[ { "id": "multiclinsum_test_1245_en.txt", "fulltext": "A 24-year-old gentleman with no past medical history had been experiencing debilitating chest pains for 2 years. He described these as varying in intensity, worsened by exercise, and relieved partially by analgesia. They were not associated with palpitations or syncope.\nUnfortunately, due to his symptoms he had to give up his studies and minimize his physical activity. He had trialled multiple analgesics (daily paracetamol and tramadol) with partial relief of symptoms.\nHe underwent serial investigations at his local cardiology unit, including a cardiac CT which identified an absence of pericardium on the left side of the heart. He was referred to the Adult Congenital Heart Disease (ACHD) team at The Royal Brompton Hospital.\nWhen first reviewed in the ACHD clinic he was not cyanosed, in sinus rhythm with no evidence of cardiovascular decompensation. His apex beat however was grossly displaced to the left and more so on lying supine. His first heart sound was normal, second split, there was also a soft one-sixth systolic heart murmur. The rest of his examination was unremarkable.\nHis electrocardiogram showed him to be in sinus rhythm at 78 b.p.m., with normal conduction times but a poor R wave progression and an intermittently changing axis.\nA chest radiography which showed situs solitus, levocardia, with a left aortic arch, no displacement of the heart to the left, and a protrusion at the level of the left atrial appendage at the left upper quadrant of the heart.\nHis echocardiogram showed a structurally normal heart with maintained ventricular function. Stress echocardiography showed mild right ventricular dilatation at rest with increase in right ventricular volume directly after exercise. A cardiac CT revealed a partial absence of pericardium on the left side with a small layer of pericardium on the upper right heart border. The heart was grossly shifted leftwards and posteriorly, highly suggestive of enhanced heart mobility. Similarly, no pericardium could be seen on cardiac magnetic resonance imaging (CMR). There was lung tissue in between the base of the heart and diaphragm , the heart was displaced to the left of the chest and the RV appeared dilated .\nThe patient’s symptoms were thought to be due to his absent pericardium and related to cardiac mobility. We were struck by the discrepancy of the erect Chest X-ray (CXR) and supine CMR with regards to cardiac position, mobility, and the severity of disabling symptoms. We thus discussed the patient at our ACHD Multidisciplinary Team meeting and offered him surgery, in an attempt to immobilize his heart, hoping to improve his symptoms and quality of life.\nThe heart was exposed via a left postero-lateral thoracotomy. There was complete pericardial agenesis on the left side and on the diaphragmatic surface. Several 0.4 mm thickness Gore-tex® patches were sutured together to create a large sheet and this was sutured down with widely spaced interrupted 3-0 Prolene® sutures to the posterior mediastinum, starting at the level of the left pulmonary artery, posterior to the left atrial appendage and along the left hilum and inferior pulmonary ligament, to the diaphragm and down. This was then brought forward to the anterior chest wall and then fixed anteriorly along the anterior chest wall with the superior end free (see Figure ). A left pleural drain was inserted and a paravertebral catheter for analgesia, followed by routine multilayered chest closure.\nThe post-operative course was uneventful apart from a small left apical pneumothorax with a small pleural effusion. Both resolved spontaneously within days.\nEchocardiography on Day 5 showed that the RV was of normal size with no evidence of pericardial effusion nor compression of cardiac chambers. The patient was discharged 8 days after his procedure on simple analgesia, mobilizing well with improved symptoms.\nThree months from surgery the patient had come off analgesia completely, with resolution of his debilitating chest pains. He was resuming his University studies and restoring normality in his life.", "summary": "A 24-year-old otherwise fit and well patient, with debilitating exertional chest pain was found to have complete pericardial agenesis on the left side and on the diaphragmatic surface. There was gross cardiac mobility demonstrated on cardiac magnetic resonance imaging. His pericardium was reconstructed surgically using Gore-tex® patches. There were no complications, and the patient was discharged 8 days later. Three months later at follow-up, the patient required no analgesia and has had complete resolution of his chest pains.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1052_en.txt", "fulltext": "A 57-year-old Greek man was referred to our facility with pain, hemorrhage and a gangrenous smell due to a so-called wound on his penis. A physical examination revealed the complete absence of his penis and a large chasm in the lower abdominal wall, which made it possible to see parts of the lower pelvis, such as the spermatic cords, the destroyed basis of the corpora cavernosa and the residual stump of the urethra. The scrotum and the testicles were stiff and were possibly invaded by the cancer. In the chasm margins, we could detect hemorrhagic and necrotic areas . The inguinal lymph nodes were palpable, hard and mobile. Our patient was in a good general condition and his body temperature was normal. From his medical history, he had discovered a lesion in his inner prepuce 18 months before. He had requested medical advice at a private health center concerning that lesion. According to his recollection, a biopsy had been taken and he was diagnosed as having penile cancer (this biopsy could not be found, as he did not ask for a copy of it at the time and the private health center failed to track our patient's data as he was never hospitalized there). The physicians at the time suggested he should undergo a partial penectomy, but he refused and stopped seeking medical treatment.\nThe lesion slowly progressed, eventually involving the whole penis. He could not specify the exact time his penis sloughed off completely. He was not circumcised. Standard laboratory test results showed that his values were within normal limits except for a small rise in white blood cell count (14,750 cells/μL) and microcellular anemia (hemoglobin = 9.8 g/dL, hematocrit = 31.2%). A chest X-ray did not show any remarkable findings. An abdominal computed tomography (CT) scan showed lymph nodes of a pathological size and number, bilateral in the iliac vessels and inguinal areas as well as an erosion of the pubic bone . We proceeded with a chest CT scan, which did not show any distant metastases or lymph nodes. On the first day of his hospitalization, we obtained biopsies from the chasm margins and identified a poorly differentiated SCC. The clinical staging was T4N3M0 and our patient was treated with chemotherapy and regional radiotherapy. We also performed a bilateral cutaneous ureterostomy, with a Gibson incision in order to protect the corroded tissues from further urine impregnation . From a combination of regional radiotherapy and bilateral cutaneous ureterostomy, total dryness of the wound was achieved. During his extended hospitalization, he presented with deep vein thrombosis in the right shin vein and seizures that were attributed to small ischemic brain strokes after a brain CT scan. Debulking and flap coverage of the wound was not considered possible, firstly because of deep vein thrombosis, epileptic seizures and his poor general condition increased the risk from operation and secondly the size of the chasm combined with very poor vascularization of the region (a topical angiography was performed). Gradually, our patient developed depression, denial of feeding and loss of weight. He died 18 months after his first admission and six months after his last follow-up admission to our clinic.", "summary": "Our case report does not concern a new manifestation of penile cancer, but an interesting presentation with clinical significance that emphasizes the need to diagnose and treat penile cancer early. It is an unusual case of a neglected penile cancer in a 57-year-old Greek man that led to auto-amputation of the penis and a large chasm in the lower abdominal wall. The clinical staging was T4N3M0 and our patient was treated with a bilateral cutaneous ureterostomy, chemotherapy and radiotherapy. Our patient died 18 months after his first admission in our clinic.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2359_en.txt", "fulltext": "A 68-year-old Caucasian male presented for surgical evaluation with a right mid-infraclavicular chest mass from referral of his primary care physician . Three years prior, the patient presented with a mass at the same location, subsequently diagnosed as a lipoma and locally excised with no surgical complications. At the time of the previous surgery, it was incidentally discovered and diagnosed that the patient also had diffuse B cell lymphoma, complete remission status post chemotherapy. Family, psychosocial, drug, and other surgical history were noncontributory. Over the past three years, the mass increased in size, but did not have any discoloration, discharge, or pain; however, due to the patient’s past medical history and immunocompromised state, the patient expressed concern and presented for elective excision of the mass.\nOn physical exam, a mass was palpated at the midclavicular line of the right upper chest, within the right first intercostal space. The mass was directly under the scar from previous excision, with no skin discoloration, telangiectasia, or visible raised nodules. The patient denied any pain, tenderness, or discharge associated with the new mass. Radiological testing was performed to determine the exact location and depth of invasion. CT chest with and without contrast revealed an ill-defined, cutaneous mass, while MRI chest was nondiagnostic.\nSurgical excision was performed under local anesthesia, with a wide, elliptical incision to the level of the underlying bony structures to ensure clean margins. Excised specimen measured 5.5 cm × 3.5 cm × 2 cm with surrounding 4 cm × 1.6 cm × 0.4 cm of skin attached. The resulting wound was closed using interrupted 4–0 plain gut sutures for the full thickness, with 3–0 Vicryl subdermal and 4–0 Monocryl subcuticular reinforcing sutures.\nThe specimen was sent to pathology for cryosectioning; however, due to increase in cellularity at the posterior margin, permanent fixation was performed. Within the submitted specimen, a subcutaneous firm nodule measuring 1.6 cm × 1 cm × 0.7 cm was located in the superior aspect with a 0.1 cm surrounding margin. Microscopic examination with hemolysin and eosin staining showed whorled myoid with spindled nuclei in storiform array throughout the dermis and extending into the subcutaneous fat with widening fibrous septa (, ). The specimen was also composed of uniform, small elongated cells and scant cytoplasm with frequent mitotic figures and giant cells. The sample stained positively for CD34 and ultimately diagnosed as DFSP .\nAs a result of the close margin excision, the patient was referred for radiation therapy with imatinib for recurrence prevention. Upon the last two follow-ups status post imatinib treatment, there have been no signs of recurrence.", "summary": "Here we present an atypical presentation of DFSP in a patient with a recurrent mass that was initially diagnosed as a lipoma, with supporting literature and clinical considerations for post-surgical management DISCUSSION: In its earlier stages, DFSP can mimic other benign or malignant dermatological pathologies, which can complicate diagnosis purely via history and physical exam. Atypical features such as prior surgical management, morphology, and location can complicate the clinical diagnosis of DFSP. Immunohistochemistry is often required for definitive diagnosis as DFSP is CD34+ and is histologically described as spindle-cells in whorled or storiform pattern, with uniform, small elongated cells.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1941_en.txt", "fulltext": "We obtained a verbal informed consent from the patient and a written informed consent from a family member of the patient for publication of this case.\nAn 83-year-old man was transported to our emergency department, with a tentative diagnosis of heat stroke. He had previously been fit and healthy, except for undergoing colorectomy for colorectal cancer 8 years ago. He also received a central venous port system, a BardPort Titanium implantable port with its and 8.0 Fr Groshong® silicone catheter (Bard Access Systems, Inc., Salt Lake, USA) which was inserted through the right internal jugular vein, for chemotherapy.\nHe was transferred to the intensive care unit, where surface cooling and intravenous hydration were performed to treat heat stroke. He made a rapid recovery, and he had become able to eat on the same day.\nNext morning, he suddenly developed ventricular fibrillation. Cardiopulmonary resuscitation was performed immediately, his trachea was intubated, and he was sedated with continuous infusion of propofol. He had not complained any chest pain before the occurrence of ventricular fibrillation, and the electrocardiography indicated neither arrythmias nor ST changes, before and after the occurrence of ventricular fibrillation. Analysis of arterial blood taken after resuscitation indicated no abnormalities in the blood gases and the blood electrolyte; troponin was not measured. A chest radiograph, which was taken after resuscitation , indicated a previously placed central venous catheter access port, without any obvious abnormalities.\nEmergency coronary angiography, performed shortly after ventricular fibrillation, identified a 90% stenosis of the left anterior descending coronary artery. The cardiologists considered that this was not the direct cause of ventricular fibrillation, as the extent to which the blood flow in the coronary artery would not bring about ventricular fibrillation. The angiography instead identified a more likely cause: a foreign body in the coronary sinus.\nClose re-examination of the chest radiograph taken after resuscitation showed a long catheter-like foreign body (approximately 20 cm) in the heart . Chest computed tomography also confirmed the presence of the foreign body . We diagnosed that a long catheter-like foreign body, likely to be a fragmented central venous catheter, was in the coronary sinus.\nIn the angiography suite, physicians attempted to remove the foreign body. However, the attempts were abandoned, as the pull of the catheter resulted in repeated ventricular fibrillation. The patient was successfully resuscitated. Surgical removal was planned the next day.\nOn the next day, the patient was transferred to an operating room, and routine monitors, such as a pulse oximeter, a blood pressure cuff, and electrocardiogram, were attached. We prepared an electric defibrillator, and cardiac surgeons were present in the room. The breathing system of an anesthesia machine was connected to the tracheal tube, and general anesthesia was induced with sevoflurane 1.5% and fentanyl 0.1 mg; neuromuscular blockade was achieved with rocuronium 50 mg. Anesthesia was maintained with sevoflurane and oxygen in air.\nThe patient underwent sternotomy, and transesophageal echocardiography identified the fractured catheter in the coronary sinus . The right atrium was opened under cardiopulmonary bypass. Surgeons could remove the fractured catheter (approximately 20 cm in length) , but with great difficulty, by gently pulling the edge of the catheter with rotating movement. After the operation, the electrocardiogram indicated no arrhythmia or cardiac ischemia.\nOn the next day of the operation, his family requested strongly to treat the coronary stenosis, and thus, cardiac physicians placed a stent in the left anterior descending artery without complications. He was discharged from the hospital on postoperative day 14, to a rehabilitation facility, and he went home at a later day.", "summary": "An 83-year-old man with heat stroke was transferred to our emergency department, where he was treated and became able to eat on the same day. He had been fit and healthy, except for colorectomy and chemotherapy using a central venous access port placed in the right upper jugular vein 8 years ago. The next day, he suddenly had ventricular fibrillation. Cardiopulmonary resuscitation was successful. Emergency coronary angiography showed a catheter-like foreign body in the coronary sinus. Physicians failed to remove the foreign body using catheter therapy, and ventricular fibrillation occurred repeatedly. After induction of general anesthesia, the fractured catheter was removed surgically. Postoperative course was uneventful.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1110_en.txt", "fulltext": "A 52-year-old female with complaints of the right knee pain and swelling was referred to us in the outpatient clinic with a diagnosis of Baker’s cyst. The pain increased on squatting and climbing stairs and was associated with swelling in the posteromedial aspect of the knee joint for the past 11 months. There was no history of the previous trauma, no intermittent locking of the knee joint, and unremarkable medical history. On examination, there was a solitary, firm, cystic, fluctuant, immobile, and slightly tender swelling on the posteromedial aspect of the right knee joint of size around 4 3 cm with no local signs of inflammation. There was joint line tenderness on the medial aspect with positive McMurray’s test for a medial meniscus tear and no ligamentous instability. Knee range of motion was 0–120 with terminal flexion painful. The plain radiograph showed evidence of early osteoarthritis of the medial compartment along with patellofemoral arthritis. MRI knee demonstrated a large well-defined cystic lesion medial to semimembranosus and semitendinosus at the posteromedial aspect of the right knee joint of size around 3.9 3.0 2.5 cm with few thin internal septations. The lesion was hyperintense on T2-weighted and fat suppression images while it was hypointense on T1 weighted image ( and ) also noted was a horizontal tear in the posterior horn and body of the medial meniscus and oblique tear in the posterior horn of the medial meniscus. The patient was posted for surgery and underwent arthroscopic partial medial meniscectomy and open excision of the cyst in its entirety along with its stalk through a medial incision under spinal anesthesia . The base of the cyst was traced and sutured with the synovial lining, thereby repairing the defect. The excised sample was sent for histopathological evaluation and found to be a cystic structure lined by flattened epithelium with walls made of fibrocollagenous tissue infiltrated sparsely with lymphonuclear infiltrate and foamy histiocytes which were consistent with a meniscal cyst. Post-operative period was uneventful and at 2-year follow-up, there was no recurrence, pain, instability, or locking of knee joint. The patient had 0–120 active, painless range of motion at the knee joint. The patient was able to return to her daily activities without any limitations.", "summary": "We report a case of a large symptomatic medial parameniscal cyst in 52-year-old female which was clinically misdiagnosed as Baker's cyst. The patient had swelling over the posteromedial aspect of the right knee with difficulty and pain on squatting. Magnetic resonance imaging (MRI) reported horizontal tear in the posterior horn of medial meniscus and parameniscal cyst adjacent to medial meniscus with features of early osteoarthritis of the knee. She was successfully treated with open excision and repair of the defect of the cyst along with arthroscopic partial medial meniscectomy.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2892_en.txt", "fulltext": "A 39-year-old woman was admitted complaining of sudden-onset headache, nausea, and vomiting for 1 day. She had a history of hypertension for 10 years and denied any history of diabetes, hyperlipidemia, autoimmune diseases, or other remarkable illnesses. She was alert and could correctly obey commands. Physical examination was unremarkable except for neck stiffness. Her blood glucose, electrolytes, blood cell counts, coagulation test, and antinuclear antibody series were within normal limits during laboratory investigations.\nHead computed tomography (CT) showed a subarachnoid hemorrhage (SAH) concentrated in the perimesencephalic cistern . CT angiography (CTA) showed that the bilateral ICAs were absent in the skull base . The bilateral MCAs and anterior arteries were normal. An aneurysm was identified in the PCA region .\nHead digital subtraction angiography (DSA) showed that the bilateral internal maxillary arteries and ascending pharyngeal arteries were confluent with the cavernous portion of the ICAs at the skull base. And the bilateral ICAs above the cavernous portion of the ICAs, the MCAs, and the anterior cerebral arteries were normal . The right vertebral artery (VA) was fine, while the left VA was well developed . Moyamoya-pattern collateral vessels could be seen in the bilateral PCAs region, the distal part of the PCA was composed of abnormally fine vessels, and an aneurysm was clearly identified in the left moyamoya-pattern collateral vessels .\nShe was diagnosed as bilateral carotid RM, moyamoya-like vessels in the PCA region, intracranial pseudoaneurysm, and SAH. Management of the aneurysm was difficult, and wait and see management was adopted. The patient experienced an uneventful recovery and was discharged 1 week later. Follow-up CT performed 44 days later showed complete resolution of the SAH . Head DSA revealed that the pseudoaneurysm disappeared spontaneously while the moyamoya-pattern collateral vessels were unchanged, the distal part of the PCAs became finer and smaller, and the transdural compensation of the posterior meningeal artery was visible . The patient was in good condition during follow-up, she returned to her normal activities.", "summary": "A 39-year-old woman was admitted complaining of sudden-onset headache, nausea, and vomiting. Further investigation revealed subarachnoid hemorrhage (SAH), carotid RM, a moyamoya collateral pattern in the PCA region, and a pseudoaneurysm in the moyamoya-like vessels. The patient was treated conservatively, recovered well and was discharged 1 week later. Follow-up angiography showed that the aneurysm had disappeared.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_51_en.txt", "fulltext": "A 67-year-old Caucasian woman with an unremarkable medical and breast history was referred to our breast unit with a screen-detected right breast mass. Mammography had revealed a fairly well-circumscribed 2 cm lesion located deep and in close proximity to the nipple and was graded as M3 on the Breast Imaging Reporting and Data System scale. Her physical examination and ultrasound showed no abnormalities. A subsequent diagnostic biopsy revealed an infiltrating ductal carcinoma with an adenoid cystic component. The patient was treated with simple mastectomy and sentinel node biopsy. The histologic examination revealed a lesion of 1.7 cm maximum diameter with mixed infiltrating NOS ductal carcinoma and ACC characteristics. The four sentinel nodes were all free of cancer. She had a good post-operative course and remained well with no evidence of recurrence 24 months later.\nThe tumor was whitish tan and was ill defined in appearance and firm in consistency. No nipple or skin involvement was present. Both the nuclear grade of the lesion and the Bloom-Richardson grade were two based on the overall appearance of the tumor. The lymph nodes were negative. The tumor was staged as T1N0M0 and was estrogen receptor (ER)- and progesterone receptor (PR)-negative. The proliferative activity was low as measured by the Ki-67 labeling method (12%). Immunohistochemically, overexpression of the proto-oncogene HER2/neu was found in 15% of the carcinomatous cells. In fluorescence in situ hybridization analysis, which was performed at a later stage, no protein amplification was ascertained.\nTwo histological patterns were blending into each other without a clear-cut boundary between them . The dominant pattern, comprising more than 70%, was an ordinary, moderately differentiated ductal carcinoma NOS. It consisted of tubule islands and cribiform structures with epithelial cells exhibiting a high nuclear-to-cytoplasmic ratio, dark nuclear chromatin, and inconspicuous nucleoli. The mitotic index was low (< 2/10 hematopoietic tissue).\nThe rest of the histological pattern was limited and mainly consisted of well-defined nests and pseudo-glandular structures occasionally filled with homogeneous basophilic (periodic acid-Schiff stain-positive) material. Interestingly, the neoplasmic nests contained predominantly basaloid cells with sparse cytoplasm coexisting with epithelial cells with more abundant eosinophilic cytoplasm .", "summary": "We describe a case of mixed adenoid cystic cancer of the breast with infiltrating ductal carcinoma in a 67-year-old Caucasian woman who underwent mastectomy with sentinel node biopsy.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1695_en.txt", "fulltext": "A 53-year-old Japanese woman with a 30-year clinical history of anorexia nervosa (155 cm, 32 kg, body mass index: 13.3 kg/m2) was found unconscious at home. On admission to the Emergency Department, she was in a coma with a consciousness level of 6 on the Glasgow Coma Scale (E3V1M2). Her other vital signs initially recorded in the Emergency Department were as follows: body temperature, 35.1°C; heart rate, 83 beats/min; blood pressure, 84/62 mmHg; respiratory rate, 12 breaths/min; and percutaneous oxygen saturation, 98% (on oxygen 10 L/min via a non-rebreather mask). A laboratory examination showed remarkable hypoglycemia with a blood sugar concentration of 8 mg/dL, anemia with a hemoglobin concentration of 8.2 g/dL, hypopotassemia with a potassium concentration of 3.4 mmol/L, thrombocytopenia with a platelet count of 128,000/μL, and coagulopathy with an international normalized ratio of the prothrombin time of 1.49 and activated partial thromboplastin time of 36.2 s. After the intravenous administration of 20 g of glucose and 20 mg of thiamine, her blood sugar concentration increased to 230 mg/dL, and her consciousness level returned to 14 on the Glasgow Coma Scale (E3V5M6). Transthoracic echocardiography showed akinesis of the heart apex and a reduced left ventricular ejection fraction of 20%, consistent with takotsubo cardiomyopathy. She was obviously emaciated and malnourished but did not have a short neck or neck deformities. A computed tomography scan showed no vascular anomaly around the neck or thorax. A diagnosis of hypoglycemia-induced takotsubo cardiomyopathy [, ] with anorexia nervosa was made. She was admitted to the intensive care unit to receive close monitoring, correction of blood sugar and electrolytes, and continuous intravenous heparin administration (5000 U/day) for the prevention of thrombus in the akinetic ventricular apex.\nOn hospital day 2, this patient suddenly developed refractory ventricular fibrillation and suffered from cardiopulmonary arrest. A rigorous resuscitation attempt using chest compressions, electrical defibrillation, endotracheal intubation, and intravenous adrenaline administration was immediately initiated by intensive care unit physicians and nurses. Spontaneous circulation was restored in approximately 15 min. A CVC was required for continuous intravenous infusion of inotropic agents and vasopressin. The right neck was then sterilized with 1% chlorhexidine digluconate, and her head was tilted to the left by approximately 30°. On a pre-procedural ultrasound (US) examination, no vascular-like structures were visible behind the posterior wall of the right IJV. The right IJV was punctured by an experienced cardiologist under US guidance with the short-axis out-of-plane technique using an 18-gauge introducer needle contained in an Arrow triple lumen central venous catheter kit (Teleflex Medical Japan, Tokyo, Japan). Although the angle and depth of the needle appeared to be appropriate if the patient had not been emaciated, the right IJV was completely collapsed at the time of the US-guided puncture, and blood was not aspirated. Non-pulsatile dark-colored blood was smoothly aspirated during gentle withdrawal of the needle, and then a guide wire, dilator, and CVC were inserted without resistance. Immediately after the CVC placement, she developed enlarging swelling around the neck. Contrast-enhanced computed tomography showed massive contrast media extravasation around the neck and mediastinum , whereas the tip of the CVC was correctly placed in the right IJV. The patient was considered at a high risk for open repair. Therefore, transarterial embolization was planned. After 6-Fr short sheath introduction (Radifocus Introducer II; Terumo, Tokyo, Japan) from her right brachial artery, a pigtail catheter (4-Fr; Cook Medical, Bloomington, IN, USA) was advanced into the brachiocephalic trunk with the support of a 0.035″ hydrophilic guidewire (Radifocus guidewire M; Terumo, Tokyo, Japan). Brachiocephalic artery angiography showed a characteristic “blush” appearance of the ruptured right thyrocervical trunk . After selective arterial embolization using a microcatheter (Masters Parkway Soft; Asahi Intecc, Aichi, Japan) and 33% N-butyl-2-cyanoacrylate, the extravasation completely disappeared and hemostasis was achieved. A schema of the operative course at the catheterization laboratory is shown in Figure S in the supplementary information file. After receiving 22 units of packed red blood cells, 20 units of fresh frozen plasma, and 20 units of platelet concentrate, the patient was returned to the intensive care unit where correction of body fluid imbalance, hypothermia, acidemia, and coagulopathy was continued. Secondary infection and multiorgan dysfunction developed approximately 2 weeks later, and the patient died from sepsis on hospital day 16. An autopsy was not performed because her family refused.\nThe search strategy was determined a priori by the survey team, which comprised anesthesiologists (YO and KS), an interventional radiologist (EU), emergency physicians (NN, IY, and JK), and a librarian (MJ, listed in the Acknowledgments). On June 2022, all reported cases of thyrocervical trunk injury associated with IJV puncture were searched for in the MEDLINE database from inception using the following keywords: “thyrocervical trunk” AND “internal jugular vein puncture”; “thyrocervical trunk” AND “central venous catheter”; “thyroid artery” AND “internal jugular vein puncture”; and “thyroid artery” AND “central venous catheter”. PubMed® was used to search the MEDLINE database. Cross-referencing was also performed using the reference list of articles included in this review. The following types of articles were excluded from the analysis: (1) they were not case reports or case letters, (2) they were not in English, and (3) they did not describe thyrocervical trunk injury associated with IJV puncture. This search produced 25 articles of which 10 relevant reports and cases were included in this review [–] . We reviewed the variables of age, sex, underlying medical conditions of the patients, number of punctures, use of real-time US guidance, characteristics of the operator, treatment, and outcome.\nThe clinical characteristics of thyrocervical trunk injury after IJV puncture described in this review, including our patient, are shown in Table . Seven articles described pseudoaneurysm of the thyrocervical trunk or its branches [–], three reported massive bleeding arising from the thyrocervical trunk or its branches [, ], and one reported CVC misplacement in the right inferior thyroid artery . The cases in the literature review consisted of four men and seven women, aged 33 to 71 years. More than 80% (9/11) of thyrocervical trunk injuries were associated with a landmark puncture without using real-time US guidance. More than half (6/11) of the thyrocervical trunk injuries were associated with multiple (≥ 2) attempts. Aneurysm or active bleeding arising from a thyrocervical trunk injury was successfully managed by surgical repair (4/11) or endovascular treatment (5/11). Endovascular embolization using 33% N-butyl-2-cyanoacrylate for the treatment of vascular complications associated with CVC placement has not been reported previously.", "summary": "A 53-year-old Japanese woman with anorexia nervosa developed refractory ventricular fibrillation. After returning spontaneous circulation, a CVC was successfully placed at the initial attempt in the right internal jugular vein using real-time ultrasound guidance. Immediately after CVC placement, she developed enlarging swelling around the neck. Contrast-enhanced computed tomography showed massive contrast media extravasation around the neck and mediastinum. Brachiocephalic artery angiography showed a \"blush\" appearance of the ruptured right thyrocervical trunk. After selective arterial embolization with 33% N-butyl-2-cyanoacrylate, the extravasation completely disappeared and hemostasis was achieved.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1012_en.txt", "fulltext": "The present case reports a 37-year-old man suffering from metastatic osteosarcoma originating in the distal part of the left femur. In March 2018, the patient entered the hospital with pain in the left leg as the major symptom. An MRI scan showed a large tumor with extramedullary parts and an intraosseous diameter of 13 cm. The histological examination of the biopsy showed a mostly epithelioid, in part osteoblastic, high-grade osteosarcoma. In the CT scans of the thorax and abdomen, there was no metastasis detectable. Before surgery, the patient was treated with a neoadjuvant regimen analog to the EURAMOS-1 trial with two cycles of doxorubicin and cisplatin and four cycles of high-dose MTX. In the intermediate staging performed by a further CT scan before surgical resection of the tumor, there was still no sign of distant metastasis. In the restaging-MRI of the left thigh the tumor showed a decrease in size. Limb saving surgical resection of the entire tumor (R0) was performed in August 2018. The tumor showed regression with 30% vital tumor cells (grade IV Salzer-Kutschnik).\nSurgery was followed by an adjuvant chemotherapy analog to the EURAMOS-1-protocol containing two cycles of doxorubicin and cisplatin, two further cycles of Doxorubicin and eight cycles of high-dose MTX. The start of adjuvant chemotherapy was delayed for two weeks because of a wound infection.\nThe final staging after the last chemotherapy cycle showed two new pulmonary metastases in the CT scan of the lung. Hence, curatively intended surgical resection was performed in April 2019.\nIn September 2019, the patient had a seizure and in an MRI of the brain multiple cerebral metastases became visible. A neurosurgical resection of a symptomatic metastasis was performed, followed by a total brain irradiation with a boost on parafalcial and occipital metastases.\nIn a systemic restaging performed by a total body FDG-PET-CT scan and an MRI of the brain, the patient then showed a rapid systemic disease-progression with metastases affecting the lung, the mediastinum, the left adrenal gland, the brain, soft tissue, bones, and the skin. (, , )\nIn a molecular testing of the most recent tissue sample of the resected brain metastases, the tumor showed a high expression of PD-L1 (TPS 90% CPS 92%) but microsatellite stability (MSS). The patient was still in a good performance state (ECOG 1). A salvage chemotherapy containing the in osteosarcoma therapy established drugs ifosfamide and etoposide was not performed because of an acute kidney failure in the patient’s history and a high amount of cumulative neurotoxicity after the total brain irradiation. Benefit-risk ratio was not considered being favorable for this option. Referring to the case of a patient with advanced osteosarcoma reported by Nuytemans et al. , who reached a stabilization of disease-progression undergoing immunotherapy with nivolumab and ipilimumab, an individual therapy attempt with the same treatment combination was conducted, as there was no further established therapy and no ongoing study available.\nStarting in December 2019, we exposed the patient to the immunotherapy combination of Nivolumab 3 mg/kg and Ipilimumab 1 mg/kg every 3 weeks for four times analog to the established treatment protocol for kidney cancer. In the following restaging performed by a PET-CT scan and an MRI of the brain 3 months after starting the therapy, the patient showed a clear response to the therapy with a profound remission of all tumor lesions (, , ). In some of the lesions, a minimally elevated uptake of FDG remained residually, whereas the lesions were not metrically measurable any more in the corresponding CT scan. In brain MRIs, minimal residual structures were interpreted as gliosis after total brain irradiation and immunotherapy. A definite distinction between inflammation or scar and minimal tumor residuals was not possible in PET-CT scans and MRIs.\nIn February 2020, the patient suffered from herpes zoster as a complication, which was treated with brivudine for 7 days.\nThe patient developed a mild facial palsy of the right side in March 2020, which can be considered as a side effect of the immunotherapy. In an examination of the cerebrospinal fluid, a slightly increased cell count of 9/nl could be detected but no signs of VZV encephalitis or meningeosis carcinomatosa, respectively.\nIn March 2020, the patient developed an immunotherapy-related pneumonitis with clinically mild symptoms but clear correlations in CT scans of the lung and noticeably reduced diffusion capacity in a subsequent lung-function examination. Therefore, immunotherapy had to be discontinued, and nivolumab maintenance could not be started according to protocol.\nFor treatment of pneumonitis, the patient received prednisolone with an initial dose of 50 mg per day (0.5 mg/kg). Because of decreasing signs of pneumonitis in control CT scans and an improving diffusion capacity in lung function, prednisolone could be quickly tapered to 7.5 mg, and re-exposure to nivolumab was feasible in June 2020. In the actual PET-CT scan and MRI of the brain, the patient still showed a profound remission of all tumor lesions, and there was no detectable sign of a relapse (, ). Currently, prednisolone is completely tapered, and the patient undergoes nivolumab maintenance (240mg) every 2 weeks. The performance state has further improved, and the patient is starting reintegration into work.\noutlines the patient’s history.", "summary": "This report presents the case of a 37-year-old patient with metastatic advanced osteosarcoma, who had no more established options for tumor treatment left. PD-L1 expression in the most recent tumor sample was high (tumor proportion score (TPS) 90%, combined positive score (CPS) 92%) but no MSI could be detected. In an individual therapy attempt, an ongoing and profound remission of all tumor manifestations due to four cycles of immunotherapy with ipilimumab and nivolumab was reached. Despite discontinuation of immunotherapy for 3 months due to therapy-related pneumonitis, remission of all tumor manifestations was ongoing, and no detectable relapse in restaging before onset of Nivolumab-maintenance could be observed.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_209_en.txt", "fulltext": "The Columbia University Medical Center IRB (AAAR0802) exempted this retrospective case report from review.\nA 36-day-old female was fussy for a day and then developed respiratory distress. She was born at term, and a New York State newborn screen was negative. In the Pediatric ER, she was tachycardic (260 beats/minute) and hypothermic (32.4 degrees C) with prolonged capillary refill and faint distal pulses. She was placed on continuous positive airway pressure via nasal prongs. Adenosine was given twice via an intraosseous line for supraventricular tachycardia with conversion to sinus rhythm. She was then intubated for worsening respiratory distress and placed on an FiO2 of 1.0. An echocardiogram was notable for decreased biventricular systolic function and left to right shunting through a patent foramen ovale. A dobutamine infusion was started. Arterial cannulation was unsuccessful; transcutaneous pulse SO2 was consistently 100% during rewarming. Results from the first blood gas (all blood gas results were reported uncorrected for temperature) drawn from a catheter placed in the IVC (by Seldinger technique; sutured in place after advancing to its full length overlying the L3 vertebral body; ) were pH 7.01, PCO2 33 mm Hg, PO2 101 mm Hg, SO2 94%, and lactate 18 mmol/L. Early during rewarming, the IVC SO2 remained above 90% and was as high as 99% ; declines in IVC SO2 and lactate during the first 3 hours of rewarming correlated (Spearman r: 0.94; p = 0.002). An arterial catheter was then placed, and the FiO2 weaned. B-type natriuretic peptide was increased (4578.5 pg/mL). Capillary refill and distal pulses improved, and dobutamine was discontinued at 8 hours; a repeat echocardiogram the next day showed improved (though still diminished) biventricular systolic function with continued left to right shunting through a patent foramen ovale. Hypothermia and hyperlactatemia resolved over 10 and 12 hours, and IVC SO2 was 80% on hospital day 2. The patient was diagnosed with supraventricular tachycardia secondary to Wolff-Parkinson-White syndrome. Prior to discharge a week later, serum acylcarnitine profile, lactate, pyruvate, thyroid stimulating hormone, and thyroxine were all normal.\nIVC PO2 values were corrected retrospectively for the patient's temperature .", "summary": "A 36-day-old female was fussy for a day and then developed respiratory distress. In the Pediatric ER, she was tachycardic (260 beats/minute) and hypothermic (32.4 degrees C) with prolonged capillary refill and faint distal pulses. Adenosine was given twice via an intraosseous line for supraventricular tachycardia, with conversion to sinus rhythm. Blood drawn from an IVC catheter was significant for uncorrected (for temperature) oxygen saturation of 94% and lactate 18 mmol/L; corrected and uncorrected IVC oxygen saturation early during rewarming were >90%. During rewarming, declines in uncorrected IVC oxygen saturation and lactate correlated. Hypothermia and hyperlactatemia resolved after 10 and 12 hours.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2314_en.txt", "fulltext": "A five-day-old full-term female baby delivered by cesarean section cried immediately at birth. The mother had no comorbid conditions and described her pregnancy as normal. During the pregnancy, the mother had an X-ray of her leg, but she was unaware of her pregnancy at the time. There is no family history of congenital anomalies, and the baby is born to parents that are unrelated. The baby was born with a trunk-like appendage that was arising from the superomedial canthus of the left eye, 26 mm × 12 mm in size, with a small tract with Cerebrospinal fluid discharge. The left nasal cavity was hypoplastic with left anophthalmia. No cleft palate, cleft lip, or choanal atresia was noted. .\nA two-dimensional Echocardiography (2D echo) revealed complex cyanotic congenital heart disease, including double outlet Right Ventricle with a large ventricular septal defect of 8 mm, malposed great vessels, mild valvular right ventricular outflow tract obstruction (RVOTO) with a pressure gradient of 26 mmHg, small Patent ductus arteriosus (PDA) with a left to right shunt and confluent branched pulmonary arteries. Two ostium Secundum atrial septal defects (ASD) were noted of 4 mm and 25 mm with a normal biventricular structure and function. On Magnetic resonance imaging (MRI) of the brain, mild hydrocephalus, bilateral colpocephaly (larger than normal occipital horns due to undeveloped white matter in the posterior cerebrum) with bilateral periventricular white matter hypodensities and corpus callosum agenesis were noted. Computed tomography (CT) imaging with a 3D reconstruction of the face revealed minor bony defects of the left nasal bone, frontal process of the maxilla, and absent nasal turbinates.Bilateral maxillary and ethmoid sinuses were underdeveloped, to a greater degree on the child's left side. CT imaging of the thorax and abdomen revealed a hemivertebra involving the T10 vertebral body causing focal scoliosis with right-sided convexity.\nOur patient’s parents deferred treatment and chose to wait till the child grows older.", "summary": "A full-term female baby delivered by cesarean section cried immediately at birth. The mother reported having a normal pregnancy but has a history of x-ray during her first trimester. The baby was born with a rare presentation of proboscis lateralis which was accompanied by multiple anomalies, including but not limited to bilateral colpocephaly, corpus callosum agenesis, complex cyanotic congenital heart disease, and hemivertebra of the T10 body.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2160_en.txt", "fulltext": "A 71-year-old female patient with thalassemia minor and hypertension was diagnosed in 1999 with early-stage right breast cancer. She underwent breast-conserving surgery and axillary dissection; pathology showed a small tumor, less than 2 cm, node-negative invasive ductal carcinoma. Hormone receptors (HRs) were positive but HER2 was negative. After the surgery, she received chemotherapy [cyclophosphamide, methotrexate, and 5-fluorouracil (CMF)] followed by radiotherapy and adjuvant tamoxifen for 5 years. She did well and was very adherent to clinical and mammographic follow-up until 2015, when she had an intractable cough. Imaging studies showed a left hilar lesion and multiple osteolytic bone metastases, including the left scapula, left shoulder, left iliac bone, and the fourth and seventh dorsal vertebrae. Fine needle aspiration from the left hilum, through endobronchial ultrasound, confirmed breast cancer metastasis. Similarly to the original tumor, hormone receptors were positive (ER 90%, PR 30%) and HER2 was negative. Therefore, she was started on endocrine therapy with fulvestrant, which kept her progression free for 2 years. She was then switched to exemestane and everolimus; however, this regimen was discontinued owing to grade II pneumonitis and prenephrotic range proteinuria. At that time, the patient was enrolled in a double-blind randomized clinical trial testing CDK4/6 inhibitors along with aromatase inhibitors. A few months later, the patient was taken off the study owing to disease progression, and was found to be on the placebo arm. Following the US Food and Drug Administration (FDA) approval of CDK4/6 inhibitors, she was started on ribociclib and letrozole, which she tolerated relatively well. Nonetheless, because of persistent neutropenia, the dose of ribociclib was reduced to 400 mg daily; on days 1–21 of a 28-day cycle.\nAlmost 20 weeks after starting ribociclib and letrozole, she noticed small, rounded, well-demarcated hypopigmented lesions over both hands with expanding patches reaching up to the mid forearms . Three weeks later, new similar lesions started to appear over her face and feet. The patient had no personal or family history of autoimmune diseases. The patient was seen at the dermatology clinic and was offered treatment with topical immunomodulators (calcineurin inhibitors), which she refused, and was kept on topical steroids with little improvement.", "summary": "A 71-year-old female patient was diagnosed initially with early-stage right breast cancer (HR+/HER2-) and was treated with breast-conserving surgery followed by chemotherapy, radiotherapy, and hormonal therapy. A few years later, she developed metastatic disease to the hilar lymph nodes, and to multiple skeletal sites, including the left scapula, left shoulder, left iliac bone, and dorsal vertebrae, for which she was treated with ribociclib and letrozole. While on treatment, she developed hypopigmented lesions involving both hands, feet, and face, which were described as vitiligo-like lesions.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2105_en.txt", "fulltext": "A 62 year old Caucasian female was hospitalized for six days for treatment of a chronic obstructive pulmonary disease (COPD) exacerbation. Management included steroids and antibiotics. Home medications included clopidogrel for coronary artery disease. Her weight was 46.58 kilograms with a BMI of 17.7 and an estimated glomerular filtration rate (eGFR) of 60.65 ml/min. During the hospitalization she was treated for DVT prophylaxis with enoxaparin 40 mg by subcutaneous injection daily.Seven days after discharge, the patient returned to the emergency department complaining of diffuse abdominal pain, increasing abdominal girth, and anterior wall bruising. An abdominal CT scan confirmed a large RSH . Both general surgery and interventional radiology were consulted. She was managed conservatively. Her hematocrit dropped from 34.8 to 25.3%; however, she declined transfusion of blood products. During the course of her stay, the patient experienced acute hypoxic respiratory failure and did not wish to be intubated. Her medical status deteriorated and, with support from her family, the patient chose to be made comfortable with minimal supportive care. She expired due to sepsis and respiratory failure two days after re-admission.\nA 79 year old male Caucasian was admitted for worsening dyspnea and management of atrial fibrillation with rapid ventricular rate and COPD exacerbation. His clinical course was further complicated by resistant gram negative and gram positive pneumonia and hypoxic respiratory failure. His weight was 59 kilograms with a BMI of 18 and initially his eGFR was 41.98 ml/min. He received subcutaneous abdominal injections of both prophylactic enoxaparin 30 mg and insulin sliding scale therapy. On day 2, his eGFR was calculated at 53.94 and his enoxaparin dose was increased to 40 mg daily.On day 5, he was noted to have abdominal pain and a noncontrast CT scan confirmed a left RSH that expanded considerably over 12 hours on follow up CT. Interventional radiology was consulted but ultimately the patient was managed conservatively with fluid resuscitation and close observation. His hematocrit dropped from 46 to 25.8%. The patient was discharged after 15 days to a long term acute care facility for further management of his respiratory failure and healthcare associated pneumonia.\nAn obese 44 year old Caucasian female with multiple chronic medical problems was readmitted to our facility from a ventilator capable extended care facility for sepsis and acute on chronic hypoxic respiratory failure. Comorbidities included spina bifida and central obesity with a BMI of 27. She was admitted on warfarin therapy for a history of recent deep vein thrombosis; however, she was subtherapeutic, with an International Normalized Ratio (INR) of 1.59. She was, therefore, treated with full dose enoxaparin 80 mg by subcutaneous injection twice daily. Her eGFR was 148.65 ml/min. Coumadin was held as the patient was treated with anitfungal and antimicrobial therapy that could prolong her INR.On day 8, she complained of abdominal pain and a CT scan confirmed a large right RSH . Repeat CT scan one day later showed extension of the hematoma and concern for possible active arterial extravasation. Both general surgery and interventional radiology were consulted. The patient underwent a failed attempt at embolization due to access complications. Due to hemorrhagic shock she required transfusion with 10 units of RBCs and pressure support ventilation in the intensive care unit. Her hematocrit dropped from 34.9 to 20.6%. Anticoagulation was reversed with fresh frozen plasma, vitamin K, and one dose of recombinant factor VIIa. The patient subsequently underwent successful embolization with thrombin and coiling of the right inferior epigastric artery by vascular surgery two days after the initial hematoma. Serial CT scans confirmed no further bleeding. After two weeks, anticoagulation therapy was reinstituted without further evidence of bleeding. The patient was ultimately discharged to a long term acute care facility for continued management of her chronic respiratory failure.", "summary": "Case number one: A 62 year old chronically ill Caucasian female develops a rectus sheath hematoma seven days after hospital discharge. The previous hospitalization included low molecular weight heparin administration for deep vein thrombosis prophylaxis. The patient ultimately chooses comfort care and expires due to sepsis and respiratory failure. Case number two: A 79 year old Caucasian male develops a rectus sheath hematoma during hospital admission where LMWH is used for deep vein thrombosis prophylaxis. He is managed conservatively; however, his hematocrit drops from 46 to 25.8%. Case number three: A 44 year old chronically ill Caucasian female is treated with therapeutic low molecular weight heparin for recent deep vein thrombosis during a hospital admission. She develops a large rectus sheath hematoma requiring embolization as well as blood transfusion.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2782_en.txt", "fulltext": "Our patient is a 70-year-old male, right-hand dominant farmer who presented at the outpatient clinic after falling on the concrete and landing on his left hand. The patient complained of pain on the ulnar side of his hand and swelling was noted on examination. No apparent deformity of the little finger was noted, finger cascade was normal, and full range of motion was elicited but with pain on motion. Ulnar nerve function was intact. Radiographs of the left hand were performed and showed no fractures or dislocations .\nThe patient denied any interval injuries but had persistent pain on the ulnar side of his left hand, prompting him to come back to the clinic 9 weeks later.\nRepeat radiographs showed complete ulno-palmar dislocation of the 5th CMCJ. A CT scan with 3-D reconstruction was also done, confirming said injury, and no associated fractures were noted .\nManual closed reduction was done under fluoroscopic guidance, but the joint was noted to be unstable when traction was removed. The patient was then indicated for open reduction of the 5th CMCJ.\nThe dislocated joint was approached from the dorsoulnar side and scar tissue was carefully removed from the 5th CMCJ . All ligaments of the 5th CMCJ were torn including the pisometacarpal ligament. Using manual longitudinal traction and dorsally directed force to the 5th metacarpal base, the 5th CMCJ was repositioned to its anatomic location. A K-wire was inserted perpendicular to the long axis of the 5th through 4th metacarpals as a temporary fixation. Thereafter, intermetacarpal fixation was performed between the 4th and 5th metacarpals using Mini TightRope® (Arthrex, FL, USA) .\nUnder C-arm guidance, 1.2-mm holes were drilled with a trajectory about 1 cm distal from and parallel to the articular surfaces of the 5th metacarpal base to the 4th metacarpal base, approximating the original location of the intermetacarpal ligament. Together with one of the stainless-steel metal buttons, a loop of the Mini TightRope® construct is passed from the ulnar to the radial hole of the 4th metacarpal. Mini TightRope® construct was then pulled to tension until the button was flush on the radial cortex of the 4th metacarpal base. The two strands of Mini TightRope® FiberWire were then passed through the radial to ulnar holes of the 5th metacarpal, and second button was then loaded into the sutures and pushed until it was flush against the ulnar cortex of the 5th metacarpal. While maintaining tension, a provisional knot is created, and range of motion and finger cascade are then checked. Once deemed acceptable, sutures were then tied for up to 5 knots over the ulnar button. Remaining strands are then cut using a blade 11 scalpel. Capsule, fascia, and skin were then closed. Immediately after surgery, the patient was allowed to use his fingers freely with unrestricted range of motion.\nK-wires were removed at 2 weeks postoperatively. Follow-up radiograph at 1 year postoperatively showed maintained proper alignment of the 5th CMCJ and the little finger had no limitation of motion. There are no plans to remove the Mini TightRope®, unless the patient begins to complain of pain related to the aforementioned hardware.", "summary": "A 70-year-old, right-hand dominant, male farmer injured his left hand when he slipped and fell on a concrete surface, landing on the ulnar side of his left hand. He was immediately seen in the clinic, just with a swollen left hand but no obvious deformity and with apparently normal PA and oblique radiographs of the hand. Nine weeks later, he came back due to persistent ulnar-sided hand pain; repeat radiographs and a CT scan of the left hand showed ulno-palmar dislocation of the fifth CMCJ. He then underwent trial closed reduction of the 5th CMCJ dislocation but failed. Open reduction, temporary K-wire fixation, and fixation using Mini TightRope® through the 4th and 5th metacarpals were done. A full range of motion of the hand was allowed immediately post-operative. Reduction was maintained and no complications were noted on subsequent follow-up visits.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_795_en.txt", "fulltext": "A 57-year-old lady with a history of diabetes, thyroid disease, dyslipidemia, and an episode of atrial fibrillation, resolved with pharmacological cardioversion several years before, presented to the Emergency Department for worsening dyspnea (New York Heart Association Classification—NYHA: IV). Her medical therapy included levothyroxine once daily, dapagliflozin 10 mg once daily, metformin 1,000 mg twice daily, rosuvastatin 5 mg, and ezetimibe 10 mg every night. She had no prior heart failure admissions. Other symptoms included orthopnea and paroxysmal nocturnal dyspnea but no chest pain or syncopal episodes. Physical examination showed a pulse rate of 95 bpm, blood pressure of 140/70 mmHg, and 97% of peripheral oxygen saturation. Cardiac auscultation revealed a pansystolic murmur of 3/6 Levine grade over the apex as well as an ejection systolic murmur over the second intercostal space at the left sternal border. There were moderate bibasal lung crepitations and mild ankles edema. Intravenous furosemide was given with resolution of the lungs and ankles edema. Her electrocardiogram showed ectopic atrial rhythm, left anterior hemiblock as well as LV hypertrophy . Laboratory tests showed troponine T of 20 pg/ml (URL of 14 pg/ml), N-terminal pro B-type natriuretic peptide (NT-proBNP) of 582 pg/ml (URL <150 pg/ml).\nA trans-thoracic echocardiogram showed a significant septal, asymmetric LV hypertrophy (basal anteroseptal wall diastolic thickness of 19 mm). Left and right ventricular systolic functions were normal. There was a clear SAM of the AML and an LVOT gradient of 56 mmHg at rest rising to 136 mmHg during the Valsalva maneuver ( and ). In addition, there was evidence of moderate to severe MR with a significant anteriorly directed jet, not very typical of SAM-related MR. A large calcification at PML-base was noted. 2D-3D trans-esophageal echocardiogram (2D-3D TEE) revealed the true MR mechanism. There was an MR combined mechanism based on: (1) PML degenerative prolapse with P2-flail from ruptured chordae with an eccentric anteriorly directed regurgitant jet and (2) a second posteriorly directed jet, related to SAM of AML ( and –). The pre-operative end-systolic mitral annular diameter was 39 mm while the end-diastolic diameter was 34 mm. There were no signs of infective endocarditis. We added beta-blockers and intravenous diuretics to pre-existing therapy and her clinical condition improved together with a significant LVOT gradient reduction (from 56 to 30 at rest). She underwent coronary-computed tomography angiography that excluded the presence of significant coronary stenoses. She was discharged and, one month later, she underwent MV surgical repair (artificial ring annuloplasty plus artificial chordae implantation) together with septal myectomy. Septal myectomy was performed according to modified Morrow’s procedure. The ruptured chorda was resected during the operation. Regarding annuloplasty, a Carpentier-Edwards Physio II ring N. 32 (Edwards Lifesciences, Irvine, CA, USA) was used. Chordal reconstruction was performed using n. 2 ePTFE Goretex neo-chordae (W.L. Gore & Associates, Flagstaff, AZ, USA). Gore-Tex chords were anchored at the papillary muscle and individually sutured to the free posterior leaflet margin. This intervention shifted the coaptation line posteriorly and contributed to fully resolving AML-SAM. The final outcome was good.", "summary": "A 57-year-old lady with a history of diabetes, dyslipidemia, and a previous single episode of atrial fibrillation (treated with pharmacological cardioversion), presented to the Emergency Department for worsening dyspnea (New York Heart Association Classification class IV). A trans-thoracic echocardiogram (TTE) showed a significant, septal, and asymmetric left ventricular hypertrophy (basal anteroseptal wall diastolic thickness of 19 mm) with normal left ventricle systolic function. A SAM of AML was evident together with a left ventricular outflow tract gradient of 56 mmHg at rest, rising to 136 mmHg during the Valsalva maneuver. In addition, there was evidence of moderate to severe mitral regurgitation (MR) with an anteriorly directed jet, not very typical of MR related to SAM. A 2D-3D trans-esophageal echocardiogram (2D-3D TEE) revealed a combined MR mechanism based on PML degenerative prolapse with P2-flail from ruptured chordae with related eccentric anteriorly directed regurgitant jet, together with a second regurgitant posteriorly directed jet, related to SAM of AML. The patient underwent MV repair together with septal myectomy, with a good final outcome.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_604_en.txt", "fulltext": "This case report has been written in accordance with the SCARE 2018 criteria .\nA 64-year-old female presented to the Emergency Department of our hospital with acute onset of a right-sided groin bulge that occurred earlier that day after doing heavy lifting. She had no significant past medical or surgical history. Her baseline functional status was exceedingly well. Her laboratory studies showed no leukocytosis or any electrolyte abnormalities. Initial radiologic studies included a transabdominal and transvaginal pelvic ultrasound, which showed a 2.4 × 2.6 cm fluid collection with some septations and absent visualized adnexal structures. A subsequent CT demonstrated a well-circumscribed 2.7 × 3.2 × 2.6 cm fluid collection containing echogenic debris. It did not have significant rim enhancement yet was noted to be most consistent with an abscess, complex seroma, or necrotic lymph node; its depth was amenable to percutaneous drainage. On further review with the radiologist, it was noted to contain the appendix, the tip of which was dilated and radiographically concerning for a cystic process, with mucocele remaining within the differential diagnosis.\nThe patient was kept nil per os, intravenous fluid resuscitation was initiated, and antibiotic therapy was begun. She was brought to the operating room for emergent surgical intervention. The patient underwent laparoscopic appendectomy with open femoral hernia repair. Intraoperatively, the appendiceal tip was incarcerated within the hernia sac . Due to the preoperative radiographic appearance and the suspicion for mucocele, extreme caution was exercised to avoid rupture and not to provide excess tension in an attempt to reduce it laparoscopically. It was easily removed through the open inguinal incision after the appendix base was divided laparoscopically . The femoral hernia was repaired in standard McVay fashion. Final pathology showed inflamed non-perforated acute appendicitis without evidence for neoplasm. The patient recovered well without notable complications and was discharged on postoperative day number two without antibiotics.", "summary": "A 64-year-old female presented to the Emergency Department of our hospital with acute onset of a right-sided groin bulge that occurred earlier that day after doing heavy lifting. Her workup revealed acute appendicitis contained within an incarcerated right femoral hernia. The patient underwent laparoscopic appendectomy with open femoral hernia repair. Intraoperatively, the appendiceal tip was incarcerated within the hernia sac. It was removed through the open inguinal incision after the appendix base was divided laparoscopically. Final pathology showed inflamed acute appendicitis without evidence for neoplasm.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1607_en.txt", "fulltext": "A previously healthy 54-year-old Caucasian man presented to the emergency department of our internal medicine ward with a medical history of aggravation of general health related to dizziness, weight gain, and two syncopal attacks. A physical examination was conducted which showed his blood pressure to be normotensive and his measured pulse rate and body temperature to be within normal limits. His initial laboratory results showed an increased hematocrit level of 69%, a hemoglobin level of 23g/dl, and a white blood cell count of 15.5×1000/μL. Initially he seemed to be hemodynamically stable with no signs of dyspnea. He experienced a rapid decrease of total proteins (5.67g/dl decreasing to 2.02g/dl within 72 hours) and began to exhibit hemodynamic instability, at which point he was admitted to our intensive care unit (ICU) and treated with catecholamines. Due to an increasing pulmonary insufficiency an endotracheal intubation was performed immediately. A massive emission of fluids and proteins from the intravascular to the extracellular compartments caused a generalized compartment syndrome to develop. Over 24 hours after admission to the ICU our patient developed compartment syndromes in both his upper and lower limbs and the abdominal compartment. The abdominal compartment syndrome was diagnosed by measuring the intra-abdominal pressure through a urinary catheter. The highest measured intra-abdominal pressure (IAP) was 26mm/Hg and therefore a diagnosis of abdominal compartment syndrome Grade IV was made. His abdomen and all four limbs required decompression by a fasciotomy of both forearms, both thighs, both lower legs, and the abdomen. The surgeries were performed 24 hours after admission to the clinic. Vacuum-assisted dressings were placed first on his lower limbs. During a second revision operation vacuum-assisted dressings were placed on his upper limbs to assist with monitoring the edema and in preparation for the definite closure of the fasciotomy wounds. The dressing of his abdomen included putting the intestine into a sac and covering it with a transparency dressing. Continuous renal replacement therapy (CRRT) was required three days after admission due to acute renal failure. Continuous venovenous hemodiafiltration (CVVHD) was applied for a total of four days. The blood levels of creatinine and urea returned to normal after three days of CVVHD and he gained back full renal function. Before CVVHD the highest creatinine level amounted to 1.4mg/dl and after renal replacement therapy (before discharge from the hospital) it decreased to 0.6mg/dl. The urea levels also decreased from 80mg/dl to 17mg/dl. The hematological parameters returned to their normal limits by the fourth day of admission . The clinical diagnostics included cultures of the blood, urine, stool, sputum, and intra-operative tissue samples which were all analyzed for aerobic and anaerobic bacteria, as well as for fungus. The results of the samples were all negative. After ruling out the differential diagnoses the diagnosis of a SCLS was confirmed, with secondary abdominal compartment and compartment syndromes in all four limbs. The secondary closure of the abdomen had been performed 16 days after admission and 23 days after admission we were able to remove the vacuum-assisted pumps and proceed in closing all wounds . His upper limbs required skin grafting . His lower limbs showed weakness in the dorsal flexion of the feet and toes, therefore peroneal splints were adjusted to his feet. His upper limbs showed residual deficits of fine motor skills, especially the left upper limb. These deficits had been improved with hand therapy. Our patient was moved to the rheumatology ward after 23 days in our ICU. He was started on medical prophylactic treatment with theophylline and terbutaline in combination with steroid therapy (prednisolone). During remission induction therapy the dose of theophylline ranged between 1200 and 1600mg per day in order to achieve serum concentrations between 20 and 25mg/dl. Before discharging him the theophylline dose was reduced to 1000mg/day. In order to obtain the remission advised to achieve peak serum concentrations between 10 and 20mg/dl, terbutaline was first given at a total dose of 20mg per day in divided doses. Before he was discharged the dose was reduced to 10mg per day. We recommended that he should continue to take theophylline and terbutaline for the rest of his life. Methylprednisolone was applied intravenously while in remission induction therapy at a dose of 40mg per day. After remission was induced the prednisolone was gradually reduced to 15mg/day. We recommended maintaining the gradual reduction of that dose.\nAfter 60 days of treatment he was discharged from the clinic. He was able to return to his previous place of work and reached the same level of athletic activity as before the illness.", "summary": "A previously healthy 54-year-old Caucasian man presented to the emergency department of our internal medicine ward with a medical history of aggravation of general health related to dizziness, weight gain, and two syncopal attacks. Due to a massive emission of fluids and proteins from the intravascular to the extracellular compartments, he developed compartment syndromes in his upper and lower limbs and the abdominal compartment. The abdomen and all four limbs required decompression by a fasciotomy of both forearms, both thighs, both lower legs, and the abdomen within 24 hours after admission. After 60 days of treatment he was dismissed from the clinic. He was able to return to his previous occupation and reached the same level of athletic activity as before the illness.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_2831_en.txt", "fulltext": "A 44-year-old woman had undergone first allogeneic cord blood transplantation (CBT, 2.35 × 106/kg nucleated cells, two locus mismatch) for acute myeloid leukemia after two courses of induction therapy that led to hematologic complete remission. The patient was pre-treated with cytarabine, cyclophosphamide, and total body irradiation conditioning. Graft-versus-host disease prophylaxis consisted of cyclosporine and methotrexate. Her medical, family, and social histories were unremarkable. The patient underwent a second CBT (2.0 × 107/kg nucleated cells) 32 days after the first due to graft failure.\nSeven days after the second CBT, she presented with high fever and shaking chills. Upon physical examination, painful induration with linear erythema along the superficial veins was observed in the peripherally inserted central catheter (PICC) site, which was inserted on the day after the second CBT. Two sets of blood samples were drawn for culture and meropenem treatment was started. The blood culture was positive on the third day of incubation (10 days after the second CBT). Gram staining of a positive blood culture revealed GPR , and vancomycin was added thereafter. Fever persisted and PICC was removed on day 11 after the second CBT (PICC was maintained for 10 days). The VITEK® 2 system (bioMérieux, Durham, NC, USA) was not able to identify the organism. Blood cultures drawn on days 10, 11, 15, and 22 after CBT were also positive for GPR. On the 14th day after the second CBT, Ziehl–Neelsen staining was performed and was found to be positive for acid-fast bacilli .\nThe nucleotide sequences were analyzed using the National Center for Biotechnology Information BLAST . The almost full-length (1442-bp) 16S rDNA gene sequence of the isolate shared 100% similarity to M. wolinskyi type strain ATCC 700010.\nIn addition to PICC removal, imipenem/cilastatin (IPM/CS), amikacin (AMK), levofloxacin (LVX), and azithromycin (AZM) were started empirically. Antibiotic susceptibilities were determined using the broth microdilution method (BrothMIC RGM®; Kyokuto, Tokyo, Japan) based on the Clinical and Laboratory Standards Institute M24 recommendations .\nAccording to the susceptibilities, IPM/CS and AZM were discontinued, and minocycline (MIN) was added. LVX was changed to moxifloxacin (MFX) based on previous case series . After 1 month of intravenous administration of AMK, the patient was discharged on an oral regimen of MFX and MIN. Although MXF had to be discontinued after 4 months due to nausea, MIN was continued for 6 months.\nAfter 3 weeks of combination antimicrobial therapy, blood cultures became negative. She showed successful engraftment by day 28. The patient responded well to therapy and no recurrence of infection was identified at 1-year follow-up.", "summary": "We present a case of CRBSI caused by Mycobacterium wolinskyi, a rare RGM, in a 44-year-old female patient who received an umbilical cord blood transplant.", "readability_score": 1, "difficulty": "hard" }, { "id": "multiclinsum_test_2546_en.txt", "fulltext": "A 73-year-old woman with pancreatic head adenocarcinoma underwent pancreatoduodenectomy (PD) at the Division of Hepato-Biliary-Pancreatic Surgery, Shizuoka Cancer Center Hospital, Shizuoka, Japan, in July 2005. The patient’s height, body weight, and body mass index were 154 cm, 56 kg, and 23.6 kg/m2, respectively. The patient was discharged 40 days postoperatively; however, approximately 1 month after discharge, she visited the hospital’s Division of Dentistry and Oral Surgery with a chief complaint of tongue pain with dysgeusia. The first examination revealed glossitis characterized by complete atrophy of the lingual papillae, which became erythematous; this is a symptom of glossitis . Furthermore, remarkable taste disorder (hypogeusia) and oral pain were reported. The angle of the mouth had stomatitis with erosive changes. The extremities showed acrodermatitis enteropathica-like eruption and abnormal keratinization . Blood test results showed hypoproteinemia and hypoalbuminemia (total protein (TP) 5.1 g/dL, albumin (ALB) 2.4 g/dL) . Examination of trace elements showed remarkably lower serum zinc and copper levels (30 μg/dL and 40 μg/dL, respectively) . We diagnosed malnutrition, dysgeusia, glossitis, angular cheilitis, and acrodermatitis enteropathica due to zinc deficiency. However, she had no obvious frequent diarrhea or steatorrhea.\nInitially, we orally administered 150 mg of Promac® granules 15% (polaprezinc, ZERIA Pharmaceutical Co., Ltd, Japan) per day (total zinc dose, 34 mg/day) to treat the zinc deficiency. However, due to the insufficient effectiveness of the replacement therapy, we additionally administered multi-trace elements (MTEs) for high-calorie infusions. Elemenmic® (Ajinomoto Co Inc, Japan) was administered intravenously, one ampule of which contained elemental iron (Fe) 35 μmol, manganese (Mn) 1 μmol, zinc (Zn) 60 μmol (= 4 mg), copper (Cu) 5 μmol, and iodine (I) 1 μmol; this was administered twice a week for 2 weeks as an outpatient treatment. However, the intravenous replacement therapy was similarly inadequate at this dosing interval and did not provide sufficient improvement in the serum copper and zinc values. The blood test results 4 months after PD were as follows: TP, 4.4 g/dL; ALB, 2.0 g/dL; Zn, 34 μg/dL; and Cu, 28 μg/dL; the patient required nutritional management during hospitalization with total parenteral nutrition (TPN) . An improvement was observed in the zinc level (99 μg/dL) and copper level (204 μg/dL) after 20 days of administering one ampule of Elemenmic® per day. Concurrently, her tongue pain and dysgeusia gradually improved. Because of a similar improvement in her nutritional status, she completed TPN (TP 5.1 g/dL, ALB 2.5 g/dL, Zn 99 μg/dL, Cu 204 μg/dL). A central venous catheter (CVC) inserted after admission was removed, and she was discharged 20 days after the second admission. On this occasion, intravenous zinc replacement therapy was discontinued.\nOne month after discharge, the serum zinc level decreased sharply, her oral pain increased again, and she had reduced food intake (Zn 35 μg/dL, Cu 44 μg/dL) . The patient was readmitted at the end of December 2005. To improve malnutrition, a CVC was re-inserted through the external jugular vein and injected with the MTE formulation and high-calorie infusions on consecutive days for 4 weeks (TP 5.9 g/dL, ALB 3.4 g/dL, Zn 75 μg/dL, Cu 47 μg/dL). In January 2006, since her oral pain and diet had improved, she was discharged from the hospital after receiving an implant of a central venous port, and she continued home self-injection of MTEs to maintain zinc levels. In February 2006, her taste function tended to improve, and in April 2006, the taste almost improved. When MTEs were self-injected daily, the serum zinc level gradually exceeded the normal range and reached 167 μg/dL in August 2006 ; therefore, the administration was switched to every other day. Subsequently, we had to confirm the blood test data repeatedly to monitor serum zinc levels to ensure that they were within the normal range . Despite continuing intravenous zinc replacement therapy, serum zinc levels decreased when additional oral zinc was discontinued in May 2007. After resuming the oral administration of Promac® due to the recurrence of dysgeusia, both the serum zinc level and dysgeusia improved. Since 2008, Pancreatin® (pancreatic enzyme, Mylan Co Inc, USA) 3 g/day had been administered alongside conventional zinc administration for this patient .\nAs of January 2012, she continued using MTEs intermittently while her serum zinc values were monitored; however, slight angular cheilitis was observed, and she exhibited no signs of glossitis and dysgeusia . Moreover, recurrence and metastasis of the primary tumor were not observed. She subsequently died of lung cancer in May 2020.", "summary": "A 73-year-old woman with glossitis, taste disorder, and acrodermatitis enteropathica-like eruption on her fingers presented to the Division of Dentistry and Oral Surgery 69 days after PD. Her serum zinc level markedly decreased to 30 μg/dL. Oral zinc administration was inadequate to treat hypozincemia after PD; therefore, multi-trace elements were injected intravenously during readmission. Her serum zinc levels recovered, and her lesions gradually improved. Furthermore, a central venous port was implanted to maintain normal serum zinc levels, and she continued self-injecting zinc at home.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_3049_en.txt", "fulltext": "A 59 year old Chinese woman underwent routine mammographic screening that revealed scattered areas of fibroglandular densities with microcalcifications developing in the lower inner quadrant of the left breast and prominent lymph nodes. Magnification mammographic views of the left breast demonstrated amorphous heterogeneous calcifications in a segmental distribution, spanning an area approximately 70 mm in maximal anterior–posterior dimension. No associated mass was identified on mammography or ultrasound. A 14 gauge (G) ultrasound-guided, spring-loaded core biopsy of the left breast lesion revealed low-to-intermediate grade ductal carcinoma in situ (DCIS) (with associated microcalcifications).\n\nThe patient was gravida 3 para 3, postmenopausal approximately 1 year previously, with no family history of breast cancer and in good health. She had no history of malignancy and was a lifetime nonsmoker.\n\nUltrasound of the left axilla showed normal morphology level I lymph nodes and one to two abnormal level II lymph nodes with focally increased cortical thickness measuring up to 5 mm and an eccentrically displaced and partially flattened fatty hilum. This clinical scenario raised the question of why this patient had enlarged nodes in the apparent presence of only DCIS.\n\nIn addition, 10 months prior to presentation, she had acute upper respiratory tract symptoms consistent with a viral illness, with a negative nasopharyngeal swab reverse transcription polymerase chain reaction test for severe acute respiratory syndrome-coronavirus-2 (SARS-CoV-2). This patient had sought medical attention after she detected swelling in her left supraclavicular fossa 7 months earlier. In addition, 7 days prior to presenting with left supraclavicular fossa swelling, she had received the first dose of the Pfizer COVID-19 vaccine in the left deltoid muscle. Just prior to her vaccination, serologic testing for total antibodies to SARS-CoV-2 nucleocapsid and spike proteins were negative.\n\nPhysical examination at the time of her presentation revealed a cluster of three soft nontender lymph nodes in the left supraclavicular fossa, each approximately 1–1.5 cm in diameter. There was no detectable cervical or axillary lymphadenopathy. Breast exam was normal, with no palpable masses. A chest X-ray revealed no abnormalities. The enlarged lymph nodes were attributed to the COVID-19 vaccine. Over the next 4 weeks, the left supraclavicular lymph nodes decreased in size and became barely palpable.\n\nInitial ultrasound-guided 25G and 22G fine needle aspiration of the level II axillary lymph node yielded insufficient lymphoid sample and was unsatisfactory for evaluation.\n\nDynamic contrast enhanced magnetic resonance imaging (MRI) was performed to further assess the breasts and lymph nodes. There was corresponding focally increased non-mass enhancement at the site of the left breast biopsy proving DCIS but no MRI evidence of grossly invasive disease. In the left axilla, there was an enhancing mass measuring 1.3 cm in diameter, deep to the pectoralis major muscle. An ultrasound-guided core biopsy of this mass was performed. Pathology revealed lymphoid tissue with interfollicular areas containing small lymphocytes, plasma cells, and rare eosinophils. No aberrant expression of CD20, CD3, BCL2, BCL6, kappa, lambda, or AE1/AE3 was identified. These histologic findings and the immunophenotype were consistent with lymphoid hyperplasia.\n\nLymphadenopathy can be caused by various etiologies, including malignancies, infections, autoimmune disorders, or miscellaneous conditions such as certain medications, foreign bodies, or vaccinations. In this case, many etiologies could be eliminated from consideration on the basis of the patient’s history, physical exam, and investigations. The patient had no known infections and no findings suggestive of an infection. She had no history of or symptoms suggestive of an autoimmune disorder. The patient was only taking hormone replacement therapy, steroid inhaler, and chronic antihypertensive medications in the 7-month period between vaccination and diagnosis of DCIS, none of which are associated with lymphadenopathy. There was no history of any implanted device or foreign body. After reviewing imaging and pathology, the clinical team concluded that the enlarged left axillary lymph nodes with reactive histology were not due to a malignancy or benign tumor. Thus the team determined by diagnosis of exclusion that the lymphadenopathy was most likely due to the prior ipsilateral upper extremity COVID-19 vaccine administration.\n\nBecause of the extent of the DCIS, the final recommendation was to do a total mastectomy of the left breast with sentinel node removal and immediate reconstruction with a deep inferior epigastric artery perforator (DIEP) flap.\n\nSurgical pathology revealed multifocal intermediate grade DCIS that was positive for estrogen receptor protein and progesterone receptor protein, with the largest area spanning 59 mm on histology. The left axilla sentinel lymph node was negative for malignancy. The patient had an uneventful recovery and received no additional treatment.", "summary": "A 59 year old Chinese woman underwent routine mammographic screening that revealed scattered areas of fibroglandular densities in the lower-inner quadrant of the left breast and abnormal lymph nodes. A core biopsy of the breast identified ductal carcinoma in situ. A biopsy of the lymph nodes was performed, and histological findings and immunophenotype were consistent with lymphoid hyperplasia. This patient had sought medical attention after she detected swelling in her left supraclavicular fossa 7 months earlier. She had received the first dose of the Pfizer coronavirus disease-2019 vaccine in the left deltoid muscle 7 days prior to presenting with left supraclavicular fossa swelling.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1066_en.txt", "fulltext": "We report the case of a 37-year-old man with a 6-month history of headaches and blurred vision. Our patient had been followed by an otorhinolaryngologist for 2 years for cervical lymphadenopathy and a right submandibular swelling. The cervical lymphadenopathy biopsy was non-diagnostic twice, showing a non-specific inflammatory disease. He had no other medical background and no personal or familiar history of an autoimmune disease.\nOn examination, he had significant swelling of the right hemi face and the neck with trismus and a decrease in the visual acuity of the right eye. The dilated fundus examination showed a right papillary paleness.\nPeripheral blood markers of inflammation were elevated. Screening for immunodeficiency and mycobacterial infections was negative.\nCerebral MRI showed a pseudotumoral lesion developing in the right pterygoid-palatine fossa spreading to the orbital and the intracranial cavity through the superior orbital fissure. The intracranial portion forms a temporal extra-axial mass mimicking a meningioma that infiltrates the lateral wall of the cavernous sinus. The lesion was strongly enhanced after the injection of gadolinium . CT scans of the chest, abdomen, and pelvis were normal.\nThe patient was operated through a pterional approach. Our first strategy was a gross total resection of the intracranial portion of the tumor. Regarding its very firm consistency, we opted for a large biopsy of the extra-axial lesion. The tumor was solid, well-delineated, and strongly adherent to the temporal lobe.\nHistological examination showed dense lymphoidplasmacytic infiltrate with storiform fibrosis [ and ]. Immunohistochemical staining revealed an increased number of IgG4-positive plasma cells . The inflammation is often focal, predominantly in a perivascular location.\nOur patient received high doses of corticosteroids (0.6 mg/kg/day) followed by progressive tapering. His neurological manifestations gradually improved and resolved after 2 months. A cerebral MRI was done 1 month after a well-conducted treatment and showed a reduction of the tumor’s size .", "summary": "We present our experience with a biopsy-proven case of IgG4-RD presenting with an intracranial extradural tumor-like mass infiltrating the temporal lobe. The patient was treated with high doses of corticosteroids followed by slow tapering. The neurological manifestations gradually improved and resolved after 2 months with a cerebral MRI showing a significant reduction in the tumoral size.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_461_en.txt", "fulltext": "A 64-year-old Caucasian woman was referred to our office for the assessment of a gigantic left neck mass . The patient reported that she had started to feel a paratracheal node 34 years previously, and that it had grown progressively during the subsequent three decades. She had undergone an operation in the area 24 years previously, although the surgery had to be terminated due to massive bleeding from the mass. During surgery, the mass was diagnosed as most likely a glomus tumor. No further surgeries to remove the mass were attempted, and it had been allowed to grow steadily.\nIn May 2013, the patient was referred elsewhere for further study of the mass. Magnetic resonance imaging (MRI) revealed a hypervascularized mass with a maximum diameter of 4 cm located at the bifurcation of the left carotid artery and causing the forward displacement of the external carotid artery and the backward displacement of the internal carotid artery. The patient refused surgery at that time and remained asymptomatic for almost 3 years. She then decided to consult a physician again due to the enlargement of the mass, which caused discomfort and mild compression symptoms when swallowing.\nIn 2016, angio MRI showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 cm of the trachea. The mass was pulsatile and slightly painful. No Horner syndrome or Hering–Breuer reflex was noted. A carotid occlusion test and a complete angiography were also performed, and these confirmed the permeability of the Willis polygon and the diagnosis of the mass.\nDue to the change in the behavior of the tumor, which had doubled in size within 3 years after remaining relatively stable for almost three decades, the size of the tumor, which had started causing compression symptoms, and the Shamblin grade II classification, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization.\nThe surgery was performed in February 2016. The tumor was approached through a wide cervical exposure after identifying the primitive carotid artery .\nThe tumor was gently dissected from the carotid bifurcation in a subadventitial plane , with great emphasis placed on maintaining hemostasis.\nThe surgeons would like to note that a partially blunt Freer periostotome is the best tool to use in high-risk areas. The vagal nerve was readily identified and separated from the mass. The hypoglossal nerve and major neck vessels were also preserved . The mass was then removed from the bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complication.\nThe postoperative phase was uneventful, and the patient was discharged 6 days after the surgery with moderate hoarseness, which was resolved within 1 month. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%.\nDue to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes. A thermoplastic mask was made to ensure that the patient remained immobilized during the treatment. A T2 MRI and computed tomography (CT) with intravenous contrast were performed to locate the above-mentioned remaining nodes of the paraganglioma. The treatment was performed separately in each affected area on two consecutive days. A coverage dose of 14 Gy and an isodose of 83% were administered using 5- and 7.5-mm collimators. The maximum dose used was 16.87 Gy. The medullary canal received a dose of less than 4 Gy, and the left VII and VIII nerves received a dose of less than 5 Gy . The treatment course was uneventful, and no complication occurred during or after treatment.\nAfter a 48-month follow-up period, no lesion recurrence or surgery-related complications have occurred. Some minor scar revision due to the tracheostomy was performed under local anesthesia. The patient is otherwise completely symptom free .", "summary": "We present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_1611_en.txt", "fulltext": "A 63-year-old female patient with a medical history of type 2 diabetes, hypertension, and hyperlipidemia visited our hospital complaining of chest, waist, and back pain radiating to both groin for 15 days. It aggravated on bending over, but there was on obvious cause. No nausea, vomiting, diarrhea, or discomfort, such as numbness or fatigue of the lower limbs, was reported. The initial treatment administered was for back pain. After admission, the patient developed a fever and abdominal pain. Peripheral leukocyte count was 12.92×109/L(normal range, 3.5–9.5×109/L) with 11.51×109/L neutrophils (normal range, 1.8–6.3×109/L) and an elevated C-reactive protein of 101.59 mg/L, and Erythrocyte sedimentation rate was 120mm/hour. The computed tomography angiography (CTA) examination revealed a thoracic aortic pseudoaneurysm. Abdominal CTA confirmed the formation of a pseudoaneurysm in the aorta, approximately in the T10-T11 vertebral body plane .\nBlood was drawn during fever, gram-negative bacilli were Gram-stained, and Salmonella paratyphi A was cultured. Susceptibility testing by the minimum inhibitory concentration (MIC) gradient method revealed that the isolate was susceptible to piperacillin/Tazobactam, Imipenem, ampicillin, and gentamicin based on the Antimicrobial Susceptibility Testing criteria (piperacillin/Tazobactam MIC≤8ug/mL; Imipenem MIC≤1ug/mL; Ampicillin MIC≤2ug/mL). Furthermore, susceptibility testing by Kirby-Bauer drug-sensitivity test showed that the isolate was susceptible to Cefotaxime, Ceftriaxone, Ciprofloxacin, and Levofloxacin based on the Antimicrobial Susceptibility Testing criteria (Cefotaxime inhibition zone diameter = 28mm; Ceftriaxone inhibition zone diameter = 30mm; Ciprofloxacin inhibition zone diameter = 26mm; Levofloxacin inhibition zone diameter = 28mm).\nThe endovascular stent-graft treatment was performed immediately. After inducing local anesthesia, the left femoral artery was punctured using the Seldinger’s method. 5F arterial sheath was implanted, and a pigtail catheter was inserted in descending aorta for aortography, which showed signs of a thoracic aortic pseudoaneurysm. An oval-shaped soft tissue-density mass measuring 2.5×2.1 cm was detected in the descending thoracic aorta at the level of the T10-T11 vertebral body. The right femoral artery was then exposed, and Ultra Stiff Wire (ASAHI, Japan) was put in. After that Hercules™ Sterile Thoracic Stent-Graft System (Microport, Shanghai) was put in along Wire and then released to cover pseudoaneurysm. The procedure lasted 45 min. Aortography was conducted again, which revealed that the pseudoaneurysm had almost disappeared without leakage.\nMoreover, piperacillin sulbactam sodium 5 g was administered once every 8 h intravenously for 14 days in combination with levofloxacin 0.4 g once a day intravenously for 7 days. Abdominal pain was relieved, and the body temperature returned to normal. The treatment was well tolerated by the patient, and she was discharged on postoperative Day 10.\nAt the 2-month follow-up, the patient was asymptomatic, and CTA confirmed the total exclusion of the pseudoaneurysm with no signs of endoleaks . After discussion with the institutional research office, formal ethical approval was deemed unnecessary if written informed consent was obtained from the patient. Verbal and written informed consent were obtained from the patient for publishing the report.", "summary": "A 63-year-old female patient had a thoracic aortic pseudoaneurysm caused by Salmonella paratyphi A infection. The patient associated with diabetes had a fever, abdominal pain, and low back pain, who was successfully treated using endovascular stents treatment and antibiotics.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_2312_en.txt", "fulltext": "A 50-year-old female patient with the main complaint of “irregular vaginal bleeding and fatigue for 6 months” visited our hospital on 12 February 2019. A mass was presented in the endometrium of the patient detected by ultrasonic examination. On pelvic MRI, a mass in the uterus involved deep muscular layer was confirmed as a 5*6 cm mass that appeared to be pelvic lymph node metastasis . Blood test showed a serous hyponatremia with Na+ level of 123∼131 mmol/L.\nThe patient received diagnostic curettage, and the pathological result showed high-grade malignant endometrial carcinoma. Tumor markers including carbohydrate antigens 125 (CA125) and human epididymis protein 4 were detected in normal ranges, and no intraepithelial or malignant lesions were found in cervical exfoliative cytology examination.\nThe uterus with enlargement in smooth and hard nature was found in the posterior position according to the gynecological examination. Hard nodules with poor activity and no tenderness can be touched in the anterior uterine wall. Based on the pathological result, endometrial carcinoma invading the serosa was diagnosed in the patient. For the huge mass in the endometrium and the poor state of patient, she received two circles of neoadjuvant chemotherapy with taxol and carboplatin, while improving the persistent hyponatremia, then staging operation of endometrial cancer was conducted under general anesthesia on 21 May 2019.\nDuring the operation, it was found that the enlarged uterus was located in the middle of the pelvis, the size was about 60 days of pregnancy, and the surface was smooth. The appearance of the main ligament and bilateral fallopian tubes and ovaries was normal, and no tumor was found on the surface of the appendix, omenta, and peritonea. The lymph nodes in the pelvis and surrounding the abdominal aorta were enlarged. First, we get the cytology of peritoneal fluid which resulted negative. And then laparotomy radical hysterectomy with bilateral salpingo-oophorectomy, and pelvic and para-aortic lymphadenectomy was performed. Incision of the uterus showed that the solid tumor of the posterior wall of the uterus with diameter of 2 cm, slightly brittle texture and pale in color protruded from the muscle wall to the uterine cavity. No lesion was found in the vaginal fornix and cervix. The normal appearance of the bilateral attachments was found, and the lymph nodes in the pelvis and surrounding the abdominal aorta were evidently enlarged.\nPathological gross examination of the tumor showed that there was no obvious tumor in the serous surface membrane of the uterus. Incision of the uterus showed that the solid tumor of the posterior wall of the uterus with diameter of 2 cm, slightly brittle texture and pale in color protruded from the muscle wall to the uterine cavity. Microscopically, the endometrium was presented with mixed carcinoma. Histologically, two kinds of carcinoma were distributed separately. And the carcinoid was distributed in the muscles of myometrium with invasive pattern, accounting for about 70% of the tumor. Diffused infiltrating carcinoma of myometrium of the uterus is neuroendocrine tumor, with no obvious cellular atypia, abundant cytoplasm, rare mitograms of 0–1/10 HPF, Ki67 index of about 2%, consistent with carcinoid, multifocal vascular and lymphatic invasion, nerve invasion. Squamous cell carcinoma is arranged in the uterine body cavity, accounting for about 30% of the tumor . There is no glandular (or tubular) structure or keratinization of the tumor cell components. The tumor has penetrated the uterine serous membrane. Multi-lesions of lymphatic vessel space infiltration were found. Of the 23 harvested lymph nodes from the pelvic, 5 metastasized lymph nodes were identified in the abdominal aortic and left pelvic lymph nodes with mixed carcinoma. Negative reaction was displayed in the cervix, bilateral ovary, and oviduct. Immunohistochemically, in carcinoid carcinoma, the tumor cells were partly positive for synaptophysin (Syn), S-100, NSE, chromogranin A (CgA), CD56, cytokeratin (CK), H-CK, P63, and P40. The progesterone receptor and the estrogen receptor were negative. CA125, P16, Napsin A, WT-1, CD99, and vimentin were negative. The Ki67 proliferation index was 10%. Elastic fibers show vascular invasion. D2–40 showed lymph node metastasis. In squamous cell carcinoma, the tumor cells were partly positive for CK, CK7, CEA, P63, P40, PAx-8, and CEA. Additionally, results of villin, CDX-2, and CK20 were all negative. The histologic features of the tumor and its immunohistochemical profile support the diagnosis of carcinoid of the endometrium with squamous cell carcinoma .\nBased on the revision of FIGO 2009 staging for endometrial carcinoma, the patient was classified as stage IIIC2. Postoperatively, the patient was given adjuvant chemotherapy with cisplatin and etoposide under interval of 3 weeks and received “sandwich” therapy of chemotherapy-radiotherapy-chemotherapy. In the first circle of chemotherapy, grade 1 nausea and vomiting and grade 3 myelosuppression were developed in the patient. The patient received radiotherapy followed by 3 circles of chemotherapy with no sign of further deterioration. A complete response was achieved in the patient on the basis of the RECIST1.1 criteria. She is currently under close follow-up with no recurrence for 4 years and 4 months after surgery.", "summary": "Here, we report an interesting case of mixed carcinoma of endometrium with both carcinoid and squamous cell carcinoma, which presented with persistent vaginal bleeding and hyponatremia.", "readability_score": 1, "difficulty": "hard" }, { "id": "multiclinsum_test_1331_en.txt", "fulltext": "A 55-year-old male patient was presented to a general hospital with fever, myalgia, and persistent cough. Polymerase chain reaction testing for COVID-19 was positive and he was admitted to the hospital due to severe respiratory symptoms. The patient is a known diabetic and hypertensive with a history of a nonfunctioning pituitary macroadenoma for which he underwent an endoscopic endonasal transsphenoidal resection 11 years ago in another hospital. Pituitary tumor recurrence was documented in the available two postoperative magnetic resonance imaging (MRI) studies . After surgery, the patient was kept on maintenance levothyroxine and hydrocortisone for panhypopituitarism.\nSix days after admission, the patient complained of severe headache and acute onset of ptosis and diminution of vision in the left eye. Ophthalmological evaluation revealed complete visual loss in the left eye, a visual acuity of 20/60 in the right eye, left oculomotor nerve palsy with ptosis, and dilated nonreactive pupil. Fundus examination revealed a pale left optic nerve head. Neurological examination was otherwise normal. Computed tomography revealed hyperdensity of the sellar and suprasellar areas. MRI was consistent with PA in a recurrent large pituitary adenoma [ and ].\nThe patient was transferred to our center and underwent an urgent endoscopic endonasal transsphenoidal surgery through which a near total excision of the tumor was achieved [ and ].\nThe procedure was performed under strict COVID-19 precautions including use of powered air purifying respirators and limited OR personnel. Intraoperative findings were similar to those usually seen in cases of PA and included dark blood mixed with purple-red adenoma tissues of variable consistencies . It was our observation that the nasal mucosa was pale and separated easily from the underlying bone. We have previously observed such features in two patients who previously tested positive for COVID-19. Histopathological examination demonstrated classic features of PA.\nWithin the first 3 postoperative days, the patient had some improvement of vision of the left eye so that he was able to perceive hand movement. Ptosis also improved partially. However, his chest condition progressed and he had to be transferred to COVID-19 intensive care unit in the referring hospital where he was intubated and put on mechanical ventilation. One week later, the patient unfortunately passed away due to complications of severe COVID-19 pneumonia.", "summary": "A 55-year-old male patient with confirmed COVID-19 infection presented by progressive decrease in visual acuity and oculomotor nerve palsy. His medical history is notable for diabetes mellitus, hypertension, and pituitary macroadenoma resection 11 years ago. He was on hormonal replacement therapy for panhypopituitarism that complicated the surgery. Previous magnetic resonance (MR) imaging studies were consistent with enlarging residual pituitary adenoma. During the current hospitalization, computed tomography revealed hyperdensity of the sellar and suprasellar areas. MR imaging revealed PA in a recurrent large adenoma. Endoscopic endonasal transsphenoidal resection was uneventfully undertaken with near total excision of the adenoma and partial improvement of visual loss and oculomotor palsy. Histopathological examination demonstrated classic features of PA. However, his chest condition progressed and he had to be transferred to COVID-19 intensive care unit in the referring hospital where he was intubated and put on mechanical ventilation. One week later, the patient unfortunately passed away due to complications of severe COVID-19 pneumonia.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_844_en.txt", "fulltext": "A 28-year-old Asian female presented to accident and emergency department with 6 weeks history of intermittent left sided abdominal pain, which got worse over last 48 hours. Abdominal pain was associated with 2 episodes of watery diarrhea not mixed with blood and several episodes of vomiting. Drug history included nothing else apart from oral contraceptive pill. On examination she was comfortable and afebrile with pulse rate of 96 and blood pressure of 160/96. Abdomen was soft but tender in left iliac fossa and left lumber region with no guarding or rebound tenderness. Digital rectal examination was unremarkable. Full blood count showed white cell count to be elevated at 24.5 × 106/L. Urea, electrolyte, amylase and liver function tests were all within normal limits. Flexible sigmoidoscopic examination up to distal descending colon did not reveal any abnormality. Computer tomographic scan of the abdomen showed dilated large bowel up to splenic flexure along with dilated loops of small bowel. She was started on supportive treatment with IV Normal Saline, O2 inhalation, catheterization and antibiotics. However after treatment she failed to respond and progressively became more unwell. Gradually she developed hypotension and oliguria. Arterial blood gas analysis at 4 litre of oxygen showed compensated acidosis with following picture:\nPo2-10.7 kPa\nPCO2-4.04 kPa\nHydrogen ion - 43.4 nmol/litre\nHCO3-17.5 nmol/litre\nOn reassessment of abdomen it was more tender with both guarding and rebound tenderness. She underwent laparotomy which showed complete infarction of small bowel, Gall bladder and spleen. Large bowel was infracted up to splenic flexure. Liver also appeared ischaemic. No procedure could be carried out and abdomen was closed. She subsequently died after around six hours in the post operative period. An autopsy was requested in view of operative findings. Post mortem examination confirmed the presence of organized thrombus at the origin of celiac and superior mesenteric arteries. In the aorta there was eccentric intimal thickening with loss of smooth muscle and a proliferation of elastic tissue [Figure ]. The nature of the lesion was confirmed as intimal fibro muscular dysplasia and thrombosis causing stenosis and subsequent occlusion of the origin of superior mesenteric and celiac artery [Figure ]. Intimal fibro muscular dysplasia of aorta causing stenosis of the origin of superior mesenteric and celiac artery was rare in medical literature.", "summary": "A 28 year old Asian female presented with acute onset left sided abdominal pain and watery diarrhea. She had a laparotomy due to further deterioration. It showed infracted small intestine, gall bladder and parts of liver. Abdomen had to be closed without any therapeutic procedure. She died in early post operative period. Autopsy showed fibro muscular dysplasia of superior mesenteric artery.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2955_en.txt", "fulltext": "We present a 30-month-old male child with a normal perinatal history who was referred to our center with a history of decreased sensation over the gluteal region along with persistent dribbling of urine for 6 months. Neurological examination was normal, except for decreased perianal sensations. He was able to walk but at a slower pace than before; however, the exact power could not be graded due to the age factor limiting communication. Magnetic resonance imaging (MRI) of the spine showed a well-defined intramedullary cystic lesion of size 12 × 8 × 8 mm in the conus medullaris at the T12-L1 level, which was hypointense on T1-weighted images and hyperintense on T2-weighted images with no perilesional edema, solid component, or contrast enhancement .\nThe patient underwent T12-L1 laminotomy under intraoperative monitoring. After a midline durotomy, the cyst was seen surfacing near the midline covered by a thin layer of gliotic tissue. The cyst was opened at its most superficial point and clear fluid akin to cerebrospinal fluid was drained. Repeated Valsalva maneuver confirmed that the central canal was separate from the cyst. There was no definite plane of dissection between the conus and the cyst. The cyst was marsupialized by suturing its wall to the arachnoid of the cord to prevent reclosure . This was followed by a watertight dural closure. The postoperative period was uneventful. On follow-up after 3 months, there was an increase in muscle strength with improved walking but dribbling continued to persist. Histopathological examination revealed arachnoid cells in the cyst wall along with glial tissue which was suggestive of an arachnoid cyst.", "summary": "We report a case of a 30-month-old child who presented with decreased gluteal sensation and urinary dribbling for 6 months. Apart from some slowness in walking, the power was normal in all four limbs. Imaging showed a non-enhancing, T2-weighted hyperintense 12 × 8 mm conus intramedullary cyst without any edema. A T12-L1 laminotomy followed by marsupialization of the cyst was done. Histopathology was suggestive of an arachnoid cyst. The postoperative course was uneventful with improvement in muscle strength and achievement of regular milestones. We also present the pertinent review of the literature to date.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1738_en.txt", "fulltext": "A 40 year old Malay male was seen at the emergency department with 1 week history of left hypochondriac pain with concurrent abdominal distention. He also complained of loss of appetite and feeling lethargic for 1 month duration. He had no fever, nausea, vomiting, changes in bowel habits or any history of bleeding diathesis. There was no history of trauma. Neither there were any significant past medical history nor family history of malignancy. He was an active smoker for 20 years but denied any alcohol consumption or substance abuse.\nOn clinical examination, he was afebrile, with an elevated heart rate of 110 beats per minute and a blood pressure measurement of 121/79 mmHg. Patient appeared pale. Abdominal examination revealed enlarged, non-tender liver and spleen. There was no ascites or peripheral lymphadenopathy. Cardiovascular and respiratory examinations were otherwise unremarkable.\nHaematological investigation revealed a low haemoglobin level at 6.4 g/dl. The patient had a white cell count (WCC) of 33.3 × 10^3 /uL and a platelet count of 568 × 10^3/uL. Differential WCC showed a predominant neutrophil count of 79.9%, lymphocyte count 8.9%, monocytes 9.6%, eosinophils 0.8%, basophils 0.8%, absolute neutrophil count of 25.63 × 10^3 /uL and absolute lymphocyte count of 2.95 × 10^3 /uL. There was an increase in lactate dehydrogenase levels (LDH) from 534 to 666 u/L. Peripheral blood film revealed leucocytosis with neutrophilia with no evidence of blast cells or atypical lymphocytes. Patient was reluctant to undergo a bone marrow aspiration and trephine biopsy. Abdominal ultrasonography demonstrated a large splenic collection. A contrast enhanced computerized tomography of the abdomen further revealed a large heterogenous splenic collection measuring 18 cm × 15 cm × 16.9 cm which was suggestive of a splenic haematoma [Fig. , and ]. There were no intra abdominal or pelvic lymph nodes enlargement. Based on computed tomography findings, a preliminary diagnosis of spontaneous splenic rupture was made. A surgical consult was obtained and an explorative laparotomy was performed on the patient. Intra operative findings showed a ruptured spleen with extensive adhesions to the omentum. No intra peritoneal lymph nodes enlargement were found. Splenectomy was then performed and subsequently, the patient was transferred to intensive care unit for close observation.\nFrom a histological perspective, the gross appearance of the obtained specimen revealed an enlarged spleen with irregular outer surfaces. A cut section of the spleen showed a firm, cream coloured layer occupying almost entire spleen with large area of necrosis with splenic infarcts. There as minimal amount of normal looking parenchyma tissues at the peripheral aspect of the specimen. Further histological examination revealed a diffuse infiltration of malignant lymphoid cells, which exhibited irregular nuclear membrane with vesicular nuclear chromatin and prominent nucleoli. The adjacent splenic parenchyma showed a congested and expanded red pulp with infiltration by atypical lymphoid cells [Fig. ].\nThe histological report confirmed the presence of diffuse large B-cell non-Hodgkin’s lymphoma (NHL) via immunohistochemical testing. Immunohistochemical staining showed the cells to be positive to CD20 (diffuse), BCL-2, BCL-6 (> 30%), and MUM-1 (> 30%) and negative to CD3, CD10, Cyclin-D1, Tdt, CD30 and ALK. Ki67 proliferative index was > 80%. In accordance with the WHO classification of Lymphoid Neoplasm [, ], these findings were consistent with the diagnosis of a diffuse large B-cell non-Hodgkin’s lymphoma, non-germinal center B-cell (non-GCB) type. Further molecular studies to assess MYC / BCL-2 / BCL-6 translocation or rearrangement was not done as the fluorescence in situ hybridization (FISH) analysis was not available in our local laboratory settings. Thirteen days later, the patient was discharged with prophylactic meningococcal, pneumococcal and influenza vaccinations. He was referred to the haemato-oncologist outpatient clinic at a tertiary care centre for post - operative chemotherapy. Unfortunately, the patient did not turn up for subsequent follow ups, rendering it difficult to further document any information with regards to treatment response in this report.", "summary": "A 40-year-old man presented with 1 week history of left hypochondriac pain associated with abdominal distention. There was no history of preceding trauma or fever. Clinical examination revealed signs of tachycardia, pallor and splenomegaly. He had no evidence of peripheral stigmata of chronic liver disease. In addition, haematological investigation showed anemia with leucocytosis and raised levels of lactate dehydrogenase enzyme. However, peripheral blood film revealed no evidence of any blast or atypical cells. In view of these findings, imaging via ultrasound and computed tomography of the abdomen was performed. The results of these imaging tests showed splenic collections that was suggestive of splenic rupture and hematoma. Patient underwent emergency splenectomy and the histopathological report confirmed the diagnosis as DLBCL.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2265_en.txt", "fulltext": "A 70-year-old male with a history of hypertension, diabetes mellitus, and atrial fibrillation presented to the emergency department with chest pain lasting for 1 hour. The patient did not have any previous neurologic deficits or surgical, family, or genetic history, although he was a heavy smoker (75 pack-years). His initial blood pressure was 109/62 mmHg with a heart rate of 103 beats per minute. There were no remarkable findings upon physical exam. The electrocardiogram (ECG) showed ST segment elevation in leads II, III, and aVF and reciprocal changes in leads I and aVL, suggestive of ST elevation myocardial infarction. Initial troponin I level (0.021 ng/mL [0–5 ng/mL]) was unremarkable. The patient was started on intravenous unfractionated heparin (UFH).\nThe patient was immediately moved to the catheterization lab for emergent percutaneous coronary intervention (PCI) of ST elevation myocardial infarction. Vital signs were normal during the femoral artery puncture and insertion of a 6 French (Fr) sheath into the right femoral artery (FA). We punctured at the femoral head level without any mispuncutures during the process. After puncture and before coronary angiography (CAG), however, the patient went into ventricular fibrillation and the blood pressure became unmeasurable. Cardiopulmonary resuscitation (CPR) was immediately initiated with defibrillation every 2 min, although normal rhythm of vital signs were not recovered. As CPR was performed for a total of 30 min, we concurrently inserted a venoarterial ECMO. A 16.5 Fr (external diameter: 5.5 mm) arterial and 21 Fr (external diameter: 7 mm) venous cannula was inserted through the right FA and femoral vein (FV), respectively. CAG revealed an extensive thrombotic occlusion of the mid right coronary artery (RCA) with thrombolysis in myocardial infarction (TIMI) 0 flow distally. A drug eluting stent (Biomime™ 4.0 × 19 mm) was inserted to the lesion and the RCA restored a TIMI 2 flow (pain-to-balloon time: 120 min).\nAfter the PCI, the patient was moved to the intensive care unit for ECMO care. Initial echocardiography showed a left ventricular ejection fraction (LVEF) of 15% and an extensive regional wall motion abnormality of the RCA territory. However, there was blood pressure drop with concurrent massive nasal and gastrointestinal bleeding. Hemoglobin level became 5.9 g/dL, which was approximately 8 g/dL decrease compared with initial levels. Blood pressure was recovered to normal after massive transfusion; however, we lowered the target activated partial thromboplastin time to approximately 40 s to prevent additional bleeding. There were no cannulation site complications such as hematoma or signs of infection during ECMO care, although diffuse swelling developed in the right lower extremity (LE) the next few days. Pneumatic compression devices were applied to both LEs to prevent DVT. ECMO therapy was maintained for 4 days, while vital signs slowly recovered. As the LVEF was restored to 46% on the 4th day, the ECMO was weaned and removed. The ECMO cannulas were removed by manual compression. Despite the risk of stent thrombosis, dual-antiplatelet therapy was stopped after ECMO removal (day 4) in concern of additional bleeding.\nOn the 7th day of admission, the patient recovered orientation. However, leg edema did not improve despite ECMO removal. He also started complaining impaired function, pain, and hypesthesia of the LE. Compartment syndrome was initially suspected. We clinically ruled out compartment syndrome , since the patient was negative for Homan’s sign and had good distal pulsation with warm circulation. The patient had burning sensation and Grade 3 weakness in hip and knee flexion motions with no deep tendon reflex. Neurologic findings were not dermatome specific. LE Doppler sonography and computed tomography (CT) showed DVT extending from the right external iliac vein to the calf vein without any evidence of puncture site hematoma or intramural bleeding . The patient was started on rivaroxaban (15 mg twice daily) for DVT (day 8). The swelling of the right LE gradually improved over the next 30 days, although his pain and weakness were not alleviated. As the patient was suspected to have post-thrombotic syndrome (PTS), which is pain and abnormal sensation after the onset of DVT, further studies such as the nerve conduction study (NCS) or electromyography were not considered until later on. The patient was discharged to a rehabilitation hospital on the 35th day.\nDuring outpatient department follow up, the patient consistently had pain, abnormal sensation, and weakness in the right LE, which resulted in insomnia and depression. Although he was given thioctic acid (600 mg qd) and pregabalin (150 mg bid) during outpatient department follow up, his pain remained. Femoral and pulmonary arterial CT angiography was performed 100 days post-ECMO insertion showed no thrombus in the right LE or pulmonary arteries. However, the NCS revealed no sensory nerve action potential in the right peroneal nerve and tibial nerve, suggestive of impaired sensation. Additionally, the compound muscle action potential was not observed in the right femoral , peroneal and tibial nerve, indicating motor nerve palsy. These results collectively suggested FN. The patient is continuing rehabilitation exercises and slowly recovering from the weakness, although the tingling sensation remains to a lesser degree. The patient shares his regrets on his previous smoking habits which caused his myocardial infarction and eventually FN. An informed consent for publishing data was obtained from the patient. A timeline of events is summarized in Table .", "summary": "A 70-year old male presented to the emergency department for chest pain. The patient had cardiac arrest before percutaneous coronary intervention (PCI) and was inserted with ECMO. Although he was successfully weaned from ECMO 4 days after PCI, he consistently complained swelling, abnormal sensation, and weakness in his right lower extremity, where the cannulas were inserted. Imaging studies showed deep vein thrombosis (DVT) in his right leg, which was further treated with anticoagulants. Symptoms, however, remained after the regression of DVT. Nerve conduction study revealed femoral neuropathy, which may have been caused by ECMO cannula compression and tissue swelling.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1914_en.txt", "fulltext": "A 43-year-old woman was referred to our institution with a neck mass in the left submandibular region. She was studied in an outside hospital, where a neck ultrasound showed a multinodular goiter and a CBT on the left carotid bifurcation. A neck Computed tomography angiography (CTA) demonstrated a carotid body tumor (3.8 cm × 2.5 cm × 3.3 cm) classified as Shamblin II and an intrathoracic multinodular thyroid gland with a right lobe extension of 11.9 cm × 9.7 cm and the left lobe of 25.2 cm × 21.3 cm with caudal retrosternal growth and evidence of slightly trachea deviation .\nThyroid function test (TFT) were normal, serum antithyroglobulin antibodies and thyroperoxidase antibody (TPO) were undetectable. Thyroid gammagram was performed reporting a multinodular goiter. Fine needle aspiration was performed demonstrating nonmalignant cells. A total thyroidectomy was performed with a transverse lower neck incision (Kocher incision), posteriorly, CBT was resected by an extension of the previous Kocher incision to the anterior border of the sternocleidomastoid muscle using the retrocarotid reported previously in our group as an effective technique, also two surrounding lymph nodes were resected to rule out malignancy . The pathology report demonstrated a paraganglioma with negative lymph nodes invasion and a multinodular goiter .\nThe patient recovered satisfactorily after the surgery and she was discharged in postoperative day number three, she currently continues her follow-up as our patient. Twelve months after the surgery, she does not present any complaints. She is currently on levothyroxine and calcium carbonate.", "summary": "We herein present the case of a 43-year old woman with the aforementioned association. As a part of the diagnostic evaluation, an ultrasound was performed, revealing both conditions simultaneously. The surgery took place with the aim of resecting both lesions during the same intervention. The patient was discharged without complications.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2033_en.txt", "fulltext": "A 66-year-old female had complained of intermittent discomfort and pain in the right gluteal area. She had also noticed an alteration in bowel habit with recent constipation. The symptoms persisted, and she was treated with analgesia and laxatives.\nHowever, there was no specific diagnosis until she fell onto her back and fractured L2, L3 and L4 vertebrae which necessitated further imaging work-up with CT scan of the thoracolumbar spine. Of note, the fractures were stable and non-displaced. Hence, no need for surgical intervention. The CT scan performed to assess the spine revealed an incidental finding of a large mass extending from the right gluteal region into the pelvis. A CT scan of the pelvis was also performed, and this showed a large fatty intramuscular lesion extending into the pelvis through the greater sciatic notch . The differential diagnosis was listed as a possible lipoma or low-grade liposarcoma. As part of the diagnostic work-up for this right intramuscular gluteal mass with intrapelvic extension, the patient underwent MRI of the abdomen and pelvis. There was no suspicious soft tissue abnormality detected on the MRI of the abdomen but the MRI of the pelvis showed a large homogenous fat lesion noted in the right pelvic side wall between the gluteus maximus and gluteus medius muscles which extended into the pelvis via the greater sciatic foramen . There was no enhancing component. No heterogeneity or thickened or irregular septations were detected. The margins were distinct. The mass measures approximately 18.9 cm × 13 cm × 22.8 cm. The intrapelvic component of the mass measures approximately 10.5 cm × 6.2 cm × 12.1 cm. There was a leftward displacement of the rectum noticed as a result of the mass effect from the central pelvic mass .\nPhysical examination revealed an area of firm induration/possible mass in the lower aspect of the right gluteal compartment. This was not tender and there was no associated redness or discoloration of overlying skin. Digital rectal examination showed normal anal sphincter tone and no mass was palpated within the anorectum.\nThe patient proceeded to have a CT guided core needle biopsy of the mass. This showed scant fragments of fibroadipose and fibroconnective tissue without features of malignancy. There was focal myxoid change. However, the cytologic atypia that would warrant a diagnosis of atypical lipomatous tumor/well differentiated liposarcoma was absent. Furthermore, FISH for MDM2 amplification was attempted, but could not be performed due to sample inadequacy. The case was presented for discussion at the multidisciplinary tumor conference and the consensus recommendation was to proceed with surgical resection of the tumor. Following this, the patient underwent wide excision of the mass through a transgluteal approach and final pathology showed well-differentiated liposarcoma .\nRegarding follow up, the patient has been undergoing surveillance for 3 years. She was seen every 3 months for the first year after resection. Computerized tomographic scans of the chest, abdomen and pelvis were done every 6 months for the first year of surveillance. The patient was seen twice a year for the following 2 years. Computerized tomographic scans of the chest, abdomen and pelvis were done annually for years 2 and 3 of surveillance. The patient has remained disease-free; that is no evidence of local or distant recurrence of the liposarcoma or the sciatic hernia.", "summary": "A 66-year-old female presented with persistent pain in the right gluteal region. Physical examination coupled with CT/MRI scans identified a firm mass extending into the right pelvic side via the sciatic foramen. It measured approximately 18.9 cm × 13 cm × 22.8 cm. The tumor was resected via transgluteal approach. Immunohistochemical and microscopic features were consistent with well-differentiated liposarcoma.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_1204_en.txt", "fulltext": "A 66 year old woman with floor of mouth squamous cell carcinoma (SCC) presented to our institution after her second relapse. Originally diagnosed in 2006, she had undergone a composite resection with a flap reconstruction and bilateral neck dissections followed by post-surgical adjuvant radiotherapy for stage IVa (T4aN0M0) disease. Immunohistochemistry (IHC) staining for p16 was negative. In May of 2009, a resectable locoregional recurrence was detected and consequently treated with a composite resection utilizing a pectoralis flap reconstruction. In November of 2013, she presented with a second non-resectable locoregional relapse. She received carboplatin and paclitaxel for 4 cycles with a partial response (PR) after 2 cycles. The patient subsequently developed regional progression and was treated with weekly methotrexate and cetuximab and she achieved stable disease (SD) for 6 months. Later, she progressed locally and was enrolled into a trial utilizing single agent pembrolizumab. She had SD for 6 cycles , and then suffered from local progression with a significant increase in the size of her neck mass, with painful ulceration and bleeding. Pembrolizumab was therefore discontinued. At this time restaging studies revealed no evidence of distant metastasis. She required multiple transfusions secondary to tumor hemorrhage and as a result was treated palliatively with radiation therapy to a total dose of 30 Gy directed at the mass. The patient experienced an excellent clinical response. Bleeding had resolved and her pain had greatly improved. A significant radiographic response was also noted on computed tomography (CT) scan, with tumor dimensions decreasing by 60 %, from 7.1 × 7.2 cm pre-radiation, to 5.9 × 3.4 cm, 6 weeks post-radiation.", "summary": "We report a case of locally relapsed non-resectable oral cavity squamous cell carcinoma, with excellent local control after pembrolizumab (MK3475) followed by radiotherapy.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2229_en.txt", "fulltext": "A 44 year old Caucasian male was referred by his primary care physician for a recurrent asymptomatic left sided massive pleural effusion. The pleural effusion was first discovered on a chest X-ray obtained to follow-up his left 8th, 9th and 10th rib fractures from an accidental fall. The effusion was recurrent over one and a half month even after drainage with thoracentesis and chest tube placement . The initial chest X-ray immediately after his fall that revealed the acute fractures did not show any pleural effusion or blunting of costophrenic angles . The patient had no significant past medical history, denied recent travel, but admitted to smoking tobacco and consuming alcohol. The patient worked, as a maintenance man, but at the time of presentation was unemployed.\nAt presentation the patient had normal vital signs with an oxygen saturation of 93% on room air. Physical examination revealed an average built man in no apparent distress, non-labored breathing, central trachea, absent breath sounds with a stony dull percussion note on the left hemi thorax. Abdomen was soft and non tender with a palpable liver 10 cm below the costal margin. Laboratory investigation revealed mild anemia, hypoalbuminemia (3.1 gm/dL), normal BUN, creatinine, electrolytes and urine analysis. Arterial blood gas (ABG) analysis showed pH 7.48, PaCO2 34 mm Hg, PaO2 63 mm Hg, HCO3 25 mEq/L, oxygen saturation 93% on room air. Pleural fluid study showed lactate dehydrogenase (LDH) 112 U/L, pH 7.80, WBC 76, Polymorphs 40%, Eosinophil 1%, glucose 139 mg/dL, albumin 0.90 gm/dL, RBC 1300/mm3, amylase 169 U/L, pleural fluid – serum LDH ratio was 0.48. Pleural fluid gram stain, cultures (aerobic, anaerobic, AFB, fungal) and cytology were all negative. Computerized tomography of chest showed the massive left-sided unilateral pleural effusion with complete collapse of the left upper and lower lobes, but no pulmonary embolism or malignancy. . Echocardiogram was normal. Ultrasound of abdomen was remarkable for fatty liver, but no ascites. Magnetic resonance cholangio-pancreatography (MRCP) showed normal pancreas and biliary system without ascites. Tuberculin skin test, ANA, ANCA, AMA, ceruloplasmin, TSH, HIV, RPR, and Hepatitis virus serology were normal. Due to frequent massive re-accumulation a diagnostic and therapeutic video assisted thoracoscopy (VATS) procedure was performed, which revealed a 2 cm diaphragmatic hernia with inflamed, friable, incarcerated omentum and small amount of bowel. This inflamed omentum was determined to be the etiology for the recurrent pleural effusion. About 3.5 liters of pleural fluid was removed by VATS. Histopathology was consistent with moderately inflamed omentum. Hernia was repaired and talc-pleurodesis was performed. There was no re-accumulation on follow-up over 2 years .", "summary": "A 44 year old male presented with recurrent pleural effusions over six weeks. His pleural effusion was first diagnosed incidentally on a chest X-ray after a fall. Extensive diagnostic studies were unyielding for the etiology of the effusion. A diagnostic and therapeutic video assisted thoracoscopy revealed a diaphragmatic hernia with inflamed, incarcerated omentum. After hernia repair there was no recurrence.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2563_en.txt", "fulltext": "A 54-year-old woman presented at our hospital with left flank discomfort and palpebral edema of 1-week duration. She underwent abdominal postcontrast computed tomography (CT) in a local hospital, which showed a low-density renal pelvic mass and hydronephrosis of the left kidney and indicated renal pelvic cancer (Figure ). Her past medical history included sicca complex for 5 years previously. In her laboratory examination, a routine urine test revealed a red blood cell count of 118.4/μl (normal reference range, 0 to 25/μl), a white blood cell count of 127.3/μl (normal reference range, 0 to 25/μl) and an epithelial cell count of 13.4/μl (normal reference range, 2 to 10/μl). No remarkable findings in the complete blood count or urine cytology were observed. A retrograde pyelogram showed a dilated left renal pelvis and stricture of the upper ureter, which had a regular surface and a filling defect (Figure ). On postcontrast magnetic resonance imaging (MRI) scans, the wall of the ureteropelvic junction was irregularly thickened and showed isointensity on T1-weighted images and hypointensity on T2-weighted images. On both T1- and T2-weighted images, the thickened wall of ureteropelvic junction showed homogeneous enhancement. Furthermore, multiple enlarged retroperitoneal lymph nodes were visualized by MRI (Figure ). Positron emission tomography/CT findings indicated that the renal pelvic mass was a malignant tumor, because the glucose metabolism was very high (Figure ). PET/CT also revealed multiple enlarged hypermetabolic lymph nodes in the supraclavicular, retroperitoneal, peritoneal and pelvic regions. All of these findings together led us to consider a possible diagnosis of a renal pelvic malignant tumor with multiple lymph nodes metastasis.\nA few days later the patient underwent a left-sided nephroureteral cystectomy and retroperitoneal lymph node dissection, in which part of the bladder was removed. The surgery was performed to establish a definitive diagnosis and for treatment if the mass was malignant.\nGross examination of the kidney showed a 5 × 2.5–cm, pale, whitish-tan, ill-defined mass located in the renal pelvis near the renal hilum. Histologic examination of the mass showed lymphatic tissue hyperplasia and diffuse infiltration of plasma cells. The plasma cells were IgG- and IgG4-positive. The IgG4/IgG ratio was approximately 40% (Figure ). Two retroperitoneal lymph nodes were dissected, which represented as reactive hyperplasia. The pathological findings did not reveal malignancy.\nAfter the diagnosis of IgG4-RD was made, a further laboratory examination was performed. The patient’s serum IgG4 level was 18.6 g/L (normal reference range, 0.03 to 2 g/L), and her high-sensitivity C-reactive protein test result was 26.3 mg/L (normal reference range, 0 to 3 mg/L). The patient underwent steroid therapy in a local hospital for 1 year. At the fourth month of her steroid therapy, her high-sensitivity C-reactive protein level had descended to 8.76 mg/L (normal reference range, 0 to 8 mg/L). Her enlarged lymph nodes in the supraclavicular, retroperitoneal, peritoneal and pelvic regions diminished with the steroid therapy.", "summary": "A 54-year-old woman visited our hospital with left flank discomfort and palpebral edema. Computed tomography, magnetic resonance imaging, retrograde pyelography and positron emission tomography/computed tomography indicated renal pelvic cancer. However, after a left-sided nephroureteral cystectomy was performed, the mass was pathologically confirmed as an IgG4-related lesion. Her elevated serum IgG4 level and a past history of sicca complex supported the diagnosis of IgG4-RD.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_444_en.txt", "fulltext": "Fifteen years ago, this now 40-year-old female had undergone a L5S1 discectomy . Ten months before this admission, due to complaints of low back pain and left leg numbness, she had undergone a lumbar; MR; it documented significant cauda equina compression at the L4-L5 level due to a large central disc herniation and marked stenosis/OYL (i.e., isointense on T2-weighted sequences) [ and ]. She now acutely presented to an emergency room with the sudden onset (i.e., over several hours) of 4/5 weakness in both lower extremities, bilateral leg numbness, and urinary retention.\nThe new magnetic resonance imaging (MRI) and CT both demonstrated worsening of her L4-L5 cauda equina compression due the large central disc herniation and severe stenosis/OYL; the CT further confirmed ossification of the OYL [-].\nNotably, the patient should have undergone emergent surgery following the completion of the MR/CT studies. However, surgery was delayed for 9 h by which time she was fully paraplegic (0/5 motor function, L4-S1 loss of sensation, and urinary retention (JOA 2/29).\nFollowing a L4-L5 laminectomy for stenosis, OYL, and a partial central discectomy (i.e., not completely removed because of its central location), the patient never fully recovered; she had just 1/5 motor function bilaterally (i.e., residual loss perineal sensation and urinary incontinence – final JOA score 9/29) 1 month later. Of interest, the postoperative MR and CT studies, despite showing mild residual ventral disc, confirmed adequate cauda equina decompression [-].", "summary": "A 40-year-old female developed an acute cauda equina syndrome (CES) attributed to an acute lumbar disc herniation and to marked canal stenosis due to OYL. As the patient underwent a 9 h delayed removal of the ossified ligament and discectomy, she sustained only minimal recovery.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2694_en.txt", "fulltext": "A 74-year-old woman, who presented with complaints of repeated upper abdominal pain for 3 days, was admitted to our hospital. She had no relevant past medical history. Abdominal ultrasonography and computed tomography (CT) scan at another hospital revealed a tumor in the pancreatic uncinate process; thus, she was referred to our hospital for a comprehensive examination.\nThe results of the laboratory tests were found to be almost normal (the italicized text indicates the test results with abnormal values): white blood cell 9130/μl, total bilirubin 1.0 mg/dl, aspartate aminotransferase 16 U/l, alanine aminotransferase 9 U/l, hemoglobin A1c 6.0%, amylase 76 U/l, C-reactive protein < 0.02 mg/dl, Ca 10.3 mg/dl, soluble interleukin-2 receptor 271.0 U/ml, IgG4 31.5 mg/dl, and antinuclear antibody 160 index. The levels of tumor markers were also normal: carcinoembryonic antigen 3.0 ng/mL, carbohydrate antigen 19-9 11.0 U/ml, DUPAN-2 < 25 U/ml, span-1 8.9 U/ml, and elastase-1 85 ng/dl.\nAn abdominal contrast-enhanced dynamic CT scan showed a 60-mm-diameter tumor in the pancreatic uncinate process, accompanied by multiple cysts , and the tumor size tended to increase over time. The gastroduodenal artery was noted to be passing through within the tumor. The pancreatic duct was not enlarged and separated from the tumor. The tumor had a poor contrast effect in the arterial phase and a small contrast effect in the equilibrium phase.\nThe gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (MRI) showed a lobulated tumor with mixed high and low signals on T2-weighted imaging . Out-of-phase T1-weighted imaging showed a low-intensity area, which was a fat component. This finding suggested that the tumor was unlikely to be a malignant tumor. However, diffusion-weighted images revealed a high signal lobulated tumor and suggested potential for malignancy . Magnetic resonance cholangiopancreatography revealed a soft tissue tumor close to the pancreatic uncinate process, and it was not continuous with the main pancreatic duct.\nAn upper gastrointestinal examination revealed that the gastric angle was pushed to the dorsal side of the stomach by the tumor. Endoscopic ultrasonography (EUS) showed a collective cystic lesion on the ventral side of the pancreatic uncinate process . The main pancreatic duct was negative for intraductal papillary mucinous neoplasm. In addition, given that the tumor was accompanied by a cystic lesion, a fine-needle aspiration was not performed.\nIn the positron emission tomography CT scan, there was no significant fluoro-deoxy-glucose accumulation in the soft tissues of the pancreatic uncinate process.\nThe tumor in the pancreatic uncinate process was thought to be the possible cause of the upper abdominal pain. Based on the abovementioned findings, we considered that the tumor was a benign vascular malformation, but because the tumor size tended to increase over time, there was a possibility of it being a malignant tumor; thus, we planned on performing a pancreatoduodenectomy (PD).\nAfter a detailed examination, we performed an elective open laparotomy. During the operation, intraoperative findings revealed that the tumor appeared to be benign and was separate from the pancreatic duct or bile duct. We therefore performed a partial pancreatectomy instead of PD. Since the right gastroduodenal artery and small vein penetrated the tumor, they were ligated and detached. The tumor was excised with a small part of the pancreatic uncinate process, and the mesentery of the transverse colon was also removed. The operation time was 200 min with 75 ml blood loss. The patient’s postoperative course was uneventful, and she was discharged on postoperative day 12.\nMacroscopically, the tumor was a 58 × 46 × 30-mm specimen with a spongioid appearance of the cut surface . Histologically, hematoxylin and eosin staining showed a mixed shape of small veins, small arteries, and capillaries . The pancreatic tissue was recognized within the tumor, suggesting that the tumor originated from the pancreas. There was no lesion with suspected malignancy. We performed immunostaining for CD31, CD34, Factor VIII, and D2-40, which revealed the following in general: CD31 was positive for vascular endothelium and histiocyte; CD34 and Factor VIII were positive for vascular endothelium; and D2-40 was positive for lymphatic endothelium. In the Elastica van Gieson staining, most vessels of the tumor had no muscular layer. These vessels were capillary blood vessels (CD31, CD34, and Factor VIII were positive, and D2-40 was negative) and lymphatic vessels (CD31 and D2-40 were positive; CD34 and Factor VIII were negative). Based on both histological appearance and immunostaining findings, we diagnosed the tumor as a capillary lymphatic malformation (CLM) according to the International Society for the Study of Vascular Anomalies (ISSVA) classification.", "summary": "A 74-year-old woman, who presented with complaints of repeated upper abdominal pain, was admitted to our institution. Contrast-enhanced dynamic computed tomography (CT) scan revealed that the tumor in the pancreatic uncinate process had a poor contrast effect in the arterial phase and a small contrast effect in the equilibrium phase, which are suggestive of a benign disease-like vascular malformation. However, we suspected that it could possibly be a malignant tumor because the tumor size tended to increase over time; thus, we decided to perform a surgery. We resected the tumor through a partial resection of the pancreas. Macroscopically, the cut surface of the tumor had a spongioid appearance. Histopathological examination findings showed a mixed shape of small capillaries and lymphatic ducts. The patient was diagnosed with CLM according to the International Society for the Study of Vascular Anomalies (ISSVA) classification, based on the histological appearance and immunostaining findings. The postoperative course of the patient was uneventful.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1165_en.txt", "fulltext": "In November 2017, an asymptomatic 14-year-old boy referred to the authors’ hospital for management of right lateral chest wall mass discovered incidently one month earlier. There was no history of trauma. Clinical examination was unremarkable. Plain chest radiography showed a smooth-walled 6.0 × 3.5 cm homogenous right lateral chest wall mass (A). Computed tomographic (CT) scan revealed right lateral extrapleural soft-tissue mass 6.5 × 4.0 × 5.6 cm in size abutting 5th and 6th ribs and filling the 5th intercostal space (B). There were neither cavitation nor calcification within the mass, and the adjacent ribs were not eroded.\nMagnetic resonance imaging (MRI) revealed right extrapulmonary and extrapleural chest wall soft tissue mass. Tl-weighted MRI demonstrated a solid mass with a low signal intensity (C), while T2-weighted MRI demonstrated a homogeneous mass with high signal intensity similar to that of fat tissue (D). Early phases after administration of contrast, showed characteristic heterogeneous enhancement of the tumor that became more uniform during the delayed phase (E and F).\nThe angiogram showed a sizeable vascular mass supplied by a branch of the internal mammary artery. That branch was subsequently embolized with Gelfoam pledgets with no further filling on the post-embolization arteriogram.\nThe patient underwent an exploratory video-assisted thoracoscopy (VAT) through the right 8th intercostal space anterior axillary line (A and B). Posterolateral thoracotomy revealed well-demarcated mass abutting the 5th and 6th ribs filling the right 5th intercostal space. En bloc resection of the mass involved ribs and the intercostal muscles were performed (C). Chest wall defect was reconstructed using polytetrafluoroethylene (PTFE) patch (GORE-TEX®, DUALMESH®, W. L. Gore & Associates) (D).\nThe surgical specimen measured approximately 6 × 4 × 3.5 cm, which included a hemorrhagic, highly vascular, mass that extended from the intercostal muscles to the extra-pleural space (E). Cut section revealed whitish homogenous material. Microscopically, there were homogeneous thick and thin-walled blood vessels with proliferating vascular spaces of capillaries of the cavernous type with intervening fibrous stroma (F). There was no evidence of recurrence at 3 and six months of follow-up.", "summary": "A 14-year-old boy who presented with asymptomatic right lateral chest wall mass with no history of trauma. Different radiological modalities were employed for diagnosis including computed tomography (CT) showed a soft tissue mass 6.5×4×5.6cm in size abutting 5th and 6th ribs. Magnetic resonance imaging (MRI) revealed iso-intense signal in T1 and hyperintense signal in T2, that is higher than that of adjacent muscles in the inferolateral right chest wall which was compatible with intercostal hemangioma. The patient underwent surgery for excision of the mass. Through right posterolateral thoracotomy, there was a well-demarcated mass abutting 5th, and 6th ribs filling the right 5th interspaces. Histopathological examination confirmed the diagnosis of intercostal hemangioma.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2233_en.txt", "fulltext": "A 75-year-old Japanese man was admitted to our hospital with a gradually growing cutaneous polypoid mass that had appeared on the skin of the left scrotum approximately one year before. The tumor measured 13mm in diameter with relatively well-defined whitish-yellow outlines . Histological examination of an excisional biopsy revealed epidermal papillary proliferation with parakeratosis, hyperkeratosis, and neutrophil infiltration . Although mitotic figures were noted in the basal layer , cellular atypia was not prominent. Besides abundant plasma cells in the upper dermis, numerous foamy macrophages infiltrated the dermal papillae, forming a characteristic clear zone beneath the basement membrane . Histopathologically, the tumor was diagnosed as a benign cutaneous verruciform xanthoma with negative lateral and deep surgical margins.\nTo determine which cells release chemoattractants for macrophages, formalin-fixed and paraffin-embedded sections were stained for cytokeratin (AE1/AE3, M3515; Dako, Carpinteria, CA, USA; 1:200), CD68 (M0876; Dako; 1:100) and MCP-1 (DA103; BD Biosciences, San Diego, CA, USA; 1:40, 1:200, 1:800). After microwave heat-induced epitope retrieval, endogenous peroxidase activity was blocked with hydrogen peroxide (H2O2) in methanol. Indirect immunohistochemistry with the use of horseradish peroxidase conjugated anti-mouse rabbit antibody revealed that cytokeratin AE1/AE3 was strongly positive in differentiating epidermal keratinocytes, and weakly positive in keratinocytes in the basal and parabasal layers . However, strong CD68 staining was observed almost exclusively in foamy cells infiltrating beneath the basal cells . The parts densely stained for MCP-1 were observed in the differentiating cytokeratin AE1/AE3-positive keratinocytes. Clusters of the infiltrating macrophages also stained positive for MCP-1 .", "summary": "A 75-year-old Japanese man with a well-defined nodule on the left scrotum was admitted to the hospital. An excision biopsy revealed epidermal papillary proliferation with parakeratosis, hyperkeratosis, and infiltration of foam cell macrophages, whereby a pathological diagnosis of benign cutaneous verruciform xanthoma was made. Immunohistochemically, monocyte chemoattractant protein-1 was observed predominantly on cytokeratin AE1/AE3-positive differentiating keratinocytes in the prickle cell layer. However, while infiltrating macrophages were densely stained for monocyte chemoattractant protein-1, keratinocytes in the basal and parabasal layers were almost negative.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_402_en.txt", "fulltext": "A 71-year-old woman with a pancreatic tumor involving the head and uncinate process was referred to our hospital with symptoms of back pain, weight loss, and diabetes. According to imaging, the tumor was approximately 30 mm in diameter. The patient was diagnosed with pancreatic duct adenocarcinoma based on findings from an endoscopic ultrasound-guided fine-needle biopsy specimen. The tumor was in contact with the right side of the SMV at the level where it received the first jejunal vein tributary. The tumor attachment to the SMV had a longitudinal extent of about 20 mm but involved less than half of the vein’s circumference, so the tumor was considered resectable. However, extension of tumor along the retropancreatic nerve plexus (RNP) approached the superior mesenteric artery (SMA; Fig. ), so based upon the results of the PREP-02/JSAP-05 trial , 2 cycles of gemcitabine and S-1 were administered before surgery.\nAlthough no radiologically evident changes of tumor status were recognized after chemotherapy, we planned to carry out pancreatoduodenectomy with SMV resection and reconstruction. The first jejunal vein tributary would be resected, provided that a vein-clamping test produced no jejunal congestion. Then the segment of the SMV caudal to its confluence with the inferior mesenteric vein (IMV) would be resected in continuity with the pancreatic tumor. Reconstruction of the SMV by simple end-to-end suturing without a venous graft would be difficult, considering that both the splenic vein and the IMV were to be preserved.\nOne notable finding from computed tomography (CT) before surgery was dilation of the right ovarian vein to a diameter of about 10 mm, similar to the SMV diameter where resection was planned . Although the patient had no active symptoms of PCS such as chronic pelvic pain, feelings of heaviness, or urinary urgency at the time of surgery, she had experienced severe dysmenorrhea prior to menopause and still had occasional feelings of pelvic heaviness, suggesting that she was at future risk for PCS. We reasoned that use of the dilated right ovarian vein as a graft might prevent or lessen such occurrences while also offering dimensions favorable for grafting.\nNo cancer dissemination was grossly evident at laparotomy; subsequent cytologic examination of abdominal lavage fluid detected no malignant cells. Dissection began with liberal application of the Kocher maneuver to the duodenum and firm retraction of the pancreatic head to the left. The peritoneum at the base of the transverse mesocolon was divided to approach the SMA and SMV, as in the mesenteric approach . When no evident jejunal congestion resulted from clamping the first jejunal vein tributary near the confluence of the tributary and the SMV, the tributary was ligated and divided. We performed the retropancreatic nerve plexus (RNP) hanging maneuver as we previously reported , making sure that the cut end of the RNP was free of cancer invasion. In brief, the RNP hanging maneuver was carried out as follows. The middle colic artery arising from the SMA and the middle colic vein both were ligated and divided, after which the SMA trunk was followed cranially to its origin. The tape for RNP hanging was placed around the RNP, which was divided together with ligation and division of the inferior pancreatoduodenal artery in the plane determined by the hanging tape prior to any division of the pancreatic parenchyma and gastric antrum. A 3-cm minimum length of the dilated right ovarian vein was removed for use as a venous graft. The tumor was removed together with the portion of the SMV that it had invaded. The SMV was reconstructed with no venous bypass, using the right ovarian vein graft. Obvious valves were not recognized within the patient’s graft after harvesting, so we carried out reconstruction in a standard manner: distal end to distal end and proximal end to proximal end. The duration of reconstruction was 38 min , while total duration of the operation was 607 min. Intraoperative blood loss was 390 mL. The pathologic diagnosis was invasive ductal carcinoma; the stage according to the classification of pancreatic carcinoma was pT3pN1b (4/40) M0, pStage IIB, with invasion of the SMV (pPV1). R0 resection status was achieved.\nThe postoperative course was uneventful, and the patient was discharged 25 days after surgery. She has finished adjuvant chemotherapy and was free from disease recurrence at 8 months after surgery. PCS symptoms have not occurred. Patency of the venous graft for SMV reconstruction was confirmed by contrast CT carried out with administration of a direct oral anticoagulant 8 months after surgery .", "summary": "A 71-year-old woman with cancer involving the pancreatic head, uncinate process, and SMV underwent pancreatoduodenectomy with SMV resection. Reconstruction used a portion of the right ovarian vein that was markedly dilated and had placed her at risk for pelvic congestion syndrome (PCS). Graft patency was confirmed 8 months after surgery. She now finished receiving adjuvant chemotherapy and has no symptoms of PCS.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_2802_en.txt", "fulltext": "A 69-year-old Japanese man who sustained an injury in a car collision received first aid at a nearby hospital and underwent craniotomy surgery for traumatic acute subdural hematoma, traumatic subarachnoid hemorrhage, and traumatic localized brain contusion. Conservative treatment was given for left-sided condylar process fracture, axis fracture, right clavicle fracture, and right fracture of the distal radius. After his consciousness disorder improved, 4 months after injury, it became clear that the patient was unable to close his mouth. His TMJ may have been dislocated during intubation. The mouth presented in an open state, with lip closure incapacity . Stenosis of the jaw dentition was demonstrated with labioclination of the front tooth, open bite, and xerostomia. The patient had a tracheostomy and could not evaluate the verbal response, Glasgow Coma Scale (GCS) 11. Traumatic brain injury left paralysis of his right upper extremity. The patient was at high risk of aspiration pneumonia due to diminished swallowing function, so the previous doctor had performed an additional gastrostomy, which recovered his ability to eat.\nPanoramic radiography examination revealed a left condylar process fracture and dislocation of the bilateral mandibular condyles. On computed tomography examination, the left condylar process fracture on the medial side and both mandibular condyles greatly exceeded the articular tubercle .\nWe attempted a manual reduction owing to the existing diagnosis of old left condylar fracture and bilateral anterior dislocation of the TMJ, but reduction was impossible. Therefore, we planned to perform right condylar resection using an intraoral approach. In addition, we evaluated the planned procedure using a three-dimensional model and confirmed that it would not disturb the jaw reposition, because the left condylar process fractures to medial. We confirmed that it was difficult to reduce and fix the old fracture and that it did not interfere with the reproduction of the original occlusion on the three-dimensional model, so we decided to treat it conservatively.\nWe performed right condylectomy with the patient under general anesthesia. Manual reduction was performed again in a state of muscle relaxation, but reduction was not possible. After an incision was made in the buccal mucosa according to the sagittal split ramus osteotomy, the inside and outside of the mandibular ramus was revealed, and the coronoid process and the neck of the mandible were confirmed . The coronoid process obstructed the visual field, so it was cut and resected with a reciprocating saw . Using a saw and osteotome, the base of the condylar was cut and separated from the surrounding soft tissue to remove the condylar head . We confirmed that the mandibular body had moved backward and that the molars could achieve the occlusion, and the wound was closed. Intermaxillary traction was started during the postoperative period using an intermaxillary fixation screw inserted in the alveolar region to improve occlusal deviation. Twenty-one days after the operation, a normal occlusion was obtained, and intermaxillary fixation screw was removed. During the 12-month postoperative follow-up period, no TMJ dislocation recurred, and the occlusion remained stable . After the operation, he was able to open and close the mouth by himself, and the lips could be closed, so that hypersalivation did not occur. His left condylar process was displaced inward owing to a fracture, but the opening and closing movements were possible because the hinge movement centered on the left condylar process.", "summary": "A 69-year-old Japanese man who sustained an injury in a car collision was unable to close his mouth. Owing to the diagnosis of long-standing temporomandibular joint dislocation, intraoral condylectomy was performed. In the case of temporomandibular joint dislocation, it is convenient to reach the condyle from the oral cavity because sufficient opening is maintained. The condyle can be clearly visualized using an approach similar to sagittal split ramus osteotomy, and the operation using surgical instruments can be facilitated by resecting the coronoid process. By separating the surrounding soft tissue and pulling the cut condyle with sufficient visual field, the condyle can be resected while addressing the hemostasis. During the 12-month postoperative follow-up period, no temporomandibular joint dislocation recurred and the occlusion remained stable.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_984_en.txt", "fulltext": "A 46 year-old man with HIV/AIDS (CD4 T-cell 115 × 106/l) presented with two weeks of left 5th digit pain and swelling. He denied any history of recent trauma to the hand, fever, weight loss, or other systemic symptoms, but did note an occasional dry cough. He had traveled repeatedly between his native country Ivory Coast, West Africa and the United States. He was not taking antiretroviral medications at the time of presentation. On physical exam his fifth digit was swollen and erythematous at the proximal intraphalangeal joint [figure ]. His laboratory values on admission were as follows: white blood cell count 5000 cells/mm3, hemoglobin 11.6 grams/dl, hematocrit 33.9%, platelets 280,000 platelets/mm3, eosinophils 9.0%, and erythrocyte sedimentation rate (ESR) of 60 mm/hour. Left hand radiography was significant for soft tissue swelling over the left finger with joint space narrowing and cortical lucencies with cystic degenerative changes in the proximal phalanx [figure ]. Admission chest radiography demonstrated right hilar lymph node enlargement with multiple scattered nodules and a resolving right lower lobe infiltrate. Computed tomography scan of the chest revealed multiple pulmonary nodules [figure ], necrotic lymph nodes, and splenomegaly. The patient underwent incision, drainage, and biopsy of the affected finger. Operative findings were significant for purulent, mottled, soft, yellow bone.\nRare acid-fast bacilli were demonstrated on biopsy of the both phalanx and synovial fluid samples [figure ]. We used the Gen-PROBE® Amplified Mycobacterium tuberculosis Direct Test which employs a transcription-mediated amplification and hybridization protection assay to qualitatively detect Mycobacterium tuberculosis complex ribosomal RNA (rRNA). Several weeks later cultures of all surgical material grew Mycobacterium tuberculosis [figure ]. Multiple Ziehl-Neelsen stains of induced sputum samples were negative for acid-fast bacilli, but all specimens sent for sputum culture grew Mycobacterium tuberculosis. Antituberculous treatment was initiated with rifampin, isoniazid, pyrizinamide, and ethambutol prior to culture results. After 12 weeks of treatment, marked improvement in the finger lesion was noted [figure ].", "summary": "We describe a case of tuberculosis (TB) dactylitis in a patient with AIDS who originated from the Ivory Coast. The diagnosis was established by direct visualization of acid-fast bacilli on joint fluid and bone biopsy of the proximal phalanx. Imaging of the chest revealed multiple bilateral nodules. Confirmation of the diagnosis was made by isolation of Mycobacterium tuberculosis from sputum and bone cultures.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_673_en.txt", "fulltext": "A 45-year-old obese male with Stanford A DeBakey type-II aortic dissection, atrioventricular re-entrant tachycardia, and atrial regurgitation was complaining of chest discomfort accompanied by palpitations, nausea, and cold sweats. Patient had a history of controlled hypertension with nebivolol 2 × 5 mg, as well as pericardial effusion and right bundle branch block 8–10 years prior. Previously, the patient was treated with anticoagulants and anti-arrhythmic medications. The patient was an ex-smoker for 16 years before quitting 6 years ago without any history of cardiac disease in the family. His vital signs on arrival were stable with normal jugular venous pressure, widened pulse pressure 99/59 mmHg similar in both arms, and soft diastolic aortic heart murmur. Laboratory findings were elevated for white blood cells, erythrocyte sedimentation rate, D-dimer, and cardiac marker. Plain chest X-ray showed aorta elongation with cardiomegaly and basal infiltrate on the right lung and middle segment of the left lung. Transthoracic echocardiography (TTE) evaluation revealed aortic root and ascending aorta aneurysm with mild atrial regurgitation secondary to dilated aortic root. Based on the assessments, the patient gave consent for Bentall procedure to replace the dissected aorta.\nAscending and hemiarch replacement Bentall procedure was conducted successfully despite severe adhesion between epicardium and pericardium layer along with resolved complication of sinus bradycardia. The tear started at the level of aortic valve commissure extending to the ascending aorta and middle arch without sign of rupture. Circulatory arrest time was 30 min. Post-intervention, patient was prescribed antibiotics and antifibrinolytic drugs. Anticoagulant medication warfarin for prosthetic valve maintenance was postponed due to concerns of high bleeding risk from aortic dissection and Bentall procedure.\n7 days post-operation, the patient unintentionally strained himself while defecating causing sudden shortness of breath, right chest pain, Visual Analogue Scale (VAS) 5–7, and cold sweats with persistent hypotension (81/51 mmHg) and tachypnea (24 breaths/min), despite fluid support and vasoconstrictors. His electrocardiogram reading was normal. Laboratory tests showed insignificant erythrocyte reduction within predicted value (from 13.1 to 11.1 g/dL in 2 days), raised troponin-T marker (341 pg/mL), hypercoagulable state, and acute kidney injury (estimated glomerular filtration rate 65.9 mL/min/1.73 m2). Transthoracic echocardiography examination showed middle right ventricle free wall hypokinesis with apical sparing. Additionally, chest X-ray evaluation revealed right lung oligaemia and abrupt tapering of the pulmonary artery . All these findings were suggestive of PE for which the patient was prescribed anticoagulant heparin.\nHowever, pulmonary computed tomography (CT) angiography showed an enlarged heart with fluid collection in the right anterolateral-superior pericardial space of density 50–60 Hounsfield unit (HU) pushing the heart laterally while compressing posterior part of superior vena cava, as well as bilateral pulmonary vein and left atrium stenosis due to extraluminal cause indicating cardiac tamponade. Location of the compression at the level of vena cava and pulmonary vein supposedly gave rise to the findings that mimic that of PE. Anticoagulant therapy was immediately ceased, and the patient was scheduled for a surgical pericardiotomy.\nTransoesophageal echocardiography (TEE) evaluations were taken for comparison . Right upper pulmonary vein and pulmonary artery were narrowed due to an external compression compared with after release ( and ). The velocity–time integral (VTI) was also shown to be improved after the clot evacuation.\nDuring the operation, active bleeding was found at the distal anastomosis of the ascending aorta with 600 mL of blood clots pressing against the right ventricle, right atrium, superior vena cava, and main pulmonary artery . After the procedure, his haemodynamics returned to normal and was eventually discharged the following week after close monitoring. At 2 month follow-up, the patient was asymptomatic with improving exercise tolerance. Echocardiography revealed no sign of periaortic leakage from the repaired aorta.", "summary": "A 45-year-old male with aortic dissection DeBakey type-II, 7 day post-Bentall procedure, presented with sudden shortness of breath and persistent shock despite therapy. Initial assessment directed towards pulmonary embolism was supported by hallmark imaging signs from X-ray and transthoracic echocardiography evaluation. However, computed tomography scan results were suggestive of cardiac tamponade mainly accumulating at the right side of the heart, compressing the pulmonary artery and vena cava confirmed by transoesophageal echocardiography, thus mimicking the findings of pulmonary embolism. After clot evacuation procedure, the patient improved clinically and was discharged the following week.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_3352_en.txt", "fulltext": "This is a healthy 22-year-old gentleman who presented with chronic hematospermia for 5 years. This has been a consistent problem, present with every ejaculation. It has severely and negatively affected his sexual life. He has no chronic medical issues apart from major depressive disorder for which he takes Trazodone and Escitalopram. He has had no previous surgeries. Interestingly, he has a congenitally absent left kidney. He denies previous sexually transmitted illnesses (STIs). The patient has denied urinary symptoms or urethral discharge at any point, and he has adequate erections for sexual intercourse without any concern for erectile dysfunction. General physical examination and genital/prostate examination are within the normal limits without any notable findings.\n\nIn attempts to address his hematospermia, the patient has received multiple courses of antibiotics (Doxycycline, Trimethoprim/Sulfamethoxazole, and Fluoroquinolones) without benefit over the past several years. A trial of Finasteride was offered but the patient declined this due to concern of possible sexual side effects at his young age.\n\nOther aspects of his work-up after presentation including labs and imaging. Serum creatinine was normal. Urinalysis, urine culture, Chlamydia/gonorrhea, and other STI tests came back negative. An ultrasound of the scrotum was performed which was unremarkable. Given the plethora of negative tests and persistent symptoms, it was agreed upon to perform a cystoscopy which was also unremarkable. At this point, Magnetic Resonance Imaging (MRI) of the pelvis was ordered and showed no evidence of tumors, cysts, or vascular abnormalities; and revealed distension of the left SV with blood. The right SV was normal.\n\n\nThe options of treatment were discussed with the patient and given the chronicity of the condition and its negative effect on his sexual life. Several treatment options were discussed with the patient. TRUS-guided SV puncture with drug instillation, TRUS-guided cyst aspiration and transurethral resection with deroofing were discussed as less morbid interventions but associated with higher failure rate. He opted for surgical removal of the left SV, despite potential for higher morbidity and potential impotence or ejaculatory dysfunction, due to his many year struggle with the condition. This was performed as a robotic-assisted laparoscopic left seminal vesiculectomy.\n\nTechnique of the primary procedure\nThe patient underwent standard induction, position preparation and draping. He was placed in the 30-degree Trendelenburg position. An Xi DaVinci robot was utilized with port placement identical to that of a robotic prostatectomy. After access was obtained and we had visualization, the small bowel was mobilized cranially. The sigmoid colon was then mobilized, exposing the cul-de-sac. The left SV was exposed and mobilized using blunt dissection and bipolar cautery to avoid neurovascular injury. The SV was traced back and dissected to the base of seminal vesicle duct. The duct of the SV was then ligated using 2-0 Monocryl suture. Transection was completed sharply, and the SV was then sent for pathologic examination. Additional examination and hemostasis using bipolar cautery and hemostatic agents. The peritoneum was then closed over the operative field once packed with SURGIFLO ® Hemostatic Matrix mixed with thrombin.\n\nOutcome and follow up\nThe patient was discharged home in good condition the next morning. He presented with abdominal pain 2 days after the procedure with abdominal pain and no fever or urinary symptoms. Unfortunately, CT scan of the abdomen & pelvis revealed pelvic hematoma. On the 3rd day post-operatively, he was brought back to the operative room and underwent robotic-assisted laparoscopic exploration.\n\nTechnique for robotic exploration\nFollowing induction, positioning and draping, the port sites of the prior operation were opened and canula inserted. Intermediate Trendelenburg (15–20-degree) was used. Insufflation then proceeded normally. The hematoma was evacuated with the Stryker® large 10 mm suction device. The peritoneum in the Cul-de-Sac was reopened and additional hematoma was evacuated upon which, a small bleeding vessel was identified in the surgical field. This was controlled safely with bipolar cautery and a drain was placed in immediate vicinity. The field was then repacked with SURGIFLO® Hemostatic Matrix mixed with thrombin to limit any further bleeding. The peritoneum was again reconstituted, and the operation was concluded uneventfully following port closure.\n\nFollowing the procedure, the patient was feeling well and was discharged home the next day in good condition. At 6 months follow-up, the patient has no pain or urinary symptoms. He still maintains normal erections with maintenance of baseline ejaculatory volume and force without hematospermia. The patient was satisfied with the results of the procedure. Pathological examination revealed left SV with congestion and focal mild chronic inflammation but was negative for malignancy.", "summary": "We present an unusual case of chronic hematospermia for 5 years in a 22-year-old patient, which failed conservative medical management. MRI was conducted which revealed left seminal vesicle (SV) distended with blood and a normal right SV. He was treated with robotic unilateral seminal vesiculectomy (RUSV). At 6 months follow-up, ejaculation was normal and hematospermia resolved.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2586_en.txt", "fulltext": "A 48-year-old Japanese male was treated for uveitis of unknown aetiology in the left eye at a general ophthalmology clinic. Prednisolone (PSL) 40 mg/day was started due to the development of macula oedema (ME) in his left eye. Although ME improved temporarily, tapering of PSL to 20 mg/day resulted in relapse of ME. He then complained of blurred vision in the left eye due to recurrence of intraocular inflammation and was referred by the local ophthalmologist to Department of Ophthalmology, Tokyo Medical University Hospital. He has no medical history. At presentation at our facility, best-corrected visual acuity (BCVA) was 20/12.5 in the right and 24/20 in the left eye, and intraocular pressure was normal. Slit-lamp examination revealed 1 + cells in anterior chamber in left eye; fundus photographs showed diffuse vitreous haze and mild exudates in peripheral retina in left eye; fluorescent angiography depicted significant retinal vasculitis characteristic of Bechet’s disease at the posterior pole and periphery retina in left eye; and OCT confirmed ME in left eye . Peripheral blood test showed elevated white blood cell count, elevated percent neutrophils, elevated LDH, positive for HLA-A26, negative for HLA- B51, negative tuberculin skin test (TST) and negative T-SPOT.TB test . He was diagnosed with incomplete type of Bechet disease based on the ophthalmological findings, recurrent oral ulcer, erythema nodosum-like rash in his legs, and HLA-A26 positivity. After a screening test, ADA was started as steroid-sparing therapy. Following the initiation of ADA, ME improved gradually and tapering of PSL to 2 mg/day was achieved, resulting in favourable outcome.\nHowever, 8 months after starting ADA, the patient developed general malaise. Peripheral blood examination was positive for T-SPOT.TB at this time, and chest X-ray and computed tomography (CT) showed granular shadows in bilateral lungs . He was diagnosed with miliary TB by a respiratory physician. ADA and PSL were discontinued immediately, and 4-drug regimen for miliary TB consisting of isoniazid (300 mg/day), rifampicin (450 mg/day), ethambutol (750 mg/day) and pyrazinamide (dose titrated from 0.8 g/day to 1.5 g/day) was started. The TB treatment was completed 6 months later based on clinical improvement , although T-SPOT.TB was still positive. During the period when ADA was discontinued, the patient received adjunctive therapy with betamethasone eye drops and sub-Tenon's injection of triamcinolone acetonide to manage the condition. However, the efficacy of these treatments approach was limited to control ME , resulting in decreased BCVA in the left eye (10/20) and gradual elevation of intraocular pressure due to topical administration of steroid. Therefore, a decision was made to restart TNF inhibitor. Although miliary TB treatment had been completed, the patient still tested positive for T-SPOT.TB, necessitating initiation of infliximab (IFX) in combination with isoniazid (300 mg/day) to address the condition. Following the initiation of IFX, subsequent fluorescein angiography demonstrated more severe vascular leakage compared to the initial examination. However, improvements of ME, vitreous opacity and BCVA in the left eye (24/20) were observed. Furthermore, there was no TB relapse during the course of treatment after starting IFX for 3 years.", "summary": "A 48-year-old Japanese male was treated for uveitis of unknown aetiology in the left eye at a general ophthalmology clinic. He was referred to Department of Ophthalmology, Tokyo Medical University Hospital because of macula oedema (ME) not responding to prednisolone (PSL) 20 mg. BD was diagnosed based on fluorescein angiographic findings of diffuse retinal vasculitis characteristic of BD, recurrent oral aphthous ulcer, erythema nodosum-like rash in his legs, and HLA-A26 positivity. After a screening test, adalimumab (ADA) was started as steroid-sparing therapy. Eight months after starting ADA, the patient was diagnosed with miliary TB. ADA and PSL were discontinued immediately due to TB. Anti-TB treatment was completed after 6 months based on clinical improvement, although T-SPOT.TB was still positive. Infliximab with isoniazid was started due to relapse of ME, worsened vitreous haze, and worsened visual acuity in his left eye. Subsequently, his ocular symptoms subsided and there was no relapse of TB.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_747_en.txt", "fulltext": "A 55-year-old Caucasian male presented in February 2005 with new onset hematuria, easy bruising, and jaundice. He also reported fevers, nausea with vomiting and fatigue in the week prior to presentation without any associated abdominal pain or pruritus. At presentation, he was afebrile and there was no skin rash, hepatosplenomegaly, asterixis, or stigmata of chronic liver disease but he was deeply jaundiced with scleral icterus and multiple ecchymoses. Initial laboratory tests included a white blood cell count of 13.7 (4.0–10.0 K/UL) with a left shift, hemoglobin 15.5 (13.5–17.5 GM/DL), platelets 200 (140–450 K/UL), AST 1466 (20–57 IU/L), ALT 1459 (21–72 IU/L), total bilirubin 5.3 (0.0–1.5 mg/dl), direct bilirubin 4.9 (0.0–0.8 mg/dl), alkaline phosphatase 219 (30–136 IU/L), INR 13, and prothrombin time 145.8 (10.0–13.5 sec). Serum liver biochemistries were normal 4 months prior (AST 32 IU/L, ALT 40 IU/L, Total Bilirubin 0.5 mg/dl). An abdominopelvic CT scan without contrast was unremarkable.\nThe patient had a history of mild depression, hypertension, and hyperlipidemia. He denied using intravenous drugs or recent travel or sick contacts. He had discontinued alcohol in 2002 but smoked a half-pack of cigarettes for the past 8 years. He was receiving warfarin for a prosthetic mitral valve since 2002 and had a previously stable and therapeutic INR. His other medications for the past 3 years included metoprolol XL, atorvastatin, and aspirin. Paroxetine had been started shortly after surgery and discontinued in May 2004 but restarted in October 2004 for recurrent depressive symptoms. Bupropion 150 mg bid was started for smoking cessation in July 2004 and was continued up until hospitalization (6 months of treatment). The patient reported never having received bupropion or other anti-depressants beyond the paroxetine previously. He also denied ingesting over the counter products such as acetaminophen or herbals. He had allergies to penicillin and sulfa drugs that caused hives.\nAfter receiving several units of fresh frozen plasma, he was temporarily placed on intravenous heparin. Diagnostic studies included a serum iron of 193 ug/dl, transferrin saturation of 55%, and ferritin of 974 mg/dl but subsequent hemochromatosis genotyping was negative. Serum ceruloplasmin was normal at 28 mg/dl. Serological studies for acute hepatitis A IgM, hepatitis B surface antigen and anti-HB core antibody, and hepatitis C RNA by PCR as well as CMV and EBV serologies were negative. However, an anti-nuclear antibody (ANA titer = 1:160; speckled pattern) and anti-smooth muscle antibody (ASMA titer = 1:40) were positive. A surface echocardiogram revealed an ejection fraction of 75–80%. Despite withdrawal of all outpatient medications, his serum aminotransferases and bilirubin continued to rise . A transjugular liver biopsy revealed severe interface hepatitis with intense peri-portal inflammatory infiltrate consisting of a mixture of lymphocytes, eosinophils, and a few scattered plasma cells . A reticulin stain showed hepatic collapse with crowding of the reticulin meshwork and loss of hepatocytes. A trichrome stain did not reveal established fibrosis. A pathological diagnosis of a severe hepatotoxic injury due to a drug with autoimmune-like features was made.\nBecause of the persistent severe biochemical injury, the patient was started on prednisone 60 mg/day. Over the next 13 days, the serum ALT levels trended down . His total bilirubin peaked at 22.7 mg/dl and his ALT reached a second peak at 1357 IU/L before trending down over the next four weeks. The patient's INR remained difficult to manage even with lower doses of coumadin, ranging between 1.6 and 3.7. However, the INR values became more stable at approximately 20 days after institution of prednisone therapy. Upon referral to the University of Michigan, a repeat ANA was higher at 1:1280 and serum IgG, IgM, and IgA levels were 1510 mg/dl, 125 mg/dl, and 367 mg/dl, respectively. At this point, the patient felt much improved and his prednisone was tapered off over 6 weeks. The patient was discharged on prednisone, metoprolol and coumadin. Three weeks later his transaminases began to rise but his bilirubin remained unchanged. Repeat testing two weeks later showed marked elevation of his total bilirubin to 23.7 mg/dl and ALT to 961 IU/L and he was readmitted to the hospital for a possible repeat liver biopsy. A decision was made to forego the liver biopsy and restart the patient on prednisone 60 mg per day and he was discharged home. However, two days later his total bilirubin increased to 37.4 mg/dl and his ALT was 1158 IU/L. He was then admitted to the hospital for liver transplantation evaluation with new onset mental status changes. The patient was started on broad-spectrum antibiotics. The patient's condition quickly deteriorated with the onset of encephalopathy and coagulopathy. On hospital day 13, he developed respiratory failure and was transferred to the ICU but he died of multiorgan failure the next day. An autopsy revealed coronary artery disease but otherwise intact myocardium. His liver was shrunken and weighed 1320 grams and there was evidence of extensive necrosis, predominantly central zone, with cholestasis. He also had bilateral aspergillus pneumonia, which had previously not been recognized. There was no evidence of other solid organ infection. His death was attributed to sepsis resulting from acute liver failure.", "summary": "We present the case of a 55-year old man who presented with jaundice and severe hepatic injury approximately 6 months after starting bupropion for smoking cessation. Laboratory evaluation demonstrated a mixed picture of hepatocellular injury and cholestasis. Liver biopsy demonstrated findings consistent with severe hepatotoxic injury due to drug induced liver injury. Laboratory testing was also notable for positive autoimmune markers. The patient initially had clinical improvement with steroid therapy but eventually died of infectious complications.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_1095_en.txt", "fulltext": "A 56-year-old Caucasian male with a past medical history of hypertension, acute coronary syndrome and atrial fibrillation presented to the emergency department (ED) with acute epigastric pain. His complaints had started 2 weeks earlier and had worsened 1 day prior to ED presentation. The pain was associated with nausea and increased on inspiration. Defecation and micturition were normal. In addition to fenprocoumon and sotalol, he was on antihypertensives, a statin and a proton-pump inhibitor. There was no history of trauma. Physical examination showed a pale, sweating and obese man in pain. His blood pressure was 113/73 mmHg, with a heart rate of 72 beats/min, oxygen saturation of 95% on room air and a respiratory rate of 13 breaths/min. The abdomen was not distended, and there were normal bowel sounds. He had epigastric tenderness without muscular defence or hepato-splenomegaly. His initial haemoglobin was 8.5 mmol/l. White blood cell count was 8.5 × 109/l with a c-reactive protein of 26 mg/l. The international normalised ratio (INR) was 2.4. Abdominal ultrasound showed an inhomogeneous aspect of the spleen without free fluid. Contrast-enhanced computed tomography (CT) imaging of the abdomen revealed splenic haemorrhage with subcapsular hematoma .\nAn acute operation was deemed unnecessary because his vital signs remained stable. Oral anticoagulation was reversed with 10 mg vitamin K. The patient was admitted to the hospital for observation and was discharged in good condition after 3 days.", "summary": "A 56-year-old male on oral anticoagulation presented to the emergency department with epigastric pain, nausea, and left upper quadrant tenderness. There was no history of trauma. Contrast-enhanced CT imaging revealed a large subcapsular haematoma of the spleen. Oral anticoagulation was antagonised with vitamin K and the patient was discharged in good condition after 3 days of clinical observation.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2783_en.txt", "fulltext": "In a 78-year-old female patient with long history of persistent AF and symptomatic AF recurrences despite multiple previous ablation procedures and persistently isolated pulmonary veins, we performed isolation of the LAA by applying an anterior and a mitral isthmus line. Following a waiting period of 6 weeks post-LAAI and oral anticoagulation with a direct novel oral anticoagulant (Apixaban), we readmitted the patient in order to implant an endocardial LAA occluder device (Watchman; Boston Scientific, Marlborough, MA, USA). A preprocedural transoesophageal echocardiography (TOE) was performed and excluded a left atrial (LA) thrombus. The novel oral anticoagulant was stopped the day of the procedure. Under deep sedation using midazolam, sufentanil, and continuous infusion of propofol, a single transseptal puncture was performed using a regular 8, 5 Fr SL1 transseptal sheath (St. Jude Medical, Inc., St. Paul, MN, USA) after a multi-electrode diagnostic catheter was inserted into the coronary sinus. Intravenous Heparin was administered with a target-activated clotting time (ACT) of 300 s. Angiography of the LAA was performed revealing a ‘chicken-wing’ morphology . The maximal diameter at the landing zone as measured in fluoroscopy and TOE was 30 mm. Accordingly, a 33 mm Watchman device was selected to occlude the LAA. The transseptal sheath was changed over a regular guidewire for the 15 Fr non-steerable Watchman sheath (Boston Scientific, Marlborough, MA, USA), which was gently advanced over the wire into the distal LAA until the according marker was in the predefined landing zone. Suddenly the patient became hypotensive and TOE showed a massive pericardial effusion . Immediate pericardiocentesis was performed, and a 7 Fr pigtail catheter introduced into the pericardial space. The 15 Fr Watchman sheath was immediately withdrawn into the inferior vena cava to avoid potential thrombus formation along the sheath. The ACT was measured with 357 s. A total of 5000 I.E. of protamine was immediately applied resulting in an ACT of 157 s. Aspirated epicardial blood was autotransfused via a right femoral vein access, but despite continuous aspiration the effusion remained unchanged indicating a massive perforation of the distal LAA.\nIn the meantime, the patient was intubated. Suspecting distal LAA perforation as the source of the massive bleeding proximal occlusion of the LAA was desired. Therefore a new transseptal puncture using a 8.5 Fr SL1 sheath (St. Jude Medical, Inc., St. Paul, MN, USA) was performed, and a guidewire was advanced into the LAA. The guidewire went via the distal LAA into the pericardial space proving the distal LAA perforation. Now, the SL1 transseptal sheath was changed over the wire for 12F steerable FlexCath Advance sheath (Medtronic, Inc., Minneapolis, MN, USA) which was advanced to the base of the LAA followed by introduction of a 28 mm cryoballoon (ArcticFront Advance, Medtronic, Inc., Minneapolis, MN, USA). The cryoballoon was gently moved over a guidewire to the base of the LAA and manually inflated using the cryoballoon manual retraction kit (Medtronic, Inc., Minneapolis, MN, USA) . Contrast medium was applied over the Flexcath sheath and demonstrated complete sealing of the LAA . Later the epicardial blood could be completely aspirated resulting in haemodynamic stability of the patient.\nSince emergency open heart surgery in this older patient would have been associated with high peri- and post-operative risk, we decided to attempt epicardial LAA closure applying the Lariat epicardial LAA suture device (Sentreheart, Redwood City, CA, USA). Another anterior epicardial puncture was performed, and the epicardial magnet was introduced via the epicardial Lariat-sheath while the endocardial magnet was advanced over a second transseptal sheath and across the temporarily deflated cryoballon into the LAA. Both magnets connected and the Lariat device was brought into the pericardium and gently advanced over the LAA and over the cryoballoon to the LAA base . After echocardiographic and fluoroscopic verification of the position of the Lariat snare, the snare was closed and the cryoballoon deflated and pulled back into the LA which resulted in complete closure of the LAA . The Lariat suture was tightened one and a second time after a waiting period of 5 min. A final TOE evaluation as well as another contrast injection via the transseptal sheath demonstrated complete LAA occlusion, no further pericardial effusion occurred. All sheaths except a pericardial 7 Fr pigtail catheter were removed and the patient was transferred to ICU for further monitoring. The patient was extubated 2 h after the LAA closure and after exclusion of further epicardial effusion during the next 24 h the pigtail catheter was removed. She was discharged 10 days after the procedure. Three months from discharge the patient had one episode of persistent AF requiring electrical cardioversion, and she had no further pericardial effusion.", "summary": "An endocardial LAA occlusion was attempted but resulted in perforation of the distal LAA and severe pericardial tamponade. To prevent open heart surgery, a cryoballoon was advanced to the base of the LAA and inflation resulted in complete occlusion and stopped further pericardial bleeding. An epicardial LAA suture device was then successfully implanted and completely sealed the LAA. No further pericardial bleeding occurred, and the patient fully recovered.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1249_en.txt", "fulltext": "A 15-year-old male presented to the emergency department with a 4-hour history of substernal chest pain and reported an episode of syncope lasting a few minutes. He also reported homicidal ideation and audiovisual hallucinations. The patient reported using “Spice”, a synthetic cannabinoid, repeatedly over the last few hours in order to maintain his euphoric mood. He used a vaporizer for consuming “Spice”. The patient was agitated and experienced audiovisual hallucinations instructing him to harm himself and others. He had no prior psychiatric or medical history. He denied drug allergies. Surgical and family history was unremarkable. Social history revealed that the patient was an emancipated minor, smoking a pack of cigarettes per day, drinking 3-5 beers per month, and a regular user of marijuana and synthetic cannabinoids. He also reported smoking crack cocaine once at age of 13. Physical examination revealed normal cranial nerve examination, tachycardia, hyperventilation, and an erythematous lesion resembling a canker sore in the lateral tongue with surrounding erythema. Blood pressure was 137/83 mmHg, temperature was 37.3 ºC, pulse rate was 75 beats per minute, and respiratory rate was 12 breaths per minute. Laboratory testing revealed no electrolyte abnormalities. Electrocardiography (ECG) showed ST segment elevation in leads V1, V2, V3, and V4, non-specific ST, T-wave changes, and T-wave inversion. Erythrocyte sedimentation rate (ESR) was mildly elevated. Cardiac enzymes were 3.2 ng/ml. Echocardiography revealed hypokinetic systolic dysfunction of the left side of the heart. Liver function tests were within normal limits. Aspirin was immediately administered to the patient and he was admitted to the cardiac catheterization lab, where no blockages in the coronary vasculature were seen. He was given a diagnosis of Takotsubo cardiomyopathy, after that catecholamine levels were seen to be elevated and urine drug screen was negative.", "summary": "A case report of an emancipated 15-year-old male experiencing Takotsubo cardiomyopathy after using the synthetic cannabinoid \"Spice\" is presented here.", "readability_score": 1, "difficulty": "hard" }, { "id": "multiclinsum_test_2176_en.txt", "fulltext": "A 27-week four days old preterm male infant was born from a dichorionic diamniotic twin pregnancy with low birth weight(1135 gr). His prenatal examinations were completed regularly without reported complications. In the 27th week of pregnancy, the mother experienced premature membrane rupture, contractions, and bleeding. She was treated with antenatal steroids and cesarean delivery was planned due to an arrested birth. During birth, the neonate was hypotonic and apneic, requiring intubation one minute after birth due to respiratory failure with an uncuffed endotracheal tube. His APGAR scores were 4,6 and 7 in the 1st, 5th, and 10th seconds, respectively. He was transferred to the newborn intensive care unit(NICU) for further management. After a diagnosis of respiratory distress syndrome was made, treatment with endotracheal poractant alfa 200 mg/kg, parenteral ampicillin-gentamicin, and caffeine citrate was initiated. His ventilation settings were arranged to assist control and volume guaranteed mode with maximum inspiring pressure of 20 cm/H²O. His cranial ultrasound showed no abnormalities.\nOn hospital day 3, he was extubated to nasal intermittent positive pressure ventilation but required reintubation for recurrent respiratory failure. His antibiotics were escalated to vancomycin-meropenem due to clinical deterioration.\nDuring hospital day 5, the baby had worsening acute hypoxic respiratory failure. A chest x-ray revealed mediastinal pneumothorax , with a follow-up x-ray one hour later showing accelerated pneumothorax on the left side and mediastinal shift to the right. To treat the pneumothorax, a thorax tube was placed through the left 5th intercostal space. Repeat x-rays showed an expanded lung on the left side and air bronchograms on the right. Ten hours later, he had persistent acidosis and hypoxia, with another x-ray displaying a repeat pneumothorax on the left side. The thorax tube was set to continuous suction, resulting in a reduction of pneumothorax on subsequent imaging.\nOn the 6th day of hospitalization, an echocardiogram revealed a 3 mm patent ductus arteriosus(PDA) with mostly left to right two-way shunts and pulmonary hypertension. His oxygen saturation levels were approximately 80%, and his arm-leg saturation differences were higher than 15 mm Hg, suggesting differential cyanosis. These findings were suspicious for persistent fetal circulation; therefore, pediatric cardiology advised against the closure of PDA. A new chest x-ray showed a collapsed left lung, and a second chest tube was placed in the left hemithorax.\nThe decision to perform a surgical intervention was made given no resolution of the pneumothorax after the second chest tube and the patient’s continued clinical deterioration. A left posterolateral thoracotomy was performed. During the exploration, a 1 cm perforated area between the left bronchus and carina was found by following the air leak through the visceral pleura. The perforated area was repaired by using a pleural patch, and suturing with 6.0 prolene was performed. At the end of the procedure, there was no visible bleeding or air leak. The anesthesiologist applied high-pressure air via the endotracheal tube to check for any air leaks. A chest tube was inserted, and the thoracotomy incision was closed by layers. Post-procedure chest x-ray showed an expanded left lung. Three days later, the neonate experienced another episode of hypoxia, which was found to be secondary to a repeat left-sided pneumothorax. Consequently, a second thoracotomy was performed using the same technique as the prior surgery, with an air leak emanating from the same area. The perforated area was repaired using a pleural patch and the left 5th intercostal muscle long peduncle . Fibrin glue and spongestan were used as anchorage on top of the patch . After the repair, no leak was found with the use of maximal airway pressure. A PDA ligation operation was also performed simultaneously during the procedure . There was no air leakage in the repeat x-ray. Both the first and second thorax tubes were removed on postoperative days 0 and 5, respectively. There were no signs of air buildup or pneumothorax on the following days. On the 161st day of admission, the patient was discharged home with a home-type ventilator and 30% oxygen support.", "summary": "In this case, we present a preterm newborn who developed pneumomediastinum and pneumothorax. The pneumothorax persisted, despite placement of a thorax tube, requiring a thoracotomy to detect and treat the bronchial rupture.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_14_en.txt", "fulltext": "The proband, female of 1 year and 3 months old, was referred to the Research Centre for Medical Genetics because of severe failure to thrive. She was born from a first pregnancy. Parents were nonconsanguineous and had normal growth. The threat of miscarriage took place at 27 weeks. Delivery was emergent at 38 weeks and 5 days by cesarean section. The condition at birth was severe. Respiratory failure was increasing. The child was on a ventilator for five days. In total, respiratory support was required for five months. The patient was diagnosed with pulmonary hypertension.\nBody weight at birth was 1490 g (Z-score −4.50), 40 cm in length (Z-score −4.54). Head circumference was 32.5 cm (Z-score −2.59). Chest circumference was 24 cm. She had hydrocephalic head, short palpebral fissures, ocular and nipple hypertelorism, depressed nasal bridge, epicanthus, microstomy, high-arched palate, hypoplastic low-set ears, short neck, low hair line, and arachnodactyly. The baby had a pronounced progeroid appearance. The subcutaneous fat layer was not pronounced. An external examination of the genitals revealed hypoplasia of the labia majora, and the clitoris was hypertrophied. The liver protruded 3 cm below the edge of the right costal arch and 1 cm below the edge of the left costal arch. Motor activity was reduced.\nUpon screening at 9 months, the patient’s condition was severe, but her consciousness was clear, and her posture as active. The baby was responsive to communication. She had a body weight of 3.3 kg (Z-score −6.54), a height of 54 cm (Z-score −6.61), and a body mass index of 11.32 (Z-score −4.67). Her head circumference was 43.5 cm (Z-score −0.76), and her chest circumference was 30 cm. The patient’s physical development was extremely low, disharmonic. Pseudohydrocephalus, high forehead, triangular face, exophthalmos, narrow nasal dorsum, smooth philtrum, microgenia, microtia, and arachnodactyly were observed in the child during the examination. The skin was clean and dry. The girl had cyanosis of the face and upper half of the trunk when crying. The subcutaneous fat was underdeveloped. The patient’s chest was barrel-shaped. Hepatomegaly was observed. The child could not support her head but tried to turn her body from prone to supine. She had a pronounced interest in toys. Her speech consisted of babbling, with chains of syllables. X-ray showed delay in bone age – less than three months vs the patient’s actual age of 9 months. The bone structure was sparse. Valgus deformity of the knee joints was observed. Data on the condition of the patella were not provided because these bones are transparent on X-ray scans in young children, and ultrasonography was not performed. EEG recordings contained no epileptiform or focal pathological activity. Echocardiography showed no signs of heart defects. Patent foramen ovale and diagonal trabecula in the left ventricular cavity were observed.\nAnother examination was performed at 1 year and 3 months was performed. The girl had a height of 58 cm (Z-score −7.06), a weight of 4 kg (Z-score −6.44) and a head circumference of 45 cm (Z-score −0.5). Hydrocephalic head, high forehead, triangular face, exophthalmos, narrow nasal dorsum, microgenia, microtia, severe hypotrophy, and areas of alopecia on the head were observed. Subcutaneous fat was not developed. The baby did not sit independently and could not steadily support herself on her feet, but she was able to stand with support. She played with toys, held objects in her hands, collected pyramids, and transferred small objects.\nThe last time the patient was examined when she was 1 year 11 months old. Short stature (63 cm, Z-score −7.04) and growth retardation (5 kg, Z-score −6.16) were noted. Clinical examination revealed dysmorphic facial features, microtia. Absent patella was noted by palpation. .\nInitially, chromosomal abnormality was suspected in the child. Cytogenetic testing was performed. Karyotype 46, XX was detected. Hutchinson-Gilford progeria was assumed. A corresponding study of the LMNA gene was performed. No pathogenic changes were observed. Clinical exome sequencing was ordered, identifying two variants in the CDC6 gene. Parental examination revealed that they were heterozygous carriers of relevant changes in the nucleotide sequence.\nNeither variant had been registered in either the GnomAD database (v.2.1.1), the clinical databases (Clinvar, HGMD Professional (v.2021.1)), or the database of variants obtained by exome sequencing (WES, CES) in the Research Center for Medical Genetics – RuExac (1337 samples). The exon 3 nucleotide sequence variant of the CDC6 gene (chr17:38447363C>T) results in a premature translation termination site (p.(Gln78Ter), NM_001254.3). According to the pathogenicity criteria, ACMG is classified as pathogenic (PVS1, PS3, PM2). The criterium PS3 was used due to the previously functional study that demonstrated pathogenicity of LoF variants in the CDC6 gene. A variant in exon 3 of the CDC6 gene (chr17:38447361A>G), resulting in a missense replacement of (p.(Lys77Arg), NM_001254.3), is classified as a variant with uncertain clinical significance (ACMG: PM2, PM3).", "summary": "The patient, a female, had extremely low physical development, neonatal progeroid appearance, lipodystrophy, thin skin, partial alopecia, cyanosis of the face, triangular face, microgenia, arachnodactyly, delayed bone age, hepatomegaly, hypoplasia of the labia majora, and hypertrophy of the clitoris in addition to known clinical signs. Differential diagnosis was performed with chromosomal abnormalities and Hutchinson-Gilford progeria. According to the results of sequencing of the clinical exome, the patient had two previously undescribed variants in the CDC6 gene, c.230A>G (p.(Lys77Arg)) and c.232C>T (p.(Gln78Ter)), NM_001254.3, in a compound heterozygous state.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_110_en.txt", "fulltext": "A 14-year-old Han Chinese woman with a history of neurogenic bladder for more than 10 years was admitted to our Department because of coma for 12 h. The patient complained for diplopia without fever or diarrhea 4 days before her coma with no incentive. She had no limb weakness and was treated with Vitamin B1and mecobalamine. Her previous medical history included neurogenic bladder caused by spina bifida occulta for 12 years and hydronephrosis diagnosed 1 year before. Sometimes she had urine retention, but she received no treatment for the neurogenic bladder except for urethral catheterization occasionally. She had no seizures and no fever. On admission, physical examination revealed that she was unconscious. The pupils did not react to light and presented different sizes in each eye (left pupil diameter, 2.5 mm; right pupil diameter, 4 mm). On stimulation, she could move her 4 limbs spontaneously. There were exaggerated deep tendon reflexes over both legs and the right Barbinski sign was positive. However, her vital signs were stable: Blood Pressure (BP) was 120/81 mmHg; pulse rate, 76 beats/min; body temperature, 36.5 °C; breath, 12 times/min. Non-contrast brain computed tomography (CT) after the onset of her coma revealed diffuse brain stem hypodensity. Brain MRI acquired 4 h after the coma onset in the county hospital showed high signal at the dorsal part of the pontine base and the mid brain on fluid-attenuated inversion-recovery (FLAIR) imaging and T2-weighted imaging. However, diffusion-weighted images presented normal signal . The non- contrast abdominal CT preformed on the next day of admission showed chronic obstructive uropathy with a distended bladder, severe hydronephrosis and dual renal atrophy.\nBlood analysis immediately after admission revealed renal failure [Urea nitrogen: 23.87 mmol/L, Creatinine: 566.8 μmol/L], and anemia [RBC: 2.5 × 1012/L, HGB: 79 g/L]. Arterial blood gas analysis revealed metabolic acidosis [pH 7.09 (7.35–7.45)]. Liver function was normal.\nThe patient was diagnosed with UE due to chronic renal failure and metabolic acidosis. She was immediately treated with 200 ml of bicarbonate at 5% conducted by quick intravenous injection, followed by 100 ml intravenous injection of bicarbonate at 5% once a day and 1.0 g of bicarbonate administered orally three times per day for 3 days. Simultaneously, she underwent urethral catheterization to relieve the urine retention. She was revived without diplopia 3 days after admission and the reviewed blood analysis after urethral catheterization showed that her renal function was returning to a normal level (Urea nitrogen: 13.10 mmol/L, Creatinine: 294 μmol/L); hence, dialysis was not necessary. She was treated with hypodermically administered erythropoietin 3000u twice a day, and ferrous sulfate 0.3 g orally thrice a day for the anemia. She was discharged 14 days after the admission without diplopia and any mental disturbance. She was still with the ureteral catheter because of the hydronephrosis. Two weeks after the discharge, the catheter was removed when abdomen ultrasonic showed decreased hydronephrosis. Follow-up MRI performed 2 months after the discharge revealed complete resolution of the brainstem UE . Reviewed blood analysis after 2 months of discharge showed that her renal failure still persisted (Urea nitrogen: 13.10 mmol/L, Creatinine: 190.3 μmol/L). However, she had no neurological disturbance or urinary retention. She underwent the comprehensive bladder-retraining program to avoid urinary retention and her renal function was regularly assessed once a month.", "summary": "A 14-year-old Han Chinese woman with a history of chronic renal failure caused by neurogenic bladder. On admission, she was unconscious and her pupils presented different sizes, while her vital signs were normal. MRI showed high signal in the dorsal pontine base and in the mid brain on fluid-attenuated inversion-recovery (FLAIR) imaging and on T2-weighted imaging while the signal was normal on diffusion-weighted images (DWI). Blood analysis revealed renal failure and acidosis. After urinary retention treatment and acidosis correction, the patient soon recovered. Follow-up MRI 2 months after the discharge revealed complete resolution of UE in the brainstem.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1257_en.txt", "fulltext": "This is a retrospective case study of a 56-year-old white man diagnosed as having an AOP infarct admitted to Umeå University Hospital, Sweden. The relevant radiology images were obtained from the hospital’s database (PACS) and evaluated by a neuroradiologist as bilateral paramedian thalamic infarcts as a result of an AOP occlusion. The medical records (SYStem Cross) at Umeå University Hospital were accessed and reviewed for his medical history, neurological work-up, and laboratory work-up (ROS). The diagnosis was based on symptoms of AOP infarction as described in the literature, radiological signs of AOP infarction, as well as exclusion of differential diagnoses.\nHe was taken to our emergency department (ED) after he was found unconscious in his home with open doors and windows. He developed ventricular fibrillation on arrival at our ED. Cardiopulmonary resuscitation (CPR) was immediately initiated and sinus rhythm (SR) was achieved on second defibrillation. He was then put on cardiopulmonary bypass for rewarming. A third ventricular fibrillation then occurred and SR was achieved on first defibrillation within seconds of onset. The total estimated duration of ventricular fibrillation was less than 2 minutes.\nHis medical history included overconsumption of alcohol. He smoked 20 cigarettes per day. On admission to our hospital there was no information available concerning his medication. On arrival at our ED his body temperature was 24.5 °C, blood pressure 145/70, heart rate 35 beats/minute, and respiratory rate 8 to 10 breaths/minute. Auscultation of his heart and lungs was unremarkable. He was unconscious with a Reaction Level Scale 85 (RLS-85) score of 4. RLS-85 gives a score between 1 and 8. An RLS-85 score of 4 indicates an unconscious patient who localizes but does not ward off when pain stimulated. He exhibited a slight anisocoria with his right pupil slightly bigger than his left.\nThe laboratory work-up at admission included moderate electrolyte disturbances and elevated liver enzymes. Drug and alcohol screens were negative.\nAn emergency CT of his head was performed and initially misinterpreted as normal with no signs of hemorrhage or acute infarction . In our intensive care unit (ICU), he was initially sedated and intubated with ventilator treatment. An attempt to extubate and wake him was made on day 3, but he still required ventilator support. He was re-intubated and sedated, and later given a tracheotomy. A complicated disease course followed with pneumothorax after CPR, pneumonia treated with antibiotics, bilateral pleural effusion requiring drainage, intestinal paralysis, acute pancreatitis, and ascites requiring paracentesis. The sedation was discontinued 2 weeks after admission to our ICU. He still required ventilation support. At this stage he could open his eyes when spoken to but otherwise he gave no contact. On neurological examination he withdrew his arms, moved his left foot, and grimaced upon pain stimulation. He had slight anisocoria, this time with his left pupil slightly bigger than his right. His pupillary reflexes were, however, symmetrical on direct and indirect stimulation. A follow-up CT performed on day 24 revealed bilateral ischemia in the medial areas of the thalami, as well as a smaller ischemic area in the left part of pons . At this point a senior neuroradiologist re-evaluated the first CT performed on admission and concluded that bilateral thalamic ischemia was discernable also on this CT . Electroencephalography (EEG) was pathological and showed an irregular theta/delta activity. However, there was no epileptiform activity.\nFour weeks after admission it was possible to extubate him. His neurological function remained unchanged. He was transferred to our medical acute ward. On day 35 of hospitalization he died and the postmortem examination revealed pulmonary infarctions and pneumonia. A timeline of events is given in Fig. .", "summary": "This is a retrospective case study of a 56-year-old white man admitted to Umeå University Hospital and diagnosed with an artery of Percheron infarction. Medical records and the neuroradiological database were reviewed, and the diagnosis was made based on typical symptoms and radiological findings of artery of Percheron infarction. We report the case of a 56-year-old man with a history of overconsumption of alcohol who was found in his home unconscious and hypothermic. He had a Reaction Level Scale-85 score of 4. He developed ventricular fibrillation on arrival at our emergency department, and cardiopulmonary resuscitation successfully restored sinus rhythm within an estimated 2 minutes of onset. He was then put on cardiopulmonary bypass for rewarming. The initial head computed tomography performed on admission was wrongly assessed as unremarkable. Bilateral ischemia in the paramedian thalamic nuclei and pons were first documented on a follow-up computed tomography on day 24 after hospitalization. He died on day 35 after hospitalization.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2715_en.txt", "fulltext": "A 23-year-old woman was referred to our hospital because of recurrent abdominal pain for 7 years, amenorrhea and dyspareunia. The patient’s mental state and movement ability was normal. She had a history of fracture of both legs and skin injury of pubic caruncle caused by trauma about 20 years ago.\nGynecological examination showed the patient had a single orifice on the urogenital region located where the urethra would be expected. There is a normally located anal perineal orifice. When forced urination, urine flowed out of the single orifice on the urogenital region. The urethral catheter was inserted about 70 mm through the single orifice, and no urine flowed out.\nPelvic CT scan showed a large calculus and a urethral catheter in the vagina . Pelvic MRI scan demonstrated the urethra and the vagina merged into a common channel about 3.45 cm long , and a large calculus about 7.8 cm × 6.8 cm × 7.7 cm in size lodging in the upper vagina, the uterus was moved upward ; a urethral catheter entered the vagina through the opening on the urogenital region; both ovaries and bladder were normal and no other congenital malformation was noted . Transabdominal sonography imaging revealed a vaginal stone, and normal two kidneys, the bladder, uterus, cervix, and adnexa.\nUnder general anesthesia, entering the urogenital tract through the median perineum approach and a 2-cm incision was performed . A hard grayish-yellow abnormal mass was observed in the vaginal cavity , and there was no adhesion to the surrounding mucosa. The tissue around the calculus was carefully separated and the calculus was completely removed.\nDuring the exploration after lithotomy, the common channel formed by vagina and urethra was detected, and the orifice of urethra was about 3 cm from the anterior vault of the vagina. The urethral catheter was inserted about 20 mm through the orifice of urethra, the urine flow was observed. Therefore, the estimated length of the urethra was about 2 cm. Hysteroscopic insertion of the vagina revealed thin endometrium and normal cervix, no other abnormality was found. The patient was diagnosed with a vaginal stone, urogenital sinus anomaly and thin endometrium.\nVaginal reconstruction (vaginoplasty) and urethra reconstruction (urethroplasty) were performed. The mucosa near the urethral orifice was taken and wrapped by 20 fr Foley’s catheter, forming a urethra about 4 cm and neourethral meatus. The vaginal mucosa was pulled down and intermittently sutured with the perineal skin to form a vagina about 8 cm that could hold two fingers.\nAfter the operation, the patient received the treatment of estrogen and progesterone, and menarche appeared 20 days later. One month after the operation, the patient recovered well, and could urinate by himself. After 8 months of follow-up, the patient had normal menstruation and urination. The patient is married and ready to conceive.", "summary": "We present a case of a 23-year-old woman with urogenital sinus anomaly who presented with a vaginal calculus. The patient was not suspected of a calculus in the vagina until the patient suffered amenorrhea and dyspareunia. Pelvic computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the patient had urogenital sinus anomaly with vaginal calculus. For the reason, the calculus was removed by surgery, and the reconstruction of vagina and urethra was performed. The postoperative recovery and follow-up were uneventful.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_497_en.txt", "fulltext": "A 48-year-old woman was admitted to the emergency department with sudden onset severe chest tightness whilst doing yoga. This was associated with pins and needles in both arms, nausea, and abdominal discomfort. It lasted for 1 hour before it self-resolved and was not related to exertion. She reported being under increased emotional stress in the preceding month prior to presentation. She had no significant past medical history and was not on any regular medications.\nShe had a mother who died suddenly from an ascending aortic dissection in her 50s. Her mother was not hypertensive and suffered no symptoms or comorbidity suggestive of systemic illness.\nThe patient was hypotensive with a blood pressure of 90/60 mmHg and a heart rate of 80 bpm with no respiratory compromise or fever. Her troponin I (high-sensitivity assay) was 54 ng/dl, 79 ng/dl, and 27 ng/dl, respectively. Her electrocardiogram (ECG) showed sinus bradycardia with no ischaemic changes. A CT pulmonary angiogram was performed and excluded pulmonary embolus and showed no other cause for chest pain. An echocardiogram showed preserved biventricular function with no significant valvulopathy and normal ascending aorta dimensions. A coronary angiogram performed on day 2 of admission showed type 1 distal left anterior descending (LAD) coronary artery dissection with thrombolysis in antiplatelets and myocardial infarction (TIMI) 3 flow . She was initially managed with fondaparinux (factor Xa inhibitor) and dual antiplatelets with low-dose beta blocker long term once spontaneous coronary artery dissection (SCAD) was confirmed.", "summary": "This case report describes a 48-year-old woman suffering from SCAD who had a mother who died from ascending aortic dissection in her 50s.", "readability_score": 1, "difficulty": "hard" }, { "id": "multiclinsum_test_2911_en.txt", "fulltext": "A 59-year-old white European man with a history of epilepsy, treated with valproic acid and carbamazepine, was admitted to our emergency department for a serious head injury after falling down the stairs at home. His family contacted our emergency department soon after the accident. An initial medical examination found a Glasgow Coma Scale (GCS) of 9/15 with no pupillary defect. His heart rate was 50 beats per minute. His blood pressure was 100/50 mmHg and his blood oxygen was 80 %.\nHe underwent orotracheal intubation and vascular filling. A clinical examination revealed a large scalp wound, which was responsible for a significant blood loss, which continued until his arrival at our hospital despite a compression bandage. He was transferred to our emergency neurosurgery unit.\nOn admission, he underwent a vascular filling (1.0 L of saline). A neurological examination found a GCS of 3/15 and his pupils were intermediate, symmetric, and reactive. The admission laboratory tests, summarized in Table , showed anemia, thrombocytopenia, hypoproteinemia and coagulation disorders.\nA brain scan without contrast (performed 2 hours after his head injury) showed an isodense, extracerebral, homogeneous SDH, 40 Hounsfield units (HU) approximately, with an estimated maximum thickness of 20 mm, situated along the right convexity . There was also a subarachnoid hemorrhage in the fold of the left convexity . The SDH had a mass effect with a 15 mm deviation of midline structures to the left and cerebral compression. In the bone window, the scan showed multiple fractures of the cranial vault in the layers above and below . This initial damage assessment revealed no other lesions. The patient became hypotensive. His blood pressure was 70/40 mmHg. He underwent hemodynamic support by continuous administration of norepinephrine (1 mg/hour) associated with 1.0 L of saline and 500 mL of Gelafundin (succinylated gelatin). Four units of packed red blood cells were administered with six units of fresh frozen plasma.\nHe was immediately transferred to our operating room. A burr hole was first drilled through his right parietal bone to assess the consistency of the hematoma and relieve the cerebral compression as quickly as possible. Correction of coagulation disorders was carried out at the same time by our anesthetists. Fresh blood mixed with clots flowed through the burr hole, thus confirming the acute nature of the hematoma.\nIn order to evacuate the hematoma, the incision was extended to carve a frontotemporoparietal flap giving access to the entire convexity and confirming the clotted appearance of the acute SDH. The flap was not put back in place when closing due to a life-threatening cerebral edema.\nHe was admitted to our neurosurgical intensive care unit. The evolution was marked by the appearance of intracranial hypertension, which was refractory to medical treatment and led to the appearance of clinical signs of encephalic death, which was confirmed 72 hours after admission to our intensive care unit.", "summary": "We report the case of a 59-year-old white European man who had a serious head injury with an acute subdural hematoma. The trauma was also responsible for blood loss and hemostasis disorders. A cerebral computed tomography scan revealed an isodense subdural hematoma. Our intervention confirmed that it was an acute hematoma.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_3323_en.txt", "fulltext": "The case report was approved by the Ethics Committee of the General Hospital of Veroia (decision No. 11, 10/12/2017). The patient’s family provided verbal informed consent. A 64-year-old man with a medical history of bipolar disorder and arterial hypertension was hospitalized for a delayed presentation ST-elevation myocardial infarction. He was admitted to our hospital 24 hours after symptom onset. Diagnostic coronary angiography revealed 95% stenosis at the distal third of the right coronary artery, and he underwent a primary percutaneous coronary intervention to the culprit lesion. Loading doses of aspirin and ticagrelor were given. During his hospitalization, he reported palpitations and shortness of breath. Clinical examination revealed normal heart sounds and a grade III systolic murmur at the cardiac apex. The patient’s blood pressure was 100/65 mmHg, pulse rate was 95 bpm, and respiratory rate was 14 breaths/minute. He had functional class II heart failure according to the New York Heart Association. Electrocardiography showed a sinus rhythm with Q-wave formation in the inferior wall leads (II, III, aVF), T-wave inversion in the same leads, and borderline QT prolongation (QTc of 490 ms). No ST elevation suggestive of left ventricular aneurysm formation was noticed. The P2Y12 inhibitor was switched from ticagrelor to prasugrel, and 24-hour ambulatory electrocardiography revealed frequent premature atrial and ventricular complexes. Transthoracic echocardiography showed a hypokinetic posteroinferior wall with a mildly reduced ejection fraction of 45% (Simpson’s method), moderate mitral regurgitation (vena contracta, 0.6 cm; proximal isovelocity surface area, 0.7 cm; effective regurgitant orifice area, 34 mm2), and mild pericardial effusion.\n\nFurosemide was added to lisinopril and metoprolol, but only mild to moderate improvement of the patient’s symptoms was noticed before he deteriorated. Furthermore, up-titration of these medications was difficult because of the borderline arterial pressure. The addition of ivabradine and the switch from ticagrelor to prasugrel did not change his clinical status. Ticagrelor may cause transient dyspnea in some patients, which reverses with discontinuation.3\n\nForty days after the myocardial infarction, the patient was readmitted because of exertional dyspnea and worsening shortness of breath. New transthoracic echocardiography showed a dyskinetic cavity that was connected to the posteroinferior wall and communicated with the left ventricle, causing damage to the posterior mitral valve leaflet (P1 scallop) and resulting in severe mitral regurgitation. A color Doppler study confirmed passage of blood from the left ventricle into the cavity through an opening in the left ventricular wall. No thrombus formation was detected. The diagnosis of left ventricular pseudoaneurysm was made.\n\nUnfortunately, the patient died while waiting to transfer for surgical treatment.", "summary": "A 64-year-old man with a history of bipolar disorder and arterial hypertension was hospitalized for delayed presentation ST-elevation myocardial infarction. He was admitted to our hospital 24 hours after symptom onset. Diagnostic coronary angiography revealed 95% stenosis at the distal third of the right coronary artery, and he underwent a primary percutaneous coronary intervention to the culprit lesion. Despite administration of a diuretic and optimization of other pharmaceutical treatment, his heart failure deteriorated. Electrocardiography showed a sinus rhythm with Q-wave formation in the inferior wall leads (II, III, aVF), T-wave inversion in the same leads, and borderline QT prolongation (QTc of 490 ms). No ST elevation suggestive of left ventricular aneurysm formation was noticed. Forty days later, cardiac ultrasound revealed a dyskinetic cavity (pseudoaneurysm) in continuity with the posterior–inferior wall of the myocardium, resulting in severe mitral valve regurgitation. Unfortunately, the patient died while awaiting surgical treatment.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_96_en.txt", "fulltext": "A 64-year-old male initially presented with painless hematuria in September 2017 and was treated by a partial cystectomy as stage I BC. The patient showed local recurrence in August 2018 and underwent transurethral resection of bladder tumor (TURBT) followed by regular intravesical chemotherapy with epirubicin after TURBT. In March 2020, the patient approached to our hospital complaining about severe anorexia and fatigue. Subsequent FDG-PET/CT scan showed bladder recurrence with local invasion of the adjacent right lower ureter, resulting in the upper urinary tract dilation. Multiple lymph node metastasis, liver metastasis and lumbar metastasis (L5) were also identified . Ureteral stent placement was performed due to obstructive renal insufficiency and hepatic biopsy confirmed metastatic high-grade urothelial carcinoma with positive PD-L1 expression on 1% of the tumor cells. Then, next-generation sequencing (NGS) by a commercial laboratory (Genecast Biotechnology Co., Ltd, Jiangsu, China) identified ERBB2 p.V842I in both circulating tumor DNA (ctDNA) and biopsy sample with an allele frequency of 3.94% and 26.07%, respectively .\nThe patient received one cycle of palliative reduced-dose albumin-bound paclitaxel due to elevated serum creatinine level (Scr 204μmol/L). The tumor biomarker CA199 was even higher after first cycle chemotherapy without any relief of his symptoms. Based on his NGS result, pyrotinib (400mg orally daily) was started from April 10, 2020. Within one month, his tumor markers dramatically improved with remission of clinical symptoms . Due to renal dysfunction, the patient performed FDG-PET/CT scan for treatment efficacy assessment, which showed significantly decreased FDG uptake of multiple metastatic sites in May 2020 . Even though the overall response was considered stable based on the RECIST 1.1 criteria, the decrease in FDG uptake and remission of clinical symptoms indicated the patient responded well to the HER2 targeting therapy. However, he developed G3 diarrhea and terminated the medication on May 20, 2020 (The pyrotinib treatment lasted for 40 days). The third-line treatment with the PD-1 inhibitor tislelizumab (200mg every three weeks) was next started but the disease progressed after 2 months . ctDNA was collected in August 2020 and ERBB2 p.V842I with an allele frequency of 2.06% was identified by NGS. Ureteral stent removal was performed with improved renal function. Four cycles of PD-L1 inhibitor durvalumab, oxaliplatin and gemcitabine were administered, but level of CA199 gradually increased. NGS was performed again showing the same ERBB2 mutation with an allele frequency of 1.58%. Reduced dose of pyrotinib (240mg orally daily) was added, the patient’s tumor again responded with significant improvement of CA199 within 20 days. Dynamic monitoring of the ctDNA again confirmed ERBB2 p.V842I by NGS with a lower allele frequency of 0.41% . The regimen was generally tolerated this time but the tumor marker gradually increased in this patient. PET/CT evaluation revealed disease progression in March 2021, and the treatment was then discontinued . The length of time for reduced pyrotinib therapy was 4.5 months. The patient passed away on July 1st, 2021, and the post-recurrence survival was 16 months.", "summary": "We described a patient with metastatic bladder urothelial carcinoma (BUC) carrying a HER2 V842I mutation both in circulating tumor DNA (ctDNA) and biopsy sample. The patient was then treated with a HER2 tyrosine kinase inhibitor, pyrotinib, and responded well. However, the targeting treatment was terminated due to G3 diarrhea. Reduced dose of pyrotinib was later added to late-line treatment, the patient's tumor again responded with a significant decrease in CA199.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_981_en.txt", "fulltext": "A 47-year-old man was initially diagnosed as dysphagia. On the next day, he developed dysarthria and tongue deviation to the left, and he visited our hospital. He had no history of trauma nor any other significant medical history. Blood pressure was 184/126 mmHg. Neurological examinations revealed leftward deviation of the tongue on protrusion and dysarthria, suggesting a left hypoglossal nerve palsy. There were no other neurological deficits. Diffusion-weighted magnetic resonance images (MRI) did not demonstrate any lesions that could cause left hypoglossal nerve palsy . Axial T2-CUBE MRI demonstrated a false lumen of ICAD that compressed the outlet portion of the hypoglossal nerve tube and dilation of the perineural space in the hypoglossal canal . The false lumen of ICAD was adjacent to the hypoglossal canal at its distal side and protruded toward the hypoglossal neural tube. These findings indicated that direct compression by the false lumen was the cause of hypoglossal nerve palsy . MR angiography (MRA) showed dissection of the left ICA accompanied with a false lumen . The patient was admitted and we started transoral administration of amlodipine (5 mg/day) and azilsartan (20 mg/day) for antihypertensive treatment. On the 2nd day, he felt headache, and transoral administration of loxoprofen was started. On the 7th day, he was started on aspirin (100 mg/day) and clopidogrel (75 mg/day) for prevention of thromboembolism from the dissected portion. Cerebral angiography performed on the 8th day showed an ICAD consistent with the findings on MRI and MRA . Cone-beam computed tomography (CBCT) showed protrusion of the false lumen to the hypoglossal canal .\nAlthough medical treatment was continued, his headache worsened, and the neurological symptoms were not improved. Therefore, CAS was performed on the 19th day to thrombose the false lumen and decompress the hypoglossal nerve. A 6Fr guiding catheter (Axcelguide MSK, Medikit, Tokyo, Japan) was inserted through the right brachial artery and a 4-6Fr catheter (Dymon catheter, Silux, Saitama, Japan) was placed in the left common carotid artery. An embolic protection device (FilterWire EZ, Boston Scientific, MA) was advanced through the lesion and a filter was deployed in the ICA at the petrous portion. A carotid stent (Wallstent, Boston Scientific) was advanced to the dissected portion and deployed. Angiography immediately after stenting showed dilatation of the true lumen and congestion of contrast medium in the false lumen . There were no neurological symptoms or vital changes throughout the procedures. Pre- or post-dilation was not performed because the vessel was sufficiently dilated and there was a risk of enlargement or rupture of the false lumen.\nHis headache gradually improved after CAS and disappeared on the 25th day. His postoperative course was uneventful and he was discharged on the 26th day. No improvement of hypoglossal nerve palsy was observed at the time of discharge. At 1 month after discharge, neurological symptoms, such as tongue deviation and dysarthria, improved. Cerebral angiography performed at month 6 showed well-dilated ICA and disappearance of false lumen .", "summary": "A 47-year-old man presented with headache, dysphagia, dysarthria, and tongue deviation to the left. He had no history of trauma nor any other significant medical history. Axial T2-CUBE MRI and MRA showed dissection of the left ICA accompanied with a false lumen. These findings indicated that direct compression by the false lumen was the cause of hypoglossal nerve palsy. Although medical treatment was continued, symptoms were not improved. Therefore, CAS was performed to thrombose the false lumen and decompress the hypoglossal nerve. His symptoms gradually improved after CAS and angiography performed at month 6 showed well-dilated ICA and disappearance of false lumen.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1626_en.txt", "fulltext": "The case in question concerns a 64-year-old Caucasian postmenopausal woman presenting with pain, abdominal distension, and an altered intestinal transit.\nThoracic and abdominopelvic computed tomography (CT) scans revealed massive peritoneal carcinomatosis, extensive ascites, involvement of the major omentum, extensive involvement small intestine mesentery, diffuse involvement of the parietal peritoneum, implants at the bottom of the Douglas pouch, and probable implants on the ovarian surface, although of normal size . Her plasma CA-125 tumour marker levels were 1,110.9 U/mL, and those of the remaining tumour markers fell within a normal range. An omentum biopsy was compatible with high-grade serous carcinoma, with positive expression for WT-1, p53, CK-7 and negative expression for CK20 and TTF-1, with tube-ovarian-peritoneal origin.\nGiven the confirmed diagnosis of a primary peritoneal carcinoma of gynaecological origin stage IIIC and after assessment by a multidisciplinary committee that considers unresectable disease due to mesenteric involvement, neoadjuvant chemotherapy with intravenous (i.v.) paclitaxel 175 mg/m2 and i.v. carboplatin AUC 6 at three-week intervals was prescribed. The patient received three cycles of this treatment, showing a significant partial response on the follow-up CT scans, and plasma CA-125 tumour marker levels of 65 U/mL (levels at diagnosis: 1110.9 U/mL).\nSurgery was subsequently performed, achieving optimal cytoreduction, and the patient had a catheter placed for the administration of the intraperitoneal chemotherapy. The pathological study of all samples obtained during the procedure revealed a high-grade serous carcinoma.\nFollowing the intervention, a total of four cycles of adjuvant intraperitoneal (i.p.) chemotherapy consisting of i.v. paclitaxel 175 mg/m2 (day 1), i.p. cisplatin 75 mg/m2 (day 2), and i.p. paclitaxel 60 mg/m2 (day 8) were administered. The patient achieved a complete response, although she developed grade-2 asthenia and grade-3 neutropenia due to treatment-related toxicity that mandated a delay in the dosing and support with granulocyte-colony stimulating factors (G-CSFs).\nSeven months after receiving the last cycle of platinum-based chemotherapy, her CA-125 tumour marker levels were 104 U/mL, and mediastinal lymph node and peritoneal recurrence were identified in the follow-up CT scans. At this time, a germline mutation study was carried out in BRCA, being negative. Given that these findings were considered to be indicative of progression after a PFI of seven months, second-line chemotherapy was started with i.v. trabectedin 1.1 mg/m2 (day 1) in combination with i.v. PLD 30 mg/m2 (day 1) at 21-day cycles. The patient achieved a partial response after the third cycle (CA-125: 11.8 U/mL) .\nChemotherapy with trabectedin and PLD was continued; however, given that the patient experienced haematological toxicity (grade-4 neutropenia), the dose of PLD had to be reduced to 25 mg/m2 and that of trabectedin to 0.9 mg/m2 during the sixth cycle. PLD was subsequently discontinued altogether due to the onset of toxicity associated with this drug (grade-2 mucositis and grade-3 asthenia). From then on, she received i.v. trabectedin 0.9 mg/m2 in monotherapy at 21-day cycles, with good tolerance and referring only grade-1 asthenia that resolved within two to three days and did not limit her during her basic activities of daily living.\nAfter ten cycles of treatment, and specifically four cycles of trabectedin in monotherapy, the dose of trabectedin had to be reduced to 0.75 mg/m2 every 21 days due to the onset of grade-3 neutropenia and grade-2 thrombopenia, which mandated frequent delays in the dosing and even G-CSF support. Nevertheless, the subsequent follow-up CT scans performed periodically continued to show evidence of complete response. The patient received a total of 35 cycles of trabectedin (six in combination with PLD and then 29 in monotherapy). During this time, she maintained an excellent quality of life, experiencing no late or cumulative toxicity, and being able to perform her usual activities.\nBecause peritoneal progression was subsequently detected after a PFI of three years, a third line of treatment with i.v. carboplatin (area under the curve [AUC] = 4) (day 1), i.v. gemcitabine 1,000 mg/m2 (days 1 and 8 of every 21-day cycle), and i.v bevacizumab 15 mg/kg (day 1) was started. A subsequent follow-up CT scan carried out after the third cycle of this therapy showed signs of partial response. Given that the response persisted after six treatment cycles, chemotherapy was suspended and treatment with bevacizumab in monotherapy was started. The patient has received a total of 12 cycles of maintenance treatment with bevacizumab in monotherapy to date, having tolerated the treatment well and maintained a partial response.", "summary": "A 64-years-old postmenopausal woman with pain, abdominal distension, and an altered intestinal transit and with partially platinum-sensitive recurrent ovarian cancer, was successfully treated with a second line of trabectedin chemotherapy in combination with PLD, followed by trabectedin in monotherapy. This case proves the effectiveness of the combination of trabectedin and PLD and demonstrates how the administration of trabectedin, even in monotherapy, allows to maintain an adequate clinical response with good tolerance to the treatment during more than two years of drug administration.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_471_en.txt", "fulltext": "A 72-year-old woman (40.3 kg, 139 cm) was referred to our pain clinic for the treatment of low back pain after two back surgeries. She had first undergone back surgery [posterior lumbar interbody fusion (PLIF at L4–5) plus spinal stabilization (L3)] for lumbar spinal canal stenosis 4 years prior to the current presentation. Two weeks after the initial surgery, she underwent reoperation because of screw placement errors, and her symptoms disappeared after surgery. However, her low back pain recurred after a fall 5 months before the current presentation. Paralysis of the lower limbs was not apparent after this episode. A sensory disturbance that had existed before the surgeries remained unchanged. No new lesions such as a lateral recess or foraminal stenosis, herniated nucleus pulposus, or fracture were found on radiographs or magnetic resonance images . The previous medical institution prescribed acetaminophen and tramadol for low back pain and performed a caudal epidural block with 5 ml of 1% lidocaine and dexamethasone 1.65 mg. However, neither treatment provided pain relief, and the patient was referred to our pain clinic. She had several comorbidities, including diabetes mellitus, hypertension, renal dysfunction, hypothyroidism, rheumatic arthritis, and gastroesophageal regurgitation, and was prescribed 27 different drugs by clinicians from five different facilities. Accordingly, we decided not to use additional medication for first-line therapy because of polypharmacy concerns and renal dysfunction and performed bilateral ESP block with the patient in the prone position. A convex type transducer was placed in a longitudinal orientation at the level of the L2 transverse process, 3 cm lateral to the midline. The L4 and L5 transverse processes could not be identified because of the echogenic artifacts due to the surgical instruments. The posterior surface of the L2 transverse process was identified using an aseptic technique. After the puncture point was anesthetized with 2 ml of 1% lidocaine, the needle was inserted in the plane of the ultrasound beam in a cephalad to caudal direction. Following confirmation of the needle tip on the surface of the transverse process of L2, 20 ml of 0.1875% ropivacaine (fourfold dilution of commercial product) was injected into the target plane between the erector spinae muscles and the transverse process . This procedure was repeated on the contralateral side. Twenty-five minutes later, the patient reported a warm feeling in her low back and almost complete relief from pain, which was approximately < 10% of its original severity. There was an area of diminished cold sensation extending from T12 to L5, with no change in the anterior and lateral abdomen. Unfortunately, the pinprick test was not performed. Pain relief lasted for approximately 10 h after the initial block. We repeated this procedure for a total of three times in a month. Finally, the patient reported that her daily baseline level of low back pain had diminished to < 40% of its original severity. She was satisfied with the extent of pain control and did not wish to undergo further treatments such as epiduroscopy or spinal cord stimulation (SCS).", "summary": "Here we report a case involving a 72-year-old woman who experienced recurrent low back pain after undergoing two back surgeries. She was treated with erector spinae plane (ESP) block, which affected the dorsal rami of the spinal nerves from T12 to L5. Pain relief lasted for approximately 10 h after the initial block, and successful low back pain relief was achieved after a total of three trials.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2048_en.txt", "fulltext": "A 57-year-old man was referred to neuro-oncology for brain MRI findings concerning for cortical laminar necrosis in the setting of metastatic melanoma. He was originally diagnosed with Stage IIIB (pT4bN1aM0) BRAF V600E-mutated cutaneous melanoma 6 years before evaluation with positive sentinel lymph node biopsy. Brain MRI at that time was normal. He had received multiple treatments throughout the years but for the past 30 months had been treated only with encorafenib and binimetinib with stable systemic disease. His medical history was otherwise unremarkable and he had no risk factors for vascular disease.\nRoutine, asymptomatic surveillance brain MRI with contrast was obtained and was notable for gyriform T1 shortening in the right pars opercularis, new from most recent imaging performed 23 months prior . Contrast-enhanced T1-weighted imaging showed no discernable enhancement, T2-weighted sequences showed no local edema, and diffusion-weighted imaging showed no diffusion restriction. These findings were most consistent with cortical laminar necrosis from chronic infarction. Nonetheless, given his clinical history and lack of vascular risk factors, metastatic disease remained a diagnostic consideration and close interval follow-up was planned.\nRepeat MRI obtained 3 months later revealed gyral expansion in the region of the T1 shortening as well as a new contrast-enhancing adjacent nodule with vasogenic edema . Given imaging features, he underwent craniotomy revealing a subpial lesion that was grossly melanotic. The lesion followed and infiltrated gray matter in a gyriform pattern, with an adjacent melanotic nodule . A total resection was achieved utilizing subpial dissection to protect nearby Sylvian vessels. The surgical specimen showed cortical gray matter with infiltration by neoplastic cells in a primarily perivascular pattern with no definitive tumor mass. The neoplasm was composed of large cells with atypical nuclei and prominent nucleoli. The majority of the tumor cells showed melanin pigment. All these findings were consistent with a metastatic malignant melanoma. The tumor expressed the mutant BRAF V600E protein by immunohistochemistry. The patient recovered well without postoperative neurological deficit. He received Gamma Knife radiosurgery to the surgical bed with a dose of 9 Gy at the 50% isodose line.", "summary": "Here, we present a case of a 57-year-old man in whom a metastatic melanoma initially mimicked the imaging characteristics of cortical laminar necrosis.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_6_en.txt", "fulltext": "A 24-year-old female presented with a 1 year history of a painful, large, irregular, lobular mass rapidly growing at the nape of the neck, accompanied by decreased sensation/numbness in the C1-C2 dermatomes . Computed tomography (CT) scans of both the head and neck revealed a moderately enhancing 10-12 cm lesion (in greatest diameter) extending from skull base to the thyroid gland. It additionally involved the posterior cervical muscles, eroded through the posterior arch of atlas and occipital bone, where it exhibited both extradural/intraspinal, and intracranial extension . An incisional biopsy documented a highly vascular lesion consistent with a cellular variant of a solitary fibrous tumor (SFT) with HPC-like features. Preoperative RT was administered to reduce the size and vascularity of the tumor, and was followed by aggressive subtotal resection of an encapsulated, soft, vascular tumor. Notably, radical resection was limited by the tumor's depth and vascularity. Further postoperative RT resulted in a good clinical outcome.\nHistopathology revealed a vascular neoplasm characterized by sheets/groups of spindle/oval cells with a stag horn pattern consistent with HPC located in between plentiful of thin-walled vessels . On immunohistochemistry, consistent with the diagnosis of HPC, the tumor cells were positive for both cluster of differentiation 34 (CD34) and vimentin . The postoperative CT scan documented only a minimal amount of residual tumor (near the atlanto-occipital junction) with near complete decompression of the craniovertebral (CV) junction . Two years postoperatively, the patient remains asymptomatic and disease free (e.g., no recurrence or metastases).", "summary": "We report a case of a 24-year-old female with a rapidly growing, highly vascular swelling in nape of the neck extending deep into the craniovertebral (CV) junction accompanied by extradural/intraspinal, and intracranial involvement. An incisional biopsy revealed a cellular, highly vascular tumor with HPC-like features. The patient received preoperative RT, which reduced both the size and vascularity of the lesion, facilitating subsequent near complete resection. Further postoperative RT resulted in a good clinical outcome, with no tumor recurrence observed at 2 postoperative years.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_520_en.txt", "fulltext": "A 56-year-old woman was referred to the emergency department due to a severe headache in the frontal area for 2 days before admission. The patient had nausea and vomiting in the morning but had no history of seizures or decreased consciousness. The examination of neurological symptoms was completely normal and showed no symptoms of meningeal irritation. The patient's vital signs were recorded as follows: blood pressure (BP)=130/85 mmHg, heart rate (HR)= 86/min, and temperature (T)=37.3 °C. In her past history, the patient had undergonetympano- mastoidectomy surgery and resection of the cholesteatoma 1 week earlier, and a canalwall down mastoidectomy (CWD) was performed due to right ear cholesteatoma. According to the information obtained from the previous surgeon, microtrauma was inflicted in the dural plate during surgery, but since no significant cerebrospinal fluid (CSF) leak occurred during the procedure, reconstruction was not necessary. After admission, examination and initial evaluation, the Mount Fuji sign was found on the brain CT scan of the patient, but no evidence of brain abscess or intracranial hemorrhage. The patient was immediately admitted to the intensive care unit (ICU).\nInitial treatments such as CBR, 30-degree head elevation, anti-fever therapy, analgesics and oxygen therapy, along with anti-compulsive drug (phenytoin), were prescribed. The patient did not undergo surgery due to a lack of neurological symptoms and a decreased level of consciousness or seizures. However, in the ICU, the patient was maintained under regular and continuous monitoring of vital and neurological signs and level of consciousness. The day after admission, the patient’s headache had completely resolved. The patient was admitted to the ICU for 5 days and was monitored for volume ofpneumocephalus every day with a CT scan. At the end of 5 days, the patient's pneumocephalus was resolved completely, and she was transferred to the ward. The patient was discharged after complete recovery.", "summary": "A 56-year-old woman was referred to the emergency department due to a severe headache in the frontal area for 2 days before admission. The patient experienced nausea and vomiting in the morning and had no history of seizures or decreased consciousness. Examination of neurological symptoms was completely normal and showed no symptoms of meningeal irritation. In terms of past history, the patient had undergone tympanomastoidectomy surgery and resection of the cholesteatoma 1 week previously. The Mount Fuji sign was found on the brain computed tomography (CT) scan of the patient. Treatments such as CBR (complete bed rest), 30-degree head elevation, anti-fever, analgesics and oxygen therapy, along with anti-compulsive drug (phenytoin), were prescribed. At the end of 5 days, the patient's pneumocephalus was resolved completely.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2019_en.txt", "fulltext": "A 74-year-old previously healthy man was referred to our institution for repeated palpitations over 6 months. A Holter electrocardiogram (ECG) from the local hospital showed persistent AT. No oral anti-arrhythmia drugs were prescribed. The initial workup included baseline ECG, routine blood tests, chest X-ray, echocardiogram, and coronary angiography. There was frequent unifocal initiating AT with a P-wave morphology of positive amplitude in I, II, III, aVF, and V1 .\nThe patient underwent an electrophysiological study and ablation. Endocardial mapping of the atrium was performed using a PentaRay catheter (Biosense Webster, Inc., CA, USA) and a smart-touch contact force (CF)-sensing catheter (Biosense Webster, Inc., CA, USA) with the CARTO mapping system (Carto, Biosense Webster). Spontaneous AT was identified and mapped throughout the procedure using activation mapping, voltage mapping, and ripple mapping. Intracardiac electrophysiology during AT [cycle length (CL) 184 ms] showed atrial signals with an eccentric pattern of atrial activation on the coronary sinus catheter . Further activation mapping showed the earliest activation zone of AT originated in the right atrial FO. Ripple mapping showed activation progressing from the FO to both the right atrium and left atrium . Voltage mapping revealed no low-voltage regions in either atrium .\nIn the FO, further high-density mapping (HDM) using the PentaRay catheter and point-to-point mapping using the CF-sensing catheter (Biosense Webster, Inc., CA, USA) demonstrated significantly long-duration fractionated electrograms covering a considerable percentage of the CL of the tachycardia within a localized region . About 107 points at 12 cm2 were taken for the map. The colours of the wavefront on the activation maps can be tracked by following the regions where red meets purple . Concealed entrainment was fulfilled only at the FO, which implied the tachycardia mechanism is micro-reentrant in nature.\nA radiofrequency catheter ablation (RFCA) current was delivered at 30 W in power-controlled mode at 43°C and a normal saline velocity of 17 mL per minutes. The RFCA was delivered to the middle of the micro-reentrant circuit, terminating the AT immediately after 3 s (, Video S2). No AT was induced during programme stimulation and isoproterenol infusion (10 µg/min). The patient had no further episodes of AT over the ensuing 12 months until he was discharged to his home without antiarrhythmic drugs.", "summary": "A 74-year-old man presented with paroxysmal atrial tachycardia (AT) presenting as palpitations. He was evaluated by an electrophysiological examination using a high-density CARTO mapping system. The mapping results showed the AT with a cycle length of 184 ms was focused on his right atrial fossa ovalis (FO). In this small area, the high-density mapping demonstrated a significant micro-reentrant tachycardia. Radiofrequency ablation at the centre of the micro-reentrant circuit successfully terminated the AT. No recurrences were observed during a 12-month follow-up.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2325_en.txt", "fulltext": "The patient was an 84-year-old man who was admitted to the emergency room with a 24-h history of acute colicky abdominal pain. He denied other signs or symptoms. The relevant surgical history included old appendectomy and inguinal hernioplasty. The patient's medical history includes coronary artery disease and atherosclerosis, despite an otherwise healthy status. The patient was hemodynamically stable and in good general condition. Abdominal palpation led to pain in the left lower abdominal quadrant with no signs of peritoneal irritation. Abdominal computed tomography (CT) revealed small-bowel loops with multiple air-fluid levels and dilated stomach with a large air-fluid level . Laboratory tests showed no significant alterations.\nDecompression with a nasogastric tube was performed, and 1250 mL of light yellow fluid was evacuated. However, the symptoms persisted and he was admitted to the surgery department for further diagnostic testing, observation, and treatment. Despite stable vital signs, the patient experienced mild residual pain during palpation without abdominal distension. The patient stated that he periodically experienced mild abdominal pain for most of his adult life. Examination with an oral water-soluble contrast revealed small-bowel obstruction which remained unresolved over several hours of observation, leading to the suspicion of adhesive small-bowel obstruction . After receiving information regarding diagnostic laparoscopy and the potential for further surgical treatment based on intraoperative evaluation, the patient provided consent to the procedure.\nLaparoscopy revealed multiple diverticula in the proximal small intestine with distinctly dilated jejunal loops. Given the extent of the disease and the high iatrogenic injury risk, the procedure was converted to open surgery. A midline laparotomy was performed, and chronic rotation of the mesentery (volvulus) was observed. Derotation of the small intestinal loops was performed. Further examination revealed extensive diverticulosis in the proximal 2-m section of the jejunum starting approximately 20 cm from Treitz's ligament . Chronic fibrous adhesions and strictures were also observed in the mesentery and the small intestine. The affected small bowel was resected to prevent recurrent volvulus and other complications, such as diverticulitis, perforation, and bleeding. Following the resection of the jejunal segment with multiple large diverticula, side-to-side entero–entero anastomosis was performed. No diverticula were observed in the remaining bowel.\nThe postoperative period was uneventful, and the patient was discharged from the hospital on postoperative day 6. The histopathologic evaluation of the resected material confirmed substantial jejunal diverticulosis with chronic fibrosis, adhesions, and strictures.", "summary": "An 84-year-old man was admitted to the emergency room with a 24-h history of acute colicky abdominal pain. He denied other signs or symptoms. The preoperative diagnosis based on physical and radiologic evaluations was challenging, and only diagnostic laparoscopy revealed extensive small-bowel diverticulosis. Midline laparotomy was performed as definitive surgery, revealing diverticulosis in the proximal 2-m section of the jejunum, starting approximately 20 cm from Treitz's ligament; the affected section was resected. The postoperative recovery was excellent. The histopathologic report confirmed substantial jejunal diverticulosis with chronic fibrosis, adhesions, and strictures.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2962_en.txt", "fulltext": "A 52-year-old female was referred to our ophthalmology clinic with decreased vision and increased tearing.\nShe was diagnosed with stage III Alport syndrome two years prior which was currently stable per her nephrologist. During these two years had gradual decline in her visual acuity that was not correctable to 20/20.\nShe had a past medical history of asthma, diabetes mellitus, and hypertension.\nFamily history was positive for Alport syndrome in her mother and brother but without any known ocular or hearing abnormalities.\nOn initial examination , uncorrected distance visual acuity was 20/60 on the right and 20/30 on the left, with a corrected visual acuity of 20/30 bilaterally.\nSlit lamp examination showed bilateral eyelid laxity, papillary conjunctival changes, prominent nerves, superficial punctate keratitis, unilateral (OD) anterior basement membrane changes, with no guttata, apical scarring, or corneal striae. Dilated fundus exam revealed bilateral floaters in the vitreous humor and normal retinal vasculature.\nInitial Pentacam (Oculus, Wetzlar, Germany) tomography revealed mean keratometry of 47.9 D in the right and 48.3 D in the left, with a 2 mo follow-up scan revealing a mean keratometry of 48.1 D and 48.5 D respectively . During this 2 mo period progressive corneal thinning occurred bilaterally. There was also bilateral anterior curvature steepening with no appreciable changes in the posterior curvature.", "summary": "A 52-year-old female was referred to our ophthalmology clinic with decreased vision and increased tearing. She was diagnosed with stage III Alport syndrome two years prior. Upon examination she was found to have average keratometries of 48 D bilaterally with tomographic evidence of keratoconus.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1676_en.txt", "fulltext": "A 52-year-old female presented to our hospital with blurred vision in her left eye for approximately 1 wk.\nThe patient reported no headache or eye pain. She had no other diseases.\nUnremarkable.\nNo abnormalities were found on slit lamp examination.\nThe patient underwent a comprehensive ophthalmic examination, including decimal best corrected visual acuity, color fundus photography, spectral domain optical coherence tomography (OCT) and fluorescein fundus angiography. On first examination, her best corrected visual acuity was 20/40 in the left eye and 20/20 in the right eye. Anterior segment and intraocular pressure findings were normal in both eyes. Fundus photography of the left eye showed a seemingly normal adult oculus fundus without any obvious hard exudate or hemorrhage . OCT demonstrated a hypo-reflective space beneath both the neurosensory retina and the pigment epithelium layer . The late phase of fluorescein angiography revealed hyper fluorescence . On the basis of these findings, a diagnosis of CSC was made. As the patient lived thousands of miles from Shanghai and did not perceive obvious changes in her eyes, she declined follow-up visits to the hospital.\nOne month later, her visual acuity deteriorated suddenly. On examination, her best corrected visual acuity in the left eye was 20/100. A sub-retinal hemorrhage, hard exudate and reddish-orange nodules were found on fundus photography . OCT demonstrated a pigment epithelium detachment and sub-retinal fluid . The late phase of fluorescein angiography revealed increased hyper-fluorescence compared to that observed one year previously . These characteristics led to the diagnosis of PCV.", "summary": "A 52-year-old woman presented to our hospital with blurred vision in her left eye for approximately 1 wk. Anterior segment and intraocular pressure findings were normal in both eyes. Fundus photography of the left eye showed a seemingly normal adult oculus fundus without any obvious hard exudate or hemorrhage. Optical coherence tomography exhibited a hypo-reflective space beneath both the neurosensory retina and the pigment epithelium layer. The late phase of fluorescein angiography revealed increased leakage. The patient was initially diagnosed with CSC. At follow-up, however, the final diagnosis turned out to be PCV.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_3327_en.txt", "fulltext": "Mr. RR, a 23-year-old male, presented at the emergency room reporting a week-long period of weakness that had worsened prior to admission, significantly impeding his daily activities. He described a four-month history of recurring, small fluid-filled sacs on the skin. These lesions evolved from reddish patches to rupturing and oozing, accompanied by itching but no pain or fever. He denied experiencing chest pain, shortness of breath, or vomiting but mentioned recent episodes of nausea. Upon examination, he appeared moderately unwell, with normal vital signs and pale conjunctiva. Physical assessment revealed palpable splenomegaly (Schuffner 6) but no lymph node enlargement or cardiac murmurs. Dermatologic examination revealed oedematous urticaria-like plaques, scaling, erosions, excoriations, and crusts all over the body, as well as a ruptured hemorrhagic bulla on the anterior body and left leg.\n\nComplete blood test showed that the White Blood Cell Count (WBC) was 23 × 109/l, the Absolute Neutrophil Count (ANC) was 644 cells/μl, the Hemoglobin (Hb) was 99 g/, and the Platelet Count (Plt) was 28 × 109/l. Bone marrow aspiration consistent with acute lymphoblastic leukemia-L1, led to the diagnosis of acute lymphoblastic leukemia in the patient. The decision for chemotherapy has not been approved by the patient, so patient only received corticosteroids and supportive therapy. At the onset of therapeutic intervention, the patient presented with erythematous patches accompanied by vesicular lesions spanning almost the entirety of his body. Subsequently, platelet concentrate transfusions were administered on the fourth and sixth days of treatment, with a total of eight bags administered on each occasion. On the eighth day of treatment, a histopathological exam showed that the patient had bullous pemphigoid because there was necrotic epithelium and chronic inflammatory cells. The decision to forgo direct immunofluorescent assay (DIF) was predicated upon the patient’s existing thrombocytopenia and heightened vulnerability to bleeding complications. Subsequently, on the twelfth day of treatment, the patient underwent a transfusion of one unit of packed red cells (PRC). Following this, on the thirteenth day of treatment, the patient received two units of PRC and eight units of platelet concentrates. On the fourteenth day of treatment, the patient experienced an abrupt loss of consciousness, preceded by a severe headache and anisocoric pupils. Laboratory findings revealed a post-transfusion platelet count of 7000/ul after eight bags of platelets were administered. Despite continued therapeutic interventions.", "summary": "A 23-year-old male with ALL-L1 diagnosis who developed bullous pemphigoid in this report. The patient exhibited typical ALL-L1 symptoms, including constitutional manifestations and signs of bone marrow compromise. Dermatological assessment revealed extensive edematous urticaria-like plaques, erosions, excoriations, crusts, and a hemorrhagic bulla. Severe thrombocytopenia was evident in laboratory tests, with histopathological examination confirming bullous pemphigoid. Despite aggressive treatment, including platelet transfusions, the patient's condition worsened.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_2870_en.txt", "fulltext": "A 30-year-old Korean woman was diagnosed with suspicious papillary thyroid carcinoma (PTC) by fine needle aspiration (FNA) at a local medical clinic. She was referred to our hospital for operation. She has no personal or family history of thyroid dysfunction and no specific medication history. Physical examination of neck revealed a diffusely enlarged and non-tender thyroid gland. There were no palpable cervical lymph nodes. US was carried out using a real time linear array 10-MHz transducer. The thyroid US demonstrated a diffusely enlarged thyroid gland with a 0.4×0.3cm sized hypoechoic nodule in the left thyroid . She wanted to diagnose the thyroid nodule again in our hospital. US-guided FNA was performed again on that nodule, and FNA cytology was diagnosed as suspicious PTC. We also examined for BRAF V600E mutation using FNA cytology materials. BRAF V600E mutation was identified by multiplex real time polymerase chain reaction assay using Anyplex™ BRAF V600E Real Time Detection (V2.0) (Seegene, Inc., Seoul, Korea), which allows for simultaneous amplification of total nucleic acid of V600E mutation of BRAF and internal control (human β globin gene). Computed tomography scan using contrast agent was done three weeks before operation. No abnormal finding was noted. The patient had no thyrotoxic symptoms during one month of the preoperative period. She underwent a left thyroid lobectomy by endoscopic thyroidectomy via a transaxillary approach for the thyroid tumor. The right thyroid was not manipulated during operation. Pathologic finding revealed a papillary carcinoma, and other portions of the thyroid parenchyma revealed lymphocytic patches predominantly in peripheral portions. There were rarely lymphoid follicles with germinal center .\nOn laboratory examination, the preoperative laboratory test was performed one month before operation was within normal limits. The serial change of thyroid function tests and the titer of autoantibodies were summarized in Table . On postoperative day (POD) 1, she didn’t definitive complain. A thyroid function test (TFT) showed hyperthyroid with Antithyroglobulin antibody and thyroglobulin (Tg) . On POD 8, the patient was consulted by the department of endocrinology. On physical examination, blood pressure was 120/80mmHg, and heart rate was 119beat/min and regular. Neck examination revealed no sign of tenderness and redness. TFT was still increased but Tg level was normalized . Tc-99m scintigraphy showed faint visualization of the right thyroid lobes, and 24-hour radioactive uptake of 131I was 0.7% , which suggested destructive thyroiditis. On POD 48, thyroid function tests revealed euthyroid. On POD 86, she complained of fatigue and weakness. A TFT revealed hypothyroidism . The patient was treated with levothyroxine for her symptoms of hypothyroidism. On POD 140, follow-up TFT revealed mild hyperthyroidism . The patient was treated with levothyroxine.", "summary": "A 30-year-old Korean woman was referred to our center for thyroid tumor operation. She was diagnosed with suspicious papillary thyroid carcinoma by fine needle aspiration at a local medical clinic. The thyroid ultrasonography demonstrated a diffusely enlarged thyroid gland with a 0.4×0.3cm sized hypoechoic nodule in the left lobe. Left thyroid lobectomy by endoscopic thyroidectomy was performed via a transaxillary approach, and the nodule was confirmed to be a papillary thyroid carcinoma. On postoperative day 1, a thyroid function test revealed hyperthyroidism, and on postoperative day 8, a thyroid function test again revealed hyperthyroidism with decreased radioactive iodine uptake. Thyroid function tests showed euthyroid on postoperative day 48 and hypothyroidism on postoperative day 86. She was treated with levothyroxine.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2466_en.txt", "fulltext": "A 55-year-old female patient was referred to the orofacial pain unit of the OPH clinic for showing symptoms of temporomandibular joint arthralgia and osteoarthritis, orofacial myofascial pain, and sleep bruxism. An experienced Orofacial Pain Specialist performed a comprehensive clinical history and examination following the Diagnostic Criteria for Temporomandibular Disorders (DC/TMD) protocol and the International Classification of Orofacial Pain-1 (ICOP-1) criteria. At the moment of the examination, the patient was taking 150 mg of Venlafaxine for major depressive disorder, 4 mg of Clonazepam for sleep onset insomnia, and hormone replacement therapy for menopause.\nAnother major complaint of the patient was difficulty initiating and maintaining sleep. Thus, an Epworth Sleepiness Scale and Insomnia Severity Index were performed to complete the sleep evaluation. Due to the high scores presented by the patient and clinical history consistent with chronic sleep insomnia, polysomnography (PSG) was requested Even though the PSG revealed no sleep breathing disorders, the exam showed severe modifications in the patient’s sleep architecture, a mild increase in the arousal index, and low-frequency sleep bruxism. The PSG results led us to hypothesize that the patient’s sleep architecture alterations were because of the chronic consumption of benzodiazepines. Thus, the patient was derived to her treating psychiatrist to gradually diminish and eliminate the intake of Clonazepam.\nDuring this time, the patient successfully underwent her osteoarthritis and orofacial myofascial pain treatment. In addition, an upper oral occlusal splint was prescribed to manage low-frequency sleep bruxism.\nIn order to manage sleep initiation and maintenance insomnia, the psychiatrist tapered the dosage of Clonazepam and progressively replaced it with Trazodone until a target dose of 100 mg was reached. Throughout this period, the patient did not experience any extrapyramidal symptoms.\nAlmost three months later, after the end of the orofacial pain treatment, the patient returned complaining of a sudden worsening of her awake bruxism. The patient presented abrupt rhythmic involuntary muscle contractions, similar to jaw-closing and jaw-deviating dystonic-like movements affecting the right side of her masticatory muscles. (See , and ) The dystonic-like episodes were spontaneous or triggered by everyday activities such as talking, chewing, and yawning. The severity of these motor contractions tended to be exacerbated by emotional stress and nervousness produced by these uncontrollable movements. During these episodes, some typical features of oromandibular dystonia were noticed, such as some sensory tricks (gentle pressure on the inferior incisors would milden the jaw-deviating dystonia as the placement of a tongue depressor between the molars for jaw-closing dystonia), and phenomena (e.g., oromandibular dystonia overflowing the right eyelid, resulting in right blepharospasm).\nA video examination protocol suggested by Yoshida was carried out to assess the type and magnitude of involuntary movements . In addition, a complete haematological and biochemical blood examination was required and all the parameters were within normal range. Thus, both thyroid and parathyroid hormonal dysfunction were ruled out.\nSince the literature indicates that Trazodone can induce extrapyramidal symptoms and abnormal oromandibular movement disorders only start after the addition of this medication. The authors, in agreement with her treating physician, suggested performing a slow taper until complete discontinuation of Trazodone was achieved.\nA progressive dose reduction of 25 mg of Trazodone was scheduled every two weeks. In the meantime, an inferior occlusal splint was prescribed, considering that mild contact in the inferior incisor tended to ease the intensity of the dystonic-like movements.\nDuring the progressive discontinuation of Trazodone, the authors noticed an immediate attenuation of the dystonic symptoms. However, only achieving a partial but clear remission of them. No video protocol was recorded during the tampering of Trazodone.\nConsidering that the patient’s mental health status was stable, it was decided to take a more conservative approach, only eliminating the most likely offending drug, namely, Trazodone. Consequently, the authors maintained that Venlafaxine intake was unchanged in order not to jeopardize the psychiatric treatment, and also closely monitored the evolution of the clinical symptoms. Additionally, the authors indicated the administration of 25 units of incobotulium toxin (Xeomin®, Merz, Germany) per masseter muscle, 20 units per temporalis muscle, and 15 units were applied to the inferior head of the right lateral pterygoid with electrical stimulation guidance (See anatomical landmarks in ).\nDuring the two-week follow-up consultation, the authors could recognize a substantial decrease in the magnitude and intensity of the oromandibular movement. Two months and seven months after the discontinuation of the offending drug and botulinum toxin application, the authors re-recorded Yoshida’s video protocol to carry out an objective before and after comparison of the treatment results. See for comparison prior to and after the treatment.", "summary": "The authors will present a clinical case and management, from the dental specialist perspective, of a 55-year-old female patient who developed tardive oromandibular dystonia induced by Trazodone prescribed for sleep insomnia.", "readability_score": 1, "difficulty": "hard" }, { "id": "multiclinsum_test_467_en.txt", "fulltext": "A 70-year-old woman was diagnosed as having PD at 65 years of age after having difficulty with walking and clumsiness with her hands. She also had olfactory impairment. She had no family history of PD. Examinations revealed asymmetric parkinsonism with limb rigidity and bradykinesia that were more prominent on the left. There were no cerebellar signs or gaze palsy. Her Hoehn and Yahr stage was 2 and her Mini-Mental State Examination score was 30. Magnetic resonance imaging of her brain revealed normal findings. She was initially treated with levodopa (L-dopa), (200 mg/day), which markedly improved her symptoms. A reduced meta-iodobenzylguanidine (MIBG) cardiac uptake on myocardial scintigraphy was detected. On the basis of these findings, she was diagnosed as having PD. At 67 years of age, she developed orthostatic hypotension and was treated sequentially with fluids, compression stockings, and midodrine. Her disease slowly progressed over the next three years, and L-dopa dosage was increased to 600 mg/day. At 70 years of age, she had a mild head trauma with loss of consciousness. One month later, she developed acute-onset lateral flexion of her trunk to the left side while standing, and she was admitted to our hospital. On admission, her neurological examinations revealed slight weakness of her left upper and lower extremities in both proximal and distal muscles, as indicated by her Medical Research Council (MRC) scale score of 5-/5. She showed a sustained 10.1°lateral flexion of her trunk to the left side on standing . This posture was alleviated in the supine position. Her Pisa angle was assessed using NeuroPostureApp© . Her right muscle strength was normal. Her sensory systems were normal and no cerebellar signs were detected. Her deep tendon reflexes were normal and her plantar reflex was flexor bilaterally. Her presurgical brain computed tomography (CT) showed CSDH in the right hemisphere resulting in a marked compression of the hemisphere . She was immediately referred to the neurosurgical department of our hospital and the subdural hematoma was successfully evacuated by single-burr-hole drainage. Her brain CT carried out four days after the operation showed improvement of her subdural hematoma . Seven days after her operation, her PS disappeared and she fully recovered to her preoperative level of function without any sequelae such as headaches and cognitive impairment caused by her CSDH. She is still being follow-up.", "summary": "A 70-year-old woman developed acute-onset lateral flexion of her trunk to the left side while standing, and she was admitted to our hospital. One month before, she had a mild head trauma with loss of consciousness. At 65 years of age, she noticed difficulty with walking and clumsiness with her hands. She was diagnosed as having PD (Hoehn and Yahr stage 2) and levodopa was initiated. Her symptoms were markedly improved. At 67 years of age, she developed orthostatic hypotension and was treated sequentially with fluids, compression stockings, and midodrine. Urgently performed brain computed tomography (CT) showed a CSDH in the right hemisphere resulting in a marked compression of the hemisphere. After surgical evacuation, her PS disappeared. She has fully recovered to her preoperative level of function.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2409_en.txt", "fulltext": "A 55-year-old man, with body mass index (BMI) of 18.36 kg/m2, was admitted into our hospital for left hip pain for 6 months. The symptom started with moderate pain after walking. Over the last few days, the pain got worsen with no remission after rest. The patient denied any history of trauma, glucocorticoid hormone consumption or radiotherapy. Examinations before admission revealed old fracture on the left femoral neck and an increase in blood uric acid. After admission, the function evaluations of his bilateral hip joint were summarized as follows: Visual Analogue Scale (VAS) score was 4, Harris score was 23 in the left hip joint, while VAS score was 0, Harris score was 90 in the right hip joint. The imaging examinations of the pelvis, including radiographs , CT and MRI scan , confirmed the existence of bilateral femoral neck fractures, with fracture nonunion and femoral neck shortening in his left femoral neck and double fracture lines in his right femoral neck. The Dual energy X-ray absorptiometry (DXA) examination of the lumbar spine showed reduced bone mineral density (BMD) and severe osteoporosis (L1–L4: BMD0.514 g/cm2, T score = − 4.6). In addition, tumor markers, tuberculosis antibodies, alkaline phosphatase and parathyroid hormone levels were found to be normal.\nWe recommended a left cementless THA, and a cannulated screws fixation on the right side. And the uric acid lowering treatment was performed on the patient. However, the patient only agreed to the left THA, while on the right side, conservative treatment was selected for patient because of financial reasons. The left THA through the lateral approach was performed for the patient under general anesthesia . During the surgery, we found sclerosis, fatty degeneration and necrosis of the fracture end of the left femoral neck . We used impactive bone grafting to strengthen the acetabular on the fracture region .\nAfter the operation, the patient was treated with 20 U Elcatonin injection (Sidinuo®, Shangdong Luye Pharmaceutical Co.LTD, Yantai, China) once a week, and 1 tablet of Calcium Carbonate D3 Granules (Langdi®, Beijing Kangyuan Pharmaceutical CO.LTD, Beijing, China) containing calcium carbonate (500 mg) and vitamin D (200 IU), twice a day. Meanwhile, He was required to exercise his quadriceps and calf muscle on bed, move with the help of wheelchairs, and avoid walking on crutches. The postoperative histological examination, as showed in Fig. , revealed that the left fracture gap was filled with fibrous tissues, with few new bone tissues and a large amount of necrotic bone tissue observed. Meanwhile, the plain radiograph of the pelvis one day after surgery revealed that the total hip replacement arthroplasty was very successful . Generally, the patient recovered well during the hospitalization and was successively discharged home.\nFour months after discharge, the radiograph image showed no signs of implant loosening or infection in his left hip . It was worth noting that CT images revealed fracture union of the right femoral neck, as shown in Fig. b and Fig. c. In addition, function evaluations of his bilateral hips at four months after surgery was satisfactory and summarized as follows: VAS score was 0, Harris score was 98 in the left hip joint and VAS score was 0, Harris score was 95 in the right hip joint.", "summary": "A 55-year-old male patient complained pain in his bilateral hip, with no history of trauma, glucocorticoid hormone consumption or radiotherapy, and imaging examination revealed fracture nonunion and shortening in his left femoral neck, and double fracture line on the right femoral neck. The patient received a cementless THA for the left femoral neck fracture and conservative treatment for the right side, followed by Elcatonin injection and oral administration of Carbonate D3 Granules. After 4 months of fellow-up, the patient presented improved functional scorings in bilateral hip joints, with no signs of prothesis infection or loosening.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2944_en.txt", "fulltext": "A 69-year-old hypertensive male was transferred to us with traumatic brain injury after sustaining a ground-level fall. On admission, his Glasgow Coma Scale was 12/15 (E4V2M6). CT scan depicted a left temporal hematoma with extensive acute subdural hematoma, diffuse edema and mass effect, and midline shift of 6.6 mm . He was admitted to the special care unit for observation where his GCS dropped to 9/15. He then underwent left-sided emergent DC and was discharged 4 weeks later. At the time of discharge, he was awake, alert, and intermittently following simple commands without gross motor or sensory deficits. His speech was slurred and intermittently, he had mildly agitated behavior (Richmond Agitation Sedation Scale or RASS + 1) with a Modified Rankin Scale of 4.\nOne week later, the patient returned to the hospital with progressively worsening agitation without a decline in consciousness. CT scan showed interval development of the right chronic subdural hematoma with interval resolution of the previous acute hematoma. The hematoma was evacuated through burr-hole craniotomy and drain placement. The procedure was uneventful but after the surgery, his agitation increased (RASS + 3) and cognitive dysfunction did not improve. Mini-Mental Score could not be assessed as his comprehension was not intact. At this point, patient was extensively evaluated for other causes of agitation such as electrolytes disturbances, endocrine abnormalities, infection, sepsis, drug intoxication, or alcohol withdrawal, all workup was within normal limits.\nOn serial CT scans, the right subdural collections decreased and his craniotomy skin flap started to sink. Suspecting “syndrome of trephined” and in view of his persistently unexplained behavioral dysfunction, we planned for early cranioplasty which was performed 6 weeks after the primary procedure . Within 48 h of cranioplasty, his agitation started to improve. On the 4th day, his RASS was + 1.\nAt 6-month follow-up, the patient was found able to walk unattended and only required minimal assistance with his activities of daily living (ADL) (MRS 3). His comprehension had also improved considerably and he was able to follow two-step commands with consistency.", "summary": "An elderly gentleman with traumatic brain injury underwent DC. He later developed a sinking flap and unexplained agitation which responded to cranioplasty by returning to a state of calm. His cognitive function further improved over a period of 6 months. This is an unusual observation reported in this case.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_57_en.txt", "fulltext": "A 73-year-old female complained of back pain and weakness in lower limbs for 2 weeks. 3 months ago, after a minor trauma, she got back pain without weakness in lower limbs. Radiography including lateral radiographs and MRI performed at a local hospital. Initially, she was diagnosed with a L1 compression fracture and accepted conservative treatment. After an asymptomatic period, she complained progressive pain at the fracture position with weakness of both lower limbs and was referred to our hospital with suspicion of Kümmell’s disease. The back pain evaluated by visual analog scale (VAS) scale was 9. According to American Spinal Injury Association (ASIA) grading criteria, the neurological function was rated as ASIA C. Sagittal MR images showed a fluid-containing IVC with high-signal intensity on T2-weighted images and STIR MR sequences at L1 and sagittal reconstruction CT scan showed a linear radiolucent IVC, accompanying spinal cord compression. The biochemical workup revealed no abnormal indications of infection (including C-reactive protein (CRP) levels, erythrocyte sedimentation rate (ESR) and T-SPOT). Furthermore, the patient denied the history of cancer or tuberculosis and she also denied hypothermia, night sweats and weakness.\nBefore surgery, we obtained the informed consent of the patient and her family to perform the operation. The patients were placed in a prone position under general anesthesia with somatosensory-evoked potentials and motor-evoked potentials for spinal cord monitoring. After the lesion was positioned with the C-arm, a standard posterior midline approach with subperiosteal stripping was used to expose the spinous processes, lamina, and facet joints. Considering the presence of osteoporosis in elder female patient, we performed a long segment pedicle screw fixation (Cox Spinal Screw-Rod System, Fule Science & Technology, Beijing, China) from T10-L3 to avoid implant failure . Then, a complete laminectomy-facetectomy was performed to decompress and fully visualize the dural sac. A temporary stabilizing rod was fixed on one side of the pedicle screws. On the contralateral side, the facet joints of the diseased vertebra were removed to reveal the pedicle. Then, the pedicle and vertebral body, including the superior and inferior disk, were piecemeal removed by rongeurs, osteotomes, or curettes. To our surprise, caseous necrosis and inflammatory granulation could be seen in the surgically resected lesions. The specimens were sent for pathologic examination. After osteotomy and debridement in anterior column at L1, we used a ‘off-the-shelf’ (OTS) three-dimensional (3D) printed artificial vertebral body instead of various materials, such as bone grafts, mesh cages, or expandable titanium cages, to reconstruct sagittal alignment . Based on preoperative 3D reconstruction of CT and MRI images, the artificial prosthesis was designed in conformity with the expected defects that may occur after the affected vertebral body resection.\nPathological examinations reported caseous necrosis tissue, epithelioid granuloma existed with the hyperplasia tissue and the acid-fast bacillus was also found .\nAfter surgery, the patient was treated with quadruple anti-tuberculous chemotherapy and hepatoprotective drug for 12 months and required to wear a brace for at least 3 months. Two weeks after surgery, the patient could walk and discharge from the hospital. Three months after surgery, the VAS scores decreased from preoperative 9 to 1 and the neurological function recovered to ASIA E. No internal fixation failure and recurrence of tuberculosis occur at last follow-up .", "summary": "A 73-year-old female complained of back pain and weakness in lower limbs for 2 weeks. 3 months ago, after a minor trauma, she got back pain without weakness in lower limbs. Initially, she was diagnosed with a L1 compression fracture and accepted conservative treatment. After an asymptomatic period, she complained progressive pain at the fracture position with weakness of both lower limbs and was referred to our hospital with suspicion of Kümmell's disease. The patient underwent posterior debridement and internal fixation for decompression and stabilization of the spine. Pathological examinations revealed the patient with spinal tuberculosis.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1167_en.txt", "fulltext": "A 74-year-old male was admitted to our hospital for the treatment of esophageal cancer. The patient had no symptoms with normal temperature at the time of admission. His past medical history included diabetes mellitus for 24 years. He underwent endoscopic submucosal dissection for early gastric cancer 4 years ago and video-assisted thoracic surgery for pulmonary adenocarcinoma in the right upper lobe 5 years ago. He occupation was making metal molds. He was ex-smoker: Brinkman index was 980. Laboratory studies showed white blood cells of 10.0 × 103/L, C-reactive protein of 6.07 mg/dL, hemoglobin A1c test of 8.8%, fasting blood sugar of 237 mg/dL on admission. Serum tumor markers, including CEA, CA19–9, SCC, CYFRA, were unremarkable. He underwent esophago-gastro-duodenoscopy for follow-up after endoscopic submucosal dissection for early gastric cancer. The upper endoscopy showed a 0-IIa lesion with relatively large granular nodules on the lower thoracic esophagus . The pathologic assessment of the biopsy revealed squamous cell carcinoma of the esophagus. The esophageal 0-IIa lesion with relatively large granular nodules let us consider the depth of esophageal cancer was deeper than M3/SM1. Chest X-Ray was normal, however, chest CT revealed a 1.3 cm × 0.9 cm pulmonary mass in the lower lobe of the right lung a month before admission . PET/CT showed the pulmonary mass had maximal standardized uptake value (SUVmax) of 3.88 (arrow) and part of the lower thoracic esophagus had SUVmax of 2.37 (arrowhead) 2 weeks before admission . Because the image of pulmonary mass had not changed between CT and PET/CT for 2 weeks and because he had no fever, even though he had inflammation reaction on laboratory studies, pulmonary mass was considered to be unlikely due to infection. Thus, he was initially diagnosed with esophageal cancer with a pulmonary metastasis or recurrence of a pulmonary adenocarcinoma. Considering the both possibilities, we started chemotherapy with cisplatin (CDDP) 70 mg/m2 day1 plus 5-fluorouracil (5-FU) 700 mg/m2 day1–4 on the day 4 after admission. On the day 8, CT revealed a slight pleural effusion in the right side . On the day 9, to rule out carcinomatous pleurisy, an ultrasound-guided aspiration of pleural effusion was performed. The effusion was serous and contained no malignant cells. After the aspiration, the patient got a high fever. Laboratory test showed white blood cells of 18.6 × 103/L (90.8% of Neutrophil), CRP of 25.87 mg/dL. Considering that fever was due to respiratory infection after the aspiration, intravenous Sulbactam/Ampicillin (6 g/day) was administered. On the day14, his fever was persistent and he also had a cough. Because CT revealed an increased amount of pleural effusion , we considered the mass was lung abscess. By the second ultrasound-guided aspiration of the pleural effusion, we got a 50 ml of white suppurative effusion. The effusion turned out to contain Actinomyces israelii , and proved pulmonary mass-like lesion was pulmonary actinomycosis. The patient received intravenous penicillin G (24 million units/day) for 4 weeks, followed by oral amoxicillin 2250 mg/day for 6 months. The patient received four courses of chemotherapy (CDDP/5-FU) and curative radiotherapy (1.8Gy/total 50.4Gy) as esophageal cancer deeper than M3/SM1. The pulmonary actinomycosis disappeared six months after the treatment with antibiotics . After the chemoradiation therapy without any complications, the esophageal cancer was in complete remission.", "summary": "A 74-year-old male who had esophageal cancer and a pulmonary mass that was positive for 18F-fluorodeoxyglucose positron emission tomography/computed tomography was initially diagnosed with esophageal cancer with a lung metastasis because he was asymptomatic. However, aspiration of pleural effusion revealed that the pulmonary lesion was actinomycosis.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1850_en.txt", "fulltext": "The patient was a 75-year-old woman who presented with severe bilateral leg edema and epigastralgia. There was no past history of chronic hepatitis or blood transfusion. Admission laboratory tests revealed an elevated serum aspartate aminotransferase level (48 U/L), mildly decreased serum albumin (3.6 g/dL), mildly decreased serum (74%), mildly elevated D-dimer (12.9 μg/mL), and mildly elevated fibrin degradation product (12.6 μg/mL). The tumor marker AFP was slightly elevated to 585 ng/mL. The Child-Pugh classification was A, and the indocyanine green retention rate at 15 min was 12%. CT scans showed a huge tumor, 21 cm in diameter, in the right liver and TT, 37 mm in diameter, in the right atrium . The TT was pedunculated, swung like a pendulum with the heartbeat, and reached the tricuspid valve on a transesophageal ultrasound video . The symptomatic patient had a risk of sudden death due to TT in the right atrium. Therefore, we explained the high surgical mortality rate to the patient and her family, and they decided to proceed with surgery.\nAt first, a partial sternotomy was made, and cardiopulmonary bypass (CPB) via the aorta, superior vena cava, and right femoral vein and electrical ventricular fibrillation were performed after intravenous injection of 25,000 units of heparin sodium. Although the TT in the right atrium was very soft and fragile, thrombectomy from the right atrium and inferior vena cava (IVC) was successful . After the removal of TT, the IVC was clamped with a tourniquet above the diaphragm and the right atrium was sutured with 5–0 Prolene. After DC defibrillation was carried out, CPB was stopped, the tourniquet on the IVC was released, and 100 mg protamine was administered for the neutralization of heparin. The open heart surgery time was 23 min, and the CPB time was 42 min. Next, a reverse T incision was made, and right hepatectomy by anterior approach and IVC tumor thrombectomy were performed. After ligation of the anterior and posterior Glissonean pedicles, the liver parenchyma was transected under the Pringle maneuver and IVC clamping below the liver. After the IVC and right hepatic vein were confirmed by anterior approach, the IVC was opened and a residual TT in the IVC was removed under total hepatic vascular exclusion (THVE) . Finally, the huge tumor was removed with the diaphragm without pulmonary embolism. The total operation time was 9 h, and the total blood loss was 8200 mL. The tumor was of the massive type macroscopically , and cancer cells showed moderately to poorly differentiated HCC with invasion to the portal vein, hepatic vein, and diaphragm. The patient’s postoperative course was uneventful, and she was discharged 14 days after surgery. She is still alive 14 months after surgery with recurrence in the lung.", "summary": "Tumor thrombectomy under cardiopulmonary bypass with heparin and electrical ventricular fibrillation prior to hepatectomy was successfully performed to prevent sudden cardiac arrest or pulmonary embolism in a 75-year-old woman with a huge HCC and TT in the right atrium. After the neutralization of heparin, right hepatectomy with tumor thrombectomy in the inferior vena cava was performed. The total operation time was 9 h, and the total blood loss was 8200 mL. The patient's postoperative course was uneventful, and she was discharged 14 days after surgery. One year after surgery, she is alive with HCC recurrence in the lung.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1992_en.txt", "fulltext": "A 26-year-old woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breathlessness, with 4 h of evolution.\nPhysical examination showed tachypnoea, tachycardia (145 b.p.m.), blood pressure of 158/95 mmHg, no fever, and oxygen saturation on room air of 92%. Cardiac auscultation was normal but pulmonary auscultation revealed bilaterally dispersed crackles. She was pale, with cold and clammy extremities and decreased capillary refill time but peripheral pulses were easily palpable.\nThe patient had a personal history of migraine and had been submitted to periodontal surgery on the previous day. She was medicated with amoxicillin/clavulanic acid and prednisolone. No relevant family history was reported.\nThe electrocardiogram (EKG) revealed sinus tachycardia, left axis deviation, V4–V6 ST-segment depression, maximum 2 mm at V5, and an inverted T wave in aVL .\nTransthoracic echocardiography showed severe LV systolic dysfunction with midbasal segments akinesia and hypercontractility of the apical segments (, , Video S1).\nThe patient underwent aortic computed tomography angiography that excluded an acute aortic syndrome, but identified signs of severe pulmonary oedema and two heterogeneous solid masses in both adrenal glands, the largest on the right adrenal gland, measuring 9.2 × 9.2 × 10.8 cm, suggestive of a bilateral pheochromocytoma .\nThe patient progressed with respiratory failure and shock signs with poor peripheral perfusion, oligoanuria, and arterial hyperlactacidemia (41 mg/dL; normal range≤ 18 mg/dL), despite her hypertensive profile (mean arterial pressure 110–120 mmHg). At this stage, she was submitted to mechanical ventilation. Due to the rapidly deteriorated clinical status, she was referred and transported to the closest hospital with extracorporeal membrane oxygenation (ECMO) capability for immediate mechanical circulatory support.\nThe patient remained with veno-arterial ECMO (VA-ECMO) support for 14 days and progressive recovery of LV dysfunction was observed, enabling the weaning of the VA-ECMO support from the 10th day, without use of inotropics. There were no significant changes on the EKG on the following 2 days, with regression of repolarization changes at follow-up. The peak troponin I level was 1.73 ng/mL (normal range ≤0.015 mg/dL), on the first day of hospitalization with a slow but progressive fall and subsequent normalization.\nUrinary catecholamines and metanephrines in 24-h urine were elevated . Alpha-blockade was started with phenoxybenzamine and subsequently beta-blockade with metoprolol.\nOn the 35th day of hospitalization, open bilateral adrenalectomy was performed, without complications, and she began supplementation with gluco and mineralocorticoids. Anatomopathological examination confirmed the diagnosis of bilateral pheochromocytomas. The patient was discharged on the 49th day of hospitalization with complete recovery of the left ventricular systolic function and preserved neurologic status.\nAfter discharge, the patient performed body scintigraphy with MIBG-123 that excluded pheochromocytoma metastases or synchronous tumours, but she was diagnosed with bilateral thyroid medullary carcinoma. Total thyroidectomy was performed and the diagnose of Type 2 multiple endocrine neoplasia (MEN) syndrome was established.", "summary": "We present a case of a young woman was admitted to the emergency department due to intense headache, chest discomfort, palpitations, and breathlessness. An ITS secondary to a pheochromocytoma crisis presenting with CS was diagnosed. The patient was managed with veno-arterial extracorporeal membrane oxygenation, until recovery of LV function. On the 35th day of hospitalization, open bilateral adrenalectomy was performed.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2816_en.txt", "fulltext": "A 79 year old man was admitted at our emergency department due to exertion dyspnoea and swelling of the upper limbs, head and neck. Five years ago the patient had undergone elective replacement of the aortic valve with a prosthetic one (Carbomedics 23 mm) and a concomitant ascending aorta aneurysm replacement with a 28 mm synthetic graft. On admission the patient was dyspnoic with malaise. His blood pressure was normal (128/82 mmHg) and the oxygen saturation without oxygen supply was 92%. Pulses were irregular and increased in rate. ECG showed atrial fibrillation with fast ventricular response (135 to 145 beats/min). The physical examination documented cyanotic and swollen head and neck and distended jugular veins. Superior vena cava syndrome (SVCS) was clinically diagnosed. Laboratory data included a prolonged INR of 7 and Ht: 35.6%. Other elements of blood count and coagulation time were within physiologic values. Cardiac enzymes were negative. LDH was 620 IU/L. The rest of his biochemical profile was normal. Arterial blood gases showed mild respiratory acidosis (PH: 7.33) due to elevated PCO2: 48mmHg.\nChest x-rays was further confirmatory of the clinical diagnosis showing a widened mediastinum. However the contrast CT angiography of the chest was remarkable, revealing a large pseudoaneurysm of the ascending aorta with a maximum diameter of 13cm compressing the superior vena cava and an extensive collateral circulation .\nTransfusions with 2 units of fresh frozen plasma were enough to lower the INR to 1.8 and the patient was then transferred to the operating room. Femoro-femoral cannulation was undertaken in order to establish cardiopulmonary bypass and decrease the patient's temperature to down to 16°C to safely commence circulatory arrest. After the completion of cooling with collection of the patient's blood within the reservoir of the extracorporeal device, repeat sternotomy was performed through safe surgical means. Retrograde cardioplegia was installed. The pseudoaneurysm was incised and the thrombotic material was carefully removed. Two sites of major leakage originating from the anterior and posterior segment of the distal anastomosis were revealed. Suturing with 3/0 prolene, reinforced by teflon patches, was performed. Eventually extracorporeal circulation was recommenced, gradual rewarming was carried out and successful weaning from cardiopulmonary bypass was achieved.\nThe patient was extubated 6 hours later and he remained in the intensive care unit for 18 hours. His postoperative course was uneventful. Ten days following his admission the patient was discharged in good condition, presenting however atrial fibrillation with good rate control under Amiodarone treatment. Three months following his discharge he remains free of symptoms.", "summary": "We present the case of a large pseudoaneurysm of the ascending aorta, five years after primary surgery, with a significant compression of the right mediastinal venous system causing superior vena cava syndrome, detected at chest CT angiography. Perioperative findings showed two rush out points both coming from the distal aortic suture line which was performed five years ago. The patient underwent reoperation under circulatory arrest facilitating safe exploration and repair of the distal anastomotic leaks", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2889_en.txt", "fulltext": "A 33-year-old Bangladeshi woman with hypertension and diabetes had initially presented to a different hospital with intermittent episodes of palpitations for 2 years, with documented electrocardiographic (ECG) evidence of AF during the episodes. She had previously been on oral propafenone, amiodarone, and metoprolol, at separate times, but continued to experience paroxysmal AF.\nShe had a 2-year history of hypertension that was well controlled with metoprolol 50 mg twice daily and ramipril 5 mg once daily. She had type 2 diabetes mellitus, diagnosed 3 years previously, that was well controlled with metformin 500 mg twice daily. There was no evidence of diabetic nephropathy or microalbuminuria. Furthermore, other etiologies of AF, such as thyrotoxicosis, were excluded by her thyroid profile, which comprised normal levels of TSH, free triiodothyronine, and free thyroxine. Ischemic heart disease was excluded by a CT coronary angiogram, which revealed normal epicardial coronary arteries.\nGiven the indication of drug-refractory AF, our patient had undergone EPS at a different hospital from which she had been discharged 24 hours after the procedure. The EPS has comprised right femoral venous catheterization followed by the insertion of three venous sheaths into her femoral vein.\nBecause an inducible arrhythmia could not be reproduced, her pulmonary vein (PV) potentials were mapped and complete PV isolation was planned. But, because there was difficulty in isolating some of the PVs along with an extended procedural time, the procedure was abandoned. A plan was made to identify all PVs more specifically using other imaging techniques such as magnetic resonance imaging (MRI), prior to attempting the procedure again.\nShe presented to our hospital 48 hours after the EPS with shortness of breath and chest heaviness, associated with palpitations and recurrent episodes of syncope. When queried, she confirmed that these symptoms were not like those she had previously experienced during her paroxysms of AF, and reiterated that they only developed after the EPS.\nShe had no prior history of thromboembolic events, long flights, prolonged immobilization, or oral contraceptive use, and she denied any family history of thrombophilia. There was no evidence of either hypertensive or diabetic nephropathy. A urine dipstick test was negative for protein. A thrombophilia work-up revealed normal levels for protein C and S, antinuclear antibody, and anticardiolipin antibody, thus eliminating other causes of hypercoagulability as possible etiologies of thrombosis.\nOn examination, she was hemodynamically stable and heart and lung auscultation was unremarkable. There was no unilateral leg swelling. An ECG showed T inversions in leads III, aVF, and V1–4. It also revealed mild tricuspid regurgitation and a pulmonary artery systolic pressure (PASP) of 50 mmHg. Her right ventricle (RV) was dilated, with diameters measuring 38 mm at the basal level and 40 mm at mid-level, with a longitudinal measurement of 90 mm on the apical 4 chamber view. Tricuspid annular plane systolic excursion (TAPSE) was measured at 12 mm. Her left atrium (LA) was of normal size and measured 33 mm. Qualitative assessment revealed that right atrium was larger than her LA. There was no evidence of left ventricular (LV) hypertrophy. Her LV ejection fraction (EF) was 65 % and there were no regional wall motion abnormalities of her LV. There was no evidence of thrombus in any of the cardiac chambers.\nA CT coronary and pulmonary angiogram revealed normal coronaries with filling defects in the descending branch of her left pulmonary artery extending into the segmental arteries, suggesting thrombus .\nSubsequently, a duplex ultrasound study of her lower limb venous system showed absent blood flow in her right external and common femoral veins, confirming acute DVT affecting her right ilio-femoral segment without signs of recanalization .\nGiven the sequence of events and their temporal relationship with the EPS, and in the absence of other risk factors for DVT, it was established that the acute DVT occurred as a consequence of the femoral vein catheterization and multiple venous sheaths inserted for EPS, subsequently leading to PE. She was treated with subcutaneous enoxaparin 60 mg twice daily for 5 days along with the administration of oral warfarin. She continued taking the warfarin for 6 months, with a target therapeutic international normalized ratio of 2.5–3. She was asymptomatic at follow-up at six months, and, due to the resolution of the DVT, oral anticoagulation was discontinued.", "summary": "A 33-year-old Bangladeshi woman with hypertension and diabetes had initially presented with recurrent episodes of paroxysmal atrial fibrillation that manifested as palpitations for 2 years. Her atrial fibrillation was drug-refractory and could not be attributed to a treatable etiology. She had undergone an electrophysiological study at a different hospital, where right femoral venous catheterization was performed followed by the insertion of three venous sheaths. However, tachyarrhythmia could not be induced and a procedure to isolate the pulmonary vein was postponed because all the veins could not be isolated. Forty-eight hours later, she presented to our hospital with shortness of breath, chest heaviness, palpitations, and recurrent episodes of syncope. She had normal coronary arteries and no other risk factors for venous thromboembolism. She was hemodynamically stable on examination. There was echocardiographic evidence of pulmonary hypertension and right ventricular dilatation and dysfunction. A computed tomography pulmonary angiogram confirmed pulmonary embolus in the descending branch of her left pulmonary artery, extending up to the segmental arteries. Subsequently, a duplex ultrasound confirmed acute deep vein thrombosis affecting her right ilio-femoral segment. She was successfully managed with subcutaneous enoxaparin and oral warfarin (target international normalized ratio 2.5-3).", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_181_en.txt", "fulltext": "Our patient was 35 year old male who presented to emergency department with history of fall by tripping on a stone (fall on outstretched hand). Post trauma patient had pain and difficulty moving both the shoulders. Patient was a known alcoholic, with previous history of one episode of shoulder dislocation on right side about 2 years prior to the present episode, which was adequately treated with closed reduction and immobilisation for 3 weeks. Patient does not give any history of convulsions (past or present) and no other neuro-muscular problems. On clinical examination, patient's both upper limbs were abducted and externally rotated. Bilaterally shoulder contour was lost with flattening. Other classical signs of shoulder dislocation viz, Bryants test, Callway sign, Hamilton's ruler test were positive. Radiographs of both shoulders were obtained and clinical diagnosis of SBAD was confirmed. Both shoulders were reduced in emergency operation theater under general anaesthesia by Kocher's method and were immobilized in sling. The left was immobilized with 90° elbow flexion and the right at 110° flexion for 3 weeks. Reduction was confirmed post operatively with X-rays. Intermitted assisted excercises where started from second week onwards and at the end of immobilization patient was advised vigorous supervised physiotherapy. By six week post injury patient had full range of adduction, flexion and internal rotation. Abduction up to 110° was possible. Patient was advised to be cautious while doing overhead activities especially which require abduction and external rotation of shoulder.", "summary": "35 year old male presented to emergency department with history fall by tripping on a stone (fall on outstretched hand). He complained of pain and difficulty in moving both the shoulders. On clinical examination, patient's both upper limbs were abducted and externally rotated. Bilaterally shoulder contour was lost with flattening. Other classical signs of shoulder dislocation viz, Bryants test, Callway sign, Hamilton's ruler test were positive. Diagnosis was confirmed on X rays. Both shoulders were reduced in emergency operation theater under general anaesthesia by Kocher's method and were immobilised in sling.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_235_en.txt", "fulltext": "In August 2008, a 44-year-old female had undergone surgery for resection of a malignant melanoma in the right lower leg and a right inguinal metastatic lymph node , followed by chemotherapy with doxorubicin, adriamycin, vincristine, and interferon beta (DAV-feron). In March 2012, computed tomography (CT) revealed brain and lung metastases, so the patient began radiation therapy to treat these lesions.\nIn September 2012, the patient was admitted to our hospital for back pain. Abdominal CT and magnetic resonance imaging detected new multiple hepatic metastases of melanoma. A transcatheter arterial infusion of cisplatin was administered, and transcatheter arterial embolization (TAE) was performed. In October 2012, she began treatment with vemurafenib, based on the finding of a positive BRAF V600E mutation in the resected primary site of the skin, which was analyzed by direct sequencing analysis using DNA from the paraffin-embedded primary cutaneous melanoma. She tolerated the treatment remarkably well, and the size of the multiple hepatic and lung metastases decreased, while the size of the brain metastases did not. In addition, the serum concentration of 5-S-cysteinyldopa (5-S-CD), a biological marker of melanoma progression, was also decreased from 40.1 ng/mL to 5.2 ng/mL.\nIn December 2012, she suddenly developed severe abdominal pain. Abdominal CT revealed ruptured hepatic metastases accompanied by massive intra-peritoneal hemorrhage. A retrospective and sequential analysis of the CT images suggested that a part of the liver metastases had enlarged rapidly and then ruptured with intratumoral hemorrhage during vemurafenib treatment . An emergency TAE was performed by selective occlusion of the right hepatic artery using gelatin sponge particles. The postoperative course was uneventful for several days. However, on the 26th postoperative day, she developed hemorrhagic shock and died of renewed intra-abdominal bleeding.\nAn autopsy examination revealed hemoperitoneum due to rupture of the liver metastases. Metastases were also discovered in the brain and lungs as well as in the kidneys, adrenal gland, and lymph nodes, although these had not been detected on imaging while she was alive. There was also massive bloody ascites (1700 mL). The background liver was completely normal, whereas exposed necrotic tissue and intratumoral hemorrhage were observed at the site of tumor rupture . We concluded that the cause of death was hemorrhagic shock from ruptured hepatic metastases of malignant melanoma. Finally, for improved understanding of the mechanism of refractory metastasis, we conducted an immunohistochemical analysis of the signal transduction molecules, phosphorylated extracellular signal-regulated kinase (p-ERK), and phosphorylated Akt (p-Akt), as well as the melanocyte marker Melan-A and Ki-67 in tumor cells of the primary malignant melanoma obtained from the right lower leg and in hepatic and lymph node metastases obtained on autopsy . Our findings showed that hepatic and lymph node metastases were positive for p-ERK and negative for p-AKT, even though the primary tumor was negative for both.", "summary": "A 44-year-old female had previously undergone surgery for resection of a malignant melanoma in the lower right leg. Four years later, hepatic metastases became apparent, and transcatheter arterial embolization (TAE) was performed. Then she underwent treatment with vemurafenib. The size of the hepatic metastases markedly decreased. Two months later, they enlarged rapidly and ruptured, requiring emergency TAE. However, the patient developed hemorrhagic shock and died of renewed intra-abdominal bleeding on the 26th postoperative day.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_614_en.txt", "fulltext": "A 39-year-old, healthy, non-smoking female patient with no family history of cancer was referred on suspicion of rectal cancer. Digital rectal examination and subsequent rigid proctoscopy revealed a low rectal tumor and multiple polyps in the rectum. Subsequent colonoscopy showed extensive polyposis of the entire colon. The tumor biopsy confirmed the presence of a rectal adenocarcinoma, while dysplastic changes were found in the polyp biopsies. No distant metastases were detected on the thoracoabdominal computed tomography (CT) scan, and baseline carcinoembryonic antigen (CEA) was 8 μg/L. Pelvic magnetic resonance imaging (MRI) revealed a locally advanced rectal cancer with extra-mesorectal growth into the rectovaginal septum and the lower posterior vaginal wall as well as a suspected growth into the anterior aspect of the coccygeal bone with multiple suspected malignant lymph nodes within the mesorectum (T4N2). Upon evaluation by our multidisciplinary team (MDT), she was scheduled for neoadjuvant chemoradiotherapy (CRT) followed by surgery. Neoadjuvant treatment was given as CT planned radiotherapy (daily 2-Gy fractions, 5 days per week; the initial 23 fractions to the macroscopic tumor and areas at risk, and the two final fractions adapted to the macroscopic tumor) with concomitant capecitabine (825 mg/m2) on days of RT. The response evaluation showed excellent tumor volume response, but with similar organ involvement as at baseline, and she proceeded to surgery 8 weeks after CRT completion. Surgery involved total colectomy, APE with resection of the posterior vaginal wall and coccygeal bone with subsequent reconstruction of the perineum with right-sided VRAM flap, and a terminal ileostomy. After an uneventful recovery, she was discharged 15 days postoperatively. Histologic examination of the specimen showed a 35-mm large adenosquamous carcinoma removed with free resection margins and metastasis in one of four local lymph nodes (pT2N1). In addition, multiple dysplastic adenomas were present in the entire colon.\nAt the routine follow-up 6 months postoperatively, clinical examination revealed a 2 × 2 cm tumor located in the muscle tissue of the VRAM flap, and an early local recurrence was suspected. Pelvic MRI showed a 10 × 16 mm rich vascularized and cell dense lesion corresponding to the clinically detected tumor . No other pathological findings were made on CT or MRI, and CEA was 1 μg/L. The lesion was surgically excised with a wide local resection of the tumor. Interestingly, histologic examination of the removed lesion described a mass composed of fatty tissue, fibrocytes, and fibroblasts arranged in broad, sweeping fascicles infiltrating the adjacent striated muscle tissue. No dysplasia was observed, but mitoses were present. Immunohistochemistry showed positive staining for β-catenin (ABCAM, Cambridge, USA), and the tumor was diagnosed as a fibromatosis . The medical geneticists suspected familial adenomatous polyposis (FAP), and testing of the APC gene, revealed a known disease-causing mutation c.3317dupG (p.Ala1107Serfs*12), confirming this diagnosis.", "summary": "A 39-year-old female patient with low rectal cancer and extensive colorectal polyposis was treated with neoadjuvant chemoradiotherapy followed by colectomy and abdominoperineal excision with resection of the dorsal vaginal wall and subsequent reconstruction of the perineum using the vertical rectus abdominis myocutaneous flap. At the 6-month follow-up, a suspected 2 × 2 cm tumor recurrence was detected in the transposed tissue and was subsequently surgically removed. Histologic examination concluded with fibromatosis. Genetic testing revealed a known disease-causing mutation in the adenomatous polyposis coli gene, confirming the diagnosis of familial adenomatous polyposis.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_746_en.txt", "fulltext": "A 26-year-old male presented with severe dyspnea, palpitation, orthopnea, and paroxysmal nocturnal dyspnea. He had hypertension, medical history of ischemic stroke 5 months ago, and repeated hospitalization caused by congestive heart failure. His blood pressure in the right arm was 180/64 mmHg. The heart rate was irregular with 150 beats/min. The jugular venous pulse was elevated to 9 cm of water. There was murmur systolic on intercostal space (ICS) 5 anterior axilla line (AAL) sinistra and murmur diastolic on ICS 2 parasternal line (PSL) dextra. Respiratory rate of 28 breaths/min, oxygen saturation at the right arm was 92% without supplemental oxygen, and body mass index of 19,5 kg/m2.\nAn electrocardiogram showed atrial fibrillation (AF) with a rapid ventricular response. A routine laboratory examination did not show any abnormality. Chest X-ray showed cardiomegaly with calcification aortic arch and pulmonary edema. Echocardiography found that left ventricular ejection fraction (LVEF) was decreased (48,5%) with left atrial and ventricular dilatation. Echocardiography also found severe aortic and mitral valve regurgitation . He was diagnosed with congestive heart failure with mildly reduced ejection fraction (HFmrEF) and classified in New York Heart Association (NYHA) class IV.\nAfter one day of hospitalization, the patient also complained of intermittent claudication in the left arm. Clinical examination found an absence of the left brachial pulse and the left carotid artery bruit. Four limbs blood pressure discrepancy was present (right arm 163/50 mmHg, left arm 96/55 mmHg, right leg 90/60 mmHg, left leg 95/55 mmHg). Computed tomography angiography (CTA) showed calcification in the left common carotid artery leading to stenosis and total occlusion of the left subclavian artery with the collateral artery branch from the left common carotid artery that supplies vascularization of the left arm. There was vascular thickness and calcification from the peri-aortic valve, ascending aorta, aortic arch, and thoracic descending aorta until abdominal aorta with high-grade stenosis on the inferior side of the renal artery branching accompanied by a post-stenotic dilatation . Inflammatory markers evaluation that needed notable are elevation C-reactive protein (CRP) level (45,1 mg/L) and erythrocyte sedimentation rate (ESR) level (25 mm/h). Other laboratory evaluations were antinuclear antibody (ANA) titer, antistreptolysin O (ASO) titer, and interferon-gamma release assay (IGRA) showed negative results. He has subsequently diagnosed with Takayasu arteritis according to the American College of Rheumatology criteria.\nFor treatment of hypertension and HFmrEF, beta-blocker bisoprolol 2,5 mg/day, angiotensin-converting enzyme inhibitor (ACE-I) ramipril 5 mg/day, and mineralocorticoid receptor antagonists (MRA) spironolactone 25 mg/day therapy were initiated. Loop diuretic furosemide 40 mg/day is given to treat congestive symptoms of heart failure and glycoside digoxin 0,25 mg/day is given as rate control of atrial fibrillation. Anticoagulant warfarin 2 mg/day was given to prevent thromboembolism. A combination of methotrexate 7,5 mg/week and high-dose methylprednisolone at 48 mg/day with subsequent tapering was given for induction of remission to inhibition vasculitis of TA. After 3 months of high-dose steroid and methotrexate therapy, the patient showed a decrease in symptoms of NYHA class II for heart failure and a decrease in the frequency of rehospitalization. The patient also did not complain of claudication in the left arm. On physical examination, the patient's right arm blood pressure was 146/55 mmHg and the left arm was 105/50 mmHg. Follow-up evaluation of inflammatory markers CRP and ESR showed normal results.", "summary": "We report a 26-year-old male presented with severe dyspnea, palpitation, orthopnea, paroxysmal nocturnal dyspnea, and claudication in the left arm. Four limbs blood pressure discrepancy was present. Chest X-ray showed cardiomegaly with calcification aortic arch and pulmonary edema. Echocardiography revealed that left ventricular ejection fraction decreased with severe aortic and mitral valve regurgitation. Computed tomography angiography showed stenosis of the left common carotid artery and total occlusion of the left subclavian artery with collateral artery. There was vascular thickness and calcification from the peri-aortic valve, ascending aorta, aortic arch, and thoracic descending aorta until abdominal aorta with high-grade stenosis on the inferior side of the renal artery branching accompanied by a post-stenotic dilatation.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_3047_en.txt", "fulltext": "A 27-year-old female smoker with no regular medication, with no previous history of SARS-CoV-2 infection, vaccinated with BCG at birth in her left arm, received the first dose of the COVID-19 mRNA vaccine (Moderna®), also in her left arm, without symptoms or immediate intercorrerences, reporting no additional changes in the following days, namely, redness, heat, change in pigmentation or exudate. Eight days after administration of the anti-SARS-CoV2 mRNA vaccine, the young woman developed a reaction of a round shape and poorly defined edges with about 2 cm in diameter at the site of the BCG scar, characterized by erythema, induration and pain, and distanced about 4 cm from the site of inoculation of the anti-SARS-CoV2 vaccine. The patient did not report fever, myalgia or other symptoms. No changes were detected in the objective examination, namely, adenopathies. The symptoms persisted for three days, and the patient opted not to be medicated. After 11 days since the onset of symptoms, a complementary analytical study was performed, revealing a positive result for anti-SARS-CoV2 IgG, with the remaining values within the normal range, including lymphocytes and viral serologies. The incident was submitted as a possible adverse reaction to medicines in the National Pharmacovigilance System.\n", "summary": "Here is a case of a 27-year-old woman with no relevant history, including SARS-CoV-2 infection, who developed inflammation of the BCG scar eight days after the first administration of anti-SARS-CoV-2 mRNA vaccine.\n", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2292_en.txt", "fulltext": "A 65-year-old female with a history of type 2 diabetes mellitus, systemic arterial hypertension and long-standing right hearing loss. She began his condition 21 days before his initial evaluation in July 2023, presenting with moderate to intense holocranial headache that partially subsided with the use of non-steroidal anti-inflammatory drugs. She had previously been diagnosed with dementia due to retrograde memory and language disorders. In the neurological examination with Glasgow 13 points (O:4, V:3, M:6), mental functions with global aphasia, isocoria of 3 mm, preserving photomotor and consensual reflexes, right side conduction hearing loss, preserved motor function, sensitive without response to pain in the right hemibody. No evidence of intracranial hypertension or history of seizures. A simple and contrasted tomography was performed, showing the presence of two lesions at the level of both ventricular trigone each one apparently, which were confirmed by magnetic resonance imaging (MRI) scan indicating periventricular lesions with extension to the white matter of the temporal and parietal lobes, a clear reduction of the ventricular space of both atrium was observed, no communication between these lesions through fibers of the corpus callosum, with mostly uniform enhancement at gadolinium contrast , and restriction to diffusion. Metastasis, high-grade glioma, and lymphoma were considered as a differential diagnosis, which was complemented by a thoracic and abdominal tomography without evidence of systemic lesions. Serological studies were performed , viral profiles for EBV, CMV, and HIV, as well as tumor markers, which were all negative. Subsequently, a spectroscopy study was performed 8 days before treatment with dexamethasone, where peaks of choline and creatine were observed, a high choline/creatine (Cho/Cr) ratio, with peaks of lipids and lactate, as well as a decrease in N-acetyl aspartate (NAA), suggestive of malignancy and presence of areas of necrosis.\nA temporal craniotomy with a transulcal T2–T3 approach guided by a neuronavigation system was performed. Biopsy samples were taken. The tumor was identified as infiltrative and diffused in the white matter, with gray color, soft consistency, and necrotic core. The ventricle was not opened. No complications were presented.\nHistopathology revealed diffuse large B-cell lymphoma with an angiocentric pattern with positive immunohistochemical staining for CD20 and Ki67 in 70% .\nThe patient had a complicated course due to pneumonia, was discharged from the hospital 15 days after surgery, and was referred to an oncology center.", "summary": "A 65-year-old female presented progressing headache, loss of memory and language alterations, as well as sensory alterations. Neuroimaging showed the presence of two equidistant periventricular lesions at the level of both ventricular atria, a spectroscopy study suggestive of malignancy. Serological studies showed no evidence of immunodeficiency or the presence of positive tumor markers; however, a biopsy was performed, which revealed a histopathological result of primary lymphoma of the CNS.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2759_en.txt", "fulltext": "A 66-year-old Maori man presented to our hospital with a history of an enlarged left testicle of 10 weeks’ duration. His clinical examination revealed nontender swelling of the left testicle. His alpha-fetoprotein (AFP) and beta-human chorionic gonadotropin (BHCG) levels were within normal limits. His lactate dehydrogenase (LDH) concentration was mildly elevated at 267 U/L (normal range 120–250 U/L). Ultrasonography of his testes demonstrated an enlarged left testicle measuring 7 × 5.5 × 4.3 cm with an estimated volume of 87 ml. A large, heterogeneous mass involved the entire testicle with increased vascularity . The patient’s right testicle measured 3.7 × 2.5 × 1.8 cm with an estimated volume of 8.8 ml. He underwent left orchiectomy. The macroscopic specimen consisted of well-circumscribed nodular lesions of varying sizes, with the largest measuring 45 × 15 mm and containing solid and gelatinous components . Sections of the specimen showed tumor composed of sheets of small, blue, round cells divided into nodules by fibrous septae . Immunostaining showed the tumor to be cytokeratin 20 (CK20)-positive with a typical paranuclear dotlike staining pattern . The stain showed a positive result for CD56, a neuroendocrine marker , as were CD117 and CK (paranuclear dots). The result was negative for CK7, placental alkaline phosphatase (PLAP), CD30, CD20, AFP, S100, SOX10, prostate-specific antigen (PSA), chromogranin, and thyroid transcription factor 1 (TTF-1). The Ki-67 level was >50 %. This immunostaining pattern raised the possibility of metastatic MCC.\nThe patient presented again to our hospital 3 months later this time with a right testicular mass. Tumor markers, including LDH, AFP, and BHCG, were within normal limits. Ultrasonographic imaging demonstrated a new lesion in the right testis measuring 3 × 2.6 × 2.3 cm. He underwent a right orchiectomy, and sections of the specimen showed diffuse infiltration of small, blue, round cells. Immunostains were positive for CD117, CD56, synaptophysin, CK20 (dotlike), and cytokeratin AE1/AE3 (dotlike). The tumor cells were negative for inhibin, PLAP, PSA, S100, CD30, CD45, CD3, CD20, TTF-1, and napsin A. The Ki-67 level was 80 %. This pattern was consistent with a poorly differentiated neuroendocrine tumor in keeping with metastatic MCC. Detailed histology reports can be found in Additional files and . A primary site was not identified, and a staging computed tomographic scan did not show evidence of other metastases. The patient received six cycles of adjuvant carboplatin and etoposide chemotherapy. He remained disease-free 18 months following completion of chemotherapy.", "summary": "A 66-year-old Maori man presented to our hospital with left testicular swelling. His alpha-fetoprotein and beta-human chorionic gonadotrophin levels were within normal limits. His lactate dehydrogenase concentration was elevated to 267 U/L. Ultrasound imaging confirmed a large testicular mass, and he underwent left orchiectomy. His histological examination revealed a neuroendocrine tumor with an immunostaining pattern suggesting Merkel cell carcinoma. He presented to our hospital again 3 months later with right testicular swelling that was confirmed on ultrasound sonography to be a tumor. He underwent a right orchiectomy, and his histological examination revealed metastatic Merkel cell carcinoma. A primary lesion was not identified, and computed tomographic imaging did not reveal spread to other organs. He received six cycles of adjuvant carboplatin and etoposide chemotherapy and remained disease-free 18 months after completion of chemotherapy.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_354_en.txt", "fulltext": "In august 2018, an 81-year-old caucasian man presented to the emergency department with fever, progressive dyspnea, dry cough and left pleuritic chest pain.\nPatients symptoms started 3 wk ago and worsened progressively.\nHis medical history was consistent with chronic lymphocytic leukemia, initially treated in 2007 with Fludarabine, Cyclophosphamide and Rituximab therapy with chronic lymphocytosis, type 2 diabetes mellitus, hypertension, hyperlipidemia and chronic kidney failure with an eGFR of 28 mL/min/1.73 m2. He reported no smoking or alcohol abuse and used to work as an upholsterer.\nNone.\nPhysical examination revealed fever (38.1°C), tachycardia (105 bpm), peripheral percutaneous oxygen saturation was 100% with 2 liters of oxygen with tachypnea, normal blood pressure and almost abolished left vesicular murmur. Adenopathy, hepatomegaly or splenomegaly were not observed. Cardiovascular and neurologic examination were normal.\nBiology tests revealed neutrophilia (18.7 g/L) and elevated CRP (297 mg/L) consistent with inflammatory response, along with stable lymphocytosis (242 g/L) and anemia (7.8 g/L).\nChest X-ray at admission showed a large left pleural effusion with contralateral tracheal deviation. Complementary computed tomography confirmed a walled-off, loculated left pleural effusion, with right lung parenchyma considered normal.\nFirst bedside pleural puncture showed a citrine exudate (pleural protein 37 g/L, pleural fluid protein to serum ratio 0.61), unfortunately cytological examination was not performed.\nDespite intravenous treatment with cefotaxime, the patient condition worsened with persistence of fever, neutrophilia, and severe hypoxemia appeared. Bacteriological standard culture of the pleural liquid sample was negative, as well as repeated blood cultures. In order to look for a tuberculosis etiology, auramine stained sputum smears and cultures were performed but remained negative. Spot-test for tuberculosis was not performed. Ten days after his admission, because of the uncontrolled large pleural effusion with acute hypoxemic respiratory failure, the patient underwent surgical thoracentesis with evacuation of 2 liters of purulent liquid. We chose surgery instead of repeated pleural drawings, because local sepsis was not controlled nor bacteriologically documented. Pleura was thick and nodular, with white pseudo-membranes. Treatment with metronidazole was added to cefotaxime after the surgery in order to cover anaerobic bacteria. Cytobacteriological examination and cultures of the liquid were negative. Lymphocyte phenotyping ruled out B-cell lymphoma as a complication of his chronic lymphocytic leukemia. Three days after, the patient progressively developed multiple organ failure requiring intensive care unit admission. As no massive transfusion was initiated during surgery and shock was the main matter, fluid overload could not explain the evolution in multiple organ failure. After a slow unfavorable evolution during the ten first days of hospitalization, the patient’s condition worse brutaly three days after surgical thoracocentesis leading to septic shock complicated of multiple organ failure. Despite antibiotherapy with cefepime and amikacin, invasive mechanical ventilation, vasopressor infusion and renal replacement therapy, the patient died of uncontrolled septic shock few hours after ICU admission.\nThe day after, mycobacterial culture of surgical pleural samples yielded Gram-negative bacilli . Rapid identification using matrix-assisted desorption ionization–time of flight mass spectrometry (MALDI-TOF MS; Microflex LT; Bruker Daltonics, Leipzig, Germany) was performed and L. pneumophila was identified. The bacteria were addressed to the National Reference Center of Legionella and molecular typing analysis by sequence-based typing was performed and showed that the strain of L. pneumophila serogroup 1 belonged to Sequence Type 1. Urinary antigen research retrospectively performed on a urine sample of the patient confirmed the presence of L. pneumophila serogroup 1.\nWe believe that chances of super infection or co-infection with another bacterium are scarce. Patient indeed received broad spectrum antibiotics directed against common bacteria causing pleural empyema (including anaerobic bacteria, gram-negative bacillus, streptococci and staphylococci), and repeated standard bacteriological cultures remained negative.\nRetrospectively, our patient presented the following risk factors: Male sex, aged more than 50 years, chronic lymphocytic leukemia with history of immunocompromising treatments, chronic kidney failure, diabetes mellitus and weaned smoking.", "summary": "An 81-year-old man presented to the emergency with a 3 wk dyspnea, fever and left chest pain. His previous medical conditions were chronic lymphocytic leukemia, diabetes mellitus, chronic kidney failure, hypertension and hyperlipidemia, without tobacco use. Chest X-ray and comouted tomography-scan confirmed a large left pleural effusion, which puncture showed a citrine exudate with negative standard bacterial cultures. Despite intravenous cefotaxime antibiotherapy, patient's worsening condition after 10 d led to thoracocentesis and evacuation of 2 liters of pus. The patient progressively developed severe hypoxemia and multiorgan failure occurred. The patient was treated by antibiotherapy with cefepime and amikacin and with adequate symptomatic shock treatment, but died of uncontrolled sepsis. The next day, cultures of the surgical pleural liquid samples yielded L. pneumophila serogroup 1, consistent with the diagnosis of pleural legionellosis.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_659_en.txt", "fulltext": "A 35-year-old female patient arrived at our department complaining of back pain, heart palpitations, and fatigue due to the large size of pericardial mass causing a pressure symptom. No symptoms of hypertension, fever, extrasystoles were found on physical examination, and no arrhythmia or rhythm disturbances were found on the electrocardiography. No previous medical and family history was declared by the patient. A chest X-ray showed that the patient’s cardiothoracic ratio was enlarged and that her cardiophrenic sinus was obscured on the right side (see Fig. ). A transthoracic echocardiography indicated pericardial effusion, tricuspid, and pulmonary insufficiencies and showed a large echogenic mass involving the pericardium beside the right heart. Pericardiocentesis was performed, and a cytologic examination of the bloody pericardial fluid obtained though fine needle aspiration (FNA) showed monotonous cells with oval-spindle vesicular nuclei with less amphophilic cytoplasm, evenly distributed chromatin, and inconspicuous nucleoli that formed groups in hypocellular samples (see Fig. ). Overlapping nuclei were observed locally. During pre-diagnosis, a spindle cell mesenchymal tumor with uncertain malignant potential was considered. Adequate sample cannot be obtained from aspiration for cell block preparation.\nA contrasted CT scan of the chest showed a heterogeneously enhanced lesion measuring 12 cm x 11 cm x 6.5 cm located in the right mid-anterior of the pericardial area (see Fig. ). The mass had a heterogeneous internal structure, creating significant pressure on the right heart and superior vena cava. There was no radiological space between the mass and the right heart, and no significant vascular, cardiac, or thoracic wall invasions were observed. No lymph node involvement was observed in the mediastinal or hilar regions. The trachea, including both main bronchi and bronchial trees, was open. Neither an active infiltrative appearance nor any nodular lesions could not be seen in the lung parenchyma (see Fig. ). All radiological findings were indicative of either a pericardial fibroma or a pericardial sarcoma, both of which were noted in the differential diagnosis. On positron emission tomography (PET)-CT imaging, only the pericardial mass showed increased F-18 fluorodeoxyglucose (FDG) uptake with a maximum standard uptake of 5.3. No lymph node metastasis was detected radiologically.\nAfter the radiological examinations, the pericardial mass was decided to be resected. During thoracotomy, the pericardial mass was found to invade the ventricular wall and main vascular structures, therefore, the mass was resected incompletely. Due to the location, extent and nature of the tumor, debulking surgery was performed and R0 surgery could not be performed. Macroscopically, any palpable lymph node was not detected during the surgery.\nHistopathologically, a spindle cell mesenchymal tumor with infiltrative limited hypercellular fascicular structure containing a small amount of loose myxoid stroma hyalinized, and necrotic areas showing mild to moderate pleomorphism were observed in fragmented mass samples (see Fig. a). A nuclear palisade was noted in the neoplastic cells, and the mitotic rate was 18 mitotic cells per 10 high power fields (see Fig. b). Mesothelioma, fibrosarcoma, a solitary fibrous tumor, leiomyosarcoma, and synovial sarcoma were all included as possibilities in the differential diagnosis. Immunohistochemical staining showed a positive reaction with vimentin, Bcl-2, TLE-1 (see Fig. c), and a negative reaction with pan-cytokeratin, HBME-1, calretinin, CD99, actin, desmin, CD34, STAT6, and S100. The Ki67 proliferation index was increased in the tumor (see Fig. d). A sample was sent to an external center where molecular studies could be performed for genetic analysis, and a real-time polymerase chain reaction (RT-PCR) test detected SYT-SSX1 fusion. With these findings, the patient was given a definitive diagnosed of monophasic synovial sarcoma.\nPostoperative radiotherapy and chemotherapy were advised but refused by the patient at first place. On postoperative 4th month, the patient decided to undergo the oncological therapies, however, a six-week pregnancy was detected, therefore, only radiotherapy was applied due to the enlargement of pericardial mass. The chemotherapy was postponed for one year. After the term delivery, the patient suffered from the respiratory distress due to enlargement of the mass in the pericardial region to 14 cm in diameter, therefore, six cycles of doxorubicin and ifosfamide combination were administered. An emboli in the left main pulmonary artery, a pleural effusion in the right hemithorax, and a metastatic mass in the liver were detected. The patient whose respiratory parameters deteriorated was followed up in the intensive care unit. During this follow-up, the patient, who had myocardial infarction 21 months after the operation, died.", "summary": "The case with back pain, palpitation and weakness, was diagnosed with pericardial effusion and suspicious mass adjacent to right heart in ultrasonography. Computerized tomography showed mass 12 × 11 × 6.5 cm in size, located in right mid-anterior pericardial area, with heterogeneous internal structure, heterogeneously contrasting right heart and prominent pressure on superior vena cava. Cytopathology of pericardial effusion showed monotonous cells with oval-spindle vesicular nuclei, less amphophilic cytoplasm, evenly distributed chromatin and inconspicuous nucleoli. The pericardial mass was resected incompletely, spindle cell mesenchymal tumor with hypercellular fascicular structure and with infiltrative margins, containing a small amount of loose myxoid stroma, occasionally necrotic areas was observed histopathologically. Immunohistochemical positive reaction was for vimentin, Bcl-2, TLE-1. Accordingly, the case was diagnosed with monophasic PSS.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2535_en.txt", "fulltext": "A 62-year-old man (175.6 cm 75 kg) was scheduled for sternum closure using the right latissimus dorsi muscle flap for mediastinitis. He had gone CABG using the bilateral internal thoracic arteries 8 months before. He underwent debridement and vacuum-assisted closure (VAC) of the infected wounds 1 month after surgery. Although sternum closure using an omental flap was performed thereafter, debridement and VAC were reintroduced because of infection. His medical history included diabetes mellitus with end-stage diabetic nephropathy. He has been receiving hemodialysis, and the right lower leg amputated for diabetic gangrene. He was receiving linagliptin and mitiglinide calcium hydrate and aspirin. His laboratory data showed renal dysfunction (estimated glomerular filtration rate 6.2 ml/min) and anemia (hemoglobin 9.6 d/dl), but no abnormal coagulation was observed, and the platelet count was 145,000/μl. He also complained of postoperative nausea and vomiting (PONV) probably due to fentanyl after multiple anesthesia histories.\nConsidering the possibility of widespread surgical wound and he was taking aspirin, continuous ultrasound-guided ESPB was planned. Aspirin was continued until the day of surgery. He received no premedication. Standard monitoring and intra-arterial blood pressure monitoring were performed. General anesthesia was induced with propofol 70 mg, fentanyl 250 μg, and rocuronium 60 mg and maintained with 1.5% sevoflurane in an inhaled oxygen concentration of 60% with a total flow of 3 l/min and remifentanil 0.1–0.2 μg/kg/min. After tracheal intubation, he was placed in the left lateral decubitus position. After performing standard skin asepsis, the linear transducer (12 MHz, LOGIQ e; GE Healthcare, Chicago IL), within a sterile sleeve, was positioned on the patient in a transverse orientation and the T6 spinous process image was taken. The transducer was moved right laterally to identify the transverse process of T6 and then rotated 90° to visualize the right transverse process and the erector spinae muscles above it. A continuous nerve block set (Contiplex Tuohy Ultra set; B BRAUN, Melsungen, Germany) with an 18-G, 100-mm Tuohy needle was inserted in-plane in a caudal to cranial direction until contact with the right T6 transverse process was made. After confirming that there was no reverse blood flow, hydrodissection with 2 ml of 0.375% ropivacaine was used to confirm the correct needle tip position. Eighteen milliliters of 0.375% ropivacaine was injected, and a 20-G catheter was advanced through the needle. After injecting 20 ml of 0.375% ropivacaine again from the catheter 55 min after the initial injection, 0.2% ropivacaine was continuously infused at 5 ml/h before the start of surgery. Hemodynamic stability was achieved without the need for an additional bolus administration of fentanyl during surgery. One gram of acetaminophen was intravenously infused 30 min before the end of the operation. There were no other events of note during the operation. The operative time was 5 h and 49 min, and the anesthesia time was 8 h and 2 min.\nAfter extubation in the operating room and admission to the intensive care unit (ICU), the nerve blockade area and Numerical Rating Scale (NRS) were confirmed when sufficient consciousness was reached. Regarding nerve blockade, the cold sign was T2–T8, pinprick was T3–T8, and NRS at rest was 1/10 immediately after surgery. The main pain area was the wound where the right latissimus dorsi muscle flap was collected, but pain of the median sternum wound was not noted. Acetaminophen at 500 mg was administered orally because the NRS temporarily increased to 7/10 due to body movement. However, the NRS at rest was 1–2/10 and that while active was 4/10. For pain during movement, 3 ml of patient-controlled analgesia (PCA) consisting of 0.2% ropivacaine was flushed, and this was performed twice in the ICU. After leaving the ICU 24 h after the operation and being transported to the general ward, pain was not aggravated and the ESPB catheter was removed 56 h after the operation. After removal of the catheter, oral administration of acetaminophen during pain provided good pain control. No ESPB-related complications were observed during ESPB catheter placement or after removal. Regarding PONV, nausea was observed immediately after admission to the ICU and only a single intravenous injection of 10 mg of metoclopramide was administered. Thereafter, the patient had no PONV.", "summary": "A 62-year-old man with mediastinitis was scheduled for sternum closure using a latissimus dorsi muscle flap. He had gone coronary artery bypass grafting and was taking aspirin. After induction of general anesthesia and tracheal intubation, a catheter was inserted for ESPB from the T6 level under ultrasound monitoring and infusion of ropivacaine was started. Tracheal tube was removed in the operating room, cold sense was absent between T2-8, and analgesia was between T3-T8 after uneventful surgery. There were no complications associated with ESPB postoperatively.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1657_en.txt", "fulltext": "A 36-year-old woman with a 2-month history of abdominal and back pain was referred and admitted to our hospital for treatment. The patient had no history of disease. On physical examination, a hard, elastic, poorly flexible mass was palpable in the right upper abdomen. No signs of obstructive jaundice or abdominal tenderness were identified. Laboratory analysis revealed anemia (hemoglobin = 10.6 g/dL), thrombocytosis (43.9 × 104/μL), and elevation of C-reactive protein (2.71 mg/dl). The remaining laboratory examinations were within normal ranges, including tumor markers α-fetoprotein (AFP), carcinoembryonic antigen (CEA), and cancer antigen (CA)19-9. Abdominal ultrasonography revealed an extensive space-occupying lesion in the right lobe of the liver, 15 cm in diameter, showing a heterogeneous internal component including hemorrhage and hypervascular mural nodule . Contrast-enhanced computed tomography (CT) of the abdomen showed a cystic mass measuring 13 × 14 × 11 cm in the right lobe of the liver with enhanced mural nodule . Magnetic resonance imaging (MRI) of the abdomen showed hyperintense components on T2-weighted imaging, compatible with the hemorrhagic area . Abnormal accumulation was seen on 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) of the liver and lower abdomen . We then planned excision of the tumor and performed hepatectomy of the posterior segment and partial resection of the omentum, which had been detected on FDG-PET. Gross examination of the hepatic tumor showed a massive cystic tumor measuring 18 × 15 cm containing hemorrhagic fluid, and mucinous, hemorrhagic brownish or yellowish multinodular tumors were observed in the extra cystic wall area . The omental tumors were two slightly brownish, nodular tumors measuring 2.1 × 1.3 and 0.3 × 0.3 cm . Microscopically, both hepatic and omental tumors comprised tubular, cystic, or spindle structures of epithelioid cells with clear or eosinophilic cytoplasm . Immunohistochemically, tumor cells were positive for AE1/AE3, EMA, CK19, CK7, CD10, and calretinin , partly or weakly positive for CK5/6, D2-40, vimentin, and WT-1, and negative for HepPer1, chromogranin A, synaptophysin, CEA, inhibin α, MUC1, melan A, HMB45, CA19-9, ER, PgR, CD34, bcl-2, and β-catenin . Ki-67 index was 5–6% . The final pathological diagnosis was multiple low-grade malignant epithelioid mesothelioma. As of the time of writing, 6 months postoperatively, the patient has shown no disease recurrence.\nMalignant mesothelioma commonly arises from the pleura, but can also arise from the peritoneum, pericardium, and tunica vaginalis testis . However, malignant mesothelioma of the liver is extremely rare and coexistence with malignant mesothelioma of the greater omentum has not been addressed in the literature. Mesothelioma of the liver may arise from Glisson’s capsule, the hepatic falciform ligament or fibrous connective tissue, and then expand to the liver parenchyma . To search the literatures, we used key words of intrahepatic, malignant, and mesothelioma and found 12 case reports which have described primary intrahepatic malignant mesothelioma [–] . In detail, these cases involved six men and six women, with a mean age of 58.4 years (range, 41–68 years). Our case represents the youngest female case among these reported cases. Although conventional mesothelioma is frequently associated with asbestos exposure, only 1 case had a clear history of asbestos exposure. Among the remaining 10 cases, 8 cases had no history of asbestos exposure and 3 were not evaluated. One case had a history of viral hepatitis, and 9 cases did not. Mean tumor size was 12.2 cm (range, 3.2–24 cm), and our case showed the third largest mass. Gross finding of the tumor was a cystic mass which is not common, and there was no report of case with cystic tumor ever. This tumor contained hemorrhagic fluid; therefore, we thought that the tumor might have bled and formed cystic mass. In 10 cases, the tumor arose in the right lobe, as in our case, and only one tumor arose in the left lobe. Surgery was performed in 10 cases, and only 1 case received best supportive care, due to systemic lymph node swelling and rupture of the tumor . Pathologically, malignant mesothelioma is classifiable into three subtypes: epithelioid, sarcomatoid, and biphasic. The epithelioid type is the most common type, and tubulopapillary or solid variations can be seen in the tumor . The tumor in our case was also diagnosed as epithelioid type, but showed atypical findings such as solid and tubular, cystic, or spindle components in the tumor, clearly partitioned from normal liver tissue by a fibrous capsule. The tumor showed partial hyalinization, but no necrosis. Typical immunohistochemical features are positive results for calretinin, vimentin, cytokeratin, WT-1, and D2-40 and negative results for CD34, CEA, AFP, and Ber-EP4, as seen in our case . In terms of tumor proliferative activity, Ki-67 index in the typical malignant mesothelioma is 15–20% , but was 5–6% in our case. The tumor was therefore diagnosed as a low-grade malignant tumor, and metastasis was considered unlikely, although a primary malignant omental mesothelioma is also a rare disease. We found only 2 case reports of malignant mesothelioma of the omentum researching with key words of greater omentum, malignant, and mesothelioma [, ]. It was difficult to distinguish multiple tumor from metastatic omental tumor in our case. Multiple malignant mesotheliomas and metastasis of low-grade malignant mesothelioma are both unlikely. The omental tumor cells were positive for AE1/AE3, CK19, CK7, EMA, C D10, and calretinin, partly for CK5/6, D2-40, and vimentin, and negative for HepPer1, chromogranin A, synaptophysin, CEA, inhibin, MUC1, melan A, HMB45, CA19-9, ER, PgR, CD34, bcl-2, and β-catenin that was same findings with hepatic tumor. However, both tumors have fibrous capsule without invasion of tumor cells. Moreover, both tumors had lower proliferated activity and considered to be low-grade malignant tumor. Basing on these pathological findings, we should diagnose the tumors as multiple mesotheliomas, although we are not able to deny a possibility of dissemination. Concerning about the outcome, lymph node relapse has only been reported in 2 cases, and they were alive at 2 or 36 months after relapse without hematogenous metastatic disease [, ]. Our patient remains alive as of 6 months after surgery without relapse.", "summary": "A 36-year-old woman was admitted to our hospital for the evaluation of an elastic hard mass in the right upper abdomen. Abdominal contrast computed tomography showed a cystic mass measuring 13 × 14 × 11 cm in the right liver lobe with enhanced mural nodule. Abnormal accumulation was identified in the liver and lower abdominal area on 18F-fluorodeoxyglucose positron emission tomography. The patient underwent hepatectomy of the posterior segment and partial resection of the omentum. The final pathological diagnosis was low-grade multiple malignant epithelioid mesothelioma based on characteristic immunohistochemical findings. As of 6 months postoperatively, the patient has shown no disease recurrence.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_723_en.txt", "fulltext": "We report the case of a 2 years and-5-month-old boy admitted in our clinic for fever with the onset 3 days before admission associating abdominal pain and diarrhea within the last 24 hours. The anamnesis did not reveal any possible exposure to COVID-19 infections or other chronic underlying pathologies.\nThe clinical exam at the time of admission revealed influenced general status, bilateral palpebral edema and conjunctivitis, mucocutaneous signs of dehydration, abdominal tenderness at palpation. The patient weighed 15 kilograms.\nThe laboratory test performed on the day of admission pointed out lymphopenia (1560/mm3), thrombocytopenia (108x103/mm3), anemia (hemoglobin – Hb 10.9 g/dL), elevated C-reactive protein – CRP (162 mg/L), erythrocyte sedimentation rate (50 mm/h) and ferritin levels (389.3 ng/mL), hyponatremia (132 mmol/ L), hypopotassemia (3.94 mmol/L), hypertriglyceridemia (476.3 mg/dL), elevated D-dimer (>5 μg/ml), increased troponin (16.6 ng/L) and NT-proBNP (19,831 pg/ml). The rapid urine exam was negative. The chest X-ray, abdominal ultrasound and echocardiography showed no pathological findings. The blood and urine culture were negative. Corroborating the previously mentioned findings we raised the suspicion of pediatric inflammatory multisystem syndrome possibly associated to COVID-19. We performed a RT-PCR test for SARS-CoV-2 infection, but it was negative. Nevertheless, the serology (IgG anti-SARS-CoV-2) for this infection was positive. Thus, established the diagnosis of PIMS-TS.\nWe initiated intravenous immunoglobulin in a dose of 2 g/kg/day associated with empirical antibiotic (ceftriaxone), anticoagulation therapy (low-molecular weight heparin) and symptomatic drugs (antipyretics, antiemetics, and antidiarrheics). In spite of this therapeutic approach, the fever persisted for the following 36 hours with no clinical improvement. Moreover, the laboratory test performed on the 3rd day of admission showed an increase of the CRP value (216.65 mg/L), and the persistence of elevated NT-proBNP (9,884.2 pg/mL) and D-dimer (1,198 ng/mL), associating also hypoalbuminemia (2.45 g/dL). Therefore, we initiated intravenous methylprednisolone (10 mg/kg/day) for 3 days followed by slow tapering and substitutive treatment with human albumin. We also repeated the echocardiography and we found minimal pericardial effusion, slight dilation of the left cavities (left ventricle - LV z score: 1.6, LV sphericity index: 1.66), regional wall motion abnormalities with dyskinesia of the inferior and infero-septal basal segments of the LV. Moreover, the LV systolic dysfunction was affected, with LV ejection fraction of 40%, with mild mitral regurgitation ; but no coronary impairment. Thus, following pediatric cardiologist’s recommendation, we associated angiotensin converting enzyme inhibitors (Lisinopril), Spironolactone and Hydrochlorothiazide. The serial echocardiography performed during the following days of admission revealed the resorption of the pericardial effusion, with improvement of LV function, correlated with decreased serum levels of NT-proBNP (123.1 pg/mL) and troponin (5.2 pg/mL).\nThe patient was discharged on the 11th day of admission without any complaints and most of the laboratory parameters within normal ranges, except of D-dimer. We recommended the continuation of anticoagulant therapy for another week, as well as Lisinopril, Spironolactone and Hydrochlorothiazide for the following 2 weeks.", "summary": "We report the case of a 2 years and-5-month-old boy admitted in our clinic for fever, abdominal pain and diarrhea. The clinical exam at the time of admission revealed influenced gen-eral status, bilateral palpebral edema and conjunctivitis, mucocutaneous signs of dehydration, and abdominal tenderness at palpation. The laboratory tests performed pointed out lymphopenia, thrombocytopenia, anemia, elevated C-reactive protein - CRP, erythrocyte sedimentation rate and ferritin levels, hyponatremia, hypopotassemia, hypertriglyceridemia, elevated D-dimer, in-creased troponin and NT-proBNP. The real-time polymerase chain reaction (RT-PCR) test for SARS-CoV-2 infection was negative, but the serology was positive. Thus, established the diagnosis of PIMS-TS. We initiated intravenous immunoglobulin, empirical antibiotic, anticoagulation therapy and symptomatic drugs. Nevertheless, the clinical course and laboratory parameters worsened, and the 2nd echocardiography pointed out minimal pericardial effusion, slight dilation of the left cavities, dyskinesia of the inferior and septal basal segments of the left ventricle (LV), and LV systolic dysfunction. Therefore, we associated intravenous methylprednisolone, angiotensin converting enzyme inhibitors, spironolactone and hydrochlorothiazide, with outstanding favorable evolution.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1013_en.txt", "fulltext": "A 15 year old Caucasian female was transferred from a secondary care paediatric unit. She presented with a two-day history of progressive dyspnoea, cough and palpitations on a background of recent onset arthralgia, alopecia and oral ulceration. Clinical examination revealed hypertension (blood pressure 170/110 mmHg), pallor with a malar rash, symmetrical polyarthritis of the interphalangeal and metacarpophalangeal joints, alopecia and oral ulceration.\nInvestigations revealed normocytic anaemia, haemoglobin 95 g/l (normal 120-160 g/l), lymphopaenia, lymphocytes 0.9 × 109/l (normal 1.2–5.2 × 109/l)), elevated inflammatory markers with an erythrocyte sedimentation rate (ESR) of 77 mm/hr. (normal 1-9 mm/hr) and c-reactive protein (CRP) of 38 mg/l (normal < 10 mg/l) and moderately impaired renal function with urea 14.4 mmol/l (normal 2.0–6.0 mmol/l), creatinine 154 μmol/l (normal 30-90 μmol/l). Coagulation screen showed a slightly prolonged prothrombin time (PT) of 13 s (normal 10.2–12.0 s) but was otherwise normal. Albumin was low (28 g/l, normal 36-50 g/l) and liver function tests were normal. Microscopic haematuria and proteinuria were present with an elevated urine albumin:creatinine ratio of 1217 mg/mmol (normal < 3.4 mg/mmol). Antinuclear antibody titres were strongly positive with a titre of 1:160, speckled pattern. Anti double-stranded DNA was positive with a titres of > 379 IU/ml (normal 0-10 IU/ml) and positive Crithidia assay >/= 1:160. Anti-Smith and anti-RNP antibodies were both positive with titres of > 480 U/ml (normal 0–5.0 U/ml) and > 240 U/ml (normal 0-5 U/ml) respectively. There was marked hypocomplementaemia with C3 0.44 g/l (normal 0.7–1.7 g/l), C4 0.06 g/l (normal 0.1–0.7 g/l) and absent CH100 classical and alternative pathway components. Antiphospholipid, anti-SSA and anti-SSB antibodies were all negative. Chest x-ray showed bilateral pleural effusions and cardiomegaly with a cardiothoracic ratio of 0.67. Initial echocardiography showed a large pericardial effusion with diastolic compression of the right atrium and ventricle suggestive of cardiac tamponade. The left ventricle was dilated with an ejection fraction of 25% and there was mild mitral, tricuspid and aortic valvular regurgitation. Treatment was commenced with high-dose intravenous methylprednisolone (30 mg/kg/dose, maximum dose of 1 g) and diuretics and immediate transfer to a tertiary paediatric intensive care unit was arranged.\nOn admission to the intensive care unit she had developed periorbital oedema and ascites with worsening dyspnoea and reduced oxygen saturation. Echocardiography revealed a large pericardial effusion, oedematous myocardium and valvulitis with an ejection fraction of 13% with no evidence of tamponade (see Fig. ). Renal function deteriorated further with a creatinine increase to 270 μmol/l (normal range 30-90 μmol/l) and the patient became anuric. Intermittent positive pressure ventilation, inotropic support, plasma exchange and haemodialysis were required. High-dose intravenous methylprednisolone was continued for 3 days and then changed to oral prednisolone at 1 g/kg/day. Cyclophosphamide was commenced at a dose of 850 mg/m2 on day four of admission due to severe renal impairment and ongoing need for haemodialysis and multiorgan involvement.\nFollow-up echocardiography showed normalisation of function by day five of admission with a small pericardial effusion as the only persistent abnormality. Renal biopsy revealed grade 4 lupus nephritis. The patient was discharged from the intensive care unit on day seven of admission and subsequently discharged from the hospital on day fourteen. Treatment at discharge included a weaning dose of prednisolone, hydroxychloroquine, enalapril and carvedilol. Cyclophosphamide treatment was continued monthly for a total of six doses after which the patient was maintained on further immunosuppression. Remission has been maintained with mycophenolate mofetil and hydroxychloroquine over the past 2 years.", "summary": "We report a case of jSLE in a 15 year old Caucasian female presenting with an acute episode of pancarditis and multiorgan dysfunction who was successfully treated with systemic corticosteroids and cyclophosphamide.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2501_en.txt", "fulltext": "A 76-year-old woman presented at our department with an abdominal pulsatile mass. She had hypertension (nifedipine 20 mg, candesartan 4 mg, indapamide 0.5 mg), dyslipidemia, congestive heart failure, and complete atrioventricular block. She had previously undergone an operation that included endovascular aneurysm repair and prophylactic accessory renal artery coil embolization for advanced uterine cancer. Enhanced computed tomography (CT) imaging revealed an abdominal aortic aneurysm (AAA) with a maximum diameter of 53 × 57 mm and a right ARA (4 mm in diameter) . Since open repair was considered risky due to her coexisting diseases, we decided to perform EVAR despite the presence of ARA.\nUnder general anesthesia, bilateral transfemoral access was obtained via surgical cutdown, and 7Fr short sheaths were placed in both common femoral arteries. Prior to EVAR, the ARA was selectively catheterized with a 6Fr RDC guiding catheter and a 5Fr RDC catheter, and embolization was performed with two 0.018-in coils (Interlock, Boston Scientific, Marlborough, MA, USA). After coiling, the main body of the stentgraft (Aorfix™, Lombard Medical, Oxfordshire, UK) was deployed with its proximal end just below the renal arteries, and bilateral common iliac arteries were used as distal landing zones. We completed the procedures having seen no perfusion defect of the inferior pole of the right kidney from the right renal artery on completion angiography .\nThe postoperative course was uneventful, and the patient was discharged 7 days later. Postoperative eGFR (58.4 ml/min) was not significantly different from preoperative (56.7 ml/min). After surgery, blood pressure was under control, and no additional blood pressure medicines were required. Postoperative enhanced CT showed that there was no endoleak. The distal portion of her right ARA from coils including the right kidney was well perfused .", "summary": "A 76-year-old woman was admitted to our hospital after noticing an abdominal pulsatile mass. Computed tomography (CT) imaging revealed an abdominal aortic aneurysm (AAA) with a maximum diameter of 53 mm. Preoperative CT scan showed a right ARA, 4 mm in diameter, which was considered likely to lead to type II endoleak following EVAR. ARA coil embolization was performed at the time of EVAR. We observed no endoleaks and no infarct of the inferior pole of the right kidney on completion angiography. The postoperative course was uneventful, and the patient was discharged 7 days later. Postoperative eGFR (58.4 ml/min) was not significantly different from preoperative level (56.7 ml/min). After EVAR, blood pressure was under control, and no additional anti-hypertensive medicines were required. Postoperative enhanced CT image showed that the distal portion of the ARA was well perfused without type II endoleak from ARA.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_697_en.txt", "fulltext": "Our patient was a 43-year-old man with a weight of 93 kg and height of 174 cm. He had been undergoing hemodialysis for 20 years because of chronic renal failure. He had been taking cinacalcet, calcium carbonate, montelukast, loratadine, and nalfurafine before the surgery. There were no preoperative symptoms of suspected pheochromocytoma and no history of hypertension. His blood pressure was 140/82 mmHg, heart rate 76 beats/min, body temperature 36.2 °C, and normal respiratory rhythm with an O2 saturation of 96% on room air. Blood tests demonstrated BUN of 60.2 mg/dL and creatinine of 13.3 mg/dL, but coagulability, liver function, and electrolytes were normal.\nA close examination of hematuria revealed a tumor in the right renal pelvis, and retroperitoneal total right nephroureterectomy was scheduled. The tumor in the right adrenal gland was followed up because there was no clear malignant finding on CT. On the day before surgery, 5100 mL of body fluid was removed by hemodialysis. Propofol and remifentanil were used for the induction and maintenance of anesthesia. After tracheal intubation, periodic systolic blood pressure fluctuations within the range of 70–260 mmHg, diastolic blood pressure fluctuations within the range of 40–90 mmHg, and heart rate of 80–110 beats/min were observed at intervals of approximately 8 min . Intermittent and continuous administration of nicardipine was not effective for suppressing the fluctuation, which lasted for approximately 3 h and disappeared spontaneously after completion of laparoscopic surgery. Intermittent and continuous administration of nicardipine was performed because of this circulatory fluctuation; however, it could not be suppressed. Periodic blood pressure fluctuations disappeared spontaneously at the end of laparoscopic surgery. Blood samples were collected during the cycle of circulatory fluctuations. The catecholamine levels of the blood samples were as follows: first blood pressure fluctuation: adrenaline 3.22 ng/mL, noradrenaline 1.98 ng/mL, and dopamine 0.02 ng/mL; second blood pressure fluctuation: adrenaline 6.32 ng/mL, noradrenaline 3.12 ng/mL, and dopamine 0.02 ng/mL; and at the end of blood pressure fluctuation: adrenaline 0.36 ng/mL, noradrenaline 0.47 ng/mL, and dopamine 0.02 ng/mL. A postoperative examination revealed that the highest blood catecholamine levels were adrenaline, with 0.03 ng/mL (normal value: 0–0.10 ng/mL); noradrenaline, with 0.52 ng/mL (normal value: 0.10–0.50 ng/mL); and dopamine, with 0.03 ng/mL (normal value: 0–0.03 ng/ml). In addition, scintigraphy identified an increased uptake of 131iodine-metaiodobenzylguanidine corresponding to the left adrenal gland, suggesting pheochromocytoma . After the diagnosis of pheochromocytoma, oral treatment with doxazosin 2 mg/day was administered, and left adrenalectomy was performed 4 months after the initial surgery.\nBefore surgery, his blood pressure was 170/92 mmHg, heart rate 80 beats/min, body temperature 36.5 °C, and normal respiratory rhythm with an O2 saturation of 96% on room air. Blood tests demonstrated Hb of 10.4 g/dL, BUN of 46.9 mg/dL, and creatinine of 11.6 mg/dL, but coagulability, liver function, and electrolytes were normal. Echocardiography showed left ventricular hypertrophy and mild left ventricular wall hypokinesis. On the day before surgery, 4300 mL of body fluid was removed by hemodialysis. Pheochromocytoma resection was performed under general anesthesia with propofol and remifentanil. As with the initial surgery, periodic circulation fluctuations occurred after tracheal intubation at the time of anesthesia induction, but the fluctuations were approximately 10 min apart, and the degree of fluctuation was smaller than that of the first surgery. Intermittent and continuous administration of nicardipine and landiolol was performed for circulatory fluctuation; however, once again, the fluctuations could not be suppressed. Dopamine was administered to maintain circulation after tumor removal. The periodic fluctuations disappeared after the ligation of the left adrenal artery and did not occur thereafter. The intraoperative course of the second surgery is shown in Fig. .", "summary": "A 43-year-old man presented with periodic blood pressure fluctuations during surgery for a renal pelvic tumor. His blood levels of catecholamines (ng/mL) changed dramatically over a short time during blood pressure fluctuations: adrenaline 0.36 to 3.22, noradrenaline 0.47 to 1.98, and dopamine 0.02. After the diagnosis of pheochromocytoma, oral treatment with doxazosin 2 mg/day was administered, and left adrenalectomy was performed 4 months after the initial surgery. Periodic circulation fluctuations occurred after tracheal intubation at the time of anesthesia induction, but the degree of fluctuation was smaller than that of the first surgery.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_12_en.txt", "fulltext": "A 15-year-old boy presented to our University Hospital, a tertiary care neurology facility, with fever, headache, and altered sensorium of 12-day duration. At admission, he was febrile (101 °F), with a pulse rate of 110/min and a blood pressure recording of 114/74 mmHg; his Glasgow Coma Scale score was 10 (E3M4V3). There were no petechiae or signs of bleeding over the skin or any mucosal surface. There was no focal neurological deficit and neurological examination was normal, including (absence of) signs of meningeal irritation. Laboratory evaluation revealed the following estimations: hemoglobin of 11 g/dl, total leukocyte count of 3300 cells/mm3, and platelet count of 22,000 cells/mm3 which dropped to 8000 cells/mm3 on next day. His aspartate aminotransferase level was 155 U/L, alanine aminotransferase was 140 U/L and alkaline phosphatase was 56 U/L. Additional biochemical parameters, renal function tests, blood sugar, electrolytes, and arterial blood gas analysis, were normal. Malarial parasite was not detected in the peripheral blood smear. The cerebrospinal fluid examination, cytological and biochemical, was normal. IgM antibody against dengue virus was positive both in serum and cerebrospinal fluid; meanwhile, dengue NS1 antigen was negative. Electroencephalography revealed generalized slowing. On magnetic resonance imaging of the brain, signal changes were seen in bilateral parietooccipital and left frontal region (left hemisphere was more involved than the right hemisphere). There were diffuse subcortical white matter changes along with suggestion of hemorrhage on gradient echo sequence. Subtle hyperintensity on T2 W images was also noted in bilateral basal ganglia. Gadolinium-contrast study revealed a gyriform enhancement suggestive of cortical laminar necrosis . He was managed conservatively and given platelet transfusion. The patient responded well to management and became fully conscious in 7 days.", "summary": "A 15-year boy presented with fever, the headache and altered sensorium of 12-day duration. On neurological examination, his Glasgow Coma Scale score was 10 (E3M4V3). There was no focal neurological deficit. Laboratory evaluation revealed leukopenia and marked thrombocytopenia. Dengue virus IgM antibody was positive both in serum and cerebrospinal fluid. Magnetic resonance imaging of the brain revealed signal changes in bilateral parietooccipital and left frontal regions (left hemisphere more involved than the right hemisphere). There was gyriform enhancement bilateral parietooccipital regions consistent with cortical laminar necrosis. Bilaterally diffuse subcortical white matter was also involved and subtle T2 hyperintensity involving both basal ganglia was noted. Gradient echo sequence revealed presence of hemorrhage in the subcortical white matter. Patient was treated conservatively and received platelet transfusion. Patient became fully conscious after 7 days.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_959_en.txt", "fulltext": "We reported a case of a 46 years old woman admitted to our hospital because of prolonged recurrent vomiting in a severe state of malnutrition. A year before she had been submitted to duodenopancreatectomy because of a stenotic duodenal adenocarcinoma. Immediately, after hospital admission she underwent to a new explorative laparotomy because of worsening symptoms. In this second abdominal surgery she underwent to gastric enteric anastomosis because of sub-occlusion.\nHer ability to absorb substances was already compromised by the first operation. During the last year she did not received an adequate nutrition, moreover she had no vitamins supplement in her diet. After the second surgery she was fed by total parenteral nutrition with glucose solutions, amino-acids solutions and vitamins complex (Cernevit – Baxter@ spa, Roma, Italy) commonly administered to post-surgical patients containing 3,51 mg of thiamine.\nAfter five days from the gastric enteric anastomosis, she developed a Pseudomonas Aeruginosa’s severe sepsis with acute renal failure; so, she was transferred to our Intensive Care Unit (ICU). At ICU admission, she was agitated but she still had a normal Glasgow Coma Scale (GCS 15/15). Brain Computed Tomography (CT) scan control was negative and altered neurological status of the patient was imputed to the severe sepsis. Her blood tests showed leucopenia (1680/ml) and thrombocytopenia (34000/μl).\nWe performed an electroencephalogram (EEG) control that evidenced slow diffused anomalies, without paroxysms or focal signs. During the night she was sedated with benzodiazepines and intubated because of respiratory worsening.\nThe following day, at the neurological examination she was drowsy, she had no response to pain stimuli, no oculocephalic reflex, limbs areflexia and negative Babinski reflex. A new EEG control pointed out a diffused cerebral suffering state. Simultaneously her renal and liver function got worse due to the evolution of the septic state. Her feeding was still composed by glucose solutions, vitamins (Cernevit – Baxter@ spa, Roma, Italy) and amino acid intravenously administered. Then, we decided to perform a new brain CT scan with contrast in order to diagnose the cause of the worsening. This CT scan was performed at day 7 after surgery. The report of the CT scan showed a faded hyperdensity of the mammillary bodies and symmetrical hemorrhagic lesions in the quadrigeminal plate . The report suggested the need of a further diagnostic study with MRI because of the suspicion of a possible metabolic encephalopathy compatible with WE complicated by petechial hemorrhages.After the CT scan, we introduced thiamine 100 mg intravenously once a day, as supplement to the usual feeding. At the following neurological examination her GCS was 7/15, she did not open her eyes, did not emit verbal responses and localized with the right arm to painful stimuli. So 2 days later, we performed a brain magnetic resonance imaging (MRI). The MRI confirmed the lesions described in the CT scan and also added some elements compatible with WE: hyperintensity lesions in T-2 weighted images in the hypothalamus, medial thalamus, periaqueductal grey matter, anatomical regions of the mammillary bodies and superior cerebellar peduncles. , Mammillary bodies and optic tracts were difficult to assess due to a sub acute blood clot occupying the lower anterior portion of the third ventricle. The study was concluded with the MRI angiography to exclude a malformation of the vessels that had been able to generate the bleeding. Anyway, MRI angiography 3D-TOF was negative for vascular malformations. All these radiological findings were consistent with the diagnosis of WE. In the meantime, patients developed septic shock due to multi-resistant drug Enterococcus Faecium with severe low platelets count needing a mean of 20 Units of platelets transfused per day to maintain a serum level greater than 30000/μl.\nNo neurosurgical indications were given for the hemorrhagic brain lesions. So, we increased thiamine dosage up to 250 mg intravenously three times per day. This therapy was prolonged for one week, and after 4 days of high-dose thiamine supplementation neurological status of the patient had a significant improvement despite severe septic shock, she was able to open her eyes spontaneously and she performed simple commands.\nNevertheless, septic shock worsened and thrombocytopenia became refractory to platelets transfusions, hemodynamic instability became unresponsive to vasopressors and liver hypoperfusion worsened. Despite severe efforts used to control septic shock and thrombocytopenia, she died on the 21st day after surgery because of massive cerebral bleeding and unresponsive cerebral edema. The macroscopic autopsy of the brain was not nullifying because intracerebral hemorrhage and severe cerebral edema led to colliquation the typical areas of the Wernicke’s disease.", "summary": "We reported a case of a 46 years old woman who underwent, one year before, to cephalic duodenopancreatectomy complicated with prolonged recurrent vomiting. She underwent to a second surgical operation for intestinal sub-occlusion and postoperatively she developed septic shock and hemorrhagic Wernicke's disease. After ICU admission, because of neurological deterioration, she underwent CT scan and MRI that highlighted a strong suspicion for Wernicke's disease. We treated her with an initially wrong low dose of thiamine, then after MRI we increased the dosage with a neurological status improvement. Despite therapeutic efforts used to control septic shock and thrombocytopenia, she died on the 21st day after surgery because of massive cerebral bleeding and unresponsive cerebral edema.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2123_en.txt", "fulltext": "A 76 year old ‘never smoker’ female with no past medical history was diagnosed with locally advanced NSCLC during investigations for a community-acquired lower respiratory tract infection. The Medical Research Council (MRC) Dyspnoea Score was 3 and there was a dry cough. Computed tomography (CT) of the chest demonstrated a 4.5 cm (anterior-posterior) × 4.1 cm (craniocaudal) left lung upper lobe mass with abutment of the mediastinal pleura and distal atelectasis and pneumonitis .\nAn 8 mm ipsilateral lymph node was visible at station 10. Multiple sub-centimetre lung nodules were noted throughout the right lung. Histological and immunohistochemical assessment of core biopsies from the primary lesion via bronchoscopy favoured the adenocarcinoma subtype of NSCLC (see Table ). The molecular analysis revealed ALK fusion protein overexpression along with ALK rearrangement. This result is in keeping with an ALK rearranged adenocarcinoma. The main lesion had an SUVmax of 16 on 18fluorodeoxyglucose positron emission topography-CT (PET-CT) imaging and no other lesions were avid. Following a review of the imaging at the multidisciplinary meeting (MDM), staging was offered at T2 N0 M0 (TNM 8 ), and in light of the patient’s fitness, radical treatment was recommended.\nDuring the assessment period for a primary lobectomy, the patient developed symptomatic atrial fibrillation. She underwent a successful direct current (DC) cardioversion and was discharged on edoxaban. Three weeks later the patient was noted to be in atrial fibrillation once more during an inpatient admission for the management of chest sepsis, for which she was discharged on digoxin. She was electively admitted to the Cardiology ward 6 weeks later for a second DC cardioversion procedure. Under conscious sedation, the patient received one synchronised shock of 120 J delivered via anterior-posterior paddles ie one placed at the left parasternal edge, one at the corresponding position on the patient’s back. One week post-procedure the patient attended the Emergency Department complaining of dysarthria and left-sided hemiparesis. CT and magnetic resonance imaging (MRI) of the brain confirmed the presence of a dense right-sided middle cerebral artery territory infarction (see Fig. ). The patient was in sinus rhythm, transthoracic echocardiography was unremarkable and mild bilateral carotid atheroma only was noted on ultrasonography (< 50% stenosis), suggestive for a stroke secondary to a delayed cardiogenic embolus related to atrial fibrillation, despite anticoagulation.\nFive days later, haemorrhagic transformation of the stoke was detected on MRI following clinical deterioration. A 3.5 cm intracerebral haematoma was identified within the right basal ganglia, causing effacement of the right lateral ventricle frontal horn, which was managed conservatively. The clinical condition stabilised and both her speech and weakness improved with rehabilitation from the department of Stroke Medicine.\nAs the patient’s ECOG PS recovered to 2 and her breathlessness resolved, work-up for radical treatment resumed, given her ongoing determination to gain control over the cancer. Updated cross-sectional imaging demonstrated complete regression of the left upper lobe lesion and a reduction of the previously documented mediastinal lymph node. Remaining atelectasis had a maximum standard uptake value (SUVmax) 2.7 on repeat PET-CT imaging (8 months since first PET). A review of the patient’s medications was undertaken searching for possible effects on FDG uptake, which was negative. The merits and risks of radical radiotherapy versus active surveillance were explored with the patient who elected to proceed with the latter.\nClinical review after 6 months of active surveillance, dry cough and mild dyspnoea were reported by the patient. Corresponding with the imaging findings, thoracic imaging with CT showed increased patchy parenchymal changes at the site of the previous left upper lobe lesion without associated hilar or mediastinal lymphadenopathy. Repeat PET-CT imaging demonstrated increased uptake (SUVmax 10.2) in a sub-pleural 4 cm mass in keeping with local relapse (see Fig. ), and no additional sites of disease.\nAs the pulmonary function tests (FEV1 90% predicted; TLCO 80% predicted) were favourable and physical fitness had stabilised (ECOG PS 2) the patient was consented for a course of radical thoracic radiotherapy without chemotherapy. She completed 55 Gy in 20 fractions planned with the intensity modulated radiotherapy technique and delivered as 6 MV arc therapy, with daily online cone beam-CT image guidance, treating Monday to Friday for 4 weeks . Target volumes were subject to peer review . Tumour shrinkage was noted during routine offline imaging review.\nThere were no acute toxicities during routine clinical assessments on treatment, or at 6 weeks post-radiotherapy. On clinical review 4 months after treatment completion the patient was more frail and had continued respiratory symptoms. Around this time, the first radiological follow-up scan demonstrated radiation pneumonitis focally in left upper lobe. At 1 year the patient returned to ECOG PS 1 and the imaging demonstrated stable disease locally and no evidence of distant relapse. At 18 months post-radiotherapy there was radiologic progression in the lungs with new pulmonary nodules and effusion and new bone metastases correlating with new symptoms of dyspnoea, cough and back pain. Owing to poor performance status, she was not considered fit for systemic therapy including ALK-targeted therapy and was managed with multi-disciplinary best supportive care until her death 5 months later.", "summary": "A 76 year old 'never smoker' female with an ALK-rearranged left upper lobe T2 N0 NSCLC experienced a stroke following elective DC cardioversion for new atrial fibrillation. Following a good recovery, updated imaging demonstrated complete regression of the left upper lobe lesion and a reduction of the previously documented mediastinal lymph node. Remaining atelectasis was non-avid on repeat PET-CT imaging, 8 months from the baseline PET-CT. When the patient developed new symptoms 6 months later a further PET-CT demonstrated FDG-avid local recurrence. She completed 55 Gy in 20 fractions but at 18 months post-radiotherapy there was radiological progression in the lungs with new pulmonary metastases and effusion and new bone metastases. Owing to poor performance status, she was not considered fit for targeted therapy and died 5 months later.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_822_en.txt", "fulltext": "A 69-year-old woman presented with pericardial discomfort and was subsequently diagnosed with advanced gastric cancer after upper gastrointestinal endoscopy. The patient underwent laparoscopic distal gastrectomy with D2 lymph node dissection, and the final pathological diagnosis was U, Less, Type 2, 46 × 38 mm, adenocarcinoma with enteroblastic differentiation, pT4a, INFa, Ly0, V0, pPM0, pDM0, pN2, and pStageIIIA (Japanese Classification of Gastric Carcinoma) . Human epidermal growth factor type 2 was negative, combined positive score of programmed cell death ligand 1 was 5 or higher and microsatellite instability was negative. The patient received oral S-1 (80 mg/m2 twice a day for 1–28 days) as postoperative adjuvant chemotherapy, but liver metastases were observed 8 months later in segments 4 (41 × 39 mm) and 8 (24 × 22 mm). The location and number of metastases were confirmed by contrast-enhanced computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET)–CT. There was no elevation observed in the tumor markers carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9). The patient was switched to weekly paclitaxel plus ramucirumab, but Grade 4 neutropenia was observed during the first cycle, and nivolumab monotherapy was initiated (3 mg/kg intravenously every 2 weeks) after consultation with the patient .\nAfter eight cycles, the target lesion of liver metastasis achieved a PR by Response Evaluation Criteria in Solid Tumors (RECIST), and nivolumab treatment was continued. After 18 cycles, liver metastases had shrunk to 24 × 22 mm in segment 4 and 11 × 9 mm in segment 8, and PET–CT showed no abnormal fluorodeoxyglucose (FDG) accumulation in liver metastases, and no new metastases appeared. CEA and CA19-9 did not show any significant changes within the normal range during the course of treatment. However, at this point, the patient developed a Grade 3 immune-related adverse event (irAE), a pemphigoid skin disorder, and the nivolumab treatment was discontinued.\nAs the metastases were controllable with anticancer agents, no new lesions appeared, and the patient’s performance status was maintained, it was decided to resect the liver metastases. The patient underwent laparoscopic partial hepatectomy for segments 4 and 8, resulting in pathological CR . After a medication withdrawal period due to the surgery, the skin disorder had resolved, and postoperative chemotherapy with nivolumab was resumed. However, due to a recurrence of the skin disorder, the chemotherapy was immediately stopped. The patient was followed up without treatment, and no recurrence findings were observed for 25 months postoperatively.", "summary": "A 69-year-old female presented with pericardial discomfort and was diagnosed with advanced gastric cancer following upper gastrointestinal endoscopy. Laparoscopic distal gastrectomy with D2 lymph node dissection was performed, resulting in a final pathological diagnosis of Stage IIIA. The patient received postoperative adjuvant chemotherapy with oral S-1 therapy, but was found to have multiple liver metastases at 8 months postsurgery. Weekly paclitaxel and ramucirumab therapy was initiated, but the patient experienced adverse side effects, leading to the discontinuation of treatment. Nivolumab monotherapy was then administered for 18 cycles, resulting in a partial therapeutic response and PET-CT revealed a complete metabolic response. However, the patient developed a Grade 3 pemphigoid as an immune-related adverse event, leading to the cessation of nivolumab. The patient underwent laparoscopic partial hepatectomy. Postoperative pathology showed no residual tumor cells, indicating a complete response. At present, 25 months after surgery, the patient was alive without recurrence.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2476_en.txt", "fulltext": "A 28-year-old mestizo man with a 3-year history of inflammatory arthritis and dry mouth and eyes was admitted to our hospital. He was admitted to our hospital at the request of an external consultant in January 2008; the initial laboratory evaluation is listed in Table . Methotrexate was initiated with good response; 1 year later methotrexate was changed to leflunomide because of liver toxicity. Globulin levels at admission and 1 year after were 5.2 and 7.2g/dL, respectively. Of interest, although he also had positive anti-double-stranded deoxyribonucleic acid (DNA) and anti-nucleosomes antibodies, which normally have a higher specificity for the diagnosis of SLE, at admission we could not make a diagnosis because of the absence of clinical manifestations other than arthritis and sicca.\nHyperemia and mild eye pain appeared in March 2009, and a diffuse scleritis was diagnosed. Therefore leflunomide was suspended and he received prednisone (1mg/kg), tapered to 5mg/day in 1 month and suspended after another month because a mouth ulcer appeared.\nMultiple important mucosal bleeding episodes (epistaxis and gingival hemorrhage) started in 2009, and coagulation tests were conducted through 2010, with the following results: prothrombin time, international normalized ratio and activated partial thromboplastin times were normal (11/10.4 seconds; 1.2; 31.1/26.9 seconds, respectively), VWF activity was observed with low ristocetin cofactor (RCo) at 33.6UI/dL, high VWF antigen (VWF:Ag) >200UI/dL, and a low VWF:RCo to VWF:Ag ratio. Furthermore, an Ivy bleeding time test of more than 15 minutes and normal factor VIII activity (70UI/dL) were observed. An in vitro test showed that the patient’s IgG inhibited the VWF:RCo of normal plasma, therefore the RCo activity inhibition test was positive, although this was not necessary for the diagnosis. A characteristic aVWD laboratory test with the identification of a causal underlying disease, integrated the diagnosis .\nThe patient was admitted to the ER in April 2011 with a 24-hour evolution of progressive dyspnea, cough, thoracic pain, and palpitations, 104beats/minute, 60/40mmHg, temperature of 38°C, pulse oximetric saturation 88% and 30 breaths/minute. Cold, pale and mottled skin was also observed; laboratory values at ER admission are listed in Table . There were no signs of arthritis or bleeding. Initial crystalloid reanimation was made with partial response, and persistent tachypnea and hypoxemia (arterial oxygen tension/fraction of inspired oxygen = 245.7) precluded a rapid intubation sequence. He was then transferred to the intensive care unit (ICU) 12 hours later. Initial laboratory ER patient data are listed in Table ; Acute Physiology and Chronic Health Evaluation II and Sequential Organ Failure Assessment values at ICU admission were 11 and 27 points respectively.\nAn electrocardiogram showed an incomplete right bundle block not previously detected. A chest X-ray revealed no infiltrates, and complementary laboratory tests did not suggest a related infection. The placement of a pulmonary artery catheter (PAC) was made under the indication of a differentiation between cardiogenic and non-cardiogenic shock (see Table ). The initial patterns showed a precapillary pulmonary hypertension; therefore, in the autoimmune context of the patient, although serial negative antiphospholipid antibodies were noted in his history, acute pulmonary embolism was the first choice for diagnosis. Pulmonary angiography was conducted, and when no clot was discovered, PAH associated with connective tissue disease was considered because pulmonary hypertension has been historically associated with connective tissue diseases.\nThese PAC parameters were the same as observed by Condliffe et al. and Murata et al. except for the pulmonary vascular resistance index, which was greater in our patient in comparison to the Condliffe et al. cases (715 versus 1525 dyne·second/(cm5·m2)). Because connective tissue disease may cause PAH because of the vasculature pathology, the difference in more severe resistance may be explained by the changes in the rheological properties (1 poise (P) = 1g·(second·cm)-1 = 1dyne·second/cm2) present in the HVS, where a decrease in the blood flow can explain the resistance increase. Laboratory blood samples hinted about the thickness of blood, so viscosity was measured and found to be 65.8cP (<1.9cP).\nLymphadenopathy and organomegaly were ruled out. A bone marrow biopsy did not show clonality of plasma cells. Serum protein electrophoresis confirmed the presence of a massive polyclonal hypergammaglobulinemia, and no paraproteinemia or monoclonal cell population was found from the electrophoretic pattern of the patient’s plasma. Hypergammaglobulinemia was the cause of HVS associated with autoantibodies. Three sessions of plasma exchange therapy were made. Albumin and frozen fresh plasma were used as the expander, and one circulating blood volume was utilized. Significant mental, hemodynamic recovery and clinical outcomes became evident. A negative Q value was observed in the HVS in this patient after taking into account the Starling vascular permeability formula, where the microvasculature is influenced by protein content and transcapillary fluid flux, specifically, where Q is equal to volume of flow across the capillary wall. This finding led to the conclusion that dilution of protein content may alleviate the HVS, and the fluid administration was considered supportive therapy with judicious consideration of the compartment shift of the solutions. His renal function returned to basal state and he was extubated on day 7 of ICU admission. He was then discharged from the ICU and hospital, respectively with good prognosis (Charlson comorbidity index of 0 points).\nThe patient now attends external consult without respiratory symptoms. One month after hospital discharge his arthritis returned and methotrexate was resumed with good clinical response. Hydroxychloroquine with steroids were initiated because of persistent elevated anti-double stranded DNA (last value at February 2013, 20.2U/mL), no bleeding sign had been present with persistent positive VWF:Ag values.", "summary": "A 28-year-old mestizo man with a 3-year history of inflammatory arthritis was admitted to our hospital. An overlap of rheumatoid arthritis with systemic lupus erythematosus was suspected; therefore methotrexate was initiated, and later changed to leflunomide because of liver toxicity. Prothrombin time, international normalized ratio and activated partial thromboplastin times were normal (11/10.4 seconds; 1.2; 31.1/26.9 seconds, respectively), von Willebrand factor activity was observed with low ristocetin cofactor at 33.6UI/dL, high von Willebrand factor antigen >200UI/dL, and a low von Willebrand factor: ristocetin cofactor to von Willebrand factor antigen ratio. He was admitted to the emergency room with a 24-hour evolution of progressive dyspnea, cough, thoracic pain, and palpitations, 104 beats/min, 60/40 mmHg, temperature of 38°C, pulse oximetric saturation 88% and 30 breaths/minute. Cold, pale and mottled skin was also observed. He was then transferred to the intensive care unit. The placement of a pulmonary artery catheter was made. The initial patterns showed a precapillary pulmonary hypertension; acute pulmonary embolism was the first choice for diagnosis. Pulmonary angiography was conducted, and when no clot was discovered, pulmonary artery hypertension associated with connective tissue disease was considered. Serum protein electrophoresis confirmed the presence of a massive polyclonal hypergammaglobulinemia, and no paraproteinemia or monoclonal cell population was found from the electrophoretic pattern of the patient's plasma. Hypergammaglobulinemia was the cause of hyperviscosity syndrome associated with autoantibodies. Three sessions of plasma exchange therapy were made, and clinical improvement was observed. He was then discharged from the intensive care unit and hospital, respectively. He is now attended by an external consult and has no respiratory symptomatology.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_719_en.txt", "fulltext": "A 49-year-old man presented with exertional dyspnea. Physical examination revealed a loud S1 and a diastolic murmur in the apical region. Transthoracic echocardiography revealed severe rheumatic MS with mild MR, as well as mild functional tricuspid regurgitation and a pulmonary pressure of 55 mmHg. Then, the patient underwent a three-dimensional transesophageal echocardiography to assess the mitral apparatus more accurately and exclude the presence of left atrial appendage thrombus . His Wilkins score was 8. This is a borderline score to take BMV. However, the patient prefers to take the percutaneous intervention rather than thoracotomy. Thus, BMV was performed via the antegrade approach under local anesthesia and X-ray fluoroscopy guidance. After transseptal puncture, a 24-mm balloon was used to achieve a single dilatation of 22 mm, after which the mean transmitral pressure gradient decreased from 19 to 11 mmHg. Intraoperative transthoracic echocardiography performed immediately afterwards revealed a significant increase in mitral regurgitation. However, the subvalvular structure could not be observed clearly because the patient was in a supine position. His vital signs remained stable with no definite symptoms, and he refused mitral valve replacement. He was discharged 4 days later after undergoing BMV. Two-dimensional transthoracic echocardiography follow-up performed one month later revealed severe MR caused by a freely mobile part of the mitral papillary muscle . The left ventricular cavity and the two groups of papillary muscle were then imaged using three-dimensional transthoracic echocardiography, which showed avulsion of the posterior papillary muscle (PMA) from the left ventricular trabecular muscle. No residual papillary muscle attachment to the left ventricular wall was observed, confirming avulsion of the posterior papillary rather than its rupture . In addition, the left ventricular end-diastolic dimension increased from 45 to 53 mm. The estimated systolic pulmonary pressure increased from 55 mmHg to 81 mmHg. Therefore, the patient underwent mechanical mitral valve replacement and tricuspid annuloplasty. After surgery, the patient recovered without symptoms. At 1-month and three-month follow ups, the patient was asymptomatic, and transthoracic echocardiography showed that the left ventricular volume and pulmonary systolic pressure were significantly lower than before surgery.", "summary": "In this case report, we describe a patient with rheumatic mitral stenosis, who experienced avulsion of the mitral papillary muscle from the left ventricular wall after undergoing balloon mitral valvotomy. Papillary muscle alvusion resulted in severe mitral regurgitation, which was finally treated by mitral valve replacement.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1784_en.txt", "fulltext": "We report the case of a 48-year-old female who checked into the emergency room (ER) having experienced pain in her chest and left shoulder since the previous evening. The patient was of normal weight and reported a positive family history for coronary artery disease, but denied other cardiovascular risk factors such as hypercholesterinaemia, hypertension, diabetes, or nicotine consumption. The patient had no past medical history and was taking no medication. At presentation, her vital signs were stable. The physical and cardiovascular examination revealed no abnormalities. The chest pain receded after one gram of paracetamol.\nA 12-lead electrocardiogram (ECG) was performed in the ER at presentation, showing inverted T-waves in II, III, aVF, V4, V5, and V6 . Bedside echocardiography revealed hypokinesis in the apical inferior segment of the left ventricle. The laboratory results showed significantly increased values for high-sensitivity troponin I (upward of 25 000 pg/mL, reference range: 0.0–51.4 pg/mL) and creatine kinase (CK) (540 U/L, reference range: 0–145 U/L). Therefore, a non-ST-elevation myocardial infarction was suspected, and the local interventional cardiology centre was contacted to perform coronary angiography (CAG).\nThe patient was swabbed before transfer to exclude a SARS-CoV-2 infection, as per the Medical University of Innsbruck (MUI) guidelines during the SARS-CoV-2 pandemic; surprisingly she tested positive for SARS-CoV-2 on the real-time reverse transcriptase-polymerase chain reaction assay. After further questioning, she reported having experienced isolated throat discomfort since the beginning of February, but denied contact with individuals with confirmed infections or flu-like symptoms; she also denied fever, cough, malaise, dyspnoea, or anosmia.\nAfter further discussion with the cardiology department, the ER team revised the working diagnosis to viral myocarditis and referred the patient to our infectious diseases ward, where she was promptly isolated and received cardiac and haemodynamic monitoring. We started therapy with acetylsalicylic acid, prophylactic-dose low-molecular-weight heparin, a statin, and a proton-pump inhibitor.\nA computed tomography coronary angiography (CTCA) was performed to exclude a coronary origin for the complaints and for the laboratory and ECG abnormalities, which revealed no significant coronary obstruction (Coronary Artery Disease Reporting and Data System: 0; Coronary Artery Calcium – Data and Reporting System: 0) .\nTo further confirm the diagnosis of viral myocarditis, a cardiac magnetic resonance imaging (CMR) was arranged. Surprisingly, CMR showed features of myocardial oedema restricted to the mid-ventricular to apical territory of the right coronary artery (RCA), as evidenced on T2 short tau inversion recovery sequence (signal intensity ratio of myocardium over skeletal muscle 2.3, ) and on T1 maps (increased native T1 of 1313 ms at inferior wall; reference value 980 ms, ). Based on subendocardial to partially transmural late gadolinium enhancement in the mid-ventricular to apical inferior wall, an acute myocardial infarction was diagnosed .\nMeanwhile, troponin T and CK values decreased , and episodes of mild chest pain were sufficiently controlled with paracetamol.\nAfter further communication with our interventional cardiology department the diagnosis was revised to a posterior MINOCA; the cardiologists advised against a CAG, due to the absence of coronary obstruction on the CTCA scan.\nFurther diagnostics with cardiac positron emission tomography–computed tomography showed evidence of reduced metabolic activity in the area affected by the infarction .\nWe prescribed dual anti-platelet therapy and an angiotensin-converting-enzyme inhibitor. A beta-blocker was not started due to a resting heart rate of 60 beats per minute. After two negative SARS-CoV-2 swab results in over 24 h and absence of COVID-19 typical symptoms, the patient was no longer considered infectious and could be assigned to a cardiac rehabilitation program. Follow-up echocardiography 2 days after discharge revealed a normal ejection fraction (58%) despite persistent inferior apical akinesia.", "summary": "A 48-year-old female, presenting with chest pain radiating to her left shoulder with no cardiovascular risk factors other than genetic predisposition, was screened for Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) and tested positive. Although computed tomography angiography excluded obstructive coronary heart disease, cardiac magnetic resonance imaging showed an acute myocardial infarction with no obstructive coronary arteries of the inferior wall. The patient was treated with dual anti-platelet therapy, an angiotensin-converting-enzyme inhibitor and a statin, and assigned to a cardiac rehabilitation program.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_3270_en.txt", "fulltext": "A Japanese 11-month-old baby boy presented at our institution with symptoms including fever, weight loss, and gallop rhythm. His fever persisted for 3 days before presentation but no treatment was provided. He was born by vaginal delivery at 37 weeks of gestation with a weight of 2612 g (36th percentile) and a head circumference of 33.7 cm. There was no family history of aortic disease and sudden death. When he was 7-months old, he had a fever of unknown origin that persisted for 2 weeks. He was poor in weight gain and was 7.55 kg (6th percentile) at the age of 10 months; his body weight decreased by 0.57 kg in the 3 weeks before presentation.\n\nOn examination at the presentation, his height was 70.8 cm, his weight was 6.98 kg, and his head circumference was 43 cm. He was ill-appearing and febrile to 38.4 °C. His blood pressure was 124/62 mmHg and pulse 146/minute. There was a notable S3 gallop and systolic murmur at the apex (Levine scale grade III/VI); however, there were no signs of rales or peripheral edema. Other physical and neurological examinations were normal. A chest X-ray revealed cardiomegaly, with 58% cardiothoracic rate. Echocardiography indicated left ventricular (LV) enlargement and dysfunction with LV diastolic dimension of 32 mm (130% of normal), LV ejection fraction 48%, moderate mitral regurgitation, and slight aortic regurgitation. Blood tests indicated the following: white blood cell count, 11.07 × 103/μl; hemoglobin, 10.6 g/dl; C-reactive protein, 5.59 mg/dl; creatine phosphokinase, 294 U/l; creatine phosphokinase-MB isozyme, 27 U/l; fibrin degradation products D-dimer, 2.1 μg/ml; brain natriuretic peptide, 2841 pg/ml; human atrial natriuretic peptide, 1360 pg/ml; and serum troponin T, 0.26 ng/ml. His blood culture at admission was negative.\n\nAs dilated cardiomyopathy was diagnosed, we initiated diuretics. However, his fever and high blood pressure (systolic blood pressure, 130–140 mmHg), which rarely present with dilated cardiomyopathy, persisted. To investigate the causes of high blood pressure, computed tomography was performed and revealed thoracic and abdominal aortic aneurysms on hospital day 3. It also revealed severe celiac artery stenosis and bilateral renal artery stenosis. From these findings, TA was diagnosed and 1 mg/kg per day prednisolone was consequently initiated as first-line therapy. His fever had resolved on day 5; his C-reactive protein levels returned to normal on day 10.\n\nAlthough his general condition improved, deterioration of vascular lesions was evident, as shown by echocardiography, on day 15. Celiac artery occlusion, severe right renal artery stenosis, and new superior mesenteric artery stenosis were also observed on day 15. We increased the dose of prednisolone to 2 mg/kg per day for 1 week due to the possibility of active inflammation around vascular lesions. In addition, we attempted continuous intravenous infusion of lipo-PGE1 at 10 ng/kg per minute to suppress the progression of angiostenosis. We performed vascular echocardiography twice a week and confirmed that there was no progression of angiostenosis following initiation of lipo-PGE1. We terminated infusion of lipo-PGE1 on day 36 and performed cardiac catheterization on day 45. His right renal artery was not visualized by angiography, and his right kidney was fed by collateral arteries, while vascular echocardiography revealed patency of his right renal artery with severe stenosis. We also confirmed celiac artery occlusion and superior mesenteric artery stenosis to be the same as those observed in previous echocardiography findings. His cardiac function gradually improved. After his C-reactive protein levels returned to normal, 1 mg/kg per day of prednisolone was administered for 1 month, after which the dose was tapered every other 2 weeks. We observed him for 13 months after the termination of lipo-PGE1. The inflammatory findings remained negative and the diameters of abdominal aortic vessels were stable.", "summary": "A Japanese 11-month-old baby boy presented with Takayasu’s arteritis and heart failure, possibly due to afterload mismatch caused by high blood pressure. Computed tomography was performed and revealed thoracic and abdominal aortic aneurysms. It also revealed severe celiac artery stenosis and bilateral renal artery stenosis. Prednisolone was initiated as first-line therapy. The fever resolved, and C-reactive protein levels returned to normal. Although his general condition improved, deterioration of vascular lesions was evident. Celiac artery occlusion, severe right renal artery stenosis, and new superior mesenteric artery stenosis were observed. We decided to use a continuous infusion of lipo-prostaglandin E1 for prevention of branch stenosis of his abdominal aorta. The progression of vascular stenosis was stopped and our patient’s cardiac function gradually improved.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_777_en.txt", "fulltext": "A 58-year-old man underwent laparoscopic radical resection of sigmoid carcinoma and ileostomy in the local hospital in June 2017. After the operation, he underwent intermittent chemotherapy six times. His physical condition recovered, and the re-examination showed no tumor recurrence. The patient was expected to undergo ileostomy takedown in the local hospital in June 2018 and April 2019, respectively, but the operation was not performed because of the COA, which was found by colonoscopy. The patient was admitted to the Department of Gastroenterology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei Province, China in May 2019. To address the difficult and complicated post-surgery problems of this cancer patient, the medical team conducted a comprehensive and careful related examination and disease evaluation. The abdominal enhanced computed tomography (CT) showed postoperative changes of sigmoid carcinoma. The intestinal lumen above the anastomotic stoma was narrow and adhered to the anterior sacral fascia. The stoma was unobstructed after the right lower abdominal fistulation . Further barium enema examination suggested that the anastomotic stoma in rectum is completely closed and separated . Blind ends were present on both sides of the sigmoid colon and the rectum, and the bilateral blind ends were approximately 3.0 cm apart. The laboratory examination of the patients showed that there was no obvious abnormality in the general hemogram and no significant change in the level of tumor markers. Physical examination showed no obvious discomfort.\nDespite the presence of COA with anastomotic separation, the examination results indicated that the blind end of the anastomotic site grew well. No obvious defect was found in the surrounding tissue. At the same time, the additional surgical operation would bring more trauma. Therefore, after speaking with the patient and his family, we planned to perform endoscopic operation on the patient to treat his disease. If endoscopic surgery cannot solve the problem, then surgery can be performed immediately or postponed as an alternative.\nAfter the improvement of the preoperative examination, along with adequate preoperative communication and informed consent of patients, endoscopic surgery was proposed. In endoscopic surgery , we first observed the two blind ends of the anastomotic stoma through colonoscopy. On the one hand, the COA can be seen at about 8 cm through the anus side of colonoscopy. The closure line showed a fine line scar, and the intestinal wall at the blind end was soft. On the other hand, the colonoscope entered through the ileostomy side to reach the rectal segment. The front of the blind end of the bowel was found to also be truncated and closed with a white linear scar, and a titanium clip was placed at the proximal blind end. The intestinal wall of this blind end was also soft when touched. Second, the distance between the blind ends of both sides of the intestine was 3–4 cm, as measured by fluoroscopy at different positions. Subsequently, the colonoscope re-entered through the anal side. The hook knife (KDL-620LR; Olympus Optical) was used to slowly cut through the scar tissue layer by layer in the center of the blind end of the intestinal canal, and the yellow adipose tissue became visible. The direction of incision was determined to be parallel to the direction of the titanium clip at the proximal blind end, as shown by repeated fluoroscopic observation. The adipose tissue was carefully cut open, and the proximal intestinal muscular layer was still not visible after 4 cm of advance. Third, EUS-FNA (19 Ga, 1.10 mm; Boston Scientific) puncture needle was used to puncture along the direction of the titanium clip under fluoroscopy, and a small amount of contrast agent was injected to observe the flow direction and the visualization of the intestinal canal. The intestine was visualized after the needle touched the titanium clip, and a yellow zebra guide wire was placed after the needle entered the proximal intestine. Finally, colonoscopy was performed through one side of ileostomy to confirm that the guide wire was located in the proximal intestine tube. The dilation tube and balloon (M00558430; Boston Scientific) were inserted over the guide wire, and the incised bowel was gradually dilated to 12 mm under fluoroscopy. Subsequently, a 60 mm * 20 mm self-expandable full-coated metal stent (Cook Medical, Bloomington, IN, USA) was placed over the guide wire to bridge both sides of the intestine, thereby achieving the repair of anastomotic closure with separation through the endoscopic operation. After completion of the operation, the patency of the stent was checked, no bleeding was found on the wound surface, and the anal canal was retained. Compared with the “drastic” surgical operation, this endoscopic surgery solved the intractable problem of COA with anastomotic separation with a more delicate and subtle operation and with minimal trauma. Endoscopic stent removal was performed on the patient 10 days later. The continuity of the bowel was observed intraoperatively, and an anal tube was placed in the proximal part of the anastomosis to observe the postoperative recovery.\nOne month later, stenosis was observed at the original anastomotic recanalization site during endoscopic examination, and scar-like hyperplasia was observed in the surrounding tissues. We successfully leaped over the narrow segment through inserting a new self-expandable full-coated metal stent of the same specification, and the stent expanded smoothly . After 1 week, the patient underwent stent removal and came to our hospital for follow-up and reexamination for several times after discharge. The anastomotic stenosis of the patient was gradually relieved. At 1 year after the endoscopic recanalization procedure for COA with anastomotic separation, the patient came to our hospital in June 2020 for ileostomy takedown. No related complications occurred during and after the operation. No obvious abnormality was found in the relevant post-operation laboratory and imaging examinations.\nThe follow-up situation at 3 months after discharge was as follows. The patient returned to a normal diet from a fluid diet and achieved normal exhaust and defecation. The results of colonoscopy showed that the anastomotic stoma healed well, and no obvious stenosis was found. At the same time, biopsy and pathological examination were carried out on the new tissue of the original anastomotic closure with anastomotic separation. The pathological results indicated that this site was normal intestinal mucosal tissue . Further endoscopic ultrasonography also confirmed the growth of normal intestinal mucosal tissues at the original anastomotic recanalization site .", "summary": "In this article, we present a case of endoscopic treatment of anastomotic closure and separation after radical resection for sigmoid carcinoma. After imaging examination and endoscopic evaluation, we found that the patient had a COA accompanied by a 3-4 cm anastomotic separation. With the aid of fluoroscopy, we attempted to use the titanium clip marker as a guide to perform an endoscopic incision and successfully achieved recanalization. We used a self-expanding covered metal stent to bridge the intestinal canal to resolve the anastomotic separation. Finally, the patient underwent ileostomy takedown, and the postoperative recovery was smooth. The follow-up evaluation results showed that the anastomotic stoma was unobstructed.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1363_en.txt", "fulltext": "A 77-year-old African American woman with a medical history of hypertension presented with a systolic blood pressure of 200 mmHg. There was no significant family or social history. A renal ultrasound showed a 12 × 9 × 7.5 cm mass medial to the left kidney. A follow-up computed tomography (CT) scan showed an enhancing 9 × 6 cm mass anterior and medial to the left kidney. Laboratory studies revealed a normal dexamethasone suppression test and an elevated androstenedione level of 182 ng/dl. Given these results, an androgen-producing adrenal tumor was suspected. The differential diagnosis also included pheochromocytoma, lymphoma, and mesenteric gastrointestinal stromal tumor. The patient underwent robotic-assisted left adrenalectomy. The intraoperative finding of “focal invasion” into the renal parenchyma raised the possibility of adrenal cortical carcinoma; therefore, an additional left upper pole partial nephrectomy was performed. The specimen received was an 11 × 7.2 × 6.8 cm adrenal mass with attached portion of kidney. The mass was golden yellow, well circumscribed, and grossly adherent to the kidney. Histologic evaluation revealed an adrenal cortical adenoma without any features of malignancy, with a Weiss score of 0 . The adjacent adrenal parenchyma shared an incomplete capsule with the kidney and was in direct contact with the renal cortex, establishing the diagnosis of fusion between the two organs . The postoperative course was uneventful.", "summary": "Our patient is a 77-year-old African American female who presented with a systolic blood pressure of 200 mmHg. Computed tomography showed an enhancing 9 × 6 cm mass anterior and medial to the left kidney. The patient underwent a left adrenalectomy with partial nephrectomy. Gross and histologic examinations revealed an adrenal cortical adenoma and renal-adrenal fusion.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1999_en.txt", "fulltext": "A 15-year-old male presented with the sudden onset of a hemiparesis (i.e., left upper limb 4/5 and left lower limb 4/5). The laboratory work up was negative for any coagulation abnormalities; normal international normalized ratio, prothrombin time, activated partial thrombin time, and bleeding/clotting times. The brain MRI was normal, thus largely ruling out a stroke. However, the cervical MR showed a dorsolateral left-sided epidural hematoma extending from C7 to D1 causing severe cord compression. The lesion was hyperintense on T1, hyperintense with a hypointense band on T2 studies, but did not enhance with contrast [- and ]. These findings were diagnostic for a SSEH. The patient underwent an emergent C7-D1 laminectomy for excision of the SSEH. Postoperatively, the patient regained normal function.", "summary": "A 15-year-old male presented with the sudden onset of a hemiparesis which recovered (4/5 weakness). The brain MR was negative, but spinal MRI revealed a dorsolateral extradural lesion extending from C7 to D1. At surgery, this proved to be a hematoma that we readily removed.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1650_en.txt", "fulltext": "A 37-year-old woman presented at the emergency department (ED) with exertional dyspnoea New York Class Association (NYHA) functional classes II and III, syncope, and new onset of T-wave inversions in the ECG . She presented in a clinically stable condition with a blood pressure of 130/80 mmHg, a pulse of 90/min, and an oxygen saturation of 96%. The clinical examination was unremarkable. Her only significant medical history was Crohn’s disease, which had been well controlled with adalimumab for 3 years. Computed tomography (CT) ruled out a pulmonary embolism and parenchymal lung disease . The transthoracic echocardiographic (TTE) findings were consistent with a cor pulmonale without signs of an intracardiac shunt or a left ventricular (LV) pathology (see , ). Right heart catheterization proved a precapillary PH with a negative vasoreactivity testing (mPAP 33 mmHg, pulmonary vascular resistance 4.8 WU, pulmonary arterial wedge pressure 7 mmHg, and central venous pressure 4 mmHg). Perfusion–ventilation scintigraphy displays small peripheral perfusion defects .\nThe presence of peripheral perfusion defects in scintigraphy was initially interpreted as possible chronic thromboembolic PH, and anticoagulation with heparin was started. However, the findings were later interpreted as in context of PH Group 1. Therefore, a PH specific therapy with macitentan and tadalafil was initiated under the continuation of heparin. The short-term course was then complicated by a heparin-induced thrombocytopenia (HIT) that was confirmed by a positive antibody test (HIT antibodies: IgG 6.96 U/mL) with otherwise normal coagulation parameters. Shortly thereafter, an episode of haematemesis occurred, and gastroscopy revealed a gastric adenocarcinoma. An escape chemotherapy regimen was launched before completion of tumour staging. Staging CT revealed subpleural nodular ground glass opacities with ‘feeding vessel sign’ compatible with PH but no metastatic spread of gastric cancer . Consequently, oncologic treatment was changed to a neoadjuvant chemotherapy and subsequent gastrectomy. The patient’s symptoms improved shortly after the first administration of chemotherapy and resolved completely within 2 months. Retrospectively, we considered a pulmonary tumour thrombotic microangiopathy (PTTM) the most probable cause of PH. Follow-up after successful oncologic treatment and cumulative 10 months of specific PH medication showed an excellent clinical response; hence, specific PH therapy was ceased (see , ; ). A subsequently performed RHC confirmed complete remission of PH at rest (mPAP 17 mmHg, pulmonary vascular resistance 1.6 WU, pulmonary arterial wedge pressure 9 mmHg, and central venous pressure 5 mmHg).", "summary": "A 37-year-old woman presented with exertional dyspnoea and syncope. The transthoracic echocardiographic findings were consistent with a cor pulmonale. Right heart catheterization (RHC) proved a precapillary PH. Specific PH therapy with macitentan and tadalafil was initiated. Shortly thereafter, a gastric carcinoma was diagnosed, and oncologic treatment with neoadjuvant chemotherapy and subsequent gastrectomy was promptly initiated. Retrospectively, we considered a pulmonary tumour thrombotic microangiopathy the most probable cause of PH. Follow-up after successful oncologic treatment and cumulative 10 months of specific PH medication showed an excellent clinical response with complete remission of PH confirmed by RHC at rest.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_374_en.txt", "fulltext": "A 71-year-old man who suffered from CLL was admitted to our hospital on June 23th, 2020 due to fever. In 2001, he was admitted to a hospital due to submaxillary mass. On physical examination, multiple enlarged lymph nodes of the bilateral jaw bone were touched with nontender and of medium in quality, among which the largest lymph node was about 1.0×1.5 cm2. Routine blood results showed white blood cell (WBC) of 148.52×109/L, lymphocyte ratio of 81.5%, hemoglobin of 101 g/L and platelet of 68×109/L. Bone marrow smear revealed active hyperplasia of nucleated cells in bone marrow and increased proportion of lymphocytes, including mature lymphocytes (91%) and immature lymphocytes (2.5%). Likewise, peripheral blood films also suggested significantly augmented proportion of lymphocytes, with 91% of mature lymphocytes and 3% of immature lymphocytes, which was consistent with the bone marrow smear of CLL. Subsequently, the patient was subjected to bone marrow biopsy. Results indicated active bone marrow hyperplasia and nodular or patchy proliferation of mature lymphocytes, without granulocyte, erythrocyte and megakaryocyte, as well as germinal center and macrocell transformation. Flow cytometric immunophenotyping showed CD5(+)81%, CD19(+)70%, CD20(+)25% and CD22(+)8%. Finally, the patient was diagnosed with CLL at Binet C stage and was given 4 courses of combination therapy of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone (R-CHOP). After discharge, he received daily oral chlorambucil 2 mg for a long time, and the disease was controlled.\nOn May 20th, 2020, the patient revisited the hospital. The bone marrow smear still conformed to CLL, and the bone marrow biopsy indicated that tumor cells were scattered or clustered (10–20%). Flow cytometric immunophenotyping performed on the peripheral blood showed that abnormal cell population accounted for 87.16% of nuclear cells, expressing CD19, CD23, CD5, CD200 and CD22, partially expressed CD20, CD123 and CD11c, and weakly expressing CD81 and CD43, suggesting CLL/small lymphocytic lymphoma (CLL/SLL) phenotype. The karyotype of the chromosome was 45,X,-Y[4]/47,XY,+12[6]/46,XY[15]. Fluorescence in situ hybridization (FISH) showed negative IGH/BCL2, ATM/CEP11, RB-1, CCND1/IGH, CEP12 and P53/CE P17. The second-generation sequencing showed that Y220C and I195F mutations in exon 6 of TP53 mutation were positive, with a mutation rate of 4.1% and 1.5%, respectively. Somatically mutated monoclonal IGHV4-34 gene rearrangement was demonstrated, with 6.7% of mutation rate. Taken together, these findings indicated that this patient was diagnosed with RaiIII-phase CLL at Binet stage C, with IgHV and TP53 mutation. Oral ibrutinib tablet 420 mg was given once a day. No increase in hemoglobin was observed in blood routine monitoring during the oral administration of ibrutinib, and intermittent transfusion for the treatment was conducted in our hospital.\nOn June 23th, 2020, the patient was referred to our hospital when he presented with fever, fatigue and anorexia. On admission, the physical examination revealed anemia, a body temperature of 37.9°C, pulse rate of 102/min, breathing rate of 20 times/min and blood pressure of 120/67 mmHg, without obvious abnormalities in the heart and lungs. There were enlarged lymph nodes in the bilateral neck and groin, with the most being 2×3 cm2, as well as splenomegaly (line A and B of 5 cm, line A and C of 6 cm, line D and V of −6 cm) with small-medium in size, nontender and pitting edema of lower limbs. Routine blood results showed WBC of 59.16×109/L, lymphocyte count of 121.04×109/L, hemoglobin of 89 g/L and neutrophile granulocyte of 17.78×109/L. Bone marrow smear indicated active bone marrow hyperplasia, and we observed 46.5% of protogranulocytes (small and unequal in size and some cytoplasm containing a few azurophilic granules), many basophilic erythroblast, megakaryocytes and small megakaryocytes, but little immature lymphocytes . The positive rate of POX staining was about 65%, indicating CLL accompanied by AML-M2 . As shown in , abnormal myeloid primordial cells were found in the specimens, accounting for 32.86% of the nuclear cells, which was in line with AML phenotype. In addition to expressing myeloid antigens, CD56-positive lymphocytes were also noticed, which could be used as a monitoring indicator for minor residual disease. A cluster of abnormal B lymphocytes with small forward scatter (FSC) intensity and side scatter (SSC) intensity was observed in the specimen of the patients, and the phenotype of the cluster of cells was consistent with CLL/SLL. Moreover, the patient’s karyotype was 44, X,-Y,del(5)(q31),-7add(12)(p13)der(17)der(18), 22,+mar([8]/44,idem,del(4)(q22)[12] .\nOn the basis of the data above, a final diagnosis of AML-M2 secondary to CLL was made, with -Y,del(4q),del(5q),-7,add(12p),der(17),der(18),-22,+mar. Azacitidine combined with B-cell lymphoma 2 (Bcl-2) inhibitor was recommended for the treatment, which was rejected by his family, and the patient died of pulmonary infection on October 17, 2020. This study followed the principles of the Declaration of Helsinki, and informed consent was obtained from the patient’s family.", "summary": "We reported a case of a 71-year-old man with a history of CLL. The patient was administrated with chlorambucil for 19 years and was admitted to our hospital due to fever. Then he was subjected with routine blood tests, bone marrow smear examination, flow cytometric immunophenotyping and cytogenetic analysis. A final diagnosis of AML-M2 secondary to CLL with -Y,del(4q),del(5q),-7,add(12p),der(17),der(18),-22,+mar was made. After rejecting the therapy with Azacitidine combined with B-cell lymphoma-2 (Bcl-2) inhibitor, the patient died of pulmonary infection.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1759_en.txt", "fulltext": "A 57-year-old male presented with 3 months of left pulsatile tinnitus and hemicranial headache without any other neurological deficits. He had no history of head trauma or central nervous system infections. Digital subtraction angiography revealed DAVF with afferents from the left external carotid artery (LECA), notably with the occipital and middle meningeal arteries draining into the ipsilateral sigmoid sinus, without leptomeningeal cortical retrograde venous drainage, and grade I of the Cognard classification .\nThe transvenous approach was chosen, together with closure of the affected sinus using coils, but the surgical team experienced difficulties when passing the microcatheter from the transverse to the sigmoid sinus, which was likely isolated. Thus, two sessions of transarterial embolization were performed 2 months apart . During the first session, the fistulous branch of the left occipital artery was occluded with Onyx® [Figure and ]. The patient recovered uneventfully and tinnitus decreased, although headaches persisted. During the second session conducted with Onyx®, the feeder branch of the left middle meningeal artery was occluded [Figure and ]. Angiography revealed complete exclusion of the fistula and successful embolization . On the first day postoperatively, the patient presented with ipsilateral peripheral facial palsy, House Brackmann grade 4 , and no other physical or neurological signs. Tomography showed no ischemic or hemorrhagic complications . The patient received 4 mg of intravenous dexamethasone every 6 h and showed progressive improvement with complete recovery in 4 days . After 4 months, the patient remains under our care, with no complaints of headache or tinnitus. A follow-up appointment is scheduled for 6 months after his last session.", "summary": "We present a male patient who complained of headaches and left-sided pulsatile tinnitus due to DAVF from the external carotid artery branches draining directly into the ipsilateral sigmoid sinus. Embolization with Onyx® was successful, obtaining angiographic occlusion and symptom remission. However, on postoperative day 4, the patient presented with left facial palsy and spontaneous regression.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1712_en.txt", "fulltext": "An 81-year-old woman, who had a history of resection of a benign eccrine spiradenoma from the parietal scalp 12 years earlier, presented with a large swelling on the same region that had been growing over the preceding 5 years without any neurological deficit. Radiological examinations demonstrated that the lesion was a single, large cystic mass invading bilateral parietal bones and attached to but not occluding the superior sagittal sinus (SSS) . Whole-body positron emission tomography revealed no lymph node or distant metastases.\nThe lesion was excised along with surrounding scalp and bone, and detached from the SSS. The dura mater covering the SSS was electrocoagulated. Reconstruction of the removed skull and scalp was then performed using titanium mesh and a pedicled skin flap from the occipital region which was replaced by a skin graft from the femoral area . Histopathological examination revealed the features of low-grade eccrine SC. Two distinct cell types were observed, comprising eosinophilic cuticular cells surrounded by poroid cells with a high nuclear-cytoplasmic ratio forming palisading or solid nests, accompanied by glandular lumens and ducts. Overall cytoplasmic atypia, some mitotic figures and partial loss of the two-cell structures were also observed . Cytokeratin 7 was broadly expressed, and Ki-67 labeling index was approximately 30% . Close follow-up was continued considering the risk of recurrence.\nThirty-three months after the first surgery, solid tumor was seen to have recurred in the SSS and a second resection was performed . The tumor was totally excised, again preserving the SSS, adjacent dura and pedicled scalp flap . Histopathological diagnosis was similar to the previous lesion, other than the loss of the large cyst formation. No adjuvant therapy was administered, in consideration of the lack of evidence of efficacy, the age of the patient, and a recent history of ischemic brainstem infarction prior to the second surgery.\nTwelve months after the second surgery, a heterogeneous contrast-enhancing mass was observed in the epidural region with extracranial extension pushing against the titanium mesh. The SSS was compressed but not occluded, and the scalp skin was about to rupture . No metastatic lesion was evident on whole-body examination.\nThe third operation was performed with resection of the skin flap, titanium mesh, and surrounding bone edge. The tumor invading the SSS could not be resected due to marked bleeding. An artificial bone flap made from polyethylene was used for the cranioplasty instead of metallic material, which would reduce the scattered radiation dose to the surrounding tissue. Scalp reconstruction was performed with a new pedicled skin flap from the left temporal region . The histopathological diagnosis was compatible with the first lesion. Two months after the third surgery, the patient received 6 MV X-ray EBRT using simultaneous integrated boost (SIB) volumetric-modulated arc therapy (VMAT) with the prescribed dose of 50 Gy in 25 fractions to the planning target volume margin (residual gross tumor invading the SSS + 5 mm, tumor cavity + 3 mm) as well as SIB with 57.5 Gy to the residual gross tumor margin. The median dose to the gross tumor volume was 60.4 Gy. The residual tumor rapidly shrank and had vanished by 3 months after completion of the EBRT .\nTwenty months after the third surgery, the patient developed left hemiparesis and a recurrent tumor was observed with intracerebral extension arising from the SSS forming a cystic lesion . The patient concurrently developed rectal carcinoma with lower gastrointestinal bleeding. Palliative endoscopic partial resection of the cystic lesion was performed, followed by stereotactic radiotherapy using CyberKnife® (Accuray Incorporated, Sunnyvale, CA) with 42.2 Gy in 10 fractions to the margin of the residual cyst wall and the tumor involving the SSS . Histopathological examination demonstrated that the features of low-grade SC had been sustained .\nThe patient was transferred to another hospital and died 7 months after the last treatment, 7 years after the first surgery, with no apparent swelling of the scalp.", "summary": "An 81-year-old woman presented with a large swelling on the parietal scalp 12 years after resection of spiradenoma from the same site. The tumor showed intracranial invasion with involvement of the superior sagittal sinus and repeated recurrences after four surgeries with preservation of the sinus. The histopathological diagnosis was eccrine SC. Adjuvant high-precision external beam radiotherapy (EBRT) proved effective after the third surgery, achieving remission of the residual tumor. The patient died 7 years after the first surgery for SC.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1523_en.txt", "fulltext": "A 20-years-old, British, male sustained cervical spinal cord injury (ASIA impairment scale B) at C-5 level in 1970 while teaching gymnastics. He was a physical training instructor in army and he landed on the wrong side of a trampoline. This gentleman had been managing his bladder by penile sheath drainage until 2013 when he underwent surgery for upper gastrointestinal bleeding. Since then, he had been draining urine by urethral Foley catheter.\nFollowing a routine change of urethral catheter by a health professional, this patient started sweating profusely. The Foley catheter was draining urine; there was no bypassing, no bleeding per urethra, and no haematuria. Patient’s wife, who had been looking after her tetraplegic husband for more than forty years, told the health professionals that the catheter might have been placed incorrectly but her concerns were ignored by health professionals. Therefore, this patient came to spinal unit for advice. On clinical examination, this patient was sweating profusely over his head and face. Blood pressure was 140/70 mm Hg. The patient did not have symptoms of urine infection nor did he feel unwell. Ultrasound scan of urinary tract revealed no urinary calculi, no hydronephrosis. The balloon of Foley catheter was not seen in urinary bladder but this finding was not recognised by radiologist and spinal cord physician. Patient continued to sweat profusely; therefore, Computed Tomography (CT) of pelvis was performed, but there was a delay of ten days. Special attention was taken to include the entire urethra in addition to urinary bladder for scanning, as misplacement of urethral catheter was suspected by the spinal cord physician. CT revealed the balloon of Foley catheter in the over-stretched prostato-membranous urethra . The tip of Foley catheter was not located within the urinary bladder but was lying distal to bladder neck . Flexible cystoscopy was performed and Foley catheter was inserted into the bladder over a guide wire. The intensity of sweating decreased; noxious stimuli arising from traumatised urethra might take a long while to settle.\n24-hours ECG monitoring revealed bradycardia: slowest being 39 beats per minute , 214 episodes, and 36 beats at 00:09:04. There was a pause of 2.08 seconds at 22:14:51 . X-ray of pelvis revealed marked osteoarthritic changes in both hips; X-ray of lumbar spine revealed slight scoliosis concave to the right; anterior hyperostosis, most marked at L4/5; disc spacing was well maintained.\nUltrasound scan revealed normal size right kidney measuring 12.5 cm with no hydronephrosis or calculi. The left kidney was atrophic measuring 8.4 cm, cortical depth of 14.1 mm. No calculus was seen. Blood tests: Urea: 5.9 mmol/L; Creatinine: 69 micromol/L; HbA1c: 36 mmol/mol; PSA: 0.15 ug/L. Urine culture yielded growth of >108/L of Staphylococcus aureus. Cytology of urine revealed abundant neutrophils and red cells. Groups of urothelial cells were seen with slightly enlarged nuclei. Some of the groups have a vaguely papillary appearance, which could be seen in low-grade urothelial neoplasms, infection, instrumentation or stones. A follow-up urine cytology revealed a few inflammatory cells and urothelial cells. No malignant cells were seen.", "summary": "A tetraplegic patient started sweating profusely following insertion of a Foley catheter per urethra. The catheter was draining urine; there was no bypassing, no bleeding per urethra, and no haematuria. Patient's wife, who had been looking after her tetraplegic husband for more than forty years, told the health professionals that the catheter might have been placed incorrectly but her concerns were ignored. Ultrasound scan of urinary tract revealed no urinary calculi, no hydronephrosis. The balloon of Foley catheter was not seen in urinary bladder but this finding was not recognised by radiologist and spinal cord physician. Patient continued to sweat profusely; therefore, CT of pelvis was performed, but there was a delay of ten days. CT revealed the balloon of Foley catheter in the over-stretched prostate-membranous urethra; the tip of catheter was not located within the urinary bladder but was lying distal to bladder neck. Flexible cystoscopy was performed and Foley catheter was inserted into the bladder over a guide wire. The intensity of sweating decreased; noxious stimuli arising from traumatised urethra might take a long while to settle.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_1756_en.txt", "fulltext": "A 76-year-old Caucasian female with known hypertension and dyslipidaemia presented with 2 weeks of intermittent palpitations, orthopnoea, and New York Heart Association functional class III–IV shortness of breath. On exam, the patient was not in acute distress; however, extremities were cool and clammy. Heart auscultation revealed normal heart sounds, no murmurs, rubs, or gallops but the rhythm was irregularly irregular at ∼100 b.p.m. The jugular venous pressure was elevated at the angle of the jaw and there was a positive abdominojugular reflux sign. Auscultation of the lungs revealed bilateral crackles up to mid chest with decreased air entry at the bases. The abdomen was soft, non-tender, and non-distended.\nIn the emergency department, she was in uncontrolled atrial fibrillation (AF) with a left bundle branch block with clinical evidence of decompensated heart failure and her chest X-ray confirming evidence of pulmonary oedema with bilateral pleural effusions. There was no prior electrocardiogram for comparison. She was admitted for intravenous diuresis [initially with Furosemide 80 mg intravenously (IV) once then Furosemide infusion 20 mg/h] and rate control (initially with metoprolol 5 mg IV once then transitioned to oral metoprolol with an intravenous digoxin load). Over the next few hours, she became increasingly diaphoretic, tachycardic, and hypotensive at 60/30 mmHg. High-sensitivity troponin increased to 2040 ng/L (reference value ≤30 ng/L) from 13 ng/L, and serum lactate was 7.1 mmol/L (reference value 0.5–2.2 mmol/L). She also developed anuric acute kidney injury with an increase in serum creatinine from 58 to 122 μmol/L (reference value 50–98 μmol/L). Other admission labs are shown in . She was started on dual antiplatelet therapy (ASA 81 mg orally daily, clopidogrel 75 mg orally daily, and fondaparinux 2.5 mg subcutaneously daily) for suspected acute coronary syndrome and vasopressors to maintain perfusion. Point-of-care ultrasound demonstrated significant biventricular systolic dysfunction, a plethoric non-collapsible inferior vena cava, and diastolic flattening of the interventricular septum suggesting volume overload of the right ventricle.\nThe following differential diagnoses were considered at that time:\nAcute coronary syndrome and possible mechanical complication with right ventricular (RV) infarction.\nUnderlying cardiomyopathy and/or pulmonary hypertension and superimposed acute decompensation.\nMassive pulmonary embolism.\nAs the patient was in extremis, she was intubated for emergent cardiac catheterization. She was initiated on epinephrine, norepinephrine, and vasopressin prior to arrival to the catheterization lab. Angiographically, the left main coronary artery (LMCA), left anterior descending (LAD), and left circumflex (LCx) arteries were very large, ectatic vessels with no flow-limiting stenosis. The right coronary artery (RCA) ostium could not be engaged from the aorta despite multiple attempts. Contrast injection into the left coronary system showed what appeared to be retrograde filling of the RCA via multiple left-to-right collateral vessels ( and Video 1A and B). On further review, it was felt that the RCA was emptying back into the pulmonary artery/right ventricular outflow tract (RVOT).\nThe possibility of an anomalous coronary artery (ACA) as the underlying aetiology of the patient’s shock prompted an ad hoc right heart catheterization for haemodynamic assessment. Measurements are shown in . An oximetry run did not suggest the presence of an intra-cardiac left-to-right shunt. Pulmonary artery angiography did not identify any pulmonary embolus or fistula involving the RVOT. The pulmonary vascular resistance was 0.87 Wood units and the systemic vascular resistance was 6.39 Wood units. An RV angiogram demonstrated significant tricuspid regurgitation (TR) with a dilated and severely dysfunctional right ventricle. It was felt that the cause of the patient’s cardiogenic shock was due to underlying severe RV dysfunction with either AF or other catecholaminergic state triggering decompensation. The elevated cardiac index prompted consideration of sepsis on the differential diagnosis and the patient accordingly received empiric antibiotics (Piperacillin/Tazobactam 4.5 g IV every 6 h, Vancomycin 1 g IV every 12 h, and Doxycycline 100 mg orally twice daily). She returned to the cardiovascular intensive care unit (CVICU) for further physiologic support.\nFormal echocardiography while in the CVICU demonstrated severely reduced RV systolic function, right atrial dilatation, and severe TR. The overall left ventricular systolic function was low-normal with an ejection fraction between 50% and 55% and no regional wall motion abnormalities. A transoesophageal echocardiogram ruled out vegetations but corroborated findings from the patient’s cardiac catheterization including a dilated and tortuous LMCA and branches, as well as a dilated and tortuous vessel draining into the pulmonary artery. This vessel was felt to be likely the RCA. Overall, these findings were consistent with a diagnosis of ARCAPA (see and Video 2).\nIn the CVICU, the patient was maintained on inotropes and vasopressors. High-sensitivity troponin plateaued at 26 040 ng/L and this was felt to be consistent with an RV infarction. In the setting of concomitant volume overload and anuric acute kidney injury, continuous renal replacement therapy was initiated. She was subsequently found to be bacteremic with positive blood cultures for Streptococcus bovis but not stable enough to pursue a colonoscopy to rule out a gastrointestinal malignancy; management consisted of antimicrobial therapy (ceftriaxone/metronidazole) with consultation from infectious diseases.\nThe patient’s clinical status eventually stabilized enough to be extubated and transferred to the cardiology ward, although she remained dependent on intermittent haemodialysis without expected renal recovery. Potential therapeutic options for ARCAPA were discussed at interdisciplinary heart team rounds but unfortunately, she was felt not to be a surgical candidate in view of her age and clinical status. The patient decided to pursue comfort measures and requested cessation of dialysis. She was transferred to a hospice for palliative care and passed away just over a month later.", "summary": "We present a case of a 76-year-old female presenting with 2 weeks of palpitations and shortness of breath who was found to be in rapid atrial fibrillation (AF) with congestive heart failure. Despite initial medical management, the patient developed cardiogenic shock with anuric renal failure. Emergent right and left heart catheterization did not demonstrate any significant obstructive coronary artery disease but showed severe right ventricular (RV) failure and raised the possibility of an ARCAPA. This diagnosis was further corroborated by findings on a subsequent transoesophageal echocardiogram. In view of profound decline and limited anticipated improvement, the patient ultimately decided to pursue comfort measures in a hospice setting.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2523_en.txt", "fulltext": "A 69-year-old female presented with chest and severe upper back pain, but neurologically intact. The first chest computed tomography (CT) was interpreted as normal . Over the next 2 months, she progressively developed anorexia and the inability to belch. Esophageal stricture and thoracic vertebra OVF were suspected based on the results of the second chest CT . Thoracic magnetic resonance (MR) and CT studies revealed a T7 OVF with a kyphotic deformity . Twice, she underwent esophagogastroscopy that showed no obstruction. As her symptoms were attributed, therefore, to the T7 OVF, she underwent a T7 BKP resulting in an improved kyphotic angle from 22.4 degrees to 17 degrees . In addition, this resulted in resolution of her symptoms.", "summary": "A 69-year-old female presented with back pain. 2 months later, she developed anorexia and difficulty belching. The thoracic magnetic resonance (MR) demonstrated a T7 OVF. As she ultimately underwent a balloon kyphoplasty (BKP), as conservative treatment was unsuccessful.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2173_en.txt", "fulltext": "A 47-year-old woman presented with complex bilateral renal stones and was referred to our hospital for surgical treatment due to failure of conventional PCNL performed at another hospital. Initially, we performed mini-ECIRS for the right renal stone, which was later identified as calcium oxalate, without any complications and residual stones.\nThree months after the first mini-ECIRS, she underwent a second mini-ECIRS for the left renal stone. Laboratory studies showed normal values of serum inflammatory markers, liver and renal function, mineral markers, and urinary parameters. Multi-slice computed tomography and kidney-ureter-bladder radiography revealed a stone (size, 6.5 × 2.2 × 2.4 cm3) with left calyceal fragment . Mini-ECIRS was performed in the prone position, and supra-12th rib access was obtained with a single pass of the 16.5/17.5-Fr metal sheath with a 12-Fr nephroscope under combined US and ureteroscopy guidance. During puncture, we identified the pleural space and pleural line under US to avoid penetration. After puncture, we checked intraoperative fluoroscopy to evaluate potential chest complications. Using the holmium YAG-laser through the rigid nephroscope, all parts of the collecting system and a part of the main stone were cleared. Finally, an ureteral stent without a nephrostomy tube was placed after the procedure. The operative time was 3 h, with no intraoperative complications and an estimated blood loss of <100 cc.\nOn POD 1, the patient developed shortness of breath. Tachypnea with normal vital signs was noted with deteriorating breath sounds on the left side. Chest radiography confirmed left pleural collection . Her hemoglobin level, which was 12.0 g/dL at discharge, had dropped to 11.0 g/dL. On consultation with the thoracic surgeon, she was conservatively managed without any treatment for several days. On POD 5, a 16-Fr tube was placed for left intercostal drainage under local anesthesia with ultrasonography guidance by the standard technique because of the aggravation of hemothorax . A small volume of old blood was drained without improved expansion of the left lower and middle lobes . On POD 9, VATD was performed for the hemothorax. During intraoperative monitoring, some abscesses were observed in the hemothorax, which appeared to be empyema; moreover, a scar hole that had been punctured through the pleural cavity during mini-ECIRS was identified . Thereafter, blood clot evacuation, decortication, and ablation were performed by VATD. At the end of the procedure, the 20-Fr chest tubes were retained . On POD 14, she was discharged after retrieval of the chest tube. Follow-up evaluation showed satisfactory expansion of the left lung with complete resolution of all chest symptoms .", "summary": "A 47-year-old woman with left staghorn kidney stone underwent mini-endoscopic combined intrarenal surgery, after which she developed shortness of breath. Chest radiography and computed tomography confirmed left hemothorax. Conservative management and left intercostal chest drainage were performed without improvement. Subsequently, video-assisted thoracoscopic debridement was performed.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_339_en.txt", "fulltext": "We present a case of 49 years old Caucasian male suffering from congenital lymphoedema of the left lower extremity who developed composite haemangioendothelioma during his adulthood. Because of the lymphoedema, the patient was first operated on at the age of 6 years, which had only a minor effect on overall condition of the patient. Later on in his life, he experienced multiple erysipelas and at the age of 47 a vascular tumour was detected in the setting of the lymphoedema as its complication. In another institution, a microscopically non-radical (R1) resection was performed and a diagnosis of angiosarcoma was concluded based on the histopathological evaluation of the tumour. The patient was not indicated for any adjuvant therapy, or re-resection. He was followed up and the first control PET/CT scan showed neither local relapse nor distant spread. Local progression was detected after one year of follow up and the patient was re-operated. Second R1 resection was performed and subsequent histopathological examination revealed an angiosarcoma again. This diagnosis was supported by an early second local relapse after a month. After that, the patient was reported to our sarcoma centre for further treatment.\nDuring the clinical investigation, two purple skin affections in the popliteal area at the edges of the dermo-epidermal mesh skin graft were found . The left lower extremity showed chronic disfiguration as a result of multiple erysipelas and chronic lymphoedema. Scattered ecchymoses were also present. In the left gluteal area, multiple small nonspecific purple skin affections were visible. For staging purposes, whole-body PET/MR was performed and revealed multiple small subcutaneous nodules located in the left gluteal area, left calf and left hamstring area . Every nodule demonstrated low metabolic activity; however, the finding raised clinical suspicion for metastases of the previously diagnosed angiosarcoma. During the multidisciplinary tumour board, a bioptic confirmation of the process within gluteal space was indicated. Under local anaesthesia excision biopsy of the skin affection in the gluteal area was performed and sent for histopathological analysis. Within dermis, there was a cavernous vascular tumour (diameter 5 mm) detected, which consisted of dilated thin-walled lymphatic vessels containing papillary protrusions at the periphery. The papillary protrusions contained hyalinised cores in the centre and were covered with columnar endothelial cells with hobnail or even matchstick-like features. There were present neither mitoses nor severe nuclear atypia. Immunohistochemistry (IHC) showed diffuse positivity of the neoplastic cells for CD31, ERG and FLI-1. Tumour showed also focal D2-40 positivity within slit-like spaces at the periphery. IHC markers CD34, HHV-8, CK-KL1 and smooth muscle actin were negative. Based on the clinical background (localization within the dermis of the gluteal area), morphology and immunophenotype of tumorous cells, the suspicion for papillary intralymphatic angioendothelioma (PILA) was raised in contrast to the previous diagnosis . The expected structures of angiosarcoma were not identified within the sample.\nTo evaluate the uncertain undergoing process, the next multidisciplinary meeting led to an indication for excision of the recurrent focus and bioptic evaluation of the nodules of the calf and gluteal area. The operation was performed in February 2022 in form of an excision of two skin affections of the popliteal area (diameter 43 and 78 mm) and another biopsy of the tumour in the calf and gluteal area (diameter 20 mm and 10 mm). Histopathological examinations showed a new finding – the dermal-based vascular tumour with the retiform arrangement was detected, which consisted of branching vascular channels lined with hobnail-shaped bland endothelial cells. At some portions, papillary protrusions with hyalinised cores were found. There were neither mitoses nor nuclear atypia again. On the other hand, no association with the lymphatic vessels was found this time. IHC showed diffuse CD31 positivity and CD34 negativity of the neoplastic cells. Proliferation marker Ki-67 was low with positivity in approximately 10% of the cells within hot spots. Even though the histopathological finding was similar to the previous sample, due to the multifocality of the process, arborizing retiform architecture of the tumour and lacking association with lymphatic vessels in the current biopsy, the diagnosis of retiform haemangioendothelioma was made.\nBecause of the absence of angiosarcomatous structures, the original bioptic material was obtained from the first resection for the second opinion. But, the second look examination confirmed the former diagnosis of HG angiosarcoma: at the border between dermis and sub-cutis there was an infiltration of solid tumour consisting of elongated oval to spindle shaped cells showing marked nuclear atypia and numerous mitoses, including atypical forms. Erythrocytes filled slit-like spaced dissecting the solid areas were detected. IHC showed a similar immune profile of the neoplastic cells (CD31 positive; CD34 and D2-40 negative) and significantly higher proliferation (40–50%) based on the Ki-67 proliferation marker. Such a finding was consistent with the original diagnosis of HG angiosarcoma . However, at the base of the sample, small areas with a morphology of retiform haemangoendothelioma similar to the previous finding were spotted.\nBased on the histopathological findings of three different vascular components resembling PILA, retiform haemangioendothelioma and HG angiosarcoma, the final diagnosis of CHE with HG angiosarcoma-like areas was established.\nRecently it has been discovered that CHE can harbour a fusion gene YAP1::MAML2, especially among children with acral localisation of the tumour . We performed next-generation sequencing (NGS) to investigate this gene fusion and to exclude other entities which could mimic such lesion. Using Fusion Plex Sarcoma V2 panel (Archer) the fusions of the following genes were excluded: ALK, BCOR, BRAF, CAMTA1, CCNB3, CIC, CSF1, EGFR, EPC1, ERG, ESR1, ETV1, ETV4, ETV5, ETV6, EWSR1, FGFR1, FGFR2, FGFR3, FOS, FOSB, FOXO1, FUS, GLI1, HMGA2, JAZF1, MBTD1, MDM2, MEAF6, MET, MGEA5, MKL2, NCOA1, NCOA2, NCOA3, NR4A3, NTRK1, NTRK2, NTRK3, NUTM1, PAX3, PDGFB, PDGFRA, PHF1, PLAG1, PRKCA, PRKCD, RAF1, RET, ROS1, SS18, STAT6, TAF15, TCF12, TFE3, TFG, USP6, VGLL2, YAP1, YWHAE).\nIn conclusion no gene fusion was identified, including YAP1::MAML2. However this finding does not exclude the diagnosis of CHE as for such gene fusion usually does not occur among adult patients outside acral localisations . Moreover, we also examined MYC amplification using fluorescent in situ hybridisation (FISH) as there is an evidence in literature that high-level MYC gene amplifications (at 8q24.21) occur in majority of post-irradiation and chronic lymphoedema-associated angiosarcomas . However MYC amplification was not found in this case, which further supports the diagnosis of CHE favouring it over angiosarcoma.\nEven though CHE usually shows indolent behaviour, the multifocality of the process in the setting of chronic lymphoedema allowed radical surgical treatment, which would have to be hemipelvectomy in this case. Such a procedure was rejected by the patient. Therefore, the current course of action is the follow up with periodical PET/MR scans. In case of local progression of any nodule, the patient would be indicated for the extirpation of the lesion. However, no growth of the remaining lesions was detected in the follow-up so far and there has been no metastatic spread of the disease since the first detection of the tumour two years ago.", "summary": "We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2880_en.txt", "fulltext": "An 11-month-old female infant presented with bilateral corneal opacity, which was first noted at the age of 3 months. The patient was born full term with an uneventful birth history, and no history of maternal infection or family history of ophthalmologic disease. Ophthalmic examination revealed central corneal opacity approximately 3.0 mm in diameter in the right eye and 2.0 mm in diameter in the left eye, with an underlying keratolenticular adhesion and a cloudy cataract, and that the patient could not trace the light . Contact ultrasound A-scans revealed axial lengths of 18.28 mm and 18.39 mm in the right and left eyes, respectively. Intraocular pressure (IOP) were 14 mmHg in both eyes, measured by a handheld tonometer (Icare Finland Oy Vantaa, Finland). There were no systemic anomalies.\nThe patient’s parents hesitated to accept PKP and fully understood the risks of performing PKP in infants because they had consulted several surgeons before coming to our clinic to seek a second opinion. Her parents were subsequently offered an option of adhesiolysis and adhesive membrane removal combined with lensectomy and vitrectomy. Surgeries were performed at the age of 11 months on 17th and 19th October 2017 in the right and left eye, respectively.\nSurgeries were performed by an experienced surgeon (Y.E.Z.) under general anesthesia using the Accurus with the venturi vacuum system (Alcon Laboratories, Inc.); the cut rate was 2000 per minute and vacuum was 350 mmHg. A corneoscleral incision was made superiorly and four 1.0 mm paracentesis were created in each quadrant. The anterior chamber was initially filled with ophthalmic viscosurgical device (OVD), and the neck of the keratolenticular adhesion was cut using intraocular scissors. There was comprehensive iris posterior synechia. The pupillary aperture was enlarged by four iris hooks through paracentesis in each quadrant after adhesiolysis. Then, a partially resorbed lens and peripheral anterior capsule contraction with zonular elongation were noted underneath the keratolenticular adhesion. The anterior capsular defect was extended to an anterior capsulorhexis of approximately 5.0 mm diameter using a 23-gauge vitrector, while the anterior chamber was maintained by a 23-gauge infusion cannula. After the mode was switched to irrigation/aspiration, the cortex was carefully aspirated. Next, a posterior capsulotomy with a 3.0 mm diameter was performed with the vitrector and the anterior part of the vitreous volume was removed using the same vitrectomy settings. Before the end of the procedure, the residual adhesion was gently peeled off by capsulorhexis forceps curvilinearly following the demarcation line. Surgery was concluded with reformation of the anterior chamber with balanced salt solution and closure of the corneoscleral incision with 10–0 nylon sutures, leaving both eyes aphakic (Additional file 1). No unexpected intraoperative complications were encountered. Clinical manifestations were similar in both eyes.\nTopical treatment consisted of steroidal eye drops gradually tapering over 4 weeks, antibiotic eye drops 4 times daily for 2 weeks, and mydriatic eye drops (phenylephrine hydrochloride and tropicamide compound) once a day for 4 weeks. A gradual reduction in central corneal opacity and improvement in the visual acuity (VA) was noted . Non-contact specular microscopy at one year postoperatively showed large heteromorphic endothelial cells at the borderline between the normal endothelium and the central defect . At the latest follow-up that was two years after surgery, the corneal had excellent transparency, with corrected Teller VA of 20/50 in both eyes and IOP of 15 mmHg in the right eye and 14 mmHg in the left eye. There was no evidence of glaucoma.", "summary": "An 11-month-old female infant of type 2 Peters anomaly presented with bilateral corneal opacity with distinct demarcation, keratolenticular adhesion and cataract, which was first noted at the age of 3 months. By peeling off the adhesion from corneal endothelium combined with lensectomy and vitrectomy, there was a gradual reduction in corneal opacity and improvement in visual acuity after surgery over a 2-year period. Her visual acuity had improved from light perception preoperatively to 20/50 at the latest follow-up. No sight-threatening postoperative complications were noted.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_1652_en.txt", "fulltext": "A healthy 10-year-old male patient presented to the Department of Pediatric and Preventive Dentistry, M.S. Ramaiah Faculty of Dental Sciences, Bengaluru, Karnataka, India, for routine dental care. Oral examination revealed mixed dentition, with deficient space for eruption of second premolar teeth ( and ). The treatment planned was distalization of maxillary first permanent molars by intra-arch pendulum appliance developed by Hilgers. Both the maxillary first permanent molars and first premolars were banded and impression of maxillary arch was made. The pendulum appliance design consisted of anterior acrylic nance portion with two posteriorly extending TMA coil springs made of 0.032\". The plane of the coil springs should be parallel to the maxillary plane; the extensions of TMA wire are then soldered to the molar and premolar bands . The appliance was cemented onto the molars and premolars. The appliance was activated extraorally and was cemented. The appliance was monitored at monthly intervals and the appliance was removed for reactivation and recementation.\nAt the end of 4th month sufficient space was regained and second premolars started erupting into the space gained. At 7th month with the complete eruption of premolars and canines proper maxillary arch alignment was achieved ( and ). The results of this case study have shown that the pendulum appliance is an effective and reliable method for distalization of maxillary molar teeth.", "summary": "The purpose of this paper is to report a case of 10-year-old male patient with loss of space in maxillary molar teeth treated by intra-arch appliance-pendulum appliance by distalization of maxillary first permanent molar teeth. Distaliza-tion of the permanent molar teeth helped in proper eruption of second premolar teeth without any extensive treatment procedures.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1603_en.txt", "fulltext": "A 57-year-old Caucasian woman reported a decrease in visual acuity for 2 weeks. Her best-corrected visual acuity for her right eye was 20/20 and for her left eye 20/100. A slit-lamp examination and intraocular pressure were unremarkable in both eyes. Fundus examination of her left eye revealed temporal intraretinal infiltrates and vitreous opacities . Her right eye was unremarkable.\nFluorescein angiography showed hyperfluorescence of her optic disc, leakage along the vessels, and chorioretinal hyperfluorescent infiltrates . The diagnosis of OT was based on the typical morphology of her ocular lesions and a positive serological testing for Toxoplasma gondii (immunoglobulin G concentration = 537IU/mL).\nShe was treated with clindamycin (Clindamycin® H) 4 × 300mg daily over 3 days without any significant improvement of visual acuity. On the fourth day of hospitalization, she received 100mg prednisolone-21-hydrogensuccinate (Solu-Decortin® H) intravenously in addition to her treatment with clindamycin (Clindamycin® H). Within 2 minutes she developed an acute erythema, particularly of her legs and abdomen , angioedema, hypotension (blood pressure 80/40mmHg), tachycardia (heart rate 140/minute), hyperthermia (38.8°C), and respiratory distress. Subsequently, she was transferred to the Intensive Care Unit to be monitored and treated with clemastine fumarate (Tavegil®), ranitidin (Ranitic®), and intravenous fluids. After 1 hour she recovered and after 12 hours she was transferred back to the ophthalmological ward. Her erythema and angioedema persisted for 32 hours. She had no history of previous steroid use. Subsequent allergy testing was conducted after 3 months in the Department of Dermatology in our hospital. The testing showed a positive skin-prick test for prednisolone and methylprednisolone in the form of a 5mm wheal, and negative results for dexamethasone and hydrocortisone , which confirmed her suspected allergy to prednisolone. Because of her allergic reaction to class A (prednisone-type) corticosteroids and possible complications due to cross-reactions to class D2 (prednicarbate-type) corticosteroids, we avoided any further treatment with systemic or intravitreal corticosteroids.\nIn addition, an oral exposure test with a step-by-step elevation of doses up to 2.0mg of dexamethasone (Fortecortin®) and 1.5mg betamethasone (Celestamine®) was conducted to find alternative corticosteroids for future treatments. For both substances, no local or systemic side effects were observed.\nAfter 1 month of treatment with clindamycin (Clindamycin® H) monotherapy without any increase in visual acuity, the patient underwent a vitrectomy with balanced salt solution filling because of heavy vitreous opacities, and to obtain a sample of the vitreous body. The polymerase chain reaction (PCR) on the sample was positive for Toxoplasma gondii.\nThe postoperative examination of her left eye revealed chorioretinal scars and infiltrates . Her right eye was still unremarkable. Fluorescein angiography of her left eye also revealed hyperfluorescent intraretinal infiltrates of the temporal hemisphere and a scar of the inferotemporal retinal vein branch . Her visual acuity recovered to right eye 20/20 and left eye 20/40.", "summary": "We present the case of a 57-year-old Caucasian woman with an anaphylactic reaction after intravenous injection of prednisolone-21-hydrogensuccinate (Solu-Decortin® H) given for the treatment of toxoplasmosis-associated chorioretinitis. Immediately after the injection, she developed an acute erythema of the legs and abdomen, angioedema, hypotension (blood pressure 80/40mmHg), tachycardia (heart rate 140/minute), hyperthermia (38.8°C), and respiratory distress. Allergological examinations showed a positive skin-prick test to prednisolone and methylprednisolone. In addition, an oral exposure test with dexamethasone (Fortecortin®) and betamethasone (Celestamine®) was conducted to find alternative corticosteroids for future treatments. After oral application, no local or systemic reactions were observed for these two substances.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_953_en.txt", "fulltext": "A 54-year-old white man with a past medical history of right maxillary sinus adenoid cystic carcinoma with metastatic lesions to lung and vertebrae, hypertension, hypothyroidism, and nonalcoholic steatohepatitis presented to our emergency room in June 2016 with mental status changes accompanied by abdominal pain, dyspnea, and fever. He was not on active therapy for his malignancy. He previously received a trial regimen of coenzyme Q10, 12 g every 3 days, from October 2015 to April 2016. His past medical history was remarkable for two prior episodes of ascites and 3 months of progressive liver dysfunction and coagulopathy . He had atorvastatin-induced myalgias leading to its discontinuation in April 2016 although his creatine kinase levels were not elevated. He was taking increasing doses of acetaminophen (2 grams daily) prior to admission. His physical examination vital signs were stable with one recorded febrile episode. In general, he appeared ill and lethargic, and was only oriented to person. He exhibited diminished bibasilar breath sounds, ascites with guarding, hypoactive bowel sounds, right lower quadrant tenderness, and hepatosplenomegaly.\nLaboratory data were remarkable for hyperferritinemia, hypofibrinogenemia, anemia, and thrombocytopenia along with elevated transaminases and coagulopathy . A peripheral blood smear showed neutrophilia, monocytosis, and reticulocytopenia. No microangiopathic changes were seen. Extensive platelet clumping was noted.\nImaging studies revealed small pleural effusions, ascites, and hepatosplenomegaly with no evidence of portal hypertension or splanchnic thrombosis. We were suspicious of HLH in light of laboratory and physical examination findings. Additional differential diagnosis workup – infectious, autoimmune, acetaminophen levels – yielded unremarkable results, including: serology for hepatitis A virus (HAV), hepatitis B virus (HBV), hepatitis C virus (HCV), hepatitis E virus (HEV), cytomegalovirus (CMV), Epstein–Barr virus (EBV), herpes simplex virus (HSV), alpha 1-antitrypsin levels, and antinuclear, anti-mitochondrial, anti-smooth muscle, and transglutaminase antibodies (immunoglobulin A (IgA) and immunoglobulin G (IgG)). ADAMTS 13 activity was >34%. HLH-specific laboratory studies were sent, including soluble CD25 (sCD25), NK cell activity studies, and bone marrow biopsy.\nHe continued to deteriorate with multiple organ failure including renal failure, myocardial injury, and respiratory failure requiring intubation. Empiric therapy, considering the evidence of liver injury and the possibility of HLH, with N-acetylcysteine on a 20-hour intravenous protocol and dexamethasone 8 mg intravenously administered three times daily was initiated. HLH chemotherapy was not done during this time as hepatotoxicity risk outweighed benefits and a definite diagnosis was not confirmed.\nAs an attempt to expedite the evaluation of possible HLH, we isolated mononuclear cells from peripheral blood and evaluated expression of surface markers in cytokine-producing NK cells and cytotoxic NK cells by flow cytometry. We compared the profile with normal controls. The results, available after 36 hours, were remarkable for an increased expression of CD69 in cytotoxic NK cells, and decreased NKG2A in cytokine-producing NK cells in our case. The expression of CD69 and NKG2A in NK cells was evaluated in four other normal donors and the results were similar to the one acquired in parallel to the HLH sample . No differences in protein expression of other markers were observed by flow cytometry (data not shown). These findings included similar surface levels of OX40, GITR, 4-1BB, TIM-3, PD-1, CTLA-4, LAG-3, and ICOS in CD8+ CD3+ T cells, as well as effector (CD127+, FoxP3-) and regulatory (CD127-, FoxP3+) CD4+ CD3+ T cells; similar expression of NKp44, NKG2C, NKG2D, 4-1BB, NKp30, and NKp46 in NK cells (CD56+ CD3-); and similar expression of CD28, CD27, ICOS, Eomes, Blimp-1, Bcl-6, T-bet, Ki-67, and cMyc in naïve (CCR7+ CD45RA+), effector (CCR7- CD45RA+), effector memory (CCR7-CD45RA-), and central memory (CCR7+ CD45RA-) CD4+ and CD8+ T cells. The frequency of all the evaluated immune cell populations was also similar, when comparing cells from our patient with those ones from a healthy control.\nOur patient’s bone marrow biopsy was performed 10 days after admission and delayed due to severe coagulopathy and demonstrated normocellularity (40 to 50%), megakaryocytic hypoplasia, and clusters of foamy histiocytes with ingested marrow cells. Fifteen days after admission, he fulfilled multiple HLH-2004 diagnostic criteria including hyperferritinemia, fever, splenomegaly, cytopenias, hemophagocytosis on biopsy of bone marrow, elevated CD25, and decreased NK cell activity by standardized studies .\nHe steadily recovered with the steroid therapy instituted for 12 days and supportive measures. He was weaned off intubation and sedation. However, he refused further interventions for HLH, namely etoposide-based therapy and continuation of steroid treatment; he chose palliative care and died 2 weeks later.", "summary": "We describe the case of a 54-year-old white man with history of metastatic maxillary sinus adenoid cystic carcinoma who had severe liver injury and cytopenias with progressive clinical deterioration. We performed an evaluation, by flow cytometry, of the expression of surface markers in his natural killer cells that revealed remarkable abnormalities. His syndrome eventually fulfilled criteria for hemophagocytic lymphohistiocytosis and he received therapy with steroids with interval clinical improvement. Unfortunately, he refused further cytotoxic treatment and died 2 weeks later.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1996_en.txt", "fulltext": "Our work is a single case report and has been reported in line with the SCARE criteria .\nA healthy 59-year-old woman on long-term therapy with Hydroxyurea 500 mg daily for essential thrombocytosis, presented with a painless slow-growing lesion of the jaw that had persisted and increased in size for six months, the appearance of the lesion is correlated to the administration of the hydroxyurea treatment. There was no history of local trauma, radiotherapy, HPV infections, or family history of malignancy. She was referred to our department's consultation for specialized care. No other personal or family history was raised during the patient interrogation. Clinical examination revealed a large nodular lesion with irregular borders of the right cheek, infiltrated into underlying tissue, the lesion extending to the free border of the right lower eyelid with dimensions 4 × 4 cm, without sensory disturbances or diplopia (.). In the nose, black horn-shaped hyperkeratosis lesion in the nasal pyramid (.).\nThere were no palpable neck lymph nodes on the left side.\nA biopsy of surgical material was performed, showing a well-differentiated squamous cell carcinoma.\nThe definitive diagnosis of malignancy was made; metastatic investigations were performed, including chest radiography, abdominal echography, PET scan. The results of these exams were normal.\nThe computed tomography (CT) was performed and found free lymph node.\nOn the biological level, the patient presented anemia at 7.4 g / dl for which she was transfused; a thrombocytosis at 548.000, the rest of the assessment was without particularities.\nBased on the positive medical history and clinical examination, the patient was diagnosed clinically with hydroxyurea-induced squamous cell carcinoma, the hydroxyurea was ceased and the hematological therapy was switched to busulfan. The surgery was indicated and performed by the chief professor of our department who has 15 years of operative experience. The surgical procedure had the aim of the restoration of the anatomic landmarks after a large excision of the tumor and reconstruction of full-thickness eyelid defect with a local flap under general anesthesia.\nDuration of surgery: 60 min; estimation of blood loss: 150 ml; duration of hospital stay: 3 days.\nThe patient received amoxicillin/clavulanic acid 1 g twice daily and antalgics for 8 days.\nPost-operative histopathology confirmed SCC proliferation; the different margins of excision were healthy.\nThe multidisciplinary team deciding on the treatment options included surgeons, oncologists, radiotherapists, ophthalmologist surgeons, radiologists. The decision was made to treat the patient with the surgical approach (surgical excision of the tumor) with 1.5 cm free margins and reconstruction; the authors used the superior pedicle nasolabial island flap; with postoperative radiotherapy (PORT) and postoperative chemotherapy (POCRT). Considering the patient's age, general history, the size of the tumor, the prognosis was average.\nPostoperative periods were favorable; the scar was clean and non-inflammatory.\nRoutine follows up 3, 6, and 12 months showed no signs of recurrence.", "summary": "The authors report the case of a healthy 59-year-old woman on long-term therapy with Hydroxyurea 500 mg daily for essential thrombocytosis, presented with a painless slow-growing lesion of the jaw that had persisted and increased in size for six months, the appearance of the lesion is correlated to the administration of the hydroxyurea treatment. Clinical examination revealed a large nodular lesion 4 × 4 cm with irregular borders of the right cheek, infiltrated into underlying tissue, the lesion extending to the free border of the right lower eyelid without sensory disturbances or diplopia. The surgery was indicated. The surgical procedure had the aim of the restoration of the anatomic landmarks after a large excision of the tumor and reconstruction of full-thickness eyelid defect with a local flap under general anesthesia.", "readability_score": 4, "difficulty": "easy" }, { "id": "multiclinsum_test_2309_en.txt", "fulltext": "A 4-day-old male infant, who was born at 40 weeks’ gestational age and weighing 3660 g. He was delivered at term by episiotomy combined with forceps and admitted to the Department of Neonatology of The Second Hospital of Jilin University due to dyspnea in February 2022. No cutaneous lesions were observed. The patient underwent brain magnetic resonance imaging (MRI), the results of which showed a short T1 strip signal shadow (hemorrhage) under the cranial plate of the occipital region on both sides. He had no family history of diseases.The right eye appeared normal in the routine retinal screening performed with RetCam 3 (Clarity Medical Systems, Inc., Pleasanton, CA, USA). However, a color fundus photograph of the left eye revealed a solitary yellowish-white flat mass with an approximate size of 1.5-disc diameters located in the nasal retina . Diffuse hyperfluorescence with slight fluorescence leakage was observed by fluorescein angiography (FA) . On B-scan ultrasound, the mass was inapparent, with no distinct foci of calcification. No treatment was administered and follow-up reexaminations were recommended. Three months later, regression in the size of the retinal mass was observed . FA showed decreased vascularization of the lesion and slight mottled hyperfluorescence over its surface . Seven months later, the fundus examination indicated no lesion in the left eye . FA showed mild tortuous vessels without leakage . The right retina was normal, and thus the clinical course suggested that the isolated RAH may undergo spontaneous regression.", "summary": "An isolated retinal astrocytic hamartoma was detected in the nasal retina of the left eye of a 4-day-old male infant. At the time of initial presentation, we detected a solitary yellowish-white flat mass with an approximate size of 1.5 disc diameters in the nasal retina. Fluorescein angiography (FA) revealed a diffuse hyperfluorescence with slight fluorescence leakage. Seven months later, the fundus examination showed no lesion in the left eye, FA revealed mild tortuous vessels without leakage.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_493_en.txt", "fulltext": "A 62-year old male patient had a robot-assisted laparoscopic prostatectomy in August 2014 for a Gleason 4 + 3 multifocal adenocarcinoma with tertiary grade 5 disease and extraprostatic extension (pT3aN0Mx). The pre-operative PSA was 5.3 μg/L and the post-operative PSA was unchanged at 5.63 μg/L, despite negative surgical margins . The PSA, 6 months post-operatively, had increased to 10.06 μg/L. Pelvic MRI showed no local recurrence or residual prostatic tissue and a bone scan was also negative. Androgen deprivation therapy (ADT) was commenced with a subsequent fall in PSA to < 0.02 μg/L, but it was discontinued after a year due to side effects.\nSerum PSA tests were undertaken regularly . 4.5 years post-operatively, the PSA increased to 2.33 μg/L and staging CT and whole-body bone scintigram showed no evidence of metastatic disease.\nFive years after prostatectomy, the PSA reached 10.6 μg/L, and the patient underwent a [68Ga]Ga-THP-PSMA PET/CT using a cold kit PSMA formulation [–]. Sixty minutes after intravenous injection of 204 MBq of tracer a [68Ga]Ga-THP-PSMA PET/CT was acquired from proximal thighs to skull base on a GE Discovery 690 PET-CT scanner (GE Healthcare, Chicago, USA) according to current guidelines (4 min per bed position with 23% overlap and axial field of view of 16 cm). A low-dose unenhanced CT scan (120 kVp, 0.5 s rotation time, 3.75 mm slices, Noise Index 45, 10–180 mA) was performed for attenuation correction and localization purposes. PET reconstructions included corrections for radiotracer decay, attenuation, modeled scatter, randoms and dead-time, both for time-of-flight (TOF ordered subset expectation maximisation with 24 subsets and 2 iterations) and scatter-limit correction series. The PET/CT showed increased focal uptake in the right mid-cranial fossa, with no focal uptake in the rest of the body. The differential considered at this point was a meningioma, glioma or a prostate metastasis, all known to express PSMA [–]. The patient had no neurological symptoms at the time of presentation.\nA subsequent contrast-enhanced MRI head showed a well-demarcated enhancing dural-based lesion in the floor of the right middle cranial fossa, projecting into the right temporal lobe, with surrounding T2 hyperintense changes in the right temporal lobe, believed to be extra-axial on MRI. The lesion was thought initially to represent a meningioma, both on the [68Ga]Ga-THP-PSMA PET/CT (SUVmax TOF = 6.2) and contrast-enhanced MRI, although the PET/CT had raised the suspicion of a prostate cancer metastasis given its unusually intense uptake .\nAs uncertainty remained between the two possible diagnoses (meningioma or prostate cancer metastasis) in the neuro-oncology multidisciplinary team meeting, the patient was referred for a neurosurgical opinion, which advised the lesion’s surgical resection and dexamethasone 4 mg bd, to reduce surrounding oedema. A second MRI head, performed 1 month later for pre-surgical planning, confirmed a 23 × 18 mm solitary tumour with surrounding oedema in the anterior part of the right inferior temporal gyrus. The tumour contained heterogeneous low and high areas of T2 signal, patchy heterogeneous contrast enhancement and dural extension to the brain surface inferiorly. The second MRI suggested that the lesion was intra-axial. The contrast-enhanced post-operative MRI head showed complete resection of the tumour with a reduction in serum PSA to 2.93 μg/L. The histopathology report confirmed an intra-axial right temporal lobe metastatic prostatic adenocarcinoma with dural extension and intense PSA and PSAP staining. Three months after neurosurgery the patient had recovered well with only mild neurological deficit (left foot drop). Surveillance MRI showed no intracranial mass or abnormal contrast enhancement to indicate residual or recurrent tumour and only expected post-operative encephalomalacia in the anterior inferior part of the right temporal lobe. The serum PSA remained elevated (2.28 μg/l). Currently, the patient is followed up with 3-monthly MRIs and PSA monitoring. ADT has not been restarted. Although the post-operative MRI head showed complete resection, the fact that histopathology documented dural involvement could explain the elevated PSA after surgery and potential presence of residual microscopic meningeal disease. The patient is currently asymptomatic, but he will undergo further PSMA PET/CT if the PSA continues to rise.", "summary": "We report the case of a prostate cancer patient presenting 5 years after robot-assisted laparoscopic prostatectomy with biochemical recurrence, no neurological symptoms, and in the absence of metastatic lesions in the body on conventional imaging. A solitary cerebral metastasis was detected using", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_998_en.txt", "fulltext": "The patient was a 43-year-old Japanese woman who underwent 70 Gy heavy-particle radiotherapy administered as 5 fractions of 14 Gy for a right choroidal malignant melanoma (T3a, N0, M0 clinical-stage IIB according to the 8th edition of the International Union Against Cancer classification) . Positron emission tomography-computed tomography (PET-CT) performed post-treatment revealed no abnormal accumulation of 2-deoxy-2-(fluorine-18) fluoro-d-glucose, and the complete response was recorded . PET-CT examination was subsequently performed regularly every 6 months, and no recurrence was noted up to 4 years after treatment, when contrast accumulation (maximum standardized uptake value 10.9) was observed on the posterior wall of the stomach .\nGastrointestinal endoscopic examination revealed a smooth, protruding lesion on the wall of the greater curvature of the stomach . Endoscopic ultrasonography (EUS) revealed extramural growth of a hypoechoic tumor with the presence of blood flow inside, on the posterior wall of the stomach that was approximately 2.2 × 2.0 cm in size . CT showed a mass with contrast enhancement in contact with the stomach wall . Based on the imaging findings, a gastrointestinal stromal tumor (GIST) at the posterior wall of the lower gastric corpus with extramural growth was suspected. Therefore, the patient was referred to our hospital for treatment.\nLaparoscopic surgery was performed under general anesthesia. A black-pigmented tumor originating from the body of the pancreas was discovered to be in contact with the posterior wall of the stomach , but continuity was not observed. Based on the intraoperative findings, metastasis of the malignant choroidal melanoma to the pancreas was diagnosed. Since no other lesions were discovered in the abdominal cavity, it was determined that resection would be possible, and laparoscopic pancreatic tail resection was performed.\nThe resected tumor comprised a single black nodule with a long-axis diameter of 27 mm . Hematoxylin and eosin staining revealed spindle cells with melanin pigment production . Immunohistological examination showed positive signals for the S100 protein, HNB-45, and Melan A, confirming the diagnosis . On postoperative day 20, a grade IIIa pancreatic fistula (Clavien–Dindo classification version 2) developed, but amelioration was achieved using conservative treatment. The patient was discharged on postoperative day 50.\nAdministration of 3 mg/kg nivolumab was initiated as adjuvant chemotherapy 2 months after surgery, but 2 months later multiple lung metastases were detected by CT. Therefore, administration of 3 mg/kg ipilimumab was initiated. Following the first dose, the patient developed colitis as an immune-related adverse event and grade 3 diarrhea (based on the Common Terminology Criteria for Adverse Events version 5.0 classification), the latter of which was ameliorated by oral administration of 30 mg prednisolone. There was an outbreak of dermatitis, which was also judged to be an immune-related adverse event, and the administration of ipilimumab was discontinued. Two courses of 800 mg/m2 dacarbazine were administered 12 months postoperatively, but the treatment was discontinued due to metastasis to the left kidney. Nivolumab administration resumed 28 months after surgery. The patient was alive after 17 courses of nivolumab without symptoms or exacerbation of the lesion.", "summary": "A 43-year-old Japanese woman underwent 70 Gy heavy-particle radiotherapy for a right choroidal malignant melanoma. Positron emission tomography-computed tomography examination was performed 4 years after treatment, when contrast accumulation was observed on the posterior wall of the stomach. Endoscopic ultrasonography and computed tomography showed a mass with contrast enhancement in contact with the stomach wall. Based on the imaging findings, a gastrointestinal stromal tumor of the posterior wall of the lower gastric corpus with extramural growth was suspected. Laparoscopic surgery was performed under general anesthesia. A black-pigmented tumor originating from the pancreas was discovered. Following an intraoperative diagnosis of metastasis of malignant melanoma, a laparoscopic distal pancreatectomy was performed. The pathological diagnosis was pancreatic metastasis of malignant melanoma. The patient was treated with adjuvant immune checkpoint inhibitors and chemotherapy after surgery, which led to long-term survival.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2906_en.txt", "fulltext": "A 28-year-old Caucasian Greek woman presented to the emergency department after sustaining an injury to her right knee during dancing. The mechanism of her injury compromised vigorous internal rotation of the femur on the tibia with the knee in flexion. She complained of mild pain in both the medial and lateral aspects of her knee joint. Her right knee was locked in 35° of flexion.\nPhysical examination demonstrated negative Lachman-Noulis and anterior drawer tests. Clicks or catches were not detected by palpation during flexion, extension, and rotary motions of her knee joint. There was tenderness in her medial and lateral joint line. Results of standard anteroposterior and lateral roentgenograms were normal. A magnetic resonance imaging (MRI) evaluation demonstrated the tears in both menisci of the right knee , while the anterior and posterior cruciate ligaments were found to be intact .\nOur patient had no relevant medical history. Her physical examination and laboratory tests were unremarkable. No diseases of the connective tissue or other deficiency of the ligaments were detected.\nShe underwent a knee arthroscopy in the next 24 hours, and the diagnosis of combined injury of bicompartmental bucket-handle tears with an intact anterior cruciate ligament was confirmed . Arthroscopic evaluation also revealed a discoid lateral meniscus. Moreover, a grade III chondral lesion (graded on the Outerbridge classification), less than 1 cm2 in size, was detected on the medial femoral condyle . A partial medial and lateral meniscectomy was then performed. The decision was made because of the discoid lateral meniscus and the quality of the ruptured part of the medial meniscus. The torn tissue - approximately 40% of the mediolateral width of each meniscus - was removed. The cartilage defect was repaired by drilling therapeutic holes (approximately 0.5 to 1.0 mm in diameter) into the subchondral bone marrow space underlying the region of the cartilage lesion (microfracture technique) .\nAt our patient's follow-up examination six months after her injury, clinical tests demonstrated that her right knee was stable, had a full range of motion and had no tenderness. The functional outcome was evaluated using Lysholm scoring. At the time of her presentation to our emergency department she had a Lysholm score of 75, while six months after her injury, her Lysholm score had increased to 85 . She was satisfied with the outcome of the operation and returned to her pre-injury activities.", "summary": "A 28-year-old Caucasian Greek woman presented to the emergency department after sustaining an injury to her right knee during dancing. An MRI evaluation demonstrated tears in both menisci of the right knee, while the anterior and posterior cruciate ligaments were found to be intact. A partial medial and lateral meniscectomy was then performed. At a follow-up examination six months after her injury, clinical tests demonstrated that our patient's right knee was stable, had a full range of motion and had no tenderness. She was satisfied with the outcome of the operation and returned to her pre-injury activities.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_2866_en.txt", "fulltext": "A 16-year-old male of East Asian descent arrived at night via air ambulance intubated and ventilated to our Heart Attack Centre with a history that during high-intensity interval training at the gym he suffered a cardiac arrest. On site, he received three shocks for ventricular fibrillation with a return of spontaneous circulation (ROSC) after 17 min. An electrocardiogram (ECG) recorded 11 min after ROSC demonstrated marked anterolateral ST-segment elevation. An ECG recorded 116 min post-ROSC on arrival to our centre showed normalization of the ST segments . On examination, he was intubated and ventilated, invasive blood pressure was 105/75 mmHg, and his heart rate was 86 b.p.m. in sinus rhythm. His heart sounds were normal, and there were no murmurs. His chest was clear to auscultation. Bedside echocardiography demonstrated a structurally normal heart with preserved left ventricular (LV) systolic function and no regional wall motion abnormalities. Initial troponin T was 798 ng/L (0–14 ng/L) and peaked at 3073 ng/L 2 h later. His parents gave a past medical history of Kawasaki disease, but that he was not taking any medications.\nIn view of the patient's stable clinical status, the lack of persistent ST-segment elevation on the ECG, and normal echocardiogram, we did not perform emergent coronary angiography as we did not suspect an acute coronary artery occlusion. He received a 300 mg loading dose of aspirin, a 180 mg loading dose of ticagrelor and continued on 75 mg aspirin once daily, and ticagrelor 90 mg twice daily with lansoprazole 30 mg once daily. In addition to antiplatelet therapy, he received a single 2.5 mg dose of fondaparinux, bisoprolol 2.5 mg once daily, and atorvastatin 80 mg once daily. He was admitted to the adult cardiac intensive care unit where he remained stable. Coronary angiography was performed the following morning.\nKawasaki disease was diagnosed at the age of 3 months in his country of origin for which he received intravenous immunoglobulin (IVIG) therapy on day 21 of his illness. He was not treated with acetylsalicylic acid nor was he anticoagulated. Echocardiography 6 days after IVIG treatment showed an aneurysm in the left main coronary artery measuring 5.5 mm. He responded well to treatment and remained under follow-up for mild left main coronary artery dilatation.\nAged 7 years following routine surveillance echocardiography suggestive of left anterior descending artery (LAD) stenosis, he underwent invasive coronary angiography. This demonstrated a small aneurysm followed by an 80% calcific stenosis of the proximal LAD, and a chronic total occlusion (CTO) of the dominant right coronary artery (RCA). Cardiac magnetic resonance (CMR) imaging showed reversible ischaemia in the inferior, inferolateral, and anterolateral walls.\nIn 2012 aged 8 years, he underwent elective intravascular ultrasound-guided rotablation-assisted percutaneous coronary intervention (PCI) to the proximal LAD with a 3.0 mm × 18 mm Multi-Link Vision bare-metal stent (Abbott Vascular, Chicago, USA; ). This corrected the CMR perfusion abnormalities initially, but from 2015 he was lost to follow-up and was not taking any medications.\nCoronary angiography during the current admission demonstrated an unobstructed left main stem; however, the previous LAD stent was occluded with the distal vessel filling retrogradely via septal and well-developed epicardial collaterals from the RCA. The left circumflex artery (LCx) was unobstructed . There was a CTO in the RCA, as before, in the mid vessel . The likeliest explanation for his presentation was the insufficient ability of the collateral circulation to meet the metabolic demand of the heart during exercise, resulting in ischaemia and ventricular fibrillation.\nComputed tomography of the brain demonstrated no acute intracranial abnormality, and his neurological course was unremarkable.\nFollowing a local Heart Team discussion, he was transferred to a different centre with expertise in both adult and paediatric cardiology and cardiac surgery. He underwent further imaging. Computed tomography established that the internal mammary arteries (IMAs) were free of disease. CMR demonstrated increased indexed LV end-diastolic volume (118 mL/m2) and normal LV wall thickness and normal indexed LV mass with preserved global LV systolic function and no regional wall motion abnormalities at rest. There was a limited infarct in the apical anterior wall and part of the apical septum . There was inducible ischaemia in 5/7 segments of the LAD territory and 4/5 segments of the RCA territory. All segments were viable.\nAt the Heart Team meeting, it was felt that either percutaneous or surgical revascularization were suitable strategies. Two days later, the case was discussed with international colleagues and a consensus reached to revascularize him surgically with the opinion being that this would provide him with a more durable result. This was particularly pertinent in his case given the RCA CTO and previously occluded stent. Following detailed discussion with the patient and his parents, he received a left IMA to the LAD and free right IMA to the posterior descending artery. His immediate post-operative course was uncomplicated. He was discharged home 7 days after surgery on aspirin 75 mg once daily, clopidogrel 75 mg once daily, ranitidine 300 mg once daily, bisoprolol 1.25 mg once daily, atorvastatin 80 mg once daily, and paracetamol as required.\nHe was last seen in clinic in December 2019. Symptomatically, he was well and had returned to the gym but was refraining from heavy weights given his sternotomy scar. Clinical examination revealed a well-healed wound, no murmurs and a clear chest. All medications were stopped except aspirin and atorvastatin. He underwent repeat CMR in January 2020, which showed no inducible myocardial ischaemia.", "summary": "Our patient, a 16-year-old boy with Kawasaki disease, presented with cardiac arrest during exercise. Coronary angiography showed that a proximal left anterior descending artery stent implanted at the age of 8 years had occluded some time ago and his right coronary artery was also chronically occluded. He has discussed in several Heart Team meetings and with international colleagues and a consensus reached to revascularize him surgically.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_1100_en.txt", "fulltext": "The proband was a preterm newborn boy, the first child of non-consanguineous parents, born at 31 weeks gestation to a 44-year old father and a 43-year old mother by cesarean section. At birth, the child weighed 1,480 g, measured 44 cm in crown-to-heel length, and exhibited multiple congenital anomalies. The newborn was transferred to the Intensive Care Units (ICU) immediately after birth. His general health condition deteriorated progressively, leading to his death at 105th days after birth. The newborn had brain malformation, including ventriculomegaly and corpus callosum dysgenesis, cleft lip and palate, retrognathism, hypertelorism, clenched hands with overlapping fingers, and hypotonia. Additionally, he revealed mild heart septal hypertrophy, ambiguous genitalia, enlarged kidneys without corticomedullary differentiation, and gallbladder with tiny cystic formations . His mother had three miscarriages from previous marriages and one miscarriage with her current husband. The remaining of his family history was otherwise unremarkable.\nBoth parents signed a written informed consent and the mother signed as the legal representative for the child. Peripheral blood was obtained to isolate genomic DNA for CMA using Qiagen QIAamp® DNA Mini kit (Hilden, Germany). Karyotyping was performed in a private laboratory through conventional cell culture, harvesting, and GTG banding with a > 550 bands resolution following standard procedures . Chromosome analyses were done using Zeiss Axio Scope (Jena, Germany) and the software IKAROS® (Metasystems Corporation, Altlussheim, Germany). All laboratory procedures were carried out following international standardized protocols and consensual criteria of quality.\nThe CMA was carried out on proband and his biological parents using the GeneChip® CytoScanHD™ (Affymetrix, Santa Clara, USA) following the manufacturer’s recommendations without modifications. Chromosomal analyses were done using the Chromosome Analysis Suite (ChAS®) software (Affymetrix, Santa Clara, USA) and the CNVs found in the patient were analyzed in comparison with public databases, including Database of Genomic Variants (DGV), Database of Chromosomal Imbalance and Phenotype in Humans using Ensemble Resources (DECIPHER), and CytoScanHD™ Array Database. Furthermore, CNVs were classified according to their nature, based on [, ].\nThe proband showed a male karyotype with a large submetacentric SMC in 90% of the analyzed metaphases after counting 50 metaphase spreads. His karyotype was 47,XY,+mar[45]/46,XY[5], suggesting 10% mosaicism. The parental karyotypes and CMA results had no visible numerical or structural alterations. The proband’s CMA revealed the marker chromosome corresponded to a de novo 70.77 Mb gain at arr[GRCh37] 9p24.3q21.11(203,861_70,974,662)× 4[0.3] dn with 30% mosaicism, encompassing 286 genes, including 152 OMIM morbid genes .", "summary": "Herein, we reported a postnatal case of a newborn who died in early infancy with multiple congenital malformations due to a mosaic de novo tetrasomy 9p detected by Chromosomal Microarray Analysis. Conventional cytogenetics analysis of the proband was 47,XY,+mar", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_2732_en.txt", "fulltext": "An 85 year old left hand dominant female presented with a six week history of rapidly growing lump on the thenar eminence. Clinical examination revealed a non-tender large lobulated mobile swelling measuring 5 × 4 cm and involving the whole thenar eminence. A sarcoma was initially suspected. Plain X-ray revealed severe osteopenia with erosive changes in the first Carpometacarpal joint, and a soft tissue swelling around an osteophyte detached from the base of the first metacarpal. MRI reported a septate thin walled cystic structure extending from the first carpo-metacarpal joint space against a background of diffuse active synovitis . In operating theater, a multilobulated lesion with a neck extending to the first CMC joint was observed. The histological analysis revealed a 3.4 g previously opened specimen consistent with a ganglion cyst. The patient made an excellent post-operative recovery with no evidence of recurrence.", "summary": "An 85 year old left hand dominant female presented with a six week history of rapidly growing lump on the thenar eminence. Clinical examination revealed a non-tender large lobulated mobile swelling measuring 5 x 4 cm and involving the whole thenar eminence.", "readability_score": 3, "difficulty": "medium" }, { "id": "multiclinsum_test_1366_en.txt", "fulltext": "A 63-year-old woman presented with a firm 7 cm multilobular tender mass located in the region of the right sternocleidomastoid directly above the clavicle. She had multiple comorbidities including renal failure, systemic lupus erythematosus, and protein S deficiency, a history of transient ischemic attack, deep vein thrombosis, and pulmonary embolism, and longstanding malnourishment requiring jejunostomy tube placement. The firm mass was identified 1 month after an unsuccessful attempt at placing a right internal jugular vein central line. When the central line was originally placed, she developed pain and swelling of her neck. The swelling was pulsatile at that time and enlarging. She was offered surgery as the standard of care for her condition which she refused because she did not want to undergo an invasive procedure and was aware of the risks involved with holding her anticoagulant medication. She had two thrombin injections in the mass since without success. She complained of right neck pain. She was not having any difficulty breathing and denied shortness of breath.\nSoft tissue ultrasound after the two thrombin injections showed pseudoaneurysm and a 7 × 3.3 × 4 cm multilobular hematoma at the base of the right neck. CTA of the neck showed a corresponding heterogeneous mass. The pseudoaneurysm was seen measuring 1.5 cm at the internal margin of the hematoma. Serial imaging with CTA demonstrated enlargement over a 2-week interval .\nAngiogram showed blush of contrast coming off of the sternocleidomastoid branch of the superior thyroid artery identifying the location of the pseudoaneurysm . This branch was embolized using glue. Repeat angiogram showed successful embolization and no contrast blush.\nOn follow-up one month later, the mass was no longer pulsatile but still present on physical exam. CTA of the neck showed a decrease in size from the previous CTA .\nOn follow-up 8 months later, she was found to have a soft neck with no mass on physical exam.", "summary": "A 63-year-old female with multiple comorbidities presented with a firm 7 cm tender mass located in the right neck. Ultrasound showed pseudoaneurysm and a 7 × 3.3 × 4 cm multilobular hematoma in the location of the previous central line. CTA showed a corresponding heterogeneous mass. Serial imaging demonstrated enlargement over 2 weeks. Angiogram showed contrast blush off of the SBSTA.", "readability_score": 5, "difficulty": "easy" }, { "id": "multiclinsum_test_707_en.txt", "fulltext": "A 30-year-old male with a known history of KTS presented with a rapidly enlarging subcutaneous central frontal mass, measuring 41 × 47 × 54 mm since its formation just 3 months earlier causing pain and an obvious cosmetic defect. The patient had no demonstrable neurological signs on examination with normal pre-operative laboratory studies, however, had previous episodes of significant hemorrhage arising from the lesion as a result of minor trauma.\nClinically, the patient was dysmorphic, most notably severe soft tissue overgrowth, bilateral lower limb lymphoedema, and cellulitis. Despite this, he had no history of other complications arising as a result of his KTS such as malignancy or peripheral hemangioma.\nRadiological findings initially began with computed tomography angiogram, which revealed a destructive expansile arterially-enhancing lesion arising from the frontal bone. Heterogenous contrast enhancement on magnetic resonance imaging further defined the lesional dural involvement and invasion into the superior sagittal sinus sparring cerebral parenchyma.\nGross total resection consisted of a T-shaped incision followed by bilateral frontal craniectomies . The vascular lesion was identified arising from frontal bone and within the superior saggital sinus. The lesion was resected en bloc from calvaria. A custom-made titanium implant was used for cranioplasty .\nPostoperatively, the patient had a full recovery. Histopathological hemotoxylin and eosin stains revealed the lesion extending through bone, with a small foci of extramedullary hemopoiesis, consistent with hemangioma. There has been no evidence of recurrence 6 months post resection.", "summary": "A 30-year-old male presented with a rapidly enlarging frontal skull lesion that had developed in only 3 months. Radiological investigation revealed a highly vascular lesion attached to the frontal bone. The lesion was surgically resected with the patient making complete recovery. Histopathology was consistent with an osseous hemangioma.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_2089_en.txt", "fulltext": "A 25-year-old woman with liver cirrhosis secondary to autoimmune hepatitis diagnosed at a local hospital presented with fatigue, painless jaundice and lower limb swelling for two weeks, there was no evidence of hypertension or protenuria excluding the possibility of pre-eclampsia or eclampsia. There was no history of ascites, spontaneous bacterial peritonitis or hepatic encephathy. Pregnancy was terminated at 27th week at the local hospital. She was on 40 mg of Prednisolone daily for possible autoimmune hepatitis, soon after, she developed steroid induced diabetes. She was referred to our hospital after 6 weeks of the intial presentation with two episodes of hematemesis, tachycardia (heart rate 120) and blood pressure of 84/50. The patient was admitted to ICU and started on octreotide and Piperacillin/Tazobactam. Gastroscopy revealed gastric varices and bleeding was endoscopically controlled. During admission, liver function tests worsened, and transjugular liver biopsy showed established liver cirrhosis with no obvious active pathology. We started tapering the steroids slowly after being on 40 mg for 4 weeks with no improvement of jaundice without evidence of active inflammation on liver biopsy. During admission, she developed painful, erythematous lesion on her right forearm at the IV access area that became necrotic and spread quickly and increased in size over a period of 24 hours. The lesion included the skin and subcutaneous tissue and was flecked with tiny black spots. Vital signs: temp 37.4ºC, BP 128/80 mmHg, pulse 66 /min, RR 20 /min, SaO2 100% on room air.\nChest was clear to auscultation and cardiovascular exam was normal. Abdomen was soft and lax, distended with ascites but no tenderness. Extremities showed mild lower limb edema. Her investigations showed a white cell count 14.24 x 103/mm3 with Neutrophils of 86%, hemoglobin 9.2 g/dl, and platelets of 103000 /mm3 , LFT: total bilirubin 457 umol/L, direct 321, alkaline phosphatase 430U/L, ALT 174U/L, AST 248U/L, gamma GT 227, total protein 51g/L, albumin 18g/L, PT 19.4 and INR was 1.7, PTT 58, renal function test was within normal, fasting glucose 9.8 mmol/L. ESR was 2, Blood culture showed no growth after 5 days.\nMRI of the right upper extremity showed inflammatory changes through the anterior compartment of the forearm suggestive of fasciitis.\nShe was taken to the operating room for debridement after 24 hours, delay was due to anesthesia issues in this high risk patients. At this point after 18 days of admission, Prednisolone dose reached 20 mg daily and antibiotic changed from Piperacillin/Tazobactam to Imipenem when the culture results from tissue biopsy grew Klebsiella Pneumonia extended spectrum b lactamase producer (ESBL).\nHistopathological examination of the debrided soft tissue revealed necrotic subcutaneous fat and skeletal muscle fibers invaded by broad hyphae, irregularly branched, with rare septations suggestive of mucormycosis ( and ). There were no clinical symptoms or signs of visceral dissemination of infection to warrant further imaging or biopsy. The patient started on Amphotericin B (Abelcet®) 300mg IV OD after 20 days of admission and was continued on imipenem. In spite of surgical debridement, her condition continued to deteriorate and ended up after 6 days with above elbow amputation, tissue-cultures for both bacteria and fungi were negative. Amphotericin B was stopped after amputation as it was considered that the source of infection was eliminated but was restarted again 3 days after discontinuation with imipenem continued all through with the addition of vancomycin. The patient continued to deteriorate and the stump showed signs of disseminated infection, which proved to be invasive mucormycosis infection. Right shoulder disarticulation done but she passed away 2 days later due to multi-organ failure after 35 days in the hospital.", "summary": "We described a rare case of forearm infection originating in a traumatic intravenous access portal in a 25 year-old woman with liver cirrhosis secondary to autoimmune hepatitis.", "readability_score": 2, "difficulty": "hard" }, { "id": "multiclinsum_test_1133_en.txt", "fulltext": "A 27-year-old male involved in a motor vehicle accident was brought to Emergency Department room with respiratory distress. He was intubated upon arrival due to low Glasgow Coma Scale (GCS) with extensive maxillofacial injuries. Thoracic examination showed reduced air entry at right chest wall region with palpable crepitus on his right neck region due to subcutaneous emphysema from the neck to the anterior chest wall. The heart sound was barely audible. A curvilinear transducer on the right second intercostal ribs shows the absence of sliding signs, suggestive of right pneumothorax. A FAST scan was performed, but, on subxiphoid view of the heart, only the right ventricle is seen during diastole. Part of the cardiac image was obscured by A-lines . This raised a suspicion of pneumopericardium given the subxiphoid window was showing partly A-lines and the other half of anatomy partially obscured. We proceeded with focused cardiac ultrasound, and only A-lines were visible on parasternal long axis (PLAX), parasternal short axis (PSAX), and apical four chamber (A4C) views.\nThe patient underwent head and chest CT scan that confirmed the diagnosis of Le Fort II facial bone injury, right pneumothorax, and right pulmonary contusion with pneumopericardium . The pneumopericardium was treated conservatively, but other injuries were treated accordingly.", "summary": "A young man involved in a car accident and sustained blunt thoracic injuries, among others. As part of primary survey, FAST scan was performed. Subxiphoid view to look for evidence of pericardial effusion showed part of the cardiac image obscured by A-lines. Other cardiac windows showed only A-lines, as well. A suspicion of pneumopericardium was raised and CT scan confirmed the diagnosis.", "readability_score": 3, "difficulty": "medium" } ]