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pmc-6320170-1
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An 18-month-old female patient was admitted to Shanghai Children's Hospital (Shanghai, China) for appearant snoring symptoms accompanied by mouth breathing and sleep apnea. In addition, the patient presented with weak aspiration and nasal leakage during fluid intake.
The routine physical examinations showed a wide fissure which split from the palate vertical anterior cleft to 1/3 of the hard palate. There was no congestion in pharyngeal mucosa with the uvula in the middle. Bilateral tonsils showed hypertrophy without exudation. Epiglottis and bilateral pear-like nest were not clear. The pear bone could be seen. Thus, the patient had incomplete cleft palate. Meanwhile, we also found an unclear-bordered uplift in the left palate and a soft mass which could be moved in the right soft palate trailing edge with endoscopy (Fig. ). Then, the patient underwent a computed tomography (CT) scan. Consistently, radiographs of the palate revealed a mass convex to the pharyngeal cavity which was about 27 mm × 21 mm × 26 mm in size (Fig. ). In addition, no obvious abnormality was found on the nasopharyngeal bone or soft tissue. According to the above examination, the diagnosis for the patient was incomplete cleft palate and soft palate vascularized space-occupying lesions (involving the left posterior wall of the nasopharynx, most likely fibrous hemangioma).
The patient was treated by palatal lump resection and cleft palate repair with endoscopic guidance from the oral cavity while under general anesthesia. During the surgical resection process, the mass was dissected from the dorsal side of the left soft palate with the pharyngeal mucosa preserved and the nasopharyngeal mucosa completely removed. At the end of the surgery, the plasma radiofrequency knife was used to ablate the residual tumor tissue. After proper hemostasis, the cleft palate was also repaired. Postoperative symptomatic treatment of infection was done with nasal feeding. Histological examination showed that there was a well-demarcated mature brain tissue with scattered sand-like calcification (Fig. ).
The patient had an excellent recovery. And one month follow up with endoscopy showed good wound healing without rupture. The snoring, mouth breathing, and sleep apnea symptoms disappeared. Also, the patient no longer experiences nasal leakage with fluid intake.
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pmc-6320199-1
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A 40-year-old Chinese male presented with a 3-day history of sudden vision loss in his right eye. He complained of a sudden onset foreign body sensation in the right eye when he was riding, and vision loss after rubbing. Eye examination showed best corrected visual acuity was no light perception (NLP) in the right eye and 20/40 in the left eye. Anterior segment examination of the right eye showed keratin precipitates+, aqueous flare++, and vitreous opacity. The right fundus examination revealed that the retina was gray, with edema as well as scattered dotted and flaky hemorrhagic foci (Fig. ). In addition, hyalocytes were visible in the left eye, without obvious abnormalities in the fundus (Fig. ). Fluorescein fundus angiography showed the following results: For the right eye, the arm-to-retinal circulation time was 19.54″, background fluorescence of the choroid in the early angiography was not uniform. At 21.10", the optic disc showed hyperfluorescence, with perfusion in some disc blood vessels. Until the late stage of angiography at 10′30.20″, perfusion was seen only in retinal arteries near the optic disc, but not in other retinal blood vessels. The optic disc showed hyperfluorescence, and hemorrhage was visible along the retinal veins, which blocked the fluorescence (Figs. and ). For the left eye, telangiectasia was visible in the optic disc, with leakage in the disc in the late stage of angiography (Fig. ). Auxiliary examination showed that erythrocyte sedimentation rate (ESR) was 50 mm/h and C-reactive protein (CRP) was 18 mg/L. There were no significant abnormalities in blood routine, antineutrophil cytoplasmic antibodies, anticardiolipin antibody, direct antiglobulin testing, Toxoplasma gondii infection, rubella virus, cytomegalovirus, and herpes simplex virus and immune examinations. Echocardiography, carotid duplex ultrasound, abdominal Doppler ultrasound, and chest computed tomography showed negative results. Past medical history revealed that the patient had complaints of recurrent oral ulcers and vulvar ulcers for 8 years, which recently worsened. The patient was diagnosed as Behcet disease at a local hospital and given long-term low-dose oral prednisone, but the recurrent symptoms persisted with a recent relapse. At our hospital, the patient was diagnosed as bilateral uveitis (obstructive retinal vasculitis in the right eye), and Behcet disease. He was given topical corticosteroids, and compound Tropicamide eye drops. The patient was also hospitalized in the Rheumatology Department, and received systemic infusion of methylprednisolone and cyclophosphamide. Two weeks later, his vision acuity of the left eye was 20/20 and that of the right eye was NLP. During the telephonic follow-up after 1 year, the patient reported 2 recurrent episodes of uveitis in the left eye, which improved after treatment at the local hospital.
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pmc-6320205-1
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A 78-year-old woman arrived at our hospital by ambulance with severe epigastric pain and vomiting at rest. Her medical history was unremarkable, and she was not receiving any oral medication. Elevated serum amylase levels of 2991 IU/L (reference range 44–132 IU/L) and serum trypsin levels of 8465 ng/mL (reference range 100–550 ng/mL), suggested acute pancreatitis. An increased white blood cell count of 23,900/μL (reference range 3300–8600/μL), indicated severe inflammation. Contrast-enhanced CT revealed pancreatomegaly, effusion extending from the peripancreatic space to the pelvic cavity, and calcified stones in the lower portion of the common bile duct. The patient was diagnosed with acute pancreatitis due to gallstones and was admitted for treatment. An APACHE II score of 12 led to the diagnosis of severe acute pancreatitis.[ Treatment with continuous intravenous infusion of nafamostat mesilate (240 mg/day), intravenous infusion of meropenem (2.0 g/day), and intravenous infusion of approximately 5000 mL/day was initiated. Two days later, a re-examination CT scan showed a lack of arterial enhancement from the pancreatic body to tail, and pancreatic arterial infusion therapy was administered for 1 week. Four weeks after admission, the patient developed pyrexia of approximately 40°C, and WOPN was suspected on CT scan. Accordingly, percutaneous drainage was performed from the left intercostal space, and a 12-French pigtail drainage catheter (Hanaco Medical, Saitama, Japan) was placed. However, drainage of the necrotic material was insufficient, and a percutaneous endoscopic necrosectomy was performed for WOPN 3 months after admission. The WOPN was resolved after 4 necrosectomies, and endoscopic extraction of the common bile duct stones was performed. Unfortunately, the PCF remained (Fig. ), and approximately 250 mL of pancreatic fluid was collected daily via percutaneous drainage. Conservative treatments such as total parenteral nutrition and octreotide were ineffective. ERCP revealed complete main pancreatic duct obstruction, which could not be crossed with a guidewire. It was decided to use EUS to internalize the PCF into the stomach. The echoendoscope (GF-UCT260; Olympus Medical Systems, Tokyo, Japan) was carefully introduced into the stomach, but because of the narrow PCF lumen, fistula visualization was difficult. This was overcome by inserting a balloon catheter (Multi-3 V Plus; Olympus Medical Systems) percutaneously into the fistula, and the inflated balloon was visualized by EUS from the stomach (Fig. ). Then, the balloon was punctured with a 19-gauge fine needle (SonoTip Pro Control; Medi-Globe GmbH, Rosenheim, Germany) through the posterior wall of the upper body of the stomach (balloon-target technique) (Fig. ). A 0.025-inch guidewire (VisiGlide 2; Olympus Medical Systems) was passed through the fistula to the outside of the body through the EUS scope (Fig. ). After the gastro-fistula space was dilated with an 8-mm Hurricane RX Biliary Balloon Dilatation Catheter (Boston Scientific, Tokyo, Japan) (Fig. ), a 7-French double pigtail catheter (Zimmon Biliary Stent; Cook Medical, Tokyo, Japan) was placed from the stomach into the PCF (Fig. ). The percutaneous drainage tube was removed after one week, and the patient was discharged 6 months after admission. Since postoperative 2 years, no adverse outcomes such as the relapse of acute pancreatitis or appearance of new pancreatic fluid collection have been reported. A timeline depicting the clinical course of the patient is presented in Figure .
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pmc-6320214-1
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A 55-year-old female had been suffering from numbness of extremities for a month, and she was admitted to a hospital in our city. On admission, magnetic resonance (MR) imaging revealed a intramedullary mass lesion located on the medulla oblongata. Because of high risk, the patient refuesd medullary lesion biopsy or surgical removal and was discharged. Two months later, she was admitted to our hospital presenting with intractable hiccough and progressive numbness of extremities.
On admission, neurological examination demonstrated marked deep sensory disturbance in distal portions extremities. The MR imaging revealed a circumscribed mass lesion located on the medulla oblongata. The mass was hyperintense on T2-weighted images, isointense on T1-WI and enhanced homogeneously with gadolinium-diethylenetriamine penta-acetic acid. Examination of the cerebrospinal fluid revealed slightly elevated protein. Analysis of cells confirmed a significant lymphocytosis 86.5% (T lymphocytes: 95.7%, CD4/CD8: 5.86). Her electrocardiogram (ECG) showed complete atrioventricular (AV) block. A computed tomography (CT) of neck and chest revealed bilateral supraclavicular, hilar, and mediastinal lymphadenopathy. The patient underwent permanent pacemaker insertion immediately. Transbranchial needle aspiration was subsequently performed. Pathological examination revealed noncaseating granuloma consisting of epithelioid cells, lymphocytes, and rare multinucleated giant cells (Fig. ). Based on these findings, pathological diagnosis was consistent with sarcoidosis.
The patient was treated with oral prednisone 60 mg/day for 4 weeks. Threedays after starting prednisone therapy, hiccough disappeared and numbness of extremities was relieved. Four weeks after starting prednisone therapy, follow up ECG and imagines showed marked improvements (Fig. ). The dose of prednisone was tapered to 20 mg/day during 5 months, and no recurrence occurred.
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pmc-6320218-1
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A 31-year-old woman (gravida 4, para 1) was referred to our department from local hospital at 33rd weeks gestation with low fever and right-sided flank pain, which had lasted for nearly half a year and severely aggravated for 5 days. As the patient recalled, previous prenatal examinations in local hospital contributed the pain to kidney stones or uterine contractions without any further inspection. After local outpatient treatment with antibiotics, progesterone and Nonsteroidal Antiinflammatory Drugs (NSAIDs), she was admitted to our department as the pain aggravated. She denied any vomiting, hematochezia, or difficulty with urination.
Moderate iron deficiency anemia, occasional dyspepsia, and diarrhea were present during the whole gestation period. At the time of presentation, patient had no family history of gynecologic or CRC. Physical examination revealed right-sided abdominal pain on palpation and normal bowel sounds. Vital signs are normal. Body mass index 24.8. Obstetric examination showed no abnormalities. Initial laboratory results included a mildly elevated white cell count and hemoglobin 7.6 g/dL and a mean corpuscular hemoglobin 26.7 pg. Her serum potassium was 4.0 mmol/L. Liver function tests showed the serum albumin was 20 g/L. Urinalysis and routine excrement examination remained normal with no occult blood. Tumor markers serum carcinoembryonic antigen (CEA) was elevated to 70.68 ng/mL. Abdominal ultrasound showed a large heterogeneous cystic mass located below the hepatic flexure of colon. Considering the extremely low risk of radiation teratogenicity in late pregnancy, an abdominal CT scan without contrast was obtained, which revealed incrassation of the ascending colon wall and exudative change around it (Fig. ). Multiple pathologically enlarged abdominal lymph nodes were observed. No colonoscopy was performed considering the site of the lesion and the possibility to induce uterus contraction of premature delivery.
After a detailed discussion in a multidisciplinary medical team, a planned delivery by caesarean section and tumor resection during the same operative procedure was performed, following the induced fetal lung maturation by dexamethasone. Intraoperatively, a palpable tumor mass at the hepatic flexure of colon with a diameter of 15 cm was identified, with the right lobe of the liver and perirenal adipose tissue involved. Mucopurulent discharge could be found through the rupture on the necrotic mass. Further abdominal exploration revealed paracolic lymph nodes, intermediate lymph nodes, and middle lymph nodes pathologically enlarged, with none metastatic lesions on pancreas or duodenum. Both adnexa and the rest of the peritoneal cavity also appeared normal. A 1550-g live female infant with an Apgar score 10-10-10 was delivered at 33rd of gestation. A right hemicolectomy and ileostomy were performed right after the cesarean section. Tumor histology of frozen section confirmed the diagnosis of ulcerative adenocarcinoma of the ascending colon with a diameter of 10 cm. Final pathologic evaluation showed a grade-1 adenocarcinoma with negative lymph nodes (16/0), R0 resection, pT4b pN0 M0, and Dukes B stage. The postoperative course was uneventful while patient received chemotherapy with folinic acid, fluorouracil, and oxaliplatin (FOLFOX) after recovery smoothly and got discharged 1 month after surgery. Patient showed no relapse or progression during the follow-up time of 2 years.
Written informed consent was obtained from the patient for publication of the present case report and any relevant images.
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pmc-6320327-1
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A 57-year-old man was admitted to the hospital due to hyperleukocytosis. Echocardiography revealed irregularly shaped vegetation (size, 25 × 15 mm) attached to the anterior leaflet of the mitral valve. The vegetation exhibited oscillation and was connected to the thickened aortic valve. Color flow imaging showed severe insufficiency of both the aortic and mitral valves with perforation in the AMC (Fig. ). Chest X-ray revealed bilateral lung congestion due to acute heart failure. Therefore, emergency surgery was indicated.
The heart was approached via median full sternotomy. An oblique incision was made in the ascending aorta under conditions of cardiac arrest. The aortic valve was bicuspid (type 1). Vegetation was observed at the non-coronary cusp, extending to the AMC. The mitral valve was exposed via the superior trans-septal approach. The anterior leaflet was thickened and had attached vegetation. Debridement of the infected tissue led to a defect in the middle portion of the anterior mitral annulus, AMC, and non-coronary cusp.
For reconstructing the defective parts, a glutaraldehyde-treated bovine pericardial patch (Model 4700, Edwards Lifesciences, Irvine, CA, USA) was folded to make a three-portion patch (Fig. a). The triangular portion (AMC portion) of two pericardial patches was sutured to the AMC remnant using continuous sutures. Pledgeted everted mattress sutures were placed around the mitral annulus, and the anterior rim was reconstructed with the pericardial patch (MV portion). A 23-mm mechanical valve (Abbott Laboratories, Chicago, IL, USA) was tied down in the intra-annular position of the aortic annulus in a manner wherein the sutures pass through the aortic annulus and the rectangular portion (AV portion) of the pericardial patch. Finally, a 28-mm mechanical valve (Abbott Laboratories, Chicago, IL, USA) was tied down in the mitral annulus (Fig. b).
Cardiobacterium valvarum was isolated on blood culture. Vancomycin and ceftriaxone were intravenously administered for 4 weeks postoperatively. Postoperative echocardiography revealed normal cardiac function with no significant perivalvular leakage. The patient displayed complete recovery and was discharged on postoperative day 33. The patient was symptom-free at his 1-year follow-up and exhibited normal laboratory and echocardiographic findings.
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pmc-6320348-1
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A 49-year-old male with metastatic colon adenocarcinoma presented with several days of fever, nausea, vomiting, jaundice and hyperbilirubinemia. His past medical history includes right hemicolectomy, right adrenalectomy, partial right hepatectomy and hepatic arterial infusion pump (HAIP) placement 4 years ago. He had received systemic and hepatic arterial pump chemotherapy. Six months prior to this admission he underwent endoscopic placement of two metallic stents across the proximal duodenal obstruction and common bile duct (CBD) obstruction from infiltrative metastases. Computed tomography (CT) scan of the abdomen showed bilobar biliary ductal dilatation due to stent occlusion. Portal vein was patent. Endoscopic biliary drainage failed as the CBD stent could not be accessed due to the presence of duodenal stent. Percutaneous biliary drainage was requested. Informed consent was obtained for all interventions. Cholangiography confirmed obstruction of the CBD stent and an internal-external biliary drainage (IEBD) catheter was placed via a segment 3 duct (Fig. ). Needle access to segment 3 duct was performed under ultrasound guidance. The patient was readmitted 2 days following discharge due to chills, bacteremia, persistent hyperbilirubinemia, right upper quadrant pain, hematochezia, and bleeding inside and around the IEBD catheter. Culture results from the implantable port showed E. coli, other enteric bacteria, yeast and candida similar to bile and peripheral blood samples confirming biliary source of infection. Patient remained afebrile on antibiotics. Intermittent peri catheter bleeding, hemobilia and hematochezia persisted. Antegrade visceral angiography was performed on post-operative day 9. This showed complete obstruction of the common hepatic artery and recanalization of the left hepatic artery via small tortuous collaterals from the left gastric artery. No significant supply was seen from the superior mesenteric artery. The segment 3 branch of the left hepatic artery could not be separated from the biliary catheter on any oblique views confirming it as the source of hemobilia. Retrograde cannulation of the left hepatic artery via the collaterals was not possible (Fig. ). The IEBD catheter was upsized from 8.5F to 12F in attempt to tamponade the injured vessel. Peri catheter bleeding and hemobilia persisted and 5 days later, he underwent repeat hepatic angiography. The common hepatic arterial occlusion was crossed with a 2.4 French microcatheter and 0.018-in. hydrophilic guidewire coaxially. This demonstrated multi-level occlusion of the hepatic arterial branches. The left hepatic artery could not be cannulated antegradely or retrogradely (Fig. ).
The indwelling IEBD catheter was exchanged over wire with a 10 French vascular sheath. Sheath cholangiography showed opacification of the segment 3 hepatic artery. This artery was successfully accessed via the vascular sheath both distal and proximal to its communication with the bile duct using a 5 French directional catheter and hydrophilic guidewire. Both areas of the artery were successfully embolized using a total of ten 0.035-in. and three 0.018-in. metallic coils of different lengths and diameters. Final sheath cholangiography showed no flow in the embolized artery (Fig. ). The peri catheter hemorrhage and hemobilia resolved over the next 2 days. Secondary biliary stenting was performed successfully 6 weeks later. The patient remained asymptomatic and expired 2 months later due to progression of disease.
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pmc-6320353-1
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A 70-year-old male ex-smoker with hypertension, dyslipidemia, and newly diagnosed prostatic adenocarcinoma was undergoing staging prior to initiation of therapy. During this workup, a SPECT/CT scan noted vertebral body notching and multiple extrapleural nodules (Fig. ). Further evaluation with CT angiography revealed multifocal saccular and fusiform aneurysms of the intercostal arteries (Fig. ). No other aneurysms of the neck, chest, abdomen, or limbs were identified. It was decided to preventatively treat three large aneurysms of the right 7th intercostal artery with endovascular embolization.
Following conscious sedation with Fentanyl and Midazolam and local anesthesia with 2% Lidocaine, the right common femoral artery was punctured utilizing a single-wall technique. A 6-Fr sheath was introduced and 5-Fr C2 Cobra catheter (Boston Scientific, Cork, Ireland) advanced selectively into the right 6th through 8th intercostal arteries. Angiography confirmed the target aneurysms of the 7th intercostal artery (Fig. ) and that no spinal artery originated from them. The 6th and 8th intercostal arteries did not provide significant collateral supply to the 7th intercostal artery. A Renegade microcatheter (Boston Scientific, Cork, Ireland) was inserted and Interlock microcoils (2 of 2 mm × 6 mm × 8 cm, Boston Scientific, Cork, Ireland) were deployed starting distally (Fig. ). To maximize the occlusive effect, the aneurysms were then embolized with a Glubran 2 (GEM, Viareggio, Italy)/Lipiodol (Guerbert, Roissy-en-France, France) mixture (1:1). Proximally, Interlock microcoils (2 of 2 mm × 4 mm × 4.1 cm, Boston Scientific, Cork, Ireland) were placed and complete cessation of flow was achieved (Fig. ). There were no intraoperative complications. The patient was discharged home the following day and made an uneventful recovery. At six-month follow-up, the patient remained asymptomatic and will be monitored with yearly CT angiograms.
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pmc-6320563-1
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A 26-year-old woman with a history of metastatic mucinous adenocarcinoma of the appendix presented to the hospital with abdominal pain. A large volume paracentesis removed 5 liters of fluid. Shortly after the procedure the patient experienced respiratory distress, followed by a cardiac arrest. Return of spontaneous circulation was achieved after 7 minutes of CPR. Upon return of spontaneous circulation, the patient’s blood pressure was 89/66 mmHg with a heart rate of 145 beats per minute. Bedside transthoracic echocardiogram (TTE) revealed a severely dilated right ventricle and a large right atrial thrombus. Labs were notable for a venous blood gas with pH 7.08, pCO2 56 mmHg, in addition to troponin 1.61 ng/mL. She received 10 mg of alteplase followed by a 90 mg infusion over 2 hours. She was transferred to the intensive care unit (ICU) receiving 30 mcg/min of norepinephrine and 7.5 of mcg/kg/min dobutamine, but remained persistently tachycardic and hypotensive upon arrival. She had continued evidence of severe right ventricular (RV) dilatation on TTE, cold extremities on physical exam, and a central venous oxygen saturation of 22%. She was deemed a poor surgical candidate by a multidisciplinary PE team. Given her persistent severe obstructive shock 6 hours later, requiring 20 mcg/min of norepinephrine and 7.5 mcg/kg/min of dobutamine, another 50 mg of alteplase was administered over 4 hours. Four hours after completion of the second alteplase infusion, the patient remained in severe obstructive shock requiring high doses of vasopressors and inotropes and an additional 50 mg of alteplase was administered over 2 hours. Overall, the patient received a total of 200 mg of tPA within a 15-hour period. Her course became further complicated by an acute drop in hemoglobin due to hemoperitoneum, with an initial 2.0 g/dL drop in Hb from 8.0 to 6.0 g/dL, occurring 16 hours after the initial tPA infusion. She was supported with a total of 10 units packed red blood cells over the next 48 hours. She was then weaned off all vasopressors and inotropes, and underwent IR-guided embolization of the right hepatic artery, after which her hemoglobin remained stable. A subsequent TTE demonstrated significant improvement in RV function. Four days later she was successfully extubated and transferred out of the ICU. Her course was ultimately complicated by aspiration pneumonia and septic shock secondary to invasive candidiasis; she expired one week later.
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pmc-6320563-2
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A 46-year-old man with history of asthma was brought into the hospital by ambulance for respiratory distress. He was initially managed in the ICU for status asthmaticus requiring heavy sedation and paralysis. His asthma improved, but his course was complicated by bilateral segmental PE detected on computed tomography angiography (CTA). TTE revealed mild RV dilatation and normal RV function. He was treated with low molecular weight heparin and eventually received a tracheostomy because of respiratory weakness, likely from critical illness polymyoneuropathy. While out of the ICU, he underwent placement of a percutaneous endoscopic gastrostomy (PEG) tube, for which anticoagulation was held for one day. The day after PEG placement the patient became acutely hypotensive, with systolic blood pressures between 70 and 80 mmHg. He received a 2 liter normal saline bolus, after which he suffered an asystolic cardiac arrest. ROSC was achieved after 6 minutes of CPR and he was transferred back to the ICU. His arterial blood gas was notable for a pH of 7.05 and pCO2 77 mmHg. He required 20 mcg/min of norepinephrine, 2.4 mL/hr of vasopressin, 300 mcg/min of phenylephrine, 5 mcg/kg/min of dobutamine, and 40 ppm inhaled nitric oxide. TTE demonstrated severe decreased RV function with bulging of the interventricular septum into left ventricle. Due to high suspicion for massive PE, 10 mg of alteplase was administered, followed by a 90 mg infusion over 2 hours. 18 hours later he had continued evidence of severe RV strain on bedside TTE, including unchanged vasopressor and inotropic support. He received 3 more doses of 50 mg of alteplase, administered over 2 hours each and 3 hours apart. In total the patient received 250 mg of alteplase in a 36-hour period. The next day inotropes and inhaled nitric oxide were weaned off. Pulmonary angiography revealed normal pulmonary artery pressures and no clot. TTE demonstrated mild decreased RV function. His course was further complicated by melena and anemia, which stabilized after transfusion of 2 units packed red blood cell, 1 unit platelets, and 2 units fresh frozen plasma. In the setting of persistent altered mental status, the patient underwent an MRI which showed evidence of embolic infarcts; a transesophageal echocardiogram (TEE) revealed a patent foramen ovale, which was later closed percutaneously. His mental status returned to baseline and he was weaned off the ventilator. He ultimately underwent decannulation of his tracheostomy, removal of his PEG tube, and was discharged home.
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pmc-6320575-1
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A 14-year-old White Italian boy came to our Emergency Unit with a headache that had worsened over 20 days together with blurred vision and diplopia over the previous 10 days. His past history was negative for significant morbidities. He reported a recent episode of fever associated with cough, which coincided with the onset of headache. For this respiratory infection he had started taking levofloxacin 500 mg once a day one week before coming to our attention but had stopped taking it after three days due to worsening headache. This headache was initially associated with daytime somnolence, myalgia and arthralgia. The somnolence and arthralgia underwent rapid and spontaneous regression, with subsequent appearance of blurred vision.
The physical examination revealed an alert adolescent with weight of 66 kg (75th -90th percentile) [], height of 169 cm (50th–75th percentile) [] and body mass (BMI) of 23.1 kg/m2 (85th–95th percentile) []. The general examination was normal. The neurological examination was normal except for a right eye abduction deficit. Eye examination showed a normal visual acuity (10/10) in both eyes with normal colour vision and pupillary light responses, but a fundus examination revealed severe bilateral papilloedema with elevated disc, hyperaemia, blurred margins and vessel tortuosity in both eyes (Fig. a-b). Lancaster red-green test confirmed a right abducens nerve palsy, and campimetry showed a restricted visual field with external right muscle deficiency on the right side. Cranial neuroimaging (CT and MRI) showed a normal brain parenchyma with no evidence of hydrocephalus, mass, structural lesion, or abnormal meningeal enhancement. MRI neuroimaging showed oedema of both optic nerves with a tapered appearance of the right optic nerve. Venography was not performed, but an angio-MRI of the cerebral circulation was normal. Visual evoked cortical potentials were normal. A 24-h Ambulatory Blood Pressure Monitoring was negative.
Blood tests showed high M. pneumoniae IgM (15.00 AU/ml, normal range 0–9) and normal M. pneumoniae IgG levels (3.89 AU/ml, normal range 0–9) suggesting a recent infection, with normal white blood cell indices and negative C-reactive protein. Clarithromycin was then prescribed for 14 days without any adverse effects.
Serological screening for Coxsackie, Parvovirus, ECHO virus, Adenovirus, Cytomegalovirus (CMV), Epstein-Barr Virus (EBV), Herpes Simplex Virus 1 (HSV1), and Herpes Simplex Virus 2 (HSV2) excluded recent infections. Thyroid function was normal. Antinuclear antibodies (ANA), anti-double stranded DNA (dsDNA), ENA screening and rheumatoid factor were negative.
During hospitalisation we observed a complete and spontaneous regression of headache and an initial spontaneous reduction in diplopia within a few days. Oral prednisone 50 mg/day (0.75 mg/kg/day) was administered for a week and ocular fundus was monitored.
Since severe bilateral papilloedema persisted one week after the first assessment, lumbar puncture was performed with the patient sedated and relaxed in lateral recumbent position. Opening cerebrospinal fluid (CSF) pressure measured with a standard manometer was 20 cm H2O and closing pressure was 19 cm H2O. These CSF pressure values have traditionally been considered borderline, but are within normal range according to a recent study in children [].
CSF biochemical tests and cultures were negative. HSV1, HSV2, VZV, HHV6, CMV, Neisseria, Haemophilus, Streptococcus pneumoniae, Streptococcus B group, Escherichia coli, Listeria and Cryptococcus neoformans, Parvovirus, Adenovirus, EBV DNA and Enterovirus and Parechovirus RNA PCR were negative. CSF oligoclonal bands were absent on CSF and blood tests.
Oral acetazolamide (1 g divided twice daily) was introduced to accelerate recovery. A gradual further improvement in diplopia was seen during hospitalisation (Fig. a-b). Ophthalmological, neurological and neurosurgical follow up was continued after discharge. The patient gradually improved, with complete resolution of the right abducens nerve palsy in one month and resolution of papilloedema in three months (Figs. and ). For this reason, acetazolamide was gradually reduced and stopped on resolution of the papilloedema (see Additional file ).
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pmc-6320586-1
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A 70-year-old, 54-kg male presented with cough and blood-stained sputum. A computed tomography (CT) scan of the chest revealed a tracheal lesion in the middle trachea (Shown in Fig. ). Bronchoscopy showed a tumor encroaching into the left tracheal wall of the middle trachea. A biopsy was positive for an adenoid cystic carcinoma of the trachea. Routine laboratory investigations were normal. The patient’s electrocardiogram showed changes of ST-T in the anterior lateral wall and the inferior wall. Coronary CT angiography confirmed that the left anterior descending branch was mildly-to-moderately stenosed with atherosclerosis. Cardiac function was at NYHA classification level II. To avoid the stress response of open thoracotomy, the patient was scheduled for tracheal resection and reconstruction under VATS.
After arrival in the operating room, routine monitoring including electrocardiography, blood pressure, and pulse oximetry were applied. Preoperative vital signs included a blood pressure of 138/82 mmHg, heart rate of 70 beats per minute and pulse oximetry of 99%. Catheterization of the radial artery was performed under local anesthesia for continuous blood pressure monitoring as well as blood gas analysis.
After preoxygenation, anesthesia was induced with intravenous sufentanil (Yichang Humanwell Pharmaceutical Company, China), propofol (AstraZeneca S.P.A., Italy), and cis-atracurium (Hengrui Medicine, Jiangsu, China). Under visual guidance of the imaging camera in the tip, an internal diameter (ID) of 7.5 mm VivaSight™ SLT was intubated through the glottis and placed above the tracheal tumor (Shown in Fig. ). The tube was then rotated until carina and tumor were both visualized on the monitor simultaneously. A 9F bronchial blocker was placed in the right main bronchus under the guidance of VivaSight™ SLT. The cuff of the bronchial blocker was inflated with the volume necessary to seal the bronchus (8 mL air). (Shown in Fig. ). General anesthesia was maintained with continuous intravenous infusion of remifentanil (Yichang Humanwell Pharmaceutical Company, China) 0.1–0.15 mcg•kg− 1•min− 1 and propofol (AstraZeneca S.P.A., Italy) 6–8 mg•kg− 1•h− 1. The depth of anesthesia was adjusted according to the Narcotrend index (MonitorTechnik, Bad Bramstedt, Germany). Cis-atracurium was intermittently administered to maintain muscle relaxation by the guidance of TOF-Watch (Organon, Netherlands).
The patient was placed in the left lateral decubitus and the position of the bronchial blocker was continuously monitored. Right video assisted thoracotomy was performed uneventfully to separate the trachea. Right lung collapse was achieved by deflating the cuff of the bronchial blocker and disconnecting the tube from the ventilator just before the surgeon broke the pleura. After a few seconds, the cuff of the bronchial blocker was inflated with the same volume of air and one-lung ventilation was achieved.
After the trachea was well exposed, the bronchial blocker was withdrawn, and a sterile SLT of ID 6.5 mm was placed to the left main bronchus through the incision of the trachea by the surgeon. One-lung ventilation was then performed through this tube. Following previous report [], the lesioned tracheal was resected. During tracheal anastomosis, the patient was intermittent ventilated through the endobronchial tube, which was inserted from the incision of tracheal by the surgeon. As tracheal anastomosis was not easily performed under VATS. When the surgeon was doing the tracheal anastomosis, the SLT was removed and the ventilation was interrupted. For a while, the SLT was re-inserted and the patient was ventilated to restore oxygenation. Until the completion of tracheal anastomosis, the patient was ventilated again through the VivaSight SLT. After surgery, the patient was transferred to the thoracic ICU with a trachea tube. He was extubated at 24 h post-operatively.
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pmc-6320590-1
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A previously healthy 57-year-old woman, with no significant past medical history, presented to the surgical department of our hospital for definite management of a primary pancreatic leiomyosarcoma, after being treated with adjuvant chemotherapy.
One year before her last admission, she was initially admitted to our emergency department due to abdominal pain, fatigue, and weight loss. She was totally healthy prior to these symptoms. She then underwent magnetic resonance imaging (MRI) that was indicative of a pancreatic head lesion along with possible metastatic liver lesions, superior mesenteric vein occlusion, and portal vein infiltration (Fig. a, b). The decision was to undergo an endoscopic ultrasound (EUS) biopsy in order to determine the exact nature of the lesion. EUS report was indicative of pancreatic leiomyosarcoma.
Multidisciplinary team’s decision was to use gemcitabine- and docetaxel-based chemotherapy as up-front treatment to assess tumor response. Follow-up CT scan and magnetic resonance imaging (MRI) after the completion of chemotherapy regimen showed downsizing of the pancreatic mass, as well as downsizing of suspicious for malignancy segment III liver lesion (Fig. c, d).
Based on the response to chemotherapy, tumor characteristics, and physical status of the patient, multidisciplinary team’s decision was to proceed to surgical exploration. Due to local expansion of the pancreatic tumor, its relation with the superior mesenteric and portal vein, and the underlying SMV thrombosis, excision of the pancreatic tumor was not feasible. Intraoperatively, a small piece of tumor was excised in order to be sent for histopathology. Surgeon’s decision was to ablate the tumor with irreversible electroporation (Fig. ). Metastatic liver lesions were identified with the use of intraoperative ultrasound. Segment III liver lesion was resected, while smaller lesions of the right lobe were ablated using microwave ablation.
The patient had an uneventful postoperative recovery and complete resolution of her symptoms. Histopathological examination of pancreatic lesion as well as segment III liver lesion revealed sarcomatous tissue of high cellularity with fascicular pattern, increased mitotic activity, and diffuse cytoplasmic immune reactivity for SMA, desmin and h-Caldesmon, and chromagen DAB (Figs. and ). Surprisingly enough, pathological report of a smaller liver lesion was indicative of angiomyolipoma staining positive for HMB45 and Melan-A. The lesion was a benign hamartomatous, circumscribed but unencapsulated hepatic mass composed mainly by mature lipocytes and limited mesenchymal component (smooth muscle cells), showing no marked atypia and thick-walled vasculature. Myoid component was positive for ΗΜΒ-45 and Melan-A. Based on the histopathological report, tumor board decided that the patient should be treated with adjuvant therapy for leiomyosarcoma after surgery. A regimen with anthracycline and olaratumab was used for 3 months. Follow-up imaging in 6 and 12 months showed no progression of the disease (Fig. a–d).
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pmc-6320594-1
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A 40-year-old Fulbe man from the Adamawa region of Cameroon presented to the out-patient department of our institution with a complaint of a progressively increasing non-tender abdominal mass associated with pollakiuria for approximately 2 months prior to consultation. He is a farmer with no chronic medical condition or past surgeries. He has never been exposed to any carcinogenic substance; he does not consume alcohol, tobacco, or any drugs. He is married and has four children; however, his birth history could not be investigated further. A physical examination revealed a patient who looked well with a blood pressure of 128/82 mmHg, heart rate at 78 beats per minute (bpm), and temperature of 37.4 °C. An abdominal examination revealed a firm, non-tender, non-mobile, hypogastric mass projecting approximately 20 cm above the pubic symphysis (Fig. ). Examination of his genitalia revealed just one testis in the right scrotum, with the contralateral scrotum and inguinal canal being empty. There were no palpable inguinal lymph nodes or ascites. A neurological assessment revealed conserved muscle forces and sensitivity in all four limbs with all reflexes, particularly the cremasteric and abdominal reflexes, conserved. Paraclinical investigations revealed: no hematuria and proteinuria on urine analysis, normal white cell and platelet count on the full blood count, no blast cells on the blood smear, and a negative human immunodeficiency virus (HIV) serology. A pelvic ultrasound revealed a heterogeneous bean-shaped mass lying above his bladder, approximately 10 cm by 7 cm in size, with five smaller satellite masses. His kidneys, bladder, and bowels had no abnormalities. Given these findings, we had as a probable diagnosis, enlarged mesenteric lymph nodes.
An exploratory laparotomy was scheduled and carried out, with intraoperative findings revealing a highly vascularized mass fixed to the left inguinal ligament, projecting into the retroperitoneum, with several other small satellite masses attached posteriorly. His peripheral bowels, mesenteries, and bladder were all without any visible structural abnormalities.
Progressive dissection and hemostasis was done to free and resect all the masses. The largest had several lobes attached together, weighed approximately 800 g, and measured approximately 11 cm by 7 cm by 5 cm (Fig. ). Seven smaller masses were removed with sizes ranging from 3 cm to 6 cm (Fig. ). Samples of the masses were obtained and sent for histopathology. His postoperative period was unremarkable; he was discharged 7 days after. Histopathology results received 2 weeks later revealed a tumor composed of sheets of fairly uniform polygonal cells having central vesicular nuclei with occasional prominent nucleoli and moderate/abundant brownish/clear cytoplasm. The tumor was divided into lobules by interconnecting thin fibrovascular septa containing a mild, patchy, mixed, inflammatory exudate. All these were suggestive of a seminoma on an undescended testis (Fig. ).
He was then counselled and referred to see an oncologist for further management. At the time of submission of this manuscript we had not yet received feedback from either our patient or the oncologist.
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pmc-6320602-1
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The patient, a 16 years old male of Kurdish ethnicity, was admitted to the pediatric lung and allergy service of Astrid Lindgren Children’s Hospital at Karolinska University Hospital due to chronic airway hypersensitivity and recurrent sinopulmonary infections. He is the third child of consanguineous parents with a family history of several early deaths due to lung failure on the maternal side (Fig. ). He had a normal vaccination history but a medical history of four hospitalizations due to enteroviral infection (at age 16 months presenting with skin rash and diarrhea), chronic cough and fever (at age 18 months due to Moraxella catarrhalis), otitis media, adenopathy and shingles (leading to tympanostomy at the age of 2), pneumonia and an asthmatic reaction (at the age of 6).
At the age of 8 years, a computed tomography was performed due to a progression of his pulmonary disease which revealed bronchiectasis and a right middle lobe atelectasis. Immunologic profiles were investigated as previously described []. Although a complete blood count and immunoglobulin levels were normal, lymphocyte populations were measured. Low CD4+ and CD8+ T cell numbers, with normal numbers of B and NK cells were detected (Fig. and Table ). The patient had low specific cell-mediated immune response in activated whole blood using mitogens and antigen, such as pokeweed mitogen (PWM), candida antigen etc. (Table ). However, normal response to mitogens phytohemagglutinin (PHA) and concanavalin A (Con A) by CD4+ T cells, but not CD8+ T cells were detected. The observation suggested that PHA and ConA stimulations for CD4+ T cells may be different from CD8+ T cells. Despite his combined immunodeficiency, the patient was free from opportunistic infections and his condition improved with temporary substitution of subcutaneous immunoglobulin and prophylactic antibiotics.
In order to identify the molecular defect, whole exome sequencing (WES) was performed. As the patient was born in a consanguineous family and showed a family history of recurrent infections and early death on the maternal side, an autosomal recessive or X-linked inheritances pattern was expected. Analysis of all variants were performed according to a standard pipeline described previously []; we identified 2 homozygous (autosomal) and 5 hemizygous (X-linked) variants which were absent from dbSNP database and 1000 Genome database (Additional file : Table S1). Comparing with the primary immunodeficiency genes database, the only variant consistent with the patient’s immunological phenotype was a novel nonsense mutation, p.R328X (c.982C>T) in exon 8 of the IL2RG gene (Fig. ). Based on this finding, the therapeutic plan of the patient was changed and he became a potential candidate for allogeneic hematopoietic stem cell transplantation.
Since the mutation causes a 42 amino acid truncation of the intracellular domain of the γC, including of the Janus kinase 3 (JAK3) binding site (Fig. ), we investigated the expression of members of the IL2/JAK3 signaling pathway by western blot. Western blot (Fig. ) demonstrated absence of IL2RG, suggesting that the mutation caused degradation of the molecule. In addition, IL2 stimulation activated JAK3 and signal transducer and activator of transcription signaling 5 (STAT5) proteins in cells from a healthy control but no activation was observed in the patient; indicating an impairment of IL-2 signaling. STAT5 expression was observed in both the control and the patient, while the main JAK3 isoform (1124 amino acids, 115kDA) was only observed in the control. However, the intensity of second isoform of JAK3 (1094 amino acids, lacking part of the kinase domain) was stronger in the patient. When the blot was reprobed with another anti-JAK3 antibody (binding to the kinase domain), expression were observed only in the control samples, indicating that the expression pattern of JAK3 is modified in the patient (Fig. ).
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pmc-6320604-1
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A 71-year old, female patient with a previous history of non-Hodgkin lymphoma and transient ischemic attack came to the emergency room of our University Hospital for sudden onset of right hemifacial paraesthesia, edema of the lower lip (Fig. ) and accentuation of an already present tinnitus. The current presentation had been preceded by a few blisters similar to those usually observed in herpes labialis, and no aphthous ulcer was detected on mouth inspection. Background therapy included aspirin and betahistine, with no personal and family history of adverse drug reactions, atopy, contact dermatitis, urticaria, angioedema, cranial nerve palsy, granulomatous or inflammatory diseases. After symptomatic treatment by intravenous steroids and antihistamines, the patient was discharged with prescription of a short-course therapy with oral prednisone and cetirizine []. This resulted in partial remission of symptoms, but 1 week later the patient was readmitted to ER for symptom recurrence and worsening of lip edema without detectable oral cavity and tongue alterations. Due to the apparent involvement of the 5th cranial nerve, a varicella-zoster virus (VZV) infection was hypothesized and therapy with valaciclovir initiated. On occasion, a blood sample was drawn showing evidence of anti-VZV IgG with undetectable IgM. One month later, on further admission at the ER for the same clinical picture associated with swelling over the left zygomatic region, an angioedema of unknown origin was suspected. Thus, a course of twice daily dose of 10 mg cetirizine was prescribed [–]. However, this approach was ineffective and also the subsequent replacement of aspirin with clopidrogel and temporary withdrawal of betahistine resulted in no improvement. IgM and eosinophil count, as well as plasma levels of angiotensin converting enzyme were in the normal range, thus helping to exclude the hypothesis of Gleich syndrome or sarcoidosis. Patch testing for dental materials was also negative, and complement screening was then performed with evidence of normal levels of circulating C1q (143 mg/L) and both antigen (302 mg/L) and functional (109%) C1-Inhibitor (C1-INH). On the contrary, C4 was low (0.03–0.04 g/L; NR 0.09–0.36 g/L) and C3 fluctuated around the lowest levels of the referral range (0.93–0.82 g/L; NR 0.9–1.8 g/L) on repeated assessments. These findings ruled out the possibility of acquired AE due C1-INH deficiency [–], prompting us to explore the (auto)immune-inflammatory state: antineutrophil cytoplasmic antibody tested negative, whilst low titer (1:160) anti-nuclear antibodies (ANA) were found along with antiphospholipid antibodies (lupus anticoagulants; anti-cardiolipin, anti-β2-glycoprotein IgM), possibly related to complement consumption [].
As ultrasound scan detected only a subcutaneous, hypoechogenic thickening of the inferior lip and we did not find any further clinical or laboratory sign of systemic inflammation, recurrent swelling was interpreted as a form of idiopathic angioedema and treated with tranexamic acid after a thrombophilia screen testing negative for further risk factors [, ]. Both this antifibrinolytic drug and a following, therapeutic course with the leukotriene receptor antagonist montelukast [] failed to solve the edema. Finally, clinical picture, blood analyses, and lack of response to any of the previous therapies suggested the possibility of Melkersson–Rosenthal syndrome []. Thus, the patient was referred to the Dental Clinic, where a mucosal biopsy of the affected lower lip was performed (Fig. ). Histopathological examination showed non caseating granulomas (Miescher’s cheilitis), consistent with a diagnosis of MRS. The two aggregates of non-caseating granulomatous inflammation consisted of lymphocytes and epithelioid histiocytes, and few multinucleated giant cells, clustered around scattered vessels (Fig. ). Special staining for identification of fungal microorganisms and acid-fast bacteria were negative. No foreign material could be detected even at polarization. One month of oral steroid (prednisone, 25 mg qd, gradually tapered to 5 mg) resulted in remission of lip swelling but not in definitive recovery. However, since then patient’s perception of both symptoms and aesthetic relevance decreased, and at present she undertakes a few-days regimen of prednisone only when feeling a relapse of edema.
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pmc-6320610-1
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A 45-year-old Japanese man received an intra-articular injection of glucocorticoid (betamethasone 2 mg) for pain in his right elbow joint 2 days prior to admission. On the day of admission, he experienced general fatigue. Two hours later, he experienced a sudden, severe headache and was brought to our emergency department in an ambulance.
He was diagnosed as having hypertension at 44 years of age, and his blood pressure was under control with lisinopril 10 mg/day. He had no other significant past medical history or any episodic headaches. He was a tobacco smoker (20 cigarettes/day) for the past 24 years, and consumed approximately 50–100 g/day of alcohol, but was not addicted to any drugs, such as cocaine. He was married and had two children (a daughter, 12-years old; a son, 1-year old). His family had no history of diabetes, cancer, or any endocrine diseases, like pheochromocytoma, medullary thyroid carcinoma, parathyroid adenoma or hyperplasia, mucosal neuroma, and kidney cancer.
His vital signs were as follows: blood pressure, 240/126 mmHg; pulse, 120 beats/minute (regular); temperature, 37.6 °C; respiratory rate, 25 breaths/minute. Except for excessive perspiration and sinus tachycardia, physical and neurological examinations showed no significant findings, such as pallor, tremor, enlarged thyroid gland or palpable thyroid nodule, enlarged lymph nodes, abnormal lung or heart sounds, meningeal irritation, and central or peripheral nerve dysfunction. Initially, subarachnoid hemorrhage was suspected due to severe headache and elevated blood pressure. However, computed tomography and magnetic resonance images of his head were normal. In addition, the cerebrospinal fluid drawn by lumbar puncture was clear, eliminating the possibility of cerebral vascular diseases, including subarachnoid hemorrhage. The results of the initial laboratory tests are shown in Table . Based on severe hyperglycemia and metabolic acidosis with normal HbA1c level on investigations, we suspected DKA caused by FT1DM.
We initiated the standard treatment for DKA, including intravenous insulin infusion and fluid replacement. The course of insulin infusion rates and plasma glucose levels is presented in Fig. . Following the initiation of insulin infusion, his plasma glucose level rapidly decreased and recovered to normal within 2 hours. In 18 hours, the lowest insulin infusion rate (0.1 U/h) was required to maintain normoglycemia. At the same time, our investigations showed that his basal insulin secretion was normal, and plasma ketone levels were not elevated, as shown in Table . These findings indicated metabolic acidosis induced by lactic acid, and excluded the possibility of FT1DM. Subsequently, he was screened for secondary diabetes. A left adrenal gland tumor (3 cm in diameter) was detected by abdominal computed tomography (Fig. a). The levels of urinary catecholamine metabolites (metanephrine and normetanephrine) and serum catecholamines were significantly elevated; however, the other hormone levels were normal (Table ). Elevated levels of serum adrenaline and noradrenaline did not reduce following the clonidine test, which indicated an autonomic catecholamine secretion. A functional scintigraphy of the adrenal gland using iodine-131 metaiodobenzylguanidine showed a strong uptake in the region of the left adrenal gland (Fig. b). These findings led to a diagnosis of pheochromocytoma. However, there were no findings suggestive of medullary thyroid carcinoma, parathyroid adenoma or hyperplasia, or any other endocrine diseases. His blood pressure was controlled with an α1 adrenergic receptor blocker doxazosin (12 mg/day), following which a left adrenalectomy was performed 85 days after his admission. The tumor size was approximately 118.75 cm3 (9.5 cm × 5 cm × 2.5 cm, Fig. a, b). On histopathological assessment, most tumor cells were positive for chromogranin A and synaptophysin, which were consistent with the diagnosis of pheochromocytoma (Fig. c–e) []. There were no signs of lymphovascular or capsular invasion. However, markers of malignancy, such as Ki-67 labeling index and Pheochromocytoma of the Adrenal Gland Scaled Score indicated borderline abnormalities (1.5% and 4, respectively), which required careful follow-up [, ]. Since this first event, he showed no signs of pheochromocytoma crisis such as paroxysmal hyperglycemia and hypertension. During the postoperative follow-up for 28 months, he did not show any symptoms or signs indicating recurrence of pheochromocytoma.
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pmc-6320617-1
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A 12-year-old boy presented with a left avulsion fracture of the ischial tuberosity. Informed consent was obtained from this patient and his family. The patient’s family history and previous medical history were unremarkable. The patient was a track-and-field athlete who felt severe pain in his left buttock while running. He visited a local hospital, where plain radiographs and computed tomography (CT) of the pelvis showed an avulsion fracture of the left ischium (Fig. ). The fragment was displaced 20 mm. No neurological deficit was present. Complete non-weight-bearing therapy was performed as a conservative treatment, but the patient’s symptoms continued, and he visited our hospital two months after injury. During the preoperative assessment, he complained of pain in the gluteal area during walking. The patient also described muscle weakness of the hamstrings, and straight leg raising (SLR) was limited to 80°/60°. The results of a blood test were all within normal ranges. Magnetic resonance imaging (MRI) at two months postinjury revealed a displacement of approximately 20 mm, with fluid accumulation between the avulsed fragments (Fig. ).
At eight weeks postinjury, we performed open reduction and anchor fixation because of non-union and displacement of the fragment after conservative therapy (Fig. ). Following administration of general anesthesia, the patient was placed in a prone position. A 10-cm incision was made longitudinally around the ischial tuberosity, and subgluteal approach was used. The plane between the gluteus maximus and the hamstring muscles were divided. The inferior edge of the gluteus maximus was elevated to identify the ischial tuberosity. The avulsed fragment was distally displaced. The hamstrings were fully mobilized distally to reduce the avulsed fragment without excessive strain. Three suture anchors were placed in the exposed ischium (Fig. d). Two holes were drilled 1 cm distal to the proximal edge of the fragment, each in line with the distal suture anchors. Three drill holes were made through the avulsed fragment, taking into account the anchor locations. The fragment was reduced with the hip extended and the knee flexed and fixed with five biodegradable suture anchors (HEALIX ADVANCE 5.5; DePuy Synthes, Tokyo, Japan) using the suture bridge technique (Fig. e, f).
A Snyder sling (Hashimoto Artificial Limb Manufacture Co., Okayama, Japan) was used to restrict knee movement to within 45° of flexion in postoperative week 3 and to within 10° of flexion in week 5, while passive assistive hamstring stretches were performed; then, active ROM exercises were started from week 6 (Fig. ). The next day after surgery, he started non-weight-bearing walking with crutches. One-third weight-bearing was permitted from week 6, and full weight-bearing was permitted from week 8. Union was confirmed on radiography and CT in week 9 (Fig. ), and the patient was therefore permitted to start jogging and gradually building up training with squats and jumps involving quick hamstring stretching. The patient returned to competitive athletics in week 13. At the final follow-up, no bilateral difference was evident in hip ROM, at 120°/120° flexion or 30°/30° internal rotation. The SLR test was 80°/80°, and no pain was experienced in the ischial tuberosity during jogging. The manual muscle testing (MMT) score was 5 for both the gluteus maximus and hamstring muscles. Assessment when the patient returned to competition found no restriction of hip joint ROM, and his visual analog pain score was zero. The Lower Extremity Functional Scale (LEFS) [] at the final follow-up was 80 points (100%).
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pmc-6320623-1
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A 20-year-old man presented with a history of right knee pain of 3-months duration without any trauma or undue exercise. Physical examination showed joint effusion and limited range of motion. There was no locking in the joint and no palpable mass. He had no other significant past history.
The patient did not receive any conservative treatments. He did not receive any plain x-radiography. An MRI of the right knee showed that the intra-articular lesion was located around the posterior cruciate ligament. The lesion showed iso-intensity or lower intensity compared to surrounding muscle in T1 weighted MRIs, and high signal intensity in T2 weighted MRIs (Fig. ). The preoperative differential diagnoses were synovial chondromatosis, pigmented villonodular synovitis or malignant soft tissue tumour. We planned to perform an arthroscopy operation to remove the lesion and to obtain a biopsy to test for malignant soft tissue tumour. If positive for malignancy, additional wide extra articular resection would be needed, and the artificial joints were prepared.
Therefore, arthroscopy of the right knee was performed to reveal synovial hyperplasia inflammation and the mass in front of the right posterior cruciate ligament.(Fig. ) The lesion was excised, and partial synovectomy was performed.
Macroscopically, the right knee mass presented with a piece of grey-red tissue measuring 2.5 cm by 2 cm by 1 cm in size. The antibodies, clones, dilutions, pretreatment conditions, and sources are listed in Table . On microscopic examination in Fig. , the tumour consisted of a bland fibroblastic proliferation arranged in irregular fasciitis with tissue-culture-like appearance. The stroma varied from focally myxoid with microcyst formation to collagenous. Extravasated erythrocytes and small lymphocytes were present throughout the lesion. No areas of necrosis or atypical mitosis were seen. Immunohistochemistry in Fig. demonstrated that the cells were positive in patches for SMA, and negative for S100, desmin, CK(AE1/AE3), nuclear stain of beta catenin and CD34 in lesion cells. Ki-67 stained 10% of cells. According to clinical features, imaging and histology, the final diagnosis was intra-articular nodular fasciitis, which is usually a self-limiting and regressing fibrous process. Recurrence after incomplete excision has been occasionally observed.
The symptoms of painful joint effusion and limited range of motion were improved 1 month after the operation. No recurrence was observed at the 6-months’ follow-up.
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pmc-6320630-1
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An 18-year-old female patient was admitted to our hospital with complaint of kyphoscoliosis after birth. Her radiographs with the spine demonstrated that the Cobb angle of lumbar scoliosis was 105° (Thoracic 11 to Lumbar 4) and the distance of trunk shift was 10 cm. The kyphosis angle from T8 to L3 was 58° (Fig. ).
At birth, the patient was noted to have hemihypertrophy and hemangioma on her face and back. Abnormal asymmetric growth became apparent along with her age. She underwent laser therapy for her facial hemangioma at the age of 4. Three years ago, venous varicosities appeared on both lower extremities. Klippel-Trenaunay Syndrome (KTS) was diagnosed for her. In addition, the patient had a history of hypoxic-ischemic encephalopathy (HIE) at birth. However, the Apgar score was unclear. Her mother took some medicine for cold at her 8th week of pregnancy.
There was no family history of KTS.
Physical examination showed hemihypertrophy of the left face, trunk, lower limb (Fig. ). There was a port-wine stain on her back (Fig. ) and venous varicosities on both lower limbs (Fig. ). Her left tonsil was swollen in 3 degrees. Her left leg was 2 cm longer than the right side. Obvious claudication was noted when she walked. Neurological examination was intact.
Positive laboratory examination results included an increased D-Dimer level of 2.02 mg/L (0~0.55, FEU), decreased hemoglobin level of 105 g/L (110–150 g/L), decreased 1,25(OH)2D3 level of 6.58 pg/mL (19.6~54.3 pg/mL), decreased Fe level of 34.7μg/dL (50~170 μg/dL), and decreased ferritin (Fer) level of 8 ng/mL (14~307 ng/mL). Thyroid function test showed increased TSH of 6.669 μIU/mL, A-Tg of 189.20 IU/mL, and A-TPO of 297.50 IU/mL. The fecal occult blood test was negative.
A computed tomographic (CT) scan of spine revealed no vertebral body deformities. Doppler ultrasound scan found no significant arteriovenous shunting. A magnetic resonance imaging of the spine showed Chiari-I-malformation without syringomyelia.
We had a consultation with endocrinologist and hematologist. However, the relation between kyphoscoliosis and other comorbidities could not be determined. Vitamin D deficiency, like other comorbidities, might be due to innutrition. Oral ferrous succinate and cholecalciferol cholesterol emulsion were administrated preoperatively. As a result, her Hemoglobin, Fe, and Fer regained normal value before surgery. However, her 1,25(OH)2D3 level was 5.21 pg/mL, which was still lower than the normal value. Subcutaneous injection of low molecular weight heparin was conducted preoperatively and maintained two weeks postoperatively. The dynamic change of D-Dimer level was shown in Fig. . Finally, posterior scoliosis correction and spinal fusion from T10 to L5 levels were performed. During surgery, we found that the scoliosis was very rigid and blood oozing from the wound surface was obvious. Bone quality was similar to other adolescent patients during pedicle screw implantation, although the patient had Vitamin D deficiency. Left pedicle of T10 poorly developed and we failed to place left pedicle screw of T10. The total operation time was about 5 hours and the amount of blood loss was 1300 mL. The motor evoked potential signal of the spinal cord was normal during the operation. Postoperative plain X-ray film demonstrated the Cobb angle of lumbar curve corrected from 105° to 60° (correction rate 43%) and the distance of trunk shift decreased from 10 cm to 1.4 cm (Fig. ). The kyphosis angle decreased from 58° to 26°. No thrombotic events or other complications occurred during perioperative period. At the 3rd month follow-up, there was no change of the curve in the coronal and sagittal planes (Fig. ). At the 8th month follow-up, the Cobb angle in the coronal and sagittal planes was 54° and 34°, respectively. The trunk shift was 1.5 cm in the coronal plane, which was not significantly different from that of postoperative (Fig. ). During the 31-month follow-up, the patient did not experience any discomfort. And her general appearance did not have any change until the last follow-up. However, she refused to take radiograph for worrying about radiation.
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pmc-6320786-1
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A 68-year-old woman with chronic kidney disease stage II presented with worsening sacral pain in 2012. Evaluation revealed multiple lumbosacral foci of DLBCL. Disease persisted despite induction chemotherapy with rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine sulfate, and prednisone alongside lumbosacral radiation. Salvage chemotherapy with rituximab, ifosfamide, carboplatin, and etoposide, supplemented with lumbosacral radiation, achieved a PET-negative complete remission.
Months later, the patient noticed a subcutaneous nodule superficial to her right scapula, and biopsy showed recurrent DLBCL. After surgical resection and adjuvant gemcitabine, rituximab, and oxaliplatin, her DLBCL remained refractory to therapy. She was enrolled in a phase II trial (clinicaltrials.gov #NCT02445248) assessing CTL019 in DLBCL (JULIET) []. Leukapheresis and CAR T manufacture were successful, but she developed postmenopausal vaginal bleeding, heralding diagnosis of stage I endometrial carcinoma which precluded further participation in JULIET. A compassionate-use IND application (#16944) was approved given CTL019 manufacture occurred prior to symptoms of endometrial carcinoma. CTL019 was infused following three days of lymphocyte-depleting fludarabine and cyclophosphamide. At this time, six subcutaneous nodules were present dorsal to her right scapula DLBCL, clinically consistent with recurrent DLBCL. She tolerated the CAR T infusion well, with no side effects, and was discharged three days later.
Her post-CAR infusion course was complicated by three presentations of neutropenic fever with autonomic instability and pancytopenic aplasia. She lacked described [] neurologic or general symptoms concerning cytokine release syndrome (CRS), neither did she develop any signs of end organ failure associated with CRS. Laboratory evaluation showed nonspecific signs of inflammation: ferritin, 864–1946 ng/mL (normal 11–307 ng/mL); lactose dehydrogenase, 98–215 units/L (normal <200 U/L); and interleukin-6 (IL-6), 12–19 pg/mL (normal <5 pg/mL). Her neutropenic fevers were each considered consistent with septic shock given positive blood and urinary cultures for Enterobacter cloacae treated with ciprofloxacin. Observation of the subcutaneous deposits of DLBCL showed regression of palpable lesions over the two months following CAR T infusion, with local breakdown of the skin over one of the lesions ().
Peripheral blood was collected for analysis on post-infusion days 1, 8, 17, 21, 31, and 58. T cell populations peaked by day 31 (A–D). CAR T cells accounted for 0.4% of the total CD3 expressing cell population at day 17. T cell immunoglobulin mucin domain 3 (Tim-3), was expressed on more cells than programmed cell death protein 1 (PD-1), with peak expressions on both the CD8 T cell (Tim-3 ≈ 50%; PD-1 ≈ 17%, G) and CAR T cell subsets (Tim-3 ≈ 78%; PD-1 ≈ 40%, H). Tim-3 was preferentially expressed on the CD8 subset, while lymphocyte activation gene 3 protein (LAG3) was more expressed on the CD4 subset, although on <10% of clones (F). Immune checkpoint inhibitor overexpression was greatest on day 8, concurrent to CAR T cell expansion, but preceding a T cell contraction phase from day 20 onward (E–H).
In order to determine the effects of CAR T expansion on other immune cells in the blood, the frequencies and phenotypes of other immune cells, at the peak of T cell expansion on day 31 post CAR T, were characterized by flow cytometry, as shown in . These data show that even at the time of peak T cell expansion, numbers of CD3+ T cells remained low (A). CD4+ T cells comprised 10.8% of the mononuclear cell population and 29.3% of all mononuclear cells were CD3+ CD8+ (B). After infusion of anti-CD19 directed CAR T, little to no CD19 expressing cells were detected, suggesting on-target CAR T function (C). The increase in CD56bright CD16-cells (D) likely represents an increase in cytolytic NK (natural killer) cells, whereas the increase in CD56dim CD16+ cells represent NK cells with replicative potential, as reviewed []. CD56bright CD16+ cells are thought to represent a population of cytotoxic T cells, with both αβ and γδ T cells expressing these antigens []. Populations of macrophages and immature monocytes (CD14dim expression, E) were increased following CAR T administration. In summary, these data in combination with a dramatic regression of subcutaneous nodules of DLBCL, apparent on examination, and confirmed by PET/CT, suggested on-target CTL019 function in depleting CD19+ targets.
To evaluate her prolonged pancytopenia (detected day 31 post-CAR T), which required repeated platelet and blood transfusions, a bone marrow aspirate was performed and immunophenotyping of marrow cells was compared to peripheral blood in . The total cellular content of bone marrow was significantly reduced across all lymphocytes, including CD3 positive cells (A). Anti-CD19 CAR T cells within both the CD4 and CD8 subsets remained detectable in the peripheral blood (B,C), and these CAR T were scarce in the marrow. Lastly, ratios of naïve (CD45RA+) and memory/activated T cells (CD45RO+) were observed to be nearly identical in both the peripheral blood and bone marrow (D); however, the total quantity of cells in bone marrow was reduced. Of note, during pancytopenic aplasia, the ratio of CD8+ CD27-/CD28-cells increased (D).
To assess the clonality of the global T cell compartment, deep sequencing of the T cell receptor-β (TCRβ) complementarity determining region-3 (CDR3) was performed (A). Clones of T cells with a productive frequency of 5% or less prior to CAR T administration were identified and increases in productive-frequency were tracked post-infusion, with A showing clones with the largest increases. A concomitant increase in CAR T was observed using flow cytometry (, Vβ-20). B shows T cell clones collected from the DLBCL nodules on day 10. Clones present at higher frequency in the tumor, present to a lesser extent in blood, suggest anti-tumor specificity of these T cell clones (purple dots, B) common to both compartments. Small numbers of clones expanded at high frequencies and homed to the tumor nodule, which are denoted by purple boxes (B). Despite these findings, CTL019 therapy failed to induce a complete and durable response for this patient. She later received monoclonal antibody against PD1, which also failed to induce a remission. She then opted for supportive care, and died from sequelae of DLBCL six months later.
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pmc-6321649-1
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The patient was a 54-year-old woman who was admitted to the hospital because of frequent palpitations for 3 months. Anti-arrhythmic drugs including mexiletine, propafenone, and metoprolol had been ineffective. She had no history of cardiovascular disease. PVCs detected by surface 12-lead electrocardiography (ECG) had the following morphology: a complete left bundle branch block, inferior frontal axis, and precordial lead transition zone >V3. The QRS in lead I was positive, and the R-wave in lead II was higher than that in lead III. The findings suggested that the PVCs were from the free wall of the RVOT. Most of the time, the ectopic beats demonstrated bigeminy with stable coupling intervals, but sometimes, the coupling intervals varied and multiplied. These findings implied that the PVCs were actually VP (Fig. a). Twenty-four-hour dynamic ECG showed more than 32,000 PVCs.
After withdrawal of anti-arrhythmic drugs for 5 or more half-lives, the patient underwent an electrophysiological evaluation. Both bipolar and unipolar electrograms were recorded by a LEAD-7000 EP Recording System (Sichuan Jinjiang Electronic Science and Technology Co., Chengdu, China) filtered at 30–500 Hz and 0.05–500 Hz, respectively. Three-dimensional electromagnetic mapping (CARTO, Biosense Webster, Diamond Bar, CA) and ablation were performed via a 7-French saline-irrigated ablation catheter with a 3.5-mm distal electrode and 2–5-2 mm interelectrode spacing. Activation mapping and pace-mapping were combined to identify the origin of VP. Activation times were assigned based on the earliest rapid downstroke of the unipolar signal (fastest dV/dt) correlating with the first sharp peak of the bipolar electrogram. The reference line was a sharp peak of QRS in lead II.
As a result, activation mapping in the RVOT region was performed and failed to indicate the earlier activation region (Fig. b). Pace-mapping also failed. The key reason for failed mapping was fusion of VP and nodal QRS. In this case, all ventricular activations during VP had two components. The degree of fusion was unstable, which accordingly made the morphology of QRS variable, as detected by surface ECG. Therefore, there was no stable reference for activation mapping (Fig. c). The precedence of local activation was unmeasurable. For the same reason, pace-mapping was also unavailable because there was no QRS template of pure VP activation. We tried to find the source of VP according to the morphology of the V-wave by unipolar ECG. Two target points were confirmed in the anterior and middle regions of the RVOT free wall, respectively (Fig. d, e). However, ablations were not effective at these positions.
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pmc-6321677-1
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In 2008 a 35-year old female patient was referred to the Glaucoma Center of the Semmelweis University in Budapest, where advanced juvenile open-angle glaucoma was diagnosed in both eyes. Her untreated intraocular pressure (IOP) was 36 and 28 mmHg, visual acuity eccentric hand motion and 1.0, and central corneal thickness 531 and 542 μm on the right and left eye, respectively. The vertical cup/disc ratio was 0.95 in both eyes. A fixed combination of bimatoprost and timolol was prescribed, and the under treatment IOP became controlled for both eyes. Over the next 10 years the under treatment IOP of the left eye ranged between 9 and 14 mmHg (typically 12 to 13 mmHg). The patient entered a long-term, prospective glaucoma structure-function investigation in the Glaucoma Center of the Semmelweis University in Budapest, for which the research protocol was approved by the Institutional Review Board for Human Research of Semmelweis University, Budapest and written informed consent was given by the patient before enrolment. The left eye was followed with various imaging methods and the Octopus 30-degree normal G2 visual field test (Octopus 900 perimeter, Haag-Streit AG, Koeniz-Berne, Switzerland) at regular 6-month intervals. Peripapillary OCTA measurement with the Angiovue OCT via undilated pupil became a part of the tests in March 2015, and was performed in all study visits at 6-month intervals until December 2017 (2.5-year follow-up and 6 visits). The peripapillary imaging was made with software version 2015.100.0.33, and it was analyzed with the 2017.1 software version and the Phase 7 update []. The 10-cluster progression analysis function of the Octopus perimeter was used to match functional progression to structural progression [, ]. All visual field tests had less than 20% false positive and less than 20% false negative response rates.
For PcVD and RNFLT progression analysis only high quality images with no artifacts or vitreous floaters were used. The image quality score was 8/10 for all but one image, for which the score was 7/10. All image acquisitions were made by the same investigator (GH). For PcVD measurements split-spectrum amplitude-decorrelation angiography was used. Motion correction was applied and the eye tracking function was activated. The 4.5 mm × 4.5 mm scan size was used. The peripapillary area was automatically defined as the area between the 2 and 4 mm diameter elliptical contour lines automatically fitted around the disc margin []. RNFLT was automatically determined as a part of peripapillary OCTA measurement. For progression analysis both RNFLT and PcVD are graphically presented and statistically evaluated with linear regression analysis, for the inferior and superior 180-degree retinal areas, respectively (Figs. and ). No exact P-value is given, significant progression is defined as P < 0.05.The software version also provides information on 360-degree PcVD, and total image area all-vessels density.
At the beginning of the OCTA follow-up the visual field mean defect was 17.1 dB. The superior and inferior RNFLT values were 48 and 43 μm (Fig. ), and the corresponding PcVD values 28.9 and 36.5% (Fig. ), respectively. During the follow-up period the uncorrected visual acuity remained unchanged (1.0). The rate of change was similar for the superior and inferior RNFLT, but only the superior RNFLT (which at the beginning of the follow-up was 5 μm thicker than the inferior RNFLT) progressed in a statistically significant manner (− 0.5 μm/year). In contrast, superior PcVD remained stable, but inferior PcVD (which was 7.6% higher than superior PcVD at the baseline visit) progressed significantly at a rate of − 2.4% per year. The difference between the first and last visits was − 0.7% for the superior and − 7.2% for the inferior PcVD (Fig. ). The Octopus visual field cluster analysis showed that the inferior clusters all progressed significantly at a rate of 2.0 to 5.1 dB/year, which spatially corresponds with the superior RNFLT progression. But for the superior visual field clusters no progression was detectable due to floor effect, as indicated by the software with the black half-arrowhead symbols which appear in Figs. and . This corresponds with the apparent stability of the very low inferior RNFLT, which is probably also caused by floor effect. No progression was detected either for the superior hemifield inner macular retinal thickness (ganglion cell complex, GCC; Pearson correlation, P = 0.638) or for the inferior hemifield GCC (P = 0.139).
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pmc-6321693-1
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A 63-year-old Japanese woman was referred to our department because of an abnormal shadow at the left side of her chest wall on computed tomography. She had undergone total hysterectomy and radiotherapy for cervical carcinoma 4 years prior. One year after the first surgery, three metastatic lung nodules appeared at the upper lobe of her right lung, the lower lobe of her right lung, and the lower lobe of her left lung. Wedge resection for upper and lower lobe of her right lung was initially performed via three-port thoracoscopic surgery. Then, wedge resection for the lower lobe of her left lung was performed via eighth intercostal single incisional thoracoscopic surgery. After the surgery, an intrathoracic chest wall mass developed which increased in size gradually. Her gynecologist introduced her to our department for surgical resection of the mass. Her family, including her parents and two sisters, had been healthy and had no inheritable diseases. She had no symptom, drug history, tobacco smoking history, or psychosocial history, and she was a social drinker. She had not received any medications since the mass developed and until admission to our hospital. She had undergone an operation three times as mentioned above and had been a carrier of type B hepatitis.
After her admission to our department, her general condition was good, and there were three operative scars at both sides of her chest and lower abdomen. Her chest sounds were clear and there was no neurological abnormality. She was 151.1 centimeters tall and weighed 49.8 kilograms. Her heart rate was 77/minute, blood pressure was 135/87 mmHg, and body temperature was 36.1 °C. The laboratory findings were white blood cells 5.25 × 103/μL, hemoglobin 12.7 g/dL, and platelets 156 × 103/μL. A liver function test revealed: albumin 4.6 g/dL, aspartate aminotransferase 15 U/L, alanine aminotransferase 13 U/L, and total bilirubin 0.3 mg/dL. A renal function test revealed blood urea nitrogen 13.6 mg/dL and creatinine 0.79 mg/dL. An electrolyte test revealed sodium 143 mEq/L, potassium 3.8 mEq/L, and chlorine 106 mEq/L. A tumor marker test revealed carcinoembryonic antigen 3.4 ng/mL and squamous cell carcinoma antigen 0.80 ng/mL. Another test revealed positive reaction to type B hepatitis surface antigen and C-reactive protein < 0.1 mg/dL. Computed tomography demonstrated a gradually increasing low-density mass measuring 2.0 × 1.8 cm in diameter (Fig. ). Magnetic resonance imaging demonstrated a low-intensity mass in T1-weighted imaging and a high-intensity mass in T2-weighted imaging (Fig. ). The mass was thought to be a singular cyst; however, this type of cyst was rare and the mass was increasing. Therefore, dissemination of cervical carcinoma could not be excluded, and surgical removal of a part or tissue of the mass was performed.
In the right lateral position, thoracoscopic excision of the mass was done with two ports (3 mm and 2 cm access ports) by two general thoracic surgeons (Fig. ). First the 3 mm port was set at the sixth intercostal space on the inframammary line. Most of her left lung was attached to her chest wall; therefore, the second port was set above the cyst and lysis of adhesions was done. After the lysis, the cystic mass was found adhering to the upper lobe of her left lung. The adhesion of the mass to her lung was not strong and could be separated without injury to the visceral pleura. Therefore, the mass was thought to derive from the chest wall pleura and was resected by adhesiolysis.
The mass was a unilocular cyst containing mucinous fluid. On microscopic examination, the cyst was lined with a single layer of cuboidal epithelium (Fig. ); immunohistochemistry showed positive staining of calretinin and D2-40 (Fig. ). Thus, the cyst was diagnosed as mesothelial cyst derived from the chest wall pleura. Five years after the surgery, our patient had no evidence of cyst or cervical carcinoma on computed tomography.
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pmc-6321697-1
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A 51-year-old woman presented to the emergency department with sudden-onset severe abdominal pain, as well as hypotension (75/48 mmHg) and diffuse abdominal tenderness with guarding on physical examination. Laboratory tests were significant for downtrending hemoglobin levels (75 g/L). Abdominal computed tomography (CT) scan with intravenous contrast showed a 2.5 cm filling defect and discontinuity in the wall of the gallbladder body, a 1.0 × 0.8 cm stone in the neck of the gallbladder, and a massive hematocele in the abdominal cavity (Fig. a). Past medical history was significant for hypertension but no history of recent abdominal trauma or past episodes of biliary colic; social history was not significant for any alcohol or tobacco use. Patient had also been taking daily aspirin (200 mg per day) for the past three years because of interventional surgery for cerebral aneurysms. The patient underwent an urgent laparoscopic abdominal exploration. A 2.0 cm defect was identified in the body of the gallbladder and an active arterial bleeding site was visualized at the edge of the defect. The remainder of the gallbladder wall appeared normal without any hyperaemia and edema. 2500 mL of fresh and clotted blood mixed with bile was evacuated from the gallbladder fossa, right supra-hepatic space, splenic recess and pelvic cavity. Final pathology demonstrated a disruption in the muscularis propria of a portion of the gallbladder wall and the abundance of eosinophils and lymphocytes infiltration in the mucosal layer, associated with chronic cholecystitis (Fig. b). The patient was discharged on post-operative day 7 without complications and recovered well.
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pmc-6321868-1
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In July 2016, a 61-year-old female suffering from chronic kidney disease, secondary to refractory hypertension, on long-term treatment with 15 mg prednisone for sarcoidosis, presented to the emergency room with presyncope, and drainage and erythema at her Hickman catheter insertion site. She had had the CVC in place for the previous 6 years for treatment of frequent episodes of malignant hypertension and congestive heart failure requiring urgent administration of antihypertensive in the setting of poor venous access. Five days prior to admission, the patient noticed that the catheter site had become erythematous and tender with copious brown discharge, which required her to change dressings daily rather than weekly. She had been experiencing presyncopal spells since the discharge started. Three days prior to admission, she started taking 250 mg each day of unused, unexpired oral levofloxacin she had left over from a prior urinary tract infection. This led to an initial decrease in erythema, tenderness and discharge with resolution of the presyncopal spells. After three days, the presyncopal spells returned with nausea, which lead her to seek treatment. In the emergency room, she had vitals within the normal range. She denied having any other symptoms, but reported that she had been showering with the catheter uncovered for over a year. Aerobic and anaerobic blood culture bottles were set up (BD BACTEC blood culture media) from the CVC, which was removed to eliminate the most likely source of infection. On admission, the patient had a total white cell count of 1.04×104 cells µl−1, a haemoglobin value of 12.5 g dl−1 and a platelet count of 3.55×105 platelets µl−1. The erythrocyte sedimentation rate was 33 mm h−1 and the C-reactive protein level was 2.1 mg dl−1. Daily blood cultures were obtained from peripheral sites over the next 3 days, which were negative. On admission, the patient was placed on 1000 mg of intravenous vancomycin every 12 hours and the oral levofloxacin was increased to 750 mg each day to cover polymicrobial infections of the catheter. The patient responded well to treatment after completing a 14 day course of therapy with a peripherally inserted central catheter (PICC) line that was placed on the third day of admission. Two months after admission, she had a port placed for permanent access with a lower risk of infection, and had not had evidence of infection since the port’s placement.
The aerobic and anaerobic blood bottles initially collected from the central line before the patient was admitted grew Gram-positive cocci in clusters, Gram-negative rods and Gram-positive rods in 24 h. Both aerobic and anaerobic blood bottles were subcultured on 5 % sheep blood agar and chocolate agar plates (Remel) per the standard protocol. The initial identification and susceptibility testing of colonies from the plate media was carried out using the BD Phoenix. The bacteria were identified as Staphylococcus aureus, Staphylococcus epidermidis, Acinetobacter sp. and Leifsonia aquatica (score of 90 %). The Hickman catheter tip grew small white and small yellow colonies that matched the colonies from the blood cultures identified as L. aquatica. The L. aquatica, designated as isolate 4120, was further analysed, since infections associated with this bacterium are uncommon. MALDI-TOF MS was used for microbial identification, following the manufacturer’s instructions. Isolate 4120 was identified as Microbacterium paraoxydans with a score of 2.13. The MALDI-TOF MS BioTyper 3.0 software queries a reference database and returns top organisms with confidence scores ranging from 0.0 to 3.0. Scores of ≥2.0 are considered high-confidence with identification to the species level, while scores of 1.6 to 1.99 are considered intermediate-confidence with identification to the genus level only. Scores of <1.6 are considered unacceptable for identification, according to manufacturer’s recommendation []. Due to the conflicting results between the BD Phoenix and the MALDI-TOF MS, the RapID CB PLUS system (Thermo Scientific) was used to further investigate the identity of this isolate. The identification came back as L. aquatica with a score of >99 %. This prompted us to do additional investigation and analyses. Reference strains of L. aquatica (ATCC 14665 and ATCC 51721) and Microbacterium sp. (Microbacterium paraoxydans ATCC 1818, Microbacterium foliorum DSM-12966 and Microbacterium oxydans DSM-20578) were obtained from the American Type Culture Collection (Manassas, VA, USA) or the DSMZ (Leibniz Institute DSMZ, Brunswick, Germany), respectively. Morphological and biochemical profiles were established using standard methods. The susceptibility testing of colonies from the plate media to MICs was carried out using the BD Phoenix system, confirmed on the Trek Sensititre automated testing platform, bioMérieux Etest strips and/or Kirby–Bauer antibiotic, following the manufacturers’ instructions. Results were interpreted as per Clinical and Laboratory Standards Institute (CLSI) guidelines []. Isolate 4120 and three of the reference strains (ATCC 1818, ATCC 14665 and ATCC 51721) underwent whole-genome sequencing using the MiSeq Genetic Analyzer (Illumina). The morphological, biochemical and susceptibility characteristics of isolate 4120 were most similar to those of ATCC 1818, which is Microbacterium paraoxydans (). Notably, isolate 4120 was highly resistant to erythromycin and rifampicin. The MIC for vancomycin was 3 µg ml−1, which is considered non-susceptible. The score-oriented dendrogram generated by the BioTyper software showed the MALDI-TOF MS mass spectra of isolate 4120 cell extracts to be phylogenetically related to Microbacterium paraoxydans (data not shown). Further, genetic analysis confirmed that isolate 4120 was most closely related to Microbacterium paraoxydans [average nucleotide identity (ANI) 94.01 %] and Microbacterium spp. CH1 (ANI 93.97 %) based on the sequences available in GenBank. Notably, the 4120 genome shared an ANI score of >97.7 % with Microbacterium paraoxydans ATCC 1818 (). Based on this data, we conclusively identified isolate 4120 as Microbacterium paraoxydans. Interestingly, with the exception of one reference strain (ATCC 14665, L. aquatica), the BD Phoenix and RapID CB PLUS systems incorrectly identified all organisms that we attempted to identify (). Of note, the BD Phoenix identified ATCC 14 665 as L. aquatica in the first run with a score of 99 %, but on the second run, the organism was identified as Cellulomonas turbata with a score of 99 %. Similarly, the system also identified the test isolate (4120) as L. aquatica in the first run with a 90 % score, but in the second run, it was identified as Micrococcus lylae with a score of 98 %. The MALDI-TOF MS only failed to identify (score was too low) reference strain ATCC 51721, which also could not be readily classified based on DNA sequence ().
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pmc-6321950-1
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A 66-year-old female from an Ashkenazi Jewish family with three successive generations of GD type 1 () presented for evaluation of GD. Her past medical history was notable for osteopenia and gallstones. One of her two sons had both colon cancer and GD discovered in his 30s after evaluation of easy bruising, thrombocytopenia and splenomegaly. Her father had colon cancer, which was successfully resected; he was diagnosed with GD in his 30s after evaluation of thrombocytopenia and splenomegaly, requiring splenectomy. In her 50s, she had colon cancer that was resected with pathology showing Gaucher cells. She was otherwise asymptomatic with no significant medical or social history. She denied bone pains, bone fractures, easy bruising, bleeding, fatigue and weight loss. The family was tested for familial forms of colon cancer, such as Lynch syndrome, which was noncontributory. On examination, her pulse rate was regular but low at 42 beats per minute. There was no evidence of hepatosplenomegaly or purpura. There was evidence of mild cervical dystonia, with her neck ratcheting to the left with several movements.
Her complete blood count was within reference range. Testing for β-glucosidase activity showed reduced levels (2 nmol/h/mg, normal 8–16), confirming the suspicion for GD. A direct gene analysis for the common 9 pathogenic variants in the GBA gene was performed; a homozygous pathogenic variant in GBA (N370S/N370S) was detected, confirming GD. Biomarker analyses of GD that reflect excess lipid storage revealed elevated activity levels of angiotensin converting enzyme (ACE; 66, 9–63 U/L), chitotriosidase (CHITO; 1603, 4–120 nmoles/h/ml) and glucosylsphingosine (lyso-Gb1; 164, <10 ng/mL). Whole exome sequencing had been done previously on this family by the laboratory of Dr. Steven Gallinger (personal communication), and no disease-causing variants relating to colon cancer were found.
ECG revealed a left bundle branch block and bradycardia. MRI of the lumbar spine demonstrated marrow infiltration, with a bone marrow burden score of 6/16. MRI of the abdomen revealed a normal liver volume of 1300 mL and mild splenomegaly of 262 mL (normal volume for her weight would be ~150 mL). Confounding diagnoses that may present with similar clinical and biochemical features include hematologic malignancies such as lymphomas and leukaemias, which must be ruled out.
Successful treatments exist for GD type 1 and include enzyme replacement therapy (ERT) and glucosylceramide synthase inhibitors. Therapy is indicated for patients with type 1 GD who exhibit clinical signs and symptoms of the disease, including anaemia, thrombocytopenia, skeletal disease, or visceromegaly. Our patient did not meet criteria for therapy. Presymptomatic treatment remains controversial. To date, the patient remains asymptomatic with no clinical or radiographic evidence of worsening hepatosplenomegaly, anaemia, thrombocytopenia, lung disease, or major bone abnormalities.
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pmc-6322227-1
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We present the clinical case of an eleven-year old boy, born from Romanian non-consanguineous parents who belonged to low socioeconomic strata, affected by T1D since he was 8 years old.
At the onset of diabetes, he was hospitalized in the Emergency Department of a Romanian hospital with a recent history of polyuria, polydipsia, weight loss and weakness. At the admission, the patient presented Glasgow Coma Scale score of 8. The following laboratory test were performed: blood gas analysis showed pH 7.04, bicarbonate serum 6 mmol/l; serum glucose was 567 mg/dl; glycated hemoglobin was 120 mmol/l and ß-hydroxybutyrate levels were 5.6 mmol/l.
He had been treated with insulin therapy, water and salt replacement according to the International Society of Pediatric and Adolescent Diabetes guidelines for management of diabetic ketoacidosis (DKA) for 48 h []. After the suspension of DKA treatment, multiple daily insulins injections were prescribed, with an initial total insulin dosage of 1 IU pro kg, insulin lispro at meals and insulin glargine at bedtime. The patient was discharged after one week, but he did not attend follow-up visit at the Diabetes Centre.
The glycometabolic control was very poor and the patient had been hospitalized with moderate diabetic ketoacidosis in two occasions. At the age of 10 years, he had moved to the Southern Italy with his family. At the age of 11 years, he was admitted due to severe DKA in an Emergency Department of a secondary level hospital. After the resolution of the DKA, he was transferred to our Paediatric Diabetes Clinic for further investigations due to the observation of marked hepatomegaly (Fig. ), short stature and for the poor metabolic control. At the admission, he presented a stature of 127.5 cm and a weight of 25 Kg (< 3° centile of expected height and weight for age and sex). Secondary sexual characters were absent, Tanner stage being 1. On clinical examination, he had a liver enlargement of 4 cm below subcostal margin. No jaundice, splenomegaly, declivous oedema or ascites were noted.
Laboratory tests showed the following alterations: serum glucose (238 mg/dl), glycated haemoglobin (114 mmol/l), total cholesterol (271 mg/dl), triglycerides (175 mg/dl). Acid base balance was normal (pH 7.39, bicarbonate serum 24 mmol/l), lactate serum was 1.1 mmol/l. Liver function tests showed normal levels of transaminases, alkaline phosphatase, total bilirubin and prothrombin time. To evaluate differential diagnosis of hepatomegaly he was submitted to further laboratory investigations. Normal levels of antinuclear antibodies, anti-smooth muscle antibodies, antimitochondrial antibodies and antineutrophil cytoplasmic antibodies excluded autoimmune hepatitis. To rule out infectious causes of hepatomegaly it was found serology for Epstein Barr virus, cytomegalovirus, hepatitis A virus, hepatitis B virus, hepatitis C virus, human immunodeficiency virus, which all resulted negative for recent infections. Normal levels of iron serum studies eliminated the suspicion of hemochromatosis. Normal cupremia and ceruloplasmin levels excluded Wilson disease. To investigate short stature, the following exams were performed: thyroid function tests resulted normal, serologic testing for coeliac disease was negative, insulin-like growth factor 1 was at the lower levels of normality according to age and sex. The skeletal age determination showed 9.9 years Greulich-Pyle atlas. Clonidine growth hormone stimulation test was performed and revealed subnormal growth-hormone peak level (6.9 ng/dl).
Abdominal ultrasound confirmed marked hepatomegaly with regular echo texture and normal portal vein. During the hospitalization, he presented a brittle glycaemic control characterized by fluctuations between hyperglycaemia and hypoglycaemia. In order to obtain a good metabolic control, the daily insulin dosage was titrated reaching a daily insulin dose of 2.3 IU pro kg. His parents received an education diabetes program. MS was hypothesized based on the association of hepatomegaly, short stature, dyslipidaemia and a history of poorly controlled diabetes.
Liver biopsy was performed, routinely haematoxylin-eosin stained 4 μ-thick sections were made from 10% neutral-buffered formalin-fixed paraffin-embedded tissue block. Parallel serial sections were also stained with periodic acid-Schiff, Sirius Red, Orcein, Perls and Masson’s trichromatic techniques. The sample showed a preserved lobular architecture with many swollen glycogen-laden hepatocytes, prominent periportal nuclear glycogen pseudo-inclusions (Fig. a) and focal macrovescicular steatosis (< 33%). Staining with periodic-acid Schiff showed an intense cytoplasmic positivity, with a strong magenta’s colour in swollen hepatic elements (Fig. b). No evidence of inflammation and fibrosis was noted. Staining for copper and iron deposits were negative. These findings confirmed the diagnosis of hepatic glycogenosis.
At the three-month follow-up visit, he presented a poor glyco-metabolic control, glycated haemoglobin 124 mmol/l and extreme glycaemic variability. On physical examination, he had a more severe hepatomegaly. Laboratory tests showed total cholesterol 450 mg/dl, triglycerides 995 mg/dl, ALT 807 UI/L, AST 694 UI/L. Therefore, he was hospitalized and intravenous continuous insulin treatment was practiced for normalization of aminotransferases and achievement of good glycaemic control, reached after eight days. At the last follow-up visit the patient maintained a good glycemic control such as demonstrated by the value of glycated hemoglobin (55 mmol/l). The improvement of glycol-metabolic control lead to a complete remission of biochemical, clinical signs and complete resolution of hepatomegaly (Fig. ). Despite to the regression of the liver disease, his stature remained < 3° centile and his growth velocity had an initial improvement only for the last months of clinical observation (Fig. ).
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pmc-6322234-1
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A 59 year old man with Crohn’s disease presented to dermatology in March 2016 with a scalp growth. Biopsy showed a spindle cell/desmoplastic melanoma (Clark level IV, Breslow thickness 1.75 mm, mitotic figures at least 5/mm2, no perineural/lymphatic invasion) with positive deep margins. In April 2016 he underwent wide excision with sentinel lymph node biopsy that revealed residual mixed spindle cell/desmoplastic melanoma that was completely excised with negative margins and negative nodes (stage IIB, pT4A). Previous to this the patient had a history of Crohn’s disease requiring hospitalization and following lymph node dissection, treatment was changed from infliximab and azathioprine to single therapy vedolizumab, an inhibitor of integrin α4β7, with the intent of limiting immunosuppression as much as possible while still optimizing therapy for Crohn’s disease.
Surrounding Inflammatory Bowel Disease (IBD), the patient was initially diagnosed with Ulcerative Colitis in 1991 and did not require treatment until developing a perirectal abscess in 1999. At that time the diagnosis was changed to Crohn’s disease rather than Ulcerative Colitis. Crohn’s disease is heterogeneous in its clinical manifestations, and the Montreal classification schema is used to better categorize a patient’s clinical course by age of onset, disease location, and disease behavior. The patient’s Montreal classification was A2 (onset between 17 and 40 years old), L3 (ileocolon location) and B3p (penetrating behavior with perianal disease). He has had no extra-intestinal manifestations of his IBD. Following the perirectal abscess in 1999 the patient was started on mesalamine and had approximately yearly flares requiring prednisone tapers for disease control. In 2010 he required more frequent tapers and his symptoms began to more aggressively emerge if his prednisone dose was reduced below 20 mg daily. In 2011 he presented to our institution’s Gastroenterology clinic. Pathology review from the outside hospital colonoscopy biopsies in 2010 showed inflammation of the cecum, descending colon, sigmoid colon, and rectum consistent with moderate to severe colitis. A repeat colonoscopy in 2011 confirmed active moderate-severe disease. He was started on azathioprine and mesalamine enemas/suppositories and continued on oral mesalamine. On this regimen he was able to be weaned off prednisone with symptom control. His course was complicated by the development of shingles, which required dose reduction of his azathioprine. He had a repeat colonoscopy in the June 2012 that showed active disease in the terminal ileum, cecum, and right colon. He was then started on infliximab infusions every 8 weeks and continued on dose-reduced azathioprine and rectal mesalamine (enemas/suppositories). Repeat colonoscopy in June 2013 showed normal terminal ileum with mild colitis proximally and mild to moderate proctitis. His rectal mesalamine therapy was escalated and repeat colonoscopy in November 2015 showed Crohn’s disease to be in remission.
In April 2016 IBD therapy was changed from infliximab and azathioprine/mesalamine to vedolizumab in response to his melanoma diagnosis. Vedolizumab has been administered 10 mg/kg intravenously every eight weeks in conjunction with IV steroids since that time. The patient underwent surveillance colonoscopy as recently as May 2017 with pathology consistent with normal ileum and colon and patchy quiescent colitis in the sigmoid and rectum (Fig. ).
In April 2016 the patient presented to our Oncology clinic for initial consultation surrounding the diagnosis of melanoma when following resection and work up revealed no evidence of disease. In July 2017 he presented with a nodule on his scalp, and biopsy demonstrated recurrence of melanoma (BRAF wildtype; NF1, SF3B1, TERT, TP53 variants). Subsequent positron emission tomography showed a hypermetabolic and large lytic lesion in the sacrum as well as a fludeoxyglucose avid lesions in the thyroid and lung, consistent with metastatic melanoma.
Immunotherapy with pembrolizumab was initiated in September 2017. Additionally, stereotactic body radiation therapy (SBRT) was pursued for treatment of the large sacral mass including 22.5 Gy in three fractions. Of note the maximum cumulative dose of radiation his rectum could have received over the course of treatment is 50 cGy. Following the fourth cycle of pembrolizumab in December 2017, CT imaging revealed resolution of the previously visualized right middle lobe nodule (Fig. ) and no growth of the sacral mass, consistent with our group’s published experience with pembrolizumab and SBRT (Fig. ) []. Likely also contributing to this response is the abscopal effect from his radiation therapy as well as the synergistic effect of radiation therapy and immunotherapy which has been well-described in an Opinion article by Ngwa et al. [] The patient now continues beyond cycle eleven of pembrolizumab with no evidence of progression of disease and having had no flare of IBD symptoms or toxicity related to radiation.
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pmc-6322324-1
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A man in his early 90s was undergoing a follow-up for mild idiopathic interstitial pneumonia. He required assistance to perform activities of daily living, spent most of the day at home, and received periodic home visits for medical care. His last hospitalization was in February 2016 for approximately 1 month due to aspiration pneumonia. Only oral commensal bacteria were cultured from his sputum during his last hospitalization. In July 2016, he was hospitalized again for aspiration pneumonia. The sputum smears obtained on the first day of admission showed the presence of polymicrobial, normal oral bacteria and polymorphonuclear leukocytes. Subsequent cultures from this sputum showed normal oral bacteria as well as a few K. pneumoniae, with high levels of resistance to all antimicrobial agents except for minocycline. Results of examination of blood culture obtained on admission were negative. Other cultures were not examined. The patient had no history of travel to other countries and had never left Japan. Ampicillin/sulbactam was started at the time of hospitalization. On the 4th day of hospitalization, the antimicrobial agent was changed to cefepime because the clinical course was exacerbated. After the 5th day, the patient’s clinical course improved, and this treatment was continued until the 12th day. The antimicrobial was not changed when K. pneumoniae was observed on a sputum culture collected on admission. The patient was discharged after his aspiration pneumonia had been successfully treated. Despite administering antibiotics that are generally not effective against K. pneumoniae, K. pneumoniae was not detected from his sputum after treatment.
During laboratory investigation, we found that gram-negative bacillus grew on 5% sheep blood agar. Carbapenem-resistant K. pneumoniae was identified by Phoenix100 and NMIC/ID-208 panel (Becton, Dickinson and Company). Minimum inhibitory concentration of both meropenem and imipenem was > 8 μg/ml, and the sodium mercaptoacetate disk test result was negative. The modified Hodge test (using ertapenem disk) result was positive for K. pneumoniae TUM16641. The DNA of K. pneumoniae TUM16641 was sequenced using MiSeq (Illumina, Inc., CA, USA), and the DNA library for Illumia MiSeq sequencing was prepared using the Nextera XT Library Prep Kit (Illumina). The Nextera XT DNA library was sequenced in a paired-end 300 cycles mode on MiSeq using 600 cycles Reagent Kit v3 (Illumina). Draft genomes (contigs) were obtained using CLC Genomics Workbench (Qiagen). TUM16641 belonged to sequence type (ST) 258 analyzed by multilocus sequence typing. A carbapenemase gene, blaKPC-2, was detected in the contigs. To characterize a blaKPC-2 carrying plasmid, we used a long reads sequencing platform, MinION (Oxford Nanopore Technologies [ONT], Oxford Science Park, UK). A MinION library was prepared from K. pneumoniae TUM16641 genomic DNA using Ligation Sequencing Kit 1D (SQK-LSK108) and Native Barcoding Kit (EXP-NBD103) (ONT). The MinION DNA library was sequenced using Flow Cell R9.4 (FLOW-MIN106) (ONT). The complete plasmid sequence was obtained using SPAdes assemblers in combination with MiSeq and MinION data []. The sequencing data showed that the K. pneumoniae TUM16641 harbored a hybrid replicon of the IncX3 and IncU plasmid (pMTY16641_IncX3-IncU) carrying blaKPC-2 (Fig. ). The nucleotide sequence of pMTY16641_IncX3-IncU plasmid (GenBank accession number BFCA01000004) highly resembled that of pKP13d, pKP1194a, and pKP64477d of K. pneumoniae obtained from different reports in Brazil (Fig. ). K. pneumoniae TUM16641 also harbored two antibiotic resistance gene carrying plasmids, a hybrid replicon of IncFIB and IncFII plasmid (pMTY16641_IncFIB-IncFII) carrying aadA2, aph(3′)-Ia, mph(A), catA, sul1, and dfrA12 and a IncA/C2 plasmid (pMTY16641_IncA/C2) carrying aac(3′)-IId, rmtB, strA, strB, blaTEM-1B, blaCTX-M-14, sul2, tet(G) (Table ).
The GenBank accession number for the draft whole-genome sequence data of the K. pneumoniae TUM16641 is DRR076334.
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pmc-6322325-1
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An 8-month-old, 11.0-kg, sexually intact male French bulldog was presented on day 1 with a 4-month history of intermittent diarrhoea and a 7-day history of focal seizures that had been observed almost every day for 7 days. Stool consistency had been very soft to watery, and stool frequency had been > 7 times/day. Blood and mucus had been observed in the faeces. Thus, diarrhoea was considered to be induced by colitis. Four months prior to the current presentation, a faecal sample of the dog was subjected to real-time PCR analysis (IDEXX Laboratories, Inc., Tokyo, Japan) for Cryptosporidium spp., Giardia spp., Clostridium perfringens α toxin, Clostridium difficile toxin A&B, Campylobacter jejuni, Campylobacter coli, Salmonella spp., Canine parvovirus type 2, canine distemper virus and canine enteric coronavirus genes by a veterinary practitioner; a positive reaction for Campylobacter jejuni was detected in the analysis. The dog was treated with tylosin (Tylan, Eli Lilly Japan K.K., Kobe, Japan; 10 mg/kg PO, q12h) for 7 days by a veterinary practitioner; however, stool conditions did not improve. Administration of an antidiarrhoeal (Diabuster, Kyuritsu, Tokyo, Japan; 1 tablet PO, q12h) containing berberine tannate, bismuth subnitrate, geranium herb, nutgalls and scopolia extract, and an antiflatulent (Bioymbuster, Kyuritsu, Tokyo, Japan; 1 tablet PO, q12h) containing Bacillus coagulans, Bifidobacterium longuin, Lactobacillus acidophilus, Streptococcus faecalis and pancreatin, improved stool conditions. However, once these drugs were discontinued, the diarrhoea recurred.
On day 1, physical and clinical examinations, including a complete blood count (CBC), a serum biochemical analysis, radiography, an abdominal ultrasound and faecal examination, did not reveal any specific causes for chronic diarrhoea and focal seizures. A faecal sample was subjected to real-time PCR analysis (IDEXX Laboratories, Inc.) to investigate an infectious cause of diarrhoea. Meanwhile, the dog was administered erythromycin (Erythromycin, Sawai Pharmaceutical, Osaka, Japan; 10 mg/kg PO, q12h) for 14 days based on the positive result for C. jejuni infection 4 months earlier.
On day 2, real-time PCR analysis of a faecal sample collected on day 1 was found to be positive for C. difficile toxin A&B genes and negative for other pathogens. The presence of C. difficile antigen and toxin A&B proteins in a faecal sample collected on day 1 was also confirmed by an immunochromatographic test kit (Techlab C. Diff Quick Chek Complete, Alere, Chiba, Japan).
In the follow-up visit on day 16, stool conditions did not improve after administration of erythromycin in the dog. Based on the clinical and investigative findings, diarrhoea in the dog was considered to be induced by C. difficile-associated colitis. Treatment with metronidazole was proposed; however, the owner rejected this treatment because of the potential for metronidazole-induced neuropathy. To investigate the cause of focal seizures, computed tomography and magnetic resonance imaging were performed. Mild ventriculomegaly was detected in the brain of the dog on imaging, but it was unclear whether the lesion was related to the seizures. After initiating treatment with zonisamide (Consave, DS Pharma Animal Health, Osaka, Japan; 10 mg/kg PO, q12h), the seizure frequency decreased.
On day 25, the dog still had large bowel diarrhoea. Real-time PCR analysis and immunochromatography confirmed that C. difficile antigen and toxin A&B genes and proteins were still positive in a faecal sample collected on day 25. Therefore, instead of treatment with metronidazole, oral faecal microbiota transplantation (FMT) was performed after obtaining written informed consent from the owner. This treatment was approved by the Research Ethics Committee of Tokyo University of Agriculture and Technology. Fresh faeces were collected from a 9-year-old, 11.0-kg, sexually intact healthy male beagle maintained for research purposes. The healthy dog was housed in a cage and fed a commercial diet (Science Diet Adult, Hill’s-Colgate Ltd., Tokyo, Japan) once daily. Water was provided ad libitum. Physical and clinical examinations, including a CBC, a serum biochemical analysis, radiography, an abdominal ultrasound and faecal examination, did not find any abnormalities in the healthy dog, and real-time PCR analysis of a faecal sample did not detect any pathogens. Immediately after faecal collection, approximately 60 g of faeces was dissolved in 50 mL of tap water. The faecal solution was filtered through a medical gauze pad twice. A total of 30 mL of a filtered faecal solution was obtained and orally administered to the recipient dog using a syringe.
Stool consistency became normal, and stool frequency was reduced to 4–5 times/day 2–3 days after oral FMT. Faecal blood and mucus were not observed after oral FMT. Real-time PCR analysis of a faecal sample collected at 7 days after oral FMT (day 32) was negative for C. difficile toxin A&B genes. Further real-time PCR analysis of faecal samples collected on days 61 and 149 confirmed that C. difficile toxin A&B genes were still negative. The absence of C. difficile antigen and toxin A&B proteins was also verified in the faecal samples by an immunochromatographic test kit after oral FMT. In addition, diarrhoea did not recur after oral FMT and further medications were unnecessary. Stool conditions are still normal on day 190.
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pmc-6322330-1
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A 65-year-old male attended the eye clinic with a past history of sudden reduction of vision in the right eye (RE) when he was 52 years old, followed a year later by sudden reduction of vision in the left eye (LE). Extensive investigation in a tertiary referral centre had identified elevated homocysteine levels resulting from a gene mutation for the enzyme methylenetetrahydrofolate reductase (MTHFR) and hypercholesterolaemia. In the absence of other findings a diagnosis of bilateral NAION was made.
When seen in our clinic he had LogMar visual acuity of 0.2 in the RE and 0.0 in the LE. Colour vision was reduced in the right eye. Three out of 17 Ishihara test plates were correctly identified in the RE and 16 in the LE. Visual field testing (Humphrey 24–2) showed absolute superior and inferior nasal scotomas in the RE, and superior and inferior altitudinal scotomas with preservation of the central 20 degrees in the LE (Fig. ). The optic nerves were pale with minimal cupping. In view of these unusual findings, further tests were arranged. A carotid ultrasound scan was normal as was an MRI scan of the optic nerves and brain. Referral to a clinical geneticist was arranged. The typical genetic mutations for Lebers herediary optic neuropathy and familial hypercholesterolaemia were not found, however a homozygous mutation in the GPIbα (VNTR B allele) was identified. The hyperlipidaemia and elevated homocysteine levels were managed by the endocrine team. Medication consisted of atorvastatin 40 mg ON, folic acid 400 mcg OD and clopidogrel 75 mg OD.
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pmc-6322348-1
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A 36-year-old white male patient suffering from headache and abdominal pain presented at our emergency department. Initial ECG showed a sinus rhythm (40/min.) with a single T-wave inversion in lead V2, and an incomplete right bundle branch block. Thrombocytes were decreased with 71.000/μl (normal range 150.000–450.000/μl), creatinine was elevated (2.0 mg/dl) with a maximum increase to 3.0 mg/dl (normal range 0.6–1.1 mg/dl) and massive proteinuria. C-reactive protein was also elevated: 8.6 mg/dl (normal < 0.5 mg/dl). Puumala virus IgG ELISA turned out to be positive, and specific antibodies (IgG and IgM) could be detected in the serum, and confirmed by immunoassay, also see Additional file . The patient was admitted to the nephrology department for supportive therapy.
Six days later, the patient reported chest pain and dyspnea. High sensitivity troponin I rose up to 0.32 μg/l (normal range below 0.04 μg/l) with an increase of the creatinkinase to 319 U/l (normal max. 190 U/l), no dynamic ECG changes could be observed. The patient was admitted to the chest pain unit. Echocardiography revealed a normal left ventricular function (65%) without regional wall motion abnormalities, no pericardial effusion or valve abnormalities. Since creatinine has normalized in the meantime, coronary artery disease was ruled out by coronary CT angiography.
CMR for work-up of suspected myocarditis was performed using a 1.5 T Magnetom Aera (Siemens Health Care, Germany). Cine-SSFPs revealed normal LV-EF (60%) with no wall motion abnormalities. A modified Look-Locker inversion recovery product sequence (MOLLI, MyoMaps) was used for T1-mapping and performed in a single mid-ventricular short-axis (SAX) slice at mid-diastole, prior and after application of contrast agent according to current recommendations []. T2-mapping was performed in the corresponding mid-ventricular SAX before administration of contrast agent using an ECG-triggered T2-prepared single-shot steady-state free precession (SSFP) product sequence with multiple T2 preparation times []. Normal values: native T1 < 1000 ms, T2 < 50 ms. Analyses were made by cvi42 software (Circle, Canada). Late gadolinium enhancement (LGE) images were acquired after contrast administration (Gadobutrol 0.15 mmol/kg) using segmented inversion-recovery fast low angle shot (IR-FLASH).
Native T1-mapping demonstrated markedly elevated T1 values with preponderance in the inferoseptal wall (1068 ± 73 ms in the entire slice vs. 1122 ± 31 ms in the inferoseptal wall), also see Figs. and . Furthermore, T2-mapping revealed increased values (entire slice 52 ± 6 ms, inferoseptal wall 55 ± 6 ms), suggesting myocardial edema representing active myocardial inflammation by hantavirus infection. In contrast, the LGE image, potentially indicating irreversible myocardial damage if positive, in the corresponding slice was negative (Fig. ). Despite negative LGE, this patient was considered having hantavirus-induced myocarditis due to: 1) clinical symptoms, 2) increased cardiac biomarkers, 3) exclusion of CAD and 4) conspicuous native T1- and T2-mapping values detected by CMR.
In the next few weeks, the patient’s state of health rapidly improved and symptoms of chest pain and dyspnea disappeared. Five months later, the patient was followed up by the same CMR protocol: Substantial decrease of native T1 values (957 ± 58 ms in the entire slice vs. 971 ± 36 ms in the inferoseptal wall) and T2 values (entire slice 44 ± 5 ms, inferoseptal wall 45 ± 3 ms) in the mid-ventricular slice position could be observed, again LGE-negative, suggesting myocardial healing (Fig. d-f).
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pmc-6322805-1
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A 9-year-old female black-skinned patient presented with monomorphic,
erythematous/desquamative papular eruptions grouped in perioral and periorbital
regions for a year, with late progression onto the genital region (), not accompanied by any other
symptoms.
Due to the exuberance of lesions, the patient was experiencing important social
limitation, pictured by her distancing from groups of children’s recreation, parties
and school environment. Over the disease course, multiple treatments were tried,
including corticosteroids, imidazole and topical immunomodulators and systemic
antibiotic therapy with cephalosporins, but lesions had no remission.
Histopathological examination of a facial skin sample showed chronic and
granulomatous findings. Dermal edema, vascular ectasia and lymphohistiocytic
inflammatory infiltrates were noted around sebaceous follicles, configuring small
granulomas surrounded by occasional neutrophils ().
The initial presentation was devoid of symptoms, but the previous use of multiple
topical agents caused local irritation, burn and pinching complaints. Topical
tacrolimus 0.03% was prescribed under monotherapy, with significant improvement of
erythema after one month. The appearance of new lesions in upper trunk and left
upper limb in spite of the satisfactory facial response to therapy, led to the
association of oral azithromycin, 320mg/day for five days, which finally provided
disease remission ().
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pmc-6322945-1
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A 58-year-old man presented with a medical history significant for chronic myelogenous leukemia (CML) status after allogenic bone marrow transplant. Ocular history was significant for severe ocular graft-versus-host disease, keratoconjunctivitis sicca, and bilateral neurotrophic keratopathy. His left eye was recently treated for a culture-positive Streptococcus viridans corneal ulcer with hypopyon, and he recovered 20/60 acuity. The patient re-presented (Fig. A, left) with new, left large central corneal epithelial ulceration, 2 paracentral areas of corneal infiltration, and a 3.5-mm hypopyon. Hourly fortified cefazolin (50 mg/mL) and topical moxifloxacin were initiated. Three days after culture, microbiology identified growth of numerous Capnoctyophaga cynodegmi species. The patient reported, while celebrating his recovery from S. viridans keratitis, that he let his dog lick him all over his face, including his neurotrophic corneas. Four days after presentation, the patient developed Seidel-positive inferior paracentral perforation requiring an emergency glue procedure (Fig. A, middle). Because sensitivities for this rare pathogen require send-out evaluation, a review of previous Capnocytophaga case reports suggested treatment with topical clindamycin. Compounded clindamycin 5% was initiated hourly. The glue remained in place for 2 months and subsequently fell off. Visual acuity improved to 20/200. The globe remained intact, and the area of previous perforation had vascularized (Fig. A, right).
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pmc-6322945-2
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A 64-year-old woman with a history of rheumatoid arthritis being treated with rituximab infusions sustained an outdoor foreign body injury after using motorized landscaping equipment. She developed ocular irritation and decreased vision and was treated at an outside facility. She presented 1 month into treatment for consultation after having failed therapy with topical prednisolone acetate 1% and topical ciprofloxacin. Her left cornea disclosed several superior mid-stromal peripheral and tiny paracentral subepithelial infiltrates (Fig. B, left). A 1-mm hypopyon was present. Multiple Gram stains, potassium hydroxide (KOH) stains, and cultures obtained from epithelial scrapings over the areas of subepithelial infiltrates were unrevealing. Confocal examination demonstrated nonspecific inflammatory changes. The stromal lesions progressed deeper. Because the scattered superficial infiltrates were clinically concerning for satellite lesions, the patient was treated aggressively with topical, intrastromal, and oral antifungal therapy (including amphotericin B, voriconazole, and natamycin). Over the next 2 months, the patient developed progressive worsening of anterior chamber inflammation associated with endothelial plaques (Fig. B, middle). Aqueous fluid from 2 anterior chamber washout procedures as well as corneal punch biopsy and patch graft of the necrotic superior mid-stromal infiltrates (Fig. B, right), did not identify any organisms using aerobic and anaerobic media. A robust inflammatory reaction persisted after a patch graft. Aqueous fluid from a third washout procedure was sent to a Clinical Laboratory Improvement Amendments-certified laboratory for universal polymerase chain reaction for fungal genomes and tested negative. Residual aqueous fluid was sent to the Proctor Foundation for metagenomic deep sequencing (MDS). MDS is an unbiased high-throughput sequencing approach that interrogates all potential genomes in a clinical sample. MDS was performed as previously described. This study adhered to the tenets of the Declaration of Helsinki. The Institutional Review Board of the University of California, San Francisco, approved the study (16-19151), and informed consent was obtained from the patient. Two species of Capnocytophaga, Capnocytophaga canimorsus, and C. cynodegmi were identified (Fig. A). Orthogonal validation with partial 16S rRNA gene reverse transcription polymerase chain reaction and Sanger sequencing of the remaining RNA from the patient's aqueous specimen confirmed the presence of Capnocytophaga genome (Fig. B). The patient was placed on topical clindamycin 5% with subsequent complete resolution of inflammation and infiltration in 6 weeks. After the MDS results, the patient reported that she lives with numerous cats and dogs. Her acuity postresolution is hand motions from irregular astigmatism from the patch graft and dense cataract that progressed during the severe inflammatory episode. Penetrating keratoplasty with cataract surgery is planned.
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pmc-6323423-1
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A 57-year-old postmenopausal lady, para 0 with an unremarkable smear history, presented to the gynaecology department with a 3-month history of left-sided abdominal pain and frequency of micturition. She previously had a laparoscopic left-sided salpingo-oophrectomy for a benign mucinous cystoadenoma 3 years earlier. Clinical examination confirmed a pelvic mass arising from the pelvis. There were no features suggestive of an acute abdomen. An ultrasound scan demonstrated a large complex thick-walled cyst mid pelvis measuring 15 × 13 × 12 cm displacing the uterus to the right. There was an additional 7 × 6 cm complex cyst seen adjacent to this mass. Neither ovary was subsequently identified. The Ca125 was 8, giving a risk of malignancy index (RMI) of 24. A subsequent MRI pelvis was consistent with right ovarian cystic adenoma/cyst adenocarcinoma, and a bulky postmenopausal fibroid uterus containing multiple fibroids displaced to the right of the midline. The patient was referred to the MDT and total abdominal hysterectomy, right salpingo-oophrectomy, and omental biopsy were recommended in view of the potential for malignant diagnosis. This was completed uneventfully. At operation, the findings confirmed a multi-fibroid uterus with a large right-sided cystic mass. She made a good recovery postoperatively and a follow-up CT undertaken 6 months after surgery did not show any evidence of disease recurrence. She was commenced on letrozole 2.5mg daily in view of the histology results. Oestrogen results were not measured pre- or postoperatively.
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pmc-6323424-1
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A 24-year-old male of Asian descent reported to the oral medicine clinic at NYU College of Dentistry. His chief concern was a painless, slowly growing mass on his hard palate that he noticed a few weeks ago. The patient denied any significant medical issues or medications. He also denied any significant family history or any similar lesions in any of his immediate family members. The extraoral examination was within normal limits. Intraoral examination revealed a large exophytic mass of the right hard palate extending to the soft palate, yellowish in color, and soft to palpation (). The lesion measured approximately 5 × 4 cm and was oval-shaped. It was compressible and had a smooth surface with numerous small blood vessels. However, the mass did not blanch or feel pulsatile upon palpation, ruling out a vascular tumor. The lesion felt fixed with well-defined margins. The working or clinical diagnosis was lipoma. The likely differential diagnosis included lipoma, a cystic lesion or other soft tissue tumor, and pleomorphic adenoma. A 5 mm incisional punch biopsy was performed in the center of the mass (). On microscopic examination, a benign salivary gland tumor consisting of pools of plasmacytoid cells and numerous double-layered ducts was seen. The stroma was composed of significant areas (approximately 50%) of adipose tissue, along with several foci of hyalinization (Figures –). The final diagnosis rendered was pleomorphic adenoma with significant adipose tissue component. The patient was then referred to oral surgery for complete surgical excision. A CBCT was performed to further delineate the lesion and confirm its benign behavior. No other investigations or diagnostic tests were performed. Surgical excision was completed and the pathology findings were consistent with the incisional biopsy results of pleomorphic adenoma with significant adipose tissue component (also approximately 50%). Upon 2-year follow-up, the patient is doing well and has no recurrences.
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pmc-6323427-1
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Mdm A, a 36-year-old Chinese lady with alpha thalassaemia (HbH disease), presented to us with a one-week history of fever and chills. There was no localizing symptom of infection. Her physical examination findings were unremarkable.
Initial investigation () revealed pancytopaenia: hemoglobin (Hb) 7.3 g/dL (11–14.7 g/dL), white cell count (WC) 1.68 × 109/L (3.37 to 8.38 × 109/L), and platelet count 92 × 109/L (172–378 × 109/L). C-reactive protein was 24 while procalcitonin was 0.14. Renal function, liver enzymes, chest X-ray, and urinalysis were unremarkable.
She was given piperacillin-tazobactam empirically. Despite antibiotic, she remained febrile on day 3 of admission. Three sets of blood culture and urine culture were negative. Dengue NS 1 antigen was negative. Piperacillin-tazobactam was changed to carbapenem.
Computed tomography (CT) of the abdomen and pelvis was arranged to investigate the cause of fever. It showed hepatosplenomegaly but no intra-abdominal abscesses ().
The pancytopaenia progressively worsened (). Peripheral blood film showed marked leukopenia, thrombocytopenia, and significant anisopoikilocytosis with microcytosis and target and tear drop cells consistent with a picture of thalassaemia intermedia (). A bone marrow examination was therefore performed. Typical bone marrow finding in a patient with HbH disease alone would reveal a hypercellular marrow with erythroid hyperplasia and marked dyserythropoiesis. Mdm. A's marrow however was hypocellular with decreased cell trails on the aspirate (). There was marked erythroid hypoplasia with rare giant erythroblasts, and inclusion bodies were seen (). There were also increased numbers of macrophages with active haemophagocytosis (). There was no evidence of lymphoma on bone marrow examination. Haemophagocytosis is however not exclusive of HLH. It may also be present in critically ill patients. Mdm. A was diagnosed with HLH based on the fact that she fulfilled 5 out of the 9 diagnostic criteria of HLH used in the HLH 2004 trial: fever >38.5°C, splenomegaly, peripheral blood cytopaenia, haemophagocytosis in marrow, and ferritin >500 ng/ml.
She was then transferred to a tertiary hospital for further treatment. 16 mg of dexamethasone once daily was initiated. Dexamethasone dose was reduced by 50% every 5 days. After 5 days of 2 mg once daily dexamethasone, the steroid was stopped. She responded well to the 20-day course of dexamethasone. She was reviewed in the clinic once a week after discharge, and other than her baseline anaemia due to HbH disease, other cell lines have recovered. Parvovirus B19 PCR was noted to be positive during her clinic visit. Diagnosis of parvovirus-induced HLH was made. She remained well in the subsequent clinic follow-up.
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pmc-6323429-1
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A 62-year-old female with a medical history of generalized anxiety disorder and hyperlipidemia presented to our clinic for further evaluation of chronic nausea and chronic intermittent abdominal pain ongoing for over 10 years. She also reported occasional nonbloody, nonbilious emesis along with the nausea which was not exacerbated by oral intake. Her symptoms were refractory to oral Ondansetron, Metoclopramide, and Promethazine. She denied bloating, weight loss, or changes in bowel habits. Her past surgical history only included an uncomplicated laparoscopic cholecystectomy.
On physical exam her abdomen was soft and nontender with normoactive bowel sounds. Laboratory study results showed a hemoglobin level of 12.2 g/ml (normal 11-15.1 g/dl), total bilirubin level of 0.8 mg/dL (normal 0.3-1 mg/dl), alkaline phosphatase of 74 U/L (normal 32-91 U/L), aspartate aminotransferase (AST), and alanine aminotransferase (ALT) levels of 32 and 41 U/L, respectively (normal 15-41 U/L, 7-52 U/L). A random cortisol level was 12 mcg/dl.
She was initially sent for a CT angiogram of the abdomen with intravenous contrast which did not show any radiographic evidence of median arcuate syndrome. She then underwent a diagnostic esophagogastroduodenoscopy which revealed a normal duodenum (). An upper gastrointestinal series with small bowel follow through using barium contrast showed no evidence of gastric outlet obstruction; however, the duodenal course was abnormal, with the proximal portion looping back on itself in the right abdomen and extending superiorly to the level of the duodenal bulb () before crossing the midline with loops of small bowel in the left upper quadrant (). Based on these characteristic radiographic findings, the diagnosis of duodenum inversum was made and the patient was referred for possible surgical management. She underwent an exploratory laparotomy which showed proximal loops of jejunum adhered to the right lower quadrant and patulous appearing first and second portions of the duodenum (). An end-to-side duodenojejunostomy was then performed successfully. The patient had no procedure related complications and began tolerating oral intake at postoperative day 4. She was subsequently discharged home in good condition and remained symptom-free at follow-up.
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pmc-6323454-1
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A 49-year-old male patient was presented at our hospital with a difficulty in ambulation. Three days prior, the patient experienced discomfort in the anterior knee joint with no preceding injury, and the symptom progressed into pain the following day. There was no swelling of the joint, but the patient showed severe restriction in the range of motion due to pain. There was no tenderness at the medial and lateral femorotibial (FT) joint, and tenderness was only observed on the proximal side of the PF joint. The Lachman test, pivot-shift test, varus/valgus instability, and McMurray test were negative. Although symptoms temporarily improved with an intra-articular injection of xylocaine, catching of the proximal-lateral knee was subsequently observed while moving the leg from full extension to flexion.
Simple radiographs showed no abnormal findings, and MRI images revealed a soft tissue mass located superolaterally to the PF joint that exhibited an ill-defined border with its surroundings. Both T1- and T2-weighted images at high signal intensities revealed the soft tissue mass, while low signal intensity was noted under fat suppression and no contrast enhancement was noted under contrast imaging (). In addition, there were no abnormal findings in the blood examination.
The patient requested surgery due to his persistent symptoms and underwent knee arthroscopy. A large fat mass with mobility in the proximal to distal direction was observed on the anterior surface of the proximal-lateral PF joint. The mass macroscopically resembled fat, and the border with its surroundings was ill-defined. There were no other intra-articular findings, and a piece-by-piece resection was eventually performed (). Histologically, the mass consisted of connective tissue that was mainly composed of fatty tissue, and there were no findings that suggested the presence of lipoma, lipoma arborescens, or pigmented villonodular synovitis ().
The symptoms improved after surgery, and no symptoms were found thereafter, including anterior knee pain (AKP) at 3 years after surgery. There was no evidence of the preoperatively confirmed fatty mass in postoperative MRI imaging, and no recurring lesions were found ().
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pmc-6323477-1
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A 41-year-old African American female presented to the emergency department with a cough, dyspnea, fevers, chills, night sweats, and fatigue.
She had never experienced pulmonary symptoms two weeks before presentation when she developed a cough and fever and was prescribed oral levofloxacin for pneumonia by her primary care physician. Completing a 7-day course of antibiotics, with unabating symptoms and worsening dyspnea, she presented to the emergency department for further treatment.
The examination was remarkable for tachypnea with a respiratory rate of 30/min, hypoxemia with an oxygen saturation of 87% in room air, and diffuse bilateral crackles, without jugular venous distension, or lower extremity edema.
Blood test was significant for white blood cell count of 30,000/µL, lactic acid of 5 mEq/L, and a normal metabolic panel. Arterial blood gas revealed a pH of 7.17, PaCO2 of 50 mmHg, HCO3 of 19 mmol/L, PaO2 of 65.3 mmHg, and SaO2 of 87%.
A CT-PE of the chest showed bilateral extensive multifocal infiltrates with significant hilar and mediastinal lymphadenopathy and no evidence of pulmonary embolism.
She was intubated for respiratory distress and admitted to the medical intensive care unit, with a tentative diagnosis of sepsis secondary to pneumonia, and started on broad-spectrum antibiotics.
A parasite smear and initial blood cultures were negative.
Bronchoscopy done on the day of admission showed mild diffuse erythema without hemorrhage. Given the patient's repeated desaturation during the procedure, transbronchial biopsies were not performed and the procedure terminated early. Lavage was sent for cytology, bacterial, mycobacterial, and fungal stain, and culture.
An echocardiogram showed a hyperdynamic left ventricle with an estimated ejection fraction of 70% with severe right ventricular dilatation and hypokinesis. Right ventricular systolic pressure was estimated at 80 mmHg with a tricuspid annular plane systolic excursion (TAPSE) of 1.3.
She continued to worsen, with worsening hypoxia and difficulty with mechanical ventilation, ultimately requiring airway pressure release ventilation and inhaled epoprostenol. Bronchoalveolar lavage remained negative, for bacteria, fungi, or Pneumocystis jirovecii sp.
Workup was expanded to include fungal blood cultures, histoplasma and coccidioides serology, and viral respiratory panel. Respiratory syncytial virus, adenovirus, influenza, parainfluenza, human metapneumovirus, human immunodeficiency virus (HIV), Ebstein–Barr virus (EBV), and cytomegalovirus (CMV) were negative. Leptospira and pertussis remained negative. Noninfectious workup along autoimmune lines including antinuclear antibody (ANA), anti-neutrophilic cytoplasmic antibody (ANCA), rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP), and anti-Smith, anti-SCL70, anti-myeloperoxidase (anti-MPO), anti-ribonucleoprotein (anti-RNP), and anti-PR3 antibodies all returned negative, with angiotensin-converting enzyme (ACE) levels on the high end of normal.
She continued to worsen, requiring hemodynamic support with norepinephrine, epinephrine, and vasopressin, with worsening hepatic function and oliguric renal failure.
On the third hospital day, she continued to be febrile. Morning ABG showed a pH of 6.99, PaCO2 of 47.4 mmHg, HCO3 of 10 mmol/L, PaO2 of 88.2 mmHg, SO2 of 83.2%, and lactic acid level of 9 mEq/L; after prompt discussion with the family, the decision was made to start the patient on continuous renal replacement therapy (CRRT) and consider extracorporeal membrane oxygenation (ECMO) support.
However, the patient developed pulseless electrical activity (PEA) arrest before CRRT could begin, with the eventual demise of the patient.
An autopsy revealed noncaseating granulomata in hilar and mediastinal lymph nodes and pulmonary, splenic, and hepatic parenchyma. Gram, Ziehl–Neelsen, and special stains were negative for bacterial, mycobacterial, fungal, or parasitic organisms.
Acid-fast bacillary and fungal cultures remained negative. No crystals were seen on polarized microscopy.
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pmc-6323478-1
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An 84-year-old woman with angina, diabetes mellitus (DM), hypertension, and Alzheimer's disease was referred to our institution for suspected right lung cancer. Physical examination revealed the following: body height, 150 cm; weight, 68.4 kg; and body mass index, 30.4. Chest computed tomography (CT) revealed a 1.8 cm nodular lesion with an ill-defined margin in the right lower lobe, suggesting lung cancer without metastasis (). Three-dimensional CT revealed normal bronchial anatomy (). Her preoperative vital capacity was 1.77 L as assessed using a spirogram, and the forced expiratory volume in 1 s was 1.35 L. Subsequently, we performed thoracoscopic right lower lobectomy without mediastinal lymph node dissection. The anesthetic and operative times were 189 and 92 min, respectively, with minimal blood loss. The total amount of intraoperative fluid replacement was 1000 mL. Final pathological finding was adenocarcinoma with hilar lymph node metastasis diagnosed as pT1bN1M0 (p-stage IIB according to the 8th IASLC classification criteria) []. Extubation was safely performed in the operating room, and she was followed up in the intensive care unit. However, postoperatively, she complained of dyspnea without chest pain and developed arterial oxygen desaturation 12 h postoperatively. Oxygen saturation reduced to 86% despite the administration of 10 L/min oxygen, corresponding to a PaO2 of 54 mmHg. An emergency chest computed tomography (CT) revealed the right upper bronchial stenosis with hilar peribronchovascular soft tissue edema (PSTE) because the middle lung lobe had been pushed upward and forward, and the right upper lung lobe had twisted dorsally (Figures and ). Three-dimensional CT scan showed severe bronchial stenosis (). Emergency bronchoscopy revealed severe right upper bronchial stenosis with an eccentric rotation and severe edema (). Echocardiography and electrocardiography revealed a cardiac ejection fraction of 55% and normal diameter of the inferior vena cava, thus ruling out ischemic heart disease. Subsequent emergency blood tests revealed normal hepatorenal function and serum albumin level. She was diagnosed with localized right upper bronchial obstruction with bronchial edema and hilar PSTE due to right upper lobe torsion after right lower lobectomy. There was no evidence of venous congestion, hemorrhagic infarction, necrotic findings, increased pleural effusion, or atelectasis. Therefore, we decided conservative treatment as primary care. ARF was treated using noninvasive positive pressure ventilation for 2 days and 40 mg methylprednisolone injection for 3 days. A follow-up chest CT on postoperative day (POD) 3 revealed improvement of the right upper bronchial stenosis; she subsequently received 30 mg oral predonine for 7 days (Figures –). 3D-CT on POD 14 showed the counterclockwise rotation of right upper lung lobe but obvious improvement of stenosis of the bronchus (). The chest tube was removed on POD 1. She was discharged on POD 16, after recovery. At the 4-month follow-up, she exhibited good health without any evidence of right upper bronchial stenosis.
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pmc-6323496-1
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The first patient was a 64-year-old male presenting with painful burning pain at the bottom of his feet for six months. The pain extended from his feet up to his legs, hips, and back in a sharp shooting manner. It was constant and it was so severe that it limited his activities. As a child, he started walking later than his peers and he was always the slowest runner. He used leg braces because his knees were “together” and he had a surgery for it at age 15. He had occasional muscle cramps and fasciculations as a child. His mother was always clumsy in her feet as well. He had no siblings or children. He had a CMT examination score of 8 out of 28. On exam, there was pes cavus bilaterally and tight Achilles tendons. His feet could not be easily brought into a neutral position. There was atrophy of the hands and feet, length-dependent pinprick and vibratory sense loss, and absent reflexes. MRI of the lumbar spine was unremarkable. Electrophysiological studies revealed moderate chronic sensorimotor, axonal polyneuropathy (). There were absent sensory responses in the bilateral sural and superficial peroneal nerves. Motor studies showed reduced amplitude in the left tibial nerve and reduced conduction velocities ranging from 32-36 m/s in the bilateral peroneal nerves and left tibial nerve. F wave in the bilateral peroneal nerve showed prolonged latency. F wave in the bilateral tibial nerve was absent. Sequencing of 72 neuropathy genes [] showed one copy of a pathogenic variant, T118M in the PMP22 gene.
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pmc-6323496-2
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The second patient was a 73-year-old man from Cuba presenting with leg pain which he described as a constant burning pain in his feet and aching pain in his legs. He was never a fast runner as a child and he was not athletic. His sister had similar symptoms of flat and painful feet. Exam revealed flat feet (), absent reflexes, and absent vibratory sense at the toes and reduced at the ankles. Electrophysiological study was unremarkable except for a mildly reduced peroneal nerve conduction velocity at the fibular head (). CMT examination score was 6 out of 28. Genetic testing revealed heterozygous T118M variant of the PMP22 gene and heterozygous R275L variant of the SLC52A2 gene. The sister was unable to undergo genetic testing.
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pmc-6323496-3
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The third patient was a 56-year-old male with past medical history of Sjögren's syndrome and rheumatoid arthritis who presented with chronic severe burning pain in the hands and feet necessitating the chronic use of narcotics to allow him to continue his profession. As a child he did have some difficulties with coordination and playing basketball. He had a daughter who also had flat feet and not athletic. He did not have other siblings. Exam showed decreased vibratory sense in the toes and flat feet with low arches (). Reflexes were present. CMT examination score was 2 out of 28. Electrophysiological study was unremarkable except for a mildly reduced tibial motor conduction velocity at the popliteal fossa (). Skin biopsy of the right distal leg and proximal thigh revealed normal epidermal small fiber densities. Sjögren's syndrome profile showed positive salivary protein IgA antibodies, parotid specific protein IgG, IgA, IgM antibodies, positive rheumatoid factor, and anticyclic citrullinated peptide antibody. ANA, double stranded DNA antibody, TSH, and free T4 were negative. Genetic testing showed heterozygous T118M variant of the PMP22 gene and heterozygous Y22C variant of the TFG gene (c.98 A> G). The daughter declined genetic testing.
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pmc-6323503-1
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A 48-year-old man was admitted to the intensive care unit with pneumonia and septic shock. He had no medical history, history of surgeries, or known allergies. He was not taking any medications. He worked as a pipefitter; otherwise, his social history was unremarkable. He had no significant family history. He tested positive for influenza B. He was treated with oseltamivir, vancomycin, piperacillin-tazobactam, and azithromycin. Chest X-ray showed patchy airspace disease in the right lung and focal consolidation in the left. Blood cultures returned positive for Streptococcus pneumoniae.
His absolute neutrophil count was 0, and peripheral flow cell cytometry showed hairy-cell leukemia, for which he received high-dose corticosteroids and rituximab.
He developed anuric acute kidney failure requiring hemodialysis and marked elevation of liver function tests. On day 5, he had new fever, for which piperacillin/tazobactam was changed to meropenem. On day 9, blood cultures were positive for Candida albicans. Caspofungin was added. Chest CT revealed multifocal pneumonia. Bronchoscopy showed erythematous airways with minimal secretions. Bronchoalveolar lavage (BAL) galactomannan was strongly positive in the left lower lobe and negative in the right lower lobe. Serum galactomannan was positive x2 (). BAL bacterial and fungal cultures were positive only for C. albicans.
Isavuconazole was added on day 15 for probable invasive aspergillosis in the setting of multiorgan, including kidney (persistently anuric), and liver (bilirubin level of 15 mg/dL), and failure. He had massive hemoptysis and died one day after. Autopsy showed disseminated mucormycosis (Figures –). Culture identified the species as Apophysomyces elegans.
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pmc-6323504-1
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A 73-year-old male veteran presented with recurrent syncope and falls. He had decreased appetite, thirst, and urine output in the setting of progressive abdominal distention, pruritus, and painless jaundice in the prior month. His past medical history was significant for obesity (BMI=40.1), hypertension, and benign prostatic hyperplasia (BPH) with pertinent medications of ibuprofen (200 mg QID), furosemide (40 mg BID), losartan, doxazosin, and finasteride. A detailed dietary history prior to admission was lacking. Physical exam was notable for diffuse jaundice, abdominal distention, and severe mid-thoracic back pain later confirmed to be T6/T7 vertebral fractures.
Initial laboratory results showed stage 3 AKI with serum creatinine (SCr) elevated to 8.98 mg/dL from a baseline of 1.04 mg/dL. Supporting laboratory findings included elevations in phosphorus (7.6 mg/dL), parathyroid hormone (319.7 pg/mL), and low calcium (7.6 mg/dL), ionized calcium (0.93 mmol/L), albumin (2.8 g/dL), and 25-hydroxy vitamin D (16.4 ng/mL). There was an anion gap (21 mEq/L) with pH= 7.145 confirmed with arterial blood gas. Jaundice workup revealed an obstructive pattern with elevated total bilirubin (5.4 mg/dL), direct bilirubin (3.7 mg/dL), AST (79 U/L), ALT (140 U/L), alkaline phosphatase (392 U/L), GGT (214 U/L), and lipase (690 U/L). Soon after admission, he developed hypotension necessitating vasopressors and broad-spectrum antibiotics for presumed septic shock. AKI workup revealed deteriorating kidney function and development of anuria, requiring intermittent hemodialysis.
The etiology of renal failure remained unclear. Urine testing showed nephrotic range proteinuria, many bacteria, no red blood cells, no crystals, few hyaline casts, and no granular casts. A renal biopsy was performed and showed diffuse calcium oxalate crystal deposition with severe acute tubular injury (ATN) and mild interstitial fibrosis (). An evident cause of oxalate nephropathy was unclear, although it was suspected that pancreatic insufficiency leading to fat malabsorption and increased intestinal absorption of oxalate were responsible. Fecal elastase returned low at 54 μg Elastase/g stool to support pancreatic insufficiency while abdominal computed tomography (CT) without contrast showed exocrine atrophy of the pancreas. Serum oxalate was 3.6 mmol/L (normal range 1.0-3.0 mmol/L). A low-fat, low-oxalate diet with calcium citrate and pancrelipase supplementation was started.
Concurrently, a jaundice workup began with abdominal ultrasound which showed hepatomegaly, splenomegaly, numerous gallstones, and no inferior vena cava or portal circulation thrombosis. Of note, the common bile duct was not visibly dilated, raising concern for an upstream malignant stricture. MRCP showed marked dilation of the left intrahepatic biliary tree with abrupt termination and mild dilation of the right intrahepatic biliary tree, further raising suspicion of malignancy. ERCP with stenting to the right intrahepatic biliary tree duct was performed. Cytology, a markedly elevated CA 19-9, and imaging confirmed the diagnosis of cholangiocarcinoma (Klatskin tumor). Medical oncology discussed chemotherapy and radiation as alternative treatments, but the patient elected to enter hospice. His kidney function ultimately improved with return of nonoliguric urine output, cessation of dialysis, and improved creatinine to 2.45mg/dL and eGFR of 26 ml/min/1.73m3 at discharge.
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pmc-6323504-2
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A 64-year-old male presented from clinic after routine lab monitoring showed new AKI and hyperkalemia. He was asymptomatic other than fatigue with no prior history of kidney disease. Past medical history was significant for hypertension, gout, and cholangiocarcinoma diagnosed in 2003. His cancer was in remission after chemotherapy and surgical interventions including complete excision of the extrahepatic biliary tree, Roux-en-Y hepaticojejunostomy, and cholecystectomy. He developed chronic pancreatitis and insulin-dependent diabetes postoperatively. Pertinent medications included losartan, triamterene-hydrochlorothiazide, and insulin. Admission vitals were notable for BP 165/71 and he was euvolemic on physical exam.
Laboratory workup revealed stage 3 nonoliguric AKI with serum creatinine of 4.61 mg/dL elevated from a stable baseline of 0.94 mg/dL. Supporting labs included elevations in potassium (5.4 mEq/dL), phosphorus (6.9 mg/dL), uric acid (10.5 mg/dL) and low bicarbonate (16 mEq/L), normocytic anemia (hemoglobin 9.9 g/dL), and hypoglycemia (blood sugar 32 mg/dL). CA19-9 was elevated to 51, but this was stably elevated and not felt secondary to signify recurrent disease. His hemoglobin A1C was 5.4%. Urine analysis showed 2-3 WBC/hpf. The etiology of his AKI was unclear but AIN was considered given his use of triamterene and leukocytes on urine microscopy.
Subsequent renal biopsy showed severe, chronic active interstitial nephritis, severe interstitial fibrosis and tubular atrophy, and oxalate nephropathy (). The oxalate nephropathy was believed secondary to enteric hyperoxaluria due to fat malabsorption from chronic pancreatitis and Roux-en-Y bypass. Followup 24-hour urine collection showed high oxalate excretion (90 mg) with low calcium (53 mg) and citrate (<28 mg) consistent with hyperoxaluria. 24-hour fecal fat was elevated at 26.3 g suggesting pancreatic insufficiency. Intermittent hemodialysis was initiated. He was started on a low oxalate diet with supplementation of pancrelipase, calcium citrate, and potassium citrate. Serum oxalate improved from 11.0 μmol/L to 7.9 μmol/L with these interventions. Serum creatinine peaked to 5.37 mg/dL before plateauing with cessation of dialysis. After further education and diet changes, his creatinine improved and plateaued to 3.57 mg/dL two years after admission.
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pmc-6323525-1
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A 69-year-old Caucasian male with a past medical history of hypertension, oromandibular dystonia treated with Botox, and recent diagnosis of gastroesophageal reflux disease (GERD) presented to his primary care provider noting an area of his left chin that was numb. The area was small and could be covered with 1 finger. He followed up acutely three weeks later with significant dysphagia for solids, but not liquids. He was urgently referred for an upper endoscopy. Upper endoscopy revealed LA Class D esophagitis with ulceration in the distal esophagus, and biopsies showed inflamed glandular mucosa with at least high-grade dysplasia. These findings were consistent with a diagnosis of Barrett's esophagus secondary to GERD.
Three weeks after his upper endoscopy, he presented to the emergency department with right jaw pain and swelling after hitting his jaw on a work bench. A CT revealed right mandibular angle fracture and coronoid fracture (). The facial trauma team was consulted and, secondary to his oromandibular dystonia, he was discharged on a liquid diet with Augmentin, Peridex, and close follow-up. He was seen in the clinic a week later and denied trismus, malocclusion, or difficulty with his liquid diet. On examination, he was found to have an exophytic mass of the right retromolar trigone, which he noted his teeth had been hitting. This mass was present before his fracture and had gotten larger over time. This mass was biopsied in the clinic and came back as likely metastatic adenocarcinoma ().
The patient underwent a second upper endoscopy. Biopsy taken during this second endoscopy was consistent with a moderately differentiated adenocarcinoma of the distal esophagus. A PET/CT revealed a large, hypermetabolic distal esophageal mass consistent with the given diagnosis of esophageal adenocarcinoma. Hypermetabolic lesions involving the regional lymph nodes, lungs, spine, and right mandible, as shown in , were found on PET/CT. These findings were consistent with a Stage IV, TX, NX, M1, and G2, esophageal adenocarcinoma. At that time, he denied smoking, alcohol, illicit drugs, and/or exposure to radiation and carcinogenic chemicals.
The hypermetabolic area of the right mandible, in conjunction with the previous retromolar trigone biopsy, confirmed a likely pathological fracture of the mandible secondary to metastatic esophageal adenocarcinoma. The advanced stage of the cancer made him a poor candidate for surgical intervention for either primary tumor or mandibular metastasis. He was referred for palliative chemoradiotherapy. He passed away one month after diagnosis.
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pmc-6323534-1
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A 60-year-old male, with a pre-existing diagnosis of Loeys-Dietz type 2, presented to the emergency room with left-sided chest pain for three weeks. He had a sudden onset of left-sided chest pain around 3 weeks ago, which was described as stabbing in nature and was 6/10 in intensity. It radiated to the back occasionally, was exaggerated by lying down, and did not increase upon exertion.
He was diagnosed with Loeys-Dietz syndrome in 2005. Genetic testing revealed a TGFBR2 mutation specifically R460H. In 2006, he underwent a prophylactic aortic root replacement for an aortic root aneurysm measuring 4.6 cm with a porcine bioprosthesis. On a routine two-year follow-up, a CT showed a left coronary artery pseudoaneurysm secondary to a left coronary artery dehiscence. He underwent another aortic root replacement and single coronary bypass with an aortosaphenous vein graft. Intraoperative findings revealed a left coronary artery sinus that had detached from the aortic graft, with increased the friable aortic tissue preventing a patch repair, necessitating an aortic replacement. In addition, other aneurysms included a right popliteal aneurysm measuring 4.4 cm, AAA measuring 3.5 cm, left internal carotid artery aneurysm measuring 9 mm, left subclavian artery aneurysm measuring 3.6 cm, and right ICA aneurysm measuring 6 mm.
On arrival to our emergency room, his vital signs were stable. Physical exam and electrocardiogram were unremarkable, serial cardiac enzymes were negative, and additional lab work-up was negative. Chest X-ray was obtained and was negative for any acute cardio pulmonary abnormalities. Keeping in mind his medical and surgical history, a cardiac CT scan with IV contrast was ordered which () revealed an aneurysm of the left subclavian artery, a sinus of Valsalva aneurysm arising from the right lateral aspect of the aorta measuring 2.4 × 3.8 × 4.0 cm and the right coronary artery arising from this aneurysm. A three-dimensional reconstruction of this pathology is shown in . This was compared to a CT scan done approximately 2 years ago which was within normal limits, showing an aortic root replacement with stable postoperative changes. He was transferred to a specialized facility for surgical repair of the aneurysm.
He underwent a redo sternotomy where the superior vena cava was cannulated, a vent was placed in the right superior pulmonary vein, the aorta was clamped, and the heart was arrested. A pseudoaneurysm was identified and recognized to be caused by dehiscence of the right coronary artery from a large defect in the old Freestyle graft. Patch and reimplantation of the right coronary artery were considered, but this was unsuccessful in prior operations. Replacement of the old prosthesis was decided upon.
The old Freestyle was excised, and the old saphenous vein interposition graft to the left coronary was detached from it. The annulus was then rimmed with mattress sutures which were passed through the base of a Valsalva graft then a magna pericardial valve. The valve and graft were lowered, and the sutures tied in and cut. The old vein graft to the left coronary could not be directly implanted, so a segment of autologous saphenous vein was interposed. The distal end of the Valsalva graft was grafted to the ascending aorta, and the right coronary artery was anastomosed to the Valsalva.
Though the heart was resuscitated, there was significant bleeding from the undersurface of the right coronary, which was not repairable. The heart had to be rearrested, and the right coronary anastomosis was taken down. The ostium of the right coronary artery had become quite macerated, so it was oversewn, opened longitudinally, and another segment of autologous saphenous vein was grafted onto it. The proximal end of that vein graft was joined to the Valsalva graft.
The aortic clamp was released again and the heart resuscitated. The patient was warmed and weaned from bypass. Hemostasis was achieved. The heart initially seemed to be functioning normally, but when LV function worsened, it introduced the possibility that the interposition vein graft to the left coronary graft was stenotic. Accordingly, the interposition graft was revised which successfully addressed the stenosis. The heart was resuscitated again, the patient was weaned from bypass uneventfully and hemostasis was achieved. The postoperative course was unremarkable. The patient received a predischarge CT scan which showed no residual pseudoaneurysm. Coronary reimplantation grafts to the RCA and left main were patent. Additionally, the left main graft had approximately 50% stenosis due to mild kinking. The patient was ultimately discharged in a stable condition.
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pmc-6323657-1
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A 58-year-old Japanese man with an 8-year history of T2DM had a symptom of pain in his right lower leg and visited the emergency room in Kawasaki Medical School. Previously, he had felt the same pain in the same region when his blood glucose was very high and thereby he was diagnosed as having T2DM 8 years before.
He had acute osteomyelitis in his right lower leg when he was a baby. Except for this, he had no past history. He had no remarkable family history. He was a barber; he smoked tobacco (pack-years = 0.75 pack/day × 40 years) and he drank alcohol every day. After the episode of acute osteomyelitis when he was a baby, there was no problem in his legs until he had general fatigue and felt pain in his right lower leg at the age of 50. He visited the emergency room. His vital signs were as follows: heart rate 76 beats/minute, blood pressure 116/70 mmHg, and body temperature 36.4 °C. He had a symptom of slight local swelling and heat sensation in the same area with pain in his right lower leg, but there were no findings in physical and neurological examinations. In addition, there was no ulcer or injury on his skin surface. Laboratory data (Table ) were as follows: white blood cell count, 7400/μL (neutrophil 64.7%); C-reactive protein (CRP), 2.50 mg/dl; plasma glucose, 382 mg/dL; hemoglobin A1c (HbA1c), 11.7%. He was diagnosed as having T2DM, but he had no diabetic complications. Magnetic resonance imaging (MRI) of his lower limbs showed an abscess and inflammatory change in his right lower leg (Fig. ). An axial T1-weighted (T1W) image of his right lower leg showed a slightly lower intensity, and an axial T2-weighted (T2W) image showed a markedly higher intensity (Fig. , upper panels). Based on these findings, we made a diagnosis of acute exacerbation of chronic osteomyelitis and T2DM. We thought that it would be better to hospitalize him and start administering antibiotics via a drip, but he did not agree to the hospitalization. Therefore, as an alternative, we started 300 mg/day of cefcapene pivoxil hydrochloride hydrate and insulin therapy (18 units of aspart) on an out-patient basis. After starting insulin therapy, his blood glucose level gradually decreased, and his leg pain was also gradually mitigated. Finally, his leg pain disappeared 2 weeks later. His CRP became within normal range, and 3 months later the focus in his right lower leg was markedly reduced on MRI. In addition, the focus was not detected in ultrasonography of the right tibia site. Just in case, however, we continued antibiotics therapy for 4 months. Since his glycemic control was improved 2 months later, we stopped insulin therapy and started orally administered anti-diabetic drugs.
He was then followed up as an out-patient with T2DM for approximately 8 years. The medication at that time was 1000 mg/day of metformin, 25 mg/day of alogliptin, 15 mg/day of pioglitazone, and 50 mg/day of ipragliflozin. However, he did not take the medicine for approximately 8 months on his own judgement at the age of 58. After his interruption of therapy for 8 months, he felt the same pain in the same right lower leg again. He immediately visited our hospital. He had symptoms of slight local swelling and heat sensation together with pain in the same area in his right lower leg, but again there were no findings in physical and neurological examinations at this time. His vital signs were as follows: heart rate 99 beats/minute, blood pressure 130/70 mmHg, and body temperature 37.0 °C. He had a symptom of slight local swelling and heat sensation in the same area with pain in his right lower leg, but there was no ulcer or injury on his skin surface. Laboratory data were as follows: white blood cell count, 6680/μL (neutrophil 63.4%); CRP, 0.32 mg/dl; erythrocyte sedimentation rate (ESR), 31 mm/hour; plasma glucose, 652 mg/dL; HbA1c, 6.9%; glycoalbumin 46.1%. Other laboratory data were as follows: red blood cell, 476 × 104/μL; hemoglobin (Hb), 15.9 g/dL; platelet, 25.1/μL; total protein (TP), 7.6 g/dL; albumin (Alb), 4.4 g/dL. Liver and renal function were within normal range as follows: aspartate aminotransferase (AST), 39 U/L; alanine aminotransferase (ALT), 50 U/L; γ-glutamyl transpeptidase (γ-GTP), 51 U/L; lactate dehydrogenase (LDH), 172 U/L; creatinine (Cre), 0.54 mg/dL; blood urea nitrogen (BUN), 13 mg/dL; Na, 131 mEq/L; K, 4.4 mEq/L; Cl, 96 mEq/L. Pathogenic bacteria were not detected. He had no diabetic complications, probably because his glycemic control was relatively good before the interruption of therapy. His leg MRI showed a spreading of the abscess and inflammatory change in his right lower leg (Fig. , lower panels). We hospitalized him in our institution but he did not agree to undergo surgery for remission. Therefore, we started 3.0 g/day of sulbactam sodium/ampicillin sodium and insulin therapy (24 units of aspart and 20 units of glargine). After starting insulin therapy, his blood glucose level gradually decreased, and his leg pain was also gradually mitigated. Local swelling and heat sensation disappeared approximately 5 days later. Finally, his leg pain disappeared approximately 2 weeks later, and he was discharged from our hospital. Just in case, however, we continued antibiotics therapy (450 mg/day of rifampicin and 4 g/day of trimethoprim) for approximately 2 months. After a total of 3-month antibiotics therapy during hospitalization and after discharge, we stopped antibiotics therapy. He was then followed up for approximately 6 months, and his leg MRI showed a reduction of the abscess and inflammatory change in his right lower leg. He had no symptoms and/or problems, and his inflammation markers remained within normal levels for at least 6 months.
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pmc-6323659-1
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On 16 December 2011, a 40-year-old white woman was hospitalized with dyspnea and a small-volume hemoptysis that had started 2 weeks before. She reported asthenia, but no weight loss, cigarette smoking (20 pack-years) that was not stopped afterwards, no exposure to toxic chemicals. Her medical history included pre-eclampsia during her two pregnancies, but no previous pulmonary disease or family history of renal/cardiac/pulmonary diseases. No other relevant finding was recorded.
Clinical examination upon admission highlighted apyrexia, hypertension (184/105 mmHg), pulse rate of 96 beats/minute, and skin pallor. A chest X-ray showed bilateral infiltrates, and the thoracic CT scan indicated diffuse and bilateral ground-glass opacification. The laboratory work-up showed normocytic normochromic anemia (hemoglobin level of 7 g/dL), but normal platelet and leucocyte counts. The creatinine level of 614 μmol/L (50 μmol/L in June 2011) indicated acute renal failure. Due to respiratory failure and renal impairment, the patients received three daily boluses of methylprednisolone (500 mg) followed by 1 mg/kg/day of prednisone.
A bronchoscopy performed on day 4 after hospitalization revealed the presence of hematic traces with a Golde score of 197 (bacterial cultures were negative). Serologic tests for auto-antibodies (antinuclear antibodies, ANCA, and anti-GBM antibodies) were negative, and the hemolytic complement fractions within the normal values (C3 = 1.22 g/L and C4 = 0.28 g/L). The ELISA test for anti-GBM antibodies using purified collagen IV alpha3 chain was negative. The renal biopsy showed fibrinoid necrosis in 10 glomeruli (among the 29 assessed; 34.5%), glomerulosclerosis in 30% of glomeruli, and cellular glomerular crescents in 28%. Immunofluorescence analysis revealed linear deposition of IgG, compatible with GS.
The patient underwent daily PLasmatic EXchanges (PLEX) for 11 days and started oral immunosuppressive therapy (100 mg of cyclophosphamide per day) on day 13 of the prednisone treatment. Due to severe renal failure and anuria, hemodialysis (3 times per week) was started on December 20, 2011. Hemoptysis stopped rapidly, but diuresis was not improved. At day 37 of hospitalization, due to neutropenia (<1G/L), the cyclophosphamide treatment was reduced to 75 mg per day, and then discontinued after 3 months. The patient remained on dialysis. As the serologic tests were all negative in 2011, the anti-GBM antibodies could not be monitored, but the patient did not show any other GS symptom in the following years.
In 2014, when the patient was still taking 5 mg of prednisone per day, a new episode of hemoptysis occurred confirmed by bronchoscopy. This was associated with acute pneumonia of the left lung lower lobe, with favorable outcome after treatment with prednisone (50 mg per day for 1 week) and fluoroquinolone-based antibiotic therapy.
In March 2015, the patient received a living donor (her mother) kidney transplant. In the years from the GS episode to the kidney transplant, all serologic tests for auto-antibodies were negative. Conversely, panel-reactive antibodies against class I and class II antigens were detected, but not against the donor’s human leukocyte antigen (HLA) (identical HLA profiles for donor and patient). Anti-Epstein Barr virus and cytomegalovirus IgGs were detected in serum samples from donor and recipient. The patient was induced with anti-thymocyte globulin (rabbit) and received standard immunosuppressive therapy with mycophenolate mofetil (MMF), tacrolimus (residual serum tacrolimus between 6 and 8 ng/mL), and prednisone. After the graft, the creatinine level was stabilized between 110 and 130 μmol/L. She developed new onset diabetes after transplantation that was treated with metformin and repaglinide.
In November 2016, microscopic hematuria without any proteinuria or renal dysfunction (creatinine level = 104 μmol/L) was detected. In February 2017, the patient was hospitalized because of hemoptysis and anuric acute renal failure (creatinine level = 1696 μmol/L). Like in 2011, blood pressure was increased (220/113 mmHg). The laboratory work-up showed normocytic normochromic anemia (hemoglobin level of 10.4 g/dL), platelet count of 100G/L, and normal leucocyte levels. At this time, the patient was taking tacrolimus (5 mg per day; residual tacrolimus level = 4.2 ng/mL), MMF (500 mg twice per day), and prednisone (5 mg per day). The serologic tests for auto-antibodies (antinuclear antibodies, ANCA, and anti-GBM antibodies) were negative, and the hemolytic complement factors within normal levels (CH50 = 79 U/mL, C3 = 1.28 g/L and C4 = 0.35 g/L). Renal biopsy of the transplanted kidney (25 glomeruli) confirmed the GS relapse with glomerulosclerosis in 36% of the analyzed glomeruli, cellular glomerular crescents in 56%, and linear IgG deposition on the GBM (Fig. ).
The patient underwent daily PLEX for 14 days, and received prednisone (500 mg/day for the first 3 days followed by 1 mg/kg/day). At the end of the 14th PLEX, a bronchoscopy indicated active hemoptysis with the presence of 99.5% sideroblasts and a Golde score of 200. Due to the absence of effect, PLEX was stopped and intravenous infusion of cyclophosphamide (500 mg/m2 every 21 days) was introduced combined with prednisone (5 mg per day) and tacrolimus (2 mg twice per day).
After five infusions of cyclophosphamide the patient has now recovered and the anemia is under control with EPO supplementation. Conversely, the grafted kidney does not work, and the patient needs hemodialysis 3 times per week. Tacrolimus has been stopped.
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pmc-6323660-1
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Case 1: A 64-year-old female presented to our hospital in late September 2017 with severe redness and discharge in her left eye. Immunochromatography revealed that her conjunctival scrapings were positive for adenovirus. She was prescribed with levofloxacin and fluorometholone instillation 4 times daily, which was discontinued after 1 week (14 days from symptom onset) because her symptoms alleviated. However, 1 week after discontinuation she presented with blurred vision in her left eye. Examination revealed a visual acuity of 10/20 in the left eye with mutton-fat KPs and multiple stellate keratitis (Fig. ). The anterior chamber had no apparent cells or flare. She was subsequently prescribed with levofloxacin and betamethasone 4 times daily in the left eye. The mutton-fat KPs and stellate keratitis disappeared after 1 week, and visual acuity recovered to 20/20.
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pmc-6323660-2
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Case 2: A 66-year-old female presented to our hospital in late September 2017 with redness in both eyes. Immunochromatography tests were positive for adenovirus. The patient was prescribed with 0.1% fluorometholone instillation 4 times daily, which was discontinued after 10 days when inflammation improved.
Three months (98 days) after the initial symptoms, she presented with MSI with a foreign body sensation and blurred vision in both eyes (visual acuity, 20/25 in each eye). Examination revealed stellate keratitis-like fluorescein staining and dark-brown pigmentation in the centres of MSI with a few cells in the anterior chamber (Figs. ). The patient was prescribed with betamethasone instillation 4 times daily in her left eye.
MSI and stellate keratitis improved within 1 week; however, mutton-fat KPs were observed in the left eye (Fig. ). The betamethasone instillations were continued for 3 more weeks until the symptoms improved.
After healing, the second steroid instillation was gradually reduced over a period of 6 and 8 weeks in cases 1 and 2, respectively. Unpleasant symptoms, such as photophobia or blurred vision, were not observed over an 8-month observation period.
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pmc-6323672-1
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The patient was a 32-year old woman with a one-year history episodes of cholecystitis treated conservatively. She did not have any other disease history. After an abdominal magnetic resonance imaging that confirmed multiple gallbladder stones (Fig. a), an elective LC was performed without intra-operative complications. The recovery was uneventful and the patient was discharged two days after operation. On the second day after discharge, the patient developed severe right upper abdominal pain and she was sent to our emergency department at 8:30 pm. At arrival, her heart rate was 110 bpm and the blood pressure was 80/55 mmHg. The hemoglobin dropped to 86 g/l from 127 g/l. The CT scan showed a 10.9 × 12.5 × 6.6 cm ISH in the right liver without obvious free fluid in abdominal cavity (Fig. b). Two hours after fluid resuscitation including 2 U red blood cell, the hemoglobin further declined to 78 g/l and the hemodynamics remained unstable. The abdominal pain was not relieved, after intravenous analgesics. A Doppler ultrasound was performed, two hours later and it found the hematoma had increased in size. Active intrahepatic bleeding was suspected. We called radiologist for consultation, however, the interventional angiography and embolization was not available at mid night. We explained the potential risk of sudden rupture of hematoma during conservative methods which may cause sudden death, to the patient and her relatives. After careful consideration of the continuous decline of hemoglobin, unstable hemodynamics after fluid resuscitation, we explained our surgical plan to the patient and her relative. We planned to perform laparoscopic exploration at first, if the hematoma continued to expand, we would evacuate or drain it, if not, we would put a drainage tube under liver which could serve as an early warning of rupture. The patient requested surgical method to reduce the risk of sudden death. Therefore, an emergency laparoscopic exploration was performed under general anesthesia. The ISH was confirmed (Fig. c). Four U red blood cell and 400 ml fresh frozen plasma were transfused. After fluid resuscitation and blood transfusion, her hemodynamic became stable. During the 3-h intra-operative observation, the hematoma did not expand. Therefore, a non-sucking drainage tube was placed under the liver and she was sent to ICU ward. Next morning, she was transferred to the ordinary ward. The upper abdominal pain gradually relieved. Five days after the laparoscopic exploration, another CT scan showed that the hematoma was largely resolved and we removed drain tube (Fig. d). She was discharged, 10 days after readmission.
Totally, 13 papers, including 16 cases of ISH after LC were reported from 1994 to 2015 (Table ). Nearly half of the patients had instability of hemodynamics. All of the cases were female patients. Age of patients ranged from 25 to 78. All hematomas were mainly located in the right lobe of liver, and some of them extended to the left lobe of liver. Only one case was ruptured at diagnosis. Hepatic capsule laceration was found in two cases, one of whom also took NSAIDS (non-steroids anti-inflammatory drugs) to control the pain after operation. Totally, 58.8% of patients took NSAIDS to control the post-operative pain, and most of them used Ketorolac, however, 35.3% of the patients still did not have definitive risk factors. The time interval to diagnose ISH after LC ranged from seven hours to six weeks. They were diagnosed most commonly (35.5%) within one day after LC. All patients had abdominal pain and 47.1% of the cases developed hypovolaemic shock.
Treatment strategies included: conservative treatment (antibiotics, blood transfusion, strict bed-reset), percutaneous drainage under CT or B ultrasound guidance, selective embolization of the bleeding vessel, laparoscopic exploration and laparotomy. Eighteen percent of patients had stable condition without fever and underwent conservative treatments. The only case of angioembolization was complicate by infection and required percutaneous drainage. For the patients with stable condition, fever and serious compression of inferior venal cava (IVC) always were indications for percutaneous drainage under CT or B ultrasound guidance. In these 17 cases, 29.4% of the patients underwent percutaneous drainage. For the patients with hemodynamic instability, emergent reoperation was adopted. Totally, nine cases underwent reoperation, including two case of laparoscopic operation and seven cases of laparotomy. For our case, we only performed laparoscopic exploration and did not perform evacuation or drainage of the hematoma, since the hemodynamic became stable after plenty fluid resuscitation and the hematoma did not expand, during the 3 h of intra-operative observation. For another case, laparoscopic exploration found small capsule laceration, and hemostasis was performed. In the seven cases of laparotomy, six patients underwent evacuation and drainage of hematoma, only one case underwent only laparotomy without evacuation or drainage.
All patients survived. Most of patients stayed one to two weeks after readmission, however, the longest hospital stay was up to 31 days after reoperation.
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pmc-6323711-1
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Patient 1 was noted to have seizures and an elevated plasma lactate level (4.95 mM, normal < 2.2 mM) when she was 3 months old. At the age of 6 years, she could only sit with support, babbled, and had hearing impairment, optic nerve atrophy, sleep apnea, proximal type renal tubular acidosis, and seizures that were controlled by a ketogenic diet. A brain magnetic resonance imaging (MRI) study revealed diffuse high intensity of white matter on T2-weighted images and a decreased N-acetylaspartate-to-choline (NAA/CHO) ratio and presence of a lactate signal on magnetic resonance spectrometry (MRS). A muscle biopsy revealed abnormal mitochondria reminiscent of mitochondrial disease, but sequencing of mitochondrial DNA revealed no pathogenic variants. She had recurrent eczema-like skin lesions. Her correct diagnosis was made after the diagnosis of her younger brother. Currently she still had apnea and needed a bilevel positive airway pressure (BiPAP) respirator at night time. She also had hearing loss.
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pmc-6323739-1
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A 70-year-old Caucasian male with past medical history of hypertension and hyperlipidemia managed with aspirin presented in the emergency room with the complaints of increasing fatigue, shortness of breath and coffee ground stool. An ulcer was found during emergency esophagogastroduodenal endoscopy. He was managed conservatively with Protonix and secession of aspirin. He returned two months later for follow-up esophagogastroduodenal endoscopy. A single 25 mm submucosal nodule was found at the gastroesophageal junction 40 cm from the incisor and appeared to extend 2 cm up into the esophagus (Fig. ). There was no Barrett esophagus or mucosal disease identified. A biopsy was taken and a diagnosis of adenocarcinoma was rendered. An endoscopic mucosal resection was subsequently conducted. Patient is disease-free for seven months since resection.
The biopsy showed proliferation of haphazard and angulated glands with focal crowding accompanied by desmoplastic stroma underlying squamous-columnar junctional mucosa (Fig. a). A diagnosis of adenocarcinoma was made.
The endoscopic mucosal resection specimen demonstrated that the adenocarcinoma invaded into submucosa (Fig. b). The tumor showed various levels of differentiation from poorly-differentiated area composed of solid tumor growth and small glands (Fig. c) to well-differentiated area composed of cystically dilated glands with attenuated epithelial lining (Fig. d). Eosinophilic secretion with focal crystallization was present in the lumina of majority of the glands. The distribution was in such a way that poorly-differentiated area was situated directly below the epithelium and well-differentiated component in the deep portion with differentiation progressing in a gradient fashion.
Despite of various differentiation, tumor cells in different areas showed similar cytomorphology (Fig. e). They had abundant cytoplasm (low nuclear: cytoplasm ratio) containing eosinophilic coarse granules and centrally located nuclei, reminiscent of Paneth cells or gastrointestinal neuroendocrine cells. The texture of the cytoplasmic granules was similar to luminal secretion, suggestive of active secretion. The tumor cells in the poorly-differentiated area displayed frankly malignant cytology with pleomorphic nuclei and prominent cherry-red nucleoli. In contrast, the tumor cells in the well-differentiated area had much blander cytology. There was no mitosis or necrosis appreciated.
The mucosa was focally eroded with focal reparative and regenerative changes characterized by pseudostratified columnar cells confined within the crypts (Fig. f). The adenocarcinoma focally colonized glandular epithelium. However, no Paneth cell metaplasia was identified. There were no goblet cells as evidence of Barrett esophagus. The adjacent gastric cardiac mucosa showed reactive foveolar hyperplasia and dilated fundic glands with proton pump inhibitor therapy effect.
To further characterize the cellular origin, special and immunohistochemical stains were performed. Both the cytoplasm of tumor cells and luminal secretion were diffusely and strongly positive for lysozyme immunohistochemical stain (Fig. b) and Periodic acid–Schiff with diastase digestion (Fig. a). Synaptophysin and chromogranin immunohistochemical stains highlighted scattered entrapped neuroendocrine cells while negative in tumor cells (Fig. c). The cytomorphology in combination with strong immunoreactivity with lysozyme antibody and absence of neuroendocrine differentiation support a Paneth cell differentiation. Ki-67 (Fig. d) and p53 (data not shown) showed similar staining pattern with positive stain in approximately 40% nuclei of tumor cells in poorly-differentiated area and only rare positivity in well-differentiate area. Mammoglobin was negative in the tumor cells (data not shown). Beta-catenin immunohistochemical stain showed membranous stain in the tumor cells (Fig. e). Her-2/neu immunohistochemical stain demonstrated nuclear and cytoplasmic staining in the poorly-differentiated area and was completely negative in the well-differentiated area (Fig. f).
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pmc-6323823-1
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A 64-year-old non-smoking Japanese man was referred to our hospital with suspected interstitial pneumonia in a health examination in 2013. He had a slightly dry cough with no desaturation. He had been a plasterer for more than 40 years without appropriate protective equipment. Chest auscultation revealed slight bilateral inspiratory fine crackles in the bilateral lower lung zones. A chest X-ray film showed enlarged hilar lymph nodes and mild reticular opacities, mainly in the upper to middle lung fields of both lungs (Fig. a). The results of a chest high-resolution computed tomography (HRCT) scan suggested a predominantly subpleural distribution of irregular linear opacities and reticulonodular shadows with interlobular septal thickening in both lung fields (Fig. b). Pulmonary function tests were close to normal (Fig. ) and a six-minute walking test performed on admission was also normal.
One year later (in 2014), forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO) had decreased (Fig. ), while reticulonodular shadows on HRCT worsened. However, his thoracic symptoms had not deteriorated and his vital signs were stable. In order to establish a diagnosis, VATS was performed from the right S2 segment of the upper lung lobe and the right S9 segment of the lower lung lobe (Fig. ). Dense fibrosis with mononuclear cell infiltration and inorganic dust particles around the respiratory bronchioles was observed in the upper lung lobe S2 segment, which was consistent with MDP (Fig. a). Furthermore, fibrously thickened interlobular septa and visceral pleura accompanied by dust, including some birefringent particles suggestive of silicates, and fibroblastic foci were detected within these lesions (Fig. b). Extensive honeycomb changes with dilated bronchioles and parenchymal collapse as well as fibroblastic foci within the cystic wall were observed in the lower lung lobe S9 segment (Fig. c). Asbestos fibers and asbestos bodies were not found in either upper lung lobe S2 or lower lung lobe S9 specimens. An electron probe microanalysis (EPMA) primarily detected silicon (Si), aluminum (Al), and iron (Fe) in the S2 and S9 areas (Additional file : Figure S1).
Based on these results, we diagnosed the patient with pneumoconiosis with the UIP pattern and monitored him without any medication because he quit his job. Although his symptoms and pulmonary function tests transiently improved, he had a dry cough with exertional dyspnea, and his pulmonary function test results markedly deteriorated (in 2016, Fig. ). We administered nintedanib with the expectation of an anti-fibrotic effect; however, the treatment was not effective, and he currently requires home oxygen therapy (Fig. ). A recent chest X-ray image (Fig. c) showed a markedly smaller lung volume than that in the initial image as well as the progression of lung fibrosis. A chest CT scan also showed the progression of fibrotic changes in subpleural regions (Fig. d).
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pmc-6323825-1
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A ten-and-a-half-year-old girl presented to her primary care physician with a three-month history of swelling and pain in the distal part of the small finger of her left hand. The pain was most intense during movement and palpation, although occasionally it was present at rest as well. There was no history of preceding trauma, acute infection, or fever. Initial physical examination showed a thickened distal phalanx of the affected finger without motion restrictions. Initial radiograph showed normal bone structure and mineralization, without signs of fracture or other pathology (Fig.
and ), and primary care physician suggested activity restriction. In the following five months, the pain became more prominent, without daily variations, and she was referred to a paediatric orthopaedic surgeon who suspected glomus tumour and ordered a magnetic resonance imaging (MRI) of the affected finger along with expanded laboratory workup. All laboratory findings, including CBC, CRP, ESR, rheumatoid factor, and antinuclear antibodies, were within the normal range. MRI showed a hyperintense signal on proton density fast spin echo sequence correlating with soft-tissue swelling surrounding distal phalanx (Fig. c and d). These features were characterized by the radiologist as trauma or tenosynovitis. Ibuprofen trial was recommended and the patient initially reported slight reduction of swelling and pain, soon followed by subsequent deterioration. Finally, paediatric rheumatologist was consulted. The initial musculoskeletal ultrasound (MSUS) examination showed increased power-Doppler activity in the distal part of the affected finger with no effusion in the distal interphalangeal joint (DIP), and a cyst-like formation connected to the extensor tendon, giving the impression of tenosynovitis. Laboratory workup remained unremarkable. Due to persistent clinical and imaging findings suggestive of dactylitis, the diagnosis of juvenile spondyloarthritis was suspected. MSUS-guided triamcinolone-hexacetonid injection was administered in the cyst. The injection, along with an oral indomethacin trial, resulted in a slight reduction of the swelling. Nevertheless, the pain persisted, affecting the quality of sleep and activities of daily living. Short courses of various other NSAIDs were attempted, with no satisfactory results, so another consultation with paediatric orthopaedic surgeon was requested, nearly two years after the initial presentation. Clinical symptoms were still suggestive of dactylitis, with distal phalanx swelling and associated increase in size of the nail bed with pain on palpation (Fig. ). Again, distal phalanx neoplasm was suspected and new radiographs and MRI were ordered, along with radionuclide skeletal scintigraphy with Technetium 99 m-MDP. At this point, two years after the initial radiographs, the newly-obtained radiographs of the left fifth finger showed diaphyseal widening of the distal phalanx with a central radiolucent zone. MRI findings were consistent with the radiographs and showed a small oval-shaped sequestrum measuring 3 mm in diameter on the dorsal aspect of the distal phalanx, along with the initially-described hyperintensity of the surrounding tissue. Radionuclide skeletal scintigraphy with Technetium 99 m-MDP showed increased radionuclide uptake in the distal phalanx/DIP joint of the affected finger in all three phases of the bone scan, a finding most typical for juvenile spondyloarthritis (Fig.
to ). OO was finally suspected. After thorough preoperative planning, the patient underwent surgical exploration and excisional biopsy. A longitudinal skin incision was made over the dorsal aspect of the distal phalanx of the left small finger. The distal interphalangeal joint was exposed together with the extensor tendon insertion, which demonstrated the presence of inflammatory changes. In order to better visualize the distal phalanx, the nail plate was removed. The dorsal aspect of the distal phalanx was clinically inflamed with prominent red discolouration in an area measuring 4 mm in diameter. Following the bone corticotomy, a complete excisional biopsy of this area was performed, with specimen sent for pathological examination. The early postoperative course was uneventful with almost immediate pain relief in the finger. Pathology report returned positive for OO, describing the nidus within bony trabeculae surrounded by fibrovascular connective tissue. Three months after the surgery, the patient was pain-free with full range of motion of the involved finger. At the most recent four year postoperative follow up, there was no evidence of local recurrence, and the patient was symptom-free.
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pmc-6323858-1
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A 35-year-old G2P0010 Cameroonian student at 39-weeks pregnancy was referred to the surgical unit of the Yaounde Gynaeco-Obstetrics and Paediatric Hospital for the management of a strangulated umbilical hernia. She had a sudden onset of localized umbilical pain three hours prior to consultation. The pain was of moderate intensity, crampy in character, aggravated by walking, without any change in bowel movement and no vomiting. An abdominal ultrasound scan revealed a parietal defect of the umbilicus measuring 55 mm in diameter with a poorly vascularised hypoechoic mass (doppler scan) measuring 50 × 30 × 37 mm, 29.6 ml in volume. In addition, the foetus was viable with a normal biophysical score and a good concordance between clinical and sonographic dating of gestational age. Hence, she was referred for surgical management of a strangulated umbilical hernia in a term pregnancy.
An episode of severe malaria during her previous pregnancy was at the origin of a spontaneous abortion at 10 weeks of gestation. Her current pregnancy was being followed at the Efoulan District hospital in Yaounde where she had attended six antenatal clinics. A urine dipstick at 24 weeks of gestation revealed a proteinuria of 600 mg/l coupled with an increase blood pressure to 152/98 mmHg and the development of lower limb oedema. She was diagnosed with pre-ecclampsia and placed on alphamethyldopa 250 mg twice daily. A second trimester ultrasound revealed the presence of two anterior and posterior interstitial myomatous nuclei, of 51 mm and 73 mm long axis respectively.
On physical examination, the patient was in severe pain (visual analogue scale of 9/10 cm) with a temperature of 38.1 °C, pulse rate of 112 beats per minutes, respiratory rate of 22 breaths per minutes and blood pressure of 170/118 mmHg. Abdominal examination showed a gravid uterus with a uterine fundal height of 38 cm. There was a tender, non-reducible umbilical swelling (Fig. ), with no cough impulse. There was no sign of peritoneal irritation. She had no costovertebral angle tenderness. Bowel sounds were present and normal. Her digital rectal examination was unremarkable. The foetus had a longitudinal lie, cephalic presentation, right-occipito anterior position and a fetal heart rate of 140 beats per minute. On vaginal examination, the cervix was posterior, non-effaced and closed. She had a bilateral pitting lower limb oedema extending to both knees. In view of this clinical picture, we thought of a strangulated umbilical hernia. All of these on a probable background of severe pre-eclampsia. The laboratory panel requested on admission is illustrated in Table .
A multidisciplinary team involving general surgeons, obstetricians and anaesthesiologists decided on a two-in one intervention wherby an emergency ceaseraean section with indication severe pre-ecclampsia, and a herniorraphy with indication strangulated umbilical hernia will be carried out within the same operation. Preoperative management consisted of placing two peripheral venous lines of large bore needle with infusion of 1000 ml of normal saline, parenteral administration of an analgesic (paracetamol 1 g), an antihypertensive drug (nicardipine 2 mg bolus) and anticonvulsant (magnesium sulphate 5 g intravenously followed by 4 g intramuscularly in each gluteus muscles). A Pfannenstiel incision performed five hours after admission permitted the extraction of a life female baby who weighed 3300 g at birth with an APGAR score of 8 and 10 at the first and fifth minutes respectively. Intraoperative findings of an anterior and posterior sub-serosal leiomyomas both measuring about 50 mm (Fig. ); anterior fibroid had an axis pointing to the umbilical ring, with irregular contours and a heterogeneous center, strongly suggestive of aseptic necrobiosis. In addition, the uterus also had several interstitial myomas. The uterine adnexae and the appendix were macroscopically normal. The herniated omentum was not necrosed. No intestines necrosis was observed. Both sub-serosal leiomyomas were surgically excised and sent for histo-pathological evaluation. A separate arciform infra-umbilical incision permitted repair of the umbilical hernia.
Histopathological analysis of the leiomyoma samples was consistent with red degeneration (aseptic necrobiosis) of the excised uterine fibroid (Fig. ). The postoperative outcome was uneventful for both the mother and the baby, with the former resuming progressive oral feeding on the first postoperative day. She was discharged five days later in a good clinical condition. Her follow-up at six weeks postoperatively was uneventful.. The six-month postoperative course was also normal.
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pmc-6324851-1
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We present the case of a 28-year-old pregnant woman with 35 weeks of gestation, from Loreto, Peru. She had four months of progressive dysphonia, loss of weight (5 kg), dry cough, and dysphagia which at the start was only for solid food and then even for liquids. One month before admission, the patient presented with odynophagia, fever, night sweats, and a productive cough. She was admitted to the emergency room due to respiratory distress and hemoptysis. At the time of her admission, she brought a laryngoscopy report that concluded laryngeal cancer; although a biopsy was not performed.
During the physical examination, she was in a bad general condition. Tachypnea, dysphonia, and a decrease of the subcutaneous cellular tissue were evident. The cardiac and respiratory frequency were increased (110 bpm and 14 vpm) and a temperature of 39°C was recorded. No other alterations were found in the rest of the examination.
Due to the history of chronic dysphonia and the laryngoscopy report that indicated the existence of laryngeal compromise, a differential diagnoses were proposed: laryngeal cancer and laryngeal tuberculosis. The diagnosis revealed respiratory and systemic symptoms suggesting the infectious etiology as the cause. A new laryngoscopy was performed, which reported a mamelonated laryngeal tumoration that compromised the arytenoid cartilage and the interarytenoid notch; the vocal cords presented irregularities with predomination of the right side, and their mobility was limited (Figures -) (Video ).
Other tests were requested to evaluate the active tuberculosis disease at pulmonary stage; a sputum bacilloscopy showed positive result (+/+++) and the chest radiography showed bibasal lesions of fine nodular pattern with predominance of the right hemithorax, reticular opacities at the left apical level and an ipsilateral elevation of the hemidiaphragm; as described earlier, it was raised with a high suspicion that the case could be laryngeal tuberculosis secondary to lung infection as the starting point (Figure ).
Nevertheless, it was not possible to perform a biopsy of the laryngeal lesions due to the level of damage that was observed in the larynx and that could be caused during the procedure; besides, the infectious etiology was already clear due to the positive sputum smear result. Empirical treatment with drugs (isoniazid, rifampicin, pyrazinamide, ethambutol) for sensitive tuberculosis was started, and after a week, the patient presented significant clinical improvement (resolution of fever, dyspnea, cough, and dysphonia), which confirmed the presumptive diagnosis and she was discharged from the hospital after being adviced to continue treatment with an ambulatory control.
The following sputum smears were negative from the second month, until after the treatment, so it was considered as healed. No adverse reactions to the drugs or serious sequels of the disease were reported.
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pmc-6324854-1
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A 60-year-old woman with a history of diabetes, ischemic cardiomyopathy (ejection fraction 30% to 35%) with implantable cardioverter-defibrillator (AICD) and pancytopenia of unclear etiology presented to the hospital with a three-day history of fever and altered mental status. On admission, the patient was febrile with a temperature of 102.6 °F, tachycardic with a heart rate of 102 beats per minute, respiratory rate of 16 breaths/minute and blood pressure of 160/87 mmHg. Her cardiac examination as well as the examination of her peripheral extremities was unremarkable. Laboratory findings revealed pancytopenia (white blood cells: 1.71 x 103/μL, hemoglobin 6.6 g/dL and platelets 88 x 103/μL). Imaging studies including chest X-ray, non-contrasted computed tomography (CT) head and CT abdomen were mostly unremarkable, except for mild splenomegaly. However, two of her blood cultures were positive for C. parapsilosis. Given that she was immunocompromised and had an indwelling prosthetic device in place, the patient was started on intravenous (IV) micafungin, and an echocardiogram was performed. The imaging revealed a large 2 x 2-cm sessile mass attached to the tricuspid valve that prolapsed into the right atrium during systole (Figure ). The patient underwent removal of the AICD, coronary sinus lead and the right atrial lead under fluoroscopic guidance. Post-procedure trans-esophageal echocardiogram (TEE) at this point still demonstrated a mobile 1-cm vegetation on the tricuspid valve. Hence, it was concluded that our patient has an infection involving not only the AICD lead but also the native tricuspid valve since even after removal of the lead, there was a persistent vegetation attached to the tricuspid valve. It was presumed that the small vegetation that was still found to be remaining would improve with medical therapy. IV micafungin was continued after the procedure, and blood cultures were followed. One of the new blood cultures continued to grow C. parapsilosis. Repeat trans-thoracic echocardiogram (TTE) a few days later still demonstrated a mobile 2 x 2-cm vegetation on the right atrial side of the tricuspid valve. As the patient failed to clear the fungemia, a repeat surgery was undertaken with sternotomy and removal of multiple tricuspid valve vegetations, resection of an infected papillary muscle with reduction and remodeling annuloplasty of the tricuspid valve. The pathology report confirmed numerous fungal yeasts and pseudohyphal forms on Gram stain. The patient’s antifungal regimen was also modified to flucytosine and amphotericin B. The patient continued to improve from the cardiac standpoint; blood cultures after the second surgery were reported to be negative.
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pmc-6324855-1
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A 62-year-old postmenopausal female, para 3, presented with a complaint of a mass coming out of the vaginal orifice for the last 10 years. Initially, there were no symptoms but recently in the last five months, the mass had become irreducible, and she developed dysuria. She had no history of any previous illness or allergy. There was no family history of malignancies. There was no significant family or psychosocial history.
The patient was weak and fragile. Her systemic examinations were unremarkable except for the mass coming out from the vaginal orifice (Figures -).
Her vitals were normal. A genital and vaginal examination revealed an irreducible uterovaginal prolapse with maggots, larvae, and eggs. Ulcers were seen over the prolapsed mass.
Further investigation of the patient revealed that her hemoglobin, total leucocyte count (TLC), platelet count, partial thromboplastin time (PTT), activated partial thromboplastin time, blood sugar levels, and urine analysis were in normal range. Hepatitis B and C profiles were negative.
The patient was treated with analgesic and broad-spectrum antibiotics. The treatment options were presented to the patient with proper counseling. Vaginal hysterectomy was selected and informed consent was obtained. There were no intraoperative and postoperative complications. The histopathology report excluded malignancy. The symptoms resolved completely after the surgery. The patient was discharged and went back to routine life.
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pmc-6324858-1
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A 63-year-old healthy woman presented for the evaluation of an itchy rash on the lower legs that developed over a period of two years. She had not initiated any new medications. Her medical history was only significant for hypothyroidism for which she took levothyroxine daily.
A complete examination of her skin and mucous membranes was performed. The distal legs showed pink plaques with peripheral hyperpigmentation (Figures -).
Purple, flat-topped papules were also present on both wrists (Figure ). In addition, white, reticulated patches were present on the bilateral buccal mucosa.
Skin biopsies of her left wrist and her right lower leg were performed. They showed hyperkeratosis with an inflammatory infiltrate predominantly composed of lymphocytes present in a lichenoid distribution along the dermal-epidermal junction with apoptotic keratinocytes. These features were considered to be those of lichenoid dermatitis and most consistent with lichen planus.
Antinuclear antibody and double-stranded deoxyribonucleic acid (DNA) antibody tests were performed to evaluate for systemic lupus erythematosus; these serologies were negative. The review of systems was negative for oral ulcerations, joint pain or swelling, and alopecia. Correlation of the clinical findings, pathology, and laboratory studies established a diagnosis of hypertrophic lichen planus.
The patient was treated with topical clobetasol 0.05% cream applied daily to the lesions on her legs as well as oral prednisone 40 milligrams daily for two weeks. At the two-week follow-up, her condition had improved; therefore, over the next month, the daily systemic prednisone was slowly tapered and she continued to apply the topical corticosteroid cream. At her subsequent follow-up appointments, a continued improvement of her condition was observed.
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pmc-6324859-1
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This was a 27-year-old male with known paraplegia and chronic osteomyelitis who presented with stage IV pressure ulcers of his sacrum and left ischium. Despite previous antibiotic therapy, he developed invasive osteomyelitis of his left femoral head and underwent a Girdlestone procedure for further care. Intraoperative findings included a necrotic femoral head as well as areas of abscess and necrotic tissue. Cultures showed Bacteroides fragilis and Staphylococcus aureus. After the completion of the Girdlestone procedure, he had NPWTi-d placed in his surgical wound with 40 milliliters (mL) of normal saline following our standard NPWTi-d protocol using Veraflo® (KCI, San Antonio, Texas, USA). Five days after the initial procedure, he underwent delayed primary closure over closed suction drains with the placement of an incisional negative pressure device. His treatment while hospitalized included dedicated offloading bedding, nutrition supplementation, and culture-driven intravenous antimicrobial medications. He was then discharged on ciprofloxacin, vancomycin, and metronidazole antibiotic therapy seven days after the initial procedure. He had no readmissions in the first 30 days after discharge.
He was discharged two days after his delayed primary closure and followed up in clinic one week after the closure. His wound was healing well. His negative pressure device was removed at that time. One month after the operation, his sutures and staples were removed due to no sign of a secondary breakdown of the wound. He was then discharged to care at his local wound clinic for the management of his pressure ulcers with no recurrence of invasive osteomyelitis of the left hip.
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pmc-6324859-2
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This patient was a 45-year-old male with known quadriplegia and stage IV pressure ulcers of the ischium bilaterally, who presented with chronic osteomyelitis in his left femoral head and chronic septic arthritis due to MRSA in his acetabular space. As his infection was resistant to intravenous antibiotics, he underwent a left Girdlestone procedure. Intraoperative findings were significant for areas of inflammation in the greater trochanter. Cultures were negative. The surgical wound was 15 cm x 5 cm x 10 cm. His wound was dressed with Veraflo NPWTi-d. Five days later, he underwent completion debridement and delayed primary closure over closed suction drains with Prevena® (KCI, San Antonio, Texas, USA). He was discharged on vancomycin and meropenem four days after the initial procedure. Postoperatively, his negative pressure dressing was removed at his follow-up appointment five days after discharge and six days after closure. He continued to have no sign of a wound breakdown over the Girdlestone at the one-year follow-up. While his left ischial ulcer healed significantly, with no recurrence of infection in his left hip, he developed a worsening of his right ischial pressure ulcer two months postoperatively. He has not had a recurrence of his osteomyelitis or septic arthritis.
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pmc-6324859-3
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This was a 70-year-old male with a history of paraplegia, a stage IV pressure ulcer of the right ischium, and new onset ulceration of the right greater trochanter in the setting of prior flap coverage and internal plating for prior fracture and pressure ulceration that had since healed well. Despite appropriate wound care, offloading, antimicrobial treatment, and removal of the hardware, the patient progressed to invasive osteomyelitis of his femoral head. The patient presented in sepsis and was admitted for a right-sided Girdlestone procedure. Intraoperative findings revealed a grossly necrotic bone with drainage of the cavity and cultures grew Cladophialophora mold, with concomitant sacral and ischial ulcers growing Pseudomonas. He was dressed with a Cleanse® (KCI, San Antonio, Texas, USA) negative pressure dressing with instillation and dwell with 50 mL of normal saline fluid instilled using our standard protocol. The wound was 10 cm x 11 cm x 5 cm and, therefore, not amenable to complete primary closure. Thus, he underwent delayed partial closure over closed suction drains four days later, with a negative pressure device over the incision and wound. He was discharged on six weeks of ertapenem and additionally had fluconazole for 10 days after the initial procedure. He had no readmissions in the first 30 days after discharge. His wound completely healed at 4.5 months postoperatively, with no subsequent infection of the treated hip one year after surgery. Figure below demonstrates the preoperative ulcer of the greater trochanter with necrosis, computed tomography (CT) imaging of the fractured right femoral neck, postoperative wound closure, and the final healed wound.
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pmc-6324859-4
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This patient was a 35-year-old paraplegic male who had a history of bilateral stage IV pressure ulcers of the ischium and presented with a disseminated Proteus infection involving his chest wall, right iliacus muscle, and right hip with associated osteomyelitis of several right-sided ribs, the acetabulum, and the femoral head. After initial stabilization with drainage of his iliacus and chest wall abscess along with nutritional supplementation, he underwent a Girdlestone procedure on his right hip. Intraoperative findings were significant for copious purulence and a grossly necrotic femoral head and a soft tissue capsule that spread to the acetabulum. These tissue cultures also grew Proteus species. His wound was dressed with a Cleanse NPWTi-d with a 50 mL lavage of ¼ strength Dakin's solution for a 10-minute dwell time every 3.5 hours. He underwent delayed primary closure over closed suction drains three days later and the incision was dressed with a Prevena. He had no readmissions in the first 30 days after discharge, no recurrent infections, and no wound complications. At the one-year follow-up, he presented with a significant reduction in the size of both ischial ulcers. Figure below demonstrates his resected femoral head, the resultant wound after Girdlestone, the placement of instillation therapy, and final closure.
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pmc-6324859-5
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This was a 40-year-old quadriplegic female with known bilateral hip dislocations and multiple decubitus ulcers. She developed chronic septic arthritis in the right acetabulum with concurrent osteomyelitis, which progressed to an ulceration of the femoral head externally through her stage IV ischial pressure ulcer. Due to this ulceration and the risk of further ulceration on the right side, she underwent a Girdlestone procedure. Intraoperatively, her femoral head and neck were grossly necrotic, although surrounding soft tissue appeared to be healthy. Intraoperative cultures grew Pseudomonas, Escherichia coli, Enterococcus faecalis, MRSA, and Acinetobacter calcoaceticus-baumannii complex. Her resulting wound bed, including the acetabulum, was dressed with a Cleanse NPWTi-d utilizing normal saline. Three days later, she underwent partial delayed primary closure over closed suction drains with the placement of a negative pressure device over the incision and an ongoing open wound, as complete closure over the ischial ulceration was not possible. She was discharged 13 days after the initial procedure on ampicillin-sulbactam, vancomycin, and cefepime. She was not readmitted in the first 30 days after discharge.
She underwent a superficial debridement of her ongoing right ischial pressure ulcer at the one-month follow-up with healthy tissue found underneath. Due to the development of a new persistent left ischial and greater trochanter ulcer, she is being evaluated for a left-sided Girdlestone.
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pmc-6324859-6
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This was a 25-year-old male with known spina bifida who presented with a chronic infection of his left acetabulum. He had been previously managed for several years for a non-healing pressure ulcer of the left greater trochanter, having undergone a partial femoral head resection and prior flap placement with subsequent failure. He presented with large volume drainage from a small ulceration over his left trochanter with CT imaging demonstrating an abscess in the gluteus muscle with osteomyelitis in the abutting femoral head. He underwent a left Girdlestone procedure. Intraoperative findings included heterotopic ossification with necrotic bone in the femoral head. Cultures grew MRSA, Proteus mirabilis, and mixed microorganisms. The surgical wound was treated with a Cleanse NPWTi-d utilizing normal saline. Three days later, he underwent partial delayed primary closure over closed suction drains with the placement of a negative pressure dressing over the incision and ongoing wound, as complete primary closure was not possible due to the dimensions of the resulting wound. He was discharged eight days after the initial procedure on ertapenem. He was not readmitted in the first 30 days after discharge.
At his one-month follow-up, it was noted that his left-sided osteomyelitis had not recurred nor progressed. At his two-month visit, the wound continued to be clean and closed, with no sign of breakdown. However, at this time, he developed the worsening of a previously existing stage IV right ischial pressure ulcer, which was treated with operative debridement. He has not had a recurrence of his left hip osteomyelitis and his wound is nearly completely healed. Figure below depicts his chronic trochanteric ulcer, the wound after Girdlestone resection, placement of negative pressure wound therapy over the closed incision, and the resultant healing wound.
Patient 7: right side
This was a 29-year-old male with a history of paraplegia who developed several stage IV ischial and sacral pressure ulcers on his right side, resulting in a dislocation of his femoral head on the right and progression of the infection into the acetabulum and iliacus muscle. His ulcer progressed despite appropriate treatment, and he also developed severe protein malnutrition; he was thus treated with a right Girdlestone procedure. Intraoperative findings were significant for necrotic exposed acetabulum and femoral head. Cultures grew MRSA and Staphylococcus epidermidis. The resulting wound bed, including the acetabulum, was dressed with a Cleanse Choice® (KCI, San Antonio, Texas, USA) NPWTi-d utilizing normal saline. Three days later, he underwent a partial delayed primary closure over closed suction drains with the placement of a negative pressure device over the incision. He was discharged 14 days after the initial procedure on doxycycline and trimethoprim-sulfamethoxazole. He was not readmitted in the first 30 days after discharge.
At his three-month follow-up visit, his wound was healing well, with no sign of recurrent osteomyelitis on the right side. However, he did have progressive ulceration of his previously existing left greater trochanter ulcer and was found to have invasive osteomyelitis in the left hip. Figure demonstrates the pre-operative ulcer, resection specimen, and resultant healing wound.
Patient 7: left side
Due to the success of the right Girdlestone procedure, the patient underwent a left Girdlestone approximately three months later. Like the right side, he had developed a chronic ulcer over the left greater trochanter with subsequent femoral head osteomyelitis. Intraoperative findings were also similar, with a necrotic femoral head and resultant cultures growing no organisms, though previous cultures grew Pseudomonas. The wound was dressed with a Cleanse Choice NPWTi-d (see Figure below). Three days later, he underwent a partial delayed primary closure over closed suction drains with the placement of a negative pressure device over the incision. He was discharged eight days after the initial procedure on doxycycline and trimethoprim-sulfamethoxazole and was not readmitted in the first 30 days after discharge.
The patient was readmitted at 60 days with concern for the protrusion of his left distal femur into the ongoing wound bed and was taken to the operating room for excisional debridement and bone biopsy. The biopsy was negative for invasive osteomyelitis. In addition, at month four, he presented with a stage IV ulceration of his sacrum. Adequate offloading, wound care and nutritional support, and intravenous antibiotics were not able to be achieved in the postoperative care of this patient due to numerous factors. He was discharged in this state five days later on trimethoprim-sulfamethoxazole with the intent to heal by secondary intention and has since re-presented with progressive malnutrition and dry gangrene of the toes of his right leg. He has refused ongoing medical care. He has not required ongoing treatment for the infection in either hip and his surgical wounds continued to decrease in size.
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pmc-6324859-7
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This was a 59-year-old male with a history of paraplegia with progressive worsening of his multiple sacral and ischial stage IV pressure ulcers, resulting in chronic osteomyelitis and dislocation of his right femoral head. He presented for a Girdlestone procedure on the right side. Intraoperative findings were significant for a necrotic femoral head. Cultures were sterile at this time, but previous cultures of the same wound grew Staphylococcus capitis, Candida albicans, Acinetobacter calcoaceticus-baumannii complex, and mixed flora. The wound was dressed with a Cleanse Choice NPWTi-d initially in addition to the application of a collagen, cellulose, and silver matrix. Instillation was started with normal saline on postoperative day one, once hemostasis was assured. Four days later, he underwent a delayed primary closure over closed suction drains with the placement of a negative pressure device over the wound incision, which was completely closed. His drain was dislodged prematurely postoperatively and he developed a partial dehiscence of his wound in the area of his previously open ischial pressure ulcer. He was discharged 17 days after the first procedure on ceftriaxone and vancomycin, with gauze dressing changes for the area of dehiscence. He was unable to continue negative pressure wound therapy. He was not readmitted in the first 30 days after discharge.
During his first two months postoperatively, he was lost to follow-up by the infectious disease and surgical clinics and presented then with a clean and healing wound bed. He was readmitted three months postoperatively with a concern for progressive osteomyelitis of his sacrum, which was negative on biopsy and was found instead to have polymicrobial urosepsis. At his six-month follow-up, he was found to have a continued decrease in his wound size without complete healing, however, there was no recurrence of his invasive osteomyelitis.
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pmc-6324859-8
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This was a 52-year-old male with a history of a severe peripheral vascular disease, right hip disarticulation, and paraplegia, with a stage IV pressure ulcer of the left greater trochanter with resultant osteomyelitis. He underwent an attempt at limited resection of his greater trochanter in a two-stage procedure but developed an invasive infection of his hip and acetabulum with ongoing wound drainage and dehiscence. Thus, he was counseled and planned for definitive therapy with a Girdlestone procedure. Intraoperative findings included a grossly necrotic femoral head with a large open wound over the greater trochanter. Intraoperative cultures grew Staphylococcus epidermidis, which had been isolated at the prior operation. The wound was dressed with a Cleanse Choice NPWTi-d. Three days later, he underwent a delayed primary closure over closed suction drains with the placement of a Prevena negative pressure device over the incision. He was discharged seven days after the first procedure on ceftriaxone and vancomycin. He was not readmitted in the first 30 days after discharge.
While his incision has healed completely (see Figure below), he has persistent low volume drainage via a surgical drain at five months postoperatively but no clear radiographic or clinical evidence of recurrent osteomyelitis.
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pmc-6324859-9
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This was a 21-year-old male with a history of paraplegia who had recurrent septic arthritis of the right hip due to MRSA, which failed to resolve after open drainage was performed at an outside hospital. He presented with sepsis, MRSA bacteremia, and acute chronic osteomyelitis in the femoral head, with an extensive invasive soft tissue infection involving the entire gluteus, posterior compartment, and hip. He underwent an emergent Girdlestone procedure. Intraoperative findings were significant for a completely necrotic femoral head surrounded by fluid, with extensive purulence throughout the acetabulum and surrounding soft tissue (see Figure below). Cultures were significant for MRSA. The wound was dressed with a negative pressure device without instillation and dwell initially and then transitioned to NPWTi-d with 75 mL instillation of ¼ strength Dakin's solution for a 10-minute dwell time at 3.5-hour intervals once his hemoglobin stabilized. Two days later, he underwent a further debridement of the right Girdlestone site and ulcer, with a changing of the negative pressure device sponge. Due to the degree of invasive infection, a third operation was performed to obtain source control with a reinitiation of instillation therapy. Four days later, a total of 13 days after the initial procedure, he underwent delayed primary closure over closed suction drains with the placement of a negative pressure device over the incision. He was discharged 20 days after the initial procedure on fluconazole, cefepime, metronidazole, and vancomycin.
He was not readmitted in the first 30 days after discharge, but he was unable to comply with ongoing offloading and wound care. Despite this, his lateral incision healed well postoperatively but he presented approximately eight weeks after the initial presentation with a progression of the ischial pressure ulcers and septic arthritis on his contralateral hip with acute dislocation of his femoral head for which he underwent a Girdlestone procedure as well.
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pmc-6324862-1
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A 57-year-old African American female with a history of diabetes presented to the hospital with severe anemia and acute change in mental status. On physical examination, the patient was noted to be lethargic and had right-sided facial drooping, right-sided tongue deviation, right-sided gaze preference, with right-sided body strength significantly diminished compared to the left. Initial laboratory results, reported in Table , showed severe anemia and thrombocytopenia (Hb 2.3 g/dL, Hct 8 %, Plt 15,000/cmm), and mild acute kidney injury (CrCl 101 mL/min). Numerous fragmented red blood cells (RBCs) (schistocytes) were noted in the peripheral blood smear (Figure ). Repeated peripheral blood smears persistently showed poikilocytosis, nucleated RBCs, immature myeloid cells, and teardrop cells. Thrombotic thrombocytopenic purpura (TTP) was suspected due to classic presentation: microangiopathic hemolytic anemia, thrombocytopenia, acute kidney injury, altered mental status, and a low grade fever.
The patient was started on daily plasmapheresis and steroids for a presumed diagnosis of TTP. However, after ADAMTS13 result came back negative, plasmapheresis was stopped and the steroid was tapered. On further evaluation, computed tomography (CT) scan of the head revealed mixed sclerotic and lytic lesions in the calvarium (Figure ), diffuse osteoblastic pelvic lesions (Figure ), and a 1.2-cm ovoid soft tissue nodular opacity in the 6 o’clock position of the right breast (Figure ). Subsequent tests including bone marrow aspiration yielded a dry tap further solidifying the concern for bone marrow infiltrative disease. Bone marrow biopsy from the ischial bone showed many atypical cells (Figure ), which were highly suggestive of carcinoma and the immunohistochemistry report was consistent with metastatic lobular breast carcinoma with the tumor cells staining positive for both estrogen receptor (ER) and progesterone receptor (PR), and negative for HER2. She was started on combination therapy with letrozole (aromatase inhibitor) and palbociclib (cyclin-dependent kinase inhibitor). The patient had significant clinical and hematological improvement within few days after starting the combination therapy. Her repeat laboratory test results are reported in Table . Two months later, the patient presented to the emergency room with deteriorated clinical status and severe pancytopenia. Despite aggressive measurements, she succumbed to her illness.
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pmc-6324868-1
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History
An eight-year-old Caucasian female presented with intermittent bleeding from the eyes and oral cavity, which started when she was two years old. Her bleeding would be exacerbated by symptoms of the common cold. Based on similar bleeding that occurred in both her sister and maternal half-brother, the family expected that the bleeding would stop at one year of age. In kindergarten, she was diagnosed with a learning disability and was reported to be 17 months behind in her developmental goals. She was transferred to special education classes for further assistance.
Her birth history included a birth weight of four pounds (lb) and four ounces (oz) (1927.77 grams) and a premature gestational age of 36 weeks. Her head circumference at birth was below the fifth percentile, consistent with microcephaly. She was in the neonatal intensive care unit (NICU) for four weeks due to intrauterine growth restriction and severe respiratory distress. A cesarean section was done due to maternal bleeding and a previous cesarean section. Her mother reported her first words were at around three years of age. According to her past medical records, she presented with grunting and tachypnea at birth. She continued to grunt despite 30% oxygen therapy, which subsided after three hours. Her group B streptococcus testing was negative. She had feeding issues during the first six days after birth and lost six ounces of weight. A nasogastric feeding tube was used for an additional three days. There was an innocent heart murmur detected at birth, which shortly subsided. This similarly occurred in both her maternal half-brother and sister at birth.
The patient had one sister and one maternal half-brother. Her sister had nearly the same appearance as the patient, as described in the physical exam. Her brother and mother had autistic-like features. Her maternal half-brother’s neurocognitive function progressively worsened after three years of age. Her mother had a severe intellectual disability, attention deficit hyperactivity disorder (ADHD), and myopia. The patient’s sister also had microcephaly, which was not present in her maternal half-brother or mother. There was also a maternal uncle with mild mental retardation. Her father also had mild mental retardation, a learning disability, and ADHD. There was no other history of congenital disabilities, mental retardation, or any known genetic disease on either side of the family. The parents were Caucasian and non-consanguineous.
Physical exam
The current vital signs were as follows: temperature 97.1 degrees Fahrenheit (ºF), pulse 84 beats per minute, respiratory rate 21 breaths per minute, and blood pressure 100/68 millimeters of mercury (mmHg). Her body mass index was 13; height was four and a half feet, and weight was 44.8 pounds. Her current audiometry testing at age eight showed a score of 500/4000 in both ears. Her appearance was prominent for a narrow-shaped face with thin, brittle hair, short stature, an extremely thin habitus, deep-set eyes, and diffuse muscular atrophy. The rest of her physical exam was unremarkable.
Diagnostic testing
Images of the patient's anterior and lateral face are shown in Figures -, respectively. Figure demonstrates the patient's fine hair and narrow-shaped face. Figure shows the patient's flat facial profile, narrow-shaped skull, and micrognathia. These features added to the suspicion of a possible chromosomal abnormality. An X-ray image of the patient’s anterior face ruled out morphological abnormalities or fractures leading to the patient's recurrent bleeding, as shown in Figure . The patient's head circumference and weight and length trends from birth to 24 months and from 24 months to eight years are shown in Figures -, respectively. The patient's neonatal screen results were normal, as shown in Table . Coagulation testing and complete blood count results were also normal, as shown in Tables -, respectively. Platelet function testing showed low levels of adenosine diphosphate (ADP) expression. She was referred for genetic testing at four months of age. All of her immediate family members were also evaluated. A comparative genomic hybridization (CGH) microarray analysis was done and showed that the patient, her mother, sister, and maternal brother all had the 15q11.2 microdeletion. The 15q interval was flanked by segmental repeats; breakpoint segments one and two (BP I and II). The region included four, non-imprinted, highly conserved genes: TUBGCP5, NIPA1, NIPA2, and CYFIP1. The breakpoint start position was 20, 301, and 966. The breakpoint end position was 20, 779, and 211. The minimum size of the deleted segment was 477 Kb. Her mother and maternal brother also had a second chromosome deletion at chromosome 1p21.3. The patient and her sister did not have this second chromosome deletion. No other significant deoxyribonucleic acid (DNA) copy number changes or copy neutral loss of heterozygosity (LOH) was detected in the 1,800,000 region-specific single nucleotide polymorphisms (SNP). Fluorescent in situ hybridization (FISH) assay was also done on the patient and family members. However, the results were inconclusive. An electroencephalogram (EEG) was also conducted on the patient and showed no seizure activity.
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pmc-6325025-1
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A 38-year-old woman (gravida 4, para 2, abortus 1) at 12 weeks of gestational age was admitted as a case of missed abortion. Ultrasound (US) showed an intrauterine single nonviable fetus with a crown-rump length corresponding to seven weeks (Figure ).
The patient refused medical termination and preferred expectant management to wait for a spontaneous abortion. After two weeks, the patient presented back at the emergency department describing the new onset of mild vaginal bleeding. She had undergone two previous Cesarean deliveries; the most recent Cesarean was six years prior to this presentation to the emergency department. Other previous surgical procedures included an appendectomy and a partial gastrectomy.
The patient was admitted with minimal vaginal bleeding and mild lower abdominal pain. Her vital signs were stable, and her cervical os was closed during per vaginal (PV) examination.
The patient was treated with misoprostol PV. After three hours, she developed severe bleeding. A PV examination revealed the os was tip-of-finger, and a speculum examination revealed no cervical laceration. At this time, the patient was passing blood clots. Bedside US showed a bulky uterus containing retained products of conception and a uterine cavity filled with blood clots.
The patient was moved to the operating theater for evacuation and curettage (E&C). Uterine content and blood clots were evacuated by ovum forceps, but E&C failed to stop the current bleeding situation. A laparotomy was performed, and the abdomen was opened via a Pfannenstiel incision. Her uterus was intact, and no tear or perforation was noted. A diagnosis of placenta accreta was made intraoperatively. The placenta was in the lower uterine segment invading the old scar. Unilateral ligation of the uterine and ovarian arteries was performed, along with a local resection of the uterine wall segment affected by the placenta accreta. The uterine wall defect was repaired. This procedure succeeded in stopping the bleeding and a need for a hysterectomy was avoided. The patient received five units of packed red blood cells intraoperatively and four units of fresh frozen plasma. Following the procedure, she received an intramuscular injection of methotrexate 50 mg as an adjuvant therapy along with an antibiotic and anticoagulant. The patient was discharged five days following the operative procedure without sustaining any noted complications.
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pmc-6325026-1
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A 21-year-old man was admitted to the emergency department for repeated episodes of vomiting in the previous 24 hours, reporting recurrent dyspepsia and weight loss. His father died of gastric cancer one year before. Abdominal examination was unremarkable except for a mild epigastric tenderness; no masses were appreciated. His blood tests were normal.
A plain abdominal radiography showed no free intra-abdominal gas. A nasogastric tube was placed (yielding 150 ml of gastric juice) and the patient was hospitalized. He was treated with starvation, proton pump inhibitors, antiemetics and IV hydration therapy, with good clinical response. According to his personal history, an esophagogastroduodenoscopy (EGD) was performed showing a mucosal bulging without superficial abnormalities. Mucosal biopsies were negative for malignancy. Tumor markers were negative. For a better investigation, the patient underwent EUS (Figure ): a capsulated, mixed echoic lesion originating from the third and fourth layers of the antral wall, with a nodular hypoechoic portion (diameter 12 mm) and some anechoic components was detected. Fine needle aspiration (FNA) was performed but was inconclusive.
For further investigation, we performed an abdominal computed tomography (CT scan) that showed an 8 x 4 x 5 cm mass originating from the gastric antrum in the lesser curvature, with both fluid and solid well vascularized components and some enlarged celiac and mesocolic lymph nodes.
According to his symptoms, his personal history, the radiological and cytological findings exploratory laparoscopy was indicated. At operation, a huge mass was identified in the posterior wall of the gastric antrum, without serosal invasion. Furthermore, some enlarged celiac lymph nodes were detected and sampled (frozen histology was negative for malignancy). Due to the lesion size and the absence of preoperative histological diagnosis, a laparoscopic distal gastrectomy with D1 lymphadenectomy and Roux-en-Y gastro-jejunal anastomosis were carried out.
Gross histology showed a pancreatic heterotopy without malignancies, localized in the submucosa, muscularis propria and subserosa (Heinrich type I). Eight normal lymph nodes were found.
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pmc-6325026-2
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A 57-year-old man was admitted to the emergency department with acute epigastric and right hypochondriac pain with vomiting; the patient was afebrile. His medical history was positive for obesity, hypertension, recurrent dyspepsia and type II diabetes. No alcohol abuse was declared. At abdominal examination, no masses or tenderness were detected. Blood tests were normal except for white blood cell (WBC) 11000/ml, C-reactive protein (CRP) 4.4 mg/dl, serum lipase 483 U/l. Abdominal ultrasonography showed no gallbladder or biliary tract abnormalities, the pancreatic region was not explorable, no free air was detected. The patient was hospitalized and initially treated with starvation, proton pump inhibitors and IV hydration therapy.
In consideration of clinical presentation and unclear first imaging findings, to rule out the suspicion of pancreatitis, an abdominal CT scan was performed 48 hours later (Figure ): no pancreatic abnormalities were found, while a diffuse gastric wall thickening with a 2 x 3 cm intraparietal nodule in the lesser curvature and some enlarged locoregional lymph nodes were detected.
An EGD (Figure ) showed marked edema and hyperemia of the mucosa of the gastric body (especially the lesser curvature), but no vegetations were found. Mucosal biopsies were negative for malignancy or gastritis.
At further control, serum lipase value was reduced to 200 U/l and tumor markers were negative. His clinical status gradually improved with medical therapy. After seven days, abdominal CT scan was repeated, showing a reduction in the diameter of his gastric nodule (1.5 x 2.1 cm), while the locoregional lymph nodes were unchanged; no pancreatic abnormalities were detected. For further assessment, EUS was performed (Figure ): a mixed-hyperechoic lesion with unclear distal margins and some anechoic areas was detected in the fourth layer of the lesser curvature; some enlarged locoregional lymph nodes were found. FNA of the gastric lesion was inconclusive.
Exploratory laparoscopy was finally indicated. Loose adhesions between the posterior wall of the stomach and the pancreatic body were found, especially involving the proximal part of the lesser curvature, where a mass was detected. No serosal invasion was found. Some enlarged celiac lymph nodes were found and sampled (frozen histology was negative for malignancy). An intraoperative EGD was performed to better clarify the proximal margin of the lesion, but it was inconclusive. Therefore, laparotomy was indicated. The lesion proximal margin was identified 3 cm below the cardias and a subtotal gastrectomy with D1 lymphadenectomy and Roux-en-Y gastro-jejunal anastomosis was performed.
At gross histology (Figures , ), ectopic pancreas with signs of chronic inflammation was found in the muscularis propria of the lesser curvature (Heinrich type II). Nineteen normal lymph nodes were identified.
In both cases, the postoperative course was uneventful and the patients were discharged at their seventh postoperative day in good clinical conditions. At clinical follow-up one month later, the patients were asymptomatic and well, and their blood tests were unremarkable.
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pmc-6325030-1
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A 79-year-old asymptomatic Caucasian male presented with progressive renal failure with abnormal serum creatinine (range of 1.82 to 2.18) in July 2012. Complete blood count (CBC) showed red blood cell count (RBC) of 3.36 x 108/uL, hemoglobin 10.9 g/dl, hematocrit 33.1%, mean corpuscular volume (MCV) 99 fL, mean corpuscular hemoglobin (MCH) 32.8 pg, mean corpuscular hemoglobin concentration (MCHC) 33.0%, red cell distribution width (RDW) 15.8% and white blood cell count (WBC) 4,000/uL, with differentials: neutrophils 50%, lymphocytes 37%, monocytes 8%, eosinophils 4%, basophils 1% and platelet count of 178,000/uL. Comprehensive metabolic profile (CMP) revealed: glucose 96 mg/dL, blood urea nitrogen 23 mg/dL, creatinine 2.18 mg/dL, sodium 140 mmol/L, potassium 4 mmol/L, chloride 107 mmol/L, carbon dioxide 25 mmol/L, albumin 4.4 g/dL, calcium 9.1 mg/dL, bilirubin (total) 0.8 mg/dL, phosphorus 3.3 mg/dL and magnesium 2.7 mg/dL. Computed tomography (CT)-guided needle biopsy of the left kidney showed severe lymphocytic inflammation mainly in the areas of tubular atrophy and interstitial fibrosis (Figure ).
Immunohistochemical staining showed atypical lymphocytes that were positive for CD20 and negative for CD5, CD10, CD19, CD22 and CD23. Molecular testing revealed an Ig heavy chain gene rearrangement; these findings were consistent with marginal zone B cell lymphoma. Furthermore, immunofluorescence (IF) microscopy demonstrated seven glomeruli with diffuse pseudo-linear staining of the glomerular capillary loops for albumin (1+). Glomerular staining for IgG, IgA, IgM, C3, C1q and kappa or lambda light chain was negative. Interstitium stained positive for fibrinogen, while protein casts were stained positive for IgA (3+), kappa light chain (3+) and lambda light chain (3+). By electron microscopy (EM), the glomerular basement membrane had a normal trilaminar structure, the mean thickness was within the normal range without electron-dense deposits or tubuloreticular inclusions and the majority of podocytes foot processes were intact (Figure ). No immune complexes were detected by IF or EM.
On serum protein electrophoresis (SPEP) and immunofixation, we found IgM kappa monoclonal gammopathy and serum M-protein level of 0.3 g/dL. Quantitative immunoglobulin testing was performed for IgG (699 mg/dL), IgA (51 mg/dL) and IgM (470 mg/dL). Serum free light chain (SFLC) showed kappa (37.9 mg/L), lambda (1.58 mg/L) and kappa/lambda ratio of 23.99. Urine protein electrophoresis (UPEP) showed a protein level of 91 mg/dL and M-spike of 18.1%. Bone marrow (BM) biopsy showed 30% BM involvement by clonal B cells, morphologically consistent with marginal zone B cell lymphoma. Positron emission tomography (PET) scan was found normal in 2012 and 2013. The patient was treated with five doses of weekly rituximab and one dose of rituximab-bendamustine combination, but renal function worsened and treatment was stopped in 2013. The patient was monitored closely without further treatment until 2017. PET scan (2017) showed a mild enlargement of the retroperitoneal lymph nodes (aortocaval from 1 x 0.8 to 1.4 x 1.2 cm, left periaortic from 1.4 x 0.5 to 1.3 x 1.0 cm) and a partially calcified subcarinal node from 3.1 x 1.1 to 3.5 x 1.5 cm (non-avid), and the spleen size increased from 12.2 to 14.2 cm in cubic centimeter dimension (Figure ). One dose of vincristine and prednisone was given at that time, but renal function declined further. The patient was offered bortezomib/dexamethasone or ibrutinib for further treatment consideration, which he declined.
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pmc-6325068-1
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This is a case of a 30 year-old gentleman with a history of Crohn's disease. He was on a regimen of infliximab, infused every 8 weeks and oral methotrexate daily. He had no other significant medical history. He was in his usual state of health until he developed a sore throat and fevers on day 0. His symptoms began while he was traveling in Europe. During his trip, he took a 10-day course of amoxicillin and felt some improvement.
After returning to the US he felt well but on day 14 he developed fevers and sore throat again. He was evaluated at an urgent care clinic and sent home with a diagnosis of a viral syndrome and instructed to treat this with non-steroidal anti-inflammatory agents. He felt some improvement initially but presented again on the day of admission with concern that he may not be well enough for his infusion of infliximab. He was found to have a temperature of 38.3 C and on day 30 was sent to the emergency department for further evaluation.
In the emergency department, he reported a non-productive cough, fevers, and sore throat. He had elevated liver enzymes: aspartate aminotransferase (AST) 340, alanine aminotransferase (ALT) 540, alkaline phosphatase 145. Additional testing was sent including a Monospot test, cytomegalovirus (CMV) and Epstein's Barr virus (EBV) serum viral levels, respiratory viral panel by PCR, adenovirus serum viral level, HIV antibody/antigen as well as HIV viral level, viral hepatitis serologic panel, human herpesvirus type 6 (HHV6) serum viral level, Varicella zoster virus (VZV) serum viral level, and syphilis IgG. He was admitted to the inpatient medicine service for fevers and hepatitis of unknown origin. His social history was remarkable for frequent trips to the Midwest for work. He did not pursue outdoors activity while traveling. He did not smoke, drink alcohol or use illicit drugs.
On day 31 he developed daily fevers to 40 C and subsequently progressed to hypoxemic respiratory failure that required high-flow supplemental oxygen and transfer to the intensive care unit. He had a chest CT showing ground-glass opacities at the lung bases and a left upper lobe nodular opacity (, ). His CT also demonstrated tonsillar enlargement and splenomegaly. He had a laryngoscopy performed that revealed an exudative pharyngitis. He was noted to have atypical lymphocytosis that peaked to 12,000 cells/uL on day 33. He was started on broad-spectrum antibiotics for presumed hospital-acquired pneumonia; including coverage of atypical organisms. He underwent a bronchoscopy on day 33. Bronchoalveolar lavage (BAL) fluid was negative for Legionella culture, AFB stain and culture, pneumocystis stain, and bacterial culture. Mycoplasma, zygomycetes and Mycobacterium tuberculosis were not detected by PCR. A respiratory viral panel by PCR from the lavage fluid was positive for Bocavirus and one of 2 samples for aspergillus galactomannan was moderately elevated but later determined to be negative on repeat testing.
On day 33 his AST peaked at 351 and his ALT at 662. His viral studies sent earlier returned negative. He had further serologic testing for atypical organisms including Blastomyces, Coccidioides, Q fever, Bordetella pertussis, as well as urine Legionella antigen that all returned negative. He was started on methylprednisolone and his fevers improved. His antibiotics were discontinued when his bacterial cultures were negative at 48 h. On day 37 the fungal PCR from BAL returned positive for Histoplasma capsulatum and cultures from that fluid later grew the organism. Urine Histoplasma antigen testing, sent earlier during his hospitalization, ultimately returned with a titer of 2.16 ng/mL (normal high 0.1 ng/mL). His Coccidioides antibody was also positive, but this was felt to be due to cross-reactivity from his Histoplasma infection.
On day 37 he was started on liposomal amphotericin B and 6 h later he had his highest fever to 42 C along with hypotension, tachycardia and worsening hypoxemia despite having received a dose of methylprednisolone earlier that day. He was felt to have a paradoxical worsening with treatment and exacerbation of immune reconstitution inflammatory state rather than an infusion reaction due to amphotericin given this time course . His infliximab was restarted the following day with subsequent resolution of his fever, hemodynamic instability and improvement in his respiratory status within hours of this infusion. He continued amphotericin B for one week before transitioning to oral itraconazole. His ALT/AST improved significantly. He was discharged on oral itraconazole with continued clinical improvement and recovery as an outpatient.
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pmc-6325093-1
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A 75-year-old man underwent EVAR for a 6.1 cm abdominal aortic aneurysm. On follow-up CT angiography (CTA) imaging obtained 9 months later the aneurysm measured 6.8 cm and a T2E was seen arising from a lumbar artery (Fig. ). Given the persistent T2E and continued enlargement of the aneurysm the decision to treat was made.
The procedure was performed under conscious sedation. The common femoral veins were accessed bilaterally with micropuncture sets (Vascular Solutions, Inc., Minneapolis, MN). The right femoral vein micropuncture sheath was exchanged for a Rosch-Uchida transjugular liver access set (Cook Medical, LLC, Bloomington, IN). The left femoral micropuncture sheath was exchange for a 9F vascular sheath through which an IVUS probe (Volcano Corporation, San Diego, CA) was advanced into the IVC.
Under fluoroscopic and IVUS guidance the aneurysm sac was accessed near the endoleak with the Rosch-Uchida liver access set (Fig. ). The inner needle was removed, and contrast was injected through the catheter confirming correct positioning within the aneurysm sac. This straight catheter was exchanged over the wire for a 5F angle tipped catheter (Terumo Medical Corporation, Somerset, NJ) which was used to select the endoleak cavity. A Progreat microcatheter/microwire set (Terumo Medical Corporation, Somerset, NJ) was advanced through the catheter into the aneurysm sac and position was confirmed with contrast injection.
Next, the microcatheter was flushed with 5% dextrose solution. Ethylene vinyl alcohol liquid embolic (Onyx®18) (Micro Therapeutics, Inc., Irvine, CA) was then administered through the microcatheter into the aneurysm sac, in the region of the T2E, under sonographic and fluoroscopic guidance (Figs. and ). The embolization was terminated once the endoleak was no longer visualized with IVUS. The catheters and IVUS probe were removed. Completion cavogram was performed through the femoral vein sheaths. Femoral vein sheaths were then removed, and hemostasis was achieved at the venotomy sites with manual compression. Total procedure time was 2.5 h. Follow up imaging (Fig. ) demonstrated good radiographic results.
|
pmc-6325611-1
|
A 17-year-old boy, from the region of Cajamarca, high Andean area of Peru, without any relevant medical personal or familial history, was admitted to the Lambayeque Regional Hospital in April, 2017. For three days the patient had muscle weakness and paresthesia in the lower limbs with an ascending evolution and proximal predominance that made his condition worse, leading to prostration and arrival by emergency route. The patient arrived at the hospital awake, hemodynamically stable, with 24 rpm tachypnea. Thyroid gland was not palpable. A neurological physical examination showed weakness in the lower limbs without a defined sensory level, and reduced patellar and ankle reflexes. There was no evidence of bulbar muscle, respiratory and sphincter involvement.
Regarding serum electrolytes upon admission, they showed hypokalemia (1.44 mmol/L [normal values NV: 3.5–4.5 mmol/L]) without sodium, chloride or calcium alterations. Regarding the ancillary examinations upon admission: hematology tests were within the normal range; normal biochemistry values; elevated thyroid stimulating hormone (TSH) of 5.5 mU/ml ([NV]: 0.27–4.2 mU/ml); low free T4 of 0.78 ng/dl (NV: 0.9–1.7 ng/dl).
The patient was evaluated by the Department of Nephrology, Endocrinology and Neurology and diagnosed with hypothyroidism and hypokalemia. He received replacement treatment with normal saline solution, IV potassium and levothyroxine (T4) 25ug/day. On the fourth day of the treatment, he showed normal potassium values (3.7 mmol/L). After the patient’s clinical condition improved, one week after his admission to the hospital, he was discharged with diagnoses of hypothyroidism (etiology to be determined) and hypokalemia resolved.
Around five weeks after the patient was discharged, he was examined in the endocrinology office and did not show any symptoms. He was indicated to continue with T4 at 25ug/d. Glucose, urea, creatinine, prolactin, morning serum cortisol, testosterone, follicular stimulating hormone, TSH, and free T4 were determined in serum; all of them within normal range. Antithyroid antibodies were positive: anti-thyroglobulin 445.5 UI/ml, anti-TPO 48.20 UI/ml. The following elements were analyzed in 24 hour urine sample: sodium 255.66 mEq., chloride 55.1 mEq., and potassium 89 mEq. Urine test: leukocytes 1–3/field, red blood cells 1–3/field, density 100.5, pH 8, glucose, blood, proteins and leukocyte esterase, all of them negative. Kidney echography: Kidneys maintained their size with multiple images compatible with nephrocalcinosis, bilateral and vesical renal lithiasis. Contrast-enhanced CT of sella turcica was normal. Electrocardiogram: bradycardia (heart rate 52 bpm), signs compatible with hypokalemia (ST-segment descent, prominent U waves and pseudo-prolongation of the QT interval). No low voltage complexes were observed.
A left-sided X-ray (
) shows the bone age corresponding to 13 years and 6 months according to the Greulich and Pyle method
. From this, it can be concluded that the diagnosis is secondary hypothyroidism to thyroiditis and distal RTA.
Three months after the first episode, over a three week period, the patient stopped the intake of levothyroxine. Around one week later, a new episode of progressive muscle weakness occurred again, which was similar to the previous one. Two days later, the patient was evaluated at a private consultation. The administration of T4 at 50 ug/day was recommend. The symptoms continued progressing, and 5 days later, the patient was admitted again to our hospital by emergency presenting with flaccid quadriparesis with predominance in the lower limbs.
Laboratory tests on second admission: normal hematology values; normal biochemistry values. Urine tests: leukocytes 10–12/field, red blood cells 1–3/field, density 100.5, pH 8, glucose, blood, proteins, and leukocyte esterase, all of them negative. No provocation tests or genetic studies were performed in search of channelopathies. A urine culture and a thorax x-ray were conducted, and both of them showed a normal outcome. Blood gases and serial electrolytes tests shown in
were also performed. Periodic hypokalemic paralysis was diagnosed in addition to the previous diagnoses.
He received treatment with IV potassium, IV sodium bicarbonate and T4 25ug/day. The patient improved clinically and was discharged at day five, with T4 25 ug/day. We could not analyze trans-tubular potassium gradient because this test was not available in our hospital. Follow up is every three months with nephrology and internal medicine departments. After continued treatment with T4 was maintained by the patient, no additional oral bicarbonate administration was necessary.
|
pmc-6325615-1
|
A previously healthy 14 year-old Caucasian female patient weighing 44 kg underwent an elective outpatient adenotonsillectomy for an initial diagnosis of recurrent tonsillitis. General anesthesia was accomplished with a sevoflurane inhalational induction supplemented with intravenous propofol (2 mg kg
-1), morphine (0.1 mg kg
-1), dexamethasone (0.2 mg kg
-1) and ondansetron (0.1 mg kg
-1) after intravenous access was established. Direct laryngoscopy revealed a grade 1 Cormack Lehane view of her airway with moderate to large tonsils and an appropriately sized cuffed endotracheal tube was placed without difficulty. Anesthesia was maintained with 1.0 minimum alveolar concentration (MAC) of sevoflurane and she was ventilated with a tidal volume of 6 mL kg
-1, positive end-expiratory pressure (PEEP) of 4 cmH
20 and fraction of inspired oxygen concentration (FiO
2) of 0.3. Her preoperative hemoglobin was 136 g L
-1 with a hematocrit of 0.4 L L
-1. The surgery was complicated by a brisk arterial bleed with an estimated intraoperative blood loss of 600 mL. The patient was resuscitated with 500 mL Pentaspan and 1000 mL Lactated Ringers intraoperatively (for a total of 34 mL kg
-1) and she remained hemodynamically stable throughout the surgery. At the conclusion of the surgery, the patient was extubated fully awake with an oxygen saturation (SpO2) of 99% and transferred uneventfully fully monitored on 6 L min
-1 blow-by oxygen to the post anesthetic care unit (PACU) where there was one-to-one nursing care.
On initial assessment in the PACU, she was alert and oriented and talking in full sentences on arrival with an SpO2 of 95% on 10 L min
-1 supplemental oxygen by facemask. At this time, bright red blood was suctioned from her oropharynx. Over the course of the next 45 minutes she began spitting up pink frothy sputum and her SpO2 could not be kept above 92% despite supplemental oxygen at 15 L min
-1. Throughout her PACU stay, a further 250 mL of blood loss was recorded by nursing. The patient was treated with a total of 4 mg (0.09 mg kg
-1) of intravenous morphine for throat pain. During this 45 minute period, the patient was talking to the PACU staff, and there was no documented observation of the patient obstructing their airway. Fifty minutes after arrival in the PACU, the Staff Anaesthesiologist of record was notified of the above course of events in the PACU. A clinical examination revealed bilateral crackles and a lethargic patient requiring continuous positive airway pressure (CPAP) to maintain an SpO2 of 92%. A prompt arterial blood gas (ABG) was obtained, demonstrating a respiratory acidosis (pH 7.24/pCO
2 55 mmHg/pO
2 101 mmHg, calculated bicarbonate of 24 mmol L
-1 and base excess of -4mmol L
-1) with a hemoglobin of 79 g L
-1. Chest x-ray (CXR) revealed bilateral pulmonary edema (
).
The patient was taken emergently to the operating room where she had an uneventful rapid sequence re-intubation and surgical hemostasis was confirmed. An intraoperative bronchoscopy demonstrated pink frothy secretions consistent with pulmonary edema distal to the endotracheal tube, with no evidence of aspiration or frank blood in the airways (
). The distal airways were patent and otherwise normal. At this time she had persistent tachycardia up to 130 beats per minute and had intermittent hypotension with systolic blood pressure less than 100 mmHg. Hemodynamics stabilized with one unit of packed red blood cells (273 mL). Post transfusion hemoglobin was 101 g L
-1 and furosemide (10 mg; 0.23 mg kg
-1) was given to compensate in the event of volume overload or heart failure. The ABG continued to show a respiratory acidosis (pH 7.20/pCO
2 65 mmHg/pO
2 75 mmHg, calculated bicarbonate of 25 mmol L
-1 and base excess of -3 mmol L
-1) with an SpO
2 of 95%, positive end-expiratory pressure (PEEP) 9 cmH
20, FiO
2 of 1.0 and end tidal CO
2 (ETCO2) of 42 mmHg.
The patient was transferred from the operating room directly to the pediatric intensive care unit (PICU). A bedside transthoracic echocardiogram demonstrated normal left ventricle and right ventricle function, with an estimated left ventricular ejection fraction (LVEF) of 56% and an estimated right ventricular systolic pressure (RVSP) of 30–40 mmHg. At that point, she required manual ventilation to enable adequate oxygenation of non-compliant lungs. Her PaO
2/FiO
2 (P/F) ratio ranged from 87 to 156 during the first two hours in the PICU on a conventional ventilator (Maquet Critical Care SERVO-I ventilator system) however, the amount of PEEP required to provide adequate oxygenation/ventilation exceeded 12 mmHg resulting in a peak inspiratory pressure (PIP) greater than 40 cmH
20. Forty minutes after arrival in the PICU the patient was placed on a high frequency oscillatory ventilator (HFOV) with a mean airway pressure of 28 cmH
2O, amplitude of 70 cmH
2O, power of 5, and FiO
2 of 1.0, SpO
2 100%. She was administered a prophylactic dose of broad-spectrum antibiotics to treat any potential infectious cause to her presentation. Over the next 12 hours the HFOV ventilator settings were titrated down as serial CXRs showed improvement (
). She was transitioned to a conventional ventilator 13 hours after her arrival in the PICU on postoperative day one. She was extubated to CPAP (8 cmH
2O) 12 hours later and her CXR showed improvement of lung injury (
). After a further 12 hours of gradual tapering of CPAP therapy, the patient was transferred to the ward with humidified 15 L min
-1 blow-by oxygen on postoperative day two. Her ABG on 15 L min
-1 blow-by oxygen was pH 7.34/pCO
2 45 mmHg/pO
2 91 mmHg, calculated bicarbonate of 24 mmol L
-1 and base excess of -3 mmol L
-1). On postoperative day three, she was discharged home without any respiratory issues and not requiring the use of oxygen therapy. Antibiotic therapy was not continued as her infectious work up was negative. While in the PICU, her parents were questioned in further detail about the patient’s medical history including a comprehensive review of systems given the postoperative course of events. It was at this time the parents revealed for the first time that the patient had an ongoing history of night-time snoring, features consistent with obstructive sleep apnea from enlarged tonsils and adenoids.
|
pmc-6325663-1
|
A 66-year-old man with a 27-year history of diabetes was hospitalized because of severe morning hypoglycemia and postprandial hyperglycemia.
At age 39, he had shown symptoms of polyuria and was diagnosed as having type 2 diabetes. At the initial treatment, an allergic skin reaction to porcine insulin occurred, requiring a switch from insulin to oral hypoglycemic agents. He often missed clinic visits, which contributed to very poor glycemic control. He was then hospitalized for glycemic control (HbA1c: 11.7% (104 mmol/mol)) when he was 54 years old. During this hospitalization, his fasting serum C-peptide level was 1.41 ng/mL and blood glucose levels were promptly improved by dietary treatment with oral hypoglycemic agents (sulfonylurea and α-glucosidase). He underwent photocoagulation therapy for proliferative-diabetic retinopathy and his creatinine level was 1.0 mg/dL at this time.
At age 56, premixed human insulin 30/70 was administered after an episode of diabetic ketoacidosis with subcutaneous abscesses, but, again, due mainly to his poor adherence, his glycemic control had remained very poor with HbA1c of approximately 10.0% (86 mmol/mol). Then, a complete cessation of treatment for three years resulted in a marked HbA1c increase to 18.9% (183 mmol/mol). At this point (61 years old), multiple daily insulin therapy using insulin analogs, i.e. aspart before each meal and detemir before bedtime, was introduced and his HbA1c levels gradually decreased. However, after one year of treatment with insulin analogs, hypoglycemic attacks in the morning manifested. In addition, postprandial hyperglycemia developed and his severe glycemic fluctuations were not reduced by switching basal insulin from detemir to degludec and glargine. The plasma creatinine level was maintained at approximately 1.0 mg/dL with proteinuria for three years after the beginning of the hypoglycemic episodes, but had recently risen to nearly 2.0 mg/dL. He had hypertension and developed peripheral artery disease with mild claudication. To prevent exacerbation of vascular complications, the patient has been treated with an angiotensin II receptor blocker, since age 64 years. He was diagnosed as having hypothyroidism with negative thyroid antibodies and had been treated with levothyroxine since age 65 years. To examine the mechanism underlying his glycemic fluctuations and to devise an effective treatment strategy, he was admitted to our hospital.
On admission, his fasting blood glucose level and HbA1c were 62 mg/dL (3.4 mmol/L) and 9.3% (82 mmol/mol), respectively. Total insulin level was extremely elevated at 4500 μU/mL. The total insulin values represented both endogenous and exogenous insulin because not only human insulin but also recombinant insulin analogs cross-react with the ARCHITECT® insulin assay (Abbott Laboratories) []. He was positive for insulin antibodies with the binding rate being 80.4% and Scatchard plot analysis (on the 2nd day of hospitalization) revealed 0.0194 × 108 M− 1 (K1) and 184 × 10− 8 M (R1) (Fig. ), indicating high binding capacity and low affinity. The characteristics were similar to those of the autoantibodies reported in IAS patients. His HLA (human leukocyte antigen) haplotypes were HLA-DRB1* 040101/ HLA-DRB1*0406 and HLA-DQB1*030201/ HLA-DQB1*050101 and, among them, HLA-DRB1*0406 is associated with the highest risk for susceptibility to IAS []. Insulin-specific IgE antibody and islet-related autoantibodies, such as those against glutamic acid decarboxylase (GAD) and insulinoma-associated antigen-2 (IA-2) were undetectable. Counter regulatory hormones against insulin action were not lowered (Table ). Estimated glomerular filtration rates were between 13 and 25 mL/min/1.73m2, showing chronic renal failure. Serum hepatic enzyme levels were within normal ranges. Neither computed tomography nor magnetic resonance imaging revealed any tumors in the pancreas, excluding the existence of insulinoma. Taking these findings together, the IAS-like insulin antibodies were considered to be the cause of glycemic instability in this patient.
First, to prevent the frequent hypoglycemic attacks, we repeatedly adjusted the treatment regimen (Table ). We stopped insulin glargine before bedtime, followed by cessation of insulin aspart before each meal. At 36 h after the cessation of all insulin administrations, insulin levels fell to 2500 μU/mL and glucose levels were elevated and remained over 360 mg/dL (20 mmol/L) throughout the day, indicating supplemental insulin, either exogenous or endogenous, to be necessary for prevention of hyperglycemia. Next, bolus insulin, restarted with altered formulations comprised of regular human insulin or insulin lispro, inhibited neither postprandial hyperglycemia nor morning hypoglycemia. Therefore, instead of increasing exogenous insulin, we administered other agents to selectively suppress postprandial hyperglycemia.
First, liraglutide, and then voglibose were added to insulin treatment, but continuous glucose monitoring (CGM) using iPro2® (Medtronic Japan, Tokyo, Japan) revealed that treatment with insulin lispro 3 U before each meal, liraglutide 0.9 mg before breakfast and voglibose 0.2 mg before lunch was not able to suppress either marked diurnal hyperglycemia or the early-morning hypoglycemia (Fig. a). Voglibose could not be used more than once a day due to exacerbation of diarrhea. The replacement of insulin lispro with mitiglinide before each meal effectively inhibited glycemic fluctuations. Typical CGM data are shown in Fig. b. The mean amplitude of glycemic excursion values for 72 h (analyzed on 51st-53rd and 61st-63rd days of hospitalization) was decreased from 216 mg/dL (12 mmol/L) to 157 mg/dL (8.7 mmol/L) by the replacement of insulin lispro with mitiglinide. To precisely evaluate endogenous insulin secretion, we used the Chemilumi C-peptide kit (Siemens Healthcare Diagnostics), which shows negligible cross-reactivity with proinsulin. Although serum C-peptide remained high due to renal failure, mitiglinide significantly raised serum C-peptide levels 2 h after, as compared with those before meals (Fig. ), indicating increased postprandial insulin secretion. Furthermore, the replacement of insulin lispro with mitiglinide decreased insulin levels from 3500 μU/mL to 2640 μU/mL, despite the persistently high binding rate (84.2%), low affinity (K1 = 0.0236 × 108 M− 1) and high capacity (R1 = 84.4 × 10− 8 M) of insulin antibodies (analyzed on the 61st day of hospitalization).
|
pmc-6325678-1
|
A 53-year-old white man presented to our knee clinic with knee pain. The pain was located in the posteromedial aspect of his left knee and first presented whilst training for a marathon. The pain was a continuous dull ache, which would often wake him from sleep. He had no improvement from conservative management trialled by his general practitioner, which included rest, ice, elevation, orally administered non-steroidal anti-inflammatory drugs, and physiotherapy. There was no history of trauma, locking, or giving way of the knee. He was otherwise fit and well with no medical co-morbidities; he was very active and had not had any previous injuries or surgeries to his left knee.
A physical examination revealed normal alignment of his knee and hindfoot, no effusion, and an area of point tenderness posteromedially, not over the hamstrings or the pes anserinus. There was full range of movement with a positive medial step off and good tracking of the patella with no gross patellofemoral crepitus. He also did not have any significant ligamentous instability and an examination of his ipsilateral hip joint was normal.
Plain radiographs taken at the time of presentation did not reveal any significant abnormalities and magnetic resonance imaging (MRI) was organized, which demonstrated the presence of a cord-like structure that originated from the fabella and passed medially, dividing into two parts around the semimembranosus tendon (Fig. ). The superficial part appeared to blend in with the semimembranosus tendon sheath itself, whereas the deeper part was thought to blend in with the superficial fascia of the gracilis and semitendinosus. This was associated with the presence of diffuse thickening of the distal semimembranosus tendon suggesting impingement of the tendon (Fig. ).
As he continued to be symptomatic, and conservative measures had failed, he underwent a knee arthroscopy which demonstrated a grossly thickened semimembranosus with fluid collection around it. A band arising from the fabella, running transversely across the popliteal fossa and around the semimembranosus tendon was noted, confirming the diagnosis of semimembranosus impingement. This band, thought to be congenital in nature, was divided, and the semimembranosus fully released (Fig. ).
Postoperatively, he recovered well and was allowed to fully weight bear with crutches. He was followed up at 6 weeks post-surgery, at which time his symptoms had resolved and he was back to training for a marathon.
|
pmc-6325729-1
|
D.M., a 72-year-old man, was admitted to our surgical unit on June 2014 with a radiological diagnosis of a suspected malignant lesion of the liver. In the clinical history: arterial hypertension, chronic renal failure and gout. He was obese (BMI 30) with a history of chronic alcoholic abuse. Among the surgical antecedents, a subtotal gastrectomy for peptic ulcer and a complex surgery for left renal cancer (left nephrectomy, distal spleno-pancreatectomy and reno-caval thrombectomy) in 1992, at the pathological examination, it revealed to be a pT3N0 well-differentiated renal adenocarcinoma, with neoplastic caval thrombosis.
After the surgery, he attended a regular follow-up, that was negative till April 2011, when the abdominal CT revealed the presence of a solid focal lesion in the eighth liver segment (size 2.3 cm) characterized by poor vascularization and fatty component; alpha-fetoprotein was negative. According to CT, the hepatic lesion was classified as an indeterminate nodule (Fig. ). MRI was not conclusive regarding the nature of the lesion and it was not typical neither for a hemangioma nor for metastasis (Fig. ). Also, positron emission tomography (PET) was negative for suspected malignant lesion of the liver, even if it has a low reliability in excluding a metastatic renal cancer. In consideration of the patient’s neoplastic history, an US-guided liver biopsy was then performed. The histological report was negative for neoplastic cells but a severe microvesicular steatosis was discovered, expression of alcoholic damage, together with an activation of Kupffer cells and a focal accumulation of histiocytis inside a granulomatous-like lesion, with enlarged cytoplasm containing crystals. At immunohistochemistry, vimentin was positive inside the histiocytis (Fig. ). So, at the end of the diagnostic phase, no suspect arose from the instrumental evaluation and the diagnosis was urate tophus of the liver. The patient continued the treatment of hyperuricemia based on oral allopurinol.
Owing to the risk of both primary and metastatic liver cancer, the patient was addressed to an instrumental follow-up each 6 months. After a first negative control, a progressive increase in the size of the hepatic lesion was noted (5.4 cm on October 2013). We took charge of the patient for the first time on May 2014. At this time, MRI showed exactly in the same area previously affected by the gouty lesion a solid nodule of 5.4 cm in size, characterized by irregular structure and rich in adipose tissue, suggestive for hepatocellular carcinoma with mosaic pattern; no evident cleavage was noted between the nodule and the middle and right hepatic veins (Fig. ). The patient was therefore submitted to CT-guided percutaneous biopsy. A macro- and microvesicular steatosis was noted without clear atypical components apart from scarce multinucleate cells; the reduction of the glycogen amount together with the straight reduction in the reticulin pattern suggested a nodule of adenomatous hyperplasia with incipient neoplastic transformation.
The patient was evaluated for surgery: he was classified as Child score A5 and MELD score 10. The viral hepatitis markers were negative. Due to the size (> 5 cm) and the site (adhesion to two suprahepatic veins) of the nodule, only surgery was considered as a radical approach. We tested the liver function and we found out a liver remnant volume after right hepatectomy of 20% with indocyanine green test (ICG) at 15′ of 16%). For these reasons, we preferred a conservative approach, also considering the good biological features of the lesion (expansive nodule with a complete capsule and favourable grading).
On June 4, 2014, we performed the resection of the VIII and the V hepatic segment; it was possible to spare both the hepatic veins (Fig. ). The post-operative course was characterized on day 3 by transient respiratory failure associated to pneumonia that required non-invasive respiratory support with continuous positive airway pressure (CPAP); after 24 h, the support was stopped as the physiological respiratory function was restored. The following post-operative course was uneventful.
The histological examination of surgical specimen confirmed the presence of encapsulated hepatocellular carcinoma with favourable grading. The non-neoplastic hepatic parenchyma was characterized by 10% of steatosis without evidence of alcoholic cirrhosis. The suspected satellite nodules observed at the macroscopic examination of the specimen just at the border of the lesion revealed to be necrotic tissue including many needle-shaped structures typical for gouty tophi (Fig. ).
The patient has been followed up by our periodic checks since today and he is free from neoplastic disease; otherwise, no other systemic localization of tophi was discovered.
|
pmc-6325776-1
|
A 41-year-old female was admitted to hospital because of unilateral proptosis in the left eye developing for about six-months. She had suffered from HT for the past 2 years and had been treated with levothyroxine 25 μg daily. She did not present any other significant comorbidities and had never smoked. Her previous personal and family history was negative for thyroid disorders. Laboratory results indicated euthyroidism - TSH level was 2.67 μU/ml (reference range 0.27–4.20 μU/ml), the free T3 and free T4 concentrations were 4.97 pmol/l (reference range 3.90–6.70 pmol/l) and 13.58 pmol/l (reference range 11.5–21.0 pmol/l), respectively. Thyrotropin receptor antibodies (TRAb) concentration was normal (TRAb 0.9 IU/l, reference range < 2). However, anti-thyroid peroxidase (TPOAb) serum levels and anti-thyroglobulin autoantibodies (TgAb) were significantly elevated: 279 IU/ml (reference range 0–34 IU/ml) and 194 IU/ml (reference range 10–115 IU/ml), respectively. The patient’s 25-OH vitamin D serum level was 25 ng/ml indicating mild vitamin D deficiency. Ultrasound examination demonstrated a thyroid gland with features suggesting chronic autoimmune thyroiditis (heterogeneous decreased echogenicity, no focal lesions, normal size and vascularity).
In the neutral position, the left eyeball was positioned convergently and downwards which implied extraocular muscle involvement. In addition, the patient also presented conjunctival erythema, eyelid redness and edema, and an enlarged, swollen lacrimal caruncle (Fig. ). Von Graefe’s, Stellwag’s, Kocher’s and Moebius' signs were positive in the left eye, whereas the Rosenbach’s sign was negative. On ophthalmic examination, vision acuity was not compromised (6/6 in both eyes). The intraocular pressure was mildly elevated in the left eye (24 mmHg), and it was normal in the right eye (19 mmHg). The patient received 4/7 points in the Clinical Activity Scale (CAS) due to the eyelid edema, redness, conjunctival injection, and inflammation of left caruncle. Furthermore, the severity of TAO was classified as IV in the NO SPECS scale due to extraocular muscles involvement in the left eye (I-0, II-a, III-0, IV-b, V-0, VI-0). In terms of the right eye, she received 0/7 points in CAS, and class 0 in NO SPECS scale.
Magnetic resonance imaging (MRI) of the orbits revealed unilateral left-sided exophthalmos (Fig. ), predominantly caused by the thickening of the medial rectus muscle (Fig. ), with an increase in T2 signal intensity (Fig. ) and prolonged T2 relaxation, indicating an active form of TAO. On MRI a 23 mm cyst was found inside the left maxillary sinus. CRP concentration was 0.3 mg/l (normal < 5 mg/l), the patient had no history of sinusitis, and presented no clinical symptoms on laryngological examination. The therapy included the administration of glucocorticosteroids intravenously according to the scheme designed and widely applied at our department, i.e. three pulses of intravenous methylprednisolone (1 g of methylprednisolone per pulse on three consecutive days), followed by an intramuscular injection regimen: one dose every three weeks (120 mg, 80 mg, 40 mg), i.e. a cumulative dose of 3.240 g of methylprednisolone during a 9-week follow-up period. The therapy was very well tolerated, with no severe side-effects observed. Simultaneously, the levothyroxine dose was increased from 25 to 50 μg per day in order to keep TSH in the lower half of the normal range. Additionally, the patient also received selenium supplementation (200 μg daily) and vitamin D (4000 IU daily). The administered therapy resulted in a complete remission of ophthalmic symptoms, and the patient has been free of symptoms in the course of a 6-month follow-up period (Fig. ). The timeline representing all vital steps in the patient’s diagnosis and therapy is presented in Fig. .
|
pmc-6325787-1
|
A 49-year-old man complained of right upper quadrant abdominal pain and jaundice for 2 days and was hospitalized in the Fifth Affiliated Hospital of Sun Yat-sen University. The pulse rate of the patient was regular and the temperature and blood pressure were normal. The patient had a clear dietary history of eating raw freshwater fish, however, no eggs of parasites were detected in the stool specimen by direct smear method (each specimen was smear onto 3 labeled slides). Magnetic Resonance Cholangiopancreatography (MRCP) revealed obstruction of the common bile duct by a stone with obviously diffuse dilation of intrahepatic ducts (Fig. ). Initial laboratory data indicated obstruction jaundice and liver enzymes elevation (Table ). Considering that the cholangitis was caused by the common bile duct stone, the gallbladder stone and cholecystitis, The laparoscopic cholecystectomy and laparoscopic common bile duct exploration was performed. A flat, leaf-like worm was found under the choledochoscope at the extremitas inferior common bile duct during the operation (Fig. ). After the surgery, a “T” shape catheter was inserted into the common hepatic duct to establish drainage. A course of anthelmintic therapy (albendazole: 16 mg/kg/day for 4 days) was administrated. During the subsequent days, the adult worms were observed in the bile duct through the “T” shape catheter. The pain of the patient relieved totally, the jaundice faded gradually and liver function indices were nearly normal.
|
pmc-6325787-2
|
A 40-year-old female patient had suffered from upper quadrant abdominal pain, with occasional nausea and fever for more than 2 years. The patient was diagnosed as cholecystitis at another hospital nearby and felt relieved very soon after treatment in the past 2 years. She came to the hospital 11 days ago due to the severe upper quadrant abdominal pain recurred with severe jaundice. Complete medical examinations, including blood pressure, pulse rate, temperature and physical examination of the abdomen, were performed in the Fifth Affiliated Hospital of Sun Yat-sen University. The clinical examinations revealed that the pulse rate, temperature and blood pressure were normal. Stool microscopy for parasite eggs by direct smear method were negative 3 times. Computed tomography (CT) scan revealed obstruction of the bile duct with dilation of the intrahepatic ducts which suggested a retained bile duct stone and a gallbladder stone (Fig. ). Laboratory data indicated obstruction jaundice, peripheral eosinophilia and liver enzymes elevation (Table ). Detailed inquiry revealed she had a history of eating raw freshwater fish. A clinical diagnosis of acute cholangitis and cholecystitis was made and laparoscopic cholecystectomy and laparoscopic common bile duct exploration was performed. Many flat, leaf-like worms appeared under the choledochoscope as deep bile duct cannulation (Fig. and Fig. ). Besides, many nodules distributed dispersedly among the surface of the liver (Fig. ). After the operation, the“T” shape catheter was placed in the common hepatic duct to allow patent drainage. The patient was treated with anthelmintic therapy (albendazole: 16 mg/kg/day for 4 days). More C. sinensis worms were drained through the “T” shape catheter (Fig. ). The clinical status of the patient improved gradually without the pain recurring.
|
pmc-6325788-1
|
A 44 years-old woman presented with right breast pain, swelling and nipple retraction. Breast ultrasonography (US) showed an irregular hypoechoic mass (30 × 10 mm) in the right retro-areolar space; a further lesion (maximum diameter 8 mm) was detected in the right upper inner quadrant. Lymph-nodes with a maximum diameter of 25 mm were also detected in the right axilla by US. A core needle biopsy revealed a poorly differentiated (G3), estrogen receptor (ER) positive (ER+) [65%], progesterone receptor (PgR) positive (PgR+) [50%], cell proliferation antigen (Ki-67) 70%, human epidermal growth factor receptor-2 (HER-2 neu) negative, IDC. A total body Computed Tomography (CT) showed no evidence of metastatic disease. After four cycles of neoadjuvant chemotherapy with epirubicin 100 mg/m2 and taxol 175 mg/m2 every 21 days, a right “skin sparing” mastectomy and axillary lymph node dissection was performed. Immunohistochemistry confirmed G3 luminal B/HER-2 neu negative IDC subtype. Eleven out of fifteen axillary lymph nodes showed metastatic deposits (TNM: pT4b N3a M0). After chest wall radiotherapy including supra−/infraclavicular lymphatic drainage area, the patient started further eight cycles of adjuvant chemotherapy with taxol 175 mg/m2 every 21 days. Tamoxifen 20 mg daily and triptorelin 3,75 mg once a month for 3 years, and letrozole 2.5 mg daily in the following 3 years were used. Seven years after the diagnosis, while still under letrozole-based hormonal therapy, the patient displayed diplopia, blurred vision, and significantly restricted upward right eye movements (Fig. ). Ocular acuity decreased from 7 to 2/10 in both eyes. A brain Magnetic Resonance Imaging (MRI) showed a lump involving the right inferior rectus extraocular muscle (Fig. a). Computed Tomography (CT) confirmed this finding without showing other sites of metastasis. A transpalpebral biopsy revealed breast cancer metastasis (ER 50%, PgR 0%, Ki67 35%, HER-2 neu negative) (Fig. ). The patient underwent orbital Stereotactic Body Radiation Therapy (40 Gy in 5 days), combined with fulvestrant 500 mg day 1, 15, 29 and, subsequently, every 28 days. A brain CT and MRI, performed 2 months later to evaluate the treatment response, showed a shrinking of the orbital mass. (Fig. b). One month after MRI, following mediastinal lymph nodes enlargement, a third line therapy using palbociclib 125 mg daily for 3 weeks/month was started. Twelve-months later, MRI showed no residual tumor mass (Fig. c) and Positron Emission Tomography (PET) confirmed no uptake in the orbit. Despite tumor regression, the right eye sight failed to improve. Currently, the patient is alive and in good general conditions after 20 months under anti-hormonal-based therapy. Patient provided informed consensus to publish her case and release case details and other personal information and images.
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pmc-6325842-1
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A 63-year-old female presented with a palpable mass in her left breast for 3 years. The lump was gradually progressive in size for the past 3 years. Physical examination revealed a painless, ill-defined, hard, large mass with no nipple discharge in the upper outer quadrant of the left breast. Skin dimpling and ulceration were also seen. The patient had no past or family history of a breast disease. A modified radical mastectomy was performed. The CAF chemotherapy was administered after surgery.
Grossly, the left breast specimen showed an ill-defined, red gray, multiple nodular, 14 × 12 cm tumor with surface skin ulceration [Fig. ]. The cut surface revealed multiple cystic spaces filled with thick, gelatinous secretions and gray-white solid areas. The individual cysts varied from 0.2 cm to 2.5 cm in dimension with cysts wall thickness from 0.1 cm to 0.5 cm. Hemorrhage and necrosis was evident.
Microscopically, multiple variable-sized cystic spaces filled with thyroid colloid-like eosinophilic secretions [Fig. ] which was diastase resistant PAS positive and thyroglobulin negative. The eosinophilic secretions were retracted from the surrounding epithelia, producing scalloped margins. The cyst lining epithelium exhibited a variable pattern. In some areas the lining was flat to cuboidal epithelium and devoid of cellular atypia [Fig. ]. In other areas the epithelium showed a proliferative change in the form of pseudo stratification, knobby tufts [Fig. ], micropapillary [Fig. ] and cribriform [Fig. ]. An invasive component comprising of irregular neoplastic glands or nests was seen [Fig. ]. Eight axillary lymph nodes showed macro metastasis and cystic areas were also seen in the lymph node metastases [Fig. ]. Immunohistochemistry shows, the cystic contents were negative for thyroglobulin. Prognostic markers were ER negative, PR negative and HER2 3+. Ki67 was 30% positive. A diagnosis of invasive CHC with axillary node metastasis was made.
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pmc-6325858-1
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We report the case of a 42-year-old Chinese man with a history of chronic tophaceous gout who presented with back pain 2 years ago. The pain was sudden, located at his lower back, radiated to his left lower limb, persisted for a few days, and was subsequently relieved with non-steroidal anti-inflammatory drugs (NSAIDs). There were no neurological abnormalities at that time and further investigations were not performed. He continued to experience episodes of the same back pain over the next 18 months. Two months prior to hospitalization, he had another episode of severe back pain which radiated down to his left lower limb with weakness of his left lower limb. There was no history of trauma, prolonged fever, cough, hemoptysis, loss of appetite, loss of weight, or incontinence.
His past medical history included gout which was diagnosed 4 years ago. He had monthly recurrent gouty arthritis, which affected his first metatarsophalangeal joints, ankles, knees, and shoulders. He noted multiple swellings over his limbs for the past 3 years. During this period, he self-medicated with NSAIDs which terminated the gouty arthritis episodes. He did not seek any medical treatment for urate-lowering therapy.
A physical examination showed normal cardiovascular, respiratory, and abdominal systems. There were multiple tophi seen over the dorsum of bilateral hands, bilateral elbows, bilateral ankles, and toes. A neurological examination showed normal tone in his bilateral lower limbs. Power was reduced for left thigh flexion and extension (3/5) and knee flexion (4/5). His left knee jerk reflex and left ankle jerk reflex were reduced. Sensation was reduced at left L4 and L5 dermatomes. There was no sensory level. His anal tone was normal. Neurology of his upper limbs was normal.
Full blood count: total white cell, 18 × 103/μL (3.99–10); hemoglobin, 11 g/dL (12.1–18.1); platelets, 526 × 103/μL (142–424). Creatinine was 165 μmol/L (60–120). Creatinine clearance was 58 ml/minute. Sodium was 130 mmol/L (135–145), potassium was 3.2 mmol/L (3.3–5.1), and urea was 7.6 mmol/L (1.7–8.3). Uric acid was 524 μmol/L (202–420). C-reactive protein (CRP) positive was 96 mg/L. An echocardiogram showed no vegetations. A chest radiograph was normal. Lumbosacral radiographs showed irregularities of the L4, L5, and S1 endplate with reduction in L4/L5 and L5/S1 intervertebral discs space and L5 vertebral body (Fig. ). MRI of his spine showed hyperintensity within the intervertebral discs spaces of L4/L5 and L5/S1 on T2-weighted imaging (T2WI) in keeping with fluid within (Fig. ). There was also irregular endplate erosion manifested as hypointensity on T1-weighted imaging (T1WI; Fig. ) which demonstrated heterogenous enhancement of the involved vertebral endplate and epidural components post contrast (Fig. ). The initial diagnosis was epidural abscess with spondylodiscitis. Staphylococcus aureus or Mycobacterium tuberculosis infection was suspected. He was started on intravenously administered cloxacillin. Investigations for tuberculosis were negative. Blood cultures were negative. A percutaneous biopsy was not performed as the clinical suspicion for epidural abscess and spondylodiscitis was high and the differential diagnoses of tumor and spinal tophi were not suspected. He underwent surgery to drain the abscess and laminectomy and posterolateral fusion.
Operative findings showed chalky white non-adherent material over the facet joints resembling gouty tophi. A small mass lesion with bony erosion was noted over the left L4/L5 facet joint extending and causing a small bony defect on the left side of the L4 lamina. There was no pus or slough seen at the operative site. Pedicle screws were inserted at the desired lumbar and sacral levels, mainly from L3 to S1. Laminectomy was performed at the L4 and L5 levels. Pre-contoured rods were inserted on both sides followed by posterolateral fusion. He was treated for spinal tophi with colchicine.
The vertebral disc was sent for histopathological examination but yielded necrotic tissue only. Tissue cultures were negative, acid-fast bacilli smears were negative, and tuberculosis culture was negative.
During the admission, he had a flare of gouty arthritis of his right wrist and metacarpophalangeal joints. He was started on colchicine and a course of steroids and the gouty arthritis subsequently resolved. During follow up, his back pain improved and he was started on allopurinol for urate-lowering therapy.
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pmc-6325863-1
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A 70-year-old man with antecedent of follicular lymphoma in complete remission presented at the Timone University Hospital (Marseille, France) in 2016 for a squamous cell carcinoma of the hypopharyngeal region. The patient categorically refused any treatment, including preservative surgery, radiotherapy, chemotherapy or supportive care.
One year later, he was addressed to our palliative care unit by the hand-surgery department after attempting suicide. The patient explained his action by the fear of suffering. No depressive state was diagnosed by our psychiatrists. Despite persistence fear of suffering, the patient rejected the idea of suicide because of his family, but still wanted to die and asks for assistance. Information on Claeys-Leonetti law was given, especially on assisted-suicide banishment and on the possibility to relieve suffering with adapted treatments.
One week after discharge, the patient was readmitted to our department for dyspnea and anxiety. Symptoms were managed by appropriate treatments (oxygen and low dose of midazolam in an anxiolytic purpose). Despite stabilisation, the patient was afraid of dying suffocated and asked for deep and continuous palliative sedation until death. Apart from the fear he expresses, the patient has no symptoms of anxiety, depression or pain after the introduction of appropriate treatments. On the other hand, he clearly states that he refuses to live again knowing that his death is approaching and that he is apprehensive of suffering. He says he wants to rush his death. For us, this is a request for assisted-suicide (active help from a third party for the administration of a lethal product) or euthanasia (act of a third party which intentionally provokes the death of another to put an end to his sufferings), rather than a real demand for deep and continuous sedation. It seems important to note that patient’s requests for deep and continuous sedation until death are not registered officially. The law does not even impose a written request. Thus, the request is most often made orally in the presence of several doctors and clinicians.
In order to try to objectify this request and therefore our answer, the patient’s request was examined and denied by palliative multidisciplinary board, in accordance with by the French Oncology Coordination Centre guidelines. This situation did not fulfil the criteria requested by Claeys-Leonetti law. Indeed, prognosis appeared not short term committed (no visible clinical progression of the disease, which commits for sure the short-term vital prognosis), symptoms were managed with appropriate treatments and no life-sustaining treatment arrest could lead to potential unbearable sufferings. Regarding the short-term criterion of life-threatening prognosis, the patient was offered to have a Computed Tomography (CT) scan to measure the progression of the disease. Indeed, no imaging had been performed for one year (time of diagnosis of recurrence). The patient refuses this proposal. The request for deep and continuous sedation was reiterated several times by the patient, who was still refusing any investigations to define the progression of his cancer and wanted parenteral hydration to be maintained. Daily, he questioned each caregiver about the rationale for the refusal of his request. How can the medical staff be sure that his prognosis is not short-term compromise? Why his psychological distress could not be considered as refractory? One week after refusing further investigation, the patient finally agrees to undergo a CT scan. Three days after the exam he dies peacefully, according to our team (no specific questionnaires or objective elements to judge the quality of death exists), of a not predictable respiratory distress certainly linked to the evolution of his cancer of the hypopharyngeal region without introduction of deep and continuous sedation, but with introduction of midazolam for anxiety. Opiates were not introduced because the patient was saying not being painful. The CT scan results, unknown at the time of death, reveal nothing conclusive (pulmonary metastases, but no lymph node involvement) and would have required additional analyzes.
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pmc-6325864-1
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A 64-year-old female patient was admitted to our department for multiple pulmonary lesions discovered by health examination for 1 month. She was asymptomatic, in good health, and had no history of pulmonary or neurologic disease. She denied history of tuberculosis, and she was a non-smoker.
Chest computed tomography (CT) revealed multiple thin-, smooth-walled cysts or cystic nodules with solid component were scarred within the lung parenchyma, sized from 0.8 cm to 2 cm (Fig. a, c). Enhanced CT scan revealed a 3.4 cm (cm) rounded mass located in the right posterior mediastinum at the inferior pulmonary vein plane (Fig. b). It showed slight enhancement in the enhanced CT scan. The result of preoperative brain magnetic resonance imaging was also negative.
Thoracoscopic lung resection was scheduled for this patient. During the operation, the larger mass, which enveloped by fat-like thin films, was located on the surface of right lower lobe rather than the mediastinum. The tumor was stripped out successfully with a right-angle electrode and we also wedge resected two cystic lesions for pathological examination (Fig. d).
Routine pathological examination of both major mass and cystic lesions revealed the tumor consisting of spindle cells arranged in swirls scattered with a small amount of typical psammoma body (Fig. a, b, c hematoxylin and eosin staining, X100). Immunohistochemical (IHC) staining was performed for both major mass and cystic lesions and all lesions were positive for epithelial membrane antigen (Fig. d, X200), CD34(Fig. e, X100), progesterone receptor (PR), Ki-67 and negative for STAT6 and CD68. The Ki-67/MIB-1 labeling index was less than 2% (Fig. f, X200).
The patient underwent an uneventful postoperative course and no additional therapy was added for her.
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pmc-6325870-1
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A 51-year-old woman was admitted to our hospital complaining of chest tightness, palpitations and dyspnoea after activity. On admission, physical examination revealed a normal state of consciousness, an average heart rate of 76 beats per minute (bpm), and a blood pressure of 111/70 mmHg. Cardiac auscultation revealed variable first heart sound intensity and a diastolic murmur in the mitral stethoscope area. The other findings were unremarkable. The electrocardiogram showed atrial fibrillation. The echocardiogram performed by our hospital also showed rheumatic heart disease (severe mitral stenosis and regurgitation) with normal cardiac function and wall motion.
After the completion of the preoperative examination, the patient was sent to the operating room for mitral valve replacement. The operation was successful. After the aorta was reopened, ventricular fibrillation occurred. Sinus rhythm was not restored until 5 rounds of electrical defibrillation had been performed. Not long after returning to the intensive care unit (ICU), this patient developed heart failure with low blood pressure (70~85/45~50 mmHg) and tachycardia (125–135 bpm). Blood gas analysis showed progressive lactic acidosis, and blood lactate increased from 2.4 mmol/L to 15.3 mmol/L. Troponin T was slightly elevated compared to the preoperative level (1.960 ng/mL vs 0.019 ng/mL). N-terminal pro-brain natriuretic peptide levels increased markedly from 821.7 pg/mL to 21,025 pg/mL. Electrocardiogram (ECG) (Fig. ) showed that the V5–6 ST-segment depression was 0.1 mV. The bedside chest film showed a small amount of fluid in the left chest. Urgent bedside echocardiography demonstrated akinesis in the middle and apical segments of the left ventricle with depressed LV function (EF 36%),while basal segments’ movement were generally normal.
To determine the blood flow in the myocardium, myocardial contrast echocardiography (MCE) was performed immediately (Figs. a, b, and a). For the MCE examination, the ultrasound system was switched to the contrast mode, with a mechanical index of 0.1–0.5. MCE was performed using intravenous administration of 2.0 mL of SonoVue (Bracco, Milan, Italy). To achieve a balance among ultrasound intensity, penetration, and the duration of myocardial opacification, the contrast-specific imaging mode needed to be adjusted. Similar enhancement intensity was observed in the basal, middle and apical segments. Quantitative analysis also showed approximately equivalent maximum intensity values in these regions.
The diagnosis was considered TCM instead of myocardial infarction. The treatment mainly involved maintaining effective circulation and reducing the postcardiac load. After the administration of epinephrine and norepinephrine, the patient’s blood pressure was still low. Considered the patient’s condition as heart failure, an intra-aortic balloon pump was inserted. Although myocardial enzyme levels were elevated, the doctors did not perform treatment for coronary heart disease, considering the reason of tissue injury after heart surgery. The therapeutic regimen for this patient included ventilator-assisted ventilation, postoperative anticoagulation therapy, anti-infection treatment and other conservative treatments.
A week later, the patient underwent coronary angiography, and the results showed no significant narrowing of the coronary artery. This patient was extubated 13 days after surgery and was later weaned from epinephrine. Myocardial contrast echocardiography performed 2 weeks later (Figs. c, d, and b) showed that apical movement was significantly improved, with a slight decrease in interventricular septal motion. In addition, the perfusion of the myocardium was normal, with an EF (Simpson) of 52%.
The clinical evolution was favourable, and the patient was discharged 3 weeks later. Before discharge, echocardiography showed that the artificial mitral valve function and the segmental wall movement were normal, with an EF of 72%. One year later, the patient remained asymptomatic and showed normalization of ventricular wall motion in the apical segment.
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pmc-6326127-1
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The patient was a 47-year-old male police officer who sought care at a consulting office and had been the victim of a perforating firearm wound to the right infraclavicular region 7 months prior to presentation. At the time of wounding he had been treated conservatively.
The patient complained of exertional dyspnea and considerable edema and pain in the right arm. He had brought the results of a chest tomography conducted some weeks before which showed considerable dilatation of the right subclavian vein and the cervical veins of the right upper limb.
Physical examination revealed significant edema of the right upper limb, with pain on palpation and holosystolic murmur in the topography of the right pulmonary apex. Right radial, ulnar, and brachial pulses were all reduced in comparison with those of the contralateral limb.
Two weeks after this consultation, the patient presented at an emergency room with exacerbation of the dyspnea, symptomatic ventricular tachycardia, and frequent premature ventricular contractions and was admitted to the hospital.
Supplementary cardiac tests were then conducted. The echocardiogram showed dilatation of the left cardiac chambers and an ejection fraction of 63%. Myocardial scintigraphy showed signs of dilated cardiomyopathy.
After clinical and cardiac stabilization, the patient underwent arteriography of the right upper limb, which showed a large arteriovenous fistula between the right subclavian vessels and a pseudoaneurysm of the subclavian artery ( ).
The treatment chosen was endovascular repair under local anesthesia with sedation. The technique employed was via puncture of the right common femoral artery with a 7F introducer and puncture of the right brachial artery with a 5F introducer. The subclavian artery was catheterized via the brachial access and the guidewire was snared and a through-and-through system constructed via the femoral access, due to difficulty in advancing the guidewire via the subclavian artery. The injury was repaired using a 8x100 mm Fluency covered stent (Bard) ( ).
After the procedure, the patient was transferred to the ward. He exhibited good postoperative recovery, with significant improvement of the pain in the right upper limb and reestablishment of symmetry of pulses with the contralateral limb. He was discharged from the hospital 2 days after the operation, on double platelet antiaggregation with acetylsalicylic acid and clopidogrel.
He was reassessed 15 days later in the consulting room. There was regression of the right upper limb edema, maintenance of the radial, ulnar, brachial pulses, and improvement of the dyspnea.
A control angiotomography conducted 15 days after the follow-up visit (i.e., 30 days after the procedure) showed that the endoprosthesis was patent and there was no premature venous filling ( ).
The study was approved by the Research Ethics Committee at the Hospital Saúde da Mulher (HSM), Belém, PA, Brazil.
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pmc-6326128-1
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A 48-year-old female patient with chronic alcoholic pancreatitis was admitted via the emergency department with a history of hematemesis. Initial tests revealed significantly elevated pancreatic enzymes, compatible with acute exacerbation of chronic pancreatitis. She underwent elective upper digestive endoscopy (UDE), which reveled a gastric swelling suggestive of extrinsic compression. The upper digestive hemorrhage recurred, causing hemodynamic instability. Initial volume resuscitation measures were successful and an urgent UDE showed the swelling covered with mucosa indicative of infiltrate, an oval-shaped erosion with a hematin background located on the large curvature of the distal stomach, and a large clot occupying the entire gastric fundus, with no signs of active bleeding.
Magnetic resonance imaging (MRI) revealed a saccular aneurysmal dilatation of the SA measuring around 2.0 x 1.6 cm. It was surrounded by an oval-shaped mass with thick/hematic content, suggesting a pseudoaneurysm of around 6.4 x 4.3 cm, in contact with the posterolateral wall of the gastric body (
).
Having been diagnosed with PASA, the patient was treated with percutaneous embolization via the right common femoral artery. The SA was accessed using a coaxial technique with a guide catheter over a Simmons 1 (SIM 1) angiographic catheter over a 0.035” x 260 cm hydrophilic guidewire (
). The decision was taken to embolize using controlled release microcoils via microcatheter, one 8 x 30 mm unit distal of the neck and two 6 x 30 mm units proximal to the neck ( ). Control angiography showed total occlusion of the pseudoaneurysm ( ).
The patient remained hemodynamically stable and there were no complications related to the embolization technique, except for mild pain in the left hypochondrium on the first day after the operation. She remained hemodynamically stable for the next 2 weeks, with no further episodes of bleeding. However, because of her severe clinical status, she died from pulmonary sepsis.
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pmc-6326130-1
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The patient was a 66-year-old male with advanced malignant prostate cancer, bone metastases, and kidney failure requiring dialysis. While an inpatient at a cancer hospital, he was transported to the intensive care unit (ICU) for catheter placement and a hemodialysis session. The professional on duty chose a left subclavian vein access, using anatomic landmarks. The blood aspirate at puncture appeared to be venous and the guidewire was advanced without difficulties, but after dilation of the tract and insertion of the catheter, retrograde pulsating flow was observed. Inadvertent positioning in the left subclavian artery (LSA) was confirmed by blood gas analysis and Doppler ultrasound ( ). The examination ruled out the possibility of injuries to the carotid or vertebral vessels, which had normal morphology and blood flow. Physical examination found 4+ brachial and radial pulses. The device was left in place and the patient was transferred to a hospital with vascular and endovascular surgery services. Inherent problems within the Brazilian National Health Service (SUS - Sistema Único de Saúde) delayed the transfer by 18 days. Since there was a risk of fatal complications, the catheter was not removed from the LSA and the patient was not given anticoagulation because of a recent history of melena. After transfer, the catheter was removed, but endovascular repair was not possible because a thrombus was seen in the arterial lumen. There was no bleeding or formation of hematoma, and left upper limb perfusion was maintained, although the brachial pulse was rated 2+ and the distal pulses were absent at that time. The patient was transferred back to the cancer hospital. Doppler vascular echography was conducted again, showing a subacute thrombus in the LSA, where flow was monophasic (
), constituting subocclusion. The arterial thrombosis was in topography distal of the emergence of the vertebral artery, in which flow was laminar, anterograde and with velocities within the limits of normality ( ). At the subclavian-axillary transition, an arterial branch was observed with reversed flow that, based on topography, may have been the dorsal scapular artery ( ). The axillary ( ) and brachial arteries were patent and exhibited slow, low resistance flow, as did the radial and ulnar arteries. The conduct adopted in this case was watching and waiting since, in addition to the contraindication to anticoagulation already mentioned, the patient’s level of morbidity was elevated for an attempt at open revascularization and predictive indicators of the success of a possible bypass were unfavorable: the time elapsed since thrombus formation (22 days), the poor prognosis of the patient’s cancer, and the presence of kidney failure. The patient was observed for a further 2 weeks and did not show any sign of cyanosis, pain at rest, or trophic lesions. He was discharged from hospital for palliative home care.
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pmc-6326135-1
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A 39-year-old woman with hepatitis C was being seen by the gastroenterology service to monitor a liver nodule. Abdominal ultrasonography identified a visceral artery aneurysm as an incidental finding. Angiotomography revealed that it was a saccular aneurysm of the pancreaticoduodenal artery, with a diameter of 40 mm, and showed subocclusive stenosis of the celiac trunk compatible with extrinsic compression ( ).
The patient underwent laparoscopic relief of celiac trunk compression ( ), thereby averting the possibility of mesenteric ischemia, as the pancreaticoduodenal artery is an important collateral route between the celiac trunk and the superior mesenteric artery and an undiscovered occlusion of this artery can cause visceral ischemia. The laparoscopic procedure was performed using a 10 mm trocar for the camera, in an umbilical position, and a further four trocars; in the right and left hypochondrium, the left flank, and a subxiphoid position. The gastrohepatic ligament, phrenoesophageal membrane, esophagus, and crura of the diaphragmatic were dissected, with inferior sectioning of the crura to enable the arcuate ligament to be viewed. Relief of celiac trunk compression was achieved by sectioning the arcuate ligament by electrocautery and the crura were drawn back together to prevent gastroesophageal reflux. Doppler ultrasonography conducted before hospital discharge showed that there was no longer compression of the celiac trunk and revealed some residual stenosis and post-stenotic dilation (the pre-stenotic celiac trunk diameter was 10 mm and at the stenosis it was 3.5 mm) ( ).
The patient returned 2 months later for pancreaticoduodenal artery aneurysm repair, which was performed under local anesthesia and sedation, via a left brachial access with selective catheterization of the superior mesenteric artery and selective embolization of the aneurysm sac with microcoils, with no intercurrent conditions ( ). Four 20 mm to 25 mm x 50 cm Axium 3D microcoils and two Axium Helical microcoils 18 mm x 40 cm and 12 mm x 40 cm were used. Follow-up Doppler ultrasonography after 3 months showed thrombosis of the aneurysm and a patent pancreaticoduodenal artery, in addition to absence of extrinsic compression of the celiac trunk.
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