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pmc-6458894-3 | A 74-year-old woman presented with body weight loss. Her medical history included hypertension and diabetes mellitus, both of which were well-controlled by medication. Laboratory data were unremarkable except for decreased hemoglobin (9.1 g/dl). Tumor markers were within normal limits. Colonoscopy revealed a circumferential tumor in the sigmoid colon. Histological examination of the biopsy revealed RAS-mutant adenocarcinoma. The RAF mutational status was not investigated. Contrast-enhanced CT showed irregular colonic wall thickening with massive involvement of the urinary bladder (). There were enlarged regional lymph nodes suggestive of metastases but no distant metastasis. The pretreatment diagnosis was LACC stage IIIC (T4bN2M0).
Similar to Cases 1 and 2, we initially performed loop transverse colostomy. Subsequently, we planned 6 courses of chemotherapy with CAPOX plus bevacizumab. The patient eventually received 5 courses of CAPOX plus bevacizumab and the remaining 1 course without oxaliplatin due to grade 2 neurotoxicity. Follow-up CT after the neoadjuvant chemotherapy revealed significant tumor shrinkage ().
Thereafter, we performed sigmoid colectomy and partial resection of the bladder. The postoperative course was uneventful, and the patient did not suffer from neurogenic voiding dysfunction or urinary incontinence. Pathological examination revealed that tumor cells or degeneration was present in less than one third of the entire lesion, which was consistent with grade 1a effect according to the Japanese Classification of Colorectal Carcinoma () []. The final diagnosis was ypT4b, ypN0, M0, ypStage IIC. She subsequently received 8 courses of adjuvant chemotherapy with capecitabine alone. There was no recurrence at 16 months after the initial diagnosis. |
pmc-6458895-1 | A 57-year-old male from Thailand with long-standing psoriatic arthritis presented with diffuse scaling of his skin. He was being treated for psoriatic arthritis with adalimumab, leflunomide, and topical clobetasol, but had stopped taking all medications two weeks prior to presentation. At baseline, the patient was a gardener and able to complete all his activities of daily living. However, during the disease course, he noticed diffuse skin scaling and had diarrhea, leading to fatigue. His only joint complaint was chronic left ankle pain, and he denied oral ulcers. Physical exam revealed diffuse yellow scaling covering his entire body with underlying erythema and tenderness without mucosal involvement (Figures and ). Empiric antibiotics were started until blood cultures ruled out infection. Labs were notable for a creatinine of 3.3 mg/dL, with no prior history of renal disease, calcium 7.8 mg/dL, phosphate 5.9 mg/dL, bicarbonate 13.0 mmol/L, urine pH 5.0, and uric acid of 12.7 mg/dL (). The patient was admitted to the intensive care unit given >80% of body surface area involvement and acute renal failure. He received 4 L of normal saline for initial resuscitation. On day 2, the patient was determined to be euvolemic based on clinical exam with moist mucous membranes. Despite aggressive fluid administration, renal function did not improve, and creatinine continued to rise and peaked at 4.61 mg/dL, with phosphate of 7.0 mg/dL and 48 hours of anuria. Renal ultrasound showed 5 mm nonobstructing renal calculi in the left kidney and small echogenic kidneys consistent with chronic kidney disease (). Subsequent urine microscopy showed diffuse polymorphic uric acid crystals, consistent with uric acid crystal-induced nephropathy (). He was managed with rasburicase, urinary alkalinization, and fluids. The underlying erythrodermic psoriasis was treated with restarting leflunomide, topical isotretinoin, and clobetasol. His renal function improved dramatically; urine output, uric acid, and electrolytes normalized. He was discharged on topical clobetasol and leflunomide and new addition of secukinumab with little to no skin involvement. |
pmc-6458909-1 | A 69-year-old man presented on May 1, 2018, to a hospital with fevers and diarrhea. The patient had a complicated history, with cardiovascular problems including an abdominal aortic aneurysm, atrial septal defect status after Amplatzer occluder device placed in 2008, and sick sinus syndrome status post dual-chamber pacemaker placement in 2015.
The patient was diagnosed with primary myelofibrosis with JAK2 mutation in April 2015, treated with lenalidomide and prednisone which were tapered off, and his treatment was switched to ruxolitinib. In November 2016, his disease transformed to secondary acute myelogenous leukemia. He was treated initially with induction chemotherapy with 7 + 3 cytarabine and daunorubicin, and follow-up bone marrow biopsies showed no evidence of leukemia. He was subsequently treated with one course of consolidation chemotherapy with high-dose cytarabine. He ultimately underwent HLA-matched related hematopoietic stem cell transplant from his brother in February 2017. His conditioning regimen was reduced-intensity fludarabine and melphalan. His transplant course was complicated by culture-negative neutropenic fevers and neurotoxicity due to tacrolimus, so he was switched to mycophenolate for graft-versus-host disease (GVHD) prophylaxis. Follow-up bone marrow biopsy showed persistence of fibrosis, but was negative for leukemia, and DNA testing proved that he had over 99% donor DNA in the cell population.
His posttransplant course was complicated by mild GVHD involving the skin and colon diagnosed by sigmoidoscopy. He was treated with high-dose steroids, budesonide, and sirolimus, which were ultimately tapered off by the end of July 2017. However, he developed chronic GVHD involving his mouth in September 2017, which was treated with prednisone, sirolimus, and dexamethasone swish and spit. His prednisone dose was tapered to 30 mg daily by November 2017, and by January 2018, he was only taking prednisone 10 mg daily. By March 2018, it was decreased to 10 mg every other day.
In March 2018, he developed an acute right lower extremity deep vein thrombosis involving his common femoral, profunda, femoral popliteal, posterior tibial, and peroneal veins. He was treated with tissue plasminogen activator, heparin, and thereafter apixaban.
He was admitted in early May 2018 for a week at an outside facility with abdominal pain, diarrhea, and septic shock requiring admission to the intensive care unit, necessitating the use of pressors and stress-dose steroids. He also developed acute renal failure but never required hemodialysis. Both blood and stool cultures were positive for S. enterica serotype Mbandaka. This was believed to be associated with the multistate outbreak of infections linked to cereal. The patient reported that he ate an entire box of cereal within one week prior to becoming ill. Blood culture susceptibilities demonstrated that the organism was susceptible to ampicillin, ceftriaxone, ciprofloxacin, tetracycline, tigecycline, and trimethoprim/sulfamethoxazole.
He was treated with IV ceftriaxone and discharged on amoxicillin/clavulanic acid, completing a total of three weeks of antibiotics. However, about a week after stopping antibiotics, at the end of May 2018, he was readmitted with fevers and diarrhea. Blood cultures were negative, but stool cultures were again positive for S. enterica serotype Mbandaka. He was treated initially with intravenous antibiotics and was discharged on 28 days of trimethoprim/sulfamethoxazole 1 DS tab twice per day. While on antibiotics, his abdominal pain, fevers, and diarrhea resolved.
Approximately seven days after stopping his antibiotics, in early July 2018, he developed a third recurrence of fevers and diarrhea. Blood cultures returned negative, but stool cultures were again positive. He was admitted to the hospital for the third time, treated with intravenous antibiotics for 3 days and discharged home with a second 28-day course of trimethoprim/sulfamethoxazole. Stool studies were also positive for Clostridium difficile, so he received oral vancomycin for treatment. His diarrhea and abdominal pain again resolved. Soon after his discharge, his prednisone dose was decreased to 5 mg every other day, and he was maintained on sirolimus 1 mg daily.
After his third admission, he was evaluated by infectious diseases for his recurrent infections with salmonellosis. A transesophageal echocardiogram was performed, which showed a 0.9–1 centimeter mitral valvular vegetation (). This appeared very mobile and was associated with mild eccentric mitral regurgitation. The Amplatz closure device showed no evidence of infection, and his pacemaker leads showed no vegetation.
The patient also had a CT scan of his abdomen which showed a mild increase in the degree of mural thrombus associated with the 2.9 × 2.7 cm calcified abdominal aorta in the lower abdomen, now measuring 10 mm compared to 7 mm on the prior exam from 14 months prior. An indium-111 white blood cell scan was negative.
The patient's antibiotics were converted from oral trimethoprim/sulfamethoxazole to ceftriaxone 2 grams intravenously daily for six weeks. He was maintained on oral vancomycin for his Clostridium difficile infection. Four weeks into intravenous therapy, however, he was rehospitalized again with fevers and diarrhea. Blood and stool cultures were negative, and stool for Clostridium difficile was negative. His fevers and diarrhea ultimately resolved, and he was discharged home.
A follow-up transesophageal echocardiogram prior to discontinuation of intravenous antibiotics showed a smaller linear echodensity, now 0.5 cm, attached to the anterior leaflet of the mitral valve. There continued to be mild mitral regurgitation. Again, both the Amplatz closure device and pacemaker leads showed no vegetation.
Upon completion of six weeks of intravenous antibiotics for endocarditis, the patient restarted trimethoprim/sulfamethoxazole. He was ultimately tapered off oral vancomycin for his infection with Clostridium difficile. Length of therapy for his salmonellosis is to be determined based on follow-up stool testing and immunosuppressive course. |
pmc-6458917-1 | A 60-year-old woman referred to our medical centre with a 2-month history of an approximately 4 cm lump in the medial part of her left breast. Clinical examination revealed no palpable left axillary lymph nodes. The patient had no other significant medical history. In mammography, a radiologic circumscribed, well-demarcated density, of her medial left breast without lymph node enlargement, was identified (). She underwent a fine needle aspiration (FNAC) of the breast lump which suggested a poorly differentiated carcinoma. Metastatic work-up including computed tomography (CT) of the thorax, upper and lower abdomen, revealed no evidence of disease elsewhere. Bone marrow scan was normal. She underwent total mastectomy and sentinel lymph node (SLN) biopsy.
A 22,5X17X5cm mastectomy specimen and sentinel lymph node biopsy (SLNB) was performed. The SLNB performed during surgery was negative for malignancy. On gross examination, serial sectioning revealed a large solid, well circumscribed, lobulated, with subtle nodularity tumor, measuring 4,8X4X3,8cm, with central necrosis ().
Surgical specimen was fixed in 10% buffered formalin, routinely processed, and embedded in paraffin. Histological slides of the formalin-fixed tumor tissue (one fragment per centimeter of the tumor was sampled) were deparaffinized and stained with hematoxylin-eosin. The final histopathological report showed an undifferentiated, high grade tumor of the “small round blue cell” morphology, suggestive of rhabdomyosarcoma (Figures –). This was further confirmed by immunohistochemistry (IHC). Immunohistochemistry (IHC) was performed according to manufacturer's protocols and included evaluation of expression of desmin (clone D33; manufacturer DAKO), myogenin (clone F5D; manufacturer DAKO), myo-D1 (clone 58A; manufacturer Immunologic), neurofilament (clone 2F11; manufacturer DAKO) and bcl-2 (clone 124; manufacturer DAKO). Positive and negative controls were used.
Staining for desmin (), myogenin (), myoD1 (), NF (), and bcl-2 () confirmed the diagnosis of RMS (solid variant of alveolar type). The tumor was staged as T2 pN0 (sn), according to TNM Classification.
After the exclusion of secondary origin, the woman was treated with chemotherapy based on Intermediate risk-good prognosis Alveolar group I-III. The patient was given 14 cycles of second-line chemotherapy with vincristine, dactinomycin, and endoxan (VAC). The patient is disease free at the follow-up of 24 months from the completion of treatment. |
pmc-6458937-1 | A 45-year-old man with no relevant medical history presented with complaints of a persistent dry cough, fever, and night sweats in August 2014. He sought urgent medical assistance a couple of times during this period and underwent empirical antibiotic therapy without improvement. A more detailed investigation using chest-computed tomography (CT) revealed thoracic lymphadenopathies; an excisional biopsy of a cervical lymph node was compatible with mixed cellularity classical HL (CD20 negative, CD3 negative, CD30 positive, CD15 negative, and PAX5 positive). Positron emission tomography-computed tomography (PET-CT) was performed for staging, which revealed involvement of the supra- and infradiaphragmatic lymph nodes, with no extranodal involvement, consistent with Ann Arbor stage IIIB disease.
The patient underwent polychemotherapy consisting of adriamycin, bleomycin, vinblastine, and dacarbazine (ABVD). After two cycles, an interim PET-CT scan was negative; he underwent a total of six cycles of ABVD treatment; nevertheless, the posttreatment PET-CT scan remained negative.
Unfortunately, five months later, in January 2015, a screening CT scan revealed iliac and retroperitoneal lymphadenopathy. A new biopsy revealed relapse of his HL. He then underwent second-line therapy with ifosfamide, carboplatin, and etoposide (ICE); however, the disease remained refractory after two cycles. A third-line therapy based on gemcitabine was successful, and hematopoietic stem cells were collected by apheresis in April 2015. A PET-CT scan after two cycles revealed complete response (Deauville score, 2), and the patient was referred to the authors' service for autologous hematopoietic stem cell transplant (HSCT) as consolidation therapy.
The autologous transplant was performed following a conditioning regimen with carmustine, etoposide, cytarabin and melphalan (BEAM) in May 2016. His transplant course was unremarkable, and neutrophil engraftment occurred 10 days later. A posttransplant PET-CT scan revealed persistent complete response.
Because the disease had relapsed <12 months after the completion of frontline therapy, the patient was started on BV according to the brentuximab vedotin as consolidation therapy after autologous stem cell transplantation in patients with Hodgkin's lymphoma at risk of relapse or progression (AETHERA) study, as posttransplant consolidation in July 2016 []. He received BV 1.8 mg/kg every three weeks, for a total of 16 doses.
In April 2017, after 10 doses of BV, the patient presented with sneezing, dry cough, and odynophagia, but without dyspnea, thoracic pain, or other associated symptoms. His workup revealed no abnormalities in whole blood count or acute inflammatory markers, and a chest CT scan was normal. Empirical treatment with levofloxacin and oseltamivir for upper respiratory tract infection was started, and he experienced partial improvement of symptoms. A polymerase chain reaction (PCR) assay for respiratory viruses was negative; therefore, oseltamivir was discontinued. Two weeks later, the patient returned to the authors' center with fever, chills, dyspnea, and asthenia. His physical examination was remarkable for a respiratory rate of 28 breaths/min, hypoxemia (arterial oxygen partial pressure 69 mmHg), and crackles on pulmonary auscultation. No adenopathies were observed.
On admission, a repeat workup revealed a hemoglobin level of 10.8 g/dL (108 g/L), leucocytes 4670/mm3 (neutrophils 3540/mm3 and lymphocytes 650/mm3), a platelet count of 105,000/mm3, and a lactate dehydrogenase level of 740 U/L (normal range, 240–480 U/L). A chest CT scan revealed areas of parenchymal consolidation and diffuse ground-glass opacities in both lungs, with predominantly peribronchovascular distribution in the upper and middle fields (). He was started on empirical therapy with piperacillin-tazobactam (4.5 g every 6 h), oseltamivir (75 mg twice per day), and trimethoprim/sulfamethoxazole (trimethoprim 17 mg/kg/day). A bronchoscopy with bronchoalveolar lavage was performed, which was positive for PJ according to toluidine blue staining and to PCR analysis.
Prophylaxis against pneumocystis was initiated in the patient at the time of neutrophilic engraftment after the transplant in June 2016 according to institutional guidelines. However, the patient had stopped it on his own due to gastrointestinal adverse effects for the preceding two months. The last CD4 lymphocyte count available from the patient in March 2017 was 317 cells/mm3.
After a 21-day course of treatment with trimethoprim/sulfamethoxazole, the patient experienced full clinical recovery and was restarted on prophylaxis. |
pmc-6458939-1 | A 25-year-old man from Ghana was admitted to our hospital for an altered mental state, weight loss, neck pain, cough, fever, nausea, vomiting, and photophobia for 7 days. He reported a cough with greenish phlegm and blood-tinged sputum and a weight loss of ∼20 lb in the last month. He reported that he could not eat because he was always nauseous and experienced nonbilious, nonprojectile vomiting. He denied chest pain, difficulty breathing and swallowing, diarrhea, dysuria, and skin rashes. His past medical history included intermittent asthma and migraines, but he was not on any medications. He did not have any significant family history or prior surgeries. He reported that he smoked 10 cigarettes a day for the past 5 years, drank alcohol occasionally, and denied illicit drug use except for marijuana. He reported that he last traveled to Ghana 6 months priorly, and he denied having contact with sick persons. He reported that he was sexually active with many partners but denied any prior sexually transmitted diseases.
Examination revealed a cachectic young male with a heart rate of 102 beats/min, a blood pressure of 108/69 mmHg, a respiratory rate of 20 breaths/min, a temperature of 98°F, and a 96% oxygen saturation in ambient air. He had a body mass index of 19.2 kg/m2 and was lethargic but oriented. He had no icterus or cervical or axillary lymphadenopathy. He had clear bilateral breath sounds, and his cardiovascular and abdominal examinations were unremarkable. He had a good pulse and no peripheral edema.
On admission, the patient tested positive for HIV with a CD4 count less than 20 and a high viral load. Pneumonia and meningitis resulting from opportunistic infections were considered, and he was treated for bacterial meningitis and tuberculosis (TB) even though the lumbar puncture was delayed because the patient was restless and uncooperative. The lumbar puncture results showed a pressure of 23 cm·H2O, a white blood cell count of 2, a red blood cell count of 57, a protein level of 102 mg/dl, and a glucose level of 25 mg/dl. The Gram stain and bacterial antigen tests were negative.
There was a high suspicion of pulmonary TB and tuberculous meningitis due to recent travel to Ghana, and the initial chest X-ray showed multiple nodular opacities suggestive of miliary TB (). The micronodular appearance was confirmed by a chest CT scan without contrast (). Despite a quantiFERON negative test result, quadruple therapy for TB with isoniazid, pyrazinamide, ethambutol, and rifampin (RIPE) was continued.
On succeeding days, yeast was growing in the patient's blood cultures and a Gram stain of the patient's cerebrospinal fluid (CSF) showed presence of yeast cells. The titer of Cryptococcus in the CSF was greater than 1 : 1024 and was eventually identified as Cryptococcus neoformans. Thus, antibacterial meningitis treatment was discontinued, and the patient was started on amphotericin B and flucytosine. Anti-TB medications were continued. He underwent placement of a lumbar drain due to a persistent headache and photophobia. Antiretroviral treatment was not started, but the patient was treated with prophylaxis medications for opportunistic infections. At this time, the patient had four persistently negative acid-fast bacilli sputum smears and was negative for the sputum Mycobacterium tuberculosis PCR test.
The patient underwent FFB with BAL and TBBx. In the BAL, >100,000 CFU/mL of Staphylococcus haemolyticus grew; thus, the treatment for bacterial pneumonia was modified. Tissue culture and TBBx showed the presence of Cryptococcus neoformans. Higher magnification microscopy showed the presence of both hyphae and yeast cells (Figures and ). The patient showed clinical improvement and was discharged on oral fluconazole. |
pmc-6458944-1 | Ethical approval and informed consent to report this case and its figures were obtained from the Ethics Committee of the State University of Campinas (approval number 2.878.038/ID 95776318.8.0000.5404).
A 19-year-old woman fell from a bicycle and hit her knee against a street guide. She presented with severe pain and an anteromedial skin bruise on the left knee and her knee locked in full extension ().
Radiographs showed superior displacement of the patella with its inferior articular surface engaging the proximal articular surface of the trochlea ().
We administered 2% lidocaine hydrochloride intra-articularly to treat her pain. Superior-inferior and lateral-lateral patellar manipulations were unsuccessful, and we subsequently grasped and anteriorized the patella in relation to the femur, which led to immediate reduction. A plain control radiograph was obtained following this procedure and showed complete reduction of the patellofemoral joint ().
To ensure patient comfort, the knee was immobilized for a week in an inguino malleolar orthosis at 20 degrees of flexion. Upon her return 10 days later, the patient was completely pain free and asymptomatic without any movement limitations. |
pmc-6458947-1 | A 29-year-old male was admitted to the intensive care unit with acute respiratory failure requiring intubation in the field. The patient was found unresponsive on the floor of his apartment. His medical history included morbid obesity with a body mass index of 43 kg/m2 and alcohol dependence syndrome.
On initial examination he was sedated on mechanical ventilation, afebrile (98 F), hypertensive with a blood pressure of 167/69 mmHg, tachycardic with heart rate of 111 beats per minute and a respiratory rate of 20 breaths per minute synchronous to the set rate on the ventilator. Peripheral capillary oxygen saturation (SPO2) was 98% with inspired oxygen saturation (FiO2) of 0.5. Lung examination revealed rales in the mid and lower zones of the right lung field. Cardiac examination revealed tachycardia with no murmurs. Abdominal, neurologic and skin exams were unremarkable.
Relevant laboratory on admission included the following: leukocytosis (white cell count of 11.2 × 103 cells/ /μL), serum creatinine of 2.1 mg/dL (baseline 0.6 mg/dL), serum creatine kinase of 39, 552 units/L, serum lactic acid of 4.2 mmoles/L, and serum ethanol level of 316 mg/dL. Initial arterial blood gas analysis revealed a pH of 7.26, an arterial carbon dioxide (PCO2) level of 40.2 mmHg and oxygen level (PO2) of 108 mmHg on a FiO2 of 0.5, and positive end-expiratory pressure (PEEP) of 5. Chest X-ray (CXR) showed right upper, middle, and lower lobe infiltrates and computed tomography (CT) of the head demonstrated no acute infarcts or hemorrhage.
He was started on broad spectrum antibiotics including vancomycin, piperacillin-tazobactam, and azithromycin for pneumonia. On day 2 of admission, the patient underwent fiberoptic bronchoscopy which revealed thick mucopurulent secretions in all segments of right lung. The respiratory cultures from bronchoalveolar lavage were negative as well as initial blood and urine cultures. Transthoracic echocardiogram (TTE) on day 2 revealed an ejection fraction of 57% with normal right ventricular structure and function and normal pulmonary artery systolic pressure (PASP). The patient clinically and radiologically improved, he was awake and tolerating daily spontaneous breathing trials with FiO2 requirements of 0.4 to keep PO2 of 104.
On day nine of admission, he was noted to be hypoxic with a PO2 of 52 mmHg with a SPO2 of 88%. He required a PEEP of 14 cmH20 and a FiO2 of 1 to achieve a PO2 of 66 mmHg. Due to body habitus prone position was not an option. Repeated CXR did not show any new abnormalities- resolving right-side infiltrates. In addition to the subcutaneous heparin started on admission for deep venous thrombosis (DVT) prophylaxis, a therapeutic dose of low molecular weight heparin (LMWH) at 1 mg/kg was administered with a presumptive diagnosis of acute pulmonary emboli as he was too unstable for transportation to radiology. A repeat TTE showed a PASP of 54 mmHg with new moderate dilatation of the RV and moderate tricuspid regurgitation () with a serum troponin T of 0.204 ng/mL and proBNP of 445 pg/mL. Inhaled nitric oxide (iNO) was given via the breathing circuit of the mechanical ventilator at a concentration of 20 parts per million (ppm) with improved oxygenation noted after one hour, PO2 increased to 81 mmHg with a FiO2 of 0.8. His blood pressure during this time ranged from a systolic of 117-134 mmHg and diastolic of 68–87 mmHg.
Chest CT angiogram revealed large embolus in the right main pulmonary artery (). Thrombolytic therapy with alteplase at 100 mg was administered over two hours and the iNO was slowly tapered off over the next four days with concomitant tapering of PEEP to 8 cmH20 and FiO2 of 0.45. Repeat TTE three days after thrombolytic therapy demonstrated a PASP of 32 mmHg with mild dilation of the RV without signs of strain (). The patient was weaned from mechanical ventilation and later discharged to a skilled nursing facility for rehabilitation on oral apixaban for three months with outpatient follow-up provided. |
pmc-6458953-1 | A 51-year-old female patient presented to our orthopedic outpatient department with a rapidly enlarging ulcerated right upper arm swelling which was growing gradually over the past six months. She recalled that it had been a pea-sized lesion and had remained the same size over the past 30 years. No history of preceding episodes of trauma or local irritation was found. The initially asymptomatic lesion had recently become painful and pruritic with slight bleeding. On examination, a large nodulo-ulcerative plaque was noted on the right upper arm, measuring approximately 12 cm × 6 cm in size (). Magnetic resonance imaging (MRI) revealed a mass measuring 12 cm × 6 cm × 1.5 cm, invading the deltoid and triceps brachii muscles (). Examination of the needle biopsy specimen demonstrated the proliferation of spindle cells in a storiform arrangement in the dermis. Results of tests for CD34 and factor VIII were negative, suggesting the possibility of a benign tumor. However, the clinical findings, which included muscle invasion, large tumor size, and rapid enlargement, suggested the possibility of malignancy, prompting the need for wide resection and myocutaneous flap-plasty. Intraoperatively, the tumor was found to have invaded the deltoid and triceps brachii muscles, as well as the cutaneous and subcutaneous tissue. Wide resection was achieved with a 3 cm margin. A latissimus dorsi musculocutaneous flap was needed to close the skin defect. The postoperative period was uneventful. Histopathological examination following hematoxylin and eosin stain revealed spindle cell proliferation with a vague storiform growth pattern and basal cell hyperplasia in the overlying epidermal cells (). The tumor tested negative for CD 34 on immunohistochemical staining but tested positive for SMA, CD 68, and factor XIIIa (Figures –). Mitoses were inconspicuous in the lesion, and the Ki 67 labeling index was also low, at 1 to 2%. These findings were consistent with a diagnosis of dermatofibroma. In 5 years of follow-up, the patient did not experience any recurrence, metastases, or other complications. |
pmc-6458957-1 | A 67-year-old man, who was suffering from long-lasting (>2 hours) severe chest pain, was admitted in our hospital. He was an ex-smoker (between the ages of 20 and 25 years) and had been treated for hypertension and hyperlipidemia. He was diagnosed with ITP 6 months before admission and maintained his platelet count between 10.0 × 109/L and 15.0 × 109/L by receiving prednisolone (10 mg daily) and eltrombopag (50 mg daily). He was immediately relieved of chest pain after admission, and there was no significant finding on a 12-lead electrocardiogram except a left axis deviation (). However, a cardiac catheterization was performed urgently due to the significant increase of serum myocardial enzymes (troponin-I 1.56 ng/mL, creatine kinase (CK) 233 IU/L, and CK-muscle/brain 20 IU/L). Although coronary angiography (CAG) revealed no artery occlusion (), left ventriculography showed mild hypokinesis in the posterior wall, and cardiac magnetic resonance imaging exhibited a delayed gadolinium enhancement in the posterior wall area (), suggesting myocardial infarction in the territory of the left circumflex coronary artery (LCX).
Because the patient persistently complained of chest pain even after discharge, the second CAG was performed 3 months after the discharge. CAG revealed de novo stenosis in the obtuse marginal branches (#12: 75% with haziness, ), and adenosine stress myocardial perfusion scintigraphy (Tc-99m tetrofosmin) showed myocardial ischemia in the posterior wall area (), where the tracer uptake was reduced at stress and restored at rest. Thus, the first PCI was planned for #12, and a dual antiplatelet therapy (aspirin 100 mg daily and clopidogrel 75 mg daily) was started more than 1 month before the procedure, where his platelet was kept at 27.4 × 109/L by eltrombopag (50 mg daily). The first PCI toward #12 was successfully performed by implantation of a zotarolimus-eluting stent (3.0 × 12 mm) and CAG revealed no residual stenosis (). However, the patient complained of severe chest pain with significant ST depression after returning to his hospital room (3 hours after the PCI). The emergent CAG demonstrated a stent thrombosis at the proximal edge of the stent (). Although the intravascular ultrasound imaging exhibited neither the underexpansion of the prior-implanted stent nor the coronary artery dissection around the stent, it showed new lining thrombus (white arrows) at the proximal edge of the stent (). An additional drug-eluting stent (3.0 × 12 mm, everolimus-eluting stent) was implanted at the proximal edge of the prior stent lesion of #12 (, second PCI) under the support of an intra-aortic balloon pumping. To avoid further stent thrombosis, the antiplatelet agent was changed from clopidogrel (75 mg daily) to prasugrel (3.75 mg daily). However, CAG, performed 3 days after the second PCI, revealed a massive thrombus with 75% stenosis in #12 (). Urokinase (UK 240.000 U) was selectively injected into the LCX, instead of balloon inflation for the third PCI, because recurrent coronary thrombosis was occurring at the area of the dual stent implantation. CAG after the UK injection revealed the residual stenosis (50%) in #12. The follow-up CAG, which was performed 14 days after the third PCI, again revealed the recurrence of stent thrombosis with 75% stenosis in #12 (). Subsequently, a coronary thrombus aspiration was performed as the fourth PCI. The final CAG after coronary thrombus aspiration exhibited 50% residual stenosis. Since his platelet maintained at >39.3 × 109/L by eltrombopag during all PCIs, the association between a recurrence of coronary thrombosis and inappropriate platelet increase induced by eltrombopag was suspected. Thus, we transiently ceased eltrombopag, and his platelet promptly decreased to 8.0 × 109/L after 7 days of cessations. CAG, after the cessation of eltrombopag, showed 50% of residual stenosis with thrombus; hence, we did not perform further PCI to #12 (). In addition, after he received a splenectomy to cease eltrombopag, he has not complained of any chest pain, and CAG after splenectomy revealed no thrombus in #12. |
pmc-6459035-1 | A 42-year old gentleman presented to the Emergency Department with a 2-day history of right knee swelling and pain. It was of spontaneous onset, with no history of trauma. He reported recurrent episodes of right knee swelling over the last two years. Previous right knee arthrocentesis revealed negatively birefringent crystals for which he was treated with colchicine. Other significant medical history included right knee grade 3 chondromalacia patella, asthma and ischemic heart disease.
Physical examination revealed warm, erythematous right knee joint with moderate effusion. This was associated with mild medial joint line tenderness. The range of motion of the right knee was 10° to 40° limited by pain. Biochemical investigations showed a raised total white blood cell count of 10.6x10^9/L (4.0-9.6x10^9/L), erythrocyte sedimentation rate 28 mm/hr (1-10mm/hr), C-reactive protein 60.5 mg/L (0.0-5.0mg/L) and uric acid level of 575 umol/L (250-550umol/L). Radiographs of the right knee revealed degenerative changes, with mild narrowing of the medial compartment (). No calcification within the joint was noted. A joint aspiration was subsequently performed and turbid straw-coloured was aspirated. Fluid analysis revealed nucleated cell levels of 19,900 cells/uL (reference range <200 cells/ul), and a neutrophil percentage of 91%. The fluid gram stain and cultures were negative. Microscopic analysis of the fluid revealed scanty crystals that were both negative and positive birefringence (), which was consistent with the diagnosis of gout and pseudogout respectively.
The patient was treated with physiotherapy and colchicine. Non-steroidal anti-inflammatory medication was not started due to a history of ischaemic heart disease and active asthma. The patient was prescribed paracetamol and tramadol for analgesia. Allopurinol was not given in the acute flare of gout. The patient’s pain improved, and the range of motion of the right knee improved to 0° to 160°. He was discharged uneventfully after two days of hospitalisation. |
pmc-6459040-1 | A 74-year old gentleman who underwent a left PS-TKA seven years previously defaulted his follow-up appointments after two years as he was satisfied with the TKA. He was symptom free until his current presentation of acute pain while getting out of a chair. Subsequently, the pain recurred mostly during climbing stairs. He had a history of poliomyelitis, which resulted in severe right lower limb weakness, but spared his left lower limb.
On physical examination, the left knee had a well-healed midline longitudinal scar. There was a small effusion with mild warmth, but without erythema. The range of motion (ROM) was from -3º to 110º with audible clicks when ranging the knee, compared to his last recorded ROM of 0º to 120º. There was a posterior sag sign, and the posterior drawer test was positive with no end point. His body mass index was 24.4 and he walked using a walking frame. The examination of his hips and spine were unremarkable.
Plain radiographs after the index TKA and on presentation (), as well as a computed tomography (CT) scan of the knee showed that the TKA had satisfactory alignment and acceptable femoral component positioning without any signs of loosening. The total white cell count, erythrocyte sedimentation rate and C-reactive protein levels were not elevated.
The impression was that of posterior instability, likely due to tibial post failure. The patient consented for a revision TKA. The index PS-TKA comprised Zimmer NexGen LPS [Zimmer, Warsaw, IN] cemented femoral and tibial components, with a 12mm thick PS HXLPE insert. Intraoperatively, the only significant finding was that the HXLPE tibial post had fractured at its base () and was freely floating within the notch of femoral component (). In the setting of balanced gaps, stable and well-positioned existing TKA components, and the absence of infection, the liner was exchanged with a 17mm-thick non-HXLPE insert. Intra-operative testing confirmed good stability and ROM (0º to 120º with no recurvatum).
At one-year post-operation, the knee pain had resolved and he was ambulant with a walking stick. His knee flexion range was from 0º to 120º and was stable. He had marked improvement in his Oxford Knee Score (42 from 27) and Knee Society Clinical Rating Knee Score (153 from 90). |
pmc-6459042-1 | An 11-year old girl was referred to our department with a ten-year history of difficulty in walking diagnosed as cerebral palsy (CP). The history obtained revealed that the patient was followed up regularly by paediatric neurologists for her CP, and she had multiple botulinum injections when she was 8 years old. The physical examination based on the walking analysis illustrated limited range of motion in addition to 55° of increased femoral anteversion in either hips. From the data of walking analysis, physical examination and radiological findings, the increased femoral anteversion and excessive soft tissue contractures due to CP were considered the primary reason for her walking difficulty. To improve her gait pattern, a combination of soft tissue procedure and distal femoral derotational osteotomy were planned.
Under general anaesthesia, the tendons of adductor longus, iliopsoas, semitendinosus, semimembranosus, gracilis, and gastrocnemius were released from the musculotendinous junction bilaterally. Bilateral distal femoral derotational osteotomy was then performed just above the metaphysis level with a lateral approach, and osteosynthesis was obtained by dynamic compression plate and screw fixation. Bilateral long leg casts were applied postoperatively to maintain osteosynthesis. The patient was discharged on the third post-operative day, casts were removed one month later and routine rehabilitative physiotherapy was instituted.
At one-year routine follow-up, there was complete union of the osteotomy. Although pre-operative radiographs of the right knee showed no evidence of any cystic or tumoral lesions around the distal femur (), the second-year follow-up radiographs revealed an asymptomatic large bone cyst in the distal part of the right femur. A computed tomography (CT) scan identified the lesion as a large cystic lesion located just above the lateral femoral condyle expanding from metaphysis to the femoral diaphysis involving the osteotomy site with cortical thinning (). The initial diagnosis was an aneurysmal bone cyst. To verify the diagnosis, an incisional biopsy was performed under general anaesthesia. Cortical bone was partially destroyed by the lesion and the cyst was filled with hemorrhagic material. Histopathological analysis confirmed the lesion as an ABC.
In consideration of the possible progression of the lesion and the risk of fracture associated with ABC, an operation for plate removal, curettage, and cementing procedure was performed under general anaesthesia. Histopathological examination of the curettage specimen obtained during surgery, reported as containing irregular blood-filled chambers with islands of bone and fibrous tissue, re-confirmed the diagnosis of ABC (). No recurrence was encountered 36 months after the curettage and cementation surgery (). |
pmc-6459044-1 | A 31-year old male patient presented to us with pain right knee while walking and running. He had history of a road traffic accident one-year back following which he had pain and swelling of the right knee. He had been taken to a local hospital. Radiographs were found to be normal and patient was given splintage for three weeks. After three weeks the splint was removed and knee bending started. The pain was found to have decreased but still present while walking. The patient had consulted traditional medicine practitioners and physiotherapists but the problem did not resolve with time. Finally, patient came to our hospital.
On examination in our clinic there was tenderness over the lateral femoral condyle. Range of motion at knee was full and pain free. There was no instability or clunking. Radiographs and computed tomography (CT) scan revealed undisplaced non-union of a Hoffa fracture of the lateral femoral condyle (, ). We decided to fix the fracture along with bone grafting at fracture site.
We accessed the lateral condyle femur through a lateral approach but intra-operatively we were not able to appreciate the fracture site. There was no abnormal mobility at the site where fracture was seen on the CT scan. We tried manipulating the fractured fragment but there was no movement of the fragment, instead cancellous bone started breaking. We decided that it was fibrous non-union and fixed the fracture in situ with two partially threaded screws which were countersunk (). Knee bending and full weight bearing walking was started on post-operative day one. There was no pain on knee bending or walking. At the last follow-up at two years the patient had full range of motion of the knee joint (00-1400) without any pain while walking or running. |
pmc-6459446-1 | A 49-year-old man had been receiving medical treatment for CTEPH for 3 years. Although he had undergone BPA four times previously at another hospital before admission to our hospital, pulmonary hypertension persisted. He had also been treated for nontuberculous mycobacterial infection with antibiotics and Crohn’s disease with mesalazine (3000 mg/day). Despite this thorough treatment and strict nutritional control, he often experienced high fever and melena.
When he was admitted to our institution, his anticoagulant control was not sufficient because of malnutrition and bleeding complications. Since admission, he repeatedly developed high fever and melena owing to active Crohn’s disease. Given that melena occurred more frequently after the administration of direct oral anticoagulant (DOAC), the DOAC treatment was switched to anticoagulant therapy using warfarin with strict control of the low-level prothrombin international normalized ratio (range 1.5–2.0). Subsequently, melena was relieved. Two weeks before PEA, because of frequent intestinal ileus, he was placed on liquid nutritional supplementation to avoid intestinal inflammation.
The preoperative laboratory data indicated mild inflammatory changes, moderate chronic kidney disease, low-level protein, and anemia, with the following results: white blood cell count, 3.1 × 103/μL; C-reactive protein level, 2.0 mg/dL; creatinine level, 1.8 mg/dL; estimated glomerular filtration rate, 33 mL/min/1.73 m2; albumin level, 2.8 g/dL; and hemoglobin level, 7.1 g/dL. He had no risk factors of thrombophilia. Chest radiograph showed cardiomegaly involving the right cavities and notable enlargement of the pulmonary arch (Fig. a). Abdominal radiograph showed a large amount of gas in the colon due to chronic intestinal paralysis (Fig. b). Electrocardiogram showed a right bundle branch block and signs of right ventricular hypertrophy (Fig. c). Echocardiogram showed severe left ventricular compression due to dilated right ventricle, and the estimated right ventricular pressure was 105 mmHg. Venous echosonogram showed old thrombi in both femoral veins. Computed tomography scan of the lung showed proximal chronic pulmonary emboli (Fig. a), infiltrations because of nontuberculous mycobacterial infection (Fig. b), and recent aspiration pneumonia (Fig. c). Lung perfusion scintigraphy revealed multiple segmental defects (Fig. d), and pulmonary arterial angiography showed a thickened proximal pulmonary arterial intima, intimal irregularities, and abrupt narrowing of both pulmonary arteries (Fig. e, f). Findings of the right heart catheter examination indicated severe pulmonary hypertension with the following parametric values: pulmonary artery pressure, 96/32 mmHg (mean, 53 mmHg); calculated total pulmonary resistance, 1247 dynes/sec/cm−5; and pulmonary vascular resistance, 1012 dynes/sec/cm−5.
After undergoing thorough treatments for active IBD, PEA using intermittent circulatory arrest under deep hypothermia was performed (Fig. ). PEA was performed through a median sternotomy using cardiopulmonary bypass with deep hypothermic intermittent circulatory arrest, similar to the techniques established by Jamieson et al. []. During PEA, the activated clotting time was strictly controlled between 350 and 500 s. Weaning from cardiopulmonary bypass was uneventful, and pulmonary hypertension improved dramatically to the following parametric values: mean pulmonary artery pressure, 16 mmHg; calculated total pulmonary resistance, 659 dynes/sec/cm−5; and pulmonary vascular resistance, 589 dynes/sec/cm−5. After PEA, anticoagulant therapy using warfarin was resumed with low-level control of the prothrombin international normalized ratio (range 1.5–2.5). Unfortunately, because mild melena and ileus occurred 2 weeks after PEA, he was placed on liquid nutritional supplementation again. Furthermore, the treatment for the mild mediastinitis with lavage and closure of the wound was needed.
Subsequently, the patient recovered completely and was transferred to the previous hospital to undergo surgical treatment for recurrent ileus. After subtotal colectomy, he was still doing well without any cardiovascular events during the 2-year follow-up. |
pmc-6459447-1 | A 72-year-old man was admitted to the emergency department for a hypoglycemic attack. The computed tomography (CT) scan detected a large tumor in the pelvic cavity, and he was referred to our hospital for closer examination. The patient had no relevant medical history and was not on any medication. On admission, his blood glucose level was 52 mg/dl (normal range 70–109 mg/dl); serum insulin level, 1.0 μIU/ml (normal range 3–15 μIU/ml); C-peptide level, 0.2 ng/ml (normal range 0.43–2.35 ng/ml), and IGF-I level, 31 ng/ml (normal range 58–198 ng/ml).
Contrast-enhanced CT indicated a heterogeneous spheroid mass with little contrast-enhancement measuring 13 × 9 × 11 cm in the pelvic cavity (Fig. ). CT-angiography revealed the presence of feeding vessels branching from the right and left internal iliac arteries (Fig. ). Magnetic resonance imaging (MRI) revealed a lobulated tumor with iso- and low-intensity areas combined in T1-weighted images, and iso- and high-intensity areas combined in T2-weighted images. No clear invasion to any adjacent organs was identified (Fig. ). Positron emission tomography (PET)-CT revealed heterogeneous accumulation on the tumor with a maximum standardized uptake value (SUVmax) of 2.5 (Fig. ).
Hypoglycemia was observed despite continuous glucose infusion, and glucocorticoid administration was initiated prior to tumor resection. The tumor was located retroperitoneally, fed by the superior vesical artery, and was completely excised. Operation time was 388 min, and intraoperative blood loss was 1410 ml.
Macroscopically, the tumor was solid and composed of partially necrotic grayish-white tissue; the tumor measured 15 × 8 × 8 cm (Fig. ) and had a fibrous capsule. When observed under the microscope, the tumor was composed of spindle cells arranged in no particular pattern (Fig. ). Seven mitoses were counted per 10 high-power fields (HPF). Immunohistochemical staining revealed that the tumor was positive for signal transducers and activators of transcription 6 (STAT6), CD99, bcl-2, insulin-like growth factor-2 (IGF-II), and CD34, and negative for CD31, EMA, S-100, SMA, and desmin. From these findings, we diagnosed this tumor as an SFT with malignant potential that secreted IGF II, which caused hypoglycemia (Fig. ).
The postoperative course was uneventful, and hypoglycemic symptoms disappeared immediately after surgery. There has been no tumor recurrence during the 1 year of follow-up. |
pmc-6459455-1 | A 73-year-old woman had been followed for giant hepatic cyst occupying the right lobe of the liver with a maximum diameter of 20 cm since 2005. Her medical history included a benign tumor in the transverse colon and an unruptured cerebral aneurysm. She had no remarkable family history.
During the follow-up for the cyst, a dorsal unenhanced mural nodule was noted within the cyst wall on computed tomography (CT) in 2008, but no change in either the morphology or size was detected until 2016 (Fig. a, b). Another ventral mural nodule newly appeared in 2016. Enhanced CT showed that the ventral mural nodule was 25 mm in diameter with weak enhancement in the early phase and centripetal prolonged enhancement in delayed phase (Fig. c, d). Magnetic resonance imaging (MRI) showed that the cyst content had a high signal intensity on both T1- and T2-weighted imaging (WI), and the ventral nodule had low signal intensity on T1WI and partially high signal intensity on T2WI (Fig. a, b). In addition, the ventral nodule showed partially strong high signal intensity on diffusion-weighted imaging (DWI) (Fig. c) and had a low apparent diffusion coefficient (ADC) value (ADCmean 0.6 × 10−3 mm2/s) (Fig. d). Fluorodeoxyglucose-positron emission tomography (FDG-PET) showed a weak abnormal uptake in the ventral nodule with a maximum standardized uptake value (SUVmax) of 2.3 (Fig. e). Furthermore, the tumor markers CA19-9 and CEA were elevated (171 U/ml and 7 ng/ml, respectively). Considering possible malignancies such as biliary cystadenocarcinoma, she was referred to us for surgery, and surgical resection was planned for diagnostic and therapeutic purposes.
We performed laparoscopy-assisted extend right hepatectomy. The surgical procedure was as follows: Under general anesthesia, a 7-cm upper midline incision was made and the cyst content was trans-hepatically aspirated (Fig. a). After ensuring that there were no malignant components in the cyst content by intraoperative cytology, a total of 3 L of brown serous fluid was sucked out using a 16-G elaster needle. We then continued to perform surgery as a negative result on cytology did not deny the possible existence of biliary cystadenocarcinoma []. The laparoscopic procedure was initiated via pneumoperitoneum with an additional four ports. First, we performed cholecystectomy and encircled the hepatoduodenal ligament (preparation for the Pringle’s maneuver). Next, laparoscopic adhesiotomy between the cyst wall and right diaphragm was performed followed by careful mobilization of the right lobe, being sure not to rupture the cyst wall (Fig. b). After preparing for a hanging maneuver (Fig. c), parenchymal division was performed under direct vision through the small laparotomy wound using a hanging maneuver and the Pringle’s maneuver (Fig. d) with the Glissonean pedicle approach. Finally, the specimen was carefully excised (Fig. e). No intraoperative complications were observed. The operative time was 673 min, and operative blood loss was 1540 ml. The postoperative clinical course was uneventful, and she left the hospital on postoperative day 13.
Macroscopically, there was a dark-red solid protuberance in the cyst wall (left side of Fig. a), consistent with the ventral enhancing mural nodule. A histological examination showed that the cyst wall mainly consisted of thick fibrous stroma and the mural nodule consisted of blood-filled dilated vessels lined by endothelial cells within the organized hematoma, mimicking cavernous hemangioma (Fig. b, c). Immunostaining of the dilated vessels showed positivity for CD31 (Fig. a) but negativity for α-smooth muscle actin (α-SMA) (Fig. b) and Elastica van Gieson (EVG) (data not shown), and murine double minute (MDM) 2 (Fig. c) and p53 (data not shown), markers for liver angiosarcoma. The Ki-67 labeling index was 19.0% (Fig. d). Therefore, we pathologically diagnosed the patient with HHC with neovascularization within the organized hematoma. On the other hand, because the dorsal mural nodule could not be detected macroscopically, we evaluated a thickened portion of the cyst wall where the dorsal mural nodule seemed to have originally existed (right side of Fig. a). The thickened portion consisted of malformed veins of various sizes (Fig. d, e) within a calcified hematoma. These veins were immunohistochemically positive for α-SMA (Fig. e) and EVG (data not shown). She has survived for 2 years without any evidence of recurrence after surgery. |
pmc-6459488-1 | A 26-year-old female (born 10 September 1988), who lived in Longhu Village, Jishan Town, Nanling County, Wuhu City, Anhui Province of China, an endemic county of schistosomiasis, was complaining of abdominal pain and distension for over 1 month, thus going to the local schistosomiasis-specialized hospital for diagnosis and treatment on 13 Oct 2014. Her diagnosis, treatment, and follow-up process is shown in . An abdominal ultrasonography was performed, indicating a mildly enlarged liver with severe fibrosis and a huge spleen. And blood routine showed thrombocytopenia. Due to her symptoms (abdominal pain and distension) and blood test results (a reduction in the platelet count) as well as contact history (living in the endemic area of schistosomiasis), she was suspected to have advanced schistosomiasis and might be in need of surgery as a result of splenomegaly, and thus was referred to the First Affiliated Hospital of Wannan Medical College (Anhui, China) for further diagnosis and treatment. She was admitted to the hospital on 21 Oct 2014 because of liver fibrosis and hypersplenism. Blood routine test showed mild anemia (HB 104 g/L) with decreased leucocytes (white blood cell [WBC] 1.7 × 109/L) and thrombocytes (platelet [PLT] 30 × 109/L). Bone marrow aspiration and biopsy revealed trilineage myelodysplasia. Ultrasounography demonstrated an enlarged liver with severe fibrosis (characteristic fish-scale pattern caused by schistosomiasis), the size of the left lobe of which was 82 × 71 mm and the size of right lobe was 117 mm with an 18-mm–width portal vein (>13 mm considered abnormal, suggesting portal hypertension). Esophagography indicated mild esophageal varices probably resulting from portal hypertension. The length of spleen was 165 mm (>120 mm considered abnormal) while the size was 78 mm (>40 mm considered abnormal).
Hepatitis virus tests were negative (HBV and HCV), and she was a nondrinker and denied long-term use of medications. Despite the fact that no egg was observed in the fecal specimen through microscopic examination, anti-SEA (soluble egg antigen) antibody was positive, which helped to make a clinical diagnosis of advanced schistosomiasis. With the purpose of alleviating portal hypertension and hypersplenism, a splenectomy was performed as well as a liver biopsy on 27 Oct 2014. The section of spleen showed chronic congestive splenomegaly while the section of liver revealed disorganized lobular structure and proliferation of fibrous tissue together with calcified eggs deposition, which provided pathological evidence for diagnosis of advanced schistosomiasis. After operation, blood routine test was performed every 1 to 3 days. A high WBC count (23.8 × 109/L) was observed on 28 Oct 2014, but it decreased steadily and become normal (9.5 × 109/L) on 6 Nov 2014. Whereas, PLT count increased apparently, from 82 × 109/L on 28 Oct 2014 to 887 × 109/L on 6 Nov 2014. Therefore, a low dose of aspirin (0.1 g per day) was prescribed to prevent blood clots at discharge on 6 Nov 2014, and regular blood routine test was recommended to monitor platelet count.
She was annually followed up by the local schistosomiasis-specialized hospital because she was entitled to the medical insurance supported by government finance as an advanced schistosomiasis patient. On her first follow-up in 2015, platelet count (362 × 109/L) was nearly normal, and a high gamma-glutamyl transferase (GGT) level (91 U/L, normal range 7–50 U/L) was detected as well as severe liver fibrosis seen from the ultrasonography, and thus glutathione and diammonium glycyrrhizinate were prescribed to protect the liver. On her second follow-up in 2016, both blood and liver routine tests were normal, and the liver fibrosis condition improved to moderate, and therefore she continued to be prescribed liver protection drugs such as glutathione. The patient was suggested to have an abdominal ultrasound examination once a year. Ultrasonography of the liver revealed severe to mild fibrosis (Grade 3 to 1) from the admission examination in 2014 to the third follow-up examination in 2018 (, ), indicating the positive effect of the surgery that facilitates the flow of blood so it is less likely to pool in the portal system. The liver is mildly enlarged in size, and its left lobe is shrunk with length dropping from 82 mm to 70 mm and thickness from 71 to 52 mm, though it is still a little oversized, whereas only a small decline was observed in the size of the right lobe (from 117mm to 109 mm). |
pmc-6459944-1 | A 70-year-old male was diagnosed with Merkel Cell carcinoma of the right thigh (). The patient underwent surgical resection with close (<1 mm) surgical margins in 2012. Surgical pathology showed the tumor to be CD56, synaptophysin, and pancytokeratin positive by immunohistochemistry, with involvement of a single right inguinal lymph node. Due to close surgical margins and inguinal nodal involvement, the patient received adjuvant intensity modulated radiation therapy (IMRT) to the right thigh and right pelvic and inguinal nodal region to a dose of 50 Gy delivered in 25 fractions (), as well as consolidative carboplatin and etoposide post-operatively. The patient then developed a radiation in-field recurrence involving the right external iliac and inguinal nodal region in 2015. Salvage right pelvic nodal dissection was performed where 15 nodes were removed with four harboring metastatic Merkel cell carcinoma.
Shortly after locoregional recurrence in the right pelvic nodal region, the patient developed metastatic disease involving bilateral pelvic nodes and the retroperitoneum on PET-scan (). Biopsy of the SUV-avid retroperitoneal lymphadenopathy confirmed metastatic Merkel cell carcinoma. The patient was treated initially with doxorubicin and cyclophosphamide and due to progressive disease was transitioned to single-agent pembrolizumab (200 mg IV every 3 weeks). After 5 months of therapy (7 cycles of pembrolizumab) the patient had a complete metabolic response with only sub-centimeter lymph nodes visible on CT. After 10 months of pembrolizumab (15 cycles), the patient had an isolated left inguinal and external iliac nodal progression (). For this reason, the patient was treated to the involved left inguinal and left external iliac nodes with IMRT to a dose of 45 Gy delivered in 25 fractions with concurrent pembrolizumab (), without any acute radiation side effects. Thereafter, the patient had complete imaging response of the left pelvic nodes as well as all other sites of metastatic disease outside of the radiation field at 3 months post-radiation by PET-response criteria in solid tumors (PERCIST) criteria (). At the present time (20 months post-radiation), the patient has received 39 cycles of pembrolizumab without evidence of cancer on serial imaging with mild rash as the only immune-related side effect. |
pmc-6460374-1 | A 70-years-old Caucasian man presented in April 2016 with a pigmented lesion of about 1 cm located in the temporal limbus of the left bulbar conjunctiva (). Patient was in good clinical conditions; the Eastern Cooperative Oncology Group Performance Status (ECOG PS) was 0. No relevant comorbidities or concomitant medications existed. The patient was an officer employee and had no familiarity for neoplasms nor environmental risk factor exposure. The patient underwent a complete escissional biopsy of the bulbar conjunctiva. The histological analysis demonstrated a conjunctival melanoma with a thickness of 0.3 cm. The extracted DNA was amplified with specific primers for exon 15 of BRAF gene and then sequenced. Codon 600 was also analyzed through Idylla™ BRAF mutation test. The heterozygote mutation of codon V600 was detected, but the specific amminoacidic substitution was not identified due to the scarcity of histological tissue.
In July 2017, the patient noticed a tumefaction of the right parotid region. The 18fluoro-desossi-glucose (FDG) positron emission tomography (PET)/computerized tomography (CT) scan showed an uptake in a nodule of the right parotid gland (ø 23 mm) and latero-cervical lymph node metastases with a maximal standard uptake value (SUV) of 4.4. The fine needle aspiration cytology (FNAC) revealed melanoma cells. Therefore, the patient underwent a right parotidectomy with latero-cervical lymph node dissection. The histologic exam confirmed the parotid metastasis from conjunctival melanoma. Metastasis of melanoma was also detected in 1/13 lymph nodes. There was no evidence of extra nodal invasion. The extracted DNA was amplified with specific primers for exon 15 of BRAF gene and then sequenced. V600E mutation (T1799A) was detected.
The 18FDG PET scan after intervention (October 2017), revealed a residual tumor in the lymph nodes located in left retro-mandibular and latero-cervical areas (maximal SUV 3.9) and a nodule in the mesenteric adipose tissue in right hypogastrium (). LDH was 700 IU/L (ULN 450 IU/L). Other cases of BRAF mutant conjunctival melanoma experienced a disease control with BRAF inhibition therapy (). In metastatic cutaneous melanoma, the combination of anti-BRAF and anti-MEK demonstrated better survival results vs. anti-BRAF alone. Indeed, we hypothesized that the target combination therapy could be the best option also for this BRAF mutant conjunctival melanoma patient. The combination of these drugs is not approved for conjunctival melanoma, thus we requested the off-label use in accordance with our institutional policy. After the institutional permission, the written consent was obtained by the patient. Then we started therapy with Dabrafenib (150 mg twice daily) and Trametinib (2 mg daily), choosing the standard dosage approved for metastatic cutaneous melanoma ().
Vital signs, dermatological exam and biochemistry analysis had been performed monthly. The therapy was complicated by fever with body temperature between 37.5 and 38°C. No signs of infections were detected. Patient took paracetamol for the fever and the adverse event resolved without squeal after 2 months. No dose reduction or interruption was needed.
The first 18FDG-PET/CT scan after 4 months of therapy revealed the persistence of lymph node disease (with increased maximal SUV to 4.4), while the mesenteric nodule was stable (). The cranial CT scan with contrast enhancement did not reveal any sign of tumor recurrence. The combination of Dabrabenib/Trametinib was continued.
The following 18FDG-PET/CT scan was performed after further 4 months of therapy. It showed the reduction of the metabolic activity of the lymph node metastases of the neck (maximal SUV 3). The mesenteric nodule was not modified (). The magnetic resonance imaging (MRI) of the brain did not reveal ocular recurrence of melanoma nor encephalic metastases.
Ophthalmic examinations have been performed every 6 months, and they detected no signs of uveitis or iridocyclitis. The patient never complained of photophobia, ocular pain, or other visual alterations.
The blood analysis performed in August 2018 revealed a grade 1 hypertransaminasemia.
On September 2018, the treatment with dabrafenib and trametinib is ongoing at the initial dose. No dose reduction was necessary. |
pmc-6460500-1 | An 80-year-old man was referred to the radiology department because of a persisting palpable mass in the left breast. Family history of breast cancer was negative. The mammography revealed a retro-areolar mass in the left breast (Figure ). There were no microcalcifications. Ultrasound of the left breast showed predominantly enlarged mammary ducts, apparently caused by an ill-defined hypo-echogenic retro-areolar mass of 2.8 cm in diameter (Figure ). An ultrasound-guided needle core biopsy of the retro-areolar mass was performed. Histology revealed a primary diffuse large B-cell type lymphoma of the breast. The patient had no prior history of lymphoma. Additional PET–CT showed a metabolic active lesion in the left breast (Figure ). Widespread disease was not present. |
pmc-6460545-1 | A 44 year-old pregnant nulliparous, weight 70 Kg, height 172 cm, BMI 23.7, at 34 weeks of a twin gestation, obtained by “in vitro” fertilization, was admitted to the General Hospital of Massa, complaining spreading pricking and lower limb edema. The patient suffered from unstable insulin-dependent type I diabetes and sciatica. Considering 4 days of immobilization in bed, low molecular weight heparin (Dalteparin© 2500 UI one per day) was administered []. The patient underwent a planned caesarean section under double-space CSE anaesthesia, using a 25-gauge atraumatic spinal needle at level L3-L4 and an 18-gauge Thuoy needle at T12-L1 (B. Braun Perifix® epidural set), placed during a single attempt. Preoperative coagulation parameters were within normal range (Platelet count 120.000 per microliter of blood; Prothrombin time 12 s, Partial thromboplastin time 30 s, fibrinogen 540 mg/dL, INR 0.90). Renal function was normal. The operation was carried out routinely and multimodal pain therapy was started (Patient Controlled Epidural Anaesthesia - PCEA - with chirocaine 0,15% plus sufentanil 0,5 mcg/ml, 4 ml/h; i.v. ketorolac 30 mg/day and oral tramadol 30 mg plus acetaminophen 1000 mg/3 times/day). Four hours after the operation, the urinary catheter was removed, the patient began to stand up and to take care of the newborns. Dalteparin 2500 U/die was continued. PCEA was discontinued 2 days after the operation and the epidural catheter was removed on the 3rd day, 12 h after the last Dalteparin administration. Pain control was optimal. The following day, the patient remained hospitalized without any complications and the two babies were admitted in neonatology.
At 06.00 a.m. of the 6th post-operative day, more than 70 h after the removal of the epidural catheter, the patient complained of acute and severe low-back pain, radiating to the right inferior limb, and paresthesia; there was no motor impairment and she was treated with analgesic drugs without any improvement. After an abdominal ultrasound, urinary retention was observed and a bladder catheter was inserted (residual volume: 1100 ml). A right lower limb motor deficit was observed at 11:50 a.m. and methylprednisolone was administered. Since no improvement in the patient’s clinical picture was seen, urgent neurological consultation was requested 8 h after the onset of symptoms (02.00 p.m.). A dorsal MRI scan showed the presence of a T12-L1 posterior SEH, predominantly on the right side, with significant mass-effect and spinal cord signal alteration in the conus medullaris region (Figs. and ). The patient was transferred to the Neurosurgical Department for decompression laminectomy and removal of the hematoma and the operation began at 07.00 p.m.. Immediately after surgery, the patient partially recovered her sensory disorder and motor functions. Five days after surgery she began intensive rehabilitation.
In the last follow-up examination (36 months later), while mostly improved, motor, sensory and sphincter deficits persisted in varying degrees. The last MRI confirmed signs of permanent ischemic injury at level T12-L1. |
pmc-6460677-1 | A 56-year-old man with no history of tobacco use or alcohol consumption presented with a three-month history of an enlarging left-sided neck mass and worsening headaches. A positron emission tomography/computed tomography (PET/CT) showed an [18 F]fluorodeoxyglucose FDG-avid soft tissue density at the left tongue base measuring approximately 1.8 × 2 cm, a centrally hypodense hypermetabolic left level IIB nodal conglomerate measuring 3.6 × 4 cm, and multiple bilateral hypermetabolic cervical lymph nodes, without evidence of distant metastasis. Brain MRI was negative for brain metastasis.
The patient underwent a core biopsy of the left neck level II node which read as a poorly differentiated neuroendocrine carcinoma consistent with small cell carcinoma.
Core biopsy of the left neck level II node revealed sheets of malignant cells with small to intermediate-sized nuclei, indistinct nucleoli, and scant cytoplasm consistent with SCC. The tumor exhibited areas of necrosis as well as abundant mitotic figures and apoptotic bodies. The neoplastic cells were positive for cytokeratin AE1/AE3, synaptophysin, p16, and TTF-1 with a nuclear staining pattern; they were negative for cytokeratin 5/6, CAM 5.2, p63, chromogranin, CD56, and EBV (by in-situ hybridization) (Fig. ).
The tumor was positive for p16, but the combined morphologic and immunophenotypic features argued against conventional HPV-associated OPSqCC. Polymerase chain reaction demonstrated that the tumor was positive for HPV16, negative for HPV18, 31, 33, 35, 39, 45, 51, 52, 56, 59, 66, and 68.
The tumor was staged T1N2cM0 (stage IVA). A percutaneous endoscopic gastrostomy tube (PEG) was placed before the beginning of treatment to meet his nutritional and hydration needs during treatment. He received four cycles of chemotherapy at 21 day-intervals. The chemotherapy regimen consisted of cisplatin 75 mg/m2 on day one and etoposide 80 mg/m2 on days one to three. On cycle two, day eight, he started radiotherapy to the oropharynx and involved neck nodes. He received a dose of 70 Gray (2 Gy/fraction) over a seven week-period. During the concomitant phase of chemo-radiation, the patient experienced grade IV mucositis, grade II nausea, and dehydration for which he received additional outpatient fluid and electrolyte replacement. Due to grade III neutropenia, the dose of cisplatin and etoposide was reduced by 25% during the last cycle of chemotherapy.
Three months after completion of therapy, a PET/CT showed complete resolution of the tumor and metastatic lymph nodes along with and no evidence of distant metastasis (Fig. ). He also had complete resolution of his mucositis and was able to resume a full oral diet resulting in removal of the PEG tube. |
pmc-6460723-1 | An 11-year-old male, with a diagnosis of pathological demand avoidance (PDA) and migraine, presented to a community optometrist with a 2-day history of a black spot in the central vision of his right eye. The optometrist reported that the best corrected visual acuity (BCVA) in the right eye was reduced to 6/10 Snellen having been normal at a prior visit. Left was 6/5 Snellen. New pigmentary changes at both macula were observed by the optometrist and referral was made to the hospital eye service (HES). He was taking pizotifen prescribed for migraine. There was no relevant past ocular, medical or family history. Six weeks later, in the HES review, he described a persistent ‘blur’ in the central vision of the right eye. Unaided VAs were 6/9 right and 6/5 left. Pigmentary changes were noted at the central macula in both eyes. SD-OCT and imaging revealed bilateral outer lamellar layer defects (Fig. ). Electrodiagnostic tests were normal but with limited co-operation. His mother accompanied him for all HES visits. In due course and following direct questioning he admitted to constructing a device made from Lego™ consisting of a laser pointer with a condensing lens used just prior to the onset of visual symptoms. The patient’s mother revealed that she had purchased the laser pointer online for him. During follow-up he reported symptomatic improvement and the VA remained stable. Centre involving structural defects at both macula persisted on clinical examination and OCT imaging to most recent follow-up 24 months later. (Supplementary Image ). The mother confirmed the laser had been purchased from a well-known UK online retailer and was still available for online purchase a year following the incident. |
pmc-6460723-2 | A 13-year-old male with attention deficit disorder (ADD) presented to the Emergency Medicine Department accompanied by his mother complaining of visual disturbance after staring into the beam from a toy laser for a few hours earlier that day. The patient stated that the toy laser belonged to a friend but the injuries were self-inflicted. The BCVA was 6/60 in the right eye improving to 6/36 with pinhole, and 6/12 in the left eye. SD-OCT images on presentation showed full-thickness hyper-reflective damage involving both fovea (Fig. ). The patient was on methylphenidate 57 mg daily treatment for ADD and was known to Child and Adolescent Mental Health Services (CAMHS). He attended mainstream school with additional classroom support but was not classified as having special educational needs, with no statement of educational needs undertaken previously. Six weeks later, his BCVA had improved to 6/12 right and 6/9 left. An improvement in SD-OCT images was observed, notably an improvement in inner retinal layers. However, the centre involving outer lamellar layer defects on OCT and fundus changes persisted throughout 24 months of follow-up but decreased. |
pmc-6460723-3 | A 15-year-old female with no past medical or psychological history was referred following a routine sight test where new discrete pigmentary changes at the right fovea were observed. The patient was asymptomatic. Her past ocular history, medical and family history were unremarkable. The unaided VA was 6/7.5 in both eyes. Two full-thickness centre involving round scars at the right fovea were observed and a third slightly eccentric. SD-OCT revealed defects in the ellipsoid zone in the outer retina in these lesions (Fig. ). The patient admitted to being involved in a ‘competition game’ with three other children about 2 years previously in the home. The ‘game’ consisted of ascertaining which child could withstand a green laser beam in one eye for the longest time. She recalled shining the laser into her right eye for short duration, perhaps 10 s twice. The laser pointer had been purchased online by the patient’s mother.
The laser pointer responsible was retrieved from the family and sent for analysis. The analysis found the laser pointer was of wavelength 532 nm with an average power of 47 mW, making it a Class 3B laser. The label on the laser pointer incorrectly stated that it was 'Class II' with a maximum output less than 1 mW (Supplementary Image ). At latest follow-up, at 24 months the macular changes persisted with 6/6 Snellen in each eye (Supplementary Image ). |
pmc-6460723-4 | A 12-year-old boy was referred with a several month history of reduced vision in both eyes. He had a history of expressive and receptive language impairment and was attending a specialist school for children with cognitive impairment and disturbed behaviour. He was under CAMHS for anger and behavioural problems. There was no relevant past ocular or family history. The presenting BCVA were 6/30 right eye and 6/75 left eye. Colour vision was reduced, with only 4/17 Ishihara plates correctly identified in the right eye and 9/17 in the left eye. Bilateral multifocal macular pigmentary changes were noted (Fig. ). To investigate abnormal visual function, electrodiagnostic tests and magnetic resonance imaging (MRI) of the brain and orbits were performed, both of which were normal.
At subsequent follow-up and on direct questioning, the boy revealed he had been playing with laser pointers at school, particularly in games involving looking directly into the beam of the laser pointer. His BCVA at 12 months follow-up was 6/19 right eye and 6/48 left eye. Fundus examination showed irregular pigmentation at the right macular and a scar at the left macular. OCT scan showed small, round, punched-out lesions more frequent in the left than the right macula. |
pmc-6460731-1 | The proband (III:1) is a 58-year-old woman with paresthesia and tendency to muscle cramps in the lower legs and feet.
She was referred to a neurologist at the age of 56 because of a sensation of warmth/cold in the lower legs and tendency to cramp in the lower legs, especially the feet. Neurological examination was normal. She was born with atrial septal defect (ASD), has had rheumatoid arthritis since the age of 28 years, has hypercholesterolemia, and recently she was diagnosed with anxiety.
MRI of the brain showed bilateral calcifications in the globus pallidus, putamen, thalamus and nucleus dentatus, as well as diffuse white matter lesions in both cerebral hemispheres consistent with chronic ischemia (leukoaraiosis). Cerebral computerized tomography (CT) scan also showed bilateral calcifications in the basal ganglia and cerebellum, and distinct periventricular leukoaraiosis.
The probands mother (II:2), have paroxysmal atrial fibrillation, and was admitted to hospital at the age of 74 years suspected of having transient ischemic attack. She presented with sudden blindness on both eyes and dizziness, lasting for about 3–4 min. Neurological examination showed decreased vibratory sensation, and the Achilles reflexes was absent bilateral. MRI of the brain showed distinct leukoaraiosis due to ischemic demyelination. CT scan was not performed.
The monozygotic twin sister of the proband (III:2) was admitted to hospital at the age of 49 years because of paresthesia on the left side. Since her mid-fifties she has had sore muscles in all four extremities. At the time of diagnosis, she suffered from restless legs syndrome with an urge to move the legs, unpleasant sensations in her legs and sometimes in the hands as well, totally relieved by the movement. Neurological examination was normal except slight insecurity at Rombergs test. A CT scan showed bilateral calcifications in the basal ganglia and cerebellum, extensive periventricular leukoaraiosis. Magnetic resonance imaging (MRI) showed white matter lesions in both cerebral hemispheres and the brain stem. She was also born with ASD. The two monozygotic twin sisters are concordant with respect to the age of onset.
A younger sister (III:3), who suffers from epilepsy, was admitted to hospital at the age of 48 years because of transient dizziness and hemiparesis at the right side. Both CT scan and brain MRI showed bilateral calcifications in the basal ganglia and cerebellar areas, as well as distinct leukoaraiosis.
The probands youngest sister (III:4) is clinically asymptomatic. Nevertheless, she has bilateral calcifications in the basal ganglia and in the nucleus dentatus on her CT scan (Table , Fig. ).
DNA from the proband and her mother was extracted from EDTA-stabilized peripheral blood lymphocytes and subjected to exome capture using NimbleGen SeqCap EZ MedExome (Roche), followed by sequencing on an Illumina NextSeq550 platform to a mean coverage of 140x, with 95% of targeted bases covered with minimum 30x coverage. Raw reads were aligned using the Burrows-Wheeler Alignment tool v. 0.7.15 [] and the GATK (Genome Analysis Toolkit) Best Practice pipeline v. 3.8–0 was used for variant calling []. Annotation and filtering of variants was performed using VarSeq 2.0.2 (Golden Helix). The sequence variant has been confirmed by bidirectional Sanger sequencing of PDGFRB (NM_002609.3) according to standard procedures.
Whole exome sequencing on DNA from the proband and her mother, and bidirectional Sanger sequencing of PDGFRB in III:2, III:3 and III:4, showed heterozygosity for a novel sequence variant, c.1834G>A; p.(Gly612Arg) in PDGFRB. The sequence variant is located in exon 13 within the tyrosine kinase domain [Table ], and can be classified as likely pathogenic class 4 variant according to the American College of Medical Genetics and Genomics and the Association for Molecular Pathology []. A CADD score of 29.5 further support the pathogenicity of the variant [].
The clinical findings in the present family are compatible with the phenotype associated with variants in PDGFRB (Fig. , Table ). |
pmc-6460740-1 | A 49-year-old male was admitted to our hospital in November 2012, with a two-month history of a sore throat and difficulties in swallowing. The oropharyngeal inspection revealed the presence of an ulcerated mass of the right tonsil extending to the base of the tongue, to the epiglottis and to the right pyriform sinus. In addition, bilateral neck lymphadenopaties were palpable. A biopsy was performed and an HPV- negative squamous cell oropharyngeal carcinoma was diagnosed. The patient was a heavy smoker (20 packs/year) but denied alcohol intake. Head and neck computed tomography (CT) scan and whole body fluorodeoxyglucose positron emission tomography/computed tomography (FDG PET/CT) scan confirmed the presence of tumor in the right tonsil area with bilateral lymph nodes involvement in levels I-III. The largest nodes measured 2-4 cm in maximum diameter. No distant metastases were found. The TNM clinical stage was cT3N2c. Following a multi-disciplinary discussion, the patient was judged suitable for concomitant chemoradiotherapy (CRT) and underwent protective tracheostomy; then, from April to May 2013, he was treated with a combination of intensity-modulated radiation therapy (IMRT) for a 70 Gy total dose, and weekly cisplatin for a total dose of 280 mg/m2.
The course of treatment was poorly tolerated, due to hematologic toxicity (grade 2 neutropenia) and grade 4 oropharyngeal mucositis, eliciting severe pain and requiring the placement of a percutaneous endoscopic gastrostomy (PEG) feeding tube. One month after the end of concomitant CRT, the patient’s clinical conditions slowly improved.
The CT scan performed at the end of treatment was negative, and a right tonsil biopsy did not reveal any residual cancer cell.
In July 2013, the patient was hospitalized because of the sudden onset of dysphagia, oral pain and extensive oral mycosis. Fiberoptic endoscopy revealed erosions and ulcerations of the tracheal, hypopharyngeal and esophageal mucosa, with fungal colonization. Biopsies confirmed the presence of Candida species hyphae and the absence of cancer cells, suggesting the diagnosis of a post-radiation tracheo-esophagitis with fungal colonization.
A few days later the patient, still in treatment with analgesic and antifungal drugs and fed through the PEG, reported a stabbing right hemithoracic pain. A plain chest X-ray showed the occurrence of a spontaneous pyo-pneumothorax that was treated by pleural purulent fluid drainage and broad-spectrum antibiotics. During the following days, as a persistent discharge of dark purulent fluid from the pleural drainage was noted, enteral nutrition reflux from the stomach to the esophagus, and then along the upper airways to the mediastinum and the pleural cavity, was suspected, possibly due to the formation of a tracheo-mediastino-pleural fistula. The diagnosis was confirmed by a CT scan (Fig. ) and the fistula was treated with the endoscopic insertion of a silicone stent.
In October 2013, a total-body CT scan was performed; it showed no evidence of malignant disease and the resolution of the fistula. Two months later, the patient was urgently hospitalized for loss of consciousness secondary to hypotension. Anemia (hemoglobin 8 g/dL), probably caused by a minor oropharyngeal bleeding from the tracheostoma, was observed in blood tests. A thoracic CT scan, the electrocardiogram and a two-dimensional echocardiogram (ECHO) revealed normal findings with the exception of the presence of sinus tachycardia. The patient died suddenly two days after hospitalization.
The autopsy revealed sparse tumor cells in the oropharyngeal and tracheal mucosa, a minimal residual tracheo-esophageal fistula, and a large number of epicardial and myocardial metastases from oropharyngeal squamous cell carcinoma (Figs. , and ). |
pmc-6460839-1 | A 64 year-old Caucasian female former smoker (4 pack-year) originally presented to the emergency department with painless jaundice. Physical exam revealed an afebrile female with scleral icterus and jaundice. Her abdomen was soft, non-tender, and non-distended in all quadrants with normal bowel sounds and no organomegaly. CT imaging demonstrated a large (10 cm) retroperitoneal mass, necessitating biliary stenting. Fine needle aspiration of the mass revealed a CD10+clonal B cell population by flow cytometry, consistent with presumptive B cell lymphoma. During the staging workup for the lymphoma, right-sided cervical level IIA and III lymphadenopathy was found incidentally during a routine dental check-up, which was initially thought to be of the same disease process. She had no supraclavicular or axillary lymphadenopathy. CT demonstrated right level II/III LN and possible right base of tongue (BOT) mass. Flexible laryngoscopy revealed an exophytic mass involving the right BOT that extended to the right glosso-tonsillar sulcus and beyond the midline measuring approximately 3 cm (Fig. ). Excisional biopsy of two right cervical lymph nodes unexpectedly demonstrated squamous cell cancer (SCC) that was positive for p16 and HPV. Subsequently, positron emission tomography/computed tomography (PET/CT) demonstrated an FDG-avid right BOT mass (2.3 × 0.9 cm) with right-sided level IIA, IIB and III lymphadenopathy (all < 3 cm), consistent with biopsy-proven HPV-associated SCC (Fig. ). There was also an intensely FDG-avid retroperitoneal mass (8.2 × 13.4 × 10.7 cm) along with left mesenteric, left periaortic, and left retroperitoneal lymph nodes (Fig. ). Laparoscopic biopsy of gastroepiploic, mesenteric, and gastrocolic lymph nodes confirmed follicular lymphoma. Pathology showed relatively low number of centroblasts (fewer than 15 per high power field) compatible with low grade follicular lymphoma (WHO grade 1–2) with significantly elevated Ki-67 proliferation index (~ 80%) suggesting clinical behavior similar to WHO grade 3 follicular lymphoma. Omentum and liver were not involved. Therefore, a diagnosis of synchronous stage IV T2N2bM0 HPV+ SCC of right BOT and stage IIAX follicular lymphoma was made. At the time, she was relatively asymptomatic from the BOT cancer. She denied dysphagia, odynophagia, trismus, otalgia, or speech or voice change. She also denied night sweats, fevers, significant weight loss, or infectious symptoms. Videofluoroscopic swallow study evaluation was normal. ECOG performance status was 1. After stenting, the patient’s bilirubin normalized and she was asymptomatic. Her case was discussed at multidisciplinary case conferences, and the initial plan was to treat the BOT cancer first due to its likely curability and shorter treatment course.
One month later, however, the patient was admitted to the hospital because of worsening abdominal pain. Given concern for lymphoma becoming increasingly symptomatic, R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy was initiated. She tolerated therapy well and had resolution of abdominal pain. After completion of 3 cycles of R-CHOP, PET/CT scan demonstrated interval markedly decreased size and uptake of the retroperitoneal mass, as well as interval resolution of the FDG-avid BOT lesion, and most of the FDG-avid cervical lymph nodes (Figs. and ). She had no symptoms referable to lymphoma at this time. Nasopharyngolaryngoscopy also revealed no residual fullness in the area of the right BOT (Fig. ). After completion of another 3 cycles of R-CHOP (in total 6 cycles), PET/CT scan demonstrated sustained metabolic resolution of the abdominal mass. However, FDG-avid right BOT lesion as well as right cervical level II and III nodes had become slightly more prominent compared to the end of cycle 3. The decision was made to start 7-week concurrent chemoradiation with weekly cisplatin 40 mg/m2 for the SCC. Unfortunately, she was found to be neutropenic and cisplatin was switched to cetuximab. At the end of the first cetuximab infusion, she developed a Grade 3 infusion reaction with rigors and chest pain and was diagnosed with NSTEMI. An attempt to re-initiate cisplatin treatment after ANC normalized was unsuccessful as patient experienced fever and altered mental status necessitating hospital admission. She had received a total of one dose of cetuximab and two doses of cisplatin before the decision was made to proceed with radiation therapy (RT) without further chemotherapy. In total, the patient received 6996 cGy, 212 cGy per day in 33 fractions with coverage of the oropharynx and bilateral neck using tomotherapy-based image-guided intensity-modulated radiation therapy. Despite experiencing significant anterior mouth sores from cetuximab early in treatment, as well as significant oropharynx mucositis late in treatment, she was ultimately able to complete RT without an enteral feeding tube. At 3-month follow-up, she had no clinical or radiographic evidence of disease on exam or PET/CT scan. At the time of this manuscript submission, 3 years after completion of the radiation therapy, she remained in remission for both cancers. |
pmc-6461054-1 | An otherwise healthy 43-year-old woman, presented with dysuria, increased urinary frequency, and recurrent urinary tract infection for the past 6 months. Physical examination revealed no abnormalities.
Image work-up was indicated and transvaginal pelvic ultrasonography showed a pedicled bladder nodule, vascularized, hyperechogenic, with 1.9 × 1.4 × 1.8 cm of diameter. The patient was then admitted for an endoscopic evaluation and treatment of the exophytic intravesical mass ().
Cystourethroscopic examination diagnosed a pedicled reddish mass projecting from the top of the right ureteral orifice with 6 cm diameter (). Complete standard transurethral resection with spinal anesthesia was performed. The exophytic mass was resected with an incision on the pedicle, after cautious withdrawal of the lesion through the urethra (). The procedure was uneventful and the patient was discharged from the hospital on day 1 post-TURBT. Follow-up was done by magnetic resonance imaging (MRI) 2 months after procedure, revealing no signs of abnormalities ().
Histopathologic examinations featured dilated glands, chronic inflammatory infiltrate, edematous, vascularized, and loose stroma covered in transitional epithelial tissue without atypias. Immunohistochemistry study has shown spindled and stellate cell proliferation scattered in both myxoid and collagenic stroma with numerous blood vessels of varying caliber. Immunoreactivity was shown for desmin, muscle-specific actin, and estrogen receptors compatible with AA ().
Surveillance with cystourethroscopy every 3 months and annual MRI are planned because of the high recurrence potential of the AA. |
pmc-6461055-1 | A 59-year-old man with hypertension and diabetes presented with progressively worsening back pain radiating down the right leg, right flank pain, nausea, and vomiting. He denied hematuria, dysuria, or any problems with urination. He also had an unintentional weight loss of 5 kg during the past 6 months.
Based on systemic symptoms, CT scan was ordered to rule out malignancy. Radiographic findings were remarkable for hypodense liver lesions, pulmonary nodules, retroperitoneal lymphadenopathy, and increased bone density in L1 and L3 vertebral bodies as well as the right iliac bone. The patient also had moderate right hydroureteronephrosis of both renal moieties with delayed nephrogram and transition point within the proximal common right ureter ( and ). Lumbosacral MRI revealed right L5 nerve root impingement by a lumbar mass. Brain MRI showed abnormal signaling within right temporal lobe concerning for metastasis.
To further characterize the ureteral mass as well as to confirm the extent of collecting system duplication, the patient underwent a right ureteroscopy. Cystoscopy revealed bilateral orthotopic ureteral orifices with no ectopic orifice. Right retrograde pyelogram showed a filling defect at the proximal ureter with reduced amount of contrast moving in two levels more proximally, corresponding to a partially duplicated system (). A wire was placed into the upper pole of the kidney. A flexible ureteroscope was advanced alongside the wire confirming a bifurcation of a bifid collecting system at the level of the filling defect. The ureteroscope was advanced into the lower pole ureter, with multiple papillary tumors noted in this segment distally by the bifurcation. Selective barbotage cytology and tissue samples using a Piranha™ grasper (Boston Scientific, Marlborough, MA) were obtained. Narrowing of the proximal right lower pole ureter precluded advancement to the lower pole kidney. Attention was then turned to the upper pole ureter where multiple papillary tumors also were observed at the same level as in the lower pole ureter. Barbotage cytology was again obtained. The upper pole kidney showed no evidence of tumor. Owing to the risk of retrograde seeding of tumor and absence of complete obstruction as demonstrated by adequate emptying films on the retrograde pyelogram, no stent was placed.
Cytology from both right side ureteral moieties returned positive for adenocarcinoma. Tissue biopsy from the right ureter demonstrated metastatic colorectal adenocarcinoma based on immunostaining (e.g., CK20 positive). The patient underwent palliative external beam radiation to the lumbosacral spine and brain followed by initiation of fluorouracil, leucovorin, and oxaliplatin chemotherapy. Although patient's pain improved after starting chemoradiation, his conditioning worsened, eventually becoming bedbound. The patient underwent four cycles of chemotherapy, but interval CT scan showed progression of disease. Ultimately, the patient and family requested termination of chemotherapy and opted for comfort care only. |
pmc-6461058-1 | Our patient is a 51-year-old female with a medical history of hypertention, familial, hypercholesterolemia, gallstones, depression, coronary artery disease (identified on calcium study because of familial hypercholesterolemia), nephrolithiasis, and recurrent cystitis. Her first stone event was in 2008 that subsequently required ureteroscopy (URS) and ureteral stent placement. She presented to our outpatient clinic initially in consultation for left-sided abdominal pain, and subsequent CT scan revealed nephrolithiasis with three separate left ureteral stones (), requiring urgent ureteral stent placement.
After urgent decompression with a stent, she was scheduled for bilateral URS with laser lithotripsy (LL) and possible bilateral percutaneous nephrolithotomy (PCNL) for definitive stone management. She ultimately underwent bilateral URS with LL and right-sided PCNL. Postoperative CT scan revealed residual right-sided stone burden, so she was scheduled for a second stage right-sided PCNL. In the interim, her postoperative course was complicated by gross hematuria and anemia, requiring transfusion of 2 U of packed red blood cells.
During her planned second stage PCNL, initial ureteroscopic observation was very poor because of extensive clot burden in the right renal pelvis and calices (). Three percent H2O2 standard preparation was diluted three times and ∼100 mL was instilled using a 60 mL Toomey syringe through the current indwelling 22 Councill nephrostomy tube to dissolve the extensive clot burden and improve observation. Approximately 45 seconds after instillation, the anesthesiologist noted that the patient's end tidal carbon dioxide (CO2) had suddenly plummeted (0–9) and she had become hypotensive. ST segment elevation was also noted on the telemetry tracings that were a change from her preoperative electrocardiogram. Owing to immediate concern for pulmonary embolus, the procedure was terminated, and the patient was taken out of dorsal lithotomy position and placed in the supine position. The patient's blood pressure increased over the next several minutes, and she became hemodynamically stable; however, her end tidal CO2 remained well below normal. She was immediately transferred from the operating room to radiology for emergent CT angiography (CTA). At the time of CTA, the patient's end tidal CO2 had risen to 30 to 40. Ultimately the CTA revealed no evidence of pulmonary thrombus; however, abdominal follow-through was notable for extensive air in the right renal collecting system (). The patient was transferred back to the operating room, extubated, and brought to postanesthesia care unit for intensivist consultation without complication. She demonstrated stability and returned to her inpatient floor. She recovered well without evidence of residual effects. Three days later, she underwent her final stage procedure to render her stone free without complications. Final postoperative CT after third stage procedure revealed no residual stones bilaterally. |
pmc-6461566-1 | A 37 y/o female from a rural area in Syria was referred to our clinic after one year of total open thoracic pericystectomy from right and left lobes of lung; the surgical management of hydatid cyst of the liver was delayed till this admission after two shots of albendazole treatment.
The patient recently developed also a symptomatic cholelithiasis. There were no other obvious findings in physical examination. Laboratory investigations were in normal limited [].
The ultrasound showed a cyst in the right lobe of liver and gallbladder stones with no signs of inflammatory. The abdominopelvic computerized tomography (CT) revealed a cystic mass of 5 × 4.5 cm2 with regular borders and minimal calcifications in the 6th segment of liver []. It was classified as a second degree in the Gharbi classification system [].
The plan of the management was discussed and included lagrot partial pericystectomy and cholecystectomy in the same time. The anatomical location of the hydatid liver cyst allowed to use the same ports for both procedures and it alerted, in the same way, to the risk of facing adhesions in the surgical field if we delayed one procedure to another operation which would be scheduled later. This judgment encouraged our team to run both procedures simultaneously. |
pmc-6461568-1 | A14-year-old female patient known to have Pica disorder since the age of 2 years with a history of recurrent trichophagia was admitted to the emergency department for nausea, vomiting, and unintentional weight loss of 7 kg in 1 month.
On physical examination, the vital signs were stable, the patient appeared pale.
Abdominal exam revealed a large, firm and hard mass in the epigastric and left upper quadrant areas.
The result of the blood tests including complete blood count, electrolytes, BUN, creatinine, liver function tests, amylase and lipase were unremarkable.
The patient underwent a computed tomography (CT) of the abdomen and pelvis, revealing a large mass measuring 30 × 17 × 12 cm, well defined, multi-layered, heterogeneous, solid appearing, non-enhancing mass in the gastric lumen, extending from the gastric fundus to the pyloric canal. Some of the layers of this mass were heterogeneously hyperdense (). The lesion was separated from the gastric walls by gastric fluid. No evidence of abnormal gastric mural thickening was noted ().
Consequently, esophagogastroduodenoscopy (EGD) was done, revealing a collection of a large hard hairball occupying the entire lumen of the stomach from the fundus through the pylorus reaching the duodenum. Endoscopic intervention failed to retrieve the mass due to its large size and hard nature.
Subsequently, surgical intervention was planned through a midline laparotomy. A large solid fixed mass was palpated in the stomach. A 7 cm longitudinal gastrotomy was done on the anterior gastric wall, 6 cm from the pylorus. A large trichobezoar, filling the entire stomach and the first portion of the duodenum, was identified and removed (). The gastrostomy site was repaired in two layers with continuous 3.0 PDS (polydioxanone Ethicon). The abdominal incision was closed in two layers with continuous Vicryl 1 suture and skin was closed with staples. The total operative time was 50 min and the weight of the bezoar was 8 kg. The postoperative course was uneventful and the patient was referred to behavioral and mental health providers. |
pmc-6461569-1 | A 66 years old male patient, known to have hypertension showed up with a painless lump in the right hemiscrotum that he has been suffering from since 3 years. He had no history of testicular trauma or infection or lower urinary tract symptoms. Examination showed a well-delimited mass of 5 cm, firm, non-mobile with irregular border over the right spermatic cord. All blood examinations were normal. The serum levels of alpha-fetoprotein, beta-hCG, lactate dehydrogenase were within the normal limits. The patient denied scrotal pain, hematuria, dysuria, fever or chills.
Ultrasound of the scrotum (-A) showed 4 × 3 × 4 cm, circumscribed heterogeneous solid extra-testicular mass located above the right testicle with increasing vascularity (-B) suggesting a neoplastic mass. The epididymis seems to be preserved, the right testicle shows homogenous echo structure. CT-scan of chest, abdomen, and pelvis were negative for local or distant metastasis.
Right radical orchidectomy along with excision of the spermatic cord mass was performed. During the surgery a mass is seen arising from the upper part of the cord while the testis and epididymis were separate from the lesion (). Microscopic examination () of the well -delineated mass demonstrated fascicles of spindle cells with eosinophilic cytoplasm of probable smooth muscle origin. Focal areas with pleomorphic morphology and hypercellularity with two to three mitoses per high power field (2-3/HPF) are present. The immunohistochemistry tested positive for Vimentin and Actin. Definitive pathological diagnosis of this patient revealed a leiomyosarcoma of the right spermatic cord with negative surgical margins. The clinical and radiologic follow-up with thoraco-abdominopelvic CT for 6 and 12 months shows no signs of local recurrence and distant metastases. |
pmc-6461581-1 | A 57-year-old female patient was referred to our hospital for further evaluation of the accidental finding of a space-occupying lesion in the upper right abdomen made in the course of a sonographic evaluation of the underlying cause of upper abdominal pain. Although the patient did not report unintentional weight loss, she complained of the presence of night sweats unaccompanied by fever over the past 12 months, as well as of the occurrence of exertional dyspnea. There were no significant pre- existing disorders, and neurofibromatosis had not been diagnosed. The CT-scan showed an axial space-occupying lesion of approximately 9.1 x 6.6 cm right-sided paramedian retroperitoneal, with displacement of the inferior vena cava and stretching of the right renal vein (A/B). [18 F]FDG-PET/CT confirmed the presence of the described right lateral paraaortic lesion, situated at the level of the renal hilus with a malignoma-typical increase of metabolic activity in the marginal areas (). No hematogenic or lymphogenic metastases were identified. Pheochromocytoma was excluded by negative catecholamines and metanephrines in the 24h-urine collector. The punch cylinders obtained on CT-guided puncture were in some instances characterized by hyalinized and cell-poor spindle cell proliferation, while siderite pigmentation was observed others. Immunohistochemically, the spindle cells were strongly positive for S-100 and showed nuclear positivity for SOX-10 at negativity for MDM2, Desmin, CD1a, Melan-A, and HMB-45, as well as for smooth muscle actin (SMA). The MIB-1 proliferation index ranged at < 1%. Molecular-biologic analysis did not yield evidence of MDM2 amplification. The overall histopathologic assessment of the described factors leads to the diagnosis of a benign schwannoma with marked regressive changes.
The observed displacement of both, the inferior vena cava and the right renal vein, as well as the malignoma-typical increase of metabolic activity in the [18 F]FDG- PET/CT led to the assumption – in spite of negative histology - that the presence of malignancy could not be excluded. Consecutively, we performed an open resection of the tumor with plastic reconstruction of the right renal vein, while the inferior vena cava could be completely preserved, as it was unaffected (A). Macroscopy revealed a solid, greyish, spherical tumor with a soft capsule and a heterogeneous center (B). The final histopathologic examination confirmed the presence of a partially myxoid schwannoma of 8.5 cm on the largest diameter, showing significant regressive changes and a partially cystic component, as well as evidence of metaplastic ossification (A/B). The tumor together with the intact capsule was resected curatively (R0-resection). The postoperative course was uncomplicated and the patient was discharged from hospital on postoperative day 8. No adjuvant therapy was indicated and regular aftercare only was recommended by the interdisciplinary tumor board. |
pmc-6461590-1 | A 63-year-old female presented to our hospital with right hypochondrium pain. She underwent appendectomy at the age of 20 years and had an uneventful recovery following that. Laboratory results showed AST of 34 U/L; ALT of 32 U/L; and ɣ-GTP of 142 U/L. A gallstone was found on computed tomography (CT) and abdominal ultrasonography. By drip-infusion cholangiography with computed tomography (DIC-CT) performed before LC, we were able to clearly visualize the cystic duct, common hepatic duct, right anterior sectional duct, and right posterior sectional duct, but the subvesical bile ducts were not visualized (). She was scheduled to undergo LC for symptomatic cholelithiasis.
One milliliter (2.5 mg/ml) of ICG was injected intravenously prior to beginning the surgery. The D-light P system (KARL STORZ, Germany) with the integrated indocyanine green fluorescence imaging (IGFI) mode was prepared to visualize the bile duct. The operative field was inspected in the IGFI mode before dissection of Calot’s triangle. The common bile duct and cystic duct were visualized on FC. LC was performed using a standard procedure under normal lighting. During dissection, FC was used when needed until the critical view of safety was confirmed. FC detected two aberrant bile ducts, 1 to 2 mm in diameter, during the dissection of Calot’s triangle (). We achieved the critical view of safety, and considered these ducts to be the subvesical bile ducts. After division of the cystic duct and cystic artery, we ligated the subvesical bile ducts with clips and divided them. Then, FC was used again to evaluate bile leakage. Dissection of the gallbladder from the liver bed was continued and the gallbladder was resected.
The resected specimen revealed that two subvesical bile ducts drained into the gallbladder (). Postoperative laboratory test results were all within normal limits. CT demonstrated no dilatation of the intrahepatic bile duct after LC. The patient was discharged uneventfully on the fourth postoperative day. |
pmc-6461729-1 | A 63-year old male patient, reported to the Dental clinic with multiple missing teeth who requested a replacement with fixed prosthesis. Panoramic radiograph was advised which revealed an incidental finding of a bilateral bifid mandibular canal (Fig. ). To the best of our knowledge and current literature search revealed only 4 earlier references on this variation (-). In these cases the bilateral canals led to two separate mental foramen. It is evident in the case at hand that the bilateral bifid canals appeared to separate into two branches from the original mandibular canal and led to the same mental foramen on either side (). In our case there was evidence of a bilateral bifurcation with varied morphology of the mandibular canal. The site of bifurcation on the left hand side could be visualised at the height of an imaginary line drawn horizontally along the occlusal plane at the height of the adjacent third molar. However the bifurcation site on the right hand side appeared to separate much lower in the mandibular ramus of the mandible. Our second case was a 37-year old male patient who reported to the Dental clinic and was curious to know if an implant could be placed in the region of upper right 1st molar region. Panoramic radiograph was advised which also revealed an incidental finding of a bilateral bifid mandibular canal (Fig. ). The site of bifurcation on the left hand side was almost similar to our first case. However determination of the bifurcation site on the right hand side was more difficult and it appeared to separate much higher in the mandibular ramus of the mandible. |
pmc-6461737-1 | A 9 year old male patient was brought by his father, to our Maxillofacial private consultation at Alcorcon Southern Hospital, referred from Public medical institution, seeking for evaluation and possibility of treatment, the reason of consult was the abscense of eruption of the permanent superior incisors. The diagnosis of EVC was made at birth, in the public institution where he was referred from. There was no family background of the disease, he had healthy parents and brothers. The parents are originally from Morocco and consanguinity related in second degree.
Among the patient’s medical background, he presented heart congenital disease: interventricular communication that closed spontaneously and interauricular communication, surgically corrected at the age of three.
At the age of four, surgical correction of epispady was performed and at the the age of nine, surgical correction of both inferior limbs axis was performed “genu valgum deformity”.
At the clinical exploration, he presented short disarmonic stature (110 cm), both superior and inferior short limbs with bilateral hexadactyly and hypoplastic fingernails.
Facial exploration, revealed normo-brachicephaly, normal eyelid fissures, wide nasal tip and narrow nostrils (Fig. ).
Intraoral exploration revealed, agenesis of lateral superior and inferior incisors [12,22,32,42], dysmorfism compatible with conical teeth at inferior incisors and canines [31,33,41,43], fusion between a superior incisor () and a supernumerary tooth (mesiodens). As for the soft tissues, multiple frenum, macroglossia and glososquissis can be find (Fig. ).
Furthermore, retention of maxillary incisors was present, due to the presence of a central supernumerary tooth. For this purpose, the retention was treated by extraction of the included central supernumerary tooth, under general anesthesia.
Radiografically, certain degree of taurodontism was present at the permanent superior first molars and more discretely at the permanent inferior first molars (Fig. ). In total 2 central supernumerary teeth were present. Maxillary compression was also evidenced
It is remarkable the maloclussion with a tendency to a class III and anterior crossbite. |
pmc-6461826-1 | A 68-year-old man with 21-year history of type 2 diabetes presented with an ulcer on the left heel. Height was 162 cm; body weight was 69 kg; body mass index was 26.3. The patient had been diagnosed with lower extremity atherosclerotic obliterans 7 years earlier. The left lower limb has been numb for 6 years, with intermittent claudication and rest pain for 1 year. The patient reported that his sleep was affected, but his degree of pain was decreased with the intermittent use of analgesic agents. For the left lower limb with claudication, walking distance was 90 m.
The patient was hospitalized on 10 July 2017. Twenty days before hospitalization, irritation and pain developed on the lateral skin of the toes of the left foot, with no obvious inducement. Purulent exudate was observed after skin ulceration, and the patient’s body temperature increased to 39.5 °C. During hospitalization, the five toes of the left foot were black, necrotic, and associated with aggravated rest pain. Although the dose of oral analgesics was increased, the patient’s pain was not relieved, and his sleep was severely affected.
The patient’s appearance on initial evaluation is shown in Fig. a and b. The five toes of the left foot were almost entirely black and necrotic. The skin extending from the bottom of the foot to the 5th metatarsophalangeal joint was red and swollen, with obvious tenderness; skin temperature was normal. The muscles of the left foot had clearly atrophied; the skin was thin, bright, and hypertonic. Incision and drainage (approx. 5.0-cm long) was immediately performed between the 4th and 5th toes. Necrotic tissue, minimal purulent exudation, and limited bleeding were observed.
The diagnostic results on obtained on July 13 are shown.Secretion cultures displayed Pseudomonas aeruginosa and Staphylococcus aureus. X-ray film showed in Fig. c and d revealed no obvious destruction in foot bone. The lower limb computed tomographic angiography (CTA) is shown in Fig. e.
The magnetic resonance imaging (MRI) of the foot obtained on July 14th is shown in Fig. f and g. Subcutaneous inflammatory tissue in the lateral 5th humerus bone was confirmed as an infectious submerged cavity (Fig. ). The first incision performed at the bedside resulted in limited purulent exudation, as well as decreased local tension and reduced foot swelling. However, progressive necrosis of the skin margin was noted (Fig. a and b).
On July 17, basal therapy consisted in controlling blood sugar with insulin (58 units/day). The initial antimicrobial application of ceftazidime infusion (2 g, twice daily) was replaced by the sensitive drug Sulperazon (Cefoperazone Sodium and Sulbactam Sodium for Injection, Pfizer), according to the bacterial culture result of the secretion, and the application of antibiotics was stopped after July 22. The patient showed improved circulation and blood pressure. Treatment was maintained with protein and iron supplements.
On July 18th, interventional therapy was performed on the lower limb. The balloon was expanded in the superficial femoral artery, anterior tibia artery, iliac artery, and tendon. A thrombus had formed in the lower segment of the superficial femoral artery. The catheter for thrombolysis was left in place until July 20 (Fig. c, d, e and f). Skin temperature in the left foot increased, and swelling extended to the center of the foot. The amount of purulent exudation increased, as did pain and body temperature. The foot was therefore incised and drained, once more. Multiple subcavities were found (Fig. ).
On July 21st, we performed patella truncation and debridement on toes 4 and 5. The procedure was performed in the operating room, with the patient under sciatic nerve block. Results are presented in Fig. . Submerged cavities were filled with povidone-iodine gauze. The amount of nonviable tissue in the submerged cavity gradually decreased. The submerged space became shallow, and the granulation filled. All subsequent treatments were performed in the outpatient department.
From July 25th to September 30th, the left foot plaster was fixed at a functional position and applied intermittently (Fig. a, b, c, d, e and f). After October 1st, the plaster was removed. The patient was given diabetic shoes, and walking was guided. The first signs of wound healing were observed on November 19 (Fig. g, h and i). |
pmc-6462037-1 | A 19-year-old male developed a primary tumour in the prostate that was diagnosed as a PAX3-FOXO1-positive aRMS. He underwent treatment according to the Italian Sarcoma Group/Scandinavian Sarcoma Group protocol III (ISG/SSGIII) consisting of doxorubicin, vincristine and cisplatin (Supplementary Table ).
The patient had a refractory and disseminated disease with multiple metastasis in the lung, sacrum, arm and neck at the time of biopsy. A sample from a palpable neck lesion was obtained by FNA for drug screening ex vivo (Fig. ).
We detected the expression of the PAX3-FOXO1A fusion transcript in the PDC (K-RMS1) by RT-PCR confirming the presence of aRMS cells (Fig. ). Among the cancer driver genes expressed in K-RMS-1 we found increments in the expression of BAX, RASD1, WT1, AKT1, cMYC and NOTCH (Fig. ).
Figure shows the selective drugs active in K-RMS1 that included several kinase inhibitors such Crenolanib (Platelet-Derived Growth Factor Receptor inhibitor); Dasatinib/Sprycel® (cSrc inhibitor); Cabozantinib/Cabometix® (cMet and VEGFR inhibitor); and Crizotinib/Xalkori® (targeting the Anaplastic Lymphoma Kinase ALK and cMET). Consistent with the patient refractory disease, the drug screening test showed poor responses for the drugs that the patient had received at the time of biopsy: doxorubicin, cisplatin and vincristine (Fig. , red dots) with sDSS below 5. The patient died of progressive disease during the course of the study. |
pmc-6462037-2 | Two cases were investigated; one expressing the fusion protein ASPS1-TFE3 (K-ASPS2) and a case where the fusion gene transcript was not detected in either biopsy nor the PDC (K-ASPS3).
K-ASPS2 originated from the tumour of a 20-year-old female, with a mass in the soleus muscle with a tumour morphology characteristic of alveolar soft part sarcoma consisting of tumour cells with eosinophilic cytoplasm and prominent nucleoli arranged in small alveoli (Fig. ). Lung metastases were detected at diagnosis. The fusion transcript ASPS1-TFE3 type 2 was detected in the tumour biopsy at routine diagnosis. The tumour was surgically removed, and the patient underwent oncological treatment with adriamycin and ifosfamide (Supplementary Table ), but developed multiple metastasis in the skeleton, mediastinum and adrenal gland. A biopsy from a skeletal metastasis was then obtained after palliative surgery for the establishment of the PDC and the expression of the ASPS1-TFE3 fusion protein was confirmed by PLA in 95% of the patient-derived sarcoma cells (Fig. ).
At the time of biopsy, the patient had received treatment with Sunitinib (Sutent®) Crizotinib (Xalkori®), Trabectedin (Yondelis®), Denosumab (Xgeva®) and Pazopanib (Votrient®) with poor responses and developed progressive disease (Supplementary Table ). Similarly, the drug sensitivity testing on the PDC showed poor activity for all these drugs except for Pazopanib (Fig. , red dots).
Active drug classes were mTOR inhibitors and other multikinase inhibitors such as Lucitanib and Tesevantinib. Interestingly, two C-Met inhibitors, Golvatinib and MK2461, were identified as selective hits (Fig. , blue dots). The ASPS1-TFE3 fusion protein is a transcriptional activator that directly targets and activates the cMET promoter. The patient died of progressive disease during the study.
The second ASPS case was a 22-year-old female with a 9 cm primary tumour in the gastrocnemius muscle and lung metastasis at diagnosis.
The primary tumour was surgically removed, and the diagnosis confirmed morphologically as alveolar soft part sarcoma (Fig. ). Fresh primary tumour was used for the establishment of the PDC (K-ASPS3). The ASPS1-TFE3 was not detected by routine diagnosis in the biopsy (data not shown) nor in the derived PDC; there the PLA showed cytoplasmic but not nuclear ASPS1-TFE3 signals (Fig. ).
Cancer driver gene expression was quantified in the ASPS3 biopsy and PDC. The apoptotic regulator (BAX), the dexamethasone-induced Ras-related (RASD1), the Wilms Tumour 1 (WT1) and mTOR (FRAP1) kinase genes were among the most expressed genes in relation to normal muscle cells (Fig. and supplemental Fig. )
Consistent with the activation of mTOR/PI3K pathway in the ASPS3 biopsy and derived PDC, several mTOR/PI3K inhibitors were active in functional screens such as PF04691502, Dactolisib and Gedatolisib with sDSS values above 10 in relation to healthy bone marrow cell controls. Other selective hits for K-ASPS3 were the purine analogue Fludarabine, the folate anti-metabolite Pemetrexed, the cell division cycle 7 homologue (CDC7) kinase inhibitor BMS-863233, and the topoisomerase inhibitor Topotecan. Similar to K-ASPS2 and K-RMS1, Dasatinib was an active drug in K-ASPS3 (Fig. , green dot).
This patient was treated with Sunitinib™, developed progressive disease with brain metastasis that was treated with whole brain radiation 4GyX5; and later developed lung metastases and was treated with doxorubicin. The patient has progressive disease at present, with new cerebellar lesions, underwent surgery and is now on treatment with Dasatinib. |
pmc-6462037-3 | A 33-year-old female presented with a 9.9 × 7 cm tumour in the gastrocnemius. The tumour consisted of spindle cells positive for TLE1 and CD99 and for the fusion transcript SS18-SSX determined in routine diagnosis. A piece of the tumour and of healthy muscle were obtained for the establishment of sarcoma and muscle PDC (Fig. ). The synovial sarcoma biopsy and derived PDC (K-SS3) but not the muscle biopsy or the cultured muscle cells expressed the SS18/SXX fusion as determined by RT-PCR (Fig. ). However, approximately 40% of the cells in K-SS3 expressed the SS18/SSX-TLE1 fusion protein complex as determined by PLA (Fig. ).
Among the genes expressed in the K-SS3 biopsy and PDC were WT1, BAX, N-MYC, HIF1α, SOX9, and autophagy-related genes (Fig. ).
Few inhibitors were selectively active in K-SS3 such as the PI3K inhibitor Copanlisib/Aliqopa™, the ALK/FAK1 inhibitor CEP-37440, the MEK inhibitor AZD-8330 and the AKT inhibitor Triciribine (Fig. ). Consistent with previous reports demonstrating the activity of HDAC inhibitors in synovial sarcomas, several HDAC inhibitors had significant activity in this PDC when normalised with normal mesenchymal controls (Fig. , green dots and supplementary Figure ); however, they showed toxicity for the bone marrow cell controls (low sDSS bone marrow controls). Several mTOR/P13K (Fig. , turquoise dots) and the cSrc inhibitor Dasatinib (Fig. , green dot) showed anti-tumour activity in K-SS3. At present, the patient has no evidence of disease. |
pmc-6462037-4 | Two Ewing sarcomas PDC were established from FNA biopsies of two patients: K-ES1 and K-ES2.
K-ES1 was obtained from an FNA of a primary tumour in the right scapula of a 26-year-old male, prior to oncologic treatment (Fig. ). The EWSR1-FLI1 fusion was detected in the biopsy (data not shown) and in 95% of the cells in the PDC confirming the presence of Ewing sarcoma cells (Fig. ). Cancer driver gene expression was characterised by the expression of the DNA damage inducible transcript 3 (DDIT3), the mTOR kinase gene FRAP1, the anti-apoptotic gene BCL2 and several autophagy-related genes of the ATG and SEC family, and downregulation of the TP53 gene among others (Fig. ).
The patient presented with bone marrow, skeleton and lung metastases at diagnosis and was treated with Scandinavian Sarcoma Group protocol IV (SSGIV) protocol consisting of cisplatin, doxorubicin, etoposide and vincristine with no clinical response (Supplementary Table ). Consistent with the patient clinical response to treatment, no activity for these drugs was observed in the drug screening assay (Fig. , red dots). However, several selective inhibitors were identified for this patient tumour cells such as cSrc inhibitors Dasatinib and Saracatinib (Fig. , green dots), the FGF inhibitor LY-2874455, the PAK inhibitor FRAX486 and the purine analogue Fludarabine (Fig. ). The patient developed systemic disease and died few months after diagnosis.
K-ES2 originated from a 31-year-old male diagnosed of Ewing sarcoma. The patient received adjuvant treatment with vincristine, doxorubicin and cyclophosphamide (SSGIV) and radiotherapy (1,8 GyX28), followed by autologous stem cell transplantation (Supplementary Table ). Thereafter, the patient underwent surgery with complete resection of the primary tumour. After a 3-year disease-free survival the patient developed metastasis in the third right rib. An FNA biopsy was taken from this lesion and the tumour cells were cultured in vitro (K-ES2). The EWS-FLI1 fusion protein specific for Ewing’s sarcoma was expressed in 95% of the cells in the PDC culture (Fig. ). The patient metastasis was treated with docetaxel and gemcitabine before surgery according to the SSGIV protocol and achieved complete response (Fig. ). Consistent with the clinical response of the patient, the ex vivo drug sensitivity testing of his tumour cells (K-ES2) showed activity for the taxanes docetaxel, paclitaxel and cabazitaxel (Fig. ), but not for gemcitabine suggesting that the complete clinical response could be attributed to the taxane treatment. |
pmc-6462314-1 | A 48-year-old male presented to the emergency department with complaints of malaise and nausea. The past medical record was relevant for type 2 diabetes mellitus that was treated at the time with neutral protamine Hagedorn insulin. On initial examination, he had signs of severe dehydration and was polypneic; blood pressure was 80/60 mmHg, and heart rate was 125 bpm, with an oxygen saturation of 95% at room air. The capillary glucose level was 620 mg/dL; venous blood gases showed a metabolic acidosis with pH < 6.94 and HCO3− of 4.1 mEq/L. A severe DKA was diagnosed, and treatment was started with aggressive hydration and IV insulin. Initial electrolytes were Na+ 119.6 mEq/L, Cl− 95 mEq/L, and K+ 5.7 mEq/L, and serum creatinine was 2.6 mg/dL; the anion gap was high, with 21 mmol/L. A 12-lead electrocardiogram (ECG) was performed and showed an ST-segment elevation of 4 millimeters in V1 and V2 leads and 1 millimeter elevation in aVR lead; also, there was a QRS complex widening and tall T-waves in V3-V5 (). Concerns were raised of possible myocardial infarction as the precipitant factor for the DKA. A cardiology consult was required, and a point-of-care cardiac ultrasound was performed, which showed a normal left ventricular ejection fraction and no regional wall motion abnormalities, cardiac troponins where ordered and reported within the normal range. The patient continued on management. After 1 hour and 30 minutes of treatment for DKA, a subsequent ECG was performed, and it showed a complete resolution of the ST-segment elevation (). DKA was resolved, and the patient was admitted for monitoring, with a favorable evolution. |
pmc-6462315-1 | The patient is a 21-year-old African American man with a past psychiatric history of schizophrenia who was transferred from the psychiatry unit to the medical floor to rule out sepsis after the development of fever and tachycardia. The patient was reported to have decompensated on account of nonadherence with his home medications resulting in frequent hospitalizations and poor functioning. As per the patient's admission records, the patient's initial labs and urine toxicology were within normal limits. The patient had been on various antipsychotics since his diagnosis with schizophrenia at the age of 19, including haloperidol, olanzapine, and risperidone, but he continued to decompensate despite adequate medication trials. The decision was made to start the patient on clozapine due to possible failure of these previous antipsychotics. Clozapine was started at 25 mg PO twice daily and titrated up to 150 mg PO twice daily over the next 12 days. His current daily dose was 300 mg when he was transferred to the telemetry unit following a sudden development of fever and tachycardia on the psychiatric inpatient floor.
On the medical inpatient unit, the patient was mostly selectively mute and did not appear to be in any pain, was not vomiting, and had no diarrhea and no reported loss of consciousness or seizures. His admission vitals were a temperature of 102.4 degrees Fahrenheit, BP of 115/81 mmHg, HR of 114 beats per minute, and an oxygen saturation of 97% on room air. Physical examination did not reveal any significant findings other than a mild dehydration, no skin rash, no jugular venous distension, basal crepitation or pedal edema. Cardiac auscultative findings were largely normal except for a tachycardic heart rate; apex beat was in the 5th intercostal space, midclavicular line. The patient's heart sounds, S1 and S2, were normal, no rubs, or murmurs, and no obvious gallop rhythm. His peripheral extremities were warm with a normal capillary refill.
The patient was given an intravenous bolus of normal saline and a stat dose of intravenous antibiotics (vancomycin and meropenem), and full workup including 2 sets of blood cultures, urine analysis, and a chest radiograph was ordered. His complete blood count (CBC) revealed a normal leukocyte count of 7000/µl with elevated eosinophils of 500 (reference 0.0–400, see for trend) and normal platelet count. He had an elevated erythrocyte sedimentation rate (ESR) of 26 (reference 0–15 mm/Hour) and CRP was 147.4 mg/dl (reference 0.0–4.9 mg/dl).
In view of the patient's current clozapine use, a presumptive diagnosis of myocarditis was made and troponin I was requested which turns out to be markedly increased, 1.12 ng/dl (reference: 0.00–0.05 ng/dl). His NT-pro B-type natriuretic peptide (BNP), lactic acid, and creatine kinase levels were normal. His electrocardiogram (EKG) showed sinus tachycardia without specific ST-T changes ( below). His chest radiograph was unremarkable. Urinalysis and urine toxicology were negative. Initial transthoracic echocardiogram (TTE) revealed a left ventricular systolic dysfunction with apical hypokinesis (ejection fraction 45%) and mild tricuspid regurgitation. Coronary angiogram showed patent coronaries; ventilation/perfusion scan resulted as low probability for pulmonary embolism.
Clozapine was discontinued on admission to the medical unit. Subsequently, the patient's fever and tachycardia resolved. Troponin I trended down (lowest being 0.22 ng/ml). Eosinophil count initially increased but later normalized as shown in . Repeat transthoracic echo after one week of discontinuing clozapine revealed normal left ventricular systolic function with mild apical hypokinesis (ejection fraction of 60–65%). The patient was clinically stable throughout the hospital course. The psychiatry consult liaison team started the patient on aripiprazole which was well tolerated by the patient without any side effects. The patient later underwent an uneventful discharge to the community outpatient clinic. |
pmc-6462316-1 | A 79-year-old Chinese female with chronic hepatitis C was referred for assessment of a liver mass. The patient was asymptomatic with no signs of decompensated liver disease. Her only comorbidity was well-controlled essential hypertension, for which she took valsartan 80 mg once daily. It was not clear from the history when she had started taking valsartan. She denied taking any specific herbal remedies. She was a nonsmoker and had no history of excess alcohol ingestion.
Her physical examination on admission was unremarkable. Her liver edge was nontender and palpable 2 cm below the right costal margin. There were no stigmata of chronic liver disease. Liver enzymes and function tests were in the normal range: (bilirubin 8 μmol/L (normal 0–20), albumin 37 g/L (normal 33–48), INR 1.0 (normal 0.9–1.1), and creatinine 55 μmol/L (normal 45–100)). The Child-Turcotte-Pugh Score was 5, and the raw MELD score was −1. Platelet count was depressed at 100 × 109/L (normal 150–450). Serum alpha-fetoprotein (AFP) was extremely elevated at 17,833 μg/L (normal 0–10; ). Other investigations for chronic liver disease (i.e., hepatitis B virus, autoimmune, and metabolic etiologies) were negative. A triphasic computed tomography (CT) scan revealed a 4.0 × 4.0 cm mass in the right hepatic lobe and three nodules in the lower lung lobes ().
The patient was given a clinical diagnosis of hepatocellular carcinoma (HCC) with probable pulmonary metastases. She declined a liver biopsy. The disseminated state of her disease precluded ablative treatments and, because of her advanced age and frailty, she was not considered a candidate for chemotherapy.
At two months, the patient did not experience a decline in her condition, and a follow-up CT was done. The lung lesions had completely disappeared, and there was a significant decrease in the size of the primary liver lesion (). Serum AFP levels had fallen to almost within the normal range at 26 µg/L (). During the subsequent 18 months, the patient remained clinically stable with no evidence of decompensated liver disease. There was a temporary rise in AFP, peaking at 4345 μg/L, and then AFP decreased again to 319 μg/L (). At 5, 8, 13, and 17 months, the liver lesion continued to shrink. Follow-up was deintensified. At 59 months, the AFP was 217. Then, at 71 months, she presented to the emergency department with ascites. A CT demonstrated a marked adverse change in the appearance of the liver: there were numerous solid and enhancing hepatic deposits (), as well as a large conglomerate solid mass measuring 10.6 × 6.3 × 8.0 cm in the right hepatic lobe. In addition, there were innumerable pulmonary nodules bilaterally. AFP was 56,034 μg/L (). She died two weeks later. The patient's clinical trajectory is summarized in Supplementary . |
pmc-6462322-1 | A 54-year-old Caucasian female with a history of lupus presented for elective left total knee arthroplasty following the development of osteoarthritis that had failed conservative measures. The patient had a history of previous left knee ACL reconstruction approximately 25 years ago using the Arthrotek bone mulch screw and WasherLoc system []. She underwent removal of the tibial WasherLoc approximately 10 years later, in the early 2000's ().
The patient was positioned supine; standard incision with a medial parapatellar arthrotomy was performed. A measured resection technique was then performed with an intramedullary guide placed in the femur. The femur was cut in 6 degrees of valgus and 3 degrees of external rotation. A size 4 femoral prosthesis was placed and noted to overhang both medially and laterally on the condyles. At this time, it was decided to downsize the femoral component. The 4 in 1 femoral cutting block was then placed back on the femur and was noted to be in contact with the bone mulch ACL screw. The bone mulch screw was located and identified in the lateral femoral condyle; a curette was used to clear the head of the screw, and it was removed. The proximal tibia was then prepared using an intramedullary guide with 3 degrees of posterior slope. A size 3 tibial component and a 9 mm poly were placed; the knee was noted to be tight in both flexion and extension. An additional 2 mm resection was performed on the proximal tibia. It was noted at this time while trying to trial the prostheses that the lateral femoral condyle was fractured. Conversion to a stemmed femoral component with a cruciate stabilizing prosthesis was attempted. The femoral canal was reamed, and the femoral box cut was made. However, during trialing, the medial femoral condyle was now noted to have a fracture as well. An intraoperative consultation with an adult reconstruction trained orthopaedic surgeon was performed. Immediate surgical correction was not possible due to improper implants being presented. The femoral and tibial canals were then reamed to accept a 200 mm × 9 mm intramedullary nail to act as a temporary internal stabilization device (). The knee was irrigated and closed, and the patient was admitted to the floor. The patient was then brought back to the OR on postoperative day 3 following the index procedure. The prior incision was utilized; the wound was copiously irrigated. It was noted that due to the patient's poor bone quality and comminution of the fractures that the only viable option was a distal femoral replacement. The distal femur was resected, the femoral canal was reamed, and a planar was used on the distal femur. A skim cut and reaming of the tibia were performed. The components were trialed. Final implants included a 13 × 127 mm hinged femoral prosthesis and small 1-stemmed tibial tray; a 32 mm patellar component was used, and a size 10 polyethylene was then inserted; all components were cemented. The knee was noted to be stable throughout range of motion with good patellofemoral tracking. The surgical wound was copiously irrigated and closed (). Estimated blood loss was 100 mL; no postoperative transfusion was necessary. She was able to bear weight as tolerated immediately postoperatively. The patient's pain was controlled postoperatively, and she worked well with physical therapy and was discharged home with home health care on postoperative day two with 3 weeks of Coumadin for venous thromboembolism prophylaxis.
The first postoperative visit was at two weeks; the patient had some swelling and quadriceps weakness, and the incision was healing well. Range of motion (ROM) was from 0-100°. At 6 weeks, she was still requiring narcotic medication; quadriceps strength was improving, ROM from 0-105°.
At 12 weeks, X-rays remained unchanged; the patient continued to have mild quadriceps weakness and was no longer requiring narcotic medications. ROM was not documented at this visit. At 6 months, ROM was 0-120°. The incision was well healed; X-rays were unchanged. The patient was doing well; however, she continued to have some residual quadriceps weakness and difficulty ambulating long distances. The patient was lost to follow-up after 6 months. |
pmc-6462326-1 | A 63-year-old Chinese woman from Wuhan was admitted to the First Affiliated Hospital of Wuhan University in October 2017 with left quadrant abdominal pain and weight loss for more than 6 months. The pain exacerbated after eating and was accompanied with abdominal distension, belching, and reduced flatus. The patient referred was taking traditional Chinese medicine that temporarily relieved the symptoms. She had previous history of hypertension with a poor control. She underwent colonoscopy examination in a local hospital and verbally reported no pathologic findings. The positive findings at the physical examination were the mesogastric tenderness and an enlarged spleen.
The blood laboratory examination reported only mild hypokalemia; the urinary and stool reports were normal. The enhanced computerized tomography showed splenomegaly, the jejunal wall with edema and a narrowed lumen, enlarged lymph nodes, mesenteric edema; the liver cleft was widened with atrophy of the right liver lobe and hypertrophy of the left and caudate lobes; calcification and periportal fibrosis signs were compatible with hepatic schistosomiasis (Figures –).
At the moment of the double balloon enteroscopy, the patient's blood pressure was not stable and we could not reexamine the colon. The upper enteroscopy showed at 150 cm distal to the Treitz ligament a 3.0 x 2.0 cm size ulcer with bottom white coating, peripheral mucosal hyperemia, and edema occupying 2/3 of the lumen so that the endoscope could not pass through. Four biopsies were taken from the ulcer (Figures –). And 20 cm proximal from the ulcer, there were many scattered irregular ulcers from where two biopsies were taken.
The histopathological examination reported chronic jejunal inflammation with ulcerative changes and old S. japonicum eggs deposition (Figures and ).
During hospitalization, the patient received symptomatic treatment with intravenous fluids, electrolytes, antispasmodic medication, and proton-pump inhibitors. Unfortunately, the patient requested for a voluntary discharge from the hospital and decided to continue her treatment and follow-up at a local hospital. |
pmc-6462336-1 | A 63-year-old man with unilateral Ménière disease (unilateral severe hearing loss, rotational vertigo attacks, dizziness episodes, caloric hypofunction, pathologic HIT, and loss of cVEMP response) and severe hearing loss and regular vestibular function of the contralateral side (caloric function, HIT, and cVEMP) was treated with an occlusion of the lateral semicircular canal, endolymphatic sac surgery, and cochlear implantation. MRI and temporal bone CT results were regular.
During a period of 9 months after the first surgery, the patient described after an initial freeness, a reoccurrence of rotational attacks, which were caused, as described by the patient, by the implanted ear and an increase of disabling dizziness. Attacks were not triggerable with a duration of up to 30 min. The attacks were independent from an electrostimulation by the cochlear implant.
We decided to perform a labyrinthectomy of the previously implanted side. After a very careful preparation of the electrode cable (), the implant receiver was removed from the implant bed/periostal pocket without pulling the electrode out of the cochlea (). The receiver was fixed by the implant magnet and Steri-Strips. The scar was removed completely from the mastoid cavity. Then, the electrode was fixed at the posterior wall of the external auditory canal with bone wax (). After that, the labyrinthectomy was performed without dislocation of the electrode from the cochlea (). During the labyrinthectomy, the complete occlusion of the lateral semicircular canal was confirmed. Finally, the implant receiver was placed back into the implant bed. The response thresholds of the electrically evoked compound action potential (NRI) on all implanted electrode contacts do not differ before and after surgery. Related to the regular NRI response, an X-ray was done the next day, which confirmed that the intracochlear insertion depth was the same as preoperatively. NRI threshold after the first surgery for electrodes 1, 6, 11, 16 was 153 CU, 135, 173, and N/A and after the second surgery was 123 CU, 151, 170, and N/A. These small changes indicate a minimal movement of the electrode.
Hearing results before and after the labyrinthectomy with the cochlear implant were 50% monosyllabic speech perception at 65 dB 6 weeks after the second surgery.
The study was approved by the institutional review board of Klinikum Bielefeld, Germany (IRB-klibi-HNO-2018/01). The patient provided written informed consent for the use of his clinical records. |
pmc-6462343-1 | Patient #A14, a 56-year-old male subject, was diagnosed with acute myeloid leukemia (AML-M4) in October 2016. A decision was made for haploidentical HSCT with his son as the donor on 28 March 2017. He received grafts from 5/10 HLA-matched peripheral blood stem cells (PBSCs) and bone marrow (BM) stem cells. The conditioning regimen was modified BUCY (busulfan: 3.2 mg/kg, iv days −9 to −6; cyclophosphamide: 1.8 g/m2, days −5 to −4); GVHD prophylaxis consisted of antithymocyte globulin (ATG), cyclosporin A (CSA), mycophenolate mofetil (MMF), and short-term methotrexate (sMTX). ATG (thymoglobulin, rabbit; Genzyme Europe B.V., Naarden, the Netherlands) was given at a dose of 10 mg/kg from days −5 to −2. CsA (3 mg/kg, iv every 12 h) was administered starting on day −10, and the trough concentration was adjusted to 150–300 ng/ml. MMF was administered orally starting on day −10 (0.5 g, every 12 h) and was withdrawn on day +45 for haploidentical donor (HID) HSCT. sMTX was administered intravenously at 15 mg/m2 on day +1 and 10 mg/m2 on days +3, +6, and +11. Neutrophil and platelet engraftment occurred on days +15 and +23, respectively.
One month later, on day +27, the patient developed abdominal tenderness and diarrhea. In addition to abdominal pain, diarrhea, and fever, patient #A14 had no GVHD-related symptoms and no impaired liver function (alanine aminotransferase, aspartate aminotransferase, total bilirubin, and direct bilirubin were normal). Because of insufficient evidence of GVHD, neither enteroscopy (sigmoidoscopy and colonoscopy) nor glucocorticoid therapy was performed for this patient. Virological tests showed that cytomegalovirus (CMV) DNA and Epstein–Barr virus (EBV) DNA were not present in the blood. Antiviral therapy was given acyclovir (0.4 Po Bid) to prevent herpes virus infection. Although the patient's temperature returned to normal and abdominal pain relieved after anti-infection treatment, the diarrhea did not alleviate. Stool screening showed no Clostridium difficile infection. Fecal neutrophils were not found in stained smears of diarrheal stools. We did not observe obvious improvement after we treated the patient with Smecta combined with sulperazone for three days. The ratio of cocci to bacilli was 9 : 1 by stool smear. One week after cessation of medical treatment, we attempted to use FMT to treat the diarrhea. |
pmc-6462374-1 | Case 1: An 71-year-old male patient had been admitted to our department with two-week history of gradually increasing headache and slight limb activity disorder and a 2-month history of minor head trauma as a result of fall to the ground. Cranial CT revealed bilateral CSDH. There was hypertension and multiple lacunar infarction were the systemic diseases which were under normal control (). Chest radiography, electrocardiogram and the blood system examinations were completed preoperatively. Fortunately, the major data was not exceptional remarkably and preoperative coagulation parameters were within normal limits. The patient underwent an urgent operation of burr hole drainage of CSDH in bilateral frontal-temporal region. During the operation, subdural hematoma launched from bone hole after the opening of the dura in spite of over high intracranial pressure (ICP). The procedure was completed without any obvious accident and the vital signs remained stable during operation. Eventually, a subdural closed system of drainage was placed when intracranial pneumatosis was eliminated absolutely. Because of the exacerbation of conscious disturbance, emergent CT scan was taken at 2 hours postoperatively and revealed multiple parenchymal hemorrhage. Postoperative recovery was uneventful. Surgical intervention was not adopted, and the patient kept to conservative treatment including continuous neurological observation for 6 days in the NICU. The hospitalization time was prolonged to 20 days because of mental disturbance after the operation which was corrected successfully in that interval. Finally, the patient was discharged from hospital with no neurological deficit except for slight language barriers. At 2-month follow-up no abnormality was noted, and the CT scan revealed hematoma was absorbed completely (). |
pmc-6462374-2 | Case 2: in case 2 we reported an 82-years-old man had been admitted to our department due to limbs weakness presented progressive aggravation with undergoing frequent falls in the previous month and a history of cerebral infarction, arterial hypertension and coronary heart disease with long-term oral rivaroxaban. The CT scan showed a right equi-density subdural hematoma and brain tissue compression. The neurologic evaluation revealed slow response and weakness of the left limbs (). The urgent evacuation of the subdural hematoma was performed with a single parietal burr-hole craniectomy on the right side without discontinuing oral rivaroxaban. A subdural drainage catheter was positioned in prefrontal region routinely. Surprisingly, we observed right visual field defect the following day and vital signs was remained within normal range all the time. The post-operative CT scan showed a bit of prefrontal pneumocephalus and right occipital lobe hemorrhage and no acute bleedings in ipsilateral subdural space. The drainage tube removal was delayed to the fourth day. Meanwhile, the CT findings occipital hematoma appeared absorbed gradually and did not require surgical intervention. The neurologic evaluation revealed a slight postural instability and for this reason that the patient was arranged to physical therapy. At two-month follow-up, the patient was able to walk unaided, and the review CT scan was normal (). |
pmc-6462374-3 | Case 3: in case 3 we reported an 80-years-old man had been admitted to our department due to moderate dizziness and headache for 20 days with a history of hypertension, ischemic heart disease, chronic obstructive pulmonary disease (COPD) and asthma. Neurological assessment confirmed mental confusion and gait imbalance without focal deficits. The CT scan revealed a sizeable right chronic subdural hematoma which caused midline left shift (). The patient underwent an instant single burr-hole craniectomy and the right subdural hematoma evacuation was satisfactory largely. A subdural drainage apparatus was positioned routinely. On the second day after surgery, sudden airways spasm occurred and blood oxygen saturation presented unstable within a short time. Emergency tracheal incubation was implemented to maintain vital signs. Simultaneously, the drainage device was closed and a emergent CT scan was performed and revealed the presence of a small area occipital intraparenchymal hemorrhage. On the third day, the patient was transferred to the ICU and assisted respiration with ventilator postoperatively. Subsequently, the patient was required tracheotomy due to difficulty of removing trachea cannula within a short time. The drainage tube removal was delayed to the fifth day postoperatively. After one week, the patient successfully detached from the respirator and returned to general ward to continue anti-infective treatment for 10 days. Finally, the patient discharged from hospital with mild pulmonary inflammation, but therapeutic process was uneventful, and the total hospitalization time was up to one month. The two-month follow-up showed the patient was in good conditions and life-independent. The CT scan of the head revealed no recurrence (). |
pmc-6462797-1 | A 35 year old healthy lady presented to our department on sixth postoperative day [POD] with an external biliary fistula and intra-abdominal sepsis. She had undergone laparoscopic converted to open cholecystectomy for acute calculous cholecystitis. She had a biliary injury that was identified intra-operatively, managed by Roux-en-y hepaticojejunostomy[RYHJ]. The anastomosis leaked. An interno-external percutaneous transhepatic biliary drainage[PTBD] extending across the leak was performed at our hospital on POD 7 for both right and left hepatic ducts.
On POD nine, she had an upper gastrointestinal bleed. Esophagogastroduodenoscopy and Contrast enhanced computed tomography [CECT] abdomen did not reveal the source of bleeding. On conventional hepatic arteriogram, a leaking cystic artery pseudoaneurysm was identified (). During angioembolisation, due to a short stump of cystic artery, coils were placed in right hepatic artery [RHA]. However, one of the coils accidentally migrated into the left hepatic artery [LHA] and could not be retrieved. LHA stenting was performed, with good flow of contrast across the stent (). However, LHA developed spasm in its distal part, resulting in complete block of LHA and RHA.
On first day after coiling, there was significant elevation of liver enzymes with features of ischemic hepatitis. CECT abdomen with arteriography revealed poor enhancement of hepatic arterial tree in the segmental branches with partial revascularization from inferior phrenic and retroperitoneal arteries. The patient’s relatives were explained the possibility of a need for an emergency liver transplant. The patient improved over the next 48 h, was transferred out of intensive care unit and oral feeds were started. Abdominal drain was removed after it stopped draining bile. She was discharged on POD 28 with PTBD catheters in situ. On POD 33, her liver function tests were within normal limits, percutaneous transhepatic cholangiogram showed trickle of contrast across the RYHJ, and the PTBD catheters were clamped. PTBD catheters were kept longer than 6 months and intervening periodic cholangiograms revealed anastomotic narrowing. Balloon dilatation of the stenosed anastomosis was performed on multiple occasions. Liver function was normal all along.
Fourteen months after surgery, cholangiogram revealed a worsening of RYHJ stricture (). A CECT abdomen and conventional angiogram performed at 18 months showed a blocked RHA and LHA. Multiple collaterals were seen arising from the right inferior phrenic artery, retroperitoneum and along the hepatoduodenal ligament (). In view of the collateral formation and persistent tight stricture with failed multiple dilatations, a surgical revision of the anastomosis was planned. Prior to surgery, right PTBD catheter was maneuvered from right to left duct across the hilum, draining externally.
During surgery, utmost care was taken not to release any adhesions in the hepatoduodenal ligament and not to mobilize liver from the diaphragmatic attachments to preserve all collaterals. Hilar dissection was kept to a minimum and the PTBD catheters guided the identification of biliary hilar confluence. A minor segement IV hepatotomy was done to expose the left hepatic duct and the roof of biliary confluence. Previous anastomosis was resected, and a Roux loop was prepared. A 2 cm single layer, interrupted 5-0 polydioxanone RYHJ was fashioned to the anterior wall of the biliary confluence with an extension onto the left duct.
Her postoperative recovery was uneventful. Cholangiogram on POD 10 showed a good flow across the anastomosis with no sign of a leak, and she was discharged with a clamped PTBD catheter. The PTBD catheter was removed 6 weeks after the surgery. The patient is doing well at 1 year follow up. Liver function is normal. She is being followed up to evaluate for secondary patency. |
pmc-6462801-1 | A 38-year-old pregnant woman (8 weeks’ gestation) presented with acute onset right upper quadrant pain. On abdominal examination, the Murphy’s sign was positive. She had leukocytosis. Ultrasonography showed features of acute cholecystitis and choledocholithiasis, which was later confirmed by MRI cholangiogram (A). ERCP was skipped due to the possible irradiation damage of developing fetus. Emergency combined LC and Lap ECBD was performed. Standard laparoscopic approach for LC was adopted. During the operation, there was gangrenous changes of inflamed gallbladder. (B). Cystic duct - common bile duct junction was defined. Dissection and isolation of 1.5 cm cystic duct was performed. Common bile duct exploration through transcystic duct approach was adopted. Choledocholithiasis was removed by endoscopic basket under the guidance of choledochoscopy (C, D and E). After ligated and divided the cystic duct and artery, the gallbladder was removed and a drain was placed beside the cystic duct stump. The operation time was 110 min and the blood loss was 30 ml. The drain was removed on postoperative Day 2, the patient recovered well and was discharged on postoperative Day 4. A healthy baby boy was delivered at 40 weeks’ gestation and no developmental problem was noted up to 3 years for the baby delivered. |
pmc-6463096-1 | A 59-year-old Brazilian man presented cachexia and disseminated and ulcerated skin lesions with one-year evolution (A). Before his illness, he worked as an agriculturist, truck driver and a sewerage system cleaner in his town. During his last professional activity, he was continuously exposed to polluted water. Eight months earlier, the diagnosis of leprosy was made without any microbiological evidence and he was unsuccessfully treated with rifampin, dapsone and clofazimine. Six months ago, HIV infection was detected and lamivudine, tenofovir and efavirenz were added. At admission, he was depressed, febrile and complaining of pain. His body weight was 40 kg, and, besides the cutaneous clinical manifestations, there were no signs of internal organ involvement. The main laboratory findings included anemia with hemoglobin of 9.1 g/dL, leukocytosis (12,100 cell/μL) and protein chain reaction (PCR) of 11mg/L. HIV test was positive with CD4 cell count of 584 cells/mm3and viral load of 1558 copies/mL (log 3.1). A skin biopsy depicted a mixed exudative and granulomatous cellular infiltrate with a few round to elongated yeast cells (B). The cultures of biopsy fragments yielded a dimorphic fungus phenotypically identified as Sporothrix sp., later identified by DNA sequence as S. schenckii. The anti-lepromatous therapy was stopped and the patient was treated with itraconazole, 400 mg per day, and cotrimoxazole 360mg/800mg per day for secondary bacterial infection. Because IRIS was suspected, prednisone at the daily dose of 20 mg per day was added and ART was changed to atazanavir/ritonavir due to probable drug-to-drug interactions between itraconazole and the previous antiretrovirals. He improved gradually and corticosteroid and cotrimoxazole were discontinued. After three months of therapy, itraconazole was reduced to 200 mg per day and discontinued after six months. The patient presented complete clinical and mycological responses. (C). |
pmc-6463145-1 | A 27-year-old female patient with a simple vascular malformation—described as ‘lymphangioma’ []—at the level of the right thigh and groin was referred for treatment (a). The lesion had been present since birth and had been partially resected at the age of 15 years. The large, but incomplete, excision had resulted in lymphedema of the right leg and thigh. At the time of referral, there were ongoing complaints of swelling of the right limb and a feeling of tension, particularly at the level of the groin. The volume difference between the affected and unaffected leg was 1222 mL, measured by an optoelectronic limb volumeter (Perometer, Pero-System, Wuppertal, Germany) [].
Conservative treatment by means of manual lymph drainage and compression garments was initiated; however, results were unsatisfactory. Given the refractory swelling and continued complaints of a feeling of pressure at the level of the groin, and given the applied gross excision in the past, a minimally invasive surgical intervention was planned.
Lymphatic mapping was performed by means of lymphography (PDE Neo II, Hamamatsu, Japan), with the fluorescent marker indocyanine green (ICG). Patent lymphatics at the medial side of the thigh, visualized by near-infrared technology, were marked on the skin [].
A lymphoscintigraphy image of the lower extremities showed a pronounced uptake of radioactive tracer on the medial side of the thigh (a). The combined examination of the single photon emission tomography (SPECT) with computed tomography (CT) in a hybrid system (SPECT-CT and lymphoscintigraphy) revealed an accumulation of radio-colloid tracer along the course of lymphatics at the medial side of the thigh, pointing to either lymph nodes, a lymphocele, or a lymphatic malformation (b).
Finally, the images from the SPECT-CT and lymphoscintigraphy were analyzed with a virtual reality program (Medicalholodeck, Zurich, Switzerland) [] that allowed the visualization in a 3D model of the localization of the lymphatic malformation (a, ), with its extension into the abdominal cavity, in a virtual reality environment (b, ).
Based on the 3D model and the mapping by ICG lymphography, the incision site was determined (a), and an end-to-end anastomosis between a patent lymphatic vessel (diameter 0.5 mm) and an adjacent vein (diameter 0.6 mm) was performed using an 11/0 suture (b). The patency of the lymphovenous anastomosis was confirmed intraoperatively by ICG lymphography. Postoperative compressive bandaging was applied. The procedure was performed under loco-regional anesthesia.
The postoperative course was uneventful. Immediately after surgery, the feeling of tension disappeared and the volume of the right thigh decreased (b). Four months postoperative, the result was very good, with further reduction of the volume of the right leg; the difference in volume between the left and right leg was reduced to 224 mL (Perometer 400 NT) [] (c).
The conventional images of the SPECT-CT and lymphoscintigraphy, stored as DICOM data, were processed by dedicated software []. The DICOM viewer of Medicalholodeck allowed us to handle the DICOM data and merge them into a single virtual reality model. In order to visualize this 3D patient-specific model, a virtual reality headset (Vive Pro, HTC corporation) and a computer with a powerful graphics card (GTX-1080ti) and a large memory (32 GB RAM) were needed. The result was a detailed 3D image of the anatomical structures and, more precisely, of the lymphatic malformation in a virtual reality environment (a,b, ).
The treatment was conducted in accordance with the Declaration of Helsinki and under the institutional ethical review board of the hospital. Written informed consent was obtained. The patient provided written informed consent for the publication and the use of the images. |
pmc-6463226-1 | A 7-month-old boy (8 kg), immunized for age, was admitted with complaints of fever, cough for 5 days, and fast breathing for 1 day. There was no significant past history and normal birth and developmental history. On examination, vitals were as follows: heart rate: 182/min, blood pressure: 85/55 (65) mm Hg (at 50th centile), temperature: 101.3°F, respiratory rate: 85/min, and oxygen saturation (SpO2) (80% on room air and 92% on oxygen by mask at 5 L/min), with anasarca. In the systemic examination, the child was in respiratory failure with crepitation heard over right side of interscapular area. Other systems were normal. Bedside lung ultrasound showed shred sign at the above-mentioned area. Initial diagnosis of bronchopneumonia was made and the child was intubated (4 mm cuffed tube, fixed at 13 cm), started on midazolam, fentanyl infusion, and antibiotics. The child was started on synchronized intermittent mandatory ventilation (SIMV) pressure-regulated volume control (PRVC) mode with settings: tidal volume: 8 mL/kg, PEEP: 8, plateau pressure: 25, rates: 45/min, SpO2 98% on inspiratory oxygen fraction (FiO2) 0.4. Positive end-expiratory pressure was chosen as per best compliance and FiO2. The clinical condition later deteriorated in the form of desaturation (SpO2 88% on FiO2 0.6), worsening chest wall edema and abdominal distension. Intra-abdominal pressure was increased to 18 cm of H2O. To improve oxygenation, the child was ventilated as per the acute respiratory distress syndrome (ARDS) net protocol (low tidal volume and PEEP titration were done by a conventional method from 8 to 15), but there was no improvement in oxygenation. At the PEEP of 15, further titration was abandoned and further adjustment was done by measuring the TPP. The TPP and esophageal pressures were recorded after inserting the esophageal catheter withholding expiration and inspiration for brief periods. By giving a 3-s hold at end-inspiration, transpulmonary plateau (Ptp plat) was recorded, and holding at end-expiration, transpulmonary peep (Ptp peep) was recorded. Esophageal pressure which corresponds to the Ppl was recorded as 20 cm of H2O ( and ). To attain Ptp plat between 10 and 15 cm of H2O and Ptp peep in the range of 0 to 2 cm of H2O, PEEP was increased from 15 (from conventional methods) to 20. Subsequently, the oxygenation improved without hemodynamic instability (). Considering worsening renal functions and fluid overload, renal replacement therapy was started in the form of peritoneal dialysis (PD). Positive end-expiratory pressure was also titrated according to the intra-abdominal pressure during PD cycle “in and out” targeting the Ptp plat and Ptp peep. As fluid overload persisted despite PD, continuous renal replacement therapy (CRRT) was done. Slowly, renal functions improved, fluid overload decreased, and the child was extubated on the 7th day. He was discharged 2 weeks after hospitalization and is currently doing well on follow-up. |
pmc-6463257-1 | We present the case of a 45-year-old Caucasian man suffering from psoriasis for more than 20 years at the time of the first visit. No relevant comorbidities were found at history collection, apart from benign prostatic hyperplasia, for which he is treated with tamsulosine. He was initially diagnosed and followed-up by dermatologists, who treated him with topical corticosteroids and salicylic acid for skin involvement and systemic corticosteroids for resistant lesions as needed ().
The skin component had always been <30% of the total body surface area (BSA). The patient reported distress in personal and social relationships with consequently reduced HRQoL. After ten years, he started experiencing dactylitis of feet and recurrent arthritis of the small joints of hands, shoulders and knees, together with inflammatory morning stiffness lasting around one hour, with negative rheumatoid factor (RF); thus fulfilling ClASsification criteria for Psoriatic ARthritis CASPAR criteria for PsA []. He was referred to the rheumatology outpatient clinic of another hospital and treated with indomethacine and systemic corticosteroids as needed for about five years, and then achieved complete remission of joint symptoms. Three years later, because of a flare of joint involvement (episodes of peripheral arthritis and dactylitis of the small joints of hands and feet once per week) and skin involvement, he was started on methotrexate 10 mg weekly with folate supplementation, in combination with cyclosporine 200 mg daily, with reduced extension and severity of skin lesions and decreased frequency of arthritis flares to once per month. However, he did not reach complete remission. Shortly after initiation, due to a 2-fold increase of liver enzymes (hepatitis excluded based on abdomen ultrasound and hepatitis B and C viral profile), methotrexate was reduced to 7.5 mg weekly []. The reduced methotrexate regimen led to normalization of the liver enzymes but was ineffective in controlling both skin and joint involvement. Two years later, due to elevated arterial pressure values (up to 150/90 mmHg) and serum creatinine increase >30% of baseline value (up to 1.4 g/L), cyclosporine was stopped.
The following year, he referred to our outpatient clinic for skin and joint flare. At referral, physical examination demonstrated mild psoriasis of trunk, arms and legs, with a psoriasis area severity index (PASI) score of 5.1 (), dactylitis of the third digit of the left foot and arthritis of the third interphalangeal joint of the left hand, which, together with a normal C reactive protein (CRP), activity visual analogue scale (VAS) and pain VAS of 4 and 4.5 respectively, accounted for a disease activity in psoriatic arthritis (DAPSA) score of 12 (low disease activity) [].
Laboratory tests showed no relevant alterations. Radiographic assessment of hands and feet showed no erosions and no signs of axial involvement were found at magnetic resonance imaging (MRI). Ultrasound imaging of the third metacarpophalangeal (MCP) joint showed inflammation of the joint and of the peritendon of the extensor tendon (a).
Considering that the patient failed to respond to two DMARDs (cyclosporine and methotrexate), that he refused a parenteral drug, that he presented mild skin and joint involvement, no bone erosions, dactylitis and no axial involvement, apremilast was chosen [,,]. At baseline, the EQ-5D questionnaire reported altered HRQoL, with some difficulties in motility, no difficulties in self-care and some difficulties in usual activities, moderate pain/discomfort and moderately anxious/depressed mood. Patient’s global health (GH) VAS was 60 and pain VAS was 45 (). Furthermore, the patient was screened at baseline for CV risk; carotid arterial doppler ultrasound demonstrated only intimal thickening of the left bulb and 24 h blood pressure monitoring demonstrated mild systolic and diastolic daily hypertension for which he started lercanidipine 10 mg/daily. At baseline, total cholesterol (TC) and triglyceride (TG) serum levels were 173 mg/dL and 113 mg/dL respectively, and TC/high density lipoprotein (HDL) ratio was 4.94 (). After 4 weeks, the extension and severity of erythema and infiltration of psoriatic plaques were substantially reduced (PASI 2.5, ). Furthermore, the patient reported a marked improvement of joint involvement with no further episodes of arthritis or dactylitis, although inflammatory morning stiffness persisted (DAPSA 6.0). At the 2-month follow-up, the patient reported several side effects such as mild headache, dizziness and hypotension and, due to further amelioration of the skin and joint involvement he stopped apremilast, which was restarted shortly after, due to worsening of psoriasis (PASI 3.0) and occurrence of a new joint flare (DAPSA 10). At the time he was still taking methotrexate 7.5 mg weekly. At the 6-month follow-up he reported no arthritis flares and no inflammatory morning stiffness. At physical examination, the third MCP joint was swollen but not tender (DAPSA 3), and ultrasound imaging showed reduction of joint and soft tissue inflammation (b). A further improvement of skin involvement was observed and reported by the patient (PASI 1.2, ). During the follow-up, we observed an amelioration of the serum lipid profile already after 4 weeks, with a 5.8% reduction of TC and a reduction of TC/HDL ratio, and after 12 months, a further reduction of TC, LDL and TG values of 15.6%, 25.7% and 17.7% respectively, and a 20% increase of HDL levels compared to baseline values. TC/HDL ratio reached the favorable value of 3.48, as shown in . We even observed a 5% weight loss—although our patient already had a normal baseline weight and body mass index (BMI)—reaching a final normal weight with a normal body mass index (BMI 21.5). After 12 months, the clinical manifestations were stable. We therefore decided to stop methotrexate, but the patient experienced a slight relapse of both skin and arthritis (PASI 2.5, DAPSA 6), shortly after. Therefore, although dosage was low (7.5 mg), we reintroduced methotrexate, leading at the 18-month follow-up to stable minimal disease activity with PASI 75 and joint remission (PASI 1.2, DAPSA 2) [,] and a dramatic improvement in HRQoL (). |
pmc-6463431-1 | A 75-year-old G5P5 patient who had been postmenopausal for 23 years was admitted with symptoms of vaginal bleeding. The patient additionally had type 2 DM and hypertension. A gynecologic examination revealed normal external genitalia, atrophic collum, intact adnexa, and free parametrium. Laboratory test results were as follows: CA125: 82 U/mL, CA19-9: 42 U/mL, and glycated hemoglobin (HbA1c): 11%. Transvaginal ultrasound revealed linear endometrium, minimal intracavitary fluid, and a 26x28 mm hypodense lesion extending to the serosa with no adnexal pathology. Abdominal computed tomography revealed no pathology in the liver, spleen, kidney, small and large bowels, and ovarian loge. Endometrial cavity had a heterogeneous appearance, and no intra- and retro- peritoneal pathologic lymph node was detected ().
Endometrial biopsy established the diagnosis of mixed EC [GCC (structural grade 3, and nuclear grade 3), and EC (structural grade 2, nuclear grade 2)]. Immunohistochemically, vimentin, and EMA produced widespread staining in the lesion (). The histologic feature is bizarre multinucleated giant cells admixed with mononucleate tumor cells ( and ). Both tumors were stained with P53 focally, and ER dye stained areas of the EC. The tumor did not stain with P16, CEA, beta HCG and P63, desmin, MyoD1, CD10, caldesmon, and cyclinD1.
The results of cytokeratin staining were as follows: microscopic examination revealed large geographic tumor necrosis, multinuclear and mononuclear giant cells, and atypical mitosis. Therefore, endometrial neoplasms involving giant cells were considered and differential diagnosis included carcinoma, carcinosarcoma, leiomyosarcoma with osteoclast-like giant cells, undifferentiated sarcoma and choriocarcinoma with osteoclast-like giant cells. B-HCG was administered immunohistochemically and a negative reaction was observed. AE1/AE3 also showed a positive reaction in giant cells.
The patient underwent laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, omental biopsy, and bilateral pelvic, and paraaortic lymph node dissection. The intraoperative frozen section result was reported as a tumor with a size of 3.8 cm, and more than half of the myometrium was invaded. Postoperative follow-up of the patient was uneventful, so she was discharged. The final histopathology report indicated a 3.8x2x9 cm GCC variant of EC and one positive external iliac lymph node metastasis. Cytology of intraabdominal specimens was unremarkable. Administration of adjuvant carboplatin and paclitaxel chemotherapy was planned upon the decision of the multidisciplinary council. |
pmc-6463606-1 | A 58-year-old menopausal woman, para 6+0, presented to the gynecology clinic with progressive abdominal swelling of three-year duration. There was no associated pain, vaginal bleeding/discharge, weight loss, gastrointestinal symptoms, or respiratory difficulties. She was eight years postmenopause. She was a known diabetic on oral medication and had been previously managed medically for uterine fibroids prior to menopause. She had no history of previous surgery. She had no family history of malignancy: breast, ovarian, or endometrial.
Examination revealed a markedly distended abdomen that moved with respiration. An abdominopelvic mass that extended to the xiphisternum was palpated. It was firm, nontender, and slightly mobile in the transverse plane. Other organs were not palpable and there was no demonstrable ascites.
Haemogram, electrolytes, urea, creatinine, and liver function tests were all normal. Serum Ca-125 was 21.3u/ml. Urinalysis showed no abnormality. Chest X-ray and ECG were normal. Abdominopelvic ultrasound scan showed a bulky nongravid anteverted uterus with multiple uterine fibroids with degenerative changes and a mid-line echo. A huge mass extending up to the epigastrium with mixed echogenicity and areas of cystic changes was seen. The liver, gall bladder, spleen, pancreas, and kidneys were grossly normal without focal mass lesion. No ascites was seen. The ovaries were not visualized.
Abdominopelvic computerized tomographic scan with intravenous contrast (Figures and ) showed a large heterogenous predominantly necrotic mass measuring about 22.4 x 16 x 25cm extending from the mid-pelvis to the level of the epigastric region, mild bilateral hydroureteronephrosis due to compression of the mid-ureters by the abdominopelvic mass and mild ascites were noted.
She had exploratory laparotomy and findings at surgery were a huge mass with cystic and solid areas filling up the entire abdominal cavity up to the epigastrium pushing the bowels loops up and to the left (). It derived its blood supply from the greater omentum to which it was attached with huge visible arteries and veins. Enlarged lymph nodes were noted in the gastrocolic ligament. The uterus had multiple myomas, but other structures (ovaries, fallopian tubes, liver, spleen, and intestines) were normal.
She had excision of the huge parasitic fibroid, partial omentectomy, total abdominal hysterectomy and bilateral salpingo-oophorectomy. The histology report () confirmed leiomyoma with cystic degeneration and lymph nodes with reactive lymphoid hyperplasia. |
pmc-6463652-1 | The patient was a 67-year-old male non-smoker with an unremarkable past medical history. He was admitted to our hospital with a complaint of increasing dyspnea for 3 months. His parents managed a workshop in a small town, and he had worked in processing aluminum and brass to make camera parts since he was 18 years old. He did not wear a dust respirator and worked closely to the cutting machine (Fig. ) because of his poor eyesight.
On presentation, the patient was fully conscious with a height of 160 cm, a body weight of 47 kg, body temperature of 36.8 °C and percutaneous oxygen saturation (SpO2) of 94% on room air. Physical examination revealed fine crackles upon auscultation, especially on the bilateral upper lung areas. Otherwise, there were no abnormal physical findings. Laboratory data demonstrated elevated serum levels of Krebs von den Lungen-6 (KL-6; 1631 U/mL) and surfactant protein D (SP-D; 157.3 ng/mL), compared to normal values of < 500 U/mL and < 110 ng/mL, respectively. Antinuclear and autologous antibodies were all negative. Chest X-ray showed linear and reticulonodular shadows with marked bilateral pleural thickening in the upper lung fields (Fig. ). Chest CT indicated bilateral dense sub-pleural consolidation, bronchiectasis and ground glass opacities in the bilateral upper lobes (Fig. ). Pulmonary function tests showed a restrictive disorder: vital capacity (VC) of 1.12 L (34.6% of predicted), forced expiratory volume in 1.0 s (FEV1) of 0.99 L (42.5% of predicted), FEV1/FVC (forced vital capacity) of 74.4% and total lung capacity (TLC) of 2.48 L (47.7% of predicted). The patient was unable to perform the diffusion test correctly. TBLB was performed with bronchoscopy to get tissue samples of the right upper lobe (B2). Bronchoalveolar lavage fluid, obtained by infusing 150 mL of saline, revealed a total cell count of 6.0 × 104/mL, comprised of 3% lymphocytes, 1% neutrophils, 2% eosinophils and 94% macrophages (Cluster of Differentiation, CD 4/8 ratio of 2.31), which was in the normal range. The biopsied specimen had fragments of bronchiolar wall with infiltration of lymphocytes in interstitial spaces; unfortunately, it did not contain alveolar tissue. A surgical diagnostic procedure was avoided due to the patient’s critical respiratory condition and instead, elemental analysis was performed on the existing specimen obtained by TBLB. The content of aluminum and other particles were measured in the TBLB sample.
On elemental analysis of the biopsy specimen, a relatively high amount of aluminum was detected in comparison to trace amounts of other elements, including iron and silica (Fig.). Based on the histological findings, the elemental analysis and the occupational history, the case was diagnosed as airway aluminosis. The patient’s condition was progressively getting worse; consequently, home oxygen therapy was implemented and the anti-fibrotic agent pirfenidone was administered. Six months later, a pneumothorax occurred and chest drainage was performed; however, his condition deteriorated, and he died of hypercapnic respiratory failure 1 month later. His family did not agree to an autopsy. |
pmc-6463815-1 | A 4-year-old boy with history of an accidental ingestion of a metallic nail presented to the emergency department few hours after the ingestion and the examination was normal at that time, the plain abdominal X-ray confirmed the presence nail in the abdomen. The parents were reassured about the possibility of the spontaneous passage of the nail with the stool and they have been advised to visit the hospital if the child develops any kind of symptom. One week later he presented with right iliac fossa pain and one attack of vomiting.
During abdominal examination, the abdomen was not distended and soft, there was tenderness and rebound tenderness at the right iliac fossa, the bowel sounds were normal and no palpable mass detected.
Plain abdominal X-ray of the abdomen showed the metallic nail in the region of the right iliac fossa, with no other abnormal finding, . Ultrasound examination of the abdomen showed no fluid collection and there was tenderness on putting the probe on the region of the right lilac fossa.
The white blood cell count was elevated (14,000 cells per microliter).
Before surgery we suspected impaction at the ileocecal junction and if surgery is delayed it may lead to bowel perforation. During surgery surprisingly the nail was impacted the lumen of the vermiform appendix causing inflammation , .
Appendicectomy done and the patient admitted for two days with no postoperative complications.
The patient discharged on the third day in a good general condition. |
pmc-6464141-1 | An 18-year-old male presented to the emergency department after an unwitnessed dive into the shallow waters of a swimming pool while inebriated where he remained submerged for two minutes. Upon extraction, the patient briefly received chest compressions and was resuscitated. The patient complained of shortness of breath, neck pain, and decreased sensory and motor function in the upper and lower extremities.
In the emergency department, the patient was awake, alert, and fully oriented. His motor exam was 5/5 in the elbow flexors and adductors, 3/5 in elbow extensors, 0/5 in finger flexors and abductors with no motor function in bilateral lower extremities. The patient demonstrated a thoracic sensory level at T7. The patient was areflexic, with no clonus or Babinski signs and the patient exhibited priapism. The patient’s clinical examination was consistent with complete spinal cord injury (SCI). This injury represents a class A injury according to the American Spinal Injury Association (ASIA) impairment scale.
Computed tomography (CT) of the cervical spine showed a burst fracture of the C6 vertebral body with retropulsion causing severe central canal stenosis with bilateral laminar fractures and inferiorly displaced spinous processes of the C4, C5, and C6 levels (Figures , ). Magnetic resonance imaging (MRI) of the cervical spine demonstrated herniation of the spinal cord into the vertebral body of C6 through the burst fracture. This was associated with significant T2 and short T1 inversion recovery (STIR) sequence signal hyperintensity of the central spinal cord from C4 through C7 with foci of gradient echo blooming at C5 and C6 and was indicative of spinal cord contusion and intramedullary hemorrhage (Figures , ).
The patient underwent a C6 corpectomy and a C5-C7 anterior interbody arthrodesis with an anterior plate from C5 to C7. Intra-operatively, as the bone fragments were resected, the spinal cord was visualized and a traumatic dural tear was observed with obvious CSF egress. On retrospective review of the MRI, spinal cord herniation into the vertebral body fragment prior to the procedure was noted. The dural defect was closed with a layer of DuraGen (Integra, Plainsboro, NJ) and Tisseel (Baxter, Deerfield, IL). After completion of the anterior procedures, The patient was then placed in the prone position to undergo a C3-T2 posterior lateral fusion followed by C4-C7 laminectomy. No dural lacerations, traumatic lacerations, or CSF was seen posteriorly.
Given the inability to primarily repair the traumatic dural tear, a lumbar drain was used to divert CSF for one week to allow healing of the dural repair. The patient’s hospitalization was complicated by acute respiratory distress syndrome (ARDS) and required multiple bronchoscopies and placement in a Rotoprone bed (Arjo Inc, Addison, IL). The patient subsequently underwent a tracheostomy and feeding tube placement prior to discharge to an acute inpatient rehabilitation facility. The patient completed a six-week course of inpatient rehabilitation and was last seen for follow up at 18 weeks post-injury. At this follow-up, he reported 4+-5/5 strength in bilateral deltoids, 4/5 in bilateral biceps, 3/5 in the right triceps, and 2/5 in the left triceps as well as some regained sensation in the lower extremities. He continues to participate in outpatient physical and occupational therapy. |
pmc-6464145-1 | We report a case of a 47-year-old male patient from Mali, West Africa, who presented with intermittent fever, chills, increased fatigue, decreased appetite, and diffuse back and abdominal pain for three weeks. He reported a 35-pound weight loss over three months. He had a history of malarial infection six months before, which was treated. He endorsed traveling through Africa until a recent illness. At the time of admission, his temperature was 102.3 F, blood pressure was 103-110/59-64 mmHg, and heart rate was 92-113 bpm, saturating at 99% on room air. A blood examination showed a hemoglobin level of 4.4, a mean corpuscular volume (MCV) of 87.5, a platelet count of 13, a white blood cells (WBC) count of 6.4, and an international normalized ratio (INR) of 1.73. Other values included serum sodium 132, lactate 4.6, alkaline phosphatase 133, total bilirubin 1.9, direct bilirubin 1.6, and albumin 2.8. The malarial screen was negative. A chest X-ray showed bilateral lower lobes infiltrates consistent with pneumonia. A computed tomography (CT) scan of the chest, abdomen, and pelvis showed mild bilateral areas of focal infiltrates and consolidation consistent with pneumonia and small right scrotal hydrocele. A peripheral blood smear (Figure ) showed few monoblasts, few dysplastic features, and erythrocytes with intracellular inclusions. He received supportive care with blood and platelet transfusions. He was treated with intravenous ceftriaxone and intravenous azithromycin for community-acquired pneumonia and atovaquone for malaria.
A bone marrow aspirate was done (Figure ), and the smear showed hypercellular marrow with monoblasts, erythroblasts, maturation defect, and few megakaryocytes. Further examination showed hypercellular marrow for age with sheets of immature mononuclear cells (blasts and monocytes) diffusely occupying marrow cavities. Table shows the differential count of the bone marrow cells. Blasts comprised approximately 50% of marrow elements, confirmed by immunostaining with CD34 and CD117. Monocytes comprised approximately 20%-30% of marrow elements. The blasts were medium to large with increased nucleus-to-cytoplasm (N/C) ratio, fine chromatin, and prominent nucleoli.
Table presents the bone marrow differential count.
These results were consistent with a diagnosis of acute myeloid leukemia (non-APL), best classified as acute myelomonocyte leukemia (AMML). Flow cytometry analysis from the aspirate showed blasts (12%-15%) that were positive for CD34 (partial), CD117, HLA-DR, CD13, CD33, and CD38. Monocytes (~25%) were positive for CD2, CD4, CD11b, CD11c, CD13, CD14, CD33, CD38, CD45, CD64, and HLA-DR. CD56 was negative.
CCAAT/enhancer-binding protein alpha (CEBPA) mutational analysis was not detected. A fluorescence in situ hybridization (FISH) test (Figure ) showed no evidence of RARA rearrangement, no evidence of BCR/ABL rearrangement, no evidence of PML/RARA gene rearrangement, and no evidence for RUNX1/RUNX1T1 rearrangement; however, a subset of cells showed an abnormal hybridization pattern, consistent with gain of 8q or trisomy 8 (Figure ). There was no evidence of MLL gene locus 11q23 translocation and no evidence of CBFB [inversion (16) or translocation (16;16)] gene rearrangement.
OnkoSight TM (Bio-Reference Laboratories, Inc., NJ, USA) NGS AML panel sequencing identified a frameshift mutation in ASXL1 (p.Gly646Trpfs*12), a frameshift mutation in RUNX1 (p.Tyr281Leufs*319), and a hotspot missense mutation in DNMT3A (p.Arg882His). Cytogenetics revealed an abnormal male karyotype with tetrasomy of 8 (49, XY, 8+, 8+) (Figure ). |
pmc-6464279-1 | A 36-year-old female patient presented for recurrent abdominal pain and her past medical history was negative except for a cesarean delivery several years prior to her presentation. The pain was localized to the lower abdomen, crampy, cyclic, and worsened over the last few months. This pain was partially relieved by taking some analgesics. Hormonal therapy was afterwards tried for a month by taking progestins. The patient was no longer able to function properly in her daily tasks due to the pain. A physical exam revealed a slightly tender, non-mobile firm mass near the cesarean scar. A probable diagnosis of abdominal wall endometriosis was made. A computed tomography (CT) scan of the abdomen and pelvis with intravenous contrast revealed evidence of a homogeneous mass at the anterior abdominal wall just at the previous cesarean section showing slight enhancement. Under general anesthesia, surgical exploration (Figures , ) revealed a 3×3 cm mass at the right lower rectus wall, and en bloc excision of the mass was performed (Figure ). A pathologic examination showed pieces of benign thick fibrous tissue with multiple endometrial glands and stroma, diagnostic for endometriosis. The patient was seen at a regular interval, up until two months of follow-up and she was symptom free. |
pmc-6464279-2 | A 40-year-old female patient presented with the same clinical presentation of cyclic pain and her past history was negative except for a cesarean delivery several years ago. She noted that the pain was more at the left rectus muscle (away from the cesarean scar). She consulted her obstetrician at first who opted for medical treatment with analgesics and hormonal therapy (oral contraceptives and gonatropin-releasing hormone agonist), which were ineffective for her pain. A diagnosis of endometriosis was confirmed by an ultrasound and CT scan that showed a mid-rectus lesion (Figure ). She underwent excision of the mass and mesh reinforcement was performed. Pathology confirmed the presence of endometrial stroma. The patient was disease-free and seen regularly up until a two-month interval. |
pmc-6464280-1 | A 54-year-old Hispanic male with a medical history significant for chronic obstructive pulmonary disease (COPD), diabetes mellitus, alcohol abuse, heroin abuse (on methadone), hepatitis C, latent tuberculosis, and ventral hernia repair presented to our institution’s emergency department complaining of progressive shortness of breath for one month duration. The patient also complained of increased abdominal girth within the same period of time, which he stated worsened his shortness of breath. Initial workup included a chest X-ray showing left lower lobe atelectasis. Notable laboratory findings included hemoglobin: 12.6 g/dL, white blood cell count: 11.5 103/uL, platelet count: 208 103/uL, creatinine: 1.4 mg/dL, blood urea nitrogen: 24 mg/dL, alanine aminotransferase (ALT): 37 IU/L, aspartate aminotransferase (AST): 52 IU/L, total bilirubin: 0.5 mg/dL, and alkaline phosphatase: 72 IU/L. The patient was then admitted to the medical floor under the impression of COPD exacerbation. On admission, surgery was consulted to evaluate the patient’s increasing abdominal girth given his medical history of ventral hernia. An abdominal CT scan was done, which showed the anterior portion of the left hepatic lobe partially herniating through the ventral hernia accompanied by hepatic steatosis (Figures -). Physical examination revealed the patient in respiratory distress, with a distended abdomen, a midline scar, and a lump protruding through the midline. Vital signs included a blood pressure of 145/95 mmHg, heart rate of 133 beats per minute, and respiratory rate of 30 breaths per minute. Due to the patient’s ongoing medical conditions, repair of the hernia was not done as an inpatient. During his hospital stay, the patient’s respiratory status improved and he was safely discharged home with a follow up appointment to the surgery clinic for hernia repair as an outpatient. |
pmc-6464283-1 | A 27-year-old male patient reported to Southern Railway Headquarters Hospital, Perambur, Chennai, with an alleged history of trauma to the face. He had a loss of consciousness after injury. He had no history of vomiting or posttraumatic amnesia. He underwent a neurological examination, and any possibilities of intracranial hemorrhage or cranial injury were ruled out. He was declared fit to undergo surgical repair of the facial fractures under general anesthesia. He underwent a detailed ophthalmic investigation by an ophthalmologist and was documented as exhibiting normal visual acuity, an absence of relative afferent pupillary defect, and normal eye movements, thus excluding injury to the globe or optic nerve. On extraoral examination, we noted circumorbital edema around the left eye and subconjunctival hemorrhage in the left eye. There was a laceration of the forehead around the frontozygomatic area on the left side. The patient had abrasions over the left zygomatic region. There were no other associated lacerations or abrasions on the face. On intraoral examination, occlusion was intact, and no fracture or mobility of any teeth was noted. The mouth opening was 38 mm. We noted normal temporomandibular jaw movements both in protrusion and lateral movements. There were tenderness and crepitation in the left frontozygomatic region and at the intraoral zygomaticomaxillary buttress region. On radiological investigation, a CT scan revealed a fracture at the left frontozygomatic region, the orbital floor involving the infraorbital rim, along with a fracture of the left zygomaticomaxillary buttress region (Figures , ).
An open reduction and internal fixation of the fractured left orbital zygomaticomaxillary complex was planned under general anesthesia, three days post-trauma. Under general anesthesia, the left zygomaticomaxillary buttress was surgically exposed via a vestibular incision after performing a subperiosteal dissection (Figure ). The fracture at the left frontozygomatic region was exposed via the already existing laceration (Figure ). A retroseptal transconjunctival approach was used to access the infraorbital rim and orbital floor. A Desmarres retractor was used to retract the lower eyelid. To prevent corneal abrasions or tearing and to protect the eyeball, a corneal shield was used. The inferior fornix was held with toothed tissue-holding forceps, and an incision was placed into the palpebral conjunctiva using Colorado tip (Stryker CMF, Chicago, IL, USA) electrocautery between the lowermost point of the eyelid and the inferior fornix (Figure ). Scissors were used to locate the inferior orbital rim, and the periorbital was reached. A subperiosteal dissection was done along the orbital floor to expose the fractured orbital floor using a malleable retractor in a dog paddle maneuver (Figure ). The dissection was continued until a stable posterior ledge of bone was reached on the orbital floor. After achieving surgical exposure of the fracture sites, the reduction was achieved by employing Rowe’s zygomatic elevator. After ensuring adequate reduction, the following fixation was carried out: 1) Fixation of frontozygomatic suture region was performed using two four-hole titanium plates (Synthes) without gap (1.5 mm × 6 mm; Figure ); 2) Fixation of left zygomaticomaxillary buttress using one straight four-hole plate without gap (2 mm × 6 mm); 3) Fixation of orbital floor and infraorbital rim using a preformed orbital titanium mesh (Universal Orbital plate, 0.4 mm Synthes Maxillofacial [Paoli, PA]), that was fixed on to infraorbital rim using 1.5-mm × 6-mm titanium screws (Figure ).
The retroseptal transconjunctival incision was closed using 5-0 Vicryl buried sutures, and a Frost suture was suspended from the lower tarsal plate for seven days postoperatively (Figure ).
The wound at the frontozygomatic region was closed in two layers, namely the muscle layer using 4-0 Vicryl simple interrupted sutures, and the skin layer using 6-0 Prolene simple interrupted sutures. The intraoral zygomaticomaxillary buttress suture was closed using 4-0 Vicryl simple interrupted sutures. The patient was prescribed postoperative topical antibiotic drops (ciprofloxacin) and eye lubricants (carboxymethyl cellulose) for five days, along with cefotaxime and metronidazole intravenous antibiotics and diclofenac sodium intramuscular injection as an analgesic for five days. |
pmc-6464285-1 | A 74-year-old, avid female gardener and active smoker with a past medical history notable for chronic obstructive pulmonary disease (COPD) and hypertension presented to the hospital with one week of progressively worsening New York Heart Association (NYHA) class III dyspnea and fatigue. She endorsed associated cough productive of yellowish mucoid sputum. She denied chest pain/discomfort, palpitations, pre-syncope, syncope, orthopnea, or paroxysmal nocturnal dyspnea (PND). There was no history of fever, arthralgia, myalgia, or rashes.
In the emergency department, the patient was afebrile, blood pressure was 85/49 mm Hg, heart rate was 150 per minute, and respiratory rate was 22 per minute, with oxygen saturation of 94% on six liters of oxygen via a nasal cannula. The patient appeared lethargic and was using accessory muscles for respiration. General examination showed pallor. The oral mucosa was dry, with a thickly coated tongue. The neck veins were flat. Heart examination revealed a fast, irregular heart rate, variable first heart sound, and normal second heart sound without any murmurs or gallops. Lung examination revealed bilateral mid to late inspiratory crackles. The abdomen was soft, distended, non-tender, with normal bowel sounds. Extremities were noted to be cold, with 1+ pitting edema and normal capillary refill time.
Routine laboratory investigations revealed a white blood cell (WBC) count of 7.5 (4.0-10.5 k/uL), hemoglobin of 9.9 (12.5-16 g/dL), and hematocrit of 32.3 (37-47%). Her baseline hemoglobin concentration was around 15 g/dL. Mean corpuscular volume (MCV) was 101.9 (78-100 fL) with elevated mean cell hemoglobin (MCH) of 34 (25-33 pg) and normal mean cell hemoglobin concentration (MCHC) of 33.4 (32-36 g/dL). Platelet count was 34 (150-450 K/uL), with elevated mean platelet volume (MPV) of 12.4 (7.4-11.4 fL). Peripheral blood smear showed intracytoplasmic parasites suspicious for Babesia along with reduced platelets (Figure ). The parasitic level was found to be at 9.04%. Lactate dehydrogenase (LDH) was 1544 U/L (125-220 U/L), haptoglobin was <6 mg/dL (27-139 mg/dL), total bilirubin was 5.4 mg/dL (0.3-1.0 mg/dL), with a direct fraction of 3.5 mg/dL (0.0-0.2 mg/dL). Aspartate aminotransferase (AST) was 202 U/L (5-40 U/L), alanine aminotransferase (ALT) was 90 U/L (7-52 U/L), with albumin of 2.3 g/dL (3.5-5.0 g/dL). Blood urea nitrogen (BUN) was 51 mg/dL (7-17 mg/dL) with a normal creatinine of 0.8 mg/dL, sodium 129 (135-145 mmol/L), potassium 4 (3.5-5.1 mmol/L), chloride 103 (98-107 mmol/L), serum bicarbonate 19 (24-32 mmol/L), and calcium 7 (8.4-102 mg/dL).
The chest X-ray was remarkable for cardiomegaly with a small right pleural effusion and small airspace opacity within the right lower lobe concerning for consolidation or segmental atelectasis. Given her presentation, she was started on intravenous fluids along with empiric antibiotic coverage for community-acquired pneumonia with ceftriaxone and azithromycin. As the peripheral smear was remarkable for Babesia, she was started on atovaquone and doxycycline for a possible co-infection with Lyme. Blood titers for Anaplasma and Lyme were sent. Positive pressure ventilation was started to help with respiratory distress. She received intravenous fluids; however, she continued to remain hypotensive and required norepinephrine for hemodynamic support. Given the severity of her disease and parasitic load, a decision was made to do erythrocytapheresis.
Following erythrocytapheresis, the parasitic load decreased to 5.54%. The patient remained in shock and clindamycin was added to the regimen. Her renal function worsened, which was believed to be secondary to pigment-induced nephropathy from hemolysis. Due to the inadequate response and development of acute kidney injury, repeat erythrocytapheresis was performed, resulting in a reduction in parasite load to 1.75%. At this point, Lyme immunoglobulin M (IgM) immunoblot was found to be positive, with negative immunoglobulin G (IgG). Anaplasma titers also showed recent/current infection, with IgG >1:1024 (reference <1:64) and IgM 1:80 (reference <1:20). Atovaquone and azithromycin were discontinued and quinine was added. Her hemodynamic status improved and she was titrated off of vasopressor support along with an improvement in respiratory status. Her complete blood count and renal and hepatic function normalized after 14 days of antimicrobial therapy. |
pmc-6464288-1 | A 38-year-old female with a past medical history of hypothyroidism, hypertension, and tobacco use presented to our emergency department with a chief complaint of intermittent burning-like retrosternal chest pain that has been occurring for the past three days but has significantly worsened over one day. In the emergency department, the patient was hemodynamically stable but symptomatic. Electrocardiogram revealed normal sinus rhythm without evidence of ST or T wave changes; however, initial troponin levels were elevated. Due to the patients' sedentary lifestyle, CT angiogram of the chest was done to rule out pulmonary embolism. Subsequently, cardiology was consulted and the decision to pursue cardiac catheterization was made for continued rising troponin levels and persistent chest pain. Cardiac catheterization revealed a SCAD of the OM branch of the left circumflex artery (Figures , ). In addition, there was isolated narrowing and diffuse vessel disease of the circumflex artery without atherosclerotic disease elsewhere. Percutaneous coronary intervention (PCI) was not performed as SCAD involved small and distal vessels, hence; medical management was initiated with dual antiplatelet therapy along with strict risk modification. During her hospital stay, further inquiry and thorough workup confirmed the diagnosis of Ehlers-Danlos syndrome. |
pmc-6464289-1 | The 83-year-old patient presented herself at a neurological emergency department with bilateral blindness and a history of severe headache, which she described the day before. An urgent computed tomography (CT) scan of the brain had been arranged, and a massive pituitary adenoma with hemorrhage was observed, and admission to the neurosurgical department immediately followed. When arriving at the hospital the hemodynamic parameters were stable (BP 130/90, 65 BPM); Glasgow coma score was 14/15. Her medical history included chronic renal failure (III°) as well as cardiovascular disease with hypertension, chronic atrial fibrillation, coronary heart disease with percutaneous transluminal coronary angioplasty, and stent. Therefore, dual oral anticoagulation with clopidogrel and acetylsalicylic acid was administered. Multiple electrode aggregometry did not detect effects of both anticoagulants. The neuro-ophthalmic examination revealed no perception of light in both eyes. Fundoscopy was normal without pallor of the optic nerves, no meningeal signs, and no overt clinical signs of hormone imbalance, but the measured pituitary hormone profile had a slight elevated prolactin level and suppression of sex hormones and thyroid profile (Table ). The blood tests revealed normal electrolytes and infection parameters. A magnetic resonance imaging (MRI) demonstrated a large 22 mm x 18 mm x 15 mm enhancing hemorrhagic pituitary macroadenoma with suprasellar extension and compression of the optic chiasm (Figure ). Emergency surgery was indicated. A transsphenoidal approach was carried out under general anesthesia. The pituitary tumor was identified, though it was not characteristic of a hemorrhagic pituitary macroadenoma. The tumor was tough and yellow colored, and debulking with a sharp curettage was difficult. Acute hemorrhage ceased after resection of tumor. Dopamine agonist was not used due to the patient experiencing complete blindness. After decompression, the surgery was concluded. After a period of cardio-pulmonary stability in the ICU, the patient was extubated. In this case, the transsphenoidal decompression of the optic chiasm provided an improvement in both eyes. Her visual disturbance improved with visual acuity from 0 to 5/100 for both eyes postoperatively. Counting fingers, identifying persons, and color vision were possible. On the first postoperative day, she was anxious and complained of gradual increase in shortness of breath. A non-ST elevation myocardial infarction was diagnosed. Additionally, to that, she developed an acute renal failure requiring temporary hemodialysis. After a short period, kidney function recovered, and cardiovascular stability was observed. The pathological examination confirmed a pituitary adenoma, most of it undergoing infarction. |
pmc-6464292-1 | A 63-year-old male with a past medical history of non-ischemic cardiomyopathy (ejection fraction 10%-15%), hypertension, chronic kidney disease stage G3A, and severe mitral valve regurgitation status post valve repair initially presented for an upgradation of his biventricular pacemaker to biventricular implantable cardioverter defibrillator (BIV-ICD) and replacement of non-functional right atrial lead. The pacemaker pocket was exposed and identified and the atrial and ventricular leads were pulled back under fluoroscopic guidance. A new left coronary sinus lead was placed. Next, right atrial (RA) and right ventricular (RV) leads were placed and access was obtained via the left coronary sinus as the left subclavian vein was found to be occluded with formation of collaterals. The leads were sutured in fascial planes via silk suture. The pacing and sensing parameters were confirmed. BIV-ICD was placed in the pocket, and the pouch was closed via vicryl sutures. The patient had electrocardiogram (EKG) (Figure ) and chest X-ray (Figure ) performed after the procedure. He had developed small pneumothorax from the procedure, which resolved on its own.
The patient was asymptomatic for the next seven months and then he developed increasing shortness of breath. At that point, the EKG (Figure ) revealed loss of appropriate pacing function. It was discovered that the patient kept massaging his pacemaker pocket site in the last seven months but he denied manipulating the device itself. He had also been moving his arm up and down frequently, which would have led all his device leads to be pulled up towards the device in a ratchet-like fashion. The patient was scheduled again for revision of BIV-ICD and on incision of the generator pocket, the left ventricular lead was found coiled up in the pocket beside the generator (Figure ). A new left ventricular lead was placed in the coronary sinus and then access was obtained via the right subclavian vein for right-sided leads. His right atrial and right ventricular leads were also found pulled up. A decision was made to place new right atrial and right ventricular leads. It is to be noted that all his device leads were undamaged and his device was found to be in the pocket in normal orientation. After securing the leads via sutures, the pacing and sensing function was analyzed. The pocket was revised and closed with vicryl sutures and the device was sewn to the pectoral fascia. A chest X-ray (Figure ) and EKG (Figure ) were obtained after the procedure to ensure the absence of pneumothorax and to document the appropriate functioning of the device, respectively. |
pmc-6464435-1 | The patient is a 37-year-old male with a past medical history of smoking two packs per day since the age of 13. He presented to the emergency department with a chief complaint of sharp, epigastric pain radiating to left upper quadrant, 10/10 in intensity, associated with non-bloody vomiting and diarrhea. The patient also endorsed having diaphoresis but otherwise denied fevers/chills, chest pain, shortness of breath, hematochezia/melena. On physical exam, he was tachycardic and abdominal exam showed tenderness on palpation of the epigastric area and right upper quadrant. Laboratory data revealed hemoglobin of 18 g/dL, hematocrit 49.6%, platelets 254,000/mm3, prothrombin time 11.1 seconds, partial thromboplastin time 26.4 seconds, international normalized ratio (INR) 1.1, white blood cell (WBC) 18.8 K/µl. Liver function tests showed alkaline phosphatase (ALP) 73 U/L, alanine aminotransferase (ALT) 24 U/L, aspartate aminotransferase (AST) 25 U/L. A computerized tomography (CT) of the abdomen and pelvis with contrast showed focal dissection of the celiac artery which extended out to the splenic artery and resulted in minimal flow through the splenic artery. Asymmetric enhancement of the spleen was seen which may have been related to diminished flow or areas of splenic infarction (Figure ).
Vascular surgery was consulted and he was placed on a heparin drip. Magnetic resonance angiogram (MRA) of the abdomen reported an abnormal celiac axis with an irregular narrowed appearance consistent with suspected dissection as seen on CT scan. There was a stenosis several centimeters after the origin likely at the junction between the celiac trunk and the common hepatic artery. No flow was detected in the splenic artery on magnetic resonance imaging (MRI) of the abdomen. Computed tomography angiography (CTA) of the abdomen and pelvis showed stenosis within the proximal celiac trunk of less than 50% with greater stenosis at the celiac bifurcation. The stenosis at the origin of the hepatic artery was approximately 75% and the hepatic artery distal to this site was normal in appearance (Figures -).
The stenosis at the origin of the splenic artery was at least 75% and continued throughout the remainder of the splenic artery. Whether the findings of the splenic artery represented stenosis secondary to thrombus or dissection was not definitively determined given the small size of the vessel; however, no dissection was seen within the aorta. There was also an accessory left renal artery without stenosis of the renal arteries bilaterally, and an area of splenic infarct.
The patient underwent percutaneous balloon angioplasty of the hepatic artery and mechanical suction thrombectomy of the common hepatic artery by vascular surgery. While hospitalized, the patient was evaluated by hematology and a hypercoagulable workup was performed with no significant findings including factor V levels, antiphospholipid antibodies, and anticardiolipin antibodies. The patient was discharged on coumadin 5 mg daily with an INR of 2.9 and was told to continue taking labetalol 200 mg orally twice a day for blood pressure control. |
pmc-6464439-1 | A 55-year-old male patient complained of burning sensation at right side tongue region for one month. The patient had a history of betel nut chewing for 30 years (10 betel nuts/day). The patient’s father passed away due to oral cancer 15 years back. Intraoral examination revealed a tender, firm, 2 × 2.5 cm ulcero-proliferative growth at right lateral border of tongue with normal tongue movements as shown in Figure . A single 1 × 1 cm tender, firm, ovoid lymph node was palpable at the right submandibular region. A provisional diagnosis of proliferative verrucous leukoplakia was made. Figure illustrates the contrast-enhanced computed tomography (CECT) findings. Biopsy confirmed infiltrating squamous cell carcinoma (SCC) involving right lateral border of tongue. Tumour node metastasis (TNM) staging: III- T2 N1 M0. |
pmc-6464439-2 | A 65-year-old male patient complained of painful growth at right lower back tooth region for 15 days. The patient had a history of chewing pan for the past 15 years (3-5 packets/day). Intraoral examination revealed a 4 × 3 cm, tender, hard ulceroproliferative growth, arising from the edentulous ridge of 45,46 extending into the alveolus and right buccal mucosa as shown in Figure . Rolled out edge and an indurated base were confirmed on palpation. A single 3 cm × 2 cm, well-defined hemispherical-shaped tender, firm, fixed lymph node was palpable at the right submandibular region. A provisional diagnosis of malignant ulcer involving right alveolus and buccal region in relation to 45,46 was made. Figure shows the panoramic view. Figures - show all the CECT findings which were suggestive of malignant mass lesion with lymph node secondaries. Biopsy confirmed well-differentiated SCC. TNM staging: IVA- T4a N1 M0. |
pmc-6464439-3 | A 65-year-old female patient complained of pain and swelling at right lower back gum and tongue region for 10 days. The patient had a history of similar intensity of pain along with burning sensation at right side tongue region two years back, for which she visited a nearby dental practitioner at her home town in a small village and excisional biopsy was done. The histopathological report had revealed early features of malignancy with severe dysplasia for which patient was prescribed a course of antioxidants and multivitamins for one month due to improper medical facilities. Afterwards, the patient was completely asymptomatic for nearly two years. The patient had no history of usage of any form of tobacco or alcohol. Intraoral inspection revealed a 3 × 3.5 cm hard, ulceroproliferative growth on the edentulous alveolar ridge mucosa of tooth 46,47,48. A single 3 × 3.5 cm hard, indurated, irregularly shaped, lobulated growth evident at the right lateral border of tongue with restricted tongue movements is shown in Figure . Tender, hard, fixed, hemispherical-shaped lymph nodes of 1 × 1 cm were palpable at the right submandibular region. A provisional diagnosis of malignant growth was made. Contrast magnetic resonance imaging (MRI) was suggestive of neoplastic lesion with metastatic lymph nodes as shown in Figures -. Biopsy confirmed SCC involving right lateral border of tongue, right retromolar trigone and right posterior alveolar ridge. TNM staging: IVA- T4a N1 M0. |
pmc-6464439-4 | A 77-year-old male patient complained of a painful non-healing growth at his right side corner of lip for three months. The patient had a history of smoking for 43 years (4-5 cigarettes/day). Extraoral examination revealed a single solitary well-defined, erythematous, tender, hard, indurated oval-shaped ulcero-proliferative growth at the right side lip region at the corner of mouth as shown in Figures -. A single 0.3 x 0.4 cm tender, hard and fixed lymph node was palpable at right submandibular region. Intraoral examination revealed a hard, tender, ulcero-proliferative growth at right commissure region approximately measuring 5 x 1 cm extending into the left buccal mucosa at the level of the occlusal plane. Tender, hard, fixed, hemispherical-shaped lymph nodes of 1 × 1 cm were palpable at the right submandibular region. A provisional diagnosis of malignant, non-healing ulcer was made. Biopsy confirmed well-differentiated SCC. TNM staging: IVA- T4a N1 M0. |
pmc-6464439-5 | A 73-year-old female patient complained of a painful, non-healing growth at her left side inner cheek region for three months. The patient had a history of usage of betel nut since childhood. The patient visited a private dental practitioner one year back for the complaint of sharp tooth at left upper back tooth region and an ulcer at its adjacent mucosal region on the left inner cheek for which she was medicated with a topical anesthetic gel. Intraoral examination revealed a sharp tooth-26, and a 4 x 4 cm tender and indurated ulcero-proliferative growth at left mid buccal mucosa region as shown in Figure . Tender, hard and fixed hemispherical-shaped lymph nodes were palpable at the left submandibular region. A provisional diagnosis of malignant non-healing ulcero-proliferative growth was made. Biopsy confirmed well-differentiated SCC. TNM staging: IVA- T4a N1 M0. |
pmc-6464458-1 | A 55-year-old male with a history of hypertension and HOCM presented to the emergency department with the acute onset of sharp, non-radiating, left-sided flank pain associated with nausea and vomiting. On admission, his vital signs were unremarkable. Physical exam was significant for a Grade III/VI systolic murmur, loudest at the apex, with no radiation. Marked tenderness on superficial palpation of the left inferior costal margin was present. There was no rebound tenderness, no costovertebral angle tenderness, and no abdominal or flank erythema. Lab workup demonstrated leukocytosis at 13,000 and acute kidney injury (creatinine: 1.3 mg/dl from a baseline of 0.7 mg/dl). Urinalysis was positive for hematuria, whereas urine toxicology was negative for any illicit substances. Computed tomography (CT) scan of the abdomen and pelvis without contrast showed no evidence of nephrolithiasis. CT scan of the abdomen and pelvis with contrast demonstrated bilateral segmental hypoperfusion indicative of a bilateral renal infarction, the left greater than the right, with no evidence of hydronephrosis (Figure ). An electrocardiogram (EKG) upon admission showed a normal sinus rhythm with no evidence of infarction, ischemia, or atrial fibrillation. The patient was started on a heparin drip soon after the infarction was noted. Further workup ruled out infection, a hypercoagulable state (anti-cardiolipin antibody, perinuclear antineutrophil cytoplasmic antibodies (P-ANCA), cytoplasmic antineutrophil cytoplasmic antibodies (C-ANCA), protein C, protein S, antithrombin antibody, and Factor V Leiden), autoimmune etiology, sickle cell disease, patent foramen ovale, and arrhythmias. A transthoracic echocardiogram (TTE) showed hyperdynamic left ventricle systolic function, a moderately dilated left atrium at 54 mm, and mild thickening of the anterior and posterior mitral valve leaflets. Later, transesophageal echocardiography (TEE) was performed which showed a peak subvalvular gradient around 20 mmHg with no obvious masses or vegetation. A small rupture in the subvalvular chord and a left ventricular outflow tract (LVOT) obstruction was also observed (Figure ). Different blood cultures were obtained throughout the hospital stay and no microbial organism was isolated, including bacteria, fungus, or acid-fast bacilli. Serologic antibody titers for Bartonella, Rickettsia, and M. pneumoniae were also negative. No obvious source of embolic origin was identified on echocardiogram and imaging of the renal arteries. The patient was placed on telemetry throughout his hospital course, and there was no evidence of any underlying arrhythmia, such as atrial fibrillation. However, it was presumed that the left atrial enlargement might be a predisposing factor to thromboembolic renal infarction via the same mechanism by which it predisposes to stroke []. The patient's renal function improved with treatment, and he was discharged on an anticoagulant, considering his left atrial enlargement and renal infarction. |
pmc-6464459-1 | A 71-year-old male with a history of hypertension, hyperlipidemia, chronic obstructive pulmonary disease, pre-diabetes, chronic smoking, and alcoholism presented with amaurosis fugax. He had suffered a previous left occipital lobe infarct. Carotid duplex and computerized tomography angiography (CTA) of the head and neck confirmed a high-grade left internal carotid artery stenosis (ICA). A five French (Fr) sheath was used for femoral access and aortic arch angiography was performed. Selective cannulation of the left common carotid artery (CCA) was done with an AR-1 catheter. Diagnostic angiography confirmed the noninvasive findings of a high-grade lesion in the left ICA (Video ).
A seven Fr Cook Shuttle (Cook) sheath was advanced into left CCA over a glide wire advantage (Terumo). The Accunet embolic protection device (EPD) was deployed and pre-dilatation of the lesion was performed with a 4-mm balloon Maverick (Video ).
An Acculink 7 x 10 x 40-mm stent was deployed without difficulty (Video ).
Post-dilatation was performed with a 5-mm balloon (Video ).
Attempts to advance the EPD retrieval system beyond the origin of left ICA were unsuccessful. Even a smaller balloon was unsuccessful in crossing the under expanded proximal stent strut. Maneuvers like having the patient turn his head to the right were unsuccessful in advancing the EPD retrieval catheter.
After multiple such futile attempts, a concept that is routinely used in transradial percutaneous coronary intervention (PCI) came handy. The shuttle sheath was extended with a guide extension catheter to enhance support. A 2.5-mm balloon was advanced into this guide extension catheter (Video ).
Balloon-assisted tracking of the guide extension catheter was performed into the left ICA beyond the point where the retrieval catheter was hanging up (Video ).
After this, advancing the retrieval catheter into the guide extension was easy and the filter was removed uneventfully.
Due to some focal weakness in the left upper extremity, a CTA of head and neck was performed which showed a small hemorrhagic transformation within the previous right occipital infarct. On discharge, the patient recovered completely and left the hospital with no neurological deficits. |
pmc-6464460-1 | A 30-year-old female had an onset of intermittent jaundice for the last six years. She was fourth gravida, with one live issue after a fourth-degree consanguineous marriage. She had episodes of jaundice during each pregnancy, which used to start in the late second trimester. The jaundice subsided slowly over two to three months after delivery each time. In between pregnancies, there was minimal or no jaundice. However, the patient was otherwise asymptomatic throughout these years. There was no history of any fever, pruritus, malaise, or abdominal pain. She was non-alcoholic and a non-smoker. There was no history of any blood transfusion, intravenous drug abuse, chronic drug intake, or occupational hazard.
Her pre-pregnancy bilirubin levels were in the range of 3.0-4.0 mg/dL (normal value 0.1-1.2 mg/dL) and would rise to about 11.0-12.0 mg/dL during the second trimester of each pregnancy. She typically had an isolated conjugated hyperbilirubinemia with conjugated bilirubin levels rising to as high as 8.0 to 10.0 mg/dL while the unconjugated bilirubin used to be in the range of 2.0-3.0 mg/dL. The liver function test was invariably normal. Serum transaminase levels were always less than 20 IU/L and serum bile acid levels and proteins were within normal range. The absence of pruritus, normal bile acid levels (0-10.0 µmol/L) and serum transaminases (<20 IU/L) excluded the diagnosis of intrahepatic cholestasis of pregnancy. There was no associated gestational hypertension or proteinuria. Her hemogram was also unremarkable and peripheral smear never showed any evidence of hemolysis. Thus, thrombotic microangiopathies were also ruled out in the patient. Ultrasound abdomen showed a normal liver span and echotexture. The viral markers for hepatitis were also negative in each pregnancy. These tests were done to rule out any active viral hepatitis and included anti-hepatitis A virus immunoglobulin M (IgM) antibody, hepatitis B virus antigen, anti-hepatitis B virus immunoglobulin G (IgG) and IgM antibodies, anti-hepatitis C virus IgM antibody, and anti-hepatitis E IgM antibody. Autoimmune hepatitis was also ruled out, as serum antinuclear antibodies and anti-smooth muscle antibodies were absent. The normal levels of gamma-glutamyl transferase excluded obstructive biliary disorders.
Urine was positive for urobilinogen owing to conjugated hyperbilirubinemia. Total urine coproporphyrin levels were normal, however, the ratio of isomer I:III was not done due to the non-availability of the test. Since the ultimate diagnosis of Dubin-Johnson syndrome is made on liver histology, a liver biopsy was done six months after her delivery, which showed normal lobular architecture. The hepatocytes showed intracytoplasmic coarse brown pigment located maximally in the perivenular hepatocytes (Figure ). The same pigment stained black with Masson Fontana stain (Figure ) and was negative for periodic acid-schiff (PAS) and Perl stain. The presence of liver pigment on liver histology and the normal levels of total urine coproporphyrin levels differentiated the condition from Rotor syndrome. Genetic studies for MRP-2 gene mutation could not be done due to financial constraints. Based on the clinical picture, investigations, and liver biopsy report, a final diagnosis of Dubin-Johnson syndrome was made.
Since it is a benign condition without any complications, she was reassured of a normal course of the disease. She became pregnant one year after the diagnosis. At 30 weeks, she again presented with an exacerbation of jaundice, which was not progressive. Her serum bilirubin levels were in the range of 10.0-11.0 mg/dL with predominant conjugated hyperbilirubinemia. She went into spontaneous labor at 40 weeks and delivered a healthy male baby of 3.2 kg.
In her previous pregnancies, her second baby had a giant encephalocele and died at 15 days of life while the third baby died of severe diarrhea at the age of six months. None of them had jaundice. |
pmc-6464475-1 | The chronological history of an otherwise healthy 13-year-old male patient started when he first appeared in the clinic complaining of a sore throat and a sand-paper like rash involving both hands for one day. Physical examination revealed mildly congested erythematous pharynx and anterior right non-exudative cervical lymph node enlargement of approximately 5 x 3 cm2. The neck mass was firm and movable, tender to touch, and best visualized when the patient asked to move his head to the opposite side. Symmetrical papular rash involving both hands with an equal distribution was also noticed. No other abnormal physical examination findings were recognized at this initial stage. For the aforementioned findings, the patient was suspected of having scarlet fever for which strep throat test was ordered and turned back negative. The patient was then instructed to report back if there are signs of fever along with close monitoring of cervical lymph node enlargement by the parents and was discharged on an anti-histamine (Cetirizine) for itching. Two days later, the patient came back with high-grade fever, bilateral conjunctivitis (Figure ), bilateral hands, and feet edema (Figure , ) with increased itching and appearance of macular rash over his body. At this stage, blood investigations revealed the following: hemoglobin (HGB) 12.2 g/dL; white blood cell (WBC) count 7.6 x103/μL, with predominating neutrophils of (82.3%); platelet count 315 x103/μL; and C-reactive protein (CRP) concentration 56 mg/L and erythrocyte sedimentation rate (ESR) of 85 mm/1 hour. Lastly, a repeated strep throat test was negative, and therefore, throat culture was ordered. The patient was started on 1 g oral augmentin (amoxicillin/clavulanic acid), and parents were instructed to report if fever persists given the use of prescribed antibiotics for more than 48 hours.
On the following day (day 4), the patient was admitted to the hospital ward due to persistent fever refractory to antibiotics. Physical examination was unremarkable for cervical lymph node enlargement suggesting resolution, whereas a neck ultrasound revealed corresponding unilateral hypo-echoic masses suggestive of cervical lymphadenitis, along with persistence of edema of hands and feet, and expansion of macular rash over his body. A few hours later during admission, the patient developed red eyes and characteristic early signs of strawberry tongue evident on physical examination (Figure ). Bacillus Calmette–Guérin (BCG) scar was not evident. Laboratory investigation at this time revealed elevated liver function test (LFT), alanine aminotransferase (ALT) of 186 IU/L, aspartate aminotransferase (AST) of 215 IU/L, gamma-glutamyltransferase (gamma-GT) of 180 IU/L, albumin serum of 28 g/L, serum protein of 78 g/L, and normal platelet count. Blood serology tests of Cytomegalovirus, measles, Epstein-Barr virus (EBV), and hepatitis A, B, and C reported a negative titer level. Urine test showed 10 cells/hpf of WBC with negative nitrite. Echocardiography was done and was normal. Given previous information, on day five (second day of admission), the patient was highly suspected of having typical KD based on clinical presentation consistent with poor outcome predictor (↑CRP, ↑LFT, ↓albumin) and was started on intravenous immunoglobulin (IVIG) 2 g/kg over 12 hours and a high aspirin dose of 80 mg/kg/d every six hours accordingly. The fever did not resolve within 36 hours, for which the patient was started on a second dose IVIG 2 g/kg over 12 hours. On day 10, the patient was noticed to have skin peeling involving both hands resembling sheet-like desquamation involving the palmar areas (Figure ). With that being said, after close observation for 48 hours, the patient was discharged home on a low-dose aspirin of 5 mg. Repeated echocardiography on day 14 was normal, and CBC follow-up showed platelet 609 x103/μL consistent with thrombocytosis. |
pmc-6464478-1 | An 11-year-old African-American male presented to our care in January 2011 because of excessive daytime sleepiness and episodes of losing muscle tone upon experiencing strong emotional stimuli. Before seen by us, the patient was evaluated by a psychiatrist in March 2010 at a request of his mother. Clinician records suggest that the patient was being seen for not being able to express himself, labile mood, lack of focus and concentration at school, anxiety, and lack of self-esteem. At the end of the evaluation by the psychiatrist, the patient was diagnosed with Adjustment disorder of childhood with mixed emotions, depressed feelings, and attention deficit hyperactivity disorder primarily inattentive type. Furthermore, the patient scored 55 (normal = 91-100) on the global assessment of functioning (GAF) score. It is a numerical scale utilized by mental health clinicians to determine the patient's day to day functionality. Specifically, it allows the clinicians to score and determine their social, occupational and psychological functioning [].
After presenting to our care, the patient was sent for further workup including the Epworth Sleepiness Scale (ESS), a nocturnal polysomnogram (NPSG), and a multiple sleep latency test (MSLT). The ESS is a subjective test utilized to measure the patient’s sleepiness []. It includes eight scenarios in which the patient rates their tendency to become sleepy. The scale ranges from 0 (no chance of dozing) to 3 (high chance of dozing). These eight scenarios are sitting and reading, watching television, sitting inactive in a public place, as a passenger in a car for an hour without a break, in a car while stopped for a few minutes in traffic, lying down to rest in the afternoon, sitting and talking to someone, and sitting quietly after a lunch without alcohol [, ]. Our patient scored 17 which is highly associated with pathologic sleepiness because a score of greater than 15 suggests that the patient is excessively sleepy and should be treated.
NPSG is a sleep study which records brain waves, oxygen levels, heart rate and breathing patterns along with eye and extremity movement as the patient sleeps []. According to the results of NPSG, the patient slept 367.00 minutes out of 440.7 minutes in bed for a sleep efficiency of 83.3%. The patient spent 66.4% of total sleep time in supine position. In addition, the patient spent 16.9%, 38.3%, 27.5% of sleep time in stage 1, 2 and 3, respectively. The study identified five REM sleep periods with REM stage lasting for 17.3% of sleep time. REM latency was recorded as normal at 87.5 minutes. Furthermore, the sleep study noted sleep latency (SL) to be 3.2 minutes. Overall Apnea-Hypopnea Index was 0.5 events/hour. The REM specific index was 1.9 events per hour. During the study, one obstructive hypopnea was with a mean duration of 14.3 seconds. There were two mixed apneas with a mean duration of 10.7 seconds along with 0 central apneas. Laboratory workup for HLA-DR15 and DQ0602 was positive.
MSLT is the primary diagnostic tool for narcolepsy and is typically performed following an NPSG to measure sleep latency []. Sleep latency can be described as the amount of time it takes to go from wakefulness to entering sleep. Also, it measures sleep onset REM periods (SOREMPs) which illustrates how quickly the patient enters REM sleep []. This test typically includes four or five naps lasting for 20 minutes each. Results of the MSLT consisted of five nap trials with a sleep latency of 1.8, 3.4, 4.7, 2.9, 6.4 minutes, respectively. In addition, REM latency during five nap trials was recorded to be at 2.5, 1.0, 1.5, 0.5 and 2.5 minutes, respectively. The result shows that the patient slept during each nap trial with mean sleep latency (MSL) to be at 3.8 minutes. Lastly, the study recorded five SOREMPs with a mean REM latency of 1.6 minutes. Based on the workup, the patient was diagnosed with narcolepsy which has been successfully managed with stimulant therapy and sodium oxybate. |
pmc-6464482-1 | A 58-year-old male underwent Ivor Lewis procedure for a T2N0 well-differentiated adenocarcinoma of the distal esophagus. The patient did well until postoperative day six when he became febrile (temperature of 102 °F) and his white cell count rose to 21 k/mm3. Chest computed tomography (CT) scan revealed a frank anastomotic leak (Figure ) with a loculated abscess formation (Figure ).
A chest tube was inserted to drain the abscess, control the leak, and obtain microbiological cultures. Broad-spectrum antibiotics coverage was initiated in the form of metronidazole 500 mg intravenous every eight hours and ceftriaxone 2 g intravenous every 24 hours. Esophagogastroscopy revealed a 1-cm disruption at the gastroesophageal anastomosis and a 23-French (diameter), 8-cm (length) fully covered metal stent was deployed by our gastroenterologist to cover the leaking area (Figure ).
Patient condition dramatically improved after stent placement with normalization of his temperature and white count. Esophagogram a week later demonstrated control of the leak (Figure ).
The patient did well and was discharged from the hospital. |
pmc-6464486-1 | We present a 77-year-old male with a past medical history of NHL/SLL diagnosed almost 10 years ago, who presented to the hospital with abdominal swelling, altered mental status, and difficulty in urinating associated with hematuria. On physical examination, diffuse bulky lymphadenopathy was found in the cervical, axillary, and inguinal areas. Detailed oncologic history and treatment regimens that were taken by the patient have been well explained in Table .
Laboratory workup showed hypokalemia, hypophosphatemia, and elevated lactate dehydrogenase levels. During the hospitalization, computed tomography (CT) scan head was done that showed negative findings for any acute events. Due to altered mental status with underlying worsening of NHL and metabolic disturbances, the toxic and metabolic encephalopathy were the differentials under consideration. Metabolic derangements were corrected during hospitalization and that improved his mental status as well. Idelalisib treatment was discontinued abruptly a week prior to patient’s presentation to the hospital due to pancytopenia and a mixed response on the CT scan imaging. Upon admission, a repeat CT of the abdomen and pelvis showed diffuse bulky lymphadenopathy in the abdomen; one of the nodes in the anterior para-aortic region was measured about 5 cm × 5 cm × 8 cm (Figure ). Bilateral iliac, inguinal, and retroperitoneal lymphadenopathy was also significantly increased in size compared with prior CT scan. There was diffuse lymphadenopathy along with axillary and cervical regions as well (Figures -).
Bone marrow biopsy was done that showed a cluster of differentiation 5 (CD5) and CD23 positive B-cell population (37% of the lymphoid gate), lambda-restricted. Almost all of the B-cells showed immunophenotypic expression of CLL/SLL with lambda light chain restriction that was found in previous cases of this patient. Interestingly, a kappa light chain restricted population of monoclonal plasma cells co-expressed with CD56 (1.1% of total events) is also identified. Urology was consulted for urinary complaints of difficult voiding and hematuria. However, the patient was further diagnosed with paraphimosis and scheduled to have an elective circumcision that alleviated his urinary complaints later. Idelalisib treatment for SLL/NHL that patient took for almost one year was stopped a week prior to current clinical symptoms. Based on progressive SLL/NHL, the patient was started on venetoclax (B-cell lymphoma 2; BCL-2 inhibitor) 20 mg/day for seven days and 50 mg/day afterward. The patient did not develop any tumor lysis syndrome after starting the therapy and is tolerating the treatment well based on both imaging and clinical finding. |
pmc-6464487-1 | A 19-year-old male, a resident of Mirpur with no known co-morbidities, presented to the emergency department (ED) of Dr. Ruth KM Pfau, Civil Hospital Karachi (CHK) in September 2018 with a history of fever, dizziness, generalized weakness and bleeding of gums since the past three days. According to the patient, he suddenly developed a continuous fever of 102°F, which was sporadic in nature and associated with chills and rigors. The fever temporarily alleviated with intake of antipyretics. He also experienced occasional bleeding from gums and dizziness with generalized weakness, for which he sought symptomatic treatment from a local health care facility, but the symptoms worsened. Therefore, he was then referred to CHK. No history of bleeding from any other site, nor hematemesis or black tarry stools was present. The patient revealed a decreased appetite and past addiction to tobacco.
On examination (O/E), the patient was of average height and built, comfortably lying on the bed, and well oriented to time, place and person. Initial vitals included blood pressure (BP) 120/70 mmHg, a regular pulse of 90 beats/min and a respiratory rate of 20 breaths/min. The patient was anemic and dehydrated. He had a soft, non-tender, non-distended abdomen without hepatosplenomegaly, and bowel sounds were audible with a rate of 3-4/min. All other systems were unremarkable.
Blood investigations revealed a hemoglobin (Hb) of 4.5 gm/dl, mean corpuscular volume (MCV) of 108.5 fl, mean corpuscular hemoglobin concentration (MCHC) of 35.4 gm/dl, total leukocyte count (TLC) of 2.7 x 109 L, hematocrit (HCT) of 12.7%, platelet count (PLT) of 12 x 109/L. The clotting profile showed an international normalized ratio (INR) of 1.11, while prothrombin time (PT) and activated partial thromboplastin time (aPTT) were 11.1 and 21.7 seconds, respectively. The various lab investigations conducted, including those for hepatitis B surface antigen and hepatitis C antibody, both came out normal, as did his X-ray chest, renal and liver function tests, spot urine examination and ultrasound of the abdomen. The need for conducting specific investigations like hepatitis and chest X-ray were to exclude any other likely cause of the fever and presence of an infection. The electrolytes were within the normal range as well. Upon serology testing, dengue antigen came out to be reactive while that of dengue virus-specific antibodies, immunoglobulin M (IgM) and immunoglobulin G (IgG), were not reactive. Malarial parasite (MP) and MP immunochromatographic test (ICT) came out to be positive for Plasmodium (P) vivax with 7% reticulocytes (RET). Therefore, a diagnosis of concurrent malaria and dengue infection was established.
The patient was treated with an oral combination of artemether and lumefantrine 80/480 twice daily for three days, along with oral acetaminophen two tablets if needed. Additionally, he was intravenously (IV) given tranexamic acid 5 mg when necessary and 1000 ml sodium chloride at the rate of 80 ml/hour. Two units of packed cells and six units of platelets were transfused alongside. Malaria and dengue were treated as separate entities with the above-mentioned treatment. |
pmc-6464489-1 | A 42-year-old male was referred to his ophthalmologist regarding the complaint of sudden blurriness of vision in his right eye. He was diagnosed five months ago with a case of ENKTCL, nasal type, stage IV-B with testicular and central nervous system involvement with 23% natural killer (NK) cells on cerebrospinal fluid (CSF) flow cytometry. He was on an active therapeutic plan consisting of cycles of chemotherapy (i.e., SMILE protocol: dexamethasone, methotrexate, ifosfamide, L-asparaginase, etoposide), to which there was a complete response in the form of negative flow cytometry and computed tomography (CT) of the chest, abdomen, and pelvis. Five months after the initial diagnosis, prior to the fourth cycle of treatment, a complaint of blurriness of right eye vision was made. Brain CT was done aiming to rule out ocular involvement, which turned out to be unremarkable. At the time of the complaint, visual acuity was 20/30 -2 in the right eye as opposed to 20/20 in the left eye. The right eye showed relative afferent pupillary defect. Extraocular muscle movements were competent in both eyes. On examination of the anterior chamber, +1 cells were visualized in the right eye only. On dilated fundus examination, there was vitritis in the right eye which obscured the vision. Left eye examination was insignificant. B scan ocular ultrasonography revealed retinal detachment in the right eye. An optic CT revealed vitritis in the right eye; an impression of disease infiltration of the eye was made. The therapeutic plan was a diagnostic vitrectomy followed by systemic therapy. A pars plana vitrectomy was made and the vitreous specimen was sampled revealing the presence of 10% viable lymphoid cells expressing CD2 and CD56 on immunohistochemistry stain. An orbital magnetic resonance imaging (MRI) was ordered revealing evidence of an enhancing retinal lesion centered on the optic disc with diffuse restriction consistent with lymphomatous infiltration; minimal proptosis of the right eye was noted (Figure ). Fundus photography of both eyes was performed postoperatively with the retina flat under the silicon in the right eye as seen in Figure and minimal disease involvement of the left eye which further confirmed the diagnosis (Figure ). In addition, CSF flow cytometry revealed immunophenotypic evidence of disease (75% mature T cells and 20% NK cells expressing CD2, CD16 and CD56). Follow-up examination revealed 6/9 vision in the left eye, as opposed to no light perception in the right eye. The patient received 35 Gray units of radiotherapy to the optic apparatus and posterior globe of both eyes and the entire cranium down to the third cervical spine region. Post-radiotherapy MRI revealed interval regression of the disease in the form of resolution of bilateral optic disc infiltrative nodule. Despite medical efforts, the patient’s condition deteriorated, and he passed away. |
pmc-6464492-1 | A 44-year-old previously healthy woman presented to the emergency department complaining of urinary retention. Transabdominal (Figure ) and transvaginal (Figure ) ultrasound showed a heterogeneous area measuring 53 x 27 mm in the superior one-third of vagina with evidence of cystic spaces containing internal echoes. Significant vascularity was seen in the lower endometrium and cervix which was extending into this heterogeneous area. The sonographic findings were concluded as a pedunculated endometrial polyp or prolapsed fibroid with cystic degeneration.
Magnetic resonance imaging (MRI) of the pelvis with contrast was then done which demonstrated a large, well-defined abnormal signal intensity polypoidal mass distending the endocervical canal and extending through the external os (external orifice) into the upper one-third of the vagina. It was measuring 46 x 46 x 58 mm in maximum dimensions. It appeared to be connected to the uterine endometrium by a T2-hypointense stalk seen within the endometrial cavity. No evidence of invasion into adjacent structures was seen. The uterine junctional zone was also thickened and ill-defined, which was suggestive of adenomyosis. The lesion contained rounded T1-hyperintense cystic spaces with fluid-fluid level within it, suggestive of hemorrhages. The lesion did not show significant diffusion restriction, and enhanced heterogeneously in the post-contrast study. Post-contrast enhancement was relatively less than that of myometrium (Figures -).
The initial differential diagnoses included endometrial polyp or submucosal leiomyoma with cystic degeneration. Prolapsed malignancy was a less likely differential because there was no diffusion restriction. The patient proceeded to have a hysteroscopy, endometrial biopsy and vaginal myomectomy. Histopathology of the specimen revealed polypoid adenomyoma, with no evidence of malignancy. |
pmc-6464980-1 | A 50-year-old African-American male with a medical history of hypertension, alcohol and marijuana use disorder, with macrocytic anemia and transaminitis, presented to our clinic to establish care. He reported a history of chronic back pain for which he took over-the-counter pain medications. He was also taking amlodipine for his hypertension. He was referred to gastroenterology for a screening colonoscopy; autoimmune and viral work-up for transaminitis were negative. An abdominal ultrasound was obtained for the transaminitis and revealed a slight fullness of the left renal collecting duct, and computed tomography of the abdomen and pelvis was suggested. The latter (Figure ) revealed an asymmetric, ill-defined soft tissue mass in the left pelvic inlet and sidewall involving the distal left ureter and seminal vesicle and prostate, producing mild left hydronephrosis, hydroureter, and a left, retroperitoneal, 1.7 cm, enlarged lymph node. CT with contrast revealed an ill-defined abnormal soft tissue in the left pelvis extending to the left pelvic sidewall and posterior presacral region concerning for neoplasm and mild to moderate left hydroureteronephrosis secondary to the encasement of the distal left ureter by left pelvic soft tissue.
Oncology was consulted, and he was referred for a biopsy. An interventional radiology-guided core biopsy was performed, which reported cores of dense fibrous tissue with extensive lymphoplasmacytic infiltrates. He was referred to urology, had a left ureteral stent placed, and, subsequently, had robotic surgery for ureteral stent reimplantation. |
pmc-6464982-1 | A 63-year-old male with a past medical history of hypertension, human immunodeficiency virus on treatment, late latent syphilis, and chronic anemia secondary to folate deficiency was referred to the gastroenterology clinic due to painless intermittent rectal bleeding for six months. He denied any associated abdominal or rectal pain, melena, tenesmus, or mucus in his stools. Laboratory results showed a hemoglobin of 11.6 g/dL, with a mean corpuscular volume (MCV) of 105.3 fL. Since the patient did not have any prior colorectal screening procedure and given his present symptoms, a colonoscopy was indicated. The colonoscopy showed multiple polyps: one 15-mm polyp at 65 cm proximal to the anus, a 10-mm polyp at the ascending colon, a 5-mm polyp at the ileocecal valve, and a diminutive at the cecum (Figure ). Pathologic results showed tubular adenoma features at the anal, ascending colon, and ileocecal polyps. On the other hand, the cecal polyp showed polygonal cells with abundant eosinophilic granular cytoplasm, which upon staining with S100 showed positivity and confirmed the diagnosis of a GCT (Figure ). |
pmc-6464983-1 | A 42-year-old Vietnamese woman with a history of chronic hepatitis B presented to the clinic for a widespread pruritic rash for the past two years. The rash was characterized by numerous 2-15 mm excoriated, indurated, hyperpigmented erythematous papules and plaques distributed on the bilateral arms and feet, trunk, and back. Punch biopsies from each arm demonstrated prurigo nodularis. Initial treatment of these lesions was twice daily clobetasol ointment.
A return to the clinic, one and a half months late, revealed an increased number of similar lesions and persistent pruritus. Given the lack of response to topical therapy, the patient was initiated on oral cyclosporine A at 100 mg twice a day dosing. A complete blood count, complete metabolic panel, lipid panel, uric acid level, and magnesium level were ordered at the time of first cyclosporine prescription. All laboratory results returned within normal limits.
The patient had good response to cyclosporine therapy, and after four months after cyclosporine initiation, her lesions were nearly all healed with the resolution of her pruritus. However, the patient noted that both she and her family felt her skin was becoming extremely tan and dry. Physical examination at that time was significant for marked hyperpigmentation mostly on the dorsal hands and feet and to a lesser degree on her arms and legs (Figure ).
Thorough patient history revealed no sun exposure more than normal, no extended length of time exposing the dorsal hands or feet (i.e., wearing sandals), with family and friends commenting on the hyperpigmentation in these areas. Despite the changes in skin color, continuation of 100 mg cyclosporine was recommended due to continued efficacious therapeutic response for her prurigo nodularis. The patient was advised to use topical emollients (CeraVe; Coria Laboratories, Aliso Viejo, CA) and use sun protection while outside. |
pmc-6464987-1 | A 21-year-old African-American male presented to the emergency department at the local community hospital after an episode where the patient was reported to have flashbacks with hallucinations. He reported that the hallucinations were “tactile in nature” and that he could “still feel the cold breath” come out of him. Upon admission to the behavioral health unit, it was discovered that he took an unknown quantity of LSD seven months prior, and he claimed he was having flashbacks to that night, where he “blacked out” and then shortly after became aware of his surroundings to discover he was beating the man who had given him the LSD. There was, reportedly, “blood everywhere.”
Because of the altercation, the patient was arrested and is serving two years of probation with community service. The patient injured his hand at the time of the assault and is unable to work, so he is physically unable to fulfill the community service requirement. The patient is under significant distress because of the position he is in and reports that he does not want to go back to work as a lumberjack. Because he is currently unemployed, his stepfather has been threatening to evict him if he does not find a new job.
The patient admits he has symptoms of poor concentration, memory problems, sleeping difficulties, and is feeling isolated without a good support system. There are no prior psychiatric hospitalizations, and additional history was benign other than the incident he had seven months prior, after taking LSD.
The patient denied a history of suicidal thoughts and attempts. He denied alcohol use but admitted to marijuana abuse. He has never been married and has no children. Upon exam, the patient looked appropriate for his stated age. There was no psychomotor agitation or retardation. He maintained eye contact and spoke with coherence. The patient was sad and his affect was constricted. Immediate retention and recall, recent memory, remote memory, and fund of knowledge appeared to be fair. There were no referential or paranoid ideations. He denied any thought broadcasting, insertion, or withdrawal. He denied any delusions but admitted to visual hallucinations. He was paranoid and guarded. There were no suicidal or homicidal ideations, intents, or plans. He denied any problems with concentration. Insight and judgment were fair, but impulse control was poor by history. The patient was not taking any prescription, over-the-counter, or herbal medications.
On completion of the interview, the patient was observed in the behavioral health unit, and a decision was made to place the patient on clonazepam 1 mg four times a day. The patient fully recovered after four days, and he was then referred to a private local mental health center for follow-up. |
pmc-6464988-1 | A 64-year-old Caucasian female with no significant past medical history presented to the emergency department with right upper extremity pain, redness, and blistering that started seven days prior to presentation. The patient denied history of trauma, fever, or night sweats, but reported progression of symptoms. She reported having mild generalized joint pain for at least five years. The patient denied also any history of joint swelling, redness or warmth, dry eyes, skin nodules, rashes or lesions, or personal or family history of RA or autoimmune disease.
On physical examination: vital signs were stable and body mass index was 25.7 kg/m2. There was evidence of diffuse redness, blistering and swelling over her right arm, elbow joint and forearm, with marked tenderness over the right arm. It was noted that the patient had mild bilateral ulnar deviation, but no evidence of joint tenderness, swelling, or redness of the small joints of the hands.
Her initial lab work included a complete blood count with a white blood cell (WBC) count of 0.9 (Ref: 4-11 × 103 cells/ul) with an absolute neutrophil count (ANC) of 387 (Ref: 1.8-0.8 × 103 cells/ul), platelet count of 109,000 (Ref: 140-400 × 103 platelets/ul) with normal hemoglobin of 13.3 g/dl (Ref: 11.5-15.8 g/dl). Initial right upper extremity radiographs did not show evidence fractures, but did show soft tissue swelling over the right forearm and the right elbow joint. Initial suspicion was for superficial cellulitis and olecranon bursitis. However, further evaluation with right upper extremity duplex showed evidence of acute deep venous thrombosis (DVT) of the brachial vein. The patient was started on intravenous (IV) heparin and IV vancomycin and cefepime empirically for the treatment of acute DVT and soft tissue infection and was subsequently admitted for further workup.
Over the next two days of hospitalization, the patient failed to show significant improvement in her symptoms. Right upper extremity magnetic resonance imaging (MRI) with contrast showed superficial and deep fasciitis with myonecrosis over the right arm. Necrotizing fasciitis was suspected. The patient underwent emergent surgical exploration with irrigation and debridement that showed an evidence of deep fasciitis. A repeat surgical exploration was done two days later. Bacterial cultures grew methicillin-sensitive Staphylococcus aureus and group C streptococcus. IV Clindamycin and cefepime were continued. She was switched to oral clindamycin after obtaining antibiotics sensitivity results.
Regarding the patient’s neutropenia and thrombocytopenia, serology results for hepatitis A and B, cytomegalovirus (CMV) and human immunodeficiency virus (HIV) were obtained and came back negative. The patient underwent a bone marrow biopsy, which revealed a hypercellular bone marrow with increased megakaryocytes and CD8 positive cells. Flow cytometry was negative for lymphoproliferative disease. Her T-cell receptor rearrangement by polymerase chain reaction was positive. Her ANC continued to drop over the following days, despite the fact she received granulocyte colony-stimulating factor (G-CSF) for five days initially. Given the bilateral ulnar deviation and elevated C-reactive protein (CRP) on presentation, work up for Felty syndrome was initiated. Labs showed a CRP of 238.8 (0-5 mg/l) and erythrocyte sedimentation rate (ESR) of 78 (Ref: 0-22 mm/h). Her rheumatoid factor (RF) was positive at 24 (Ref: 0-11 IU/ml), anti-citrullinated protein antibodies (anti-CCP) of >340 (Ref: 0-6.9 U/ml), and antinuclear antibodies (ANA) of 1:320 (<1:40). She had negative anti-SSA and SSB antibodies as well as normal levels of C3 and C4. Hepatosplenomegaly was noted on computed tomography (CT) scan of the abdomen. Bilateral foot and hand X-rays revealed osteopenia, arthritis, and erosive changes in the metatarsophalangeal (MTP) and metacarpophalangeal (MCP) joints, respectively. A diagnosis of Felty syndrome was made. Oral prednisone 40 mg and methotrexate were initiated prior to hospital discharge. Her ANC improved dramatically from a low value of 40 to 1530 within two weeks of initiating treatment. |
pmc-6464990-1 | A sixty-four-year-old female with a past medical history of newly diagnosed hypertension (HTN), type two diabetes mellitus, and hyperlipidemia presented with worsening generalized weakness and bilateral lower extremity swelling for two to three months. She also reported occasional flushing and easy bruising. She gained 20 pounds over six months with centrally distributed fat.
She was recently diagnosed with HTN and diabetes type two by her primary care physician. Routine lab work also showed hypokalemia. Examination revealed high blood pressure and tachycardia with two plus pretibial pitting edema bilaterally.
Due to the combination of recently diagnosed HTN and diabetes type two with hypokalemia, the patient underwent a workup for Cushing syndrome. Cortisol level in the early morning was 45 ug/dL (normal range: 7–28 μg/dL), and ACTH was 444 pg/mL (normal range: 10––60 pg/mL). High-dose dexamethasone suppression test was positive. Prolactin, insulin-like growth factor 1 (IGF-1), and thyroid-stimulating hormone (TSH) were all within normal limits. Subsequently, the patient had an MRI of brain which was negative for any mass. However, the CT scan of abdomen/pelvis with contrast revealed a mass in the pancreas with multiple liver lesions and lymphadenopathy (Figure ). Pathology of the pancreatic mass which was obtained via endoscopic ultrasound (EUS) confirmed a well-differentiated intermediate-grade NET. Subsequently tumor markers were checked. Carbohydrate antigen (CA) 19-9 was 41.1 U/mL (normal range: 0–35 U/mL). Carcinoembryonic antigen (CEA) was 4.7 ng/mL (normal range: 0–5 ng/mL). Chromogranin A was 210 ng/mL (normal range: 0–95 ng/mL). The patient was evaluated for possible Whipple procedure. However, she was deemed not a candidate due to significant liver tumor burden. Her clinical course deteriorated rapidly despite medical management. She passed away two months after the diagnosis of the pancreatic NET. |
pmc-6465391-1 | A 71-year-old man who presented with anemia was diagnosed with rectal cancer with a synchronous adrenal metastasis. His serum level of carcinoembryonic antigen (CEA) was 529.8 ng/ml; and other laboratory data showed no abnormalities. Preoperative abdominal computed tomography (CT) revealed a mass in the right adrenal gland of 4.3 × 3.2 cm in size (Fig. ). Abdominoperineal rectal resection with regional lymph node dissection was performed. The pathological findings revealed well-differentiated adenocarcinoma. According to the TNM classification, the disease was stage IV (fT3N1M1[ADR]). The metastatic adrenal lesion increased in size and the CEA level became elevated after the operation (Fig. ). After chemotherapy with 12 cycles of FOLFOX, the adrenal mass shrunk in size, the CEA level markedly decreased, and no new lesions were detected (Fig. ). On admission, the results of general blood tests including the adrenal hormone levels were normal. Right adrenalectomy was performed for 8 months after the first surgery. The pathological examination of the adrenal gland confirmed the diagnosis of adenocarcinoma, and was consistent with metastatic rectal cancer (Fig. ). The CEA level normalized after the right adrenal resection. Capecitabin was administered as post-operative chemotherapy for 9 months after adrenalectomy. Thereafter, the patient was closely followed without therapy. The patient is currently alive at 9 years after adrenalectomy with no evidence of metastasis and a normal CEA level. |
pmc-6465391-2 | A 53-year-old man underwent sigmoidectomy for sigmoid colon cancer. A pathological examination showed poorly-differentiated adenocarcinoma. According to the TNM classification, the disease was stage IIIb (fT3N2M0). Preoperative laboratory analyses, including the serum level of CEA (0.8 ng/ml), showed no abnormalities. After surgery, chemotherapy with 5-fluorouracil and folinic acid followed by tegafur uracil were administered. At 46 months after the operation, the patient’s CEA level increased to 13.9 ng/ml and positron emission tomography (PET)-CT showed an abnormal uptake in the upper lobe of left lung, and left upper lobectomy was performed. The pathological examination revealed metastatic adenocarcinoma from the previously resected sigmoid colon cancer. Oral adjuvant chemotherapy with tegafur uracil and calcium folinate was administered after lobectomy. Although the patient’s serum CEA level normalized after surgery, it increased to 23.3 ng/ml at 20 months after lobectomy, and PET-CT revealed a left adrenal metastasis (Fig. ). No additional recurrence was observed on CT or colonoscopy. On admission, the results of general blood tests, including the patient’s adrenal hormones levels, were normal. Left adrenalectomy was conducted nearly 6 years after the first surgery. The histopathological findings showed metastatic adenocarcinoma of sigmoid colon cancer (Fig. ). Additional chemotherapy with FOLFOX was administered for 12 cycles after adrenalectomy. At the time of the most recent follow-up examination (9 years after the last chemotherapy treatment following resection of adrenal metastasis), no recurrence was detected by CT or colonoscopy and the patient’s CEA level was normal (Fig. ). |
pmc-6465601-1 | A 37-year-old male peasant presented with a 4-week history of low spirit, bad mood, suicide behaviors, and suspicion prior to hospitalization. He was diagnosed with severe depression and received sertraline (50–100 mg) and olanzapine (10 mg), but the situation became worse with declined cognition function and epileptic seizures after 7 days of treatment. The CSF pressure was 240 cmH2O and leukocyte count was 10 × 106/L. The anti-NMDAR antibodies in CSF and serum were 1:32 (). Initial electroencephalography (EEG) showed epileptic activity with sharp-slow waves in the right anterior frontotemporal region (). The chest and abdomen were detected with B-ultrasound and CT to exclude tumor. He received intravenous immunoglobulin (IVIG; 25 g/day, 5 days), methylprednisolone (1,000 mg, 3 days + 500 mg, 3 days), and prednisolone (0–60 mg, 12 weeks) for two courses; levetiracetam (1,500 mg, bid) and valproic acid (500 mg, bid) were used to control epilepsy. The patient showed severe heart failure and respiratory failure, with persistent psychiatric symptoms, such as visual hallucination, auditory hallucination, and delusion. When given olanzapine (10–20 mg/day, 3 days) and aripiprazole (2.5–10 mg/day, 7 days), these psychiatric symptoms could not be alleviated. Aggressive behaviors occurred when given olanzapine; muscle stiffness and slurred speech occurred when given aripiprazole. After cessation of olanzapine and aripiprazole, the use of clonazepam (2 mg, bid) led to clinical improvement. Thus, he was sedated with midazolam (2–4 mg/h, 45 days) during the period he was in the intensive care unit (ICU). The patient received quetiapine (50 mg/day to 0.4 g/day, 30 days) and clonazepam (2–6 mg/day, 35 days) from the ICU, but he still had severe visual hallucination and auditory hallucination after 6 months of treatment. Positive and Negative Syndrome Scale (PANSS) total score () was 112. The anti-NMDAR antibodies in CSF and serum were 1:10 and 1:320, respectively (), and the antibodies against AMPA1, AMPA2, LGI1, CASPR2, and GABAb were negative (). Head-enhanced magnetic resonance imaging (MRI) showed encephalatrophy (), and no epileptic waves were found in EEG. Then, he was given clozapine (50–300 mg/day), with 218.8 ng/ml plasma concentration (; ). Meanwhile, he was still treated with valproic acid (500 mg, bid) for epilepsy control. Eighteen months later, the anti-NMDAR antibodies in CSF and serum were 1:10 and 1:32 (), respectively. Up to now, the patient was able to live and work normally, with stable situation and no psychiatric symptoms. PANSS total score was 26. |
Subsets and Splits
Exclude ER emergencies
Retrieves 100 descriptions that do not contain the terms 'ER' or 'emergency', providing a basic filter of the dataset.