_id stringlengths 7 16 | description stringlengths 55 95.2k |
|---|---|
pmc-6420325-1 | A 19-year-old male presented to our emergency room with one day history of left flank pain and dysuria. He endorsed mild chronic mild low back pain, which did not impair his daily activities and which he attributed to a previous car accident. During initial workup for hematuria, abdominal computed tomography (CT) scan revealed diffuse lumbar spinal osseous lesions with both osteoblastic and osteolytic features (Figure ). The patient was therefore admitted to internal medicine service for workup of potential metastatic malignancy. His grandmother had cervical cancer; otherwise his family history was unremarkable. Physical examination showed mild tenderness to palpation on thoracic and lumbar spine. His right orbital rim was slightly more prominent than left side and there was a dark skin mark on right front thigh along L1-2 distribution (Figure ).
During the hospital stay, the patient’s laboratory tests were significant only for bacteriuria and vitamin D deficiency with a normal complete blood count/comprehensive metabolic panel, including alkaline phosphatase. Serum protein electrophoresis was negative for any M protein. Tumor markers including CEA and PSA were negative.
Chest, abdominal and pelvic CT scan with contrast was carried out, which showed additional similar thoracic spinal lesions. No primary tumor lesion was identified, except a small 4 mm non-specific pulmonary nodule. Single photo emission computed tomography (SPECT) and whole body nuclear scan were carried out for further characterization of patient multiple bone lesions. SPECT showed ground-glass sclerotic lesions on maxillofacial region and right calvarium, which is typical for fibrous dysplasia (Figure ). All bony lesions are associated with increased nuclear signal uptake (Figure ). Retrospectively, the patient reported to have a small lump on right forehead at the age of five, for which he had an X-ray. We were able to retrieve the original image and found that the same sclerotic lesion on right orbital rim had remained stable in the past 14 years, further suggesting FD, a benign disease process (Figure ). Finally, a bone biopsy was performed, which showed fragment of cartilage and bone without malignancy (Figure ).
Based on clinical presentation and imaging, this patient was considered to have PFD with possible McCune-Albright syndrome, although there was no sign of endocrinopathy yet. The patient was discharged with calcium and vitamin D supplements. Intermittent low dose acetaminophen or non-steroid anti-inflammatory drugs (NSAIDs) were recommended for symptomatic relief of mild lower back pain. Consultation for surveillance of future endocrine disorder was also provided before the patient was discharged. |
pmc-6420328-1 | An 84-year-old female, nursing home resident, with a past medical history of type II diabetes mellitus, hypertension, cerebrovascular accident, anoxic brain injury with permanent percutaneous endoscopic gastrostomy (PEG) tube and tracheostomy to ventilator was noticed to have a high peak pressure on the ventilator on a routine check by the nursing home respiratory therapist. Suctioning was attempted but was unsuccessful. The tracheostomy tube was changed and during the process, increased resistance was felt. Soon thereafter it was noticed that blood was leaking from around the tracheostomy tube. Emergency medical services were called and the patient was brought to the emergency department. On arrival the patient was found to be hypertensive with a blood pressure of 160/74 mmHg, heart rate of 80 beats per minute, respiratory rate of 18 breaths per minute, O2 saturation was 100% on 100% FiO2 fraction of inspired oxygen (FiO2) on assist control-volume control + ventilator mode and a temperature of 37.8°C. On examination, the patient appeared to be generally edematous and was found to have crepitus on palpation starting at the forehead proceeding all the way down to the chest, abdomen, pelvis and upper thighs. In addition, her upper arms bilaterally also appeared swollen with crepitus present on palpation. Examination of the head and neck revealed a tracheostomy with tracheal tube in place with slightly pink secretions and what appeared to be dried blood on gauze surrounding the tube. On auscultation of the chest Hamman’s crunch, a crunching sound, synchronized with the heartbeat was present, as well as slightly decreased breath sounds at the right apex. Stat chest X-ray was performed which revealed extensive PM associated with subcutaneous emphysema in the neck and right chest wall (Figure ).
The patient was emergently assessed by an otolaryngologist and tracheoscopy was performed through the stoma with the tracheal tube in place. It was determined the tracheal tube was in proper position and no obvious tear or leakage in the tracheal wall was noted. Further imaging was warranted and a computed tomography (CT) of the chest, abdomen and pelvis was conducted. CT of the chest showed what appeared to be a 7-mm linear gas density projecting from the posterior wall of the upper trachea which was not seen on tracheoscopy. In addition, it also showed extensive subcutaneous emphysema of the neck and thoracic walls, extensive PM, gas density surrounding the lungs within both the pleural and extra-pleural spaces, measuring 10 mm in the anterior lower right hemithorax and less than 10 mm in the anterior lower left hemithorax (Figure ). CT of the abdomen and pelvis showed large pneumoperitoneum with posterior displacement of the viscera, numerous gas densities in the mesenteric fat and retroperitoneum, extensive subcutaneous emphysema in the soft tissues extending to the level of the labia and anterior thighs bilaterally (Figures , ).
For further monitoring, the patient was transferred to the medical intensive care unit (MICU). Lab results at the time of admission and discharge from the MICU are displayed (Table ).
The patient remained on the ventilator through the tracheostomy at 100% FiO2 with a positive end expiratory pressure (PEEP) of 0 cmH2O. Due to the substantial pneumoperitoneum, there was concern for abdominal compartment syndrome. Bladder pressure was assessed which revealed a pressure of 11 mmHg (normal range: 0-15 mmHg). Repeat chest X-ray 12 hours later showed a slight improvement in subcutaneous emphysema as less free air was evident in the soft tissue. The patient had episode of fever and was treated with cefepime for possible pneumonia. She remained hemodynamically stable and was transferred to telemetry floor for continuing cardiac monitoring. While on the floor the patient continued to receive antibiotics and remain on ventilator support. Five days later on nursing rounds, she was found to be unresponsive without a pulse. She was assessed and found to be deceased. |
pmc-6420332-1 | A 68-year-old man presented for evaluation of a new asymptomatic bump on his left arm. His previous skin examination had been six months earlier and the lesion had not been present. He previously had three basal cell carcinomas (on the left temple, left side of his upper lip, and left mid back excised 32 years, four years and two years earlier, respectively) and one squamous cell carcinoma (on his right upper back that was excised seven years ago). He also had actinic keratoses that were treated with liquid nitrogen cryotherapy.
His past medical history was significant for severe acne vulgaris as an adolescent, hypercholesterolemia, hypertension, and prostate cancer that was diagnosed one year ago. He is currently with no evidence of malignant disease after treatment which included a robotic-assisted laparoscopic prostatectomy (with negative margins for tumor) and a bilateral pelvic lymph node dissection (with none of eight nodes positive for cancer). His current oral daily medications included amlodipine 10 mg and simvastatin 20 mg.
Cutaneous examination showed a six by six millimeter flesh-colored nodule on the extensor aspect of his left arm near the elbow (Figure ). A shave biopsy of the superficial portion of the nodule was performed. The site was treated topically with mupirocin two percent ointment, three times daily, until it had healed.
Microscopic examination of the hematoxylin and eosin-stained tissue specimen showed strands and nodular aggregates of atypical basaloid tumor cells in the dermis reaching the deep margin of biopsy; the overlying epidermis was thin with effacement of the rete ridges and sparse overlying orthokeratosis (Figure ). Deposition of amorphous material filled the dermal stroma between the tumor aggregates (Figure ); the amount of amyloid present was abundant (+++). The lateral aspect of the specimen showed solar elastosis in the upper dermis above the tumor and below the epidermis (Figure ).
Correlation of the clinical presentation and pathological findings established the diagnosis of a nodular basal cell carcinoma with extensive amyloid deposition and adjacent solar elastosis. The residual tumor was excised and a side-to-side layered closure was used to resolve the surgical wound. The surgical site was healed without recurrence at a follow-up examination nine months later. |
pmc-6420334-1 | A 52-year-old otherwise healthy female presented with clear nasal discharge. The nasal discharge reportedly began happening in 2016 and spontaneously resolved. It resumed in April 2018 with no inciting events. The watery nasal discharge worsened with bending over, activity, and coughing. She reported that the nasal discharge was associated with headache as well as a continuous salty taste in her mouth and a feeling of fullness in her ear. The patient denied any trauma. A review of systems was negative except for the headaches and nasal discharge. The patient denied a history of any connective tissue disease, rhinitis, or recurrent sneezing. On physical exam no other abnormalities were noted other than a clear liquid coming from the right side of her nose. Her neurological examination was unremarkable as well.
Testing of the fluid for beta-2 transferrin was positive, indicating that the fluid was CSF. An MRI of the brain showed an empty sella, intracranial hypertension, and a right middle fossa encephalocele associated with a possible CSF fistula (Figure ). A CT cisternogram was performed to confirm the diagnosis, which revealed a discrete focal region of severe bone thinning and probable cortical discontinuity in multiple locations within the anterior, inferior aspect of the right middle cranial fossa just lateral to the foramen rotundum, cribriform plate, and bilateral fovea ethmoidalis (Figures -). The Neurosurgery and Ear Nose Throat (ENT) departments were consulted. Endonasal repair was recommended and the patient was instructed to follow up in the clinic for elective surgery. |
pmc-6420335-1 | Clinical history
A 41-year-old male was initially diagnosed with cervical spinal stenosis and a C3-C6 laminectomy and discectomy were performed at an outside institution (Figure -). MRI in a neutral position two weeks postoperatively indicated decreased stenosis of the cervical spine (Figure ). In the postoperative period, the patient reported particularly concerning episodes of neurological symptoms with new deficits, and increasing levels of tetraparesis.
Two months postoperatively, the patient came to our institution and underwent MRI for reevaluation of the cervical spine in three different positions: neutral, flexion, and extension. While neutral MRI did not show any significant stenosis, flexion and extension MRIs were significant for cervical compression due to spondylosis and compression by paraspinal muscles (Figure ). Compression was quantified by measuring the anteroposterior (AP) diameter of the spinal canal, with more significant compression occurring during extension (Table ).
Surgical management and technique
A reoperative C2-C7 posterior decompression with laminectomies, medial facetectomies, and foraminotomies was performed. Limited undercutting was performed at C2 due to ventral compression visible on the extension at C2. Residual and recurrent compression was encountered and decompressed. Bilateral C3-C7 lateral mass screws were placed, and C2-C7 posterolateral arthrodesis was performed with local autograft and allograft (Figure ). Due to clinical and radiographic findings indicating residual nerve root and spinal cord compression, anterior cervical surgery was performed two days later. At surgery via an anterior cervical approach, significant spondylosis and impingement of nerve roots and spinal cord were found at C3-C7. C3-C7 anterior cervical discectomies were performed with posterior osteophytectomies. Structural allograft pieces were contoured and used for the arthrodeses. C3-C7 segmental anterior cervical screw-plate instrumentation was placed (Figure -). The patient tolerated the procedure well without complications.
Postoperative course
The patient remained neurologically intact, postoperatively, and had a relatively stable physical exam with occasional numbness and weakness in the right upper extremity. His postoperative course was otherwise uneventful, prompting discharge to rehabilitation. The patient remained stable 6 months postoperatively. |
pmc-6420533-1 | A 63-year-old man with a past history of rectal cancer was admitted to our hospital for the treatment of CRLM. The patient had undergone laparoscopic high anterior resection to treat rectal cancer 7 months previously. The pathological stage of the rectal cancer was T1N1aM0 stage IIIA, according to the Union for International Cancer Control classification (seventh edition). The patient did not experience any perioperative complications. He refused to receive adjuvant chemotherapy. Computed tomography (CT) performed 7 months after primary surgery revealed liver metastasis in the left lateral section. Indocyanine green retention at 15 min (ICGR15) was 4.1%. The patient’s Child-Pugh classification was class A. Tests for hepatitis B virus surface antigen and antibodies against hepatitis C virus were negative. Abdominal contrast-enhanced CT revealed a hypovascular tumor of 38 mm in size in the left lateral section (Fig. ). The ligamentum teres was observed on the right side of the gallbladder (Fig. a, b). Three-dimensional CT clearly showed that the umbilical portion of the portal vein was located on the right anterior portal vein, where the RSLT connected (Fig. ). Considering the segmentation of the liver according to the Brisbane 2000 terminology [], the right anterior branch of the portal vein was ramified from the right portal vein. The lateral superior branch of the portal vein (P2) branched off behind the bifurcation of the portal vein, running separately from the common branch of the lateral inferior branch (P3) and left paramedian branch (P4). The patient was diagnosed with CRLM, and LLLS was planned. We fixed the patient in the supine and open-leg position and inserted four ports. The intraoperative findings showed an RSLT with a left-sided gallbladder (Fig. a). After mobilizing the left lateral section, the resection line was carefully decided by identifying the origin of the P3 and P4 of the portal vein using intraoperative ultrasonography (IOUS) (Fig. b). The P2 was independently ramified from the left portal vein and was identified by IOUS (Fig. c). The resection line was on the left side of the falciform ligament. Liver resection was performed using an ultrasonic scalpel (Harmonic Scalpel; Ethicon, CO) and repeatedly using IOUS to ensure the resection of the lateral superior branch of Glisson (G2) and lateral inferior branch of Glisson (G3) (Fig. d). The G3 and the G2 had to be separately clipped and resected, because of the wide distance between the two branches on the resection line (Fig. e, f). The vein draining the left lateral superior section (V2) and left hepatic vein (LHV) were resected at the cut surface, and the left paramedian branch of Glisson (G4) was correctly preserved using intraoperative ultrasonography. The operation time was 2 h and 58 min and the intraoperative blood loss was 50 g. The patient had a favorable clinical course without any complications and was discharged on postoperative day 5. |
pmc-6420555-1 | An 88-year-old male presented in the outpatient surgical setting with a chief complaint of a right groin bulge that had been present for 6 weeks. He had sharp pain initially when he first developed the abnormality but had been asymptomatic ever since. He did not recall any inciting factors. He was concerned that a previously repaired right inguinal hernia had recurred from its original tissue repair in 1977. Details of the original right inguinal hernia repair were unknown to the patient, other than no implantation of mesh occurred. On physical examination, a 3 cm × 3 cm firm, nontender mass was palpable in the right groin just lateral to the pubic tubercle. A computed tomography scan of the abdomen and pelvis was performed to elucidate the cause of the mass in his groin (Figs. , , and ). The imaging was relevant for a right inguinal hernia with the appendix present within the sac. Preoperative laboratory testing revealed a white blood cell count of 4.7 × 109/L. The patient elected to proceed with surgical intervention for hernia repair.
The patient presented to the hospital setting for his elective right inguinal hernia repair. A classic oblique incision was made in the right groin using the anterior superior iliac spine and pubic tubercle as landmarks. The external oblique aponeurosis was opened and the hernia isolated and examined. The hernia was noted to be comprised of an extremely hard and dense amount of omentum that had a chronic, scarred appearance. The base of the appendix could be seen exiting the internal inguinal ring, but the densely adhered omentum prevented reduction of the appendix back into the peritoneal cavity. Initially, there was no indication to perform an appendectomy at the time of the procedure if the appendix could be successfully reduced into the abdominal cavity. However, the chronic appearing adhesions in the area prevented this step. In order to reduce the appendix at that point, a relaxing incision was then made in the typical transverse fashion in the right lower quadrant through the rectus sheath, and the peritoneum entered. The appendix was clearly visualized exiting the abdominal cavity into the inguinal defect. The appendix and its adhered omentum were then carefully reduced back into the abdominal cavity using intraperitoneal countertension without any rupture or spillage. Due to its densely adherent chronic inflammatory tissue, an incidental appendectomy was performed as there was significant tension on the cecum after placing the appendix back in its anatomical location. There was concern for the development of appendicitis post operatively due to the manipulation performed during the procedure. The appendix was then stapled at its base using a standard gastrointestinal anastomosis stapler and passed off the field. The indirect hernia defect was very small and closed with a medium size lightweight mesh plug. The patient was discharged from the post-anesthesia care unit the same day as surgery and had no complications from his recovery course. No additional antibiotics were given other than a single prophylactic dose during the surgical case. At his 2-week follow-up, he had no recurrence of his hernia and was doing well. On pathologic examination, there was no evidence of appendiceal inflammation or appendicitis. The periappendiceal fat did exhibit some fat necrosis, however, supporting the chronic periappendiceal adhesive changes. |
pmc-6420736-1 | A 70-year-old Japanese woman who had undergone resection of a thymic cyst by video-assisted thoracic surgery via the left thorax 10 years earlier was found to have a solid nodule with a multilocular lesion at the site of the previous operation. She had no remarkable medical history aside from the tumor and was not taking any medications. She had no tobacco smoking or drinking of alcohol habits, or significant family history. In addition, she was a clerical worker with no exposure to cancer-causing agents. Her physical examination and laboratory findings, including tumor markers, were within normal ranges. Contrast-enhanced computed tomography (CT) of her chest revealed a solid nodule with a multilocular lesion (30 × 30 × 15 mm) at the anterior mediastinum. The solid component was heterogeneously enhanced, and the cystic component was not (Fig. b). Retrospectively, the CT findings obtained before the previous operation showed a thymic cyst and a small nodule in the cranial section of the cyst (Fig. a). Only the cyst was resected with the partial thymus, and the nodule remained after the previous operation 10 years earlier. Magnetic resonance imaging (MRI) revealed that the solid component was iso-intense on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI), while the cystic component was iso-intense on T1WI and highly intense on T2WI; there was no evidence of invasion to the surrounding organs.
Given the possibility that the tumor was malignant (for example, thymoma or thymic carcinoma), we performed trans-sternal radical thymectomy. The tumor was located at the left inferior pole of the thymus, and the cystic component contained serous fluid. There was no dissemination or adhesion. A pathological examination showed that the tumor size was 3.7 × 2.5 × 1.0 cm. On microscopic examination, the tumor was composed of small nodules and abundant lymphoid stroma (Fig. a, b). The nodule consisted of short spindle-shaped and oval-shaped epithelial cells that resembled type A thymoma. The lymphoid stroma was composed of normal lymphocytes, and normal lymphocytes formed lymphoid follicles partially. On immunohistochemistry, the epithelial tumor nodules were positive for cytokeratin (AE1/3), and the lymphoid stroma was positive for CD3 and CD20 (Fig. c–e). Furthermore, there was linear connective tissue in another slice of the tumor that was probably a scar from the previous operation (Fig. f–h). Reassessment of the thymic cyst resected 10 years earlier revealed a few of the same nodules and lymphoid stroma in the wall of the cyst. The nodule and stroma showed the same immunohistochemical staining pattern as the present case (Fig. ). This indicated that only the cystic component of MNT was removed at the previous operation, and the residual lesion grew over the subsequent 10 years.
The residual lesion was completely removed with the thymus, and there was no recurrence at 2 years after the operation. |
pmc-6420961-1 | A 26-year-old Thai woman presented with a foreign body in her left orbit that had been retained for 8 months. Eight months previously, she had sustained a gunshot injury to her left eye. There was only a small wound on her left eyelid (Fig. ); she had normal eye movement, a normal visual field, and no pain. A plain radiograph was performed at that time; it detected a round-shaped metallic foreign body located medially in her left eye globe. She was then diagnosed as having a retained foreign body in her left orbit and she was advised to have clinical observation. However, 8 months later, she developed pain in her left eye without any sinonasal symptoms. After discussion about the risk of surgery and retention of an orbital foreign body, an ophthalmologist referred her to our department for the minimally invasive procedure option of removal of the foreign body using an endoscopic transnasal approach. On examination, the movements of her left orbit were not restricted and there was normal visual acuity (20/20) with no proptosis or chemosis. A computed tomography (CT) scan of her left orbit revealed a round-shaped metallic foreign body in the medial intraconal space, and lateral attachment of posterior ethmoid sinus, measuring 6 mm (Fig. ). A transnasal endoscopic approach, with navigator assistance (Brainlab), was used to remove the bullet. Uncinectomy and anterior-posterior ethmoidectomy were performed. The location of the intraconal metallic foreign body was confirmed with a navigator system, then part of the lamina papyracea was removed and the periorbita incision was done. The defect was enlarged and the fibrotically encapsulated bullet was found lying in the orbital fat (Fig. ). The fibrotic capsule was dissected, and the bullet was delivered through her left nostril with probes and curetted (Fig. ). No intraorbital bleeding or damage to any soft ocular structures was noted. A relative afferent pupillary defect was found in her left eye during removal of the foreign body, but it resolved and no postoperative complications were observed. |
pmc-6420994-1 | A 61-year-old man was diagnosed with prostatic adenocarcinoma on routine work-up for nocturia and back pain in 2015, and a radical prostatectomy with a pelvic lymph node dissection was performed two months later. The lymph nodes were negative for metastatic prostate cancer but, however, showed partial effacement of the nodal architecture. Immunohistochemical studies performed on select lymph nodes showed nodular/follicular areas mainly composed of B-lymphocytes positive for CD20, CD5 (dim), CD23, and BCL2. These lymphocytes were negative for CD3, CD10, and cyclin D1. The interfollicular areas were almost entirely composed of T-lymphocytes expressing CD3, CD5 (bright), CD43, and BCL2. Interestingly, the pattern of CLL/SLL in the lymph node was unusual, as it seemed that the neoplastic cells were restricted to lymphoid follicles, a pattern known as the follicular pattern of CLL/SLL (Figures –). A complete blood count showed a white blood cell (WBC) count of 12.5 × 109/L and flow cytometry immunophenotypic analysis showed that 26% of blood cells had the following immunophenotype: CD20 (+), CD5 (+), CD19 (+), CD22 (+), CD23 (partial +), CD79b (+), CD200 (+) with surface immunoglobulin lambda light chain restriction, supporting a diagnosis of CLL/SLL.
A referral report showed that conventional cytogenetic analysis revealed a complex karyotype and fluorescence in situ hybridization (FISH) screen for CLL/SLL revealed del(11q) and del(13q). Mutational status of the immunoglobulin heavy chain (IGH) revealed hypermutation of the variable region. The peripheral blood WBC doubling time was estimated at 6 months and hence was determined as not susceptible of chemotherapy. Mutational analysis using next-generation sequencing (NGS) 51-gene panel for hematologic neoplasms showed mutations in ATM and MDM2.
Due to the low-stage disease as determined by the lack of any major clinical or laboratory abnormalities, the patient was followed up with observation (“watch and wait”). At the time of initial diagnosis of CLL/SLL in early 2016, the patient noted a skin rash, mainly in the back with a waxing and waning clinical course (Figures and ). Multiple skin biopsies were performed on these lesions diagnosed as superficial and deep dermis small T-cells and rare small lymphocytes with a periadnexal and perivascular distribution; a diagnosis of B-cell lymphoma was excluded (Figures –). In one of these biopsy specimens, polymerase chain reaction- (PCR-) based assay to assess TRG revealed a small monoclonal T-cell population in a background of oligoclonal T-cells. PCR for IGH in the same specimens was consistently negative (Figures –).
In August 2017, the patient developed anorexia, fatigue, headache, and drenching night sweats. The rash on his back increased in size while on topical treatment. The peripheral blood WBC count rose to 117 × 109/L, compared with 12.5 × 109/L at diagnosis (). A new staging computed tomography (CT) scan showed multicompartmental lymphadenopathy and splenomegaly (Figures and ).
The clinical diagnosis of progression of CLL/SLL was established and bone marrow aspiration and biopsy were performed before the initiation of planned therapeutic regimen of B-cell receptor inhibitor ibrutinib and anti-Bcl2 monoclonal antibody venetoclax. The biopsy specimen showed ~80% cellular bone marrow involved by three distinct aberrant cell populations: (a) T-PLL, representing ~70% of bone marrow cells, (b) minor population of CLL, representing 1.2% of bone marrow cells, and (c) CD5-negative small monotypic B-cells, representing 2.8% of bone marrow cells. The concurrent peripheral blood smear showed predominance of small-to-medium-sized lymphocytes with irregular nuclear contours, clumped chromatin, and conspicuous nucleoli (Figures –).
Conventional cytogenetic analysis showed a complex karyotype: 40~45,X,-Y,add(3)(q29),-11,add(12)(p13),-13,inv(14)(q11.2q32),-15,der(15;22)(q10;q10),-16,-18,-19,-20,+6~10mar[cp3]/46,XY[17]. FISH analysis showed TCL1 rearrangement and deletions of ATM, D13S319 locus, and LAMP1. Gene clonality assays showed both monoclonal TRB and IGH rearrangements. Amplicon-based targeted next-generation sequencing (NGS) assay performed using 28-gene panel on genomic DNA extracted from bone marrow aspiration showed ATM mutation (NM_000051.3(ATM):c.8078_8080del p.A2693del) at high frequency (variant allele frequency [VAF] of 28%) and MYD88 (NM_002468.4(MYD88):c.794T>C p.L265P) gene mutation at a very low frequency (VAF of <5%). Clinical and pathologic features of CLL/SLL and T-PLL as seen in our patient are summarized in .
The patient was diagnosed with composite T-PLL and CLL/SLL. The patient was started on alemtuzumab (total of 3 months of treatment with 30 milligrams 3 times weekly with continuous venous infusion) with excellent clinical and laboratory response showing reduction in size of mediastinal, hilar, axillary, abdominal, pelvic, and inguinal adenopathy. A substantial reduction in previous splenomegaly was observed ().
In light of the new diagnosis of T-PLL, the diagnostic tissues from original pelvic lymph node, obtained at the time of prostatectomy and from one of the skin biopsy specimens, were reanalyzed with TCL1 immunostain to evaluate if T-PLL was present at the time of diagnosis of CLL (Figures –). Both the lymph node and the skin biopsy specimens showed the presence of T-PLL cells highlighted by strong nuclear and cytoplasmic TCL-1 expression.
Flow cytometry immunophenotype of the bone marrow showed that 46% of analyzed cells were positive for CD3, CD7 (bright), and CD26. Also identified were two monotypic B-cell populations with 1.2% of total cells being CD5-positive and 2.8% of total cells being CD5-negative (Figures and ).
FISH analysis was also performed on formalin-fixed paraffin-embedded pelvic lymph node to evaluate for rearrangements of TCL1 and ATM. TCL1 rearrangement was mainly seen in the interfollicular area (Figures and ), whereas ATM was detected in both follicular and interfollicular areas. Therefore, TCL1 rearrangement was mainly confined to the T-cell component, while ATM deletion was detected in both the T-cell and B-cell components. Although desirable for a more definitive assessment of the mutations, cell sorting was not performed on analyzed specimens.
illustrates the chronological order of patient's diagnoses and clinical management. |
pmc-6421014-1 | A 10-week-old 9.6 kg sexually intact, female, golden retriever puppy was purposefully bred and born via cesarean section with ten other littermates (seven females and three males). The female dog in question had two smaller than anatomically normal slit-like anal openings with haired skin coursing between the two distinct orifices. The referring veterinarian examined and explored the female puppy's abnormality after birth and ruled out a persistent anal membrane. No further treatment or surgery was performed since the female puppy did not appear to have difficulty with defecation. The dog was then referred to the University of Florida Small Animal Hospital for further evaluation and exploration of surgical treatment options.
On presentation, the dog was bright, alert, and in good condition, with a body condition score of 5/9 and normal vital signs. The owner reported the patient defecated through both anal openings without tenesmus and noted that the fecal material was mostly soft and of poorly formed consistency. During the physical examination, the dog became excited and dribbled urine in several small spots. Further discussion with the owner revealed that she frequently found urine on the floor but did not recognize this as a sign of incontinence. On physical examination, the left anal orifice was situated 3-4 mm off midline, was smaller in diameter, and had a slit-like appearance compared to the right anal opening, which was 1-2 mm to the right of midline (). On rectal digital palpation of both orifices, mild discomfort was initially noted, and a pain response was observed when palpated more cranially. Additionally, a soft tissue septum with a palpable mucosal surface between the two communicating anal openings was found. This surface and septum was palpated and extended approximately 5 cm orally in the colon. In addition, a lack of palpable circumferential rectal musculature was appreciated on the ventral aspect suggesting this dog had an incomplete external anal sphincter. Urogenital physical evaluation revealed a complete ventral to dorsal soft tissue septum bisecting the vulva, which can be seen in .
Abdominal radiography was obtained to rule out possible orthopedic and vertebral anomalies and to examine the abdominal contents. Abdominal radiography revealed a normal sized stomach containing a small amount of gas and soft tissue material. The small intestines were normal in diameter and had a normal distribution within the abdomen, and a few segments had a small amount of central contiguous gas. The colon was normal in size and contained a small amount of fecal material and gas that extended caudally within the rectum to the perineal margin. Serosal detail was normal with no other abdominal abnormalities noted. No orthopedic or vertebrae malformations were noted. The visible portion of the thorax was within normal limits. Based on these findings, contrast computed tomography (CTA) was recommended to further define the congenital anomalies.
A packed cell volume: 32% (reference range 37-54%), total protein: 4.6 g/dL (reference range 5.4-7.1 g/dL), and AZOstick (within normal limits at 5-15 mg/ml) were performed prior to induction of anesthesia. Premedication and induction of general anesthesia were performed with 1 mg of butorphanol intravenously (0.1 mg/kg), and 30 mg of Propofol (3.13 mg/kg) plus 15 mg ketamine (1.56 mg/kg) respectively and the patient was maintained on isoflurane inhalant ranging from 1 to 2% and oxygen at 1L/min for the duration of the CTA imaging series. Heart rate, electrocardiogram, indirect oscillometric blood pressure, oxygen saturation (by means of pulse oximetry), and end-tidal CO2 were monitored throughout the procedure. Lidocaine was administered to the patient during the procedure at 1.66 mg/kg intravenously.
Advanced imaging, by computed tomography (Toshiba Aquilon 8 CT Scanner, Toshiba Medical Systems, Tustin, Calif.) of the abdomen and pelvis, was performed with intravenous administration of a nonionic, iodinated contrast medium, Iohexol (300mg I/ml), for a total of 4.8 g (500 mg/kg). Images were obtained after contrast administration during both arterial and venous phases. Helical CT images were obtained in a volume data set and were reconstructed in soft tissue, bone, and lung algorithms and then reformatted in transverse, dorsal, and sagittal planes.
A vaginocystourethrogram was performed using two 10 French Foley catheters (one in each vulva), using approximately 15 mL of nonionic, iodinated contrast medium (Iohexol 300 mg I/ml) in each catheter to confirm complete or incomplete duplication as well as establish whether communication was present. Subsequently, a retrograde colonogram was performed using two 10 French red rubber catheters (one in each anal orifice), secured by purse string sutures of 2-0 Nylon (Ethilon®) suture. Approximately 30 mL of barium sulfate paste positive contrast medium was administered in each red rubber catheter. The dog was rescanned, and images were acquired after vaginocystourethrogram and retrograde colonogram.
CT images and abdominal radiographs were reviewed and interpreted by a board-certified radiologist. A vertically oriented soft tissue septum was present and measured 0.37 cm thick and 4.8 cm in length, which extended from the anus to the caudal rectum diverging the positive contrast medium laterally. The contrast then joined again cranially at the level of the second caudal vertebra (). The vulva was divided in the sagittal direction by a soft tissue septum that measured 0.38 cm in thickness. The vestibule, urethra, and urinary bladder were duplicated and positioned side-by-side. The urethra was seen coursing ventral to the uterine horns and had minimal contrast medium filling after urethrogram. The left and right portions of the urinary bladder were mildly to moderately filled with fluid and contrast medium (). The kidneys and ureters were bilaterally symmetric and within normal limits. The left ureter entered the ureterovesicular junction of the left urinary bladder, and the right ureter entered the ureterovesicular junction of the right urinary bladder, in the region of the trigone at the level of the first caudal vertebrae. The ovaries were present and in a normal anatomic location, immediately caudolateral to their respective kidney. The caudal mesenteric artery was completely absent, and no vessel was observed branching from the aorta between the deep circumflex iliac arteries and the external iliac arteries. In addition to this vasculature anomaly, the cranial mesenteric artery took an aberrant path, coursing caudally, in a left lateral direction immediately to the left of the median sacral artery rather than the typical right lateral direction. The cranial mesenteric artery traveled dorsally to the descending colon, then bifurcated at the level of the L7 vertebra, giving off a cranial rectal artery branch, instead of this vessel normally originating from the caudal mesenteric artery. Orthopedically, there were no abnormalities noted.
Surgical options for correction of the anal abnormalities were presented to the owner. However, due to the urogenital findings and concurrent urinary incontinence, the owner instead elected humane euthanasia. The dog was euthanized with 3 ml of sodium pentobarbital (390mg/ml) given intravenously at 122 mg/kg.
A complete necropsy was performed by a board-certified pathologist (MJD). The urinary bladder was abnormal when viewed externally with a cranial-to-caudal oriented central depression. Internally, the urinary bladder was completely bifurcated by a soft tissue septum extending from the apex to the trigone. The trigone had separate urethras exiting in each urinary bladder chamber. The urethras each extended approximately 4.5 cm caudally and then entered separate duplicated vaginas, each with a separate vaginal canal and opening to the outside of the body (). The uterine horns each were separate, with each connected to a single ovary and having a separate cervix. The urethras joined the uterine horns at the level of the cervices. The vaginal canals exited caudally through distinct vestibules as seen from the exterior. Approximately 2 cm from the rectum, there was a similar bifurcation that diverged the colon into two rectal portions and separate anal openings (). The external anal sphincters of both orifices were thin lacking normal muscular layers and incomplete circumferentially. No significant lesions were detected in the remainder of the organs. The necropsy diagnosis was urinary bladder bifurcation with vaginal and colonic duplication. |
pmc-6421035-1 | Eighty-six-year-old female (case no. 21) received staged spinal corrective surgery on her ASD (Figures and ). XLIF was performed on L2/3,3/4,4/5 with 2 hours 29 min., estimated bleeding of 30 ml. Eight days later, open posterior surgery was conducted from T10 to S2 level with 7 hours and 27 min., estimated bleeding of 1100ml. Postoperative hospitalization days were 64 days. Clinical outcomes improved in magnitude of JOA score, VAS of lumbar pain and leg pain, ODI from 23, 64.8, 44.3, and 41.8, preoperatively to 23, 46, and 37, and no data at one month after the surgery, 25, 12, 20, and 17.8 at one year, respectively (). C7PL-CSVL improved from 62 mm to 3 mm at one month after the surgery and 2 mm at one year. SVA decreased from 119 mm to 12 mm at one month after the surgery and 11 mm at one year (). Cobb angle improved from 29° to 4° at one month after the surgery and 8° at one year. LL increased from 4° to 42° at one month and 45° at one year. In consequence, PI-LL improved from 50 to 12 at one month and 9 at one year. PT did not change during the course, from 24° to 21° at one month after the surgery and 27° at one year (). |
pmc-6421046-1 | A 15-year-old woman with no medical history was admitted to our intensive care unit (ICU) a few hours after a longboard fall without initial loss of consciousness or head trauma. The patient was not able to walk and she had to be transported to find help. When the medical team arrived, she was conscious, Glasgow coma scale (GCS) of 15, without hemodynamic or respiratory instability and with a left femur fracture. During the medical transport, she received analgesics medications and immobilization after the reduction of the fracture. The initial body CT scanner, performed 3 hours after the trauma, found a left femur fracture and an anterior left pneumothorax, without cerebral lesions.
She presented secondarily a neurologic status impairment with a GCS of 11, initially attributed to an excess of analgesic therapy. Anyway, she was operated with a left femoral nailing during which a prolongated hypotension without hypovolemia or other obvious causes occurred. At the end of the surgery, 7 hours after the initial injury a new brain scan was performed. It showed the appearance of a cerebral swelling ().
Postoperatively, she was admitted to the ICU because of consciousness disorders requiring a drug induced coma to permit a mechanical ventilation. A cerebral fat embolism was rapidly suspected. Despite a hemodynamic stability and a normality of the PaCO2, the transcranial Doppler ultrasound found a bilateral high pulsatility index at 2.2 and low end-diastolic flow velocity below 20 cm/s. These Doppler profiles led us to suspect an intracranial hypertension. A new brain CT scan, performed 16 hours after the trauma, confirmed a diffuse major cerebral edema. No other organ dysfunctions, rash, or petechiae were noticed.
The patient was managed as a severe brain injury. An intracranial pressure catheter was inserted and found a very high intracranial pressure (ICP) of 75 mmHg. Despite a maximal medical treatment including osmotherapy, hypothermia, barbiturate sedation, and use of neuromuscular-blocking drugs, the ICP remained above 35 mmHg. Twenty-two hours after admission, the patient presented a bilateral reactive pupillary enlargement. The neurosurgeon immediately performed a decompressive right fronto-temporo-parietal hemicraniectomy. Afterwards, the intracranial pressure remained between 20 and 25mmHg and an external ventricular derivation was inserted. A control brain CT scan was performed ().
On the fourth day, the patient presented signs of brainstem injury with a bilateral unreactive mydriasis and loss of oculocardiac reflex despite the normalization of the ICP under 20 mmHg. The patient was still under sedative drugs. A cerebral magnetic resonance imaging (MRI) was carried out. T2-weighted, fluid-attenuated inversion recovery, and diffusion-weighted magnetic resonance imaging showed diffuse punctate hyperintense foci of restricted diffusion in both cerebral and cerebellar hemispheres, with susceptibility artifacts on susceptibility weighted MRI sequences in keeping with petechial hemorrhagic foci, in a starfield pattern ().
Progressively, her consciousness improved with a GCS 9 (M4, V1, E4) ten days after the trauma. One month after her admission she was discharged to a rehabilitation center with a GCS 11 (M6 V1 E4). At two months, she was still improving with a GCS 15, but with persistence of few cognitive disorders evaluated by brain trauma scales: Montreal Cognitive Assessment scale at 26/30; Ranchos scale at VII. The cerebral MRI at three months showed a regression of the multiple punctate hypersignal lesions on diffusion sequences and a disappearance of the hypersignals FLAIR and diffusion of the striatum (). At 6 months after the trauma, she could reintegrate her school. She kept only some headaches and an asthenia one year later.
We have the patient's consent for publishing this case report. The ethic committee approval was not required according to French legislation. |
pmc-6421267-1 | An 81 year-old woman was admitted to our oncology unit (Military Training Hospital Bégin, Unit of Oncology, Paris, France) for the management of grade 3 diarrhea due to fluoropyrimidine-related toxicity. She suffered from an invasive ductal carcinoma of the left breast (Elston-Ellis grade 3, RH+ HER2-) diagnosed in 2007. Medical management was initially based on surgery, radiation therapy and chemotherapy (adriamycine and cyclophosphamide). Hormonal therapy (anastrozole then exemestane) was performed during the following 5 years (2008–2012), and then she entered a regular clinical and radiological follow-up program. As bone and hepatic metastases were diagnosed in 2016 and 2017, respectively, hormonal therapy (exemestane) and chemotherapy (paclitaxel) were reintroduced. In May 2018, given evidence of CNS progression with multiple cerebral metastases, capecitabine (1,500 mg twice a day) was administered. The patient presented signs of major toxicity requiring urgent hospitalization in our department 20 days after capecitabine treatment, and the main symptoms included diarrhea (grade 3) and asthenia (grade 3). She was admitted to our department for close monitoring, with intensive fluid and nutritional support.
Biological investigations performed at admission revealed hematological toxicity with grade 4 neutropenia (absolute neutrophil count: 0.31 × 109/L; reference interval, 1.5–4 × 109/L) and grade 4 thrombocytopenia (platelet count: 35 × 109/L; reference interval, 150–300 × 109/L). The consequences of the severe diarrhea resulted in low blood levels of potassium (2.8 mmol/L; reference interval, 3.5–4.5 mmol/L), phosphate (0.4 mmol/L: reference interval, 0.81–1.45 mmol/L) and magnesium (0.63 mmol/L; reference interval, 0.7–1.05 mmol/L). Moreover, prothrombin time was increased (prothrombin ratio: 41 %; reference interval, >70 %) with a mild elevation of liver transaminase levels (ALT: 60 UI/L; reference interval, <33 UI/L and AST: 106; reference interval, < 32 UI/L) and a marked hypoalbuminemia (albumin level: 22.3 g/L, reference interval, 35–52 g/L). Stool cultures failed to detect bacterial pathogens, including C. difficile strains.
Capecitabine treatment was suspended upon the patient's admission to our service. The clinical evolution was slowly favorable: no diarrhea was observed after 72 h, and cell blood counts were normalized in 1 month.
DPD deficiency was suspected as a potential explanation for the severe toxicity following the first cycle of treatment with capecitabine. DPD phenotype assessment was performed by measurements of plasma uracil (U) and dihydrouracil (UH2) using an LC-MS/MS method (). Analysis revealed a partial DPD deficiency according to the established criteria (U: 40.4 ng/mL, deficiency cut-off > 16 ng/mL and/or UH2/U ratio: 5.0, deficiency cut-off < 6) (). The patient signed written informed consent for genotyping and related data for scientific research. The four DPYD SNPs most commonly observed in the Indo-European population were genotyped according to the current recommendations of the Clinical Pharmacogenetics Implementation Consortium (). The SNPs (c.1905+1G>A, rs3918290; c.2846A>T, rs67376798; c.1679T>G; rs55886062 and c.1236G>A, rs56038477) were genotyped using LAMP Human DPD deficiency kit (LaCAR MDx Technologies, Lièges, Belgium). None of the four variant were detected. However, an atypical profile of the melting curve relative to rs3918290 genotyping was observed (). As the presence of an uncommon SNP was suspected, sequencing of DPYD exon 14 was performed which revealed that the patient harbored a SNP in a heterozygous state: c.1903A>G (p.Asn635Asp) (chromosome position 1:97915617, A/G). To the best of our knowledge, this is a novel variant and is not identified in any of the specific Ensembl, dbSNP, ExAC, Alfred, HGMD or LOVD databases.
In order to evaluate the impact of the proximity of this variant to the intron 14 mRNA splice donor site, we used the most popular in silico tools to predict potential functional alterations involving splicing sites. SIFT (Scale-Invariant Feature Transform, , accessed 28 September 2018) and MutationTaster (, accessed 28 September 2018) predictions suggested a deleterious effect for this variant, with scores of 0.002 and 23, respectively, while PolyPhen-2 prediction (, accessed 28 September 2018) indicated a benign effect, with a confidence score of 0.371. The PROVEAN (Protein Variation Effect Analyzer—, accessed 28 September 2018) tool indicated a neutral prediction with a score of −1.770, while the Human Splicing Finder (htt://www.umd.be/HSF3/index.html, accessed 28 September 2018) tool referred to an exonic splicing enhancer (ESE) mutation “Alteration of an exonic ESE site. Potential alteration of splicing.” Furthermore, the SwissModel web tools (, accessed 28 September 2018) indicated that the missense variant did not affect the final structure of the protein.
Therefore, in order to characterize the potential pathogenic features of the identified variant, a peripheral blood sample stored in PAXgene Blood RNA Tube (Qiagen, Hilden, Germany) was sent to the Oncogenomic Research Center (University of Bari, Italy) for further molecular analysis. Total RNA was extracted from whole blood using the PAXgene Blood RNA Kit (PreAnalytiX GmbH, Hombrechtikon, Switzerland) and reverse transcribed into cDNA with an iScript cDNA Synthesis Kit (BioRad, Hercules, CA, USA). Using a pair of primers spanning exons 13 and 15 of DPYD, the cDNA was amplified and sequenced using a 3500 Genetic Analyzer (Applied Biosystems, 3500 Genetic Analyzer (Applied Biosystems, Foster City, CA, USA). Sequence analysis performed on the cDNA tract including exons 13–15 did not indicate exon 14 skipping ().
The cDNA was then used to perform real-time quantitative PCR assays using the iTaq Universal SYBR Green Supermix (BioRad) in the Step One Plus instrument (Applied Biosystems). The mRNA levels were measured using 3 replicates per sample, with the comparative threshold cycle (Ct) method using glyceraldehyde-3-phosphate dehydrogenase (GAPDH) and beta-actin (ACTB) as endogenous controls, and with related values calculated by ΔCt. As controls, equal amounts of RNA extracted from whole blood of 3 subjects, previously genotyped and identified as wild-type for variants in the DPYD gene, were used. DPYD mRNA levels in the patient carrying the variant were reduced by an average of 45% compared to those observed in healthy subjects expressing the wild-type DPYD gene. A potential limitation in this analysis is that it is difficult to normalize across patients in samples containing multiple cell types that have different expression levels of house keeping genes, but normalizing with either a structural gene and a metabolic gene gave similar results, suggesting the effective reduction of gene expression in the patient.
As a subsequent analytical step, 5 pairs of primers were designed to perform sequencing of a 3296 bp cDNA segment, including the 3078 bp coding sequence of the DPYD gene ().
Analysis of the results allowed the identification of the variants c.85T>C (Cys29Arg) and c.496A>G (Met166Val) recognized as the reference SNP (refSNP) Cluster Report rs1801265 and rs2297595, described with clinical significance of “Pathogenic” and “With drug-response allele” respectively ( accessed 28 September 2018).
The re-introduction of capecitabine at lower doses (1,000 mg twice a day) was associated with recurrence of adverse effects (grade 3 diarrhea and thrombocytopenia—platelet count: 88 × 109/L) and treatment was stopped. Following this, new therapies were adopted (Palbociclib and Fulvestrant) until the patient died 3 weeks later. |
pmc-6421298-1 | An 8–10-year-old patient with a mRS of 2 experienced a sudden onset of headaches accompanied by dysphagia 2 months before being admitted to our hospital. CTA performed in another hospital revealed a giant dissecting aneurysm located in the VBJ, which was confirmed on DSA performed in our hospital (). The LVA was treated with two PEDs (3.5 × 35 mm), and the RVA underwent parent artery occlusion with coils. Immediately postoperative angiography showed excellent reconstruction of the LVA (), and complete occlusion of the RVA (). After the procedure, the clinical symptoms were mildly improved compared with preoperatively. One day post-treatment, MRI demonstrated a giant mass effect with an intramural hematoma, resulting in severe brainstem compression. Six months post-treatment, follow-up DSA revealed complete occlusion of the LVA and RVA (). Compared with MRI performed at 1 day post-treatment (), follow-up MRI showed a marked reduction in the mass effect (). At 6 months after the procedure, the patient had no clinical problems and/or focal neurological function deficiency, with a mRS of 0. As this patient had weak dual posterior communicating arteries preoperatively (), the good clinical outcome might be attributed to the presence of robust dual posterior communicating arteries after the procedure (). |
pmc-6421341-1 | A 92-year-old male reported to his primary care provider with complaints of pain due to OA in his left knee. The patient received an ultrasound-guided injection containing 48 mg Hylan G-F 20 (Synvisc-One; Sanofi Genzyme) without complication. Approximately 8 h following the procedure, the patient reported stabbing pain and swelling in his left knee and ultimately presented to an emergency department 10 h post-injection. The patient’s vital signs were normal and outside of pain, the only other symptom was a decreased range of motion in his knee (limited to 90–120° of movement) compared to a full range of motion prior to the injection and effusion. Following discussion between the primary care provider who performed the injection and the emergency department provider, the patient’s joint was aspirated and nearly 100 ml straw-coloured synovial fluid was removed and sent to a microbiology laboratory for culture. Following aspiration, the patient reported improvement and was able to leave the emergency room without assistance. The patient returned to his primary care provider the following morning for follow-up, and while the knee remained swollen, the complaints of pain had decreased. The primary care provider collected an additional synovial fluid specimen for culture and submitted it to the microbiology laboratory. Seven days after the initial ultrasound-guided injection, the patient was seen by his primary care provider for further follow-up. At this time the symptoms were nearly completely dissipated and the patient’s range of motion had returned to baseline without undergoing additional treatment. In addition, the complaints due to OA had also subsided, indicating that aside from the initial pain the medication performed as expected. |
pmc-6421342-1 | A 66-year-old man presented to the hospital emergency department in August 2014 complaining of redness and pain in his left eye, with blurred vision. His past medical history revealed no human immunodeficiency virus infection or other immunodeficiency disorders. The patient reported a history of perforating trauma to his left eye with a splinter of a plastic hose while working in the countryside. On examination, a central corneal wound was detected, which was self-sealing but opened up to pressure. A positive Tyndall effect was also observed. Topical (0.5 % moxifloxacin 1 eye drop/5 h) and oral (ciprofloxacin, 500 mg/8 h)) antibiotics were prescribed for the following 6 months. Intravenous (IV) vancomycin (1 g/12 h) and ceftazidime (1 g/8 h) treatment was administered for 6 weeks. Samples of corneal scrapings and conjunctival smears sent to the microbiology department yielded no relevant results, and an anatomopathological study of the cornea did not present conclusive data. Given the torpid evolution, a cornea transplant was finally performed in June 2015.
The patient was admitted again to the hospital on November 2015 due to an endophthalmitis in his left eye, despite having been treated with 0.5 % moxifloxacin and 0.1 % dexamethasone drops since the cornea transplant. IV vancomycin (1 g/12 h) and ceftazidime (1 g/8 h) were prescribed, in addition to topical (1% drop/2 h) and oral voriconazole (200 mg/12 h). An aspiration from the anterior chamber of the eye was performed, and an aqueous humour sample was sent to the microbiology department. Bacterial culture was performed on Columbia blood agar, chocolate agar, McConkey agar and in thioglycolate broth with incubation at 35±2 °C in atmospheric conditions supplemented with 5 % CO2. After 72 h, slightly whitish dry-looking colonies were observed on blood agar and on chocolate agar (). Gram staining yielded branched Gram-positive rods, and modified Ziehl–Neelsen stain revealed acid-fastness. The micro-organism was initially identified as (99.9 % identity) using matrix-assisted laser desorption/ionization-time of flight (MALDI-TOF) MS (Vitek MS; bioMèrieux). Sequencing of the 16S rRNA gene and analysis using blast () showed 99.9 % similarity to ATCC 33726 (GenBank accession number: NR115835.1). Subsequently, applying an updated software version for the MALDI-TOF MS, the isolate was identified as . Antimicrobial-susceptibility testing was performed using Etest strips (bioMèrieux) [] and susceptibility clinical categories were defined according to Clinical and Laboratory Standards Institute (CLSI) guidelines for mycobacteria, nocardiae and other actinomycetes []. The micro-organism was reported as susceptible to ceftriaxone (MIC=0.5 mg l−1), imipenem (0.02 mg l−1), amikacin (1 mg l−1), clarithromycin (0.03 mg l−1) and linezolid (0.016 mg l−1), and resistant to benzyl-penicillin (>32 mg l−1), amoxicillin-clavulanic acid (>256 mg l−1), tobramycin (128 mg l−1), ciprofloxacin (>32 mg l−1), moxifloxacin (8 mg l−1) and co-trimoxazole (>32 mg l−1). Although no susceptibility breakpoints have been established for vancomycin by CLSI, the MIC value was high (8 mg l−1).
After the microbiological diagnosis of endophthalmitis caused by , the patient was prescribed amikacin, 1 g/24 h/IV, and imipenem, 500 mg/6 h/IV, for 2 months and discharged. Treatment was continued with ambulatory amikacin, 1 g/24 h/IV, and ceftriaxone, 2 g/24 h/IV, for 1 month; linezolid, 600 mg/12 h/orally for another month; and clarithromycin, 500 mg/12 h/orally for 2 more months (total treatment time: 6 months). The patient was followed-up with consultations with the ophthalmology and infectious diseases departments for 2 years, and satisfactory evolution was observed. |
pmc-6421492-1 | We reported the case of a 57-year-old male with painful proptosis (approximately 3 weeks) associated with defective ocular motility, decrease of visual acuity and strength deficiency on the upper limbs.
Ophthalmological examination
• visual acuity RE (right eye) = 0.9, LE (left eye) = 0.8;
• OU: proptosis and limitation of adduction, abduction, depression and elevation;
• LE: palpebral ptosis;
• OU: normal pupillary reflex.
Neurological examination
No signs of meningeal irritation, brachial diparesis with inability to raise the right arm over 90 degrees; motor shortage for prehension and fist extension, more emphasized on the globally diminished right side reflexes.
Electromyography
Does not detect significant damage to the peripheral nerves of upper limbs.
Initiation of corticotherapy with Medrol 80 mg/ daily (presumptive diagnosis of myositis), the patient’s progression is unfavorable with the increase in proptosis and the marked decrease of visual acuity LE (NLP).
Craniocerebral MRI
Retrobulbar space replacing processes; with thickened wall structure, external contours well delimited to the extraocular muscles RE; maximum diameter 25/12/18 mm; without delimitation from medial rectus (MR), superior rectus (SR) and levator palpebrae superioris LE; maximum diameter 35/25/27mm ().
Thoracoabdominal CT
Infiltrative tissue lesion in the posterior median cervix, with a maximum thickness of 5.3 cm in the sagittal plane, which incorporates the descending thoracic aorta without deviating it (-). The set of lesions described may be compatible with multisystemic infiltrative lymphoma.
It was decided that the patient should be urgently transferred to a university neurosurgical clinic for therapeutic management. Emergency surgical treatment was performed: bilateral orbital decompression by the orbital ceiling frontal resection, microscopic ablation of the left intraorbital formation and partial abortion of the right intraorbital formation.
Postoperative cranial CT
Histopathological examination
Tumor proliferation consisting of diffuse atypical lymphocytes, intratumoral capillary vessels are present without obvious alterations. Final anatomopathological diagnosis: Small cell diffuse non-Hodgkin’s lymphoma.
Differential Diagnosis
• Wegener lymphogranulomatosis (excluded by immunological dosages of pANCA and cANCA antibodies);
• Langerhans/ non Langerhans cells (the diagnosis of certainty is performed by specific immunohistochemical stains from 2 tissue samples).
Evolution
The patient is currently under the supervision of Suceava Oncology Clinic, tetraplegic, blind (progressive decrease of visual acuity RE) under antalgic treatment (opiates). |
pmc-6421495-1 | A 32-year-old healthy Indian male presented with complaints of painful diminution of vision in his left eye for the past three days. The ocular evaluation revealed a best corrected visual acuity (BCVA) of 20/ 20 in the right eye and 20/ 80 in the left eye, with severe anterior chamber inflammation and hypopyon. There was a mild spillover inflammation seen in the vitreous cavity but third order vessels in the fundus could be visualized.
The patient was started on intense topical steroids and cycloplegics. All routine systemic investigations including HLA-B27, and X-ray sacroiliac joint were done. He was found to be HLA-B27 positive by deoxyribonucleic acid based molecular method: single specific primer- polymerase chain reaction (PCR). Over the next three days, the anterior segment inflammation considerably reduced, with the resolution of the hypopyon, but the patient noticed profound diminution of vision in the same eye, dropping to 20/ 400. The examination of the left eye revealed intense vitritis with media clarity decreased to Grade III, with the optic disc and vessels being faintly visible (), and optical coherence tomograph (OCT) showing macular fold and gross macular thickening. He was started on a high dose of oral steroids (1.5mg/ Kg body weight) in order to control the inflammation over the next one week, but the posterior segment inflammation persisted. In view of hypopyon at presentation and dense vitritis with no response to high dose oral steroids, a differential diagnosis of endogenous endophthalmitis was kept in mind and the patient was subjected to a diagnostic and therapeutic pars plana vitrectomy. The vitreous sample was negative for bacteria and fungi on smear as well as culture. The vitreous sample was negative for Mycobacterium tuberculosis (MTB) by PCR (Xpert MTB RIF assay G4).
There was a slight improvement in the posterior segment inflammation as well as media clarity after the surgical procedure, with examination revealing optic disc edema, as well as macular folds on fundus photograph () as well as on OCT. When the patient started showing adverse effects of high dose steroid therapy, he was started on oral Methotrexate 15 mg/ weekly, with the posterior segment inflammation and disc edema still persisting, and with a mild improvement in vision to 20/ 200. The patient developed an idiosyncratic reaction to methotrexate in the form of raised serum transaminases by the third week of methotrexate therapy, which was therefore stopped.
The patient continued to have a significant posterior uveitis with optic disc edema despite being on high dose oral steroids, having undergone pars plana vitrectomy, and having been on immunosuppressive therapy. In view of the long-term requirement of an anti-inflammatory agent in this case, a sustained release dexamethasone implant (Ozurdex) was placed in the vitreous cavity with slow tapering of oral steroids. The patient showed a good response to the implant with a mild increase in intraocular pressure, which was controlled with topical anti-glaucoma medications. There was a complete resolution of the dense vitritis and disc edema with visual acuity improving to 20/ 40 four weeks after Ozurdex implant injection (). |
pmc-6421557-1 | Patient 1: The first patient was a 78-year-old African male who first presented to his local hospital with constitutional symptoms and abdominal distension, which progressed over a four-month period. He was later referred to us for further management. This was his first-ever consultation and admission to any medical facility. There was no past surgical or medical history of note and no history of illicit drug use. However he was an occasional drinker and a smoker with a three-pack year history. He had no family history of note.
Physical examination revealed skin hyperpigmentation, lower abdominal mass and features of severe tricuspid regurgitation with right heart failure. The rest of his clinical examination was unremarkable. Carcinoid syndrome with CHD was suspected during routine transthoracic echocardiographic assessment, and his images are shown in .
His biochemical laboratory results revealed a markedly elevated serum 5-hydroxyindoleacetic acid (HIAA), which was more than 10 times the upper limit of normal. An octreotide scan was positive for the primary lesion localised around the periprostatic area. Additional blood results revealed normal renal and hepatic function. His full blood count revealed features in keeping with anaemia of chronic diseases. The prostate-specific antigen level was mildly elevated. Chest radiography revealed a mildly increased cardiothoracic ratio and hyperinflated lungs. Unfortunately, the patient refused further hospital management, including surgery, and died a year later. |
pmc-6421557-2 | Patient 2: The second patient was a 32-year-old male of Indian descent who presented with a two-month history of abdominal pain, weight loss and diarrhoea. He had no significant past medical, surgical, family or occupational history. Patient 2: The second patient was a 32-year-old male of Indian descent who presented with a two-month history of abdominal pain, weight loss and diarrhoea. He had no significant past medical, surgical, family or occupational history.
His clinical examination revealed features of right heart failure with severe tricuspid and moderate pulmonary valve regurgitation. The rest of his examination was unremarkable. Chest radiography revealed a mildly increased cardiothoracic ratio and an electrocardiogram revealed sinus rhythm. Blood results revealed mild pre-renal dysfunction, anaemia of chronic disease, normal liver function test and normal comprehensive metabolic panel. Further biochemical results revealed an elevated 5-HIAA level and prior to referral, his private practitioner had already commenced medical therapy, which included octreotide.
Two weeks later the patient was referred for a specialist’s opinion and further management. His echocardiographic and computed tomographic images are presented in and , respectively. His symptoms improved dramatically on medical therapy and he was subsequently referred to the surgical team, where an elective tricuspid valve replacement (TVR) was successfully performed six months later. His intra-operative and postoperative periods were uneventful, and his symptoms continued to improve on subsequent follow-up visits. |
pmc-6421597-1 | We present the case of a 63-year-old male of African origin (Kenya) with an occupational work exposure to boilers, and with a known medical history of uncontrolled hypertension, diabetes mellitus type 2, brain aneurysm that developed after a motorcycle accident, and a large left testicular hydrocele (8 × 3 cm) developing for over 1 year. The patient was seen at another hospital with complaints of testicular enlargement but did not receive medical treatment at that time. More recently, the patient underwent a spectral and color Doppler ultrasound revealing a large complex left hydrocele with a 1.7 cm regular, heterogeneous solid structure within the upper anterior aspect possibly representing the left testis. Surgery was then performed a month later, 1300 cc of straw-colored fluid was noted, and the hydrocele sac and left testicular nubbin were removed via scrotal orchiectomy. After pathological review and consultation, the specimen was confirmed to be malignant mesothelioma, epithelioid type. The tumor demonstrates predominantly an exophytic papillary growth although in areas it is solid and invasive into the underlying tunica vaginalis. The neoplastic cells are epithelioid with cuboidal to oval nuclei and eosinophilic cytoplasm (-). By immunohistochemistry, the tumor cells are positive for WT-1, calretinin, and focally for D2-40, while BAP-1 is retained.
Follow-up for positron emission tomography/computed tomography scan did not reveal any hypermetabolic foci except for a small 9 mm hypodense nodule in the right lobe of thyroid gland.
Radical excision is planned and chemotherapy planned after more extensive surgery. |
pmc-6421649-1 | A 55-year-old Japanese woman was referred to our hospital because of hepatic tumors detected by abdominal ultrasonography during a screening examination. Her medical and family history was unremarkable; her occupational and residential history showed no apparent exposure to asbestos. She received no medical drugs, and neither smoked tobacco nor drank alcohol. On admission, her general status was unremarkable; her temperature was 36.2 °C and her blood pressure was 110/70 mmHg with a regular heart rate of 80/minute. A physical examination revealed no abnormal findings; neurological examinations were unremarkable. All laboratory data, including levels of the serum tumor markers carcinoembryonic antigen, cancer antigen 19-9, alpha fetoprotein, protein induced by vitamin K absence or antagonist-II, and cancer antigen 125 were within normal ranges (Table ). An abdominal enhanced CT scan revealed four hypervascular, round hepatic tumors. One tumor, which was 32 mm in diameter, was in contact with the lateral liver segment and the hepatic hilus; the other three tumors were in contact with the diaphragm and measured 7 mm in segment 4, 17 mm in segment 7, and 15 mm in segment 8 (Fig. a–d). There was no pleural effusion or ascites, and no other nodular lesions in her chest or abdomen were observed. Similar to the CT findings, magnetic resonance imaging (MRI) revealed a tumor of lower intensity than the liver on T1-weighted images, and with higher intensity than the liver on T2-weighted and diffusion-weighted images (Fig. a–c). Upper and lower gastrointestinal endoscopic examination findings were normal. We hypothesized that the tumor arose from her liver, and diagnosed our patient as having multiple hepatic hemangiomas, at first. However, imaging findings were not typical of hepatic tumor: all tumors were on the edge of the liver and the tumor margins were clear. Moreover, the tumor in segment 7 appeared continuous with the diaphragm with similar high-density contrast medium (Fig. d). We then performed CT angiography to obtain further details.
CT findings during arterial portography showed enhancement in none of the tumors (Fig. a and b), but CT during hepatic arteriography revealed strong enhancement in the tumor in the lateral segment and that this tumor was fed by the left gastric artery. In contrast, the remaining three tumors had no enhancement and were fed by the right inferior phrenic artery (Fig. c and d). Imaging findings indicated that the liver was not the origin of these tumors. Moreover, all tumors exhibited increased uptake of 18F-fluorodeoxyglucose, with a standardized uptake value of 7.8 with positron emission tomography (PET)-CT (Fig. ). We considered these tumors to be peritoneal malignant tumors, preoperatively, and performed surgical resection. Laparotomy revealed that the tumor in the lateral segment was clearly in contact with the hepatoduodenal ligament, and that the other three tumors were on the diaphragm (Fig. a–c). We performed tumorectomy with left liver lobectomy (Fig. ) and partial diaphragmatic resection. Histopathological and immunohistochemical examinations of the resected specimens were positive for CD34, CD31, D2-40, and calretinin, which confirmed epithelioid mesothelioma (Fig. a–h). Our patient was not given any medication except a general drip infusion and prophylactic antibiotics; she was discharged 10 days after surgery without complications. CT and PET-CT revealed no recurrence 20 months after surgery without adjuvant therapy. |
pmc-6421665-1 | A one-day-old Han Chinese female infant was referred to our neonatal intensive care unit (NICU) after experiencing repeated seizures for half a day. She was the first-born child of a healthy, non-consanguineous Chinese couple. She was born at 40 weeks’ gestation via vaginal delivery. The birth weight was 3100 g. There was no intrauterine distress, birth trauma or asphyxia. There was no placental preface or abruption and no amniotic fluid pollution, and the umbilical cord was not wrapped around the neck. The Apgar scores were 10 and 10 at 1 min and 5 min after birth. Umbilical artery cord gas analysis was not done. She was breastfed after birth. A few hours after birth at the local hospital, the newborn suddenly experienced limb tremors with notable left side twitching after crying. The onset lasted for 3–4 min before subsiding on its own. Within half a day, a similar phenomenon occurred 6–7 times, with no observations of fever, tachypnea, screaming or vomiting. Upon her admission to the NICU at our facility, the infant appeared well and active. Her vital signs were normal: T37.3°C, P 119 bpm, RR 42 bpm, and BP 70/42 mmHg. The physical examination showed normal consciousness, good responses, and pink skin color. No jaundice was observed. The anterior fontanelle was flat and soft, with a size of approximately 1.0 × 1.0 cm. No cephalohematoma or bruising was present. The lungs were clear with equal aeration. Heart function was normal with no murmurs, and the abdomen was soft without hepatosplenomegaly. The parents did not have a similar disease, and no inherited metabolic diseases were known among family members.
After admission, the patient was fed with formula milk and treated with phenobarbital followed by intravenous maintenance doses. Epileptic electrical activity was observed on a continuous normal voltage background pattern with immature sleep-wake cycling during amplitude integrated electroencephalogram(aEEG) monitoring (Fig. ). Lab tests revealed normal serum glucose and high lactic acid levels (5.5 mmol/l), while ammonia levels were not tested. Blood gas tests revealed respiratory alkalosis, with PH 7.54, PCO2 of 20 mmHg, HCO3 of 21.5 mmHg, and BE of − 3.29 mmol/L. Electrolytes, liver enzymes, and creatine phosphokinase levels were normal. The newborn had no fever, vomiting, drowsiness or irritability. The physical examination indicated good responsiveness, a flat anterior fontanelle, normal muscle tone and no signs of nervous system dysfunction. Additionally, the complete blood count was normal: the leukocyte count was 12.3 × 109/L with neutrophils prevailed, hemoglobin levels were 130 g/L, and platelet count was 371 × 109/L. In addition, C-reactive protein was < 1.0 mg/L. There was no basis for intracranial infection; thus, we did not conduct a lumbar puncture. There were no obvious abnormal signs found in the head CT. Echocardiogram showed an atrial septal defect without cardiomyopathy.
On day 3 in the NICU, without additional anticonvulsants were administered, the convulsion frequency decreased. By day 5, no further clinical seizures were noted, and a repeated aEEG showed that epileptic electrical activities had resolved (Fig. ). Phenobarbital was switched to oral treatment of 2.5 mg/kg twice a day. However, there were various degrees of hyperintense signals and diffusion restriction in the deep white matter of both hemispheres on T1-weighted (Fig. ), T2-weighted, FLAIR, and DWI brain magnetic resonance imaging (MRI). On day 7, the blood bacteria culture was negative. On day 9, tandem mass spectrum of blood spots from day 2 revealed that free carnitine and several acyl carnitine levels had decreased (Table ), and amino acid and organic acid levels were normal. The urinary tandem mass spectrum showed normal carnitine, amino acid, and organic acid levels. Decreased plasma free carnitine levels and several acyl carnitine levels suggested carnitine deficiency. The patient was treated with 150 mg/kg/d of L-carnitine intravenously on the following days. On day 13, the metabolic work up of the sample from day 9, just before L-carnitine treatment, indicated that plasma free carnitine and acyl carnitine levels increased to the normal lower limits (Table ). The patient was discharged from the hospital on day 14. Considering brain lesions assessed by MRI and normal lower carnitine levels, the patient was given oral L-carnitine supplementation and phenobarbital after discharge. Half a month later, phenobarbital was discontinued.
Genetic testing showed that the patient had heterozygote mutations for c.1400C > G of the SLC22A5 gene. Her mother had homozygous mutations for c.1400C > G. Her father had a normal SLC22A5 gene (Fig. ). It was confirmed that the patient’s mother had low plasma carnitine levels and was advised to start L-carnitine supplementation and to monitor carnitine levels at regular intervals, especially during future pregnancies.
When the patient was 3 months old, head MRI showed resolution of abnormal signals (Fig. ). At more than 3 months of age, her repeat carnitine levels were elevated; thus, L-carnitine supplementation was decreased and then stopped 1 week later. At the 8-month follow up, levels of growth and development were assessed using the Gesell Developmental Scale [], which yielded a developmental quotient (DQ) of 97.8, which is well within the normal range. |
pmc-6421673-1 | A 48-year-old male with no relevant medical history was referred to our service from another hospital with a soft-tissue mass on his right knee. The patient noticed the mass several years earlier, but due to its size and the absence of symptoms, he did not seek medical assistance. The mass progressively increased in size and deep flexion became uncomfortable. No history of previous trauma was reported.
Physical examination revealed a mass of soft tissue in the superomedial aspect of the right knee. Upon palpation, the mass was soft, nontender and adhered to deep planes. There was no localized temperature increase or joint effusion. The range of movement was 0–110° with no mechanical symptoms, although deep flexion was painful. There was no evidence of muscular atrophy. Complementary tests and diagnostic imaging: Blood test results were within normal limits. Weight-bearing X-rays of the knees were normal, presenting only radiolucent soft tissue and no signs of degenerative joint disease. T1-weighted (T1-w) and T2-weighted (T2-w) MRI sequences revealed a soft-tissue mass with high signal intensity containing linear structures of low signal intensity (Fig. a, b and c) that were isointense with the subcutaneous fat. The tumor was located in the medial suprapatellar bursa but crossed into the lateral region and occupied the patellofemoral joint in extension.
Treatment: A limited medial parapatellar arthrotomy excision was performed (by using only the superior part of the midline longitudinal approach). We encountered a soft ovoid mass measuring about 13 × 8.5 cm, with a fibrous capsule (Fig. a), that was not adhered to deep planes, although it was anchored by a fibrous tract to the suprapatellar bursa; we cauterized this tract. The lateral portion of the lesion passed through the patellofemoral joint into a lateral location. We performed a complete resection of the lesion and sent it to the pathology department. They confirmed it was a true intra-articular lipoma, observing typical images of mature adipocytes without an atypical nucleus and separated by fibrous septa (Fig. b).
Outcome and follow-up: The case coursed with a favorable postoperative evolution. The surgical site incision healed without complications, although a keloid scar formed on the skin. The patient suffered from postoperative rigidity that was resolved with physiotherapy sessions. He was discharged without symptoms and with a complete range of motion. Table shows a timeline of the postoperative evolution.
Lipomas are commonplace soft-tissue tumors, and can be found anywhere in the body []. Intra-articular lipomas, however, are a very rare entity, with approximately 27 cases published to date, of which 19 affected the knee []. Initially, intra-articular lipoma can be difficult to diagnose, especially when it is small and there is no apparent lesion discernible on conventional radiographs. If a lesion can be identified, it appears as an area of well-defined radiolucency. The next step in the study of this lesion is MRI, which is considered the tool of choice for the detection of intra-articular masses and meniscal-ligamentous lesions []. It manifests as a high intense signal in T1-w and T2-w sequences, which is analogous to the signal intensity of the subcutaneous fatty tissue. However, lipoma can also appear with nonspecific characteristics on MRI, such as a signal intensity analogous to fluid, that is thought to be due to mucoid degeneration [].
Differential diagnosis should be made to rule out lipoma arborescens, intra-articular liposarcoma, pigmented villonodular synovitis (PVNS) and Hoffa’s disease [].
Macroscopically, lipoma arborescens has the appearance of villous synovial proliferation of fatty tissue. It is also associated with some clinical conditions such as previous trauma, osteoarthritis and other chronic inflammatory conditions (e.g., rheumatoid arthritis and psoriatic arthritis), whereas, intra-articular lipoma occurs de novo without any previous history. On MRI, lipoma arborescens produces “hairy” projections in the synovium with a high signal intensity in T1-w and T2-w sequences, that is saturated on Short-Tau Inversion Recovery Images (STIR) [].
Low-grade liposarcoma affects middle-aged people. It usually presents as a painless, slow-growing, locally aggressive tumor that rarely metastasizes. Intra-articular liposarcoma is rare. On MRI, it appears as a large lesion with thick septa, accompanied by non-lipomatous soft tissue with a low fatty component.
PVNS is a rare intra-articular lesion that affects the synovial membrane of joints and tendon sheaths. MRI tends to reveal a low signal intensity on T1-w and T2-w sequences with “blooming effect” [], which is due to the magnetic susceptibility of hemosiderin deposits.
Hoffa’s disease refers to impingement of infrapatellar fat, first described by Hoffa in 1904. Infrapatellar fat becomes hypertrophic due to previous trauma. In this case, MRI will show a mass of low signal intensity in T1-w and T2-w images, attributable to subacute or chronic fibrosis. On T2-w images, there may also be an increase in signal intensity in cases of inflammation or hemorrhage. Hoffa’s disease is occasionally accompanied by ossification. However, unlike intra-articular lipoma, it is rarely found in the suprapatellar bursa or intercondylar region [].
Histopathologically, intra-articular lipoma is consisted on mature adipocytes covered with a synovial membrane and may also contain a vascular fibrous septum. That is why it is a true neoplasm of uncertain etiology. The natural history of the disease has not been studied in depth, although it is known that it grows slowly and follows a silent clinical course until the symptons appearance due to a space-occupying lesion.
The gold-standard treatment has not yet been established for intra-articular lipoma. Arthroscopic excision has been performed as well as open arthrotomy. There have been no recurrences of the lesion following arthroscopic excision in previous studies, which suggests this treatment is valid so long as it is practicable. Arthroscopy did not seem to be an option in our case given the large size of the patient’s lesion, so we deemed limited arthrotomy to be a much more realistic option. |
pmc-6421687-1 | The patient was a 32-year-old man with severe obesity (Body Mass Index (BMI) = 35) and heavy smoking (ten cigarettes per day for more than ten years). He was diagnosed to have moderate HTG (fasting serum TG concentration, ~ 500 mg/dL (~ 5.65 mmol/L)) three years ago (at the age of 29) but had not taken lipid-lowering drugs. His father had diabetes mellitus, his mother was healthy, and there was no known history of HTG in the family.
He was hospitalized elsewhere due to severe abdominal pain associated with nausea and vomiting on June 5, 2017. He was transferred to our Surgical Intensive Care Unit for further treatment on June 11, 2017. The study was performed in accordance with the Helsinki Declaration and was approved by the Ethics Committee of Jingling Hospital, Nanjing University. Bio-samples were obtained after written informed consent. |
pmc-6421695-1 | The 3-year-old male proband was referred to Clinical Genetics for evaluation of a history of developmental delay. He was the second child of non-consanguineous parents of Mexican descent, and his pedigree was remarkable for a mother and maternal male first cousin who required special education mainly for learning disabilities. The prenatal history included a maternal chlamydia infection at 3 months gestational age (treated with antibiotics) and intrauterine growth restriction. He was born vaginally at 37 weeks gestational age, weighing 1.98 kg, and spent a total of 23 days in the NICU requiring nasogastric feeds and phototherapy. He walked and began to speak at 14 months of age and was subsequently diagnosed with global developmental delay by a behavioral pediatrician. He began early intervention services at the age of 2 years, receiving occupational, physical and speech therapy. At 3 years of age he was placed in special education classes getting speech and occupational therapies. At the time of his initial genetics evaluation, the patient could understand directions and point to body parts. He did not know the alphabet, colors, or numbers. In addition to these developmental delays, the proband has a history of anemia that is followed by his pediatrician. On physical exam, the patient was found to have a low anterior hair line. His palpebral fissures were slightly downward slanting, and infraorbital puffiness was noted. He has overfolded helices bilaterally, a broad nasal bridge, a wide grin and thin upper lip, which generally resembled the facial features of his mother (Fig. ).
Clinical Fragile X testing on DNA isolated from the proband was normal with 29 CGG repeats; however, CMA testing using the SurePrint G3 ISCA CGH + SNP 4 × 180 microarray (Agilent Technologies, Santa Clara, CA) [, ] detected a heterozygous 1.21 Mb deletion of chromosome 3q29 (reported as arr[GRCh37] 3q29(195804728_197016624)× 1) (Fig. ). This microdeletion is smaller than the 1.6 Mb recurrent 3q29 microdeletion defined above, but still nested within its segmental duplication boundaries (Fig. ). It included 28 genes and transcripts, and had only minimal overlap with copy number variants (CNVs) reported among healthy individuals in the Database of Genomic Variants (DGV; ) []. The interstitial chromosome 3q29 microdeletion was confirmed using the higher resolution CytoScan® HD platform (Affymetrix, Santa Clara, CA) and metaphase fluorescent in situ hybridization (FISH) using subtelomeric DNA probes (Fig. ). Notably, familial CMA testing determined that the pathogenic chromosome 3q29 microdeletion was maternally inherited and not present in the healthy older sibling (Fig. ), nor was it prenatally detected in a subsequent maternal pregnancy. |
pmc-6421697-1 | A 51-year-old male visited us complaining of right hip pain in June 2011. He had undergone bilateral THA more than 10 years prior. X-rays revealed wear of the acetabular linings of both hips, and severe osteolysis of the proximal femora and acetabula (Fig. a). Preoperative laboratory data revealed no sign of infection; we thus revised the right hip on June 24, 2011. During operation, we found that the acetabular cup was firmly fixed; we thus replaced the acetabular polyethylene lining and the alumina ceramic head. The femoral stem was loose so a DePuy Solution stem (diameter 12 mm) was placed after removing the old stem. The DePuy stem is a distally fixed, extensively porous-coated cylindrical revision femoral stem (Fig. b). We grafted alloallergic cancellated bone onto the acetabular side; we did not graft the proximal femoral bone defect. On April 11, 2013, we revised the left hip. Again, we found that the acetabular cup was firmly fixed and the femoral stem loose. We grafted alloallergic cancellated bone onto the acetabular side, replaced the acetabular polyethylene lining and the alumina ceramic head, and again used a DePuy Solution stem (diameter 12 mm) for femoral revision (Fig. c and d). However, on June 122,014, the patient returned to hospital complaining of left hip pain and an inability to move after changing his sitting posture. Blood tests revealed a normal erythrocyte sedimentation rate and a C-reactive protein level of 56.8 mg/L. A stem fracture was evident on X-rays (Fig. a and b). We revised the left hip 11 days later; the cup and acetabular polyethylene lining were satisfactory. We replaced the femoral head and used a 14-mm-diameter DePuy Solution stem to revise the femoral side. Allograft bone augmentation of the proximal femur followed by cerclage wiring was performed (Fig. c and d). However, the patient returned again in September 2017 with a right femoral prosthetic fracture (Fig. a and b). We replaced the femoral head and the acetabular polyethylene lining, and used a 14-mm-diameter Wagner SL stem (Zimmer, Warsaw, IN, USA) to revise the right hip. We performed allograft bone augmentation of the proximal femur followed by cerclage wiring (Fig. c and d). |
pmc-6421719-1 | A 78-year-old woman was diagnosed with stage IV adenocarcinoma of the left lung in November 2015 after presenting with a history of haemoptysis. Her only medical history was hypercholesterolaemia. She underwent a bronchoscopy and biopsy of a lesion in the LLL, which confirmed TTF-1-positive adenocarcinoma of the lung. Her tumour status was epidermal growth factor receptor (EGFR) mutation and anaplastic lymphoma kinase rearrangement negative. Her initial CT at diagnosis showed a large LLL tumour measuring 5.3 × 7.9 × 6.3 cm with volume loss, satellite nodules, and surrounding interstitial changes. There was a severe encasement and narrowing of the pulmonary vessels, pleura infiltration with discrete pleural nodularity in the left upper lobe, and a small effusion. Bilateral pulmonary metastases were seen with a large nodule in the RLL measuring 2.2 × 2.9 cm. There were also enlarged necrotic appearing lymph nodes in the left hilar and subaortic region, which measured 12 mm.
She was initially commenced on palliative chemotherapy with carboplatin and pemetrexed. After 3 cycles of chemotherapy, her restaging CT showed an increase in size of the nodular lesion of RLL measuring 3.8 × 3.5 cm with LLL measuring 5.3 × 3.5 × 5.9 cm and subaortic node of 9 mm (). She was commenced on second-line treatment with Nivolumab (3 mg/kg) on the early access to medicine scheme in May 2016, which she tolerated well. An interval restaging CT post 3 cycles of Nivolumab in June 2016 showed a stable RLL mass measuring 3.6 × 3.7 cm, and the LLL mass was smaller measuring 3.1 × 3.6 cm. No mediastinal lymph node enlargement was seen.
A restaging scan after 9 cycles of Nivolumab in September 2016 showed some reduction in the RLL mass measuring 3.1 × 2.8 cm, an increase in LLL lesion 4.3 × 3.9 cm (). A further interval CT restaging after 15 cycles of Nivolumab in December 2016 showed that the RLL mass had further reduced in size measuring 2.9 × 2.6 cm. The LLL mass was, however, significantly larger measuring 7.7 × 7.3 cm. This mass has lobulated margins and showed marginal and almost septated more central enhancement. Stable pleural thickening is shown in . Her case was discussed in the lung multidisciplinary team meeting, and she went on to have an ultrasound-guided biopsy of LLL mass in January 2017. The histopathology report concluded fragments of lung parenchyma with necrotic areas and reactive inflammatory changes, with no evidence of residual malignancy, features which were in keeping with immunotherapy effect. We concluded based on the biopsy findings that the significant radiological size increase was due to pseudoprogression, and she continued with the immunotherapy.
A further repeat CT scan was performed after further 4 cycles in March 2017 which confirmed tumour regression with the LLL mass measuring 6.5 cm × 5.3 cm. The mass in the RLL also showed a reduction in size measuring 2.1 × 2.0 cm in maximum axial diameter (). |
pmc-6421721-1 | An 81-year-old woman with a left bipolar hemiarthroplasty performed 10 years previously presented with a left femoral shaft fracture that occurred without trauma (). Her femur broke while she was standing, and then, she fell down. She had visited our hospital with new-onset left thigh pain two years prior to this episode. Radiographs showed no evidence of a fracture, but slight localized periosteal thickening of the lateral cortex was observed (). In addition, she had undergone osteoporosis treatment and had been taking alendronate for more than five years. She was instructed to discontinue alendronate, prescribed vitamin D, and continue with limited weight bearing with cane. After 6 months (1.5 years before the injury), the fracture line became clearer; however, the pain had disappeared. Thus, the conservative treatment was continued. At 12 months (1 year before the injury), a fracture line was visible; however, there was no complaint of pain. However, at 24 months (10 days before the injury), the patient reported pain (). After the injury, radiographs showed a noncomminuted transverse fracture located at the tip of the stem with localized periosteal thickening of the lateral cortex or a “beak sign”; we judged Vancouver type B1 periprosthetic fracture (). The fracture was complete, extending through both cortices. We used a locking plate with cable grip to perform open reduction and internal fixation (). Postoperatively, the patient was allowed non-weight-bearing exercise. She was prescribed weekly subcutaneous injection of 56.5 μg teriparatide and low-intensity pulsed ultrasound (LIPUS). One year later, radiographs revealed nonunion (). At the two-year follow-up, complete bone union was achieved (). At the most recent follow-up (three years), there was no tenderness over the fracture site and radiographs revealed no displacement or loosening of the implants. |
pmc-6421721-2 | An 85-year-old woman visited our hospital due to a right femur fracture that occurred after minor trauma as a result of having fallen down while walking. She had received a right total hip arthroplasty 18 years earlier due to rapidly destructive coxopathy and a revision arthroplasty 9 years earlier because of the loosening of the femoral stem. In addition, the patient had been taking alendronate for more than five years. Radiographs showed localized thickening of the lateral femoral cortical bone and complete transverse fracture with internal spikes. In addition, a third bone fragment was seen, showing the same findings as the atypical femoral fracture. We judged Vancouver type B1 periprosthetic fracture (). In the radiographs taken five months before hospitalization, the cortical bone appeared to be thinning slightly in proximity to the tip of the stable femoral stem and the transverse lucency was emitted on the outside (), which resulted in a complete fracture from the incomplete fracture. We used a locking plate with cable grip to perform open reduction and internal fixation (). Bisphosphonate use was discontinued and prescribed weekly subcutaneous injections of 56.5 μg teriparatide and LIPUS. The patient was allowed non-weight-bearing exercise. At the follow-up three months after surgery, the patient was using a wheelchair without pain and a radiograph revealed no displacement or loosening of the implants. |
pmc-6421722-1 | A 20-month-old Chinese boy was seen with a 7-day history of high-spiking fevers. The child broke out with a nonpruritic widespread reddish rash 1 day after the onset of fever. On the third day of the fever, he developed nonpurulent conjunctival injection. The child was irritable and had decreased oral intake. His mother brought him to see a family physician who treated the child with azithromycin and acetaminophen. The fever persisted in spite of the treatment. The child had not been exposed to anyone with a known infectious disease. His past medical history was unremarkable. The family history was noncontributory.
On examination, the child was irritable and lethargic. His weight was 10.4 kg, height 82 cm, and head circumference 48.5 cm. His temperature was 39°C, heart rate 115 beats per minute, blood pressure 84/40 mm·Hg, and respiratory rate 33 breaths per minute. The child was noted to have bilateral nonpurulent bulbar conjunctival injection; fissured red lips (); strawberry tongue diffuse erythema of the oropharyngeal mucosa; a generalized blanching polymorphous maculopapular rash over his face, trunk (), and groin; erythema and firm edema of the dorsa of the hands and feet with sharp demarcation at the ankles and wrists and two enlarged firm tender lymph nodes each measuring 2 × 3 cm in the right cervical area. The rest of the physical examination was normal. In particular, there was no hepatosplenomegaly or a heart murmur.
The child was admitted to the hospital for investigations and management. Laboratory tests on admission revealed the following results: hemoglobin 12.6 g/dL (126 g/L), white blood cell count 21.3/μL (×109/L) with 88% neutrophils, platelet count 277 × 103/μL (×109/L), and C-reactive protein 21.2 mg/L (201.7 nmol/L). Urinalysis showed 15 white blood cells per high-power field with no bacteria. Serum electrolytes, albumin, liver enzymes, and renal function were normal. Urine culture and throat swab culture were negative. The baseline chest radiograph, electrocardiograph, and echocardiograph were normal.
A diagnosis of Kawasaki disease was made based on the findings of fever for seven days, conjunctival injection, polymorphous rash, oral mucosal changes, changes in extremities, and cervical lymphadenopathy. The child was treated with intravenous immunoglobulin (2 g/kg) infused over 12 hours and high-dose aspirin (80 mg/kg/day divided into 4 doses) given orally. Over the next 36 hours, the child became afebrile, and the maculopapular rash resolved completely. He was discharged after 4 days of hospitalization on high-dose aspirin (80 mg/kg/day divided into 4 doses) for a total of 14 days followed by low-dose aspirin (4 mg/kg/day) in once-daily dosing for 8 weeks. At 2-month follow-up, the child's fingernails and toenails were found to be partially shed on both hands and feet, with the proximal nail beds covered by new nail (). At 4-month follow-up, the old fingernails and toenails were fully shed, and the new fingernails and toenails were normal. Follow-up echocardiogram at 4 weeks and 3 months was normal. |
pmc-6421763-1 | Permission was taken for publication from the patient. A 31-year-old female patient was admitted due to a mass in the left submandibular region in 2015, and mass excision surgery was performed. The result was reported as pleomorphic adenoma. Two years later, the patient was again referred with a mass in the same region. Fine-needle aspiration biopsy accompanied by ultrasonography was performed, and the result was reported as chondroma. Neck exploration was performed, and the mass was excised from the neck. During the operation, it appeared that the mass was very hard and could only be removed from the mylohyoid bone with sharp dissection. Pathology was reported as a low-grade chondrosarcoma. On control PET, which was performed for the patient again due to mass complaints, there was a mass with left submandibular gland localization, invading the left lateral wall of the larynx and destroying the left side of the hyoid bone ().
On neck CT scan, a mass of 4.5 × 2.5 cm, which was considered to have hyoid bone origin, was localized on the left side of the hyoid bone ().
When MRI images are taken, the metastatic lymph node nodules are seen (Figures –).
With these results, left supraomohyoid neck dissection and hyoid resection of the tumor were performed on the patient.
Histologically, chondrosarcomas, cell atypia, and cellularity are divided into three subgroups according to their characteristics. Myxoid change is frequently observed. This is considered to be intermediate if there is a mycoid structure even if the cellularity is low (Figures –). Pathology was reported as an intermediate-grade chondrosarcoma ().
During the postoperative period, the patient received radiotherapy treatment. No recurrence was observed after radiotherapy. The patient is currently seen once a year. |
pmc-6421772-1 | A 48-year-old female came to our observation due to a limitation in mouth opening range. She also reported crepitus sounds at the left TMJ as well as pain, exacerbated by function (e.g., chewing) and increasing in intensity over the past three months. Clinical assessment showed a limited mouth opening (i.e., 22 mm) and pain with palpation at the left joint and all the main masticatory muscles, more severe on the left side. At the first appointment (T0), mandible manipulation was performed to achieve a forced opening of about 40 mm. A magnetic resonance (MRI) was prescribed to assess the disc-condyle relationship as the possible source of limitation in mouth opening and to gather some pictorial evaluation of the presence of joint effusion (). Despite the clinical suspicion of TMJ arthritis, computerized tomography was not prescribed at this stage due to the expected low impact on treatment planning decisions.
MRI showed a regularly shaped condyle, with an anteriorized disc at closed mouth. At the maximum mouth opening, the condylar translation is reduced and the disc is not recaptured. Joint effusion of severe entity is also present. A conservative approach to provide pain relief and to manage muscle tension was provided, based on counseling, a home program of self-exercise and a stabilization appliance to wear at night. After three months, symptoms improved only partially, with a reduction of pain with muscle palpation but a steady pain at the left joint.
Based on that, a cycle of five arthrocentesis plus viscosupplementation with hyaluronic acid (Sinovial, IBSA) has been performed weekly. Clinical data has been recorded at each time point before each injection and 15 days after the last one ().
After one month from the last arthrocentesis plus viscosupplementation, the patient still showed some pain and, more important, still felt a limitation in the unassisted mouth opening and right laterotrusion. A diagnosis of TMJ intermittent locking on the left side was thus performed, and given the difficulties to stabilize clinical symptoms and mouth opening with the usual conservative approaches, the patient was planned for a surgical removal of the TMJ disc with concurrent HAM positioning ().
TMJ surgery provided condyle remodelling and discectomy (Figures –), after which a HAM patch is positioned within the intra-articular space (), and stratified stitching is performed to avoid postoperatory scars (Figures and ).
Three months after the intervention, the patient showed no negative exitus or postsurgical side effects. Jaw range of motion was increasing, both as for unassisted mouth opening (38 mm) and right laterotrusion (8 mm). The patient reported an overall improvement in chewing efficiency as well as the absence of pain ().
Five months after surgery, a new MRI was performed (). The images showed the physiological excursion of the TMJ condyle during mouth opening (). |
pmc-6421778-1 | An 87-year-old woman with an intractable pain radiating over the left chest, suggestive of intercostal neuralgia, was referred to our spine center for further evaluation and management. Five weeks earlier, she experienced a ground-level fall, causing severe back pain. According to the initial evaluation conducted elsewhere, she was diagnosed as having OVF of the T10 vertebral body and managed conservatively with a brace and pain medication for one month. Her chief complaint gradually changed from bilateral back pain to an aching pain extending to the left thorax before her first visit to our hospital. What is noteworthy about the history of this case is that 8 years previously, she had had a painful OVF of the T12 vertebral body at the age of 79 years, which was treated with vertebroplasty using hydroxyapatite (HA) blocks and posterior pedicle screw instrumentation followed by iliac bone graft fusion from T11 to L1 at another hospital [].
On clinical examination at our hospital, the patient had no neurologic deficits, complaining of nothing but reporting a position-dependent severe chest pain on the left side, which intensified when sitting up from a supine position. Imaging studies revealed neither rib fractures nor abnormal findings of the chest cavity as a possible cause of the chest pain.
On the plain radiographs of the thoracic spine (), the anteroposterior (AP) views and the lateral views taken in the supine position showed the IVC within the T10 collapsed vertebral body as a gas-like radiolucent area, which disappeared on the lateral view obtained in the sitting position (i.e., the opening-closing phenomenon) most likely indicating an OVF of nonneoplastic and noninfectious origin [, ]. The lateral radiograph in the sitting position also demonstrated that the fracture line extended through the posterior fusion mass bone, grafted 8 years ago, indicating a potentially unstable flexion-distraction injury. Close observation of the AP radiograph obtained in the supine position revealed a gas-like radiolucency at the extravertebral space just lateral to the T10 collapsed vertebral wall ipsilateral to the side of her chest pain. The computed tomography (CT) scans, which have a higher sensitivity in detecting gas than radiography and magnetic resonance imaging (MRI) scans, more clearly showed an accumulation of gas located just below the head of the left 10th rib, the place radiologically termed the T10-T11 extraforaminal zone ().
In terms of the IVC contents of radiolucency on the plain radiographs, sagittal MRI revealed a gas-like signal void within the T10 collapsed vertebral body. Corresponding to the extravertebral area of radiolucency shown by the AP radiograph and the CT, both T1- and T2-weighted axial MRI showed combined gas-like and fluid-like signal intensities; i.e., a gas-like low signal intensity area with a fluid-like iso-signal intensity area on the T1-weighted image and a gas-like low signal intensity area with a fluid-like high signal intensity area on the T2-weighted image ().
All these findings from the imaging studies suggested that the patient's chest pain probably resulted from T10 nerve root compression at the T10-T11 extraforaminal zone by a mixed accumulation of gas and fluid, which most likely originated from the adjacent IVC within the collapsed T10 vertebral body as a result of the “force pumping mechanism,” as previously postulated [].
Because of the unstable nature of the fracture involving all structural components from anterior through posterior spinal columns, in addition to her persistent disabling pain not responding to conservative therapy, we carried out posterior spinal fusion from T7 to L2 with pedicle screw instrumentation followed by iliac bone graft (). Immediately after the surgical stabilization, the patient reported a complete resolution of the pain. This patient had no history of antiosteoporosis medications before the referral to our spine center. We administered pharmacological treatments postoperatively, consisting of teriparatide injections initially, followed by oral bisphosphonates. At a 2-year follow-up, she remained asymptomatic, and CT scans showed neither a gas accumulation nor a fluid collection both inside and outside of the T10 vertebral body. |
pmc-6421788-1 | A 56-year-old female patient with an overall healthy oral state visited the clinic for implant placement after the removal of a retained right mandibular 1st molar (tooth 46) root (Figures and ). After local anesthesia, the dental root was separated using a high-speed handpiece and #558 bur, followed by careful atraumatic extraction using an elevator. After drilling into the interradicular septum using only a 4 mm diameter trephine bur to obtain autologous bone, a 10 mm long and 5 mm diameter implant (AnyOne, Megagen Implant Co., Seoul, Korea) was inserted 1 mm deeper than the height of the buccolingual alveolar ridge. The gap between the inserted fixture and extraction socket was about 4 mm in both the buccolingual and mesiodistal directions (). The gap between the extraction socket and implant was filled with autologous particulate bone obtained by grinding the bone core made by the trephine bur (). After equipping it with a cylinder-type healing abutment (), a silicone cap was fabricated as described earlier and then placed over the healing abutment because it was easily stretched using the pincettes (). Except for the force that exerted light pressure on the soft tissues around the extraction socket, the installed silicone cap showed no movement (). Thus, without making an incision or using sutures and a barrier membrane, the extraction socket was sealed using only autologous bone and a silicone cap. The silicone cap is seen as a radiopacity on a radiograph ().
The patient was instructed to be careful not to dislodge the cap with their tongue, masticatory movements, or by brushing. The patient was advised to rinse with a 0.1% chlorhexidine digluconate solution (Hexomedine solution, Bukwang Pharm. Co., Seoul, Korea) twice daily for 2 weeks. Additionally, the patient was prescribed with antibiotics (Augmentin 625 mg, Ilsung Pharm. Co., Seoul, Korea) three times a day for 7 days. On clinical images taken at 2 weeks postinsertion, plaque bacteria were observed adhering to the healing abutment, but not the silicon cap, with only slight discoloration (). The cap also did not create a foreign body reaction or cause pain to the patient. Although the surrounding gingival margin was temporally suppressed by compression due to the silicon cap, healthy soft tissue regenerated, completely sealing the extraction socket (). At 2 months after the removal of the cap, the suppressed soft tissue had healed completely (). A final prosthesis was provided at 6 months after implant insertion, and healthy attached gingiva formed around the final prosthesis (), showing integration with the surrounding gingival margin, including the mucogingival junction (MGJ) line (). Furthermore, regenerated bone tissue was observed on the radiograph (). A radiograph taken at 22 months postoperatively also showed a well-maintained alveolar bone height (). |
pmc-6421788-2 | A 42-year-old male patient was admitted for a retained dental root and root apex lesion at tooth 16 and required an implant prosthesis for an extracted tooth 17 (). Computed tomography () revealed that the retained bone height was insufficient due to the large root apex lesion of tooth 16. After atraumatic extraction of tooth 16, bone-added osteotome sinus floor elevation (BAOSFE) was performed using allogenic bone (SureOss, Osstem Implant Co., Seoul, Korea) to enhance the primary fixation of the implant. This BAOSFE procedure was performed using a drill and osteotome according to Summers' original technique []. A 13 mm long and 6 mm diameter implant (AnyOne, Megagen Implant Co., Seoul, Korea) was then immediately inserted with insertion torque exceeding 40 N cm (); the implant was inserted 1 mm deeper than the height of the alveolar ridge of the extraction socket. After collecting 10 cm3 of peripheral blood, platelet-rich fibrin (PRF) was prepared using a centrifuge (MF550, Hanil Science Industrial Co., Incheon, Korea). After application of the cylinder-type healing abutment, the gap in the extraction socket was filled with allograft material (SureOss, Osstem Implant Co., Seoul, Korea) up to the height of the alveolar ridge of the extraction socket, followed by filling with the PRF membrane up to the height of the soft tissue in the extraction socket (). Due to gingival tearing during extraction, two interrupted nonabsorbable sutures (Dafilon 5-0, B. Braun, Melsungen, Germany) were inserted. After fabricating a silicone cap as described earlier, it was placed over the healing abutment (Figures and ). Using a flapless technique, a nonsubmerged 13 mm long and 5 mm diameter implant (AnyOne, Megagen Implant Co., Seoul, Korea) was inserted into the region of tooth 17, where the ridge had healed, accompanied by BAOSFE to enhance the primary fixation of the implant.
At 2 weeks postinsertion, the cleanly maintained silicone cap and sutures were removed (); the extraction socket showed satisfactory healing, with soft tissue formation (). The final prosthesis was applied 8 months postoperatively. The soft tissue initially suppressed by the silicone cap was restored, and a well-formed attached gingiva was observed (). In addition, stable bone tissue was observed around the inserted implant neck, and increased vertical alveolar bone was observed under the implant (). Clinical and radiographic evaluation at 2 years postoperatively revealed a healthy gingival margin and satisfactory stability (Figures and ). |
pmc-6421800-1 | A 35-year-old female with a past medical history of type 1 diabetes mellitus, hypertension, and hyperlipidemia presented to emergency care with 4 hours of continuous sharp midsternal chest pain. At the time of admission, she admitted to intermittent issues with hyperglycemia over the last several weeks as well as night sweats and malaise but denied fever, weight loss, cough, nausea, vomiting, abdominal pain, dysuria, or vaginal discharge. There was no history of recent travel, cardiac abnormalities, dental procedures, or exposure to tobacco, alcohol, or illicit drugs. Surgical history was pertinent for a Cesarean section performed 3 months prior to admission complicated by postpartum hemorrhage from placental abruption. On exam, she was awake and alert with no respiratory distress. Her vital signs were BP 116/72 mmHg, pulse 97 bpm, temperature 99.7°F, and respiratory rate 24 breaths/minute on room air. The physical examination was normal except for a systolic ejection murmur at the left lower sternal border and a well-healed low transverse Cesarean section incision. Her lab data showed a WBC count of 10,500 c/mm3 (normal: 4,800–10,800 c/mm3), hemoglobin of 7.6 g/dl (normal: 12.0–16.0 g/dl), MCV 84.5 fl (normal: 80.0–9.0 fl), creatinine of 1.58 mg/dl (normal: 0.60–1.30 mg/dl), and Troponin I of 8.29 ng/ml (normal: <0.03 ng/ml). EKG demonstrated ST elevations in leads II, III, and aVF consistent with inferior acute myocardial infarction (). She was taken emergently to the cardiac catheterization lab where she was noted to have a completely occluded right coronary artery (RCA) (). Consistency of the occlusion and absence of calcium suggested acute thrombus or embolus. An aspiration was attempted but was unsuccessful, so a 3.0 × 38 mm bioabsorbable drug-eluting stent was placed into the RCA along with balloon dilation of the proximal aspects of the posterior descending artery and distal RCA (). Left-sided coronaries were angiographically normal (). A postcatheterization transthoracic echocardiogram then revealed large mobile aortic valve vegetations on all 3 leaflets with the largest measuring 1.4 × 1.7 cm, moderate aortic insufficiency, severe tricuspid regurgitation with a 1.1 × 0.5 cm mobile vegetation on the anterior leaflet of tricuspid valve, and moderate mitral regurgitation without lesions. Ejection fraction was estimated at 50% with inferior and inferolateral wall hypo/akinesis. These findings were later confirmed with transesophageal echocardiogram, which also identified a patent foramen ovale with continuous right-to-left shunt (Figures and ). Chest CT imaging noted no embolic disease.
Blood cultures grew Enterococcus faecalis in 4 of 4 vials. Vancomycin and ceftriaxone were initiated and the patient was transferred to an outside facility where she underwent urgent cardiac surgery with the placement of bioprosthetic aortic and tricuspid valves, as well as PFO closure. Bioprosthetic valves were used as per patient preference. Cultures of removed cardiac tissue were positive for Enterococcus faecalis. No evidence of aortic root abscess was noted during the surgery. CT of the chest, abdomen, and pelvis was unrevealing for sepsis source. Endocarditis was ultimately attributed to prior placental abruption.
After valve replacement, she was continued on IV antibiotics. Blood cultures cleared, and on hospital day 15, she was discharged on IV ampicillin and ceftriaxone for synergy. Three weeks after discharge, a repeat ECHO revealed an ejection fraction of 47% and severe hypokinesis of the basal to mid inferior wall with normal function of both aortic and tricuspid bioprosthetic valves. After completing 6 weeks of IV antibiotic therapy, she was transitioned to chronic oral suppression with amoxicillin/clavulanic acid. At phone follow-up 6 months later, the patient reported tolerating oral suppressive antibiotic with no clinical signs or symptoms of recurrent infection or complications. |
pmc-6421801-1 | A 41-year-old female patient attended the Specialty in Cosmetic Dentistry, Cosmetic, Restorative, and Implantology Clinic of the Faculty of Stomatology, Autonomous University of San Luis Potosí, Mexico, for consultation due to the mobility of the clinical crown of tooth #21 because of a trauma The patient did not have relevant medical records, and thus, we requested a computerized axial tomography (CT) scan to assess the thickness of the buccal bone table and the coronal fracture.
In the intraoral clinical evaluation (), mobility grade 3 of the clinical crown of tooth #21 was diagnosed. A panoramic X-ray was taken to assess the root morphology of tooth #21 (). The CT revealed that tooth #21 was fractured at the cervical level of the clinical crown () and that dental extraction was indicated without the possibility of placing a postextraction implant, since there was insufficient thickness of the external vestibular table and the available remnants would lead to a fenestration or lack of primary stability.
Atraumatic extraction of tooth #21 was planned, along with alveolar preservation to minimize bone reabsorption, placing a provisional restoration with the same autologous clinical crown of extracted tooth #21, which would maintain the same gingival level as that of the natural tooth.
The tooth extraction procedure for tooth #21 consisted of first separating the fractured clinical crown () to subsequently and atraumatically extracting the root remnant with #65 forceps. The alveolus was curetted to eliminate any remaining granulation tissue and periodontal ligament, and to induce bleeding, an InterOss® alveolus was placed in the 0.5 g bone xenograft (Anorganic Cancellous Bone Graft Granules) () to subsequently seal the alveolus surface with a collagen membrane of 8 mm × 12 mm (Colla Tape, Zimmer) sutured with Vicryl 4-0.
Once the clinical crown was separated from the root of tooth #21, it was washed with physiological serum and disinfected with 2% chlorhexidine, and a pulpotomy of the crown was performed to eliminate the pulp tissue and to seal the pulp chamber with a conventional composite and the ovoid pontic (). The crown was fused with a metal ligature and conventional composite on the palatal surfaces of teeth #11 and #22 (). The cervical part of the provisional crown should exert slight pressure in the area of the extraction, thus beginning to create a natural emergence profile for at least 3 months after this alveolar preservation to set the implant in place. The provisional restoration was omitted from the occlusion in the opening-closing and mandibular eccentric movements ().
The patient was scheduled for an appointment 1 week after the tooth extraction to remove the sutures () and to evaluate possible inflammation after extraction. Nevertheless, a healthy gum was observed, including a coral pink color, and the dental papilla had maintained its dimensions. At 1 month after surgery, the soft tissue was evaluated and was not collapsed, did not present gingival inflammation, and demonstrated a maintained confirmation of the volume and dimension of the papillae (). The autologous clinical crown was removed to evaluate the edentulous flange. Next, we evaluated the conformation of the ovoid pontic, and we decided to add a small amount of the conventional composite on the pontic to provide a natural emergence profile (Figures and ). At 3 months after the extraction, the level of the soft tissue was observed (Figures and ). An intraoral scanner (3Shape) was applied for dental implant placement and surgical guidance (Figures and ). |
pmc-6421802-1 | An 89-year-old gentleman with no significant past medical history presented to the emergency department (ED) of a district general hospital with atraumatic right hip pain and inability to weight-bear. The patient had a long history of right hip osteoarthritis causing pain and functional limitation, for which he had been referred by his general practitioner to the orthopaedic team for consideration of total hip replacement three years previous. However, the pain experienced during the week preceding ED presentation was much more severe in character. Of note, he had been seen by his general practitioner in the week preceding ED attendance with intense right hip pain on weight-bearing and passive movement and had been referred to the elderly care team with a view to optimising pain management.
Plain film radiography obtained in the emergency department () demonstrated a lytic lesion within the right femoral neck, intertrochanteric region, and proximal femoral metaphysis with evidence of cortical breach and progressive sunburst periosteal reaction.
The patient was admitted for further investigation and assessment. Computed tomography (CT) of the chest, abdomen, and pelvis () confirmed a bony lesion affecting both the right acetabulum and proximal femur with pathological intertrochanteric fracture and abnormal surrounding soft tissue. Magnetic resonance imaging (MRI) () findings were felt to be consistent with that of a primary bone tumor; tumoral necrosis was evident, and thigh muscles demonstrated oedema to the knee level. No lymphadenopathy was seen. Nuclear medicine scanning () demonstrated avid uptake in the right acetabulum and proximal femur with cortical breakthrough and marked extension to the surrounding thigh compartments but no uptake suggestive of distant disease. Fluorodeoxyglucose-positron emission tomography (FDG-PET) was not performed preoperatively.
Histological analysis of needle core biopsies obtained via a direct lateral approach under ultrasound guidance of the right femur was undertaken; the findings of which were in keeping with that of diffuse large B-cell lymphoma (DLBCL). Sections through the cores demonstrated tissue infiltration by large round blue cells staining positively for leukocyte common antigen, CD20, BCL2, and BCL6 and negatively for CD10, CD3, CD5, and cyclin-D1. The Ki67 proliferation index was high at approximately 80%. Interface fluorescent in situ hybridisation detected a BCL2 translocation but no BCL-6 or c-myc translocations.
This gentleman was transferred to Northern Ireland's Regional Trauma Centre where he underwent proximal femoral replacement with acetabular reconstruction, owing to the presence of pathological fracture as visualised on CT. Pathological analysis of the operative specimen demonstrated a tumor present within the medullary cavity with cortical, periosteal, and soft tissue involvements. The osseous resection margin was clear; however, the soft tissue resection margin was involved laterally. Histological analysis confirmed DLBCL of germinal centre phenotype staining positive for CD20, BCL6, and BCL2 and negative for CD3, CD5, CD10, MUM-1, cyclin-D1, C-MC, and TdT. The proliferation index was again high with MIB-1 of over 90%.
The patient received postoperative care in the intensive care unit where he required inotropic support ahead of ward transfer. FDG-PET undertaken two weeks postprocedure demonstrated high FDG uptake at the surgical site, but metabolically active disease at other sites was not seen.
He was discussed at the haematological multidisciplinary meeting postoperatively, and the diagnosis diffuse large B-cell lymphoma (DLBCL), NOS (M9680/3), was agreed. He was deemed stage IEA due to the involvement of contiguous bones. The patient embarked upon six cycles of R-Mini-CHOP (a regimen of rituximab with decreased dose cyclophosphamide, doxorubicin, vincristine, and prednisolone []), a regimen which is in widespread use within the unit amongst those greater than 80 years of age.
Throughout his R-Mini-CHOP treatment, the gentleman was able to fully weight-bear and made excellent progress with the rehabilitation team. Radiographically, implant alignment was satisfactory, and there was no evidence of prosthetic loosening. The patient subsequently declined radiotherapy treatment. Repeat FDG-PET was conducted five months following diagnosis; no further disease was demonstrated. |
pmc-6422875-1 | A 53-year-old woman with a history of asthma bronchiale and chronic obstructive pulmonary disease had a cardiorespiratory arrest due to status asthmaticus. After successful cardiopulmonary resuscitation the normal sinus rhythm returned after 10 min. Tonic-clonic seizures, action myoclonus and clinical pyramidal syndrome developed. The patient's condition improved after 7 weeks and she was weaned from mechanical ventilation and referred to the neurological and then rehabilitation unit.
She was first admitted to our neurological department 9 months after the cardiac arrest. Neurological examinations revealed: the patient was fully conscious, attentive, and oriented; she had mild cognitive concentration deficits and echolalia, right lateral end-gaze nystagmus and central VII cranial nerve deficit. Her motor strength was 4/5 throughout muscle tone was markedly increased in the lower extremities with brisk deep reflexes, and a positive Rossolimo sign on both sides. She also had ataxia in both upper limbs without any sensory deficit. Intention myoclonic jerks were noted in the face, trunk, and upper and lower extremities.
She was partly able to feed herself, sit up, get up, and walk with the help of a walker and other persons.
Nine months after CPR, EEG showed correct basic activity and symmetric and synchronous paroxysmal sharp wave discharges and sharp and slow wave complexes on both sides. A hyperventilation test increased the number of discharges. Quantitative EEG (QEEG) analysis was performed in a frequency domain using the fast Fourier transform (FFT) algorithm following the guidelines given in Gupta and Caviness () and revealed a predominance of slow frequencies (1–7.5 Hz) measured as absolute spectral power (p < 0.01), with peaks at ~7.1 Hz in most of the 19 channels. On analyzing the ratios of relative power [in bands: delta (1.0–3.5 Hz), theta (3.5–7.5 Hz), alpha (7.5–12.5 Hz), beta-1 (12.5–18 Hz), and beta-2 (18–24 Hz)] as an amount of EEG activity in a frequency band divided by the amount in all bands, disproportions in spatial distribution (p < 0.05) were observed for both occipital channels (caused mainly by alpha activity) and the central area (electrodes Cz and Pz, caused by slow frequency predomination). We found no hemispherical asymmetry (p < 0.05) (, ).
Hemispherical asymmetry was found in the multimodal evoked potentials. The latency of P100 of the visual evoked potential was bilaterally prolonged, more so on the right side (left P100 = 138 ms, right P100 = 146 ms). We also found a slight discrepancy between sides in inter-wave latencies III-V, I-V of the brainstem auditory evoked potentials, longer on the right side (2.32 and 4.57 ms vs. 1.94 and 4.12 ms). All somatosensory evoked potential parameters from the median nerve were within normal limits.
At the same time, we performed a brain MRI with a 1.5T unit (Signa Hdx, GE Medical System) using a 16-channel coil design for head and spine imaging. The imaging protocol included conventional axial, sagittal and coronal T2-weighted images, axial and coronal T1-weighted images and axial FLAIR (fluid-attenuated inversion recovery sequences) images, as well as MR spectroscopy (MRS). The MRS examinations were performed using the Single Voxel Spectroscopy (SVS) technique (PRESS sequence). Using localizing axial T2-weighted images, voxels of 2 × 2 × 2 cm (8 cm3) were placed in the posterior cingulate gyrus (PCG) and left parietal white matter (PWM). Ratios of N-acetylaspartate (NAA), choline (Cho) and myo-inositol (mI) to creatine (NAA/Cr, Cho/Cr, mI/Cr, respectively) were calculated and analyzed.
The MR images showed diffuse, mild cortico-subcortical brain atrophy (). MR spectroscopy revealed a decreased NAA/Cr ratio in the PCG region, as well as within the parietal white matter (); the NAA/Cr ratios were 1.25 and 1.28, respectively. The other metabolite ratios presented values within the normal limit, as follows: Cho/Cr = 0.53, mI/Cr = 0.57 in PCG and Cho/Cr = 0.86, mI/Cr = 0.69 in the PWM area.
No other abnormalities were noted. Laboratory results were within normal limits.
The patient received intensive rehabilitation and antiepileptic treatment (sodium valproate 2,000 mg/d, levetiracetam 1,000 mg/d).
Twenty six months after cardiac arrest the patient was admitted to our department for the second time because of an increased amount of myoclonic jerks. The patient presented a similar neurological status as before. She could do everyday activities, such as feeding or toilet on her own.
The levetiracetam dosage was increased (up to 3,000 mg/d) leading to a marked reduction in the myoclonus.
EEG performed 26 months after CPR showed the normal basic function of both hemispheres in comparison with the previous reduced results. The number and amplitude of slow wave discharges and sharp and slow wave complexes were reduced. QEEG revealed statistically significant (p < 0.05) differences in the proportions of the contribution of each frequency band in the spectrum between the current and previous examinations; proved for 17 of 19 EEG channels (with the exception of Fz and Cz). This is due to a change in the relative spectral power calculated for the 3.5–7 Hz band and (to a lesser extent) the 18–24 Hz band in the signal recorded 26 months after CPR in comparison to the EEG measured 17 months earlier (). The first study presented a smaller relative-magnitude of theta and a larger one of beta-2 waves, and the ratio of energy possessed by EEG at both frequency bands (theta/beta-2) changed from 1.1, 1.7, and 1.4 to 3.4, 3.9, and 4.0 on average for frontal, occipital and tempo-cervical areas, respectively (p < 0.01). The dominant peak frequency in the theta band moved its position to ~6.2 Hz. No statistically significant disproportions were found in the spatial distribution of the relative spectral power. The spectral maps prepared for EEG recorded 9 and 26 months after CPR presented some changes in this distribution ().
The follow-up MR image () revealed an increased rate of brain atrophy after 2 years, MRS study—a slight improvement in NAA/Cr ratios in the cortex of PCG and a moderate one within white matter (), but still the NAA/Cr ratios remained decreased (NAA/Cr = 1.29 in PCG and NAA/Cr = 1.42 in PWM).
The patient gave her written informed consent to participate in the study, and for the publication of this case report. |
pmc-6423350-1 | A 48-year-old lady presented for the last 6 months with dull aching epigastric pain, early satiety and weight loss, she has not recorded the degree of weight loss but she knows that from her clothes. There are episodic attacks of vomiting which non-bilious and not containing blood. |
pmc-6423352-1 | A 47-year old male patient presented to the emergency department of our center with a 4-day history of moderate abdominal pain increasing gradually in severity over the duration of illness. The pain started as generalized all over the abdomen, and then mobilized to the right iliac fossa. The pain was associated with frequented vomiting which started as greenish juice then became food particles. It was also associated with increased an abdominal girth and obstipation. Physical assessment showed a distressed patient with a respiratory rate of 22 and heart rate of 120 bpm. His Blood pressure was 90/60 and Temp 37.8° C orally. O2 Saturation was 90 at room air. Abdominal examination revealed marked abdominal distention, with localized guarding in right iliac fossa. On Digital rectal examination there was no masses or blood and no stool. On presentation, laboratory findings included elevated Creatinine (1.5 mg/dl), BUN (27.0), Na (143) and normal WBC count (5900 cell/mm3), hemoglobin (10.4 g/dl), platelets (136 × 103/μl), INR 1.7. Plain abdominal radiographs showed dilated small and large bowel loops ().
The abdomen CT scans with contrast enhancement was advised after resuscitating the patient and stabilizing his condition, and showed dilated small and large bowel with sigmoid volvulus plus large amount of preihepatic and pelvic free fluid. Swirl sign at the mesentery of cecum suspected strangulated bowels due to transmesentric defect (). Intraoperative findings: 2 × 4 cm defect in the mesentery of cecum, Gangrenous sigmoid volvulus and part of the terminal ileum entrapped through the mesenteric gate, and much free fluid (). Surgical treatment started with deflating the sigmoid colon, and reducing it through the defect. We also reduced the small bowel. After that, resection of all nonviable segments was performed. End-to-end anastomosis of small bowel, colostomy on the left side, and closure of defect was done. Immediately after recovery from surgery, the patient was transferred to ICU and stayed for three days, during which he developed atelectasis and systemic inflammatory response with acute kidney injury and blood hemolysis but these rapidly reverted to normal. Following that, the patient was transferred to surgical ward in which he spent seven days with a complication of superficial wound infection and ileus. The infection was treated with IV antibiotics according to antimicrobial susceptibility testing and dressing was applied. Ileus was treated conservatively. On the tenth postoperative day, the patient was discharged with functioning stoma and excellent general condition. We are planning to restore GI continuity after 3 months. |
pmc-6423710-1 | An 80-year-old man with post cerebral infarction, depression, epilepsy, gastrostomy, lower limb arteriosclerosis obliterans, and angina pectoris had recurrent empyema in the left thoracic cavity secondary to pulmonary suppuration. He presented with high fever. CT scan revealed empyema, and Streptococcus constellatus was detected by wound culture. Irrigation and thoracic drainage (20-Fr trocar) were performed at the eighth intercostal space in the anterior axillary line, and broad-spectrum antibiotics were started. VATS decortication could not be performed due to chronic empyema. Thoracic drainage was continued for 21 days; however, the cavity did not noticeably shrink. As the patient could not undergo OWT due to his poor general condition, plastic surgeons were consulted, and on day 24, non-OWT NPWTi-d was initiated. Initially, NPWTi-d was installed as explained in method 1. However, it was changed to method 2 on day 6, in order to more effectively wash the deep part and apply uniform negative pressure. After initiation of non-OWT NPWTi-d, the cavity began to shrink. Since NPWTi-d is indicated for medical insurance only for 28 days in Japan, the patient bears the full expenses if further treatment is continued. Therefore non-OWT NPWTi-d was continued for 31 days until the fistula closed. However, three days later, he had a recurrence of high fever. CT scan revealed the recurrence of empyema, and Staphylococcus aureus was detected by wound culture. The fistula, which was closed with NPWTi-d, remained epithelialized. Thoracic drainage (12-Fr aspiration catheter) was performed under CT guidance, and broad-spectrum antibiotics were re-initiated. On day 85, the empyema healed, and he was transferred to another hospital for long-term care. |
pmc-6423710-2 | A 65-year-old man with diabetes, hypertension, and spinal cord injury had empyema in the right thoracic cavity after partial lung resection for adenocarcinoma. He presented with high fever on postoperative day 10. CT scan revealed empyema, and Streptococcus caprae was detected by wound culture. Irrigation and thoracic drainage (20-Fr trocar) were performed under fluoroscopy. Broad-spectrum antibiotics were started, and treatment was continued for 16 days. Although the cavity was confirmed to be reducing in size, the infection persisted. Hence, plastic surgeons were consulted, and on postoperative day 36, non-OWT NPWTi-d was installed by method 2. It was continued for 20 days until the CT scan confirmed that the empyema disappeared. Four months after completing non-OWT NPWTi-d, his CT scan showed no empyema recurrence (Fig. ). |
pmc-6423748-1 | A 35-year-old G4P2012 presented to labor and delivery at full term in early labor with spontaneous rupture of membranes. She had undergone living donor kidney transplant 8 years prior for ESRD suspected secondary to IgA nephropathy versus post-streptococcal glomerulonephritis. She was maintained on Prograf and prednisone throughout her pregnancy with a stable baseline serum creatinine 1.2 mg/dL. Obstetrical history was significant for two prior uncomplicated Cesarean deliveries, both prior to her kidney transplantation. It was unclear from her records whether she had undergone counseling regarding pregnancy post-transplant. She declined a of trial of labor after Cesarean section despite counseling on the risk of injury to the transplanted kidney given its location in the operative field, which was confirmed on transabdominal ultrasound prior to the case.
The patient was taken to the operating room for and indicated repeat Cesarean section. Routine dissection was performed down to the level of the fascia through a Pfannenstiel incision and the fascia was carefully transected horizontally in the usual fashion. When the superior fascial edge was lifted for traction to allow for dissection from the underlying rectus muscle, brisk pulsatile bleeding was noted. The transplanted kidney was noted to be densely adherent to the overlying anterior abdominal wall, and avulsion of a portion of the transplanted kidney was confirmed (Fig. ). Direct pressure was applied to the organ while the Trauma Surgery team was consulted. The avulsion was repaired using 3–0 pledgetted Prolene sutures in interrupted horizontal mattress fashion and using Evarrest compound. At this time, the decision was made to extend the skin incision in the vertical midline for improved access to the uterus given the concern for further shearing forces to the exposed transplanted kidney in the process of delivering the baby. The rest of the case was uneventful. No blood product transfusion was necessary. After the transplanted kidney was repaired, a viable male infant with Apgars of 8 and 9 weighing 2460 g was delivered.
Postoperative serum creatinine levels remained stable between 1.1–1 .3mg/dL and the patient maintained adequate urine output. Renal ultrasound immediately postpartum and 1 month postpartum demonstrated normal arterial and venous flow with no evidence of ischemia or perinephric hematoma. |
pmc-6423750-1 | A 30-year-old man complained of a gradually enlarged mass in the right lower abdomen. The results of an abdominal ultrasound taken at the local hospital 3 months prior showed a heterogeneously mixed echo mass located in the right lower abdomen, and the size of the mass was approximately 8.6 cm × 7.3 cm. He had no family history of malignancy but had a history of bilateral undescended testis since birth. The local medical officer suspected a testicular tumor according to the history of cryptorchidism. At the same time, his rapid HIV-1 antibody test showed positive results, and the baseline CD4 count was 70 cells/ml (normal: 404–1612 cells/ml) upon further testing, which suggested severe immunosuppression. The patient initially received combination antiretroviral therapy (cART) but refused treatment for the abdominal mass. As the mass rapidly grew for 3 months, he came to our hospital for treatment of abdominal neoplasm. Upon examination, an immobile and nontender mass was visibly noticeable and palpable in the right lower abdomen. The bilateral testis was not visualized and could not be palpated. The patient had significantly elevated levels of alpha-fetoprotein (AFP), slightly elevated levels of beta-human chorionic gonadotropin (β-HCG), moderately decreased levels of hemoglobin and a low CD4 count (Table ). Further evaluation revealed a low viral load, which was less than 40 copy/ml. Computed tomography (CT) images showed a large, lobulated, ill-defined heterogeneous retroperitoneal mass measuring 17 cm × 16 cm × 24 cm without fat or calcifications and with marked inhomogeneous enhancement due to the presence of necrotic-colliquative areas (Fig. a). The lesion displaced the bilateral lower ureters, resulting in bilateral hydronephrosis. The lesion also compressed the surrounding small intestine, with possible infiltrating signs. Around the mass, ascites was detected, but no enlarged lymph nodes were found. Cystic-solid masses (5 cm × 5 cm) were detected in the bilateral inguinal regions, and their density and enhanced characteristics were similar to those of the retroperitoneal neoplasm (Fig. a, b). A diagnosis of testicular tumors with bilateral inguinal region metastases was suspected. The patient underwent routine clinical staging and prognosis evaluations according to the results of radiological and laboratory examinations. Clinical staging and risk group categorization were classified as stage IIIC and poor prognosis using the American Joint Committee on Cancer staging system (AJCC) [] and the International Germ Cell Cancer Collaboration Group (IGCCCG) prognostic scoring scheme (IGCCCG, 1997), respectively [].
An ultrasound-guided biopsy was performed on the 5th day after admission, and histology revealed a malignant yolk sac tumor of the testis. Then, the patient underwent exploratory laparotomy, retroperitoneal neoplasm resection, partial ileum resection, ileal anastomosis, and double J ureteral stent implantation under general anesthesia on the 11th day after admission. Histopathological examination revealed the lesion as a malignant mixed GCT of the undescended testis composed predominantly of yolk sac tumors with foci of embryonal cell carcinoma and seminoma (Fig. a, b). The repeated hematological and tumor markers 2 days after the operation are listed in Table . Hemoglobin level was still low; then, 4 Units of suspended red blood cells were transfused to correct anemia with no obvious transfusion reaction. The level of AFP was still high, and the follow-up CT scan showed a gradually enlarged right inguinal mass (Fig. c, d) 1 week and 2 weeks after the surgery, respectively. On the 17th day after the operation, the patient underwent right inguinal mass resection under local anesthesia. Pathological examination revealed mixed GCT metastasis (Fig. c), accompanied by hemorrhage and necrosis. Subsequently, the patient received salvage chemotherapy with a regimen of cisplatin, etoposide, and ifosfamide. Hematology was closely monitored during the treatment. After the first cycle of chemotherapy, the full blood counts remained within the normal range. CD4 count did not decrease during the course of treatment and remained at 118 cells/ml. Then, the patient was discharged and waited for the next cycle chemotherapy. Unfortunately, on the 4th day after discharge, he showed several symptoms of cancer cachexia including fever, progressive weight loss, pain, and severe weakness. Moreover, a low platelet count, which is a side effect of chemotherapy, was found (20 × 109/L, normal 125–350 × 109/L). Therefore, IL-11 was administered subcutaneously at a dosage of 50 μg/kg/day for thrombocytopenia. Two days later, the platelet count was still low (23 × 109/L); on the next day, the patient died because of severe immunosuppression, a low platelet count and cancer cachexia. |
pmc-6423802-1 | An 8-year-old Moroccan girl presented to our institution with painful swelling of both forearms which initially appeared on her left forearm and 6 months later on her right forearm. Her family history and medical history were unremarkable. Given the exacerbation of the pain she consulted a doctor who obtained plain radiographs and noted a lesion in both forearms. She was then referred to us for further evaluation. She reported that despite daily use of nonsteroidal anti-inflammatory medications and narcotic analgesics, the pain in her forearms continued to progress. On physical examination she had no fever and had a good general condition. She presented a swelling in the upper third of her right forearm and the upper two-thirds of her left forearm with inflammatory signs (Fig. ). Laboratory studies found a moderate anemia (hemoglobin at 10 g/dL) and a white blood cell count of 11,210/μL with 80% neutrophils. Her C-reactive protein level was 60 mg/L. We obtained plain radiographs (Fig. ) that showed: an osteolytic lesion of the upper one-third of the right ulna and osteolytic lesion of the upper two-thirds of the left radius. On both forearms, we did not note mineralized matrix production, but a cortical breakthrough and internal trabeculations were present. We therefore performed magnetic resonance imaging (MRI) (Fig. ) which showed: an osteolytic lesion mass (arrow) of the upper one-third of her right ulna and the upper two-thirds of the left radius. The mass was invading her elbow joint whose matrix was in hyposignal T1 (Fig. a), hypersignal T2 (Fig. b), and short T1 inversion recovery (STIR) (Fig. c), containing septa and enhanced annularly after injection of gadolinium (Fig. d). The lesion began in the diaphysis and crossed the physis. A soft tissue mass and cortical breakthrough were noted. A soft tissue edema was also seen. A biopsy curettage of her left forearm was done. A histopathologic examination revealed a proliferation of histiocytes with an infiltration of eosinophils (Fig. ). These histiocytes were positive for S-100 protein (Fig. a) and for CD-1a (Fig. b). No chemotherapy was necessary. Our patient’s symptoms disappeared after a short (5 days) period of nonsteroidal anti-inflammatory therapy. A repeat X-ray was obtained (Fig. ) and showed a partial improvement of the osteolysis. She remained asymptomatic after 6 months. |
pmc-6423812-1 | A previously healthy Japanese 63-year-old male was referred to the AIDS Clinical Center, National Center for Global Health and Medicine (Tokyo, Japan; day 0) because of protracted dyspnea on effort (DOE) after pneumocystis pneumonia (PCP) treatment.
Fifty-seven days before referral (day − 57), he was admitted to a local hospital for progressive dyspnea of one month duration with diffuse interstitial infiltration in bilateral lung fields (chest computed tomography (CT), Fig. a, b). Human immunodeficiency virus (HIV) infection was first pointed out upon initial blood examination, and the non-acute phase of HIV-1 infection was confirmed by western blotting. Cluster of differentiation-4 counts and HIV-RNA loads were 45/μL (7.3%) and 56,000 copies/mL, respectively. Bronchoscopy identified Pneumocystis jirovecii in bronchoalveolar lavage fluid, and levels of β-D glucan in serum was increased (> 300 pg/mL) at that time point. With a diagnosis of HIV-related PCP, trimethoprim-sulfamethoxazole (TMP-SMX) was initiated with corticosteroids at that hospital (day − 47). Hypoxia under rest was improved rapidly, but DOE remained 2 weeks after completion of PCP treatment (day − 12). He was referred to our hospital for the further examination and treatment (day 0). Physical examination revealed “Velcro rales” in bilateral lower back auscultation, whereas no other abnormalities were identified by a review of systems (including neurologic examination). Arterial blood oxygen pressure was within normal range (93.5 mmHg) at rest, but decreased rapidly 30 s after a slow walk (44.5 mmHg). Respiratory function tests showed severe restrictive ventilator impairment (vital capacity = 36.5%; forced expiratory volume in 1 s = 107.4%). Re-examination of chest CT showed severe fibrotic changes at bilateral basal parts and diffuse fibrotic changes (Fig. c, d) in which PCP lesions were seen initially in previous images. Levels of SP-D (214.2 ng/mL), KL-6 (2249 IU/mL) and lactate dehydrogenase (234 IU/L) were increased, but β-D glucan was not elevated (14.8 pg/mL) and P. jirovecii was not detected in saliva at referral. Cytomegalovirus DNA in plasma and surrogate markers of autoimmune diseases (anti-centromere antibody, anti-Jo-1 antibody, c-ANCA, p-ANCA, anti-nuclear antibody, anti-RNP antibody, anti-SS-A antibody, anti-SS-B antibody, anti-Scl-70 antibody, anti-CCP antibody) was negative. The drug-induced lymphocyte stimulation test (DLST) against TMP-SMX was negative, but we changed secondary prophylaxis of PCP to atovaquone. We initiated combination antiretroviral therapy (cART) comprising dolutegravir, tenofovir alafenamide and emtricitabine at day 2, and added tacrolimus at day 16, but respiratory function and imaging findings were not improved. Corticosteroids (methylprednisolone [1000 mg/day, p.o.] for 3 days followed by prednisolone [1 mg/kg/day, p.o.]) were re-initiated at day 37. However, we stopped corticosteroid therapy because it caused mediastinal emphysema without eliciting any positive effects upon lung fibrosis. We started nintedanib at day 48, and are following up symptoms carefully. Oxygen requirement until the last follow-up date (day 120) was unchanged, that is 2 L/min at rest or 4 L/min at light labor. |
pmc-6423814-1 | A 56-year-old Caucasian man presented for evaluation of intermittent dysphagia to solids for the past few weeks. He had no significant past medical history. He had the sensation of food getting stuck in substernal area. Otherwise he reported good appetite and no weight loss. A physical examination revealed a well-nourished man with no palpable mass or lymph nodes. An abdominal examination revealed no localized tenderness or organomegaly. No family history of stomach or colon malignancy was reported. An esophagogastroduodenoscopy (EGD) examination revealed a single 6 mm nodule at GEJ (Fig. ) and Los Angeles grade A (less than 5 mm mucosal breaks) distal esophagitis. A pathology examination from the GEJ nodule showed squamous mucosa with mucosal ulcer and associated acute and chronic inflammatory infiltrates. Nests of atypical cohesive cells with clear cytoplasm, and mild nuclear pleomorphism were noted in submucosa. Multiple immunostains were performed to further characterize atypical cells with following staining pattern: vimentin (+), PAX-8 (+), CD10 (focally and weakly positive), and epithelial membrane antigen (EMA; focally and weakly positive) (Figs. , , , and ). These cells were negative for RCC, thyroid transcription factor 1(TTF1), S100 protein, CD68, cytokeratin 5/6, pancytokeratin, p63, cytokeratin 7 and 20, p40, and pan melanoma marker. Histological features and staining patterns were consistent with atypical clear cell infiltrate involving squamous mucosa, which was consistent with metastatic RCC. A computed tomography study of his chest, abdomen, and pelvis performed with orally and intravenously administered contrast showed unremarkable esophagus and no mediastinal lymphadenopathy.
A solid-appearing, partially exophytic mass involving his superior left kidney was seen. The renal mass measured 5.8 × 5.3 cm. The mass was heterogenous and displaced portions of upper pole. The mass was abutting the inferior aspect of his spleen, but a thin fat plane separating his spleen from the mass was seen. A small 0.6 cm gastrohepatic lymph node was seen which radiologically appeared benign. A positron emission tomography (PET) scan with 16 mCi of 18-flurodeoxyglucose showed a left upper pole renal mass measuring 5 cm with standardized uptake value (SUV) of 3, suspicious for renal malignancy. No other abnormal uptake was seen to suggest metastatic disease or lymphadenopathy. A pathology conference and expert opinion concluded that the GEJ nodule biopsy was consistent with metastatic renal clear cell carcinoma (Fuhrman grade 2) involving esophageal mucosa with erosion. Clear cell morphology showed sharp cell borders and lack of glands or mucin, consistent with RCC. A multidisciplinary conference recommended left renal nephrectomy. Our patient underwent left radical nephrectomy and path showed clear cell RCC, with no sarcomatoid features, Fuhrman nuclear grade 3. Resections margins were negative and no involvement of adrenal gland was noticed. Endoscopic submucosal dissection of area of GEJ nodule was performed, which showed squamous and gastric-type mucosa with chronic inflammation and underlying adipose tissue in submucosa, negative for malignancy. He has been doing well clinically for the past 16 months, after left nephrectomy. Oncology services recommended close surveillance and conservative management after long discussion with our patient and other consultants. |
pmc-6423826-1 | A 67 years old previously healthy male, a retired clerk presented to the medical casualty with a history of fever for three days. It was associated with arthralgia, myalgia, headache and loss of appetite. He did not have respiratory, urinary symptoms and bowel habits were normal. He denied any history of exposure to leptospirosis or contact history of fever. On admission, his general examination was normal with a heart rate of 80 bpm and blood pressure of 100/70 mmHg. Other system examination was unremarkable. After admission it was noted that his urine output is low while he was on maintenance fluid. Initial investigations revealed neutrophilia with normal white blood cell count, thrombocytopenia, elevated blood urea, serum creatinine, C-reactive protein and AST. Urine analysis showed 4–6 pus cells, 1–2 red cells with granular casts. Clinical diagnosis of leptospirosis was made on high index of suspicion although there was no significant history of exposure to leptospirosis. Patient was started on intravenous cefotaxime.
By the day five of illness, he developed confusion (GCS-14/15), low blood pressure (80/40 mmHg) with tachycardia (117 bpm), high fever spike (103 F), and mild dyspnea with SpO2 98% on air. ECG showed sinus tachycardia, non-contrast CT brain was normal, 2D echocardiogram revealed ejection fraction of > 60%, chest X ray-PA was normal, and troponin I titer was marginally positive. Ultrasound abdomen showed renal parenchymal changes with normal sized kidneys. Serum creatinine was rising. Patient was started on inotropic and vasopressor support to maintain blood pressure. Even after achieving mean arterial pressure > 65 mmHg patient went in to anuric acute renal failure. Meanwhile he developed rapid atrial fibrillation which was settled with electrical cardioversion. He was given hemodialysis on day 6 of illness. On day 7 of illness again patient developed rapid atrial fibrillation and it did not respond to electrical cardioversion and started on IV amiodarone infusion and patient regained sinus rhythm and could tail off inotrope and vasopressor. Since day 8, he gradually improved clinically with good urine output, hemodynamic stability and confusion settled. But he did not recover from acute kidney injury and renal functions remained rising again. He was given another hemodialysis on day 12 of illness. Then his renal functions slowly improved and discharged on day 17 of illness with a follow up plan at nephrology clinic.
On discharge patient had normal platelet count, C-reactive protein, liver transaminases, ECG. Serum creatinine was static around 250 micromol/l. Repeat 2 D echocardiogram which was done three weeks after recovery was normal. Leptospirosis antibody titre (MAT) on day 7 of illness was positive. (1:10240). |
pmc-6423826-2 | A 17 year old male patient presented with fever for two days. Fever was associated with chills, rigors, arthralgia, myalgia, frontal headache, faintishness, lower back pain, loss of appetite, vomiting, loose stool 3–4 times/day for two days. Patient denied a significant exposure to leptospirosis. There was no contact history of fever. He was a manual worker. On admission he was ill looking, febrile (temp-104 F), dehydrated, mildly dyspnoec (RR-24 breaths/min) with SpO2 98% on air and had conjunctival suffusion. His pulse rate was 124 bpm with blood pressure 80/50 mmHg. Other system examination was unremarkable.
Ultrasound scan of abdomen showed acute renal parenchymal changes and there was no evidence of free fluid in the abdomen. Initial investigations revealed neutrophil leukocytosis with thrombocytopenia, high C-reactive protein (360 mg/L), high blood urea (172 mg/dL) and serum creatinine (355 micromol/L), marginally elevated liver transaminases (AST > ALT), microscopic hematuria, ECG showed sinus tachycardia with mild T inversions in V4-V6. Chest X ray was normal.
Possible diagnosis of leptospirosis was made on clinical grounds and he was started on intravenous cefotaxime. His blood pressure was improved after fluid resuscitation and he had good urine output. His 2D echocardiogram was normal, but his troponin titer increased and then came down. Patient was discharged from the ward on day 7 of illness with complete recovery and normal full blood count, renal and liver function tests. CRP and trop I titer was coming down. 2 D echocardiogram which was performed after three weeks of recovery was normal.
His dengue antigen test, blood and urine cultures were negative. The Leptospirosis qPCR test performed on day three of the illness was reported as not detected though one out of triplicate samples was positive. Patient was clinically diagnosed as a “possible” case of leptospirosis. |
pmc-6423826-3 | A 55 year old male laborer presented with fever for four days duration. He was previously diagnosed to have diabetes mellitus, but he was not taking treatments. Fever was associated with arthralgia, myalgia, headache, lower back pain, dysuria and reduced urine output for two days, cough for one week producing scanty amount of whitish sputum. He had a history of muddy contact within one week prior to symptom onset.
On admission, patient was febrile (temp-102F), ill looking, mildly dehydrated and had conjunctival suffusion. His pulse rate was 124 bpm with blood pressure of 90/50 mmHg. Other system examination was unremarkable. Initial laboratory work up showed neutrophilia with normal white cell count, thrombocytopenia, high C-reactive protein (250 mg/L), high serum creatinine (146 micromol/L) and normal liver transaminases. ECG showed sinus tachycardia and chest X ray-PA was normal. Depending on clinical grounds, diagnosis was made as leptospirosis and started on intravenous cefotaxime while fluid resuscitation is being carried out.
Despite adequate fluid resuscitation patient developed shock with low urine output on the same day of admission. (Day 4 of illness- Pulse rate- 130 bpm, BP-85/60) Then vasopressor support was given and small dose of frusemide infusion was started after achieving normal blood pressure with noradrenalin. 2D echocardiogram was performed on D5 of illness and it showed mild global hypokinesia with ejection fraction 45–50%, dilated left ventricle with concentric left ventricular hypertrophy and concluded as hypertensive heart disease with or without myocarditis. Cardiac troponin I titre became positive and had rising titre when repeated and then came down by the time of recovery. US scan of abdomen revealed bilateral renal parenchymal changes with normal sized kidneys. Noradrenalin was tailed off within 24 h and urine output was improved with maintenance fluid therapy. Patient had rising serum creatinine till day 6 of illness and then started to come down. Serum electrolytes were normal throughout and there was no acidosis. Patient was improved dramatically and was discharged from the hospital by day 9 of illness. On discharge he had rising platelet count, normal serum creatinine and dropping troponin I titre and CRP.
2 D echocardiogram was repeated after 4 weeks of discharge and his ejection fraction was improved to 60% and there was mild left ventricular hypertrophy with grade I diastolic dysfunction. His diabetes was controlled with soluble insulin during acute illness and changed to oral hypoglycemic treatment with the recovery. Leptospirosis antibody titre (MAT) done on day 7 of illness was positive (1:5120). |
pmc-6423826-4 | A 73 years old male patient presented with fever for 4 days. It was high fever associated with arthralgia, myalgia and mild difficulty in breathing. He also complained of reduced urine output and loose stool (two episodes) for one day. There were no other respiratory or urinary symptoms. He denied a significant exposure to leptospirosis. He had a past history of hypertension for which he was not taking treatment and past history of renal calculi for which he has undergone surgery several years back.
On admission he was ill looking, febrile (Temp-102 F), and anicteric. Pulse rate was 112 bpm and blood pressure 96/66 mmHg. Other system examination was unremarkable. Initial investigations revealed marked thrombocytopenia, neutrophilia with low normal white blood cell count, high C-reactive protein (236 mg/L), high serum creatinine (267 micromol/L), elevated liver transaminases (AST > ALT), urine analysis showed pus cells 65–70, red cells 15–20 and albumin 2+ (urine culture became negative). Chest X Ray-PA was normal.
Possibility of dengue fever could not be excluded with his full blood count and clinical presentation, but all other initial investigations were supportive towards leptospirosis although there was no history of significant exposure to leptospirosis. On admission ultrasound scan of the abdomen was performed inward and there was no evidence of fluid leakage. Therefore, patient was started on intravenous cefotaxime in addition to hydration with maintenance fluid. Patient had low urine output and went in to shock (PR-114, BP-78/41 mmHg) despite of adequate fluid resuscitation (on day 4 of illness). He was started on IV Noradrenalin to maintain blood pressure.
Ultrasound scan of the abdomen revealed right side scarred kidney with left side renal parenchymal changes with normal size kidney. There was no evidence of leaking by the time of developing shock. 2D echocardiogram showed severe mitral regurgitation with and there was no evidence of myocarditis. Troponin I titer became marginally positive and later came down. ECG showed sinus tachycardia. Histological diagnosis or cardiac MRI to diagnose cardiac involvement was not accessible due to lack of resources in the hospital. Noradrenalin could be tailed off within 24 h. (On day 5 of illness). By day five of illness urine output was gradually improved but serum creatinine remained rising with normal serum electrolytes. Dengue NS1 antigen was negative, but IgM and IgG antibodies were positive with dropping platelet count and white cell count (neutrophilia persisted).
Dengue pre-critical monitoring was continued while giving maintenance fluid therapy. Daily ultrasound scans were performed to exclude fluid leakage. Patient remained hemodynamically stable and platelet and white cell count started to increase by day 7 of illness and serum creatinine started to come down by day 10 of illness. He was discharged from the hospital on day 11 of illness with a plan to be followed up in nephrology clinic for possible chronic kidney disease. 2D echocardiogram was repeated after three weeks of recovery and it was normal other than trivial mitral regurgitation. Leptospirosis antibody titer done on day 7 of illness was positive. (1:2560). |
pmc-6423866-1 | A previously healthy 62-year-old woman living in a rural area developed fever, headache, and fatigue starting on July 27, 2018. She also had gastrointestinal symptoms, such as anorexia, nausea, and diarrhea later, and visited a local clinic on July 30. She was prescribed antipyretic analgesics and returned home. The following day, she developed a mild consciousness disorder and visited a local clinic again accompanied by her family. She underwent a magnetic resonance imaging examination, but no abnormality was noted. However, laboratory data showed leukocytopenia, thrombocytopenia, and increased aspartate aminotransferase (AST) and creatine kinase (CK) levels. She was transported to Kagoshima University Hospital.
On admission, she had a slight disturbance of consciousness [Glasgow Coma Scale of 14: E3, V5, M6], a body temperature of 37.4 °C, a respiratory rate of 22/min, blood pressure of 109/73 mmHg, heart rate of 97/min, SpO2 of 99% (room air), normal heart and respiratory sound, no swelling of the superficial lymph nodes, crusty bite wounds in the left inguinal region, although there was no tick. Laboratory testing showed leukocytopenia (total white blood cell count of 910 cells/mm3); thrombocytopenia (platelet count of 63,000 cells/mm3); mildly elevated AST (68 U/L), lactate dehydrogenase (364 IU/L), and CK (317 U/L) levels; elevated ferritin level (1947 ng/mL); and normal CK-MB fraction (CK-MB) (4 U/L) and C-reactive protein (CRP) level (< 0.02 mg/dL). A urinary general examination showed protein (4+) and occult blood (3+). Electrocardiography (ECG) and chest radiography findings were normal on the day of admission (day 1).
Because of these clinical findings, she was suspected of having SFTS and transferred to the intensive care unit. Several hours after admission, SFTSV RNA was detected in her serum samples by RT-PCR, so she was definitively diagnosed as having SFTS.
Her clinical course is shown in Fig. . Her blood pressure gradually started to decrease from day 2 evening. Fluid resuscitation (100 ml/h for 10 h; crystalloids 500 ml, colloids 500 ml) was performed until day 3 morning because hand-held ultrasound on day 2 midnight showed collapsed IVC (inferior vena cava). Then, repeat hand-held ultrasound showed recover from collapsed IVC; nevertheless, her blood pressure had not recovered and administration of low doses of dopamine and norepinephrine was started. To exclude cardiogenic shock, trans-thoracic echocardiography was performed and showed diffuse left ventricular (LV) wall motion depression (with an EF: ejection fraction of 34.4%), diffuse LV wall thickening, and small amount of pericardial effusion. Further, ECG showed ST elevation in the II, III, aVf, and V2–6 leads and a low-voltage complex in all leads (Fig. ), and the level of troponin I was elevated (120.9 ng/mL), but that of CK-MB was not. She did not have any chest symptoms. She met several diagnostic criteria for clinically suspected myocarditis [], which include unexplained cardiogenic shock, elevated level of troponin I, abnormal ECG and echocardiographic findings. In addition, she also met criteria for fulminant myocarditis [], which include acute illness (history of < 2–4 weeks since the onset of symptoms), hemodynamic instability due to cardiogenic shock, and need for hemodynamic support (inotrope). Therefore, she was suspected of having fulminant myocarditis caused by SFTSV. However, we did not perform coronary angiography (CAG) or endomyocardial biopsy because thrombocytopenia and leukocytopenia greatly increased the risk of an invasive procedure. We screened for other viruses, including enterovirus, adenovirus, and influenza viruses that may cause myocarditis, but none of them were detected in nasal swab samples.
On day 5 of hospitalization, repeat echocardiography showed further LV dysfunction (with an EF of 27.9%), diffuse LV wall thickening (septum/posterior wall: 14.7 mm/13.5 mm), and narrowing of the left ventricular cavity (Fig. ). On the basis of these results, administration of low doses of dobutamine and milrinone was added. On day 6 of hospitalization, repeat echocardiography showed cardiac tamponade findings of the dilated IVC without respiratory variations and right ventricular diastolic collapse (Fig. ). However, no surgical pericardial puncture was performed, because there was no safe puncture space and a circulation agonist was able to maintain blood pressure. After that, echocardiography showed steady improvement of LV function (EF) and wall thickness, the urine output also continued to increase and the water balance gradually leaned toward negative as a result (Fig. ). Additionally, the pericardial effusion gradually decreased and the cardiac tamponade findings naturally disappeared. On day 11 of hospitalization, her mild disturbance of consciousness from admission had improved, and she no longer needed a circulatory agonist. On day 13 of hospitalization, ECG showed improvement of ST elevation in the II, III, aVf, and V2–6 leads. She was eventually transferred to a general ward on the same day. On day 17 of hospitalization, repeat echocardiography showed normal LV function (with an EF of 70.3%) and wall thickness (septum/posterior wall: 8.9 mm/9.4 mm) (Fig. ).
On day 20 of hospitalization, laboratory testing and ECG showed no abnormal findings. After exercise rehabilitation, she was discharged home on day 28 of hospitalization.
She had no history of cardiac diseases, allergy, or use of drugs that may cause myocarditis. During 28 days of hospitalization, CRP and bacterial culture were always negative, and there was no evidence of eosinophil elevation. Viruses, other than SFTSV, were not detected by virus isolation test. Based on these results, she was clinically diagnosed as having fulminant myocarditis caused by SFTSV. |
pmc-6423868-1 | A 38-year-old Chinese female was first admitted to our hospital in December 2011 due to a complaint of constipation for 1 month. Her diet and lifestyle were normal. She had no history of serious illness or family genetic diseases. During the physical examination, no abnormalities were identified. The peripheral blood counts revealed a white cell count of 1.3 × 109/L, a hemoglobin level of 93 g/L, and a platelet count of 94 × 109/L. The blood chemistry findings showed normal lactate dehydrogenase, C-reactive protein, and albumin levels. Her bone marrow was hypercellular, exhibited infiltration and included 91.5% blast cells comprising primitive monocytes and naive monocytes. The immunophenotype analysis showed that 54% of the cells were abnormal, and positive labeling for CD34, CD10, and CD71 and negative labeling for CD19 were observed. The overall findings were consistent with acute monocytic leukemia. G-banding revealed 45, XX, − 2, der(11)(p15) []/46,XX[16]/92,XXXX []. The genetic tests, including screens for FLT3, IDH1/2 and tp53 mutants, were all negative. The patient was diagnosed with high-risk acute monocytic leukemia. The patient did not respond to idarubicin and cytarabine (IA) or subsequent aclacinomycin, cytarabine, and etoposide (AAE). Then, the patient achieved CR following one additional AAE regimen as previously described. Furthermore, she received aclacinomycin and cytarabine (AA) twice, mitoxantrone and cytarabine (MA) once, and intermediate-dose cytarabine once as consolidation chemotherapy. Immediately thereafter, the patient underwent sibling HSCT from her HLA-identical sister in October 2012. The patient achieved continued CR but still exhibited microresidual disease of 0.01–0.05% in the following 4 years. Additionally, she did not suffer from acute or chronic graft-versus-host diseases after her first transplantation.
The patient was admitted to our hospital again with complaints of fever and cough in April 2017. The peripheral blood counts revealed a white cell count of 4.8 × 109/L, a hemoglobin level of 117 g/L, a platelet count of 170 × 109/L and 10% abnormal cells. Her bone marrow was hypercellular, exhibited infiltration and included 51% blast cells comprising primitive monocytes and naive monocytes. The level of donor chimerism in her bone marrow was 47.9%. The genetic tests, including screens for FLT3, IDH1/2 and tp53 mutations, were all negative. Subsequently, the patient did not respond to mitoxantrone, cytarabine and etoposide (MAE) or donor lymphocyte infusion. Because the patient was refractory and exhibited AML relapse, subsequent D-CLAG chemotherapy (decitabine, 25 mg d1–5; cladribine, 5 mg/m2 d6–10; cytarabine, 2 g/m2 d6–10; and granulocyte-stimulating factor, 150 μg twice daily from d4 until the neutrophils exceeded 0.5*109/L) was administered (Fig. ). A bone marrow test performed 3 weeks after the D-CLAG regimen showed CR. Moreover, the neutrophils and platelets recovered quickly (Fig. a, b). Additionally, the patient did not suffer from any severe complications after chemotherapy. Subsequently, the patient was given one more D-CLAG and donor lymphocyte infusion. A bone marrow test performed 1 month after the second D-CLAG showed CR. During her follow up, she underwent haploidentical HSCT from her daughter in August 2017. The conditioning regimen consisted of cytarabine, busulfan, cyclophosphamide, methyl-N-(2-chloroethyl)-N-cyclohexyl-N-nitrosourea, and anti-thymocyte globulin. Methotrexate, cyclosporin A and mycophenolate mofetil were used for graft-versus-host disease prophylaxis. The numbers of mononuclear cells and CD34+ cells were 15.07 × 10/kg and 5.68 × 10/kg, respectively. The neutrophil and platelet engraftments were achieved on day 17 and day 29, respectively (Fig. c, d). A short tandem repeat analysis showed complete donor-type engraftment. She suffered gastrointestinal bleeding on day 20 but quickly recovered with supportive treatment. Epstein-Barr virus infection was observed 2 months after transplantation with a maximum Epstein-Barr virus DNA load of 3 × 10 copies/ml. The patient did not develop a posttransplantation lymphoproliferative disorder, and the Epstein-Barr virus DNA load decreased below the normal level with intravenous rituximab. Following haploidentical HSCT, her marrow showed continuous CR, and the microresidual disease remained below 0.01%. Currently, one year after transplantation, the patient continues to be in good general condition. |
pmc-6423896-1 | A 38-year-old man presented with deterioration of memory, which had begun 1 month prior and was accompanied by impaired extension of the right upper limb. The patient had suffered from intermittent right frontal headache after catching a cold 10 days prior, during which hypomnesia was especially pronounced. His history included cholangiolithiasis, pancreatitis, and nasosinusitis. A metal biliary endoprosthesis had been placed endoscopically 8 months prior. The patient's family history was unremarkable. He was lucid but displayed poor comprehension, slow reaction time, decreased computational capabilities, and amnestic aphasia. Neurological examinations demonstrated no abnormalities except for impaired extension of the right upper extremity. Bilateral exophthalmos and cervical lymph node enlargement were found during the physical examination. The left upper eyelid was touching an active mass.
Laboratory tests for variables including tumor markers, relative levels of rheumatologically relevant antibodies, thyroid hormones, and routine blood parameters were all normal except for a rise in the erythrocyte sedimentation rate (ESR) to 46 mm/h and an elevated eosinophil percentage at 11.4%. Parasite infection was considered the primary diagnosis. Doppler ultrasound showed grade II enlargement of the cervical lymph nodes. Initial MRI and diffusion-weighted imaging (DWI) showed temporal and occipital lobe inflammation and colloid cysts in the right lateral ventricular trigone (). Ocular MRI revealed increased volume of the bilateral tear glands, sinusitis in the entire group of paranasal sinuses and mastoiditis. Lumbar puncture indicated that the intracranial pressure was 170 mm H2O. The cerebrospinal fluid was sent out to be examined for cerebrospinal fluid-related viruses, rheumatic immune-related antibody, cerebrospinal fluid biochemistry, and autoimmune encephalitis antibodies. However, none of these tests were positive.
Four days after admission, the patient began to cough. A CT scan showed bronchitis as well as inflammation of the left apex pulmonis and pleura. Two days later, extension of the right fingers suddenly became impaired. MR spectroscopy (MRS) demonstrated an obvious rise in choline (Cho) and a decrease in N-acetyl aspartate (NAA) in the left lateral ventricular trigone, indicating the possibility of a tumor lesion (). We began to administer 120 mg methylprednisolone q.d. as an experimental therapy on day 9 of hospitalization, although the nature of the inflammation was unknown. On day 14 of hospitalization, the patient underwent microsurgery. Routine blood reexamination on the second day after the operation showed that the percentage of eosinophils had returned to normal. Except for a mild increase to 37.6°C 3 days after surgery, body temperature was normal. The patient quickly recovered, and his memory and right upper limb movement returned to normal 10 days after surgery.
The patient developed swelling of the right lower extremity 2 months after neurosurgery and was admitted to the hospital again in May 2018. Doppler ultrasound indicated thrombosis in the bilateral popliteal veins, right superficial femoral vein, anterior tibial vein, posterior tibial vein, and fibular vein. The percentage of eosinophils in peripheral blood had increased again to 14.8%. MRI reexamination showed stable enhancement of the right lateral ventricular trigone and normal postoperative changes in the left temporal-occipital lobe. The patient underwent percutaneous transluminal balloon dilatation and was discharged 5 days later.
Three months later, the patient came to the hospital for follow up. The disease seemed to have progressed this time. MRI showed a more pronounced degree of enhancement and a wider range of edema than before. A CT scan indicated double pneumonia and multiple enlarged lymph nodes in the mediastinum and under the axilla. The patient was then diagnosed with EGPA. He then received 1 g methylprednisolone p.o., q.d. for 3 days, and 60 mg prednisone q.d. was administered for 14 days, followed by 45 mg q.d. thereafter. Beginning 2 weeks after discharge from the hospital, 125 mg cyclophosphamide per day was taken orally. One month later, the symptoms had been alleviated, and MRI, CT, and Doppler ultrasound showed an excellent response to the treatment.
A subtotal resection of the right lateral ventricular trigone lesion was achieved via the subtemporal approach. The lesion was yellow-brown and unevenly textured and grew infiltratively in the shape of a cuff around the temporal-occipital artery. We performed piecemeal resection in the gliosis zone. Subsequently, the left temporo-occipital region was exposed. Multiple abnormal lesions around the Labbe vein showed infiltrative growth and abundant blood supply with soft material. Considering the presence of the lesion around the angular gyrus, subtotal resection was performed with careful manipulation.
Hematoxylin and eosin staining of tumor specimens revealed inflammatory cell infiltration dominated by histiocyte and perivascular lymphoid sheath formation in the left temporo-occipital region. Microscopically, glial hyperplasia was observed in tissue from the right lateral ventricle trigone. Immunohistochemistry showed positivity for CD68, glial fibrillary acidic protein (GFAP), S-100, and synaptophysin (Syn) as well as focal positivity for Oligo-2. No reactivity was observed with antibodies against myelin basic protein (MBP), neurofilament (NF) or isocitrate dehydrogenase-1 (IDH-1). The MIB-1(ki-67) proliferation was approximately 10–20% ().
Genomic DNA was extracted from the patient's peripheral blood using the QIAamp DNA Mini Kit (QIAGEN). Reads were mapped to the human reference genome assembly GRCh37 using BWA-MEM in the BWA package (version 0.7.17) with the default parameters. Human leukocyte antigen (HLA) genotyping was performed from the sequencing reads of the patient. We used three different genotyping algorithms, OptiType (), BWAkit () and xHLA (). Single nucleotide variants and indels were identified by the Freebayes tool (version 1.1.0) (). The SNPs in this patient were compared with those reported in the previous literature () (). |
pmc-6424055-1 | A 92-year-old neglected female patient, living on her own, presented to our Department with a large ulcerated nodule on the left supraclavicular region that had been present for 10 months, growing progressively and consistently.
Physical examination showed a patient in poor general conditions with a 11 × 8 cm, ulcerated, cauliflower-like, with polilobulated margins, nodule in her left supraclavicular region. The lesion appeared to invade the clavicle bone and cervical lymphnodes were not palpable ().
A punch biopsy was performed in order to assess the type of cutaneous malignancy, but unfortunately came back not diriment because of the large amount of necrotic tissue with rare isolated keratin pearl.
Then we decided to perform a surgical excision with two centimeters margins en-bloc with the periosteum of the clavicle, to which the lesion appeared to be adherent, under local anesthesia and sedation ().
The loss of tissue substance was then reconstructed by direct closure performing a wide undermining of the wound margins in a subfascial plane, under the fascia of the pectoralis major muscle anteriorly and of the trapezius muscle posteriorly.
The choice of including a fascia in the direct closure of the wound has been made in order to give a better coverage to the clavicle bone, whose periosteum was previously removed ().
A drain tube was positioned and the surgical wound was closed by direct suture ().
The postoperative period was uneventfully, the drain tube was removed after 4 days and stitches was removed in two weeks.
The histopathological examination revealed large tumor cells with abundant eosinophilic cytoplasm and nuclei with finely dispersed chromatin and prominent nucleoli. Immunohistochemistry revealed positive stain for CK AE1-AE3 and CDX2, highlighting an epithelial differentiation and likely origin from large intestine. The tumor cells showed negative stain for TTF-1, CK7, Mammoglobin, P63, neuroendocrin markers (CD56, sinaptofisina) and S100 (, ). Final diagnosis was a cutaneous metastasis from an occult adenocarcinoma of the colon. All the resection margins were clear.
Two weeks after the surgery, a colonoscopy was scheduled and a large vegetative mass of the descending colon was found. A punch biopsy was performed that came back positive for moderately differentiated adenocarcinoma composed by cells with abundant cytoplasm, nuclei with dispersed chromatin and prominent nucleoli () positive stain CK AE1-AE3 and CDX2 ().
We then programmed a PET scan one month after surgery to assess the presence of the primary tumor, but unfortunately the patient died due to a myocardial infarction. |
pmc-6424056-1 | We present the case of a 70 year old male who had an elective right inguinal hernia repair. He reported a longstanding history of a right inguinal lump which had been causing increasing discomfort over the previous 12 months. His past medical history included an emergency laparoscopic cholecystectomy 5 years prior, as well as atrial fibrillation. On examination, the patient had a mildly tender right inguinoscrotal hernia. Despite being tender, the hernia was reducible and there were no overlying skin changes. Abdominal and testicular examinations were otherwise unremarkable.
Open right inguinal hernia repair was performed using a modified Kugel technique. Intraoperative findings validated clinical examination and a large indirect hernia was reduced. Upon reduction, the hernia sac was found to have multiple 5 mm foreign bodies embedded into the wall. On closer inspection these foreign bodies were macroscopically consistent with gallstones (see , ). The hernia sac and foreign bodies were sent to the pathologist who confirmed the foreign bodies to be cholesterol gallstones.
The immediate post-operative recovery was uneventful and the patient was discharged home the following day. He was reviewed four weeks later in the outpatient surgical clinic where he reported a good recovery. |
pmc-6424059-1 | A 65-year-old Russian male, not known to have chronic medical illnesses, came to the ED complaining of painful swelling in the lower abdomen which had been going on for five days. Abdominal pain was severe colicky in nature with no relieving factors, associated with nausea and vomiting multiple times. There had been no change in bowel habits, fever or change in appetite. The patient had a history of lower abdominal surgery at the age of two, but he had no medical report
On physical examination the patient was conscious and had a normal body built. His blood pressure was 126/92, pulse was 88 and temperature was 36.2 °C. is symmetrically distended with a swelling in the lower abdomen 12 × 15 cm in size with negative cough impulse, erythema and tenderness on the overlying skin. The rest of the abdomen was soft on palpation with positive bowel sounds. Investigation of his hemoglobin gave 10.8 wbc’s with 11.5 sodium 139 potassium 3.2 creatinine 0.7.
The patient was admitted as a case of abdominal pain for investigation. The CT of abdomen and pelvic with IV and oral contrast was done showing thickened terminal ileum with marked luminal narrowing which appeared adherent to the urinary bladder wall with no line of cleavage. Two fistula tracts were seen superior and inferior; the superior one lead to a pocket of collection filled by contrast 36 × 20 mm in size. The inferior tract was connected to an anterior abdominal wall collection measuring about 18.7 × 14.4 mm with marginal enhancement denoting an abscess. There was diffuse anterior abdominal wall fat stranded with subcutaneous pockets of air denoting infection. Subcentemetric mesenteric lymphadenopathy was observed ().
Patient was taken to the OR for exploratory laparotomy and drainage of the abscess. Upon internce to the abdomen a large pocket of pus in subcutaneous layer was opened and evacuated and a swab was sent for culture and sensitivity. A firm mass inclosing the pelvic was dissected and found to be a large diverticulum 10 cm from the ileocecal junction. The mass was attaching to the urinary bladder and was fistulating to the subcutaneous pus collection. Urology was called in at this point and the urinary bladder was checked by injecting methylene blue dye; there was no leak. Limited right hemicolectomy was performed with a primary iliocolic anastomosis ().
Histopathology was consistent with diverticulum of the small bowel and serosal lipoma with a pocket containing multiple staghorn-type black stones, negative to tuberculosis (). Patient wound culture from OR showed E. coli which was sensitive to Tigacyclin. Treatment was started with this antibiotic and patient’s condition improved. Postoperative course was uneventful except for a small dehiscence at the lower part of the abdominal wound, which was treated conservatively with VAC dressing. Patient was discharged to travel to his country, and the wound was left for secondary closing. |
pmc-6424340-1 | A 23-year-old male presented with lumbar back pain radiating to the lower
extremities and worsening right foot weakness that began four days before. Patient
also reported unintentional fifteen-pound weight loss over the past four months.
Past medical history included treatment for sexually transmitted infections
(syphilis and chlamydia) and three years prior, and episode of Guillan-Barre
syndrome (GBS), presumed to be secondary to Lyme disease from which he had recovered
completely. Social history was significant for high-risk sexual behavior and a
long-term HIV-positive partner with undetectable viral load. Neurological exam
revealed normal tone throughout but reduced strength (3/5) in the right lower
extremity with reduced sensation distal to the right patellofemoral joint. The right
patellar reflex was absent with 2+ reflexes elsewhere. The rest of the clinical
examination was normal.
On examination, temperature was 36.7C, pulse of 43 beats per minute, blood
pressure 112/73 mmHg, respiratory rate 17 breaths per minute, and oxygen saturation
was 100% on ambient air. Initial blood tests showed: white blood cell count
3.1×109 and platelet count 147,000. CSF examination was
unremarkable: no mononuclear cells or polymorphonuclear leukocytes, protein level of
32 mg/dL, and a glucose level of 53 mg/dL. Further blood tests revealed negative
immunoglobulins M/G for Lyme disease, negative HIV antibody testing, positive IgG
for Epstein Barr Virus, and positive treponemal serology with a rapid plasma reagin
(RPR) of 1:8. An MRI of the lumbar spine was remarkable for abnormal enhancement of
the cauda equina roots (). MRI of the
head, cardiac MRI, and transthoracic echocardiogram were all within normal limits.
Cardiology was consulted for bradycardia and recommended outpatient follow-up in the
background of an otherwise normal EKG with bradycardia and normal ejection fraction
on echocardiogram.
The suspected diagnosis was neurosyphilis and CSF treponemal antibodies were
ordered. The patient received intravenous penicillin G with frequent neurological
examinations however, the right lower extremity sensory deficit did not show any
improvement. Electromyogram revealed an early demyelinating predominantly motor
polyneuropathy. Patient had been successfully treated for syphilis two years ago
with RPR titers that had reduced from 1:128 to 1:2. CSF studies including polymerase
chain reaction analyses, and antibody testing were negative for Lyme disease,
syphilis, herpes simplex virus, herpes zoster virus, Epstein-bar virus, and
cytomegalovirus.
Screening for other blood-borne viruses, opportunistic infections, and MRI of
the brain were all negative. In view of the established risk factors and new
findings, a repeat HIV test was done on hospital day 4 and resulted positive. The
HIV viral load was >500,000 copies/ml and the CD4 count was 574
cells/mm3. There was a high clinical suspicion for inflammatory
demyelinating polyneuropathy secondary to an acute HIV infection and patient was
started on highly active antiretroviral therapy (HAART) along with intravenous
immunoglobulin (IVIG). His strength improved (4/5) with therapy and physical therapy
recommended acute rehabilitation. On hospital day 12, patient was discharged with
close follow-up. |
pmc-6424473-1 | A 50-year-old male presented to the emergency room with abdominal pain. An abdominopelvic computed tomography (CT) scan showed a perforated appendicitis with a contained abscess. Laparoscopic exploration revealed a neoplastic appendiceal lesion with peri-appendicular and pelvic mucin as seen in PMP. A laparoscopic appendectomy was performed.
Pathological examination revealed the coexistence of mucinous and neuroendocrine appendicular tumours (Figures -). The former corresponded to a low-grade mucinous adenocarcinoma which had developed from a low-grade appendiceal mucinous neoplasm (LAMN). The tumor was 5.5 cm long and occupied the entire appendix. A perforation site was identified as well as neoplastic cells in the lumen of the resection margin. The second tumour was a well-differentiated NET measuring 1.6 cm, with infiltration of the muscularis propria and minimal infiltration of the mesoappendix. The proliferation index as evaluated by the immunohistochemical marker MIB-1 was approximately 3%, corresponding to a histologic grade of G2/3. Perineural invasion, but no vascular invasion, was visualized. The resection margin was negative for NET. No lymph nodes were identified in the appendectomy specimen.
A complete workup, including thoracic and abdominopelvic CT scans, total colonoscopy, and evaluation of serum markers (carcinoembryonic antigen and chromogranin A) was normal. As the prognosis was considered more likely linked to the PMP rather than the NET component, right hemicolectomy and cytoreductive surgery (CRS) with hyperthermic intraperitoneal chemotherapy (HIPEC) were performed five months after the appendectomy.
At laparotomy, there were mucinous lesions on the peritoneal surfaces of the right hemidiaphragm, right abdominal wall, greater omentum, right colon, and pelvis. The peritoneal carcinomatosis index (PCI) was 22. Right hemicolectomy was performed as well as total omentectomy, cholecystectomy, and peritoneal stripping of the right hemidiaphragm, abdominal wall and pelvis were performed. A completeness of cytoreduction score of 0 – with no residual peritoneal seeding – was achieved. HIPEC with oxaliplatin (460 mg/m2) for 30 minutes at 42°C was then administered.
Histopathologic examination revealed the presence of acellular mucin in the ligamentum teres hepatis, the peritoneal surface of the right colon, greater omentum, and Douglas' pouch. A suspicious right colic artery lymph node was analyzed intraoperatively and was found to be covered by acellular mucin. The right diaphragmatic peritoneal surface displayed mucinous epithelial cells with high-grade dysplasia. The right hemicolectomy resection margins and 17 lymph nodes were cancer-free. The patient recovered uneventfully after the surgery and remains cancer-free after 20 months of follow-up. |
pmc-6424540-1 | A 63-year-old male, with a past medical history of type II diabetes mellitus, hypertension, chronic kidney disease stage G3a, and heart failure with preserved ejection fraction, presented with generalized lethargy and weakness. His history, obtained from his family, revealed that he was taking his home medications: metformin 1000 mg twice daily, lisinopril 10 mg once daily, and furosemide 40 mg once daily but not eating or drinking adequately at home due to a lack of money. On physical examination, he was found to be confused and in a state of lethargy. He had a dry oral mucosa and scored 14/15 on the Glasgow Coma Scale. The vitals were a blood pressure of 70/40 mmHg, pulse of 70 beats/minute, a temperature of 92 degrees Fahrenheit, and a respiratory rate of 14/minute. Tables - list the major laboratory and hematology findings.
His urine toxicology screen was negative and blood alcohol level was undetectable. The computed tomography (CT) scan of the head and the posterior-anterior (PA) / lateral chest X-ray were unremarkable.
The patient was admitted to the intensive care unit for a hypovolemic shock, with the resultant acute chronic renal failure secondary to hypotension worsened by the ongoing use of furosemide and lisinopril. He was treated initially with intravenous fluids and norepinephrine for blood pressure support. It was postulated that the elevated lactic acid level and anion gap level were due to hypoperfusion, likely exacerbated by the concomitant use of metformin in the setting of acute kidney injury. His altered mentation was likely due to metabolic encephalopathy secondary to high lactate.
The patient received broad-spectrum intravenous antibiotics on the day of admission, but they were discontinued later, as no source of infection was found and sepsis was ruled out. The patient also received a bicarbonate drip until the bicarbonate improved to 22 mmol/L. He subsequently underwent emergent hemodialysis. Post hemodialysis, lactic acid trended down to 8.3 mmol/L and pH improved to 7.24 with an anion gap of 35 with the first session; subsequently, lactate became undetectable, as he underwent further sessions of hemodialysis during the hospital course. His creatinine at the time of discharge was 3.12 mg/dl, bicarbonate was 31 mmol/L, the anion gap had closed, and pH was 7.34. His urine output normalized and mentation improved to GCS 15/15. He was discharged in a stable condition. His metformin, lisinopril, and furosemide were stopped, and he was advised to keep himself adequately hydrated. Outpatient creatinine was 1.4 mg/dL. |
pmc-6424541-1 | A 50-year-old Hispanic lady presented with an eight-month history of dull, aching generalized abdominal pain that worsened after meals, and was associated with nausea and reduced appetite. Her stool had become softer than before, but there was no melena, and no hematochezia. She had episodes of severe abdominal pain that disrupted her sleep every few days. Review of systems revealed no weight loss. Her past medical history was significant for hypertension, and past surgical history revealed the following four surgeries within the last 10 years: laparoscopic cholecystectomy, laparoscopic appendectomy, umbilical hernia repair, and tubal ligation. She had been a lifelong nonsmoker and had never used alcohol. Her family history revealed breast cancer in her mother and prostate cancer in her father. Her BMI was 38. Physical examination was unremarkable. She underwent an upper gastrointestinal endoscopy that showed a nonobstructing, nonbleeding cratered ulcer in the posterior wall of the gastric body. Biopsies were taken that revealed moderate-poorly differentiated adenocarcinoma. She underwent positron-emission tomography (PET) scanning that showed increased uptake in two regions: one in the medial gastric fundus (maximum SUV=14.2) and a second one near the cecum (maximum SUV=18.6). Further the PET scan showed abnormal circumferential thickening and pericolonic inflammatory changes involving the cecum, and numerous small lymph nodes were noted in the right lower quadrant (largest lymph node=12 mm × 18 mm). One week later, she underwent a colonoscopy that showed a fungating, partially obstructing, nonbleeding, circumferential mass in the cecum. Biopsy showed a moderately differentiated adenocarcinoma. The blood tests showed anemia (hemoglobin= 9.3) while all other tests were unremarkable. Carcinoembryonic antigen (CEA) was 0.819 preoperatively. Based on the above investigations it was decided that the patient has synchronous gastric and colon cancer and that it is surgically resectable. A week later, she underwent an exploratory laparotomy, subtotal gastrectomy with Roux-en-Y reconstruction and right colectomy with resection of terminal ileum and ileocolostomy. The description of the procedure is as follows: in the supine position on the OR table after being appropriately identified, induction of anesthesia, endotracheal tube placement, area of the abdomen prepped, and draped in the standard sterile fashion. A midline laparotomy incision was made from the xiphoid down to the umbilicus. That incision was carried down from the skin and subcutaneous tissue until the abdomen was entered. An Alexis wound retractor was placed for better exposure. Internal organs were grossly unremarkable. Liver was inspected and noted to be free of any metastatic lesions. There were two malignant appearing tumors, one in the body of the stomach and another one in the cecum. Both tumors were easily identified by palpation although both were tattooed. We commenced the operation with the gastric portion. We created an opening to the lesser sac and transected the branches of the gastroduodenal artery along the greater curvature of the stomach. Then we excised the branches of the right gastric artery supplying the lesser curvature. The tumor itself was located in the body of the stomach that would save the junction between the proximal third and the middle third of the stomach. So we fired a thoracoabdominal (TA-90) stapler with a few centimeters margin into the proximal stomach. Then we proceeded by firing a gastrointestinal anastomosis (GIA-75) stapler along the pylorus, transecting approximately two-thirds of the stomach. The specimen was opened, and we decided to perform a Roux-en-Y reconstruction. We went a couple of feet from the ligament of Treitz and a GIA stapler was used to separate the jejunum. The jejunum was brought retrocolic all the way up into the proximal stomach. To create an anastomosis we chose an end-to-end anastomosis (EEA-25) stapler. The anvil was placed inside the proximal stomach creating an enterotomy that was approximated using another fire on the GIA stapler. Then we proceeded by making an opening in the small bowl and through that opening the EEA-25 stapler was then carefully introduced and it was opened. It was then connected to the anvil previously placed in the proximal stomach under direct visualization; the stapler was approximated and fired. The stapler was removed showing two donuts indicating a patent anastomosis. The opening where the stapler entered the small bowel was sealed using an Echelon stapler. Then we proceeded by curetting the anastomosis between the two limbs of the jejunum. It was a side-to-side anastomosis using GIA and TA. Anastomosis reinforced using 3-0 Vicryl sutures and noted to be widely patent with good coloration and tension free. Anesthesia proceeded by advancing a nasogastric tube all the way down to the anastomosis. Evicell was sprayed in the area to ensure complete hemostasis.
We then concentrated our attention on the right lower quadrant where the colon was visualized. The tumor was in the right colon near the cecum. We started mobilizing the colon medial to the white line of Toldt ensuring hemostasis. The proximal transverse colon was also mobilized by transecting the hepatocolic ligaments. We proceeded then by firing a GIA in the terminal ileum; another GIA was fired at the proximal transverse colon. Then using the Echelon stapler, series of fires were used to transect the right mesocolon. We then proceeded by anastomosing the terminal ileum and the transverse colon. It was side-to-side functional end-to-end anastomosis using GIA and TA. Anastomosis reinforced using 3-0 Vicryl and it was noted to be widely patent with good coloration and tension free.
The abdomen was then irrigated with saline. The fluid was aspirated. No evidence of bleeding or injury to intra-abdominal organs was noted. The Alexis retractor was removed. The fascia was approximated using a running PDS, and the skin was approximated using staples followed by sterile dressing applied on top. The patient tolerated the procedure well without any complications. She was then transferred to recovery in stable condition. The counts were correct ×2.
Both surgical specimens were sent to pathology. The surgical margins of both masses and the 21 excised nodes were tumor free. The distal gastrectomy specimen showed poorly differentiated adenocarcinoma of the intestinal type (pT2aN0Mx) (Figure ).
The right colectomy specimen showed moderately differentiated adenocarcinoma that was invading the muscularis propria and extending into the subserosa (pT2aN0Mx) (Figures -).
Postoperatively the patient remained well (she was ambulating, tolerating oral diet, and pain was under control) and was discharged on the fourth postoperative day. She followed up in clinic up to two months after the surgery. She was about to begin chemotherapy. She missed her latest clinic appointment. |
pmc-6424542-1 | A 40-year-old woman with newly diagnosed AML undergoing induction chemotherapy developed blurry vision on admission day 16. Findings of bilateral papilledema and severe retroorbital headaches on day 18 prompted a computed tomography (CT) scan of the head which was unrevealing. Persistent symptoms prompted a repeat CT on day 27 which revealed findings concerning for DST (Figure ).
This was further shown by magnetic resonance venography (MRV) to involve the bilateral transverse sinuses (TSs), superior sagittal sinus (SSS) and straight sinus (Figure ). Systemic anticoagulation via continuous IV heparin drip was immediately initiated. Despite medical therapy, the patient's symptoms continued to worsen.
The patient was taken to the angiography suite on day 28, where extensive DST was confirmed by catheter venography (Figure ). Following venography, an alteplase drip was placed via infusion microcatheter (RenegadeTM Hi-FloTM, Boston Scientific, USA) in the dominant left TS and infused overnight. The following day, the patient returned to the angiography suite where repeat catheter venography revealed multiple, small, and irregular channels within the thrombus of the left TS. However, a very large clot burden was still noted, which precluded access of the SSS. The alteplase infusion microcatheter was again advanced into the large burden of clot within the left TS and infusion resumed throughout the day. Later the same day, repeat catheter venography revealed a recanalized left TS but with persistent outflow obstruction. The microcatheter was then successfully advanced into the SSS where it was left for overnight alteplase infusion at the same rate (Figure ).
On the morning of admission day 30, the patient again returned to the angiography suite where venography revealed persistent large clot burden within the same distribution and rethrombosis of the left TS. Mechanical thrombectomy was then performed using a 6 mm x 30 mm stent retriever (SolitaireTM, Medtronic, USA). A large volume of adherent clot was successfully removed from the left TS, however, a large clot burden remained. Final venography during this intervention revealed a partially recanalized left TS and partial underlying outflow obstruction thought due to either focal stenosis or a venous web at the junction with the left sigmoid sinus. Large volume clot persisted within the SSS, therefore the alteplase infusion microcatheter was left in the SSS for infusion throughout the day.
On the evening of admission day 30, repeat catheter venography revealed rethrombosis of the left TS and persistent large volume clot within the SSS. In addition, stenosis near the junction of the left TS and sigmoid sinus was noted (Figure ). The decision was made to perform suction thrombectomy utilizing a reperfusion catheter (ACE68TM Reperfusion Catheter, Penumbra, USA) with a suction pump (Pump MAXTM, Penumbra, USA). Suction thrombectomy of the SSS, left TS, and sigmoid sinus yielded a large volume of clot. The in situ stenosis persisted at the junction of the left transverse and sigmoid sinuses. Thus, angioplasty was performed utilizing a 5 mm x 20 mm balloon (ViatracTM 14 Plus, Abbott, USA) followed by successful deployment of an 8 mm x 40 mm self-expanding stent (Zilver®, Cook Medical, USA) with good venographic outcome (Figure ).
The patient experienced improvement in visual deficits and headache the next day, with resolution of these symptoms by day 32. She was converted from a heparin drip to a twice daily dose of enoxaparin 1 mg/kg and discharged home on day 36 with only mild blurry vision.
Eight days after discharge, the patient was readmitted with a lower extremity hematoma, likely a complication of systemic anticoagulation. Upon admission, she reported worsened blurry vision and intermittent headaches. These prompted reevaluation of the dural venous sinuses by CT venography. This revealed in-stent thrombosis of the left TS with ~75% stenosis and nonocclusive thrombus within the SSS along with the confluence of sinuses. She was then discharged home again, returning shortly thereafter as an outpatient for catheter venography. This demonstrated the progression of thrombosis to completely occlusive near the junction of the left TS and sigmoid sinus, along with markedly elevated pressure within the left TS of 53 mmHg (Figure ).
Thus, suction thrombectomy proved of limited benefit. Therefore, balloon angioplasty was performed and a second self-expanding stent was placed. This procedure demonstrated excellent venographic outcome and significant decrease in measured pressure within the left TS to 22 mmHg (Figure ). The headaches quickly resolved, although mild blurry vision persisted.
One month following the placement of the second stent, follow-up imaging confirmed patency of the dural sinuses and left TS stents. Twelve weeks after placement of the second stent, the patient remains headache-free with only mild blurry vision. |
pmc-6424548-1 | A 39-year-old female was brought to our emergency room by a private vehicle after being shot. An evaluation revealed a wound at the lower left back and at the mons pubis. Exploratory laparotomy and resection of the sigmoid colon, left ovary, and fallopian tube with a colostomy was performed. Postoperative deep venous thrombosis prophylaxis in the form of enoxaparin, 30 mg every 12 hours, was given and then changed to heparin, 5,000 units subcutaneously every eight hours, due to worsening renal function. The patient did well until postoperative day 4 when she complained of increased abdominal pain out of proportion to the clinical findings. Her symptoms worsened the following day. A computed tomography (CT) scan of the abdomen and pelvis was obtained, the results of which were consistent with postoperative ileus and raising concerns for right colon ischemia.
The patient was managed conservatively, and bedside drainage of the superficial wound infection was done on postoperative day 6 with improvement in abdominal pain. On the following day, a repeat CT of the abdomen and pelvis raised more concerns for bowel ischemia; however, the patient refused reexploration (Figure ).
A computed tomography angiogram (CTA) of the chest was obtained for worsening pulmonary symptoms on postoperative day 8 and was consistent with lower lobe pneumonia.
Feculent drainage around the stoma was noticed on postoperative day 9, and an exploratory laparotomy was performed with resection of multiple small bowel ischemic loops, cholecystectomy, and right hemicolectomy. The rest of the small bowel appeared dusky and friable. After multiple visits to the operating room, she was left with only a few inches of the proximal jejunum. A CTA of the abdomen was done on postoperative day 13 from her initial surgery and was consistent with a superior mesenteric artery occlusion (Figure ).
Admission platelet count was 281, down to 82 on postoperative day 11 when she tested positive for heparin-induced antibodies. The pre-test probability for HIT score was high (2 for thrombocytopenia, 1 for timing, 2 for thrombosis, 2 for no other cause = 7). The heparin was stopped and argatroban was used. The patient did survive her injury and was referred to a small bowel transplant center. |
pmc-6424549-1 | A 45-year-old female patient with a past medical history of hypertension and dyslipidemia presented with an 11-month history of numerous episodes of chest pain, palpitations, and dyspnea. These symptoms were associated with persistent edema of the hands, legs, and face. An ambulatory electrocardiogram and cardiac stress test were normal. The patient was treated for hypertension with enalapril 20 mg twice daily, hydrochlorothiazide 25 mg once daily and propranolol 20 mg twice daily. On physical examination, she had bilateral nonpainful mobile supraclavicular lymph nodes that were larger on the right side (1.5 cm), and a cushingoid habitus with abdominal striae, centripetal obesity, a full moon face, and a buffalo neck. Blood tests, including a complete blood count, electrolytes, albumin, renal function tests, and thyroid function test, were within the normal range. Laboratory evaluation for Cushing’s syndrome confirmed the diagnosis of an adrenocorticotropic-independent Cushing’s syndrome (Table ).
An abdominal computed tomography (CT) scan showed a 3.7-cm diameter right adrenal mass with clearly identifiable borders and soft tissue and fatty density consistent with an adrenal adenoma. The left adrenal gland was normal. The patient underwent a laparoscopic adrenalectomy without complications. A 3.0 x 2.5 x 2.5-cm mass was resected. Pathological examination confirmed an adrenal adenoma. The patient was discharged with prednisone temporary replacement.
Five months after the adrenalectomy, the patient developed generalized arthralgias, malaise, and dry cough. Additionally, some erythematous nodular lesions on the skin of the lower extremities were present. A chest X-ray was suggestive of interstitial pneumonitis. A chest CT showed multiple enlarged mediastinal lymph nodes occupying the perivascular space. A ground-glass pattern was present in a segment of the right superior lung lobe, apical and posterior segments of the right inferior lung lobe and posterior segments of the left inferior lung lobe. Additionally, there was bilateral pleural effusion, mainly on the right side. Fibrobroncoscopy was performed, tuberculosis, fungal infection, and malignancy were ruled out by Ziehl-Neelsen staining (ZN), methenamine silver staining, and cytology, respectively.
During endocrinology follow-up, parathyroid hormone (PTH)-independent hypercalcemia (12.7 reference value 8.7-10.4 mg/dL) and hypercalciuria (412 mg/24 hours, reference value 100-200 mg/24 hours) were found. The patient required mediastinoscopy and biopsy of the enlarged lymph nodes. Fungi and acid-alcohol-resistant bacilli infection were ruled out. Due to the presence of a non-necrotizing granulomatous lymphadenopathy, a diagnosis of sarcoidosis was made. The patient was started on oral prednisolone 40 mg per day (0.7 mg per kg per day), azathioprine 100 mg per day and a calcium/vitamin D supplement. Ophthalmic sarcoidosis was ruled out. Follow-up at 13 months showed no signs of recurrence of sarcoidosis or Cushing’s syndrome. The patient remained well with prednisolone treatment, and her symptoms of sarcoidosis did not reappear. The prednisolone dosage was kept unchanged. |
pmc-6424550-1 | A 48-year-old man was admitted with a recurrence of previously diagnosed right knee septic arthritis requiring multiple surgical interventions and treatment with high-dose narcotic analgesia. During his hospitalization, he attempted to place his upper left dental bridge, but he accidentally ingested it. He noted that the partial dental appliance had an exposed screw. On evaluation, the patient felt that the appliance was stuck in his upper chest and was associated with significant chest discomfort. Examination revealed audible upper airway wheezing, but normal bowel sounds and no abdominal tenderness. Chest X-ray showed a radiopaque foreign body (denture) near the gastroesophageal junction, and emergent endoscopy (EGD) was done, but the appliance had passed beyond the reach of the upper endoscope and was not visualized. Serial abdominal X-rays were performed to observe denture passage through the gastrointestinal (GI) tract where it eventually came to rest in the area of the cecum/ascending colon (Figure ).
There was no further advancement after three days and bowel preparation, and the patient continued to complain of abdominal pain. Therefore, a colonoscopy was performed for removal. On entry into the proximal ascending colon, the appliance was visualized clearly (Figure ).
A snare was used to grasp the exposed screw, and it was slowly removed with one attempt. The patient’s abdominal pain resolved post-procedure and was discharged in a stable condition. |
pmc-6424554-1 | A 23-year-old Caucasian female with a past medical history of heparin-induced thrombocytopenia (HIT), deep venous thrombosis (DVT) in her left lower extremity (LLE), and pulmonary embolism (PE) came to the hospital with low-grade fever, worsening LLE swelling, and redness for two weeks. She denied any recent history of trauma, prolonged immobility, chest pain, shortness of breath, or weight loss. She also denied any history of alcoholism, tobacco, or illicit drug use. Her family history was significant for systemic lupus erythematosus (SLE) in her mother and factor V Leiden mutation in her father. Her medication included coumadin and over-the-counter painkillers. Her initial vitals revealed a low-grade fever of 100.6 F. The physical exam showed LLE swelling extending up to the proximal calf, with mild diffuse redness of the skin and no demarcation. Her lungs were clear to auscultation bilaterally, and her oxygen saturation was 98% on room air.
Investigation
Her blood cultures were obtained and admitted to the general medical floor on broadspectrum antibiotics and pain medications. On the first day of admission, her blood workup was significant for the following: international normalized ratio (INR) = 2.7; partial thromboplastin time (PTT) = 45 seconds; blood urea nitrogen (BUN) = 13 milligram per deciliter (mg/dL); and serum creatinine = 0.74 mg/dL. Complete blood count was evident for white blood cell (WBC) count = 5.5 k/UL; hemoglobin = 10.4 g/dL; and platelet count = 324 k/UL. Urinalysis and chest X-ray (posteroanterior (PA) view) were within standard limits. Venous Doppler of her LLE was significant for a large DVT in the left common femoral vein. Ultrasound also revealed an abnormal compression of her left common femoral (Figure ) and popliteal vein.
She was started on argatroban infusion due to her history of HIT. She was initially started on broadspectrum antibiotics, which were stopped eventually upon negative culture data and no source of infection.
Differential diagnosis
Upon presentation, we suspected either LLE acute cellulitis, recurrent DVT, hypercoagulability or warfarin failure due to skipped doses, or thrombophilia associated with antiphospholipid antibody syndrome (APLAS). But our suspicion of MTS grew after getting a normal blood culture, WBC count, therapeutic range INR upon presentation, and normal blood titers for the anticardiolipin antibody, antinuclear antibody, and factor V Leiden.
Treatment
Due to the extent of the thrombosis and persistent severe pain, it was decided to send her for IR-guided mechanical thrombectomy. She underwent successful in-line pharmacokinetic thrombolysis with alteplase and IR-guided mechanical thrombectomy. The patient remained on argatroban infusion for four days. Her LLE venous Doppler scan was repeated, which revealed a recurrence of the clot. Vascular surgery was taken on board, and she underwent iliofemoral venous stent placement. Eventually, she was discharged from the hospital on Day 10 on fondaparinux.
Outcome and follow-up
The patient followed up with the hematologist after four weeks of her discharge. She had a repeat venous Doppler of her left leg, which showed no recurrence of her DVT. |
pmc-6424557-1 | A healthy 13-year-old female with an unremarkable medical history was referred to our outpatient clinic due to a one-year history of headache. Routine hematological tests and serum chemistry were normal. T1-weighted gadolinium-enhanced brain magnetic resonance imaging (MRI) (Figure ) revealed a large, homogeneously enhancing intraventricular mass in the right lateral ventricle with associated obstructive hydrocephalus.
Gross total removal of the tumor was achieved. Surgery was uneventful and a right external ventricular drain (EVD) was placed after tumor resection. The histopathology of the tumor was a transitional meningioma, World Health Organization (WHO) I.
The patient’s immediate postoperative recovery was marked by two episodes of wound cerebrospinal fluid (CSF) leak, which were treated conservatively with local stitches. However, five days after surgery, she developed a high fever (39.8°C) and a purulent discharge from the EVD. The physical examination revealed neck stiffness. She had marked leukocytosis (38.5×109/l) although her biochemical parameters were within normal limits. The CSF examination obtained via the EVD revealed severe hypoglycorrhachia (2.2 mg/dL versus an expected level of 66–77 mg/dL at blood glucose level 111 mg/dL), with a hyper proteinorachie of 2581 mg/L (normal <450 mg/L). After sending CSF, urine, and blood samples for cultures, IV vancomycin (2g/day) and ceftazidime (6g/day) were initiated empirically. On the following day, the CSF culture was positive for carbapenem-resistant A. baumannii and the antibiotic protocol was changed to the maximum recommended colistin dose according to patient weight (6 million units/day). After three days, the patient’s high fever (40.1°C), neck stiffness, and leukocytosis (33.7×109/l) persisted, with worsening hypoglycorrhachia (0.44 mg/dL versus an expected level of 58–68 mg/dL at blood glucose level 99 mg/dL) and hyper proteinorachie (6967 mg/L). The CSF culture remained positive for carbapenem-resistant A. baumannii. T1-weighted gadolinium-enhanced (Figure ) and diffusion-weighted imaging (DWI) MRI studies (Figure ) revealed a right subdural enhancing collection with a diffuse bilateral intraventricular restriction, subdural empyema, and severe ventriculitis.
The craniotomy site was reopened, the subdural empyema was drained, and the EVD was replaced by a new catheter, without surgical complications. Intraventricular colistin (150,000 units (10 mg) once daily) was added to the antibiotic treatment after surgery. After five days, fever (38.1°C) and neck stiffness were somewhat reduced and the leukocytosis was improved (18.1×109/l); however, hypoglycorrhachia persisted (11 mg/dL versus an expected level of 52–61 mg/dL at blood glucose level 89 mg/dL), hyper proteinorachie had deteriorated (9433 mg/L), and CSF culture for carbapenem-resistant A. baumannii remained positive. The isolate was found to be sensitive to tigecycline (minimum inhibitory concentration (MIC) of 0.38 µg/ml), and IV tigecycline was added to the combination therapy (50 mg twice daily, following a loading dose of 100 mg). After one week, the patient showed complete clinical recovery, resolution of the leukocytosis, and sterile CSF; however, hyper proteinorachie persisted (1996 mg/L). The EVD was removed two weeks after the second surgery and intraventricular colistin was discontinued. IV tigecycline-colistin continued for four more weeks. The patient developed headaches and blurred vision. Contrast-enhanced head CT revealed an improvement of the intraventricular enhancement and subdural collection but an enlargement of the ventricular system and transependymal edema with a communicating hydrocephalus. The patient underwent ventriculoperitoneal (VP) shunt insertion. There were no complications or signs of further VP shunt infection or hydrocephalus. She was discharged at the end of therapy, six weeks after the reopening of the craniotomy site, without any evidence of infection. At the six, 12, 18, and 24-month follow-up, the patient had reached a Glasgow Outcome Score of 5 with no sign of a new infection. |
pmc-6424585-1 | A 73-year-old male with a significant asbestos exposure and a recent history of recurrent diverticulitis presented to the gastroenterology clinic with persistent left lower quadrant (LLQ) pain despite several courses of empiric antibiotic therapy. Computed tomography (CT) scan completed during a previous hospitalization which showed fluid near sigmoid colon suggested nonspecific colitis (Figure ). Repeat CT performed due to nonresolving symptoms showed possible nodularity of the mesentery (Figure ). Subsequent positron emission tomography (PET) scan demonstrated multiple hypermetabolic mesenteric lesions, notably in the left paracolic gutter and portion of pelvis (Figure ). The findings favored carcinomatosis. A colonoscopy was subsequently performed which demonstrated severe diverticulosis, but no obvious luminal lesions. Tumor marker serology was negative. The patient was eventually referred to colorectal surgery and an exploratory laparoscopy was done. He was noted to have extensive peritoneal carcinomatosis involving all mesenteric surfaces and partial involvement of the right diaphragm. The disease involved predominantly the LLQ, with encasement of the left colon over the pelvic brim and into the pelvis between the bladder and colon. A sigmoid colon resection with diverting colostomy was performed to provide symptomatic relief. Final pathology revealed malignant epithelial mesothelioma with peritoneal seeding. The patient was referred to oncology and was started on hyperthermic intraperitoneal chemotherapy (HIPEC) and cytoreductive surgery (CRS). |
pmc-6424587-1 | A 41-year-old male with a past medical history significant for a reported history of post-traumatic coma, post-traumatic epilepsy with prolonged (three to four days) Todd’s paralysis, bullet wounds to the head, two ocular strokes with left eye blindness, coronary artery disease (CAD), post-percutaneous coronary intervention (PCI), hypertension, benign prostatic hyperplasia (BPH), spinal stenosis, attention deficit hyperactivity disorder (ADHD), and bipolar disorder presented to the hospital with reported multiple seizures and left-sided post-ictal paralysis. The patient had a history of multiple admissions to the hospital due to seizures. He stated that he began having seizures after being attacked 24 years ago, where he sustained multiple injuries to the head, and claimed that he was in a coma for one year. Since then, he has carried the diagnosis of post-traumatic epilepsy for over 20 years. He reports having eight to nine seizures per month with associated “Todd’s paralysis,” which, according to him, resolves on its own after three to four days. He has been seen by multiple neurologists and has tried multiple antiepileptic drugs without seizure control. During the present admission, the patient was on phenytoin 400 mg and levetiracetam 1000 mg. He had therapeutic levels of both medications during this admission.
On examination, the patient’s mental status and cranial nerves examination were normal; the motor exam was significant for paralysis; and strength was 0/5 in the left arm and leg. He also complained of hemi-sensory loss on the left side of the body that was significant for no reaction to noxious stimuli. Reflexes were symmetrical and 2+ bilaterally. The patient was not able to ambulate due to the weakness. The psychiatric examination was significant for anxiety and auditory hallucinations. During an interview, the patient was slightly guarded, irritable, and talkative but redirectable.
Magnetic resonance imaging (MRI) of the brain was normal; specifically, there was no evidence of traumatic brain injury or any bullet injury as reported to and by the patient (Figure ). Routine electroencephalography (EEG) was normal (Figure ). Due to the lack of definite evidence of epilepsy, a video-EEG with medication titration was performed continuously for five days. The study was normal, with no focal or generalized epileptiform abnormalities noted. During the recording, there was no EEG correlation with the patient’s complaints of left-sided weakness. The EEG background was normal (typically, a patient with such severe weakness should have a slowing in the right hemispheric region). On the first day of the recording, when he had reported left-sided weakness, he was noted to have movements on the left side during sleep. Given that there was no evidence of epilepsy and psychogenic weakness, the patient was successfully weaned off all anti-seizure medications.
The patient was discharged home after six days fully functional: walking, speaking, and eating on his own. He was glad to know that he did not have epilepsy and was very thankful for the care and diagnosis received. |
pmc-6424588-1 | A 49-year-old woman was admitted for an elective abdominal hysterectomy. She was an unmarried psychiatrist originally from Argentina, living in Northeast USA. She had an 11-year history of asthma and a history of hypertension. Her surgical history included tonsillectomy at age 11, right carpal tunnel release at age 37, cholecystectomy for cholelithiasis at age 41, right breast nodule excised at age 45, which showed intraductal hyperplasia, adenosis and chronic cystic mastitis (fibrocystic change), and cervical laminectomy at age 47. Her social history included a 10 pack-year history of smoking ending at age 32 and no history of alcohol use.
On admission, she was afebrile, with a pulse of 96 beats/minute, blood pressure of 168/104 mmHg, respirations of 20 breaths/minute, and obesity (body mass index 35.5 kg/m2). Her chest was clear and she had a 14 cm "nodular fibroid uterus." Preoperative white blood cell count was 10,300/cu mm, hematocrit 36.8%, potassium 3.7 mEq/L, sodium 142 mEq/L, blood urea nitrogen 11 mg/dL, and urinalysis negative. Chest X-ray showed "poor inspiratory effort with low lung volumes." The electrocardiogram showed small Q waves in the inferior leads.
The patient underwent a total abdominal hysterectomy with bilateral salpingo-oophorectomy. The surgical specimen showed a 5 cm leiomyoma, endometriosis, and a hemorrhagic corpus luteum of the right ovary. The first postoperative day was uneventful. On the second postoperative day, the patient developed postprandial nausea and vomiting relieved with prochlorperazine. The abdominal X-ray showed an ileus. The patient's potassium was 3 mEq/L. This was corrected to 4 mEq/L but the ileus persisted.
On the third postoperative day, repeat chest X-ray showed atelectasis despite incentive spirometry. That night, the patient had insomnia, which she had also suffered each of the previous three nights. There was a night shift nurse on duty each of these nights, who helped the patient pass the time, talking to her when she was not busy with other patients. That night, at midnight, the nurse took the patient's vital signs: her temperature was 39.1 degrees C (102.4 degrees F), pulse 88 beats/minute, blood pressure 160/92 mmHg, and respiration 24 breaths/minute.
At 01:00, the patient told the nurse that she had slight surgical incision pain and emesis. The nurse paged the obstetrics/gynecology resident on call, who gave an order over the phone for oxycodone/acetaminophen and calcium carbonate. This was given.
At 02:00, the patient told the nurse that she was having an anxiety reaction. In fact, the patient told the nurse "I'm really nervous because I am going to die tonight." The patient told the nurse she needed a benzodiazepine and suggested one, with a specific dose. The nurse again paged the resident on call and told him that the patient was having an anxiety reaction and wanted a benzodiazepine sedative. The resident gave an order over the phone for diazepam. This was given.
At 03:40, the patient told the nurse that she was experiencing progressive dyspnea. Her temperature was 38.0 degrees C (100.4 degrees F), pulse 118 beats/minute, blood pressure 118/70 mmHg, and respirations 24 breaths/minute. The nurse again paged the resident on call, who gave an order over the phone to get a respiratory therapist to administer inhaled bronchodilator therapy. There were very few respiratory therapists on duty and the soonest one of them could get to this patient was 40 minutes later.
At 04:20, when the respiratory therapist arrived, the patient had respiratory distress with wheezing and a respiratory rate of 36/minute. She was diaphoretic, cold, and clammy. Her blood pressure was 100/60 mmHg. At 04:30, inhaled bronchodilator therapy was only partially successful in relieving the patient. The nurse again paged the resident on call who gave an order over the phone for intravenous aminophylline therapy.
At 05:00, an attempt by the nurse to start an intravenous line for aminophylline therapy was unsuccessful. While a second attempt was underway, the patient began vomiting large amounts of bilious yellow-green fluid. Shortly after this, the patient suffered a respiratory arrest. Cardiopulmonary resuscitation was begun, but it was to no avail and the patient was pronounced dead at 06:15.
The postmortem examination revealed small amounts of freshly aspirated gastrointestinal contents within the tracheobronchial tree and within lower lobe alveoli. In addition, the autopsy demonstrated mucoid fluid secretions in the bronchi, bilateral areas of atelectasis, patchy acute bronchitis, and pneumonia within the lower lobes, more on the right side. The stomach and entire length of small and large intestines were massively dilated. The liver had diffuse marked steatosis. |
pmc-6425290-1 | A 74-year-old man presented to the Emergency Department (ED) on April 2016, after 4 days of fever, productive cough, and abundant “yellow” sputum; he was started on oral levofloxacin on the diagnosis of tracheobronchitis. As symptoms worsened, he returned to the ED.
On initial evaluation in our ED, he complained of persistent moderate dorsolumbar pain and productive cough, without shortness of breath. There was no history of vomiting, aspiration, or other abdominal complaints.
On physical examination, there were no signs of respiratory distress and no jaundice and, upon cardiac and pulmonary auscultation, no abnormal sounds were detected. He was hemodynamically stable, with no fever; pain was recorded as 6/10 (numeric rating scale for pain) on arrival but resolved soon after admission. Thoracic X-ray showed no pleural or lung parenchymal lesions. According to previous sputum microbiological isolate, Escherichia coli ESBL+, he was started on endovenous meropenem and was admitted to the general ward.
He had a metastatic colorectal cancer (CCR), diagnosed in 2013; the tumor was RAS mutated; the disease was staged as oligometastatic liver disease at the beginning. He was treated with primary chemotherapy, mFOLFOX6 (5-fluorouracil, leucovorin, and oxaliplatin) and bevacizumab, from November 2013 to November 2014 and had a selective right portal vein embolization in June 2014, in order to be able to perform liver surgery with adequate remnant liver. On February 2015, a sigmoid colectomy was performed as well as liver segmentectomy of the VI and VII segments, conditioned by proximity to vascular structures and cholecystectomy. Surgical resection specimen confirmed liver metastasis with complete pathological response of the primary lesion, staged as ypT0N0M1. It was considered a R1 surgery due to liver margin intersection (vascular margin).
Postoperative period was prolonged due to bilioenteric fistula and liver abscess adjacent to the segmentectomy scar that evolved to septic shock; blood cultures and peritoneal drainage were positive for Escherichia Coli, ESBL+.
He was discharged on the 40th postoperative day (May 2015) after successful medical treatment (large spectrum antibiotic and interventional radiology drainage), with normal liver tests and no abnormal findings on CT scan.
He had no further complementary chemotherapy.
On January 2016 lung and liver progression (relapse on the surgical scar) were detected on CT scan. Lesions were considered unresectable. Chemotherapy was restarted with mFOLFOX6 combined with bevacizumab, receiving 4 cycles until this hospital admission.
Initial cultural exams confirmed Escherichia Coli ESBL+ respiratory infection associated with same agent bacteremia; the antibiotic susceptibilities test was identical to the previous one; cultural exams turned negative after the first week of antibiotic. Although inflammatory parameters and arterial gasometry improved, he kept daily fever (>38°C) and expelled large volumes of yellow-green sputum (>1L per day); as symptoms kept ongoing he had a chest and abdomen CT scan on hospitalization day 15. In the liver, there was a lesion with air and necrotic component adjacent to the metastatic subdiaphragmatic lesion scar relapse and adjacent lung atelectasis (). No fistula trajectory was evident. There was no pleural or pericardial effusion.
A bronchoscopy was performed showing right middle bronchus obstruction with a large amount of thick and brown secretions (with microbiological isolate of Escherichia coli ESBL+). Interventional radiology drainage for the subdiaphragmatic metastatic abscess was performed; the possibility of a fistula between the abscess and the lung was confirmed after local instillation of radiological contrast that the patient expelled with cough.
After drainage, the patient had a significant clinical improvement; 1 month later, with only residual liver drainage, the drain was removed.
While on antibiotics, the patient received inpatient rehabilitation and respiratory therapy and was successfully discharged on the 63rd day.
He was then followed-up as an outpatient in the gastrointestinal cancer clinic, with progressive resolution of the respiratory symptoms and no clinical evidence of recurrence of the fistula. |
pmc-6425300-1 | A 61-year-old female with poorly controlled diabetes mellitus with severe bilateral PDR presented to the emergency department at Upstate University Medical Center. Initially, the patient presented to an outside emergency room with conjunctival injection, copious purulent discharge and associated painful vision loss to no light perception (NLP) in the left eye. Patient was then transferred to Upstate University Medical Center for higher level care and was evaluated by the ophthalmology service 18 hours after symptom onset. She underwent lateral canthotomy and cantholysis at the outside hospital 6 hours prior to arrival, due to severe pain and concern for orbital compartment syndrome; however patient endorsed complete loss of vision 12 hours prior in her left eye. She had no ocular trauma or history of intravenous drug abuse. However, she noted to have a two-month history of intermittent left abdominal pain and diarrhea, along with an abdominal computed tomography (CT) reporting diverticulitis. On presentation, examination revealed an edematous, erythematous, and proptotic left eye with severely restricted extraocular movements (). The anterior segment exam revealed diffuse conjunctival injection, mucopurulent discharge from superotemporal globe (), diffuse corneal edema and haze, and extensive fibrin in the anterior chamber. The dilated exam was limited due to corneal edema, diffuse anterior chamber reaction, and dense vitritis. Ophthalmic ultrasonography revealed a subluxed lens with diffuse vitritis (). Orbital CT confirmed no retained intraocular foreign body () or occult penetrating injury.
Upon admission, patient's blood and specimens from ocular mucopurulent discharge were sent for gram stain and culture. The gram stain revealed gram-positive bacillus. The patient was admitted and started on intravenous (IV) broad spectrum antimicrobial therapy (vancomycin, meropenem, and amphotericin B). Due to progressive clinical decline with associated leukocytosis and encephalopathy 48 hours after initiating systemic antimicrobial treatment and gram stain results, she underwent enucleation of the left eye. Postoperatively, the patient completed a full course of IV vancomycin and meropenem.
Reflex anaerobic culture from the mucopurulent collection grew Clostridium perfringens and gross specimen from the enucleation revealed numerous gram-positive bacillus microbes. Anaerobic microbes seen on staining and samples were inoculated onto prereduced anaerobically sterilized Brucella blood agar, phenylethyl alcohol blood agar, kanamycin-vancomycin laked blood agar, and Bacteroides bile esculin agar (Oxyrase, Inc., Mansfield, OH). The inoculated plates were incubated at 35 degrees C anaerobically using the AnaeroPack-Anaero Anaerobic Gas Generator (Mitsubishi Gas Chemical America, New York, NY). After 48 hours of incubation, 2+ growth of a gram-positive bacillus was noted on the Brucella blood agar. These colonies were identified as Clostridium perfringens by Matrix-Assisted Laser Desorption Ionization-Time of Flight Mass Spectrometry (Vitek MS, bioMerieux, Inc., Durham, NC). No other growth was observed.
Amphotericin B was discontinued once fungal etiology was excluded. Subsequently, both the periorbital edema and erythema resolved. Additionally, the patient regained consciousness, as her encephalopathy and infection improved. She was subsequently transferred to local rehabilitation facility. A repeat CT abdomen demonstrated the previous inflammation had resolved, likely due to the aggressive inpatient broad spectrum antibiotics. No comorbid occult distal gastrointestinal malignancies were found. |
pmc-6425302-1 | A 38-year-old man came for orthodontic treatment with tooth abrasion as his chief complaint. His medical and dental histories showed good general health, except for a stressful lifestyle. He did not report any mandibular movements with nocturnal bruxism but did report functional movements or clenching in the daytime. He also stated that, when brushing, he applies too much force.
Dental analysis showed an Angle Class I malocclusion, normal overjet, a moderate overbite, and a slightly maxillary midline, shifted to the right (a posterior crossbite on the right side involved the first molar and both bicuspids (). Gingival retraction and dental abrasion in the facial cervical region were localized in the cervical areas of the maxillary canines and incisors (). There was wear on the occlusal and tip of the canines, premolars, and molars (Figures , , , , and ) and severely worn facets on the palatal surface of the maxillary canines and incisors, due to probable strong contact with the tip of the mandibular incisors ().
During the clinical examination, the patient had excellent plaque control and good gingival health. The cephalometric analysis showed a skeletal Class III relationship (ANB = -1°; AoBo = -5 mm), brachyfacial pattern (FMA = 17°), upright mandibular incisors (1-NB = 15°), and a concave profile (Z-angle = 78°) (). The panoramic radiograph showed extensive restorations on the maxillary and mandibular molars ().
The primary goals of the treatment proposed were as follows: transverse skeletal expansion of the maxilla, creation of a condition to restore the palatal side of the maxillary canine and incisors, and transverse dentoalveolar expansion of the maxillary right side.
Based on the treatment objectives, the following treatment options were suggested: surgically assisted maxillary expansion, associated with a hyrax-type expander; transpalatal arch; intermaxillary elastics; expansion with arches and intermaxillary elastics; or mini-expandex-type expander for dentoalveolar expansion.
The patient did not accept the surgically assisted palatal expansion, so the first step was to affix the fixed mini-expandex-type expander to the maxillary first molars. On the left side, an extension was established on the second premolar and the second molar to increase the anchorage (Figures and ). The activation was once daily for 10 days. After one month, fixed 0.022 in × 0.028 in standard edgewise brackets were bonded first on the maxillary arch, and 45 days later, on the mandibular arch. A 0.016 in SS archwire, with a mesially bent helicoidal loop, was used to jump both premolars, buccally (Figures and ). After removing the mini-expandex, vertical crossbite elastics were attached to the archwire ().
After completing the orthodontic treatment, three sessions of dental whitening were performed, with 38% hydrogen peroxide. The technique of direct restoration of the composite resin in the anterior (palatal surface) and posterior (cusp tip and cervical/facial lesion) teeth, using a universal adhesive system, allowed a better control of the restorative sequence []. The restorations were performed to the enamel and dentin and then photopolymerized for 10 seconds. The resins used were Charisma (Heraeus/Kulzer) on color OA2, Amelogen (Ultradent) color A2, and Durafill (Heraeus/Kulzer) color A2. After 14 days, the finishing and polishing of the composite resin restorations were carried out, achieving an excellent aesthetic result with this restorative technique ().
The posttreatment records showed the correction of the maxillary transverse deficiency and the restoration of palatal surface abrasion of the maxillary canine and incisors. Ideal overjet and overbite relationships were maintained, with good intercuspation. The buccal cuspids of the maxillary right molar and premolars were built-up with composite resins (). Retention was performed by removal of the wraparound-type appliance on the maxillary arch and bonded lingual arch, from the mandibular canine to canine. The treatment time was nine months.
The cephalometric analysis did not show changes in the skeletal relationships and did show maintenance of the incisors' inclinations (Figures and and ). The panoramic radiograph showed no significant root resorption (). The transverse dimension was changed slightly after treatment, where the intermolar width was slightly changed from 37.0 mm () to 41.0 mm ().
After 37 months, follow-up photographs showed a stable occlusion, with maintenance of the overjet and overbite, as well as the molar and canine relationships, except for a slight rotation of the mandibular left central incisor. The patient stopped using the retainer one year after debonding (). |
pmc-6425303-1 | An 8-year-old Caucasian boy with ASD presented to our clinic with a history of limping and recurrent left knee pain mainly in the back of the knee, exacerbated with activity and partially alleviated with rest. Clinical examination was difficult to perform, due to the strong opposition of the autistic child. His left knee was not swollen and no joint line tenderness was elicited. Range of motion (ROM) of the left knee was slightly limited in extension compared with the opposite side, and hyperflexion was painful and slightly limited as well. The McMurray, Lachman, and varus/valgus stress tests were all negative. Plain radiographs were performed and resulted normal. A second-level imaging was needed, but the presence of ASD complicated the execution of the test, so MRI of the left knee was performed under general anesthesia with sevoflurane. Scans revealed a 29 mm × 16 mm × 17 mm well-defined septated cyst located in the intercondylar notch between the ACL and PCL, abutting predominantly posteriorly to the PCL. The round-shaped cystic mass encasing the PCL depicted homogeneous low-signal intensity, slightly hyperintense relative to the muscles, on proton density-weighted image (PDWI) and on turbo spin echo (TSE) imaging and high-signal intensity on turbo inversion recovery magnitude (TIRM) images ().
Arthroscopic surgery was performed under general anesthesia and a tourniquet was used. The location of the cyst correlated with the MRI findings. Arthroscopic examination, performed through standard anterolateral and anteromedial portals, revealed a large white encapsulated ganglion cyst, with blood vessels on the surface, filling the femoral notch (). The cystic mass, arising from the PCL, enveloped PCL fibers and extended posteriorly in intimate connection with the posterior capsule. Both cruciate ligaments appeared intact as medial and lateral menisci were without tears under arthroscopic examination. Before excising the cyst, we used punch forceps to obtain a specimen for histopathology, then a motorized shaver was used to excise the cyst completely from the PCL and the posterior capsule. After debridement, any persistent bleeding has been controlled using a radiofrequency ablation probe to ensure hemostasis. A pressure dressing was applied onto the affected knee after the operation.
Histologic examination revealed the proliferation of synovial cells lined with dense fibroconnective tissue, widespread thick bundles of collagen and capillary proliferation, confirming the diagnosis of posterior cruciate ligament ganglion cyst (). The symptoms improved immediately after the operation, and the patient was addressed to a short postoperative rehabilitation program due to his neurological condition. At 3 months postoperatively, the patient had an International Knee Documentation Committee (IKDC) score of 97, as he was able to perform all activities of daily living, including squatting and sitting in the crossed leg position, and had full ROM. Twelve months after successful arthroscopic excision, no recurrence was detected under clinical examination. |
pmc-6425306-1 | A 22-year-old female was referred to our endocrine clinic with 2-year history of gradually progressive proximal muscle pain and weakness involving both upper and lower extremities. Her pediatrician had treated her with prednisone for a period of two months without any improvement. MRI of pelvis and thighs completed before her referral had shown linear hypointense foci in both proximal medial femoral necks and the right ischium consistent with insufficiency fractures. She had a normal childhood development and pubertal growth and at presentation had a height of 154cm and a body weight of 57.1kg. There was no reported family history of rickets, osteomalacia, or any other metabolic bone disease. Her menstrual history was unremarkable.
Past medical history was significant for a diagnosis of carnitine palmitoyl transferase 2 (CPT2) deficiency at the age of 14 which was diagnosed in the context of a work-up for diffuse muscle pain and weakness. At that time, she was evaluated in the Neurology clinic and was found to have low serum carnitine levels, elevated serum alanine, normal lactate, and low pyruvate levels. C16, C18:2, C18:1, and C18 levels were elevated suggesting carnitine palmitoyltransferase II deficiency. However, genetic testing was negative for the S113L variant. Results of other biochemical testing at that time were not available in her medical records. Her symptoms had reportedly completely resolved a few months after she was started on oral carnitine replacement therapy.
Physical exam did not show any apparent kyphosis or scoliosis of spine, vertebral tenderness, or hyperextensibility of joints. There were no lower extremity deformities. She had proximal muscle weakness with 4/5 strength in upper and 3/5 in the lower extremities. There was tenderness in the thighs. Her neurological exam was otherwise normal. She had a waddling gait.
Laboratory work-up revealed the following results: calcium, 9.1 (ref: 8.6-10.2 mg/dl); phosphate level, 2.0 (ref: 2.5-4.5 mg/dl); alkaline phosphatase, 243 (ref: 39-136 Units/L); bone specific alkaline phosphatase, 119.6 (ref: 0-21.3 mg/dl); 25-hydroxy vitamin D, 8.2 (ref: 30-100 ng/ml); and PTH, 145 (ref: 22-84 pg/ml). A bone density scan showed an abnormally low bone density matched for age, gender, and ethnicity with the lowest Z score of -3.3 at the left femoral neck. Vertebral fracture assessment score was within normal limits.
A 24-hour urine collection revealed low calcium of 27.6 (ref: 100-300.0 mg/24 hr) and inappropriately normal urine phosphate excretion of 445 mg/dL (ref: 400.0-1300.0 mg/24 hr). Fractional excretion of phosphate was inappropriately normal at 19% (ref: 15–20%). Renal tubular reabsorption of phosphate (TmP/GFR) was low at 0.50 (ref: 3.18-6.41 for 16- to 25-year-old females). These results suggested renal phosphate wasting as a potential etiology for osteomalacia and we considered Tumor-Induced Osteomalacia (TIO) and Hereditary Hypophosphatemic Rickets (HRR) as diagnostic possibilities. However, in the absence of any family history of rickets and her normal childhood growth and stature, rickets appeared to be less likely and subsequent work-up was directed at ruling out TIO. An Octreotide scan with SPECT imaging came back negative and was followed by F-18 FDG PET/CT scan, which too failed to reveal any tumor. However, an FGF-23 level was found to be elevated at 580 (ref: 44-215 RU/mL). The assay used is a second-generation C-terminal assay that measures both the intact FGF-23 and its C-terminal fragments. At this stage, following the negative localization studies for TIO, the patient was counseled on genetic testing to look for the possibility of hereditary hypophosphatemic rickets. She consented for the genetic testing which revealed a heterozygous known pathogenic missense variant in FGF23: c.527 G>A p. Arg176Gln (R176Q) consistent with a diagnosis of “Autosomal Dominant Hypophosphatemic Rickets”.
Genetic screens for variants in PHEX (X-linked Hypophosphatemic Rickets) and DMP-1 (Autosomal Recessive Hypophosphatemic Rickets 1) were negative. These results were therefore consistent with a diagnosis of Autosomal Dominant Hypophosphatemic Rickets (ADHR) caused by a mutant FGF23. When we informed the patient about the genetic diagnosis, she did some further research regarding her family history and found out that her father's maternal aunt and grandfather had ‘bone disorder' and were wheelchair-bound in early adulthood but her mother remained in good health and had no symptoms
She was started on Ergocalciferol 50,000 IU weekly and was encouraged to increase her dietary calcium to 1000 mg daily. She was also prescribed Potassium-Sodium-Phosphate 250 mg (8 mmol) 4 times a day. She continued to have pain and discomfort in her inner thighs. She was prescribed Diclofenac and Flexeril for pain. MRI of the pelvis was repeated to follow up on the insufficiency fractures and showed persistent bilateral femoral stress insufficiency fractures (). She was referred to orthopedic surgery and underwent pinning of bilateral femoral necks. She had significant improvement in pain and discomfort in the right thigh and groin region but still had some discomfort in the left upper thigh.
Her 25(OH)D levels normalized to 46.2 ng/ml as did her serum phosphate level (2.7 mg/dL) with oral replacement therapy.
Due to persistent pain in her left groin, she was prescribed narcotic analgesics and underwent a steroid injection of the psoas muscle by orthopedics with no relief. A repeat X-ray of pelvis showed new insufficiency fractures of the left superior and inferior rami ().
The patient had difficulty adhering to the regimen of potassium-sodium-phosphate 250 mg (8 mmol) four times daily. Therefore, she was started on calcitriol 0.25 mcg once a day. The dose of calcitriol was gradually increased up to 2 mcg per day. The patient reported significant improvement in her pain after the introduction of calcitriol to her regimen.
Although she missed a few follow-up appointments in the endocrine clinic, she was eventually seen a year later and reported feeling significantly better on the same treatment regimen of calcitriol 2 mcg daily, potassium-sodium-phosphate 250 mg (8 mmol) twice daily, vitamin D3 2000 units daily, and 600 mg of calcium supplement daily. She had complete resolution of her pain and was not on any pain medications. Her physical exam showed normal strength in the upper and lower extremities and no tenderness. Laboratory work-up at that time showed complete resolution of the previous abnormalities: serum phosphate level was 3.3 (ref: 2.5- 4.5 mg/dl); calcium, 9.4 (ref: 8.6-10.2 mg/dl); alkaline phosphatase, 88 (ref: 39-136 units/l); and 25(OH)D, 44.6 (ref: 30-100 ng/ml). A repeat X-ray of pelvis showed resolution of the previous left superior and inferior pubic rami fractures.
The patient subsequently enrolled in a clinical trial of iron supplementation for patients with ADHR at another institution. Correspondence received from that institution showed a hemoglobin 10.1 (ref: 12-15.5 g/dl), hematocrit 31 (ref: 39.4-44.5%), MCV 78 (80-96 fL), platelets 313, 000 (ref: 150,000-400,000/ml), WBC 8.400 (ref: 4,000-11,000 ml), Total Iron Binding Capacity (TIBC) 378 (ref: 240-450 mcg/dl), ferritin 6.4 (ref: 12-300 ng/ml), and percent iron saturation 3% (ref: 25-35%). These results are consistent with iron deficiency anemia. The patient has not followed up with our endocrine clinic since then and we have not been able to reach her despite multiple attempts to obtain any update on her condition following iron supplementation trial. |
pmc-6425315-1 | A 71-year-old gentleman who was diagnosed at age of 59 with high grade urothelial carcinoma involving a bladder diverticulum with associated carcinoma in situ underwent a radical cystectomy, prostatectomy, extended lymphadenectomy, and orthotopic Studer ileal neobladder. Pathology confirmed a high grade urothelial carcinoma arising in a right-sided diverticulum with extension into the perivesical fat and associated carcinoma in situ. There was no evidence of extension into the urethra or ureters and all surgical margins were negative. None of the 40 lymph nodes removed showed carcinoma (pathologic stage pT3aN0Mx). He underwent adjuvant chemotherapy (methotrexate, vinblastine, doxorubicin, and cisplatin), but only tolerated 2 cycles before discontinuing due to side effects. He continued to have surveillance imaging and follow-up every 6-12 months.
On routine follow-up, 11 years following cystectomy, voided urine cytology was positive for high grade urothelial carcinoma. Follow-up CT imaging showed normal upper tracts and interval development of focal mural thickening involving the left anteroinferior neobladder (). He underwent a cystoscopy, which revealed a normal urethra, and a solitary lesion at the left anteroinferior wall of the neobladder. Biopsies of this mass revealed an invasive high grade urothelial carcinoma with focal squamous differentiation.
He subsequently underwent resection of the orthotopic neobladder and conversion to an ileal conduit. Intraoperatively, the mass involved the neobladder with possible extension into the pelvic sidewall and pubic bone periosteum. All gross disease was resected. Frozen section margins from pubic bone periosteum and urethra were negative. On gross examination of the final pathologic specimen, a 4.2cm x 2.3cm exophytic, friable mass was noted protruding into the neobladder lumen. Upon sectioning, this mass was noted to have variable depths of invasion, the highest being 9mm through the mucosa, submucosa, and serosa with involvement of the surrounding adipose tissue. Final pathology confirmed high grade urothelial carcinoma involving the neobladder and extending into the surrounding fat (Figures and ). No tumor was seen at the neobladder-urethral anastomosis and all surgical margins were negative. |
pmc-6425318-1 | A 34-year-old woman gravida 5, para 3, abortion 1 presented to the ER of our hospital, at 29 weeks' gestation, due to uterine contractions that increased in frequency and intensity in the last 5 hours, with no other symptomatologies added (). Her past medical history was unremarkable with O + hemotype; she had an abortion due to an anembryonic pregnancy that required curettage, which was performed without complications. The patient had 3 healthy previous pregnancies which resulted in 3 healthy living children. Currently in her fifth pregnancy, she denies pregnancy care; only one obstetric ultrasound performed at 24 weeks' gestation in another clinic reported the following: harmonic fetal growth and no fetal malformations; however polyhydramnios was present. During her observation in the ER, a new ultrasound examination was ordered, which revealed an apparently large placenta with approximate weight of 1,800 gr, suggestive of placental edema; the fetus appeared with polyhydramnios, and no heartbeats nor fetal movements were registered; she was then referred to the high risk obstetric department, where she was found to have normal vital signs, mild edema of the ankles without fovea, and a gravid uterus occupied by a single longitudinal cephalic fetus with lateralized back to the left; fetal heart rate was not detected with doptone; she had regular uterine dynamics palpable at a rate of 3 contractions lasting 60 seconds each, within a time frame of 10 minutes; at vaginal examination the cervix was softened with 4 cm of dilation and 70% thinned, intact amniotic membranes, without bleeding or leucorrhoea. Laboratory tests reported the following: hemoglobin 11.5 g/dl, hematocrit 34.8%, and no other abnormal results including normal renal and hepatic function. Due to increased frequency of contractions, she was immediately sent to the expulsive room, where a single female sex without vitality was spontaneously delivered, with data of hydrops and macerated skin, weight: 1,730 gr, with a grayish and hemorrhagic sacral mass (). After resection of the tumor and pathologic examination the following report was made: An 820 gr Type I sacrococcygeal teratoma (SCT) with mature and immature elements as well as blood sequestration areas, partially encapsulated with extensive areas of coagulation and necrosis. Placenta was grossly edematous and weighed 1,280 gr with a marked hydropic change of the villi, with an area of intervillous fibrinoid infarct was noticed. Amnion and chorion had no alterations. The umbilical cord had three blood vessels with marked edema of Wharton's jelly. The patient received counseling and was discharged 3 days after delivery without edema or any other physical alteration. |
pmc-6425334-1 | A woman in her late 60s was transferred in July 2012 from her local hospital because of anemia of at least one and a half year duration. She had no relevant family history, but had been treated for diabetes type 2 and hypertension for decades and paroxysmal atrial fibrillation for a few years. She had suffered from recurrent urinary tract infections, several times with fever and once with verified urosepsis. A concrement had been removed from her right ureter a few months ago.
Her history of anemia started gradually with fatigue in 2010-11. Her hemoglobin (Hb) level was 8.9 g/dL in February 2011, as compared to 13.4 at the last known previous assessment in 2008. She had received an erythrocyte transfusion at her local hospital without any transfusion reaction or other problems, and she had already suffered several exacerbations of anemia during febrile infections. There was no history of acrocyanosis or Raynaud phenomena.
On admission, she was in good general condition and did not present any pathological findings by physical examination. In particular, there was no acrocyanosis, lymphadenopathy, or splenomegaly. Chest radiography and abdominal ultrasonography were unremarkable. Hb was 8.2 g/dL, leukocytes 7.8 × 109/L with normal differential count, platelets 263 × 109/L, mean corpuscular volume (MCV) 99 fL, reticulocytes 88 × 109/L, and C-reactive protein (CRP) 11 mg/L. Serum levels of iron, transferrin, cobalamin, and folic acid as well as transferrin saturation were within the reference range, however, with elevated ferritin at 1257 μg/L. Genetic screening for hemochromatosis showed no HFE gene abnormalities. Concentrations of electrolytes, calcium, creatinine, and liver transaminases were normal. Lactate dehydrogenase (LDH) was elevated at 544 U/L, bilirubin elevated at 43 μmol/L, while haptoglobin was undetectable (less than 0.1 g/L). Her urine was negative for hemoglobin and protein. Serum albumin was 41 g/L with normal immunoglobulin levels (IgG 7.0 g/L, IgA 3.2 g/L, and IgM 0.69 g/L). Serum electrophoresis identified a small spike of monoclonal IgAλ. Free λ chains in serum were slightly elevated at 53 mg/L, however, with a κ/λ ratio within the reference range. The direct antiglobulin test (DAT) was strongly positive for C3d and negative for IgG, IgM, and IgA. CA titer at 4°C was 128. Serum erythropoietin was slightly elevated at 36 IU/L.
Based on these findings, she was diagnosed with CA-mediated AIHA. With a chronic course, no signs of malignancy so far, and no recent specific infection, this AIHA was further classified as primary CAD. A bone marrow trephine biopsy showed erythroid hyperplasia and small lymphocytic infiltrates interpreted as lymphoplasmacytic lymphoma (LPL). Flow cytometry in bone marrow aspirate revealed two small, clonal populations: one of B-lymphocytes that displayed a κ phenotype and the other one of λ positive plasma cells.
The further development in Hb and IgA levels is shown in . During the next couple of years, Hb ranged from 9.0 to 10.0 g/dL; she had only mild fatigue and no transfusion requirement. Management consisted of regular follow-up, avoidance of cold exposure, and prompt antibiotic therapy in case of febrile bacterial infection but no CAD-directed pharmacological therapy. By 2014, a second bone marrow biopsy showed erythroid hyperplasia and lymphoid infiltrates, now interpreted as probable CAD-associated LPD []. Flow cytometry in bone marrow aspirate again demonstrated 2 pathological clones of κ positive B-lymphocytes (approximately 5%) and λ positive plasma cells (0.7%), respectively.
Her condition remained stable until late spring 2016, when she deteriorated. Her fatigue worsened, her atrial fibrillation relapsed more frequently, and her Hb level declined to 8.5 g/dL. The reticulocyte count was now elevated at 191 × 109/L, LDH was 303 U/L, bilirubin 96 μmol/L, haptoglobin <0.1 g/L, while IgA had increased to 12.5 g/L and CA titer to 512. The DAT findings were unchanged. We decided to give her rituximab monotherapy [], four weekly doses of 375 mg/m2, which she received from August 2016. Hb rose to 12.5 g/dL by December 2016, while bilirubin and LDH normalized. Unexpectedly, however, her IgA level continued to increase from 12.5 g/L immediately before rituximab treatment to 19.6 g/L by January 2017.
From June 2017, Hb started again to decline and IgA continued to increase. In January 2018, she experienced severe lumbar pain after having removed snow from her courtyard, which can be heavy work in Norway. One month later, Hb was 9.5 g/dL, while the absolute reticulocyte count was 153 × 109/L and the levels of LDH, bilirubin, and haptoglobin had not changed significantly. The IgA level was 24.5 g/L and β2-microglobulin 3.5 mg/L. A new trephine biopsy showed hypercellular bone marrow affected by 20% infiltration of monoclonal, λ positive plasma cells, and some small lymphocytic infiltrates. A CT scan showed osteolytic lesions of the skull but no relevant pathological findings in her spine, pelvis, or femora. She was diagnosed with development of MM.
In February 2018, a local visiting hematologist initiated a cycle of melphalan and prednisolone. During the next couple of weeks, Hb declined further to 8.0 g/dL, however, without any neutropenia or thrombocytopenia. Concomitantly, a rise in bilirubin and LDH was observed, and she was diagnosed with an exacerbation of CAD. Melphalan and prednisolone were discontinued after the first cycle.
Hoping to induce remission of both MM and CAD, we decided in March 2018 to offer her bortezomib-based therapy [] with the addition of dexamethasone and rituximab [, , ]. She received four cycles at four weeks' interval of bortezomib (1.3 mg/m2 weekly for 3 weeks), rituximab (375 mg/m2 once per treatment cycle), and dexamethasone (20 mg on days 1, 2, 8, 9, 15, and 16). She tolerated this combination well; her Hb level increased from 8.0 g/dL to 13.6 g/dL following the first cycle and reached 14.4 g/dL after the second one. Bilirubin and LDH normalized within a couple of weeks, and reticulocyte count declined to the normal range (65 × 109/L). IgA had declined to 3.8 g/L by the start of the second cycle. Her back pain improved. Ten weeks after start of therapy, Hb was 13.2 g/L, bilirubin 7 μmol/L, LDH 247 U/L, haptoglobin 0.24 g/L, and CA titer 64. IgA remained in plateau at 3.9 g/L, while serum electrophoresis did not show any monoclonal spike. DAT was still positive for C3d.
The lymphoma pathologist in our group (U.R.) did a centralized, parallel review of all three bone marrow samples based on extensive experience with CAD-associated bone marrow pathology []. The 2012 biopsy shows, in addition to erythroid hyperplasia, intraparenchymatous, κ positive B-cell infiltrates consistent with CAD-associated LPD. There are scattered κ positive plasma cells, more of them near the lymphocytic infiltrates, and one distinct infiltrate of λ positive plasma cells. In the 2014 biopsy, the picture has not changed significantly. The trephine biopsy from February 2018 shows an IgAλ positive plasma cell population that has expanded, but IgMκ positive B-cell infiltrates can still be seen. In retrospect, therefore, all three biopsies show two clones; and in the 2018 sample, the CAD-associated IgMκ plasma cell population seems “hidden” among the expanding, myeloma-associated IgAλ plasma cells (). |
pmc-6425342-1 | A 64-year-old woman with no past medical history and no prescribed medications was found unresponsive at home after ingestion of 208 tablets of Tylenol PM™ (APAP 500 mg with diphenhydramine 25 mg) approximately three hours prior to transportation to the ED. She was endotracheally intubated by prehospital staff due to decreased level of consciousness, vomitus, and agonal respiration. On arrival to the ED, vital signs included temperature of 33.9°C, heart rate of 57 bpm, blood pressure of 139/102 mmHg, respiratory rate of 19 rpm, and oxygen saturation of 99% on 100% FiO2. Physical examination demonstrated a Glasgow Coma Scale score 3 without spontaneous respiration. The patient subsequently became hypotensive requiring five IV push-dose epinephrine doses (total 100 µg), followed by dopamine (10 µg/kg/min increased to 15 µg/kg/min), and then a norepinephrine infusion (10 µg/min). An electrocardiogram (ECG) showed sinus rhythm of 58 bpm, PR of 144 ms, QRS of 112 ms, and QTc of 659 ms. Serum chemistries measured as follows: lactate 7.6 mmol/L (0.5-1.0 mmol/L), glucose 193 mg/dL (70-100 mg/dL), Na+ 142 mEq/L (136-144 mEq/L), K+ 3.2 mEq/L (3.7-5.2 mEq/L), Cl− 110 mmol/L (96-106 mmol/L), CO2 18 mmol/L (20-29 mmol/L), BUN 16 mg/dL (7-20 mg/dL), Cr 1.17 mg/dL (0.6-1.1 mg/dL), AST 21 IU/L (10-34 IU/L), ALT 99 IU/L (8-37 IU/L), and INR 1.2 (0.8-1.1). Initial ABG measured as follows: pH 7.32 (7.35-7.45), pCO2 30 mmHg (35-45 mmHg), pO2 249 mmHg (80-100 mmHg), and HCO3 16 (CMV FiO2 65%, PEEP 6, RR 16, and TV 500 mL). Initial serum APAP concentration measured 1,017 µg/mL. Serum salicylate and ethanol concentrations measured 7 mg/dL and negative, respectively. A preliminary urine drug screen of abuse detected only methadone. Expanded serum liquid chromatography/mass spectroscopy detected caffeine, dihydrocodeine/hydrocodol, lidocaine, monoethylglycinexylidide, and diphenhydramine. A chest X-ray revealed mild interstitial edema. Head computed tomography was unremarkable. The patient was admitted to the intensive care unit. There was no repeat ECG performed prior to transfer to transplant center; however, no dysrhythmias were observed on telemetry monitoring.
The medical toxicology service was consulted and recommended IV sodium bicarbonate (for prolonged QRS interval), IV NAC, IV 4-MP (15 mg/kg), and immediate HD. APAP concentration decreased to 825 µg/mL after initiation of IV NAC, and serial concentrations exponentially decreased during “triple therapy” (Figures and ). During HD, the IV NAC rate of administration was doubled to 200 mg/kg and then tripled to 300 mg/kg, and a subsequent dose of IV 4-MP 10 mg/kg was administered; these were performed due to concern of HD removal of both antidotes.
The patient's mental status improved during HD; however, she did not follow commands. Due to concern for potential severe liver injury given the massive initial APAP concentration and lack of institutional transplant services, she was transferred to a liver transplant center on hospital day one. On arrival to the liver transplant center, she was maintained on IV NAC and received an additional HD treatment. 4-MP was not readministered. IV NAC was discontinued when APAP concentrations were undetectable. The patient was awake and following commands but failed extubation due to respiratory distress and pneumonia. She was ultimately extubated to BiPAP and discharged to an inpatient psychiatry unit approximately eight days after ED presentation in a normal state of health. |
pmc-6425366-1 | A 7-year-old girl was seen with an esotropia of 6-month duration. According to the parents the esotropia was intermittent at first and became constant after several weeks. Another ophthalmologist prescribed glasses 4 months prior to our first appointment with the patient. With glasses on, she still had esotropia during the morning and early afternoon hours. Her eyes were “straight” after 3:00pm. No other signs or symptoms were present.
Our first examination was done around noon. She presented VA of 20/30 OU with her glasses of +5.50 sph OU. She measured with an esotropia of 50 PD at distance and 60PD at nearness, both with and without her glasses on. No diplopia was elicited. Anterior segment evaluation and dilated fundus exam were normal. Cycloplegic refraction with Cyclopentolate 2% showed +6.00 = +1.00 X 90 OU.
The mother had many photographs on her cell phone, which showed left esotropia in the morning (with glasses) and orthotropia (with glasses) in the afternoon, specifically after 3:00 pm.
Her next appointment was scheduled for late afternoon, at 5:00 pm. She was orthotropic at both distance and nearness with stereopsis of 100” of arc. Cycloplegic refraction with Atropine 1% showed the same refractive error as with Cyclopentolate 2%. This new prescription was dispensed.
Two weeks later, she was seen with the new glasses. She was esotropic at her morning appointment and orthotropic at her late afternoon appointment.
A neurological evaluation was performed which was normal. MRI was normal. Blood work to rule out thyroid disease and ocular myasthenia was also normal.
The diagnosis of cyclic esotropia was made. A 5.5 mm bimedial rectus recession was performed 6 months after her first appointment.
Postoperatively, she was orthotropic all day, with glasses. At 3-, 6-, 9-, and 12-month follow-ups, she was orthotropic. After 2 years, the patient is still orthotropic all day long.
Her prescription has changed to +4.00=+1.00X90 OU. |
pmc-6425372-1 | A 70-year-old woman complained of systemic edema and excessive weight gain. Since she has hypertension and a history of subarachnoid hemorrhage at the age of 50 years, she had taken antihypertensive agents, including amlodipine besylate and candesartan cilexetil. In year X-25, she was diagnosed with SS because of dry eyes confirmed by the Schirmer and Rose Bengal test, mononuclear cell infiltration around the salivary gland, and the presence of anti-SSA antibodies. In July of year X-1, she visited our hospital due to body weight gain of 3 kg in a month, lower leg edema, and dyspnea. Computed tomography (CT) showed thoracoabdominal fluid. She was admitted in September.
Upon admission, she had normal blood pressure of 119/83 mmHg, and oxygen saturation was 97%. She had no cardiac murmurs. Her respiratory sound attenuates in both lower lungs and marked subcutaneous edema in the abdomen and legs was noted.
Laboratory findings revealed elevated Ht level of 45.6%, with lower total protein (TP) (6.1 g/dL) and albumin (ALB) levels (2.9 g/dL) (). Thyroid function was normal. Antinuclear antibody showing a centromeric pattern and anti-SSA antibody were positive. Serum M and urinary Bence Jones proteins were not detected. CT showed moderate pleural effusion and ascites. Echocardiography showed small amount of pericardial effusion, no ventricle expansion with normal tricuspid valve systolic pressure gradient, and normal diameter of the inferior vena cava with respiratory fluctuation, indicating that her cardiac function was normal.
At first, we considered the thoracoabdominal fluid as serositis with SS and started celecoxib 400 mg/day (). However, pleural effusion did not decrease, and body weight still increased. Prednisolone (PSL) 35 mg/day (0.5 mg/kg/day) was started, and body weight gradually decreased; however, difficulty in breathing, subcutaneous edema, and pleural effusion worsened. In addition, hypoalbuminemia worsened, and the Ht level continued to increase. Other causes resembling SCLS, i.e., angioedema, monoclonal gammopathy of undetermined significance, and CTD other than SS, were distinguished by her clinical history, physical, and laboratory findings. We decided to treat the condition as chronic SCLS, administering terbutaline 6 mg/day and theophylline 300 mg/day starting that day. However, they were ineffective, and hypoalbuminemia progressed. Therefore, 35 g/day (0.4 g/kg/day) of IVIG was administered for 5 days from hospital day 31, in addition to 30 mg/day of PSL. After three days, the Ht level decreased and hypoalbuminemia began to improve. Subcutaneous edema and pleural effusion also improved, and she was discharged.
After discharge, the dose of PSL was gradually decreased. However, after decreasing the PSL dose to 2 mg/day in March of year X, abdominal fullness was noted. Subcutaneous edema and pleural effusion redeveloped and worsened. In May of year X, she had difficulty in breathing and was rehospitalized.
Upon admission, her blood pressure was 102/60 mmHg, and severe abdominal edema was observed. Hemoconcentration, hypoalbuminemia, and thoracoabdominal fluid, as in the previous condition, were also present.
She was diagnosed with relapse of chronic SCLS, and 35 g/day (0.4 g/kg/day) of IVIG was started without increasing the PSL dose because her clinical history suggested that only IVIG might be essential. However, severe abdominal edema did not improve even after 5 days of IVIG therapy. We thought that IVIG alone seemed ineffective. Because the difficulty in breathing worsened, PSL dose was increased to 35 mg/day (0.5 mg/kg/day). Subsequently, shortness of breath, abdominal edema, and thoracoabdominal fluid improved. Hypoalbuminemia also improved. She was then successfully discharged. |
pmc-6425374-1 | A 74-year-old woman with a history of SLE on hydroxychloroquine without known renal involvement, chronic kidney disease stage (CKD) IIIa, heart failure with reduced ejection fraction, hypertension, and type 2 diabetes mellitus (T2DM) presented to the emergency department with progressive chest “heaviness” which had started several days prior to admission. In addition, she complained of swelling in both feet and at least three-pound weight gain.
Upon admission she was afebrile, blood pressure ranged 150-180/70-80 mm Hg, heart rate was about 50 bpm, and oxygen saturation was above 95% on room air. She was a well-developed, nonobese woman in no significant distress and nontachypneic. Pertinent physical exam findings included no jugular venous distention, no crackles audible at base of lungs, heart with regular rate and rhythm with no extra sounds or murmurs, nondistended abdomen, trace peripheral leg edema, and no visible rashes. Laboratory tests were significant for sodium 129 mEq/L, potassium 4.2 mEq/L, chloride 98 mEq/L, CO2 20 mEq/L, BUN 46 mg/dL, and creatinine 3.4 mg/dL. Her baseline creatinine based on the most recent reading one month prior to admission was 1.1 mg/dL. Urinalysis was significant for a specific gravity of 1.006, RBC 142, and WBC 8 per HPF. No casts of dysmorphic cells were seen. Proteinuria was 2+, and a random urine protein-to-creatinine ratio was 1.48.
Her creatinine continued to rise rapidly, and by day 6 of hospitalization it was 6.4 mg/dL. Due to these findings, a renal biopsy was performed. Up to 21 glomeruli were present per tissue section, and two of them showed global sclerosis. Two other glomeruli showed segmental sclerosis and the remaining glomeruli were open. There was global diffuse marked mesangial sclerosis and hypercellularity, and there was no obvious endocapillary cell proliferation or inflammatory cell infiltrate. Three of the open glomeruli also displayed segmental or circumferential predominantly cellular crescent. There was multifocal chronic tubulointerstitial injury characterized by atrophic tubules, interstitial fibrosis, and mild mononuclear inflammatory cell infiltrate, all which accounted for about 20-40% of the cortical tissue area. Figures and are biopsies seen in light microscopy with the former showing mesangial proliferation and the latter demonstrating a crescent on silver stain. Immunofluorescent staining showed marked IgA and C3 mesangial deposition, weak linear IgG staining of the glomerular basement membrane, and absence of C4 and C1q which was consistent with IgA nephropathy. There are also changes suggestive of diabetic nephropathy, in keeping with the clinical history of diabetes. These changes include thickened lamina densa and linear IgG of the glomerular basement membrane and mesangial sclerosis. Immunofluorescent staining positive for IgA and C3 is seen in Figures and , respectively. Staining for C4 and C1q was both negative with the latter seen on . In addition, electron microscopy showed uniform thickening of lamina densa of glomerular basement membrane, marked mesangial sclerosis, and hypercellularity, and some electron dense deposits were identified in the mesangial areas and subepithelial location which can be seen in Figures and . Ultimately, given the histological findings, the biopsy was diagnosed as mesangial proliferative glomerulonephritis with crescent formation. Findings were not comparable with lupus nephritis, especially the immunofluorescent findings. The significant mesangial IgA staining raised the likelihood of IgA nephropathy.
Her serologic work-up included anti-dsDNA antibody (1:80), ANA (1:320), and normal serum complement levels. With these biopsy results in the setting of worsening kidney function, she was diagnosed with immune complex RPGN secondary to IgAN.
She subsequently received intravenous methylprednisolone 500 mg daily for three days and five rounds of plasmapheresis. Unfortunately, her renal function continued to deteriorate, and she was started on hemodialysis. She was discharged with instructions to take prednisone 60 mg daily for one month with a taper. Her clinical symptoms showed mild improvement, however, there was no significant recovery in kidney function, and she was declared to have end-stage renal disease (ESRD). |
pmc-6425384-1 | A 28-year-old man sought medical attention reporting that he had been bitten by the snake Bothrops jararaca. He presented only pain and a punctate wound on the lateral aspect of the middle third of the right leg and without other signs (). Because it was not a characteristic snakebite lesion and a thorn sting was suspected, symptomatic treatment was performed. The physician did not valuate the patient report, and he was then discharged with analgesics. The victim returned to the health unit 5 days after the accident, complaining of severe pain, edema, pallor, regional lymphadenopathy, bruising ecchymosis, myonecrosis, and bullous erythema in the right lower limb, specially around the fang marks (). All other clinical data evaluated during the physical examination were normal. At this second admission, white cell count was normal (4.7 × 109/L) but then leukocytosis developed in the second day (14.7 × 109/L) and persisted for approximately 8 days. Creatine kinase levels were very high (3.006 IU × 103/L) at the admission and then begun to decrease during hospital treatment, suggesting recovering of rhabdomyolysis. Thrombocytopaenia (54.300 × 109/L being the lowest registered value); coagulopathy; increase of C-reactive protein (445.28 mg/dL being the highest registered value); sedimentation rate of erythrocyte, γ-glutamyltransferase, and lactate dehydrogenase; and a slight alteration in liver transaminase levels were also registered. Acute kidney injury was not observed; creatinine levels were within the normal limits, and serum urea levels were increased (ranging from 60–110 mg/dL during the first 4 days after admission), suggesting in this case increased protein catabolism caused by skeletal muscle injury. He was then treated with 10 vials of antibothropic serum and then 20 vials of a polyvalent antibothropic laquetic antivenin serum. Nevertheless, he progressed to compartment syndrome and required decompressive fasciotomies, aiming to reduce pressure within the affected compartment in order to prevent irreversible sequelae (). The victim underwent analgesia with opioids and antibiotic therapy first with ampicillin and sulbactam (for 8 days) and subsequently imipenem with cilastatin and vancomycin (for 21 days) according to the ongoing protocol to control nosocomial infections. He was discharged 71 days after the accident. |
pmc-6425386-1 | A 62-year-old female presented for evaluation of recurrent left lower extremity swelling. Her medical history was notable for prior deep vein thrombus in the right distal lower extremity while on hormone replacement therapy (HRT). She denied the active use of HRT and tobacco use during this admission. Venous Doppler ultrasound completed in the emergency room revealed extensive thrombosis of the left lower extremity extending superiorly towards the left common iliac vein. Further imaging with ultrasound revealed compression of the left iliac vein by the right iliac artery as well as a significantly elevated reflux time of the left great saphenous vein (14.2 seconds) suggestive of MTS. The patient was taken to the operating suite and during the procedure the common iliac vein appeared normal distally, but more proximally the vein was narrowed significantly to a diameter of less than 2 mm. Prior to entering the inferior vena cava, the common iliac vein normalized. Using intravenous ultrasound, measurements were taken and a 14 x 60 mm Luminexx stent was deployed at the area of stenosis. The stent was noted to have migrated upward into the inferior vena cava and a buttressing of this stent with a 16 x 40 mm Wallstent was placed to ensure adequate apposition. Unfortunately, this caused further migration upward into the IVC and a 14 mm Atlas balloon was used to help secure the migrated IVC stent. The area of stenosis was no longer stented given this migration. Therefore, stenting of the left common iliac vein stenosis was ultimately achieved with a 14 x 80 mm Luminexx stent (). The patient was started on warfarin with heparin bridging postoperatively. Early ambulation and the routine use of elastic stockings were encouraged following the procedure. The following day the patient complained of severe abdominal pain and an abdominal x-ray revealed only two stents located in the abdomen (). A chest x-ray was obtained and revealed the initial 14 x 60 mm Luminexx stent projecting over the right atrium (). She underwent open-heart surgery for stent retrieval and had a postoperative course complicated by atrial fibrillation and recurrent left sided lower extremity DVT managed with catheter directed thrombolysis. Hypercoagulable work-up revealed homozygosity of the Factor V Leiden gene mutation. One week after discharge, she developed hypotension and lightheadedness. She presented to the emergency department and was found to have pericardial tamponade requiring blood transfusion, pericardiocentesis, and pericardial window. Anticoagulation treatment was stopped during hospital stay and not resumed upon discharge.
Three weeks later, she had a syncopal episode secondary to a massive pulmonary embolus (PE). Imaging also revealed residual DVT in bilateral lower extremities. She underwent thrombolysis with tissue plasminogen activator and subsequently developed a thoracic hematoma. Given the residual clot burden in the bilateral lower extremity, she underwent IVC filter placement and mechanical thrombectomy. No additional stents were placed. Throughout the hospitalization the patient required multiple blood products after developing a hematoma related to recent thoracic surgery. The patient was eventually stabilized and given the Factor V Leiden mutation and life-threatening PE, she was started on rivaroxaban indefinitely. Since these events, she has been followed closely as an outpatient with no known hospitalizations related to bleeding or thrombosis. At 5-year follow-up, the patient reports that she is doing well. She is not experiencing any complications related to rivaroxaban. She does have residual postthrombotic syndrome (CEAP class 3, Villalta Score 8) well managed with daily compression stockings. |
pmc-6425396-1 | A late preterm (36 5/7 weeks) male neonate was born at an outside facility to a 29-year-old, Caucasian, Gravida 5 Para 5 mother needing cesarean section for category II fetal heart rate tracing. Mother had limited prenatal care. Antenatal ultrasound (US) during second trimester was normal. Maternal TORCH, Parvovirus B 19, and Epstein Barr Virus workup was negative. She had no family history of anemia, gall bladder surgery, splenectomy, or hydrops fetalis. All her older children were born healthy except one, who required phototherapy at birth for hyperbilirubinemia.
At birth, the newborn was appropriate for gestational age (birth weight 2600 g) with Apgar score of 4, 6, and 7 at 1, 5, and 10 minutes, respectively. He was intubated for poor respiratory effort and was admitted to neonatal intensive care unit (NICU). On examination, he appeared pale and noticed to have firm, distended abdomen with hepatosplenomegaly and generalized body wall edema. There was no cephalhematoma, subgaleal bleed, or bruising. Cord blood analysis showed severe congenital anemia with hematocrit of 20.3% and packed red blood cells (PRBC) were transfused. Infant's blood group was A+ with direct coombs 2+ for anti-IgG with anti-E found on elution. Mother's blood group was A+, but antibody screen was unknown at birth hospital.
Infant was placed on a mechanical ventilator and initial chest radiograph revealed cardiomegaly. Fresh frozen plasma and platelets were also transfused for active bleeding from the umbilical stump. Diagnosis of hydrops fetalis was confirmed by echocardiogram showing a structurally normal heart with small pericardial effusion, ascites on abdominal US, and generalized edema on exam.
Laboratory workup showed white blood cell count 10,400/ml, hemoglobin 9.1 g/dl, and hematocrit 25.1%; reticulocyte count was 18%, lactate dehydrogenase (LDH)>4000 u/l, and platelets 154,000/ml; and peripheral smear showed numerous nucleated red blood cells with moderate schistocytes, target cell, and few burr cells. Total bilirubin at 3 hours of life was 4.6 mg/dl.
Infant was transferred to our level IV NICU for further management due to hypoxemic respiratory failure. He continued to have labile oxygen saturation despite being on 100% oxygen, for which inhaled nitric oxide (iNO) was started at 20ppm and repeat echocardiogram showed features of persistent pulmonary hypertension of newborn (PPHN) including suprasystemic right ventricular pressure with severe tricuspid valve insufficiency. He became hypotensive requiring multiple vasopressor support.
Partial exchange transfusion was deferred due to hemodynamic instability and PRBC were transfused in small aliquots for severe anemia. At 12 hours of life, he was started on phototherapy for total bilirubin of 13 mg/dl.
Although anti-E hemolytic disease rarely leads to severe hydrops, consultant hematologist agreed with the suspected diagnosis due to evidence of high reticulocyte count (18.1%), LDH >4000 mg/dL, numerous nucleated red blood cells, and normoblasts on peripheral smear (transfused specimen) along with positive direct coombs test findings. Maternal blood group antibody screen and placental pathology remained unavailable from the birth hospital. Cord blood ferritin was normal ruling out chronic fetomaternal blood loss. Alpha thalassemia was ruled out as initial mean corpuscular volume was high and there was no evidence of many target cells on peripheral smear. Congenital aplastic or dyserythropoietic anemia was ruled out due to initial high reticulocyte count. Infectious etiology was ruled out due to negative herpes simplex virus 1 and 2 polymerase chain reaction (PCR), cytomegalovirus (CMV) PCR, urine CMV culture, and parvovirus PCR. Baby was seropositive for Epstein Barr Virus, possibly from maternal antibody.
He continued to remain hypoxemic despite maximal ventilatory support and inhaled nitric oxide, so eventually placed on extracorporeal membrane oxygenation (ECMO). Head US prior to initiating ECMO was normal. Repeat head US on day 2 of life while on ECMO showed development of large left intracranial hemorrhage (ICH) with midline shift. ECMO was promptly discontinued and the patient was placed back on mechanical ventilation. Parents were counselled about poor prognosis in view of hypoxemic respiratory failure and large ICH. Parents agreed for comfort measures. Soon after withdrawal of life support, the patient passed away. Autopsy report showed extensive extramedullary hematopoiesis within multiple organs including liver and spleen. |
pmc-6425407-1 | A 78-year-old female with follicular lymphoma came to the hematology-oncology department for a routine checkup in May 2016. She had been suffering from intermittent fevers, tiredness, significant weight loss, and night sweats for several weeks. She was treated repeatedly with antibiotics with no clinical effect. Up to that point, the patient was still working and was an active woman with no other diseases or chronic medication.
Initially, the patient was diagnosed with a follicular lymphoma (FL) of stage IIIA in 1999 and underwent standard treatment consisting of 6 cycles of CHOP with the achievement of a complete remission. In 2003, the patient developed asymptomatic, low-burden, histology-proven relapse of indolent FL, but therapy was only started in October 2004, when bulky disease and symptoms were revealed. The patient was treated with 6 cycles of R-COPP immunochemotherapy, resulting in a complete remission.
Since 2015, PET/CT had shown mild lymphadenopathy (up to 25 × 15 mm; SUVmax up to 5.79), but the patient remained asymptomatic and no treatment was administered. In May 2016, the progression of cervical lymphadenopathy and systemic symptoms occurred, accompanied by a significant elevation of the liver enzymes, LDH, and CRP (ALT 6.01 μkat/l, AST 5.96 μkat/l, ALP 22.69 μkat/l, GGT 20.40 μkat/l, LDH 8.19 μkat/l, and CRP 27.6 mg/l). However, lymph biopsy showed no evidence of malignant cells; only reactive inflammatory infiltration with central coliquation was found. The patient's condition was deteriorating rapidly with continuous fevers, and she developed ascites. Serological and PCR tests (CMV, EBV, hepatitis A, B, C, HIV, HHV-8, chlamydia, and mycoplasma) gave negative results. The bone marrow and paracentesis were free of pathology. Flow cytometry identified an elevated count of T-lymphocytes and a nearly zero level of B-lymphocytes concordant with deep hypogammaglobulinemia (IgG < 0.4 g/l, IgA < 0.05 g/l, and IgM = 0.09 g/l). Thorough immunological analysis revealed a significant elevation of CD8+ cells in the blood up to 57% (normal 39%), mildly elevated CD3+ cells; conversely, the population of CD19+ was almost undetectable. The examination of the liver, portal system, and biliary tract by CT and ultrasound showed no pathology.
In conclusion, based on a finding of severe B-lymphocytopenia, agammaglobulinemia, and unusual CD8+ cell population, we assumed that our patient suffered from a rare CD8+ lymphoproliferation. We performed additional special (not routinely performed) staining of bone marrow and lymph node specimens targeted at the CD8+ cells, where an evident clonal CD8+ cell population was found. Corresponding T-cell clonality in the peripheral blood and bone marrow was also confirmed.
There is currently no standard treatment available for this disorder. Based on published case reports, we administered a combination of intravenous immunoglobulin (IVIG 0.3 g/kg—once every three weeks) and corticosteroids (initial dosage of prednisone 1 mg/kg/day) []. Within four weeks, the patient's condition had improved significantly, lymphadenopathy diminished, ascites and systemic symptoms disappeared, and liver enzymes decreased (ALT 1.71 μkat/l, AST 1.32 μkat/l, ALP 7.32 μkat/l, and GGT 11.41 μkat/l). Thus, we could slowly reduce the dosage of corticosteroids. Since April 2017, the patient has been asymptomatic with laboratory tests within the norm and now is on a small dose of prednisone (5 mg/day). Up to now, the patient is well without symptoms of lymphoma or CD8+ lymphoproliferation, but regular IVIG substitution is necessary. |
pmc-6425584-1 | An 8-year-old girl, who had a laparoscopic appendectomy for gangrenous appendicitis at another hospital, presented with postoperative persisting non-bilious vomiting and progressive abdominal distension initially diagnosed as paralytic ileus and treated with conservative management. Despite this, her clinical condition did not improve; she passed spontaneously flatus, but she couldn’t have bowel movements without administration of enemas. Laboratory findings were within normal range. Ten days later, as the clinical conditions failed to improve, she was transferred to our hospital.
On physical examination at admission, the abdomen was distended and tympanitic to percussion, but soft with no tenderness, rebound or guarding. Bowel sounds were present. Laboratory findings were within normal range and no free fluid or collections were found at the US scan.
The abdominal x-ray showed marked colonic gaseous dilatation, without evidence of mechanical obstruction. Gut decontamination with oral ciprofloxacin and metronidazole was started and oral intake was progressively resumed.
The symptoms improved temporarily, but, unfortunately, they recurred 4 days after admission with greater severity in presence of bilious vomiting. The abdominal x-ray showed worsening colonic dilation (Fig. a) and rectal stool impaction. Because of the diminished bowel sounds, an abdominal computed tomography scan was performed and confirmed the severe colonic dilatation form the cecum to the splenic flexure in absence of colonic mechanical obstruction (Fig. b). A nasogastric tube and a rectal tube were inserted to put bowel at rest, parenteral nutrition was started, the patient was kept on nil by mouth and a combination of stool softeners and enemas were administered. The clinical and radiological findings suggested the diagnosis of Ogilvie’s syndrome. Erythromicyn (250 mg two times a day) through the nasogastric tube was then started followed by an improvement in clinical symptoms. The medication was stopped after seven days. The patient made a full recovery, resuming her normal diet and spontaneous bowel movements, and was discharged in good conditions 25 days after the surgery. At the follow up appointment one week after discharge she was well and completely recovered. |
pmc-6425598-1 | The patient was a 71-year-old female with a history of progressive cerebellar ataxia. She first experienced the onset of her current illness nine years ago, when she developed unsteady gait with difficulty performing a tandem walk.
She was initially treated for suspected inner ear problems, but approximately two years ago, magnetic resonance imaging (MRI) revealed cerebellar atrophy, which has led to her current diagnosis. Neuro-ophthalmologic evaluation indicated ophthalmoplegia, requiring her to wear prisms lenses to assist with reading.
To date, her symptoms have gradually progressed with notable worsening over the past year, and include balance disorder, increased risk of falling, reduced manual dexterity, fatigue, and episodes of speech slurring. She uses a cane for ambulation with difficulty turning and moving between a standing to seated position. She tried several pharmacological treatments over the years without any lasting clinical benefits. She completes daily-prescribed physical rehabilitation training exercises at home. |
pmc-6425646-1 | A 46-year-old man weighting 176 kg with BMI 53.7 kg/m2 was scheduled for gastric volume reduction surgery to reduce body weight under general anesthesia. The patient was diagnosed of obstructive sleep apnea (OSA) 3 years ago, without treatment. Airway inspection showed short neck with circumference of 51 cm, limited neck extension due to its thick fat and the Mallampatti score-Ш. The patient felt tired preoperatively because of his sleep deprivation secondary to OSA. He was very nervous and refused to consent for awake fiberoptic intubation under sedation.
We elected to perform tracheal intubation after anesthesia induction but keeping patient’s spontaneous breathing to avoid hypoxia, with initial direct laryngoscopy using video laryngoscope, and back up with fiberoptic intubation and then laryngeal mask airway (LMA). Bispectral index (BIS) was used to monitor anesthesia depth.
Vital signs showed Bp 142/79 mmHg, HR 88 bpm, SpO2 96%, RR 22 bpm before anesthesia induction. Midazolam 3 mg and sufentanil 10 μg was given intravenously to reach BIS at 62 for sedation. Thereafter, intravenous 100 mg propofol was given and BIS fell to 51. Mask pressurized ventilation could be performed to maintain SpO2 100% with patient under continuous target controlled infusion (TCI) at propofol 3μg/mL.Direct laryngoscope with video laryngoscope was tried twice but failed because of the invisible glottis obstructed by Huge epiglottis (Grace IIb view). SpO2 fell to 75% at the end of second laryngoscopy. Two hand pressurized mask ventilation was initiated and became difficult, although SpO2 could be maintained around 88% with following vital signs: BP 133/73 mmHg, HR 86 bpm, normal sinus rhythm, BIS 57.We then placed WEI Nasal Jet Tube (WNJ), (Well Lead Medical Equipment Ltd., Guangzhou, China. Production batch number: 20140901) (Fig. ) in the patient’s right nostril to provide SJOV. The jet catheter of the WNJ was connected to an automatical jet ventilator-TKR-400 (Well Lead Medical Equipment Ltd.,Guangzhou, China.) with following working parameters: driving pressure (DP) 35 psi, respiratory rate (RR): 55 bpm, I/E ratio 1:3.SpO2 began to rise again and reached 100% at 1 min after initiation of SJOV. Thoracic cage moved ups and downs during SJOV indication of both oxygenation and ventilation. We then administered intravenous rocuranium 60 mg, TCI propofol 4μg/mL and controlled ventilation was achieved using SJOV. Fiberoptic bronchoscopy guided endotracheal intubation was performed via mouth approach. Fiberoptic intubation was difficult due to hypertrophy of the patient’s tongue and epiglottis but eventually succeded 5 min later. The vital signs at the end of successful intubation were as followings: SpO2 100%,BP 125/64 mmHg, HR 71 bpm, sinus rhythm, BIS 45.PetCO2 was not monitored during fiberoptic intubation due to the both Ports being not consistent and hurry to raise oxygenation. However,instant blood gas analysis showed pH 7.36, PaO2 124 mmHg and PaCO2 49 mmHg. Total time of SJOV via WNJ was about 7 min. No obvious barotrauma, nose bleeding etc.,was seen at the end of intubation.
The operation was successfully completed after 3 h. The patient was transferred to the ICU and was extubated in ICU without event. Patient recovered smoothly in hospital for 8 days and did not have any recall inside the operating room. |
pmc-6425683-1 | In June, a 68-year-old Chinese woman with severe hypertension and renal dysfunction was admitted to our hospital for further treatment. The patient had no remarkable medical history or family history. The patient had noticed puffy swollen fingers and leg edema 1 year previously, as well as the gradual development of skin thickening and Raynaud phenomenon. She had been diagnosed with SSc by a local doctor and had been prescribed PSL 20 mg/day 25 days before admission. At 11 days before the current admission, the patient had visited the previous hospital with acute-onset chest and back pain with blood pressure 101/51 mmHg and pulse rate 111 beats/min, and contrast-enhanced computed tomography (CT) showed massive intraperitoneal bleeding (Fig. ). At this time, contrast-enhanced CT showed normal findings of renal arteries, and laboratory findings showed serum creatinine (Cre) 1.1 mg/dl and blood urea nitrogen (BUN) 37.4 mg/dl. The patient accordingly underwent emergency laparotomy, and the bleeding from the gastroepiploic artery was stopped surgically. After surgery, she developed severe hypertension (260/129 mmHg) and progressive renal dysfunction (Cre, 6.4 mg/dl; BUN, 125.3 mg/dl). She was transferred to our hospital for further treatment.
On admission, physical examination showed bilateral lower limb edema, trismus, nail fold telangiectasia, and diffuse skin thickening. The patient’s modified Rodnan total skin thickness score was 33 points. No lung lesions were detected on chest CT. Laboratory findings (Table ) showed normocytic, normochromic anemia and severe renal dysfunction with elevated levels of plasma renin activity at ≥ 20 ng/ml/h (normal, 0.3–2.9 ng/ml/h) and plasma aldosterone concentration 433 pg/ml (normal, 35.7–240 pg/ml). Immunological tests revealed positive antinuclear antibody and anti-RNA polymerase III antibody. Echocardiography showed mild left ventricular hypertrophy, a small amount of pericardial effusion, preserved ejection fraction of 80%, and no pulmonary hypertension. Renal artery ultrasound examination showed no evidence of renal artery stenosis.
The patient was diagnosed with SSc and developed severe hypertension and progressive renal dysfunction with proteinuria and hematuria, thus fulfilling the criteria for SRC [, , , ]. The patient also had several risk factors for SRC (duration of disease < 4 years, diffuse cutaneous form, administration of PSL ≥ 15 mg/day, and anti-RNA polymerase III antibody) [–]. No obvious thrombocytopenia, schistocytes, or elevation of direct bilirubin was present; hence, we had little evidence to diagnosis the patient with complications of thrombotic microangiopathy.
The pathological examination of excised gastroepiploic artery at the previous hospital showed the dissection at the media–adventitia junction, and the dissected cavity was filled with hematoma, indicating gastroepiploic artery aneurysm (Fig. a–c). In addition, multiple vacuoles were revealed in the medial muscle layer (Fig. d), which was characteristic of SAM [, ]. We diagnosed a gastroepiploic artery aneurysm caused by SAM, with intraperitoneal bleeding due to rupture of the aneurysm. This patient was therefore confirmed as having a very rare case of coexisting SSc with SRC and SAM.
From the first day of admission, she received the angiotensin-converting enzyme inhibitor (ACE-I) enalapril at 5 mg/day, and maintenance hemodialysis was started. PSL was gradually reduced to 2.5 mg/day because of its increased risk of SRC. Her plasma aldosterone concentration decreased gradually (to 75.6 pg/ml on hospital day 45); however, the control of blood pressure was insufficient, and use of a combination of multiple antihypertensive drugs was required. Eventually, maintenance antihypertensive therapy included enalapril 5 mg/day, olmesartan 40 mg/day, and nifedipine 80 mg/day. Despite these treatments, her renal dysfunction did not improve at all. She was discharged on hospital day 57 and continued maintenance hemodialysis and blood pressure control thereafter. At the 1-year follow-up visit, she remained on dialysis, and her blood pressure was well-controlled with enalapril 2.5 mg/day and nifedipine 10 mg/day. |
pmc-6425685-1 | A 60-year-old man consulted a previous physician complaining of upper abdominal pain. Although he was prescribed H2 blocker, his symptoms did not improve, and esophagogastroduodenoscopy (EGD) was performed. EGD revealed a type 2 lesion in the posterior wall of the lower body of the stomach (Fig. a), and the biopsy indicated carcinoma. He was referred to our hospital for detailed examination.
On admission, abnormal symptoms, such as fever, anemia, and jaundice, were not observed, and his performance status was good (Eastern Cooperative Oncology Group score of 0). He had hypertension and hyperuricemia and was taking medication. He had a history of eradication of Helicobacter Pylori (HP). He had no history of smoking, and had no family histories of malignant diseases, either. Laboratory data on admission showed no remarkable findings, and tumor marker levels (carcinoembryonic antigen and carbohydrate antigen 19–9) were also not increased. The EGD and biopsy specimens were re-examined, and they revealed poorly differentiated adenocarcinoma. Contrast-enhanced computed tomography (CT) from the neck to the bottom of the pelvic floor indicated swelling of the perigastric lymph node (Fig. b) but showed no other distant metastasis.
Based on these findings, the patient was diagnosed with cT2N1M0, cStage IIA gastric adenocarcinoma (according to the Union for International Cancer Control (UICC) TNM classification of malignant tumors, 8th edition).
Distal gastrectomy with D2 lymph node dissection was performed. The primary lesion was 4 × 4 cm in size and extended into the subserosal layer. Thirty-eight lymph nodes were harvested, and there were 14 metastatic lymph nodes. Stations of metastatic lymph nodes were nos. 3a, 3b, 4d, 6, and 7. Although the preoperative biopsy suggested adenocarcinoma, histopathological examination of the resected specimen indicated SCC, and it was diagnosed as pT3N3aM0, pStage IIIB (according to the UICC TNM classification of malignant tumors, 8th edition). Hematoxylin and eosin (HE) staining of the tumor specimen showed that the tumor cells had hyperchromatic nuclei and an abundant amount of eosinophilic cytoplasm, and proliferated in a sheet-like structure with solid nests. We also detected intercellular bridges in some sections (Fig. ). HE staining of the metastatic lymph nodes showed findings similar to those of the primary tumor. Atrophic change with intestinal metaplasia was observed in the mucous membrane around the tumor, and former infection of HP was suggested. And the tumor specimen did not show the pattern like lymphoid stroma, and infection of Epstein-Barr virus (EBV) was not suggested. Immunohistochemistry of the resected tissue specimen was positive for pan-cytokeratin (AE1/AE3), cytokeratin (CK) 5/6, and caudal-type homeobox protein 2 (CDX2), slightly positive for CK20, and negative for p63, p40, synaptophysin, α-fetoprotein, and CK7 (Fig. ).
He was discharged without any complications 9 days after the operation. He received adjuvant chemotherapy with S-1 (100 mg/body/day of S-1 on days 1–28, every 6 weeks). After 5 cycles of S-1 therapy, a follow-up CT scan showed multiple metastases in the liver and around the duodenal stump. Then, he received two courses of CapeOX therapy (3000 mg/body/day of capecitabine on days 1–14 and 130 mg/m2 of oxaliplatin on day 1, every 3 weeks), but failed because of skin trouble. Furthermore, he received three courses of weekly paclitaxel and ramucirumab-combined therapy (80 mg/m2 of paclitaxel on days 1, 8, and 15 and 8 mg/kg of ramucirumab on days 1 and 15, every 4 weeks), but failed because of the tumor progression. At this time, CT showed worsening of the liver and duodenal stump metastases and the new lesion of multiple subcutaneous and distant lymph node metastases, and he noticed melena. We selected chemotherapy regimens according to that of the Japanese Gastric Cancer Treatment Guidelines but could not obtain desirable results. So we selected docetaxel, cisplatin, and 5-fluorouracil (DCF) therapy (60 mg/m2 of docetaxel on day 1, 60 mg/m2 of cisplatin on day 1, and 700 mg/m2 of 5-fluorouracil on days 1–5, every 4 weeks), according to the Japanese regimen of esophageal SCC. After two courses of DCF therapy, CT showed a significant reduction of metastatic lesions and his complaint of subcutaneous nodules and melena disappeared, and got efficacy evaluation of partial response (PR), according to the Response Evaluation Criteria in Solid Tumors (RECIST). After five courses of DCF therapy, his tumor was getting worse; hence, best supporting care (BSC) was selected. He died 1 year and 5 months after the operation. |
pmc-6425689-1 | A 57-year-old man (weight, 89.3 kg; height, 175 cm; body mass index, 29.16 kg/m2) initially presented to our outpatient clinic with complaints of lateral knee pain. Radiographs revealed lateral compartment osteoarthritis and valgus deformity of the knee joint. He underwent a closing wedge DFO (Fig. ). The target alignment was adjusted to the contralateral limb, and biplanar osteotomy was performed. Fixation was performed using a locking plate (Ohtofix®, DFO Plate, Hwaseong, South Korea). A bicortical lag screw was inserted initially at the combination hole for indirect reduction of the shaft. Subsequent self-tapping locking screws were inserted using a power driver after predrilling the screw holes and measuring with a depth gauge. Final manual tightening of the locking screws were performed in sequence with a torque-limiting screw driver. The rehabilitation protocol was routine, and tolerable weight bearing with crutches was performed after postoperative 1 week.
During follow up, he complained of an unusual painful swelling at the operated area. Aspiration was performed at postoperative 2 weeks, and 50 cc of blood was aspirated. At postoperative 1 month, he visited the emergency department because of recurrent painful swelling. More than 50 cc of joint fluid mixed with blood was aspirated. Radiograph revealed mild bone resorption at the osteotomy site. No signs of infection were found. Our laboratory results showed normal C-reactive protein (CRP) level. We encouraged him to perform partial weight bearing, and applied compressive dressing. At postoperative 3 months, he revisited our outpatient clinic on a wheelchair with similar symptoms. CRP level was normal and he was afebrile throughout the follow-up period. Radiograph revealed four broken distal fixation screws, with one screw pulled out and positioned within the knee joint. Computed Tomography (CT) revealed a widened gap and fractured lateral hinge (Fig. ).
Re-operation was performed. The distal screws were removed first. Among the four screws, the head portions of the three screws were removed, however one screw could not be found at the previous surgical site. The tip portions of the four screws were removed using a coring reamer and the remaining hardware was removed. The osteotomy site had minimal stability. At the anterior portion of the osteotomy site, the patellofemoral joint had a connection with the operated site. The pulled-out portion of the distal screw was assumed to have moved into the joint via this path, further strengthening the reason why joint fluid was aspirated from an extra articular site. The connection was closed and arthroscopy was performed to remove the migrated screw (Fig. ). Two cannulated lag screws were inserted between proximal-lateral and distal-medial fragments for compression of the lateral hinge. Subsequently a locking plate with a larger profile was reinserted for a more stable fixation (Fig. ). After the re-operation, his symptoms improved and the swelling disappeared. He was satisfied with the outcome of the re-operation and union was achieved at postoperative 6 months. Informed consent was obtained from the patient for all procedures. |
pmc-6426013-1 | An 85-year-old man was referred to our hospital for the treatment of left lower extremity oedema. His past medical history included localized prostate cancer and cardiogenic cerebral infarction caused by AF. He was taking warfarin (1.5 mg daily) at the time. His prothrombin time-international normalized ratio (PT-INR) was checked monthly by his primary care physician. It was almost always between 2 and 3 over the past 2 years. The most recent PT-INR, approximately 2 months before admission, was 2.66. Three years ago, after prostate cancer was diagnosed, an anti-androgenic agent and a luteinising hormone-releasing hormone agonist were started. Prostate cancer was in remission with these medicines. He had no other risk factors for VTE.
On admission, he was afebrile, heart rate was 96 b.p.m., blood pressure was 170/104 mmHg, and respiratory rate was 24 b.p.m. His oxygen saturation was 96% on room air. There was no obvious jugular venous distention or audible murmurs. The lung fields were clear. His left lower extremity was erythematous and swollen.
Laboratory tests revealed high levels of inflammation, as evidenced by the high white blood cell count, 11 100/μL (reference range 3500–8000/μL); C-reactive protein level, 7.55 mg/dL (<0.2 mg/dL); and d-dimer level, 37.0 μg/dL (<1.0 μg/dL). He had normal antinuclear antibody titres. Lupus anticoagulant, anticardiolipin IgG antibodies, and anti-β2-glycoprotein titres were negative. His serum creatinine level was 0.86 mg/dL (0.5–1.2 mg/dL) and his creatinine clearance was 62 mL/min (70–130 mL/min). At presentation, PT-INR was 3.75 (0.9–1.1). Electrocardiography showed AF and inverted T waves in V1 and V2. Computed tomography (CT) with contrast revealed intraluminal filling defects in the LAA, right pulmonary artery, and from the left superficial femoral vein (SFV) to the left popliteal vein (Figure ).
Anticoagulation was switched from warfarin to unfractionated heparin (UFH). The target activated partial thromboplastin time was 60–80 s (25–40 s). We administered UFH for 1 week, with no changes in symptoms. No obvious decrease in the size of the thrombi was observed on follow-up CT. Given the patient’s refractory symptoms, refusal to undergo catheter-directed thrombolysis, and no absolute contraindications to urokinase, we decided to perform systemic thrombolysis (360 000 to 540 000 units/day of urokinase) for 1 week to improve his acute lower extremity symptoms and prevent post-thrombotic syndrome. He was then switched to a DOAC after complete symptom resolution and ultrasonography showed no lower extremity thrombi. The direct factor Xa inhibitor apixaban was started at 5 mg twice daily. Enhanced CT approximately 1 month after hospital discharge showed complete resolution of the LAA thrombus, deep vein thrombosis (DVT), and pulmonary embolism (Figure ).
During 12 months of follow-up, the patient was doing well with significant improvement in his quality of life. Venous thromboembolism and LAA thrombus were not detected by enhanced CT at 12 months. |
pmc-6426033-1 | A 74-year-old female with a history of hypertension and mild to moderate valvular aortic stenosis (AS) (mean gradient 20 mmHg) suffered cardiac arrest due to ventricular fibrillation (VF) at a restaurant. Bystander cardio pulmonary resuscitation was initiated immediately, and after two defibrillations she was cardioverted to atrial fibrillation. Return of spontaneous circulation was achieved after 10 min of CPR. The patient was intubated on site and transferred to the nearest primary coronary intervention centre. During transport she showed signs of awakening and was sedated with propofol. Adrenaline infusion was titrated to a mean arterial pressure (MAP) >70 mmHg.
The coronary angiogram showed severe proximal left anterior descending (LAD) artery stenosis. Complete revascularization was achieved after implantation of a single drug-eluting stent in the LAD artery. Troponin T values were only mildly elevated (257 ng/L) and the electrocardiogram without ST-segment elevation. The bedside transthoracic echocardiogram showed a hyperdynamic and hypertrophic left ventricle with ejection fraction of 50–60%. There were signs of LVOTO, SAM, and moderate MR, but no aortic regurgitation (AR).
The patient was transferred to the intensive care unit (ICU) for haemodynamic stabilization. Targeted temperature management was omitted due to haemodynamic instability and because the patient had been partly awake.
Within 6 h her haemodynamics deteriorated further with tachycardia 110–130 b.p.m., lactic acidosis (9.2 mmol/L), increasing demand for vasopressor therapy (norepinephrine: 0.7 μg/kg/min) to maintain MAP >60 mmHg and concurrent severe pulmonary oedema with a fall in peripheral oxygenation saturation to 60% despite 100% oxygenation supply and titration of positive end expiratory pressure settings of the mechanical ventilator. Efforts to increase pre- and afterload by volume and additional vasopressor therapy (vasopressin 1.0 U/kg/d) only worsened the degree of congestion.
Repeated transthoracic and transoesophageal echocardiograms revealed progression of LVOTO and SAM resulting in severe MR and pulmonary oedema (Figure ; , Videos –).
After a multidisciplinary team conference, it was decided to attempt to establish mechanical circulatory support with femoro-femoral veno-arterial extracorporeal membrane oxygenation (VA-ECMO) for oxygenation and restoration of tissue perfusion. Right femoral vein and artery was cannulated via Seldinger’s technique and VA-ECMO established using a Cardiohelp ECMO system. A 21 Fr venous catheter was placed in the right atrium guided by transoesophageal echocardiography for venous drainage. A 17 Fr arterial cannula was placed in the femoral artery and advanced for return of oxygenated blood. Veno-arterial extracorporeal membrane oxygenation blood flow was set at 4.0 L/min. This led to immediate improvement in haemodynamics and resolved SAM.
Lactate was normalized within 24 h and oxygenation improved. Demand for vasopressor therapy was minimized. On Day 4, haemodynamics had been restored. The echocardiogram revealed cessation of MR severity to mild and after VA-ECMO flow had been reduced to 1.5 L/min it was removed. After 5 days sedation weaning and extubation was achieved without complications despite intermittent use of low-dose norepinephrine to maintain MAP >70 mmHg.
A Day 13, the patient was discharged from the ICU to the coronary care unit. She was neurologically intact without echocardiographic signs of LVOTO or SAM. Overall, the patient was hospitalized for 16 days. She was discharged with beta-blocking agents after implantation of secondary prophylactic implantable cardioverter-defibrillator (ICD) to ambulatory cardiac rehabilitation. At 6 months of follow-up, the patient was in high spirit and fully asymptomatic. Transthoracic echocardiogram demonstrated normal ejection fraction, mild septal hypertrophy, and unchanged mild AS and discrete AR. No Doppler signs of MR.
Pressure gradient in LVOT at rest was not elevated. However during Valsalva manoeuvre the gradient in the LVOT increased to 42 mmHg, suggesting a latent LVOTO. The patient gave informed consent and all identifiable information has been removed. |
pmc-6426513-1 | A 38-year-old pregnant woman [28+1 weeks of twin pregnancy, gravida 3, para 2 (G3P2)] was transferred from a local hospital to our emergency room on March 28, 2018 due to complaints of persistent productive cough, with increased expectoration, dyspnea, dizziness, fatigue, and irregular abdominal pain. The admission diagnoses were as follows:
suspected pulmonary infection and heart failure;
single chorionic twin pregnancy;
moderate anemia;
pregnancy with severe rachiterata accompanied by a huge dorsal mass; and
G3P2 28 + 1 weeks of intrauterine pregnancy of twin live fetus.
The patient has been suffering from severe congenital scoliosis and kyphosis deformity with a huge dorsal mass, with ulcers repeatedly appearing in the surface of the dorsal mass for more than 10 years. She had a history of 2 deliveries. The first induction in 2008 was an 8-month-old stillbirth. In 2011, she delivered a premature infant who fortunately survived. As she and her husband wanted a male child, they risked another pregnancy.
The last menstrual period before the third pregnancy was unknown by the patient, but it was assumed that the patient was approximately 3-months pregnant during the admission. The patient had not had prenatal care since the beginning of pregnancy. She did not have any discomforts during the earlier weeks of pregnancy. However, she started having persistent cough without sputum production on the 24+ weeks of pregnancy, which continued to worsen. She had 2-week treatment in a local hospital prior to her admission in our institution.
Upon admission, the patient's vital signs were as follows: temperature of 36.5°C, heart rate of 107 beats per minute (bpm), respiratory rate of 35 breaths per minute, blood pressure of 107/67 mmHg, and blood oxygen saturation of 92%. Her weight and height were 46 kg and 133 cm, respectively. She was conscious and half-lying on her left side in a decubitus position owing to a huge dorsal mass (Fig. ). Her head was slightly advanced forward. She had a barrel-shaped chest, showing obvious shortening in length. The right shoulder was slightly higher than the left one. The outline of the right chest was larger than that of the left, and the tri-retraction sign was visible upon inhalation. Auscultation of both lungs revealed coarse rales, especially obvious at the lower portion of the right lung. A huge brown soft mass was found covering almost the whole back of the patient, with a size of approximately 26 × 22 × 8 cm and an ulcerative area (10 × 4 cm) at the center. No obvious abnormalities were found in other systems as well as the abdomen and pelvis.
At admission, the laboratory examination results were as follows: white blood cell (WBC), 15.9 × 109/L; neutrophil percentage (N%), 86.3%; hemoglobin concentration (HGB), 76 g/l; C-reactive protein (CRP), 67.0 mg/l; and PCT (calcitonin), 11.96 ng/ml. Mixed bacterium (Streptococcus, a small amount of yeast-like fungus, and Neisseria) were found in the sputum culture.
The initial diagnosis of heart failure was excluded because the electrocardiogram (ECG) showed sinus tachycardia, and the left ventricular ejection fraction was 0.69%, measured by color Doppler echocardiography, which indicated that the diastolic and systolic pressures of the left ventricle were normal. The myocardial marker, B-type natriuretic peptide (BNP), was normal as well.
Chest radiographs showed bilateral thoracic asymmetry (Fig. ), spinal protrusion with lateral bending deformity, kyphosis with “S”-type distortion, partial fusion of the left rib, consolidation of the lower portions of both lungs, and bilateral pleural effusion, which may have been caused by the pulmonary infection.
The pathogeny of the large dorsal mass could not be identified; however, the possibility of it being either a hemangioma or a neurofibroma cannot be excluded. Nevertheless, Staphylococcus aureus was cultured from the exudate of the local skin rupture.
B ultrasound showed the following: fetal 1 (left): heart rate: 154 bpm, left occipitoanterior (LOA), biparietal diameter of 6.44 cm, and femur length of 4.76 cm; and fetal 2 (right): heart rate of 148 bpm, right sacrum anterior (RSA), biparietal diameter of 6.41 cm, and femur length of 4.66 cm. Fibrous membrane echoes were found between the twins.
Before delivery, we had carried out a series of active symptomatic treatments and intensive monitoring for both the patient and her fetuses. For the mother, we tried to control the lung infection by administering third-generation cephalosporin and transfusing erythrocyte suspension to address her anemia. Daily nursing and treatment of surface ulcers of the dorsal mass were implemented as well. For the fetuses, in addition to fetal electronic monitoring every day, the patient had intramuscular injections of corticosteroid to promote fetal lung maturation.
Two weeks later, the patient's condition worsened. She was weak, anorexic, and in a low mood. She was also dyspneic and had persistent cough with thick sputum, which is difficult to expectorate. After multidisciplinary consultation, we decided to induce labor as soon as possible, in consideration of the critical situation of the patient, to ensure safety of the mother and fetuses.
The Cook Cervical Ripening Balloon (Fig. A) was used for mechanical dilation of the cervical canal prior to labor induction at term because the cervix was unfavorable for induction. On April 11, 2018 at 14:28 pm, owing to the complete dilation of the cervical, the patient was transferred to the delivery room. Given that she was dyspneic and had spinal deformity accompanied with a huge dorsal mass, she was placed on a right side-lying position while the upper body was elevated 45° to help facilitate the delivery process (Fig. B). At 15:00 pm, transvaginal examination and palpation revealed that fetal 1 had a head presentation, while fetus 2 had breech presentation. Moreover, the foot of fetus 2 was found on the right side of fetus 1's head. Considering that they come from 2 different chorionic cavities, our experienced midwife immediately prepared the patient for delivery. As soon as the rupture of membrane of fetus 1 occurred, the midwife pushed the foot of fetus 2 to the upper right until the foot could not be palpable using her right index and middle fingers, while simultaneously assisting the head of fetus 1 to descend using her ring and little fingers (Fig. C). Fetus 1 was successfully delivered at 16:15 pm, and fetus 2, who had breech presentation, was born at 16:19 pm via transvaginal-assisted delivery (Fig. D and E). The Apgar scores were 6-9-9 for both 2 premature infants. After tracheal intubation, the newborns were transferred to the neonatal intensive care unit immediately (Fig. F and G)
The mother's vital signs postpartum were stable. Interestingly, at 24 hours after delivery, the mother's dyspnea and coughing episodes gradually improved. One week later, the patient's temperature was normal, and the uterus contracted well with less vaginal bleeding. Then, she gradually recovered and was discharged soon enough.
The 2 neonates survived after treatment and were discharged from the hospital 1 month later. |
pmc-6426533-1 | A 52-year-old Chinese woman was hospitalized because of liver mass diagnosed 2 weeks ago. The magnetic resonance imaging (MRI) examination of the upper abdomen showed a well-defined, 30 × 29 mm2 approximately, a nearly-circular shadow in the IVb segment of the liver, with low signal intensity on T1W imaging and slightly high signal intensity on T2W imaging (Fig. ). After the hospitalization, the laboratory findings revealed that human anti-hepatitis B surface antibody (HBsAb), human anti-hepatitis B virus e antibody (HBeAb), and human anti-hepatitis B core antibody (HBcAb) were positive. Because of the MRI results considering liver tumor, the laparoscopic hepatectomy of the IVb segment was implemented. The liver neoplasm removed was about 3.0×3.0×3.0 cm3 (Fig. ). The pathological examination reported that the neoplastic cell was arranged in a nest-like pattern, in which a small number of lipoid cells could be seen. And the results of immunohistochemical analysis showed as follows: Hepatocyte (Negative), Ki- 67 (approximately 1% Positive), CK (Negative), CK 19 (Negative), HMB45 (Positive), Melan-A (Positive), S-100 (Negative), Desmin (Negative), SMA (Positive), Caldesmon (focal Positive), GPC-3 (Negative), GS (focal Positive) (Fig. ). According to pathological findings a large number of smooth muscle fiber bundles, with abundant thick-walled vascular structures, were observed without nuclear atypia or significant mitosis which was commonly seen in malignant cells, and immunohistochemical staining that HMB45 (Positive), Melan-A (Positive), SMA (Positive), the liver neoplasm was diagnosed ultimately as hepatic angioleiomyoma. Four months after discharge, the patient was reported no recurrence of angioleiomyoma and any postoperative complications. |
pmc-6426533-2 | A 64-year-old Chinese woman was admitted to hospital with symptoms of left lumbago and dorsalgia 2 years ago. First, the B-type ultrasonic inspection showed left kidney lesions. Then the MRI examination showed an approximately 8.4×5.8 cm well-demarcated mass with mixed signals in the lower pole of the left kidney, with low signal intensity on T1W imaging and slightly high signal intensity on T2W imaging (Fig. ). The mass of the left kidney was considered to be an angiomyolipoma and the mass of liver needed to exclude the possibility of a metastatic tumor. The computed tomography (CT) examination revealed a lobulated low-density soft tissue mass in the left liver, showing a ring-shaped enhancement at the edge of the mass in the enhanced scanning arterial phase and a lower enhanced density than the surrounding liver tissue in the venous phase and the parenchymal phase. And in the lower left kidney, an irregular mixed density tumor was found, which contained fat density, flocculent soft tissue density, and calcified nodules. The soft tissue was obviously strengthened during the enhanced scanning, and large vessels were visible at the margin and inside of the tumor (Fig. ). Moreover, we also performed contrast-enhanced ultrasonography, showing that the left kidney mass was rich in blood supply and was unevenly enhanced, presenting a “fast-forward and fast-out” mode, which was considered to be kidney cancer. The mass of the liver was rich in blood supply, showing a “fast-forward” mode, which was considered to be metastatic tumor (Fig. ). Ultimately, after consultation with urology department, hepatobiliary surgery department, medical imaging department, and oncology department, a suggestion was made that the left kidney was radically removed. Then choose surgery or regular follow-up of the liver mass based on pathological results of the kidney's mass. The kidney mass removed was 6.0×0.8×2.0 cm3. The pathological examination suggested that many blood vessels were visible in the mass, as a small-diameter fissure surrounded by many differentiated smooth muscle cells and some adipose tissue. And the results of immunohistochemical analysis showed as follows: HMB45 (Positive), CD10 (Negative), RCC (Negative), PAX-2 (Negative), CK (Negative), Ki-67 (< 1% Positive), S-100 (Negative), SMA (Positive), Vim (Positive) (Fig. ). The pathological examination suggested that abundant thick-walled vascular structures in the mass were mixed with a large number of smooth muscle fiber bundles, and no obvious atypia or significant mitosis of malignant tumor cells was observed. Based on these, the renal mass was diagnosed as angioleiomyoma. The liver mass was not eventually removed, but a regular radiographic examination was selected.
At this time, enlargement of the hepatic mass was revealed in follow-up. The B-type ultrasonic inspection showed that a hyperechoic nodule could be seen in hepatic left lateral, about 4.5 × 3.9 cm2 in size, with a clear boundary, which was considered hepatic hemangioma (Fig. ). And CT examination showed that in the left hepatic lobe, a kind of nearly-circular low density with a little fat solid density shadow can be seen, with a maximum diameter of 3.8 cm. After enhancement, the mass was significantly unevenly enhanced in the arterial phase and reduced in the venous phase and the delayed phase. It was considered to be primary hepatic carcinoma (Fig. ). The MRI reported that a nearly-circular abnormal signal shadow, about 4.4×4.2 cm2 in size, was seen in hepatic left lateral, presenting a lamellar high signal in phase, a low signal out phase, and an equal or low signal in T2 fat-suppressed imaging. It was considered as liver cancer with steatosis (Fig. ). Considering the possibility of liver cancer, laparoscopic hepatic left lateral lobectomy was performed. The hepatic mass after excision was about 5 cm. Pathological examination reported that the tumor tissue consisted of many adipose tissues, epithelioid cells, and thick-walled blood vessels, and a large amount of lymphocyte infiltration was seen in the tumor stroma. And the results of the immunohistochemical analysis showed as follows: HMB45 (positive), Melan-A (positive), S-100 (focal Positive), Ki-67 (5% Positive), CD34 (vascular Positive), SMA (Positive), Desmin (Negative), Hepatocyte (Negative), CD68 (focal Positive), P53 (Negative), Vim (Positive), CD117 (Negative) (Fig. ). According to the pathological features described above, the mass of the liver was diagnosed as hepatic angioleiomyoma. From the first kidney surgery to the rehospitalization, the follow-up time was 2 years, and the follow-up time was 4 months after the liver surgery during the second hospitalization. The patient's tumor did not relapse and no other surgical complications occurred. |
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.