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pmc-6350156-1
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A 33-year-old male with known CLS presented to the hospital with a 2-day history of
cough, hypoxia, and shortness of breath. On admission, the patient’s vitals were
significant for pulse rate of 103 beats per minute and oxygen saturation of 88% on
room air and an arterial blood gas pH of 7.38 with PCO2 58 and
HCO3 34. Physical examination revealed characteristic findings of
CLS, including broad nose, large ears, hypertelorism, down-slanted palpebral
fissures, oligodontia, pectus excavatum, and severe kyphoscoliosis with decreased
breath sounds in the lower lung fields, worse on the right side. The lung
examination was limited, secondary to the significant skeletal abnormalities. With
concern for aspiration pneumonia, a chest X-ray was ordered, which suggested left
basilar airspace disease ( and ). This
study was followed by a computed tomography of the chest revealing the extent of
skeletal abnormality ( and ).
This patient suffered from undiagnosed chronic respiratory failure caused by
restrictive lung disease secondary to congenital kyphoscoliosis. During
hospitalization, there was an initial concern for aspiration pneumonia because of
leukocytosis and declining respiratory function with a new arterial blood gas pH of
7.23 with PCO2 84 and HCO3 35; however, induced sputum
cultures solely grew normal throat flora. Initially, the patient was started on
nebulized ipratropium bromide/albuterol but required intubation for declining
oxygenation and fatigue. As the patient’s condition improved, he was extubated the
following day and managed on BiPAP (bilevel positive airway pressure). Although the
patient continued to demonstrate improvement, he required oxygen via nasal cannula
after a failed trial on room air. Withholding oxygen for approximately 10 minutes
resulted in arterial carbon dioxide and oxygen pressures of 75 mm Hg and 47 mm Hg,
respectively, indicating a need for oxygen supplementation. The patient was
discharged on 2 liters of oxygen via nasal cannula and BiPAP PRN as recommended by
pulmonology. At the 6-month follow-up, patient is reported to be doing well.
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pmc-6350311-1
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In 2017, a 61-year-old Japanese man was referred to an oral and maxillofacial surgeon in Tokai University Hospital, Isehara, Japan, because of trismus and general fatigue. He complained of gradually worsening trismus and a painful ulcerated wound in the right buccal mucosa that had failed to heal for the past 6 months. He was on medication for hypertension and had no other specific systemic disease. On physical examination, facial swelling without redness was observed on the middle right side of his face, and trismus was noted (inter-incisor distance was 17 mm). Ulceration was observed in the right buccal mucosa, and an indurated mass could be palpated on the skin of his right cheek. Multiple palpable cervical lymphadenopathies were observed. He underwent workup for suspected malignancy of the buccal mucosa. There were no neurological and cardiologic abnormalities.
Computed tomography (CT) showed a mass in the right buccal mucosa that extended superiorly destructing the lateral wall of the maxillary sinus, inferiorly to the retromolar trigone, and laterally to the buccinator and anterior border of the masseter muscles, with multiple cervical lymph node enlargements (Fig. and ). Whole-body 18F-fludeoxyglucose (FDG) positron emission tomography (PET)/CT was performed. The PET scan showed increased uptake of FDG in multiple lymph nodes in the right cervical area, scapula and erector spinae muscles, and the right femur (Fig. ).
Laboratory tests on admission showed high white blood cell count (13,400 cells/μL) and elevated levels of SCC marker (4.5 ng/mL), but did not show any disorder in other tests including blood coagulation tests and tumor markers: cancer antigen (CA) 19-9, 31 U/ml; and carcinoembryonic antigen (CEA), 1.0 ng/ml.
An incisional biopsy of the right buccal mucosa was performed, which confirmed the diagnosis of SCC. He was given a diagnosis of right buccal carcinoma (T4bN2bM1). Induction chemotherapy was planned, and he was admitted at our hospital. Five days after hospitalization and prior to the initiation of chemotherapy, he experienced aphasia and lost consciousness. He had right hemiparesis with right upper and lower extremities manual muscle test (MMT) grade 0 [, ], and his National Institute of Health Stroke Scale (NIHSS) was 19 [, ].
The first set of laboratory tests right after onset revealed a platelet count of 31.1 × 104/μL, a prothrombin time-international normalized ratio (PT-INR) of 1.06, and high levels of fibrinogen degradation product (FDP) at 9.2 μg/ml and D-dimer at 5.4 μg/mL. No marked abnormality was observed on other blood chemistry tests, and the condition did not fulfill the diagnostic criteria for DIC. Brain CT, 30 minutes after the onset of symptoms, showed scattered hyperdense curvilinear areas suggestive of developing petechial hemorrhage in the region of his right middle cerebral artery (MCA) (Fig. ). Magnetic resonance imaging (MRI) was performed 100 minutes after the onset of symptoms. Diffusion-weighted image (DWI) showed a scattered lesion affecting the cortical part of the region supplied by his right MCA and perfusion imaging showed corresponding deficit (Fig. ). Head magnetic resonance angiography (MRA) showed attenuated flow-related signal in his right MCA region beyond the M1 segment, but its superior division was not visible (Fig. ). All imaging findings indicated right MCA infarction. A Doppler ultrasound scan of his neck revealed thrombosis of his left internal jugular vein (IJV), and compression of his right IJV by metastatic lymph nodes (Fig. and ). He was diagnosed as having TS by multifocal cerebral infarction.
Intravenous recombinant tissue plasminogen activator (t-PA) (alteplase 0.6 mg/kg) was administered directly after the MRI scan. Electrocardiogram (ECG), Holter monitoring, echocardiography, and blood culture tests did not show any abnormalities. A head CT on 1, 3, and 7 days after onset showed that the infarction in his right MCA area had not recovered. Seven days after the onset of brain infarction, systemic heparinization was started (PT-INR, 1.5 to 2.0). He did not recover from his cerebral infarction and died 16 days after admission, 21 days after diagnosis, due to pneumonia. A pathological autopsy was not performed as the family did not consent. Family consent was obtained for this case report.
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pmc-6350335-1
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A 74-year-old Taiwanese man had ESRD secondary to essential hypertension and started hemodialysis therapy since 2002 until now. On 16 June 2005, parathyroid investigations showed the following values: serum intact parathyroid hormone (i-PTH) concentration of 757 pg/ml (reference range 10–73), serum total calcium concentration of 11.2 mg/dl (reference range 8.4–10.2), and serum phosphate concentration of 6.5 mg/dl (reference range 2.7–4.5). As a result, the patient was diagnosed as having tertiary hyperparathyroidism. The ultrasound examination of parathyroid glands revealed the right inferior parathyroid gland 15.5 × 12.0 × 11.9 mm in size and the left inferior parathyroid glands 21.6 × 12.3 × 7.4 mm in size. The patient did not receive the examination of parathyroid scan with Tc-99 m MIBI.
On 5 December 2007, endocrine surgeon performed parathyroidectomy to remove all four parathyroid glands and transplanted right superior parathyroid gland into the subcutaneous fat over the internal part of the right thigh. The pathology of the right and left inferior parathyroid glands showed oxyphil cells and chief cell hyperplasia of both parathyroid tissues. Pre-operative laboratory tests revealed serum i-PTH of 2148 pg/ml, serum total calcium of 11 mg/dl, and serum phosphate of 13.6 mg/dl. Post-operative laboratory tests showed serum i-PTH of 71 pg/ml, serum total calcium of 5.9 mg/dl, and serum phosphate of 8.0 mg/dl.
In December 2017, the patient was found to have elevated i-PTH concentration again to 1135.9 pg/ml, hypercalcemia (total calcium 11.0 mg/dl) and hyperphosphatemia (phosphate 8.4 mg/dl). Therefore, we performed parathyroid scan with Tc-99 m MIBI and scanned with early and delayed imaging, which showed focal tracer uptake in retrosternal region (Fig. ). There was no evidence of recurrent parathyroid gland in the neck or right thigh. Besides, the patient did not have sterna related symptoms or physical findings. So, we suspected ectopic functioning parathyroid gland in the retrosternal region. Post contrast chest and mediastinal computed tomography (CT) scan showed a nodule around 1.3 cm in size in the retrosternal region (Fig. ), which can be consistent with an ectopic parathyroid gland. Both investigations revealed evidence of an ectopic parathyroid gland in the retrosternal region.
On 27 February 2018, a thoracic surgeon performed a neck incision with partial sternotomy and resection of a 1.5 cm mediastinal nodule at the upper mediastinal above the left innominate vein and thymus. The ectopic parathyroid gland is located extra-thymic because we didn’t find any thymic tissue in the histological examination of the resected specimen. Microscopic examination of the specimen showed a parathyroid gland composed of nodular hyperplasia of oxyphil cells and chief cells. Immunohistochemically, the parathyroid gland was positive for GATA-3, while negative for CD5 and synaptophysin (Fig. ). The pathologic findings were compatible with a diagnosis of an ectopic mediastinal parathyroid gland. We compared the laboratory tests between pre-operation and the second post-operative day: serum i-PTH level decreased from 1135.9 pg/ml to 272.7 pg/ml, serum phosphate level decreased from 7.9 mg/dl to 5.9 mg/dl, and serum total calcium level decreased from 11.0 mg/dl to ionized calcium 0.88 mmol/L (reference range 1.1–1.4). We continued by recording ionized calcium values as 0.81 mmol/L, 0.76 mmol/L, 0.73 mmol/L, 0.95 mmol/L, and 0.85 mmol/L for the past 5 post-operative days, respectively. We immediately administered intravenous calcium chloride 20 ml every 12 h, along with calcium acetate 667 mg four tablets three times a day and vitamin D 0.25 mcg daily from the second post-operative day.
According to the clinical history, the patient was diagnosed to have recurrent tertiary hyperparathyroidism before total parathyroidectomy and ectopic parathyroidectomy. In addition, hungry bone syndrome was present after ectopic parathyroidectomy.
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pmc-6350341-1
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A 76 year-old male presented with a 3 week history of lightheadedness, olfactory hallucinations, confusion, and intermittent agitation. An MRI was performed, which showed significant edema in the right anteromedial temporal lobe and insula concerning for herpes encephalitis. An electroencephalogram (EEG) revealed a few right frontal sharp waves and diffuse slowing concerning for possible seizure activity. Remarkable laboratory data included a sodium level of 125 mEq/L. CSF revealed a glucose of 62 mg/dL (normal 40–70 mg/dL), total protein of 71 mg/dL (normal 0–44 mg/dL), and 6,750 RBCs with 2 WBCs. CSF testing was negative for human cytomegalovirus (HCMV), herpes simplex virus (HSV), and varicella zoster virus (VZV) by polymerase chain reaction (PCR). CSF was also negative for Coccidioides antibodies and cytology for malignant cells.
The patient was started on intravenous (IV) acyclovir for presumed herpes simplex encephalitis and concomitant levetiracetam to mitigate seizure risk. His symptoms improved significantly, and he was discharged on a 21-day course of IV acyclovir. At follow up, roughly 15 days after admission, his prior symptoms of lightheadedness, olfactory hallucinations, confusion, and agitation had all resolved.
A repeat MRI was performed 3 months after symptom onset, showing a ring enhancing lesion concerning for glioblastoma. The patient underwent right temporal craniotomy for resection of the lesion. Pathology was consistent with glioblastoma.
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pmc-6350341-2
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A 77 year-old male presented with headache, profound confusion, aphasia, and MRI findings of a non-enhancing left frontal lesion which was hyperintense on T2-weighted and FLAIR images (Figures ). The MRI also revealed non-enhancing lesions in the temporal lobes and corpus callosum. The patient's vital signs on admission were: BP 159/69 mmHg, HR 105 bpm, RR 24, and a temperature of 37.3°C. The patient presented with left carotid bruit. He could not follow commands. His past medical history was significant for hypertension, diabetes mellitus diagnosed 10 years previously, coronary artery disease, and moderately differentiated prostatic adenocarcinoma status post-prostatectomy 10 years previously. Remarkable laboratory data included blood glucose 179 mg/dL and arterial blood gas pH 7.37, pCO2 49, pO2 72, SaO2 94% on 2 L/min O2 by nasal cannula.
A neurology consult suggested a possible diagnosis of GBM, but biopsy was deferred due to lack of a ring enhancing lesion. No CSF sample was taken, and IV acyclovir was initiated to treat possible herpes simplex encephalitis. On post-admission day 2, a left internal cerebral arteriogram was performed which demonstrated normal left common, external and internal carotid arteries and normal left anterior and middle cerebral arteries. Acyclovir was administered for 4 weeks. Steroids were not given at any point during the patient's hospitalization. The patient displayed remarkable clinical improvement over the next 2 weeks, with neurological function returning to baseline. MRIs performed on post-admission days 7 and 14 showed decreased edema but interval increase in the size of the focal enhancing lesion along the left frontal lobe gray matter concerning for glioblastoma (Figures ).
Approximately 3 weeks after his original hospitalization, the patient was readmitted due to neurological deterioration. A fourth MRI scan showed increased enhancement of the left frontal lesion (Figures ). A fifth MRI, performed ~4 months after his original hospitalization, demonstrated a bifrontal “butterfly glioma.” The tumor was subsequently resected, and pathology confirmed a diagnosis of glioblastoma.
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pmc-6350341-3
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A 78-year-old male presented with severe confusion, receptive aphasia, headache, and dizziness. MRI revealed hyperintensity in the posterior medial left thalamus, bilateral hippocampi, and the left precentral gyrus on T2 FLAIR imaging with no contrast enhancement. Computerized tomography (CT) imaging showed mild microvascular disease but no evidence of acute intracranial process or stenosis. The patient's vital signs upon admission were: BP 103/65 mmHg, HR 64 bpm, RR 18, and a temperature of 36.9°C. His medical history was significant for atrial fibrillation, for which he was prescribed Xarelto but was non-compliant. EEG did not indicate seizure. CSF cultures were negative for HSV and VZV and revealed normal differentiated cell count. Remarkable laboratory results included a CBC with elevated lymphocyte levels of 3.89E9 cells and low creatinine levels of 0.73 mg/dL.
The patient was immediately started on IV acyclovir. No steroids were administered at any point during his hospital stay. The patient displayed clinical improvement and returned to baseline neurological function over the following week.
An MRI performed 2 weeks post-admission revealed stable asymmetric non-enhancing T2 FLAIR hyperintensity involving the left thalamus and increased size of enhancing intra-axial lesion in the left precentral gyrus with surrounding T2 FLAIR hyperintensity, concerning for a neoplastic process. The patient was readmitted 3 months after his initial hospitalization for resection of a brain mass which was determined to be glioblastoma following biopsy.
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pmc-6350352-1
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A 49-year old Caucasian male with no significant medical history and no prior complaints of chest pain (smoking was his only known cardiovascular risk factor) was referred to our cardiac emergency department with acute chest pain during exertion. The ambulance ECG showed extreme ST-segment elevation anterolateral (‘tombstone elevations’), which had resolved completely at arrival in the hospital (Figs. and ).
In the hospital the patient immediately went to the catheterization laboratory for an emergency coronary angiography, which showed no significant lesions. At the bifurcation between LMCA and LAD wall irregularities were visible, possibly indicating either a small dissection or a passed thrombus (Fig. ). Dual antiplatelet therapy was continued afterwards. During the next two days the patient did not have any complaints and no arrhythmias occurred. Ultrasonography showed no regional wall motion abnormalities, LV ejection fraction of 50% and no significant valvular disease. Patient received dual antiplatelet therapy, statins and ACE-inhibition. A beta blocker was also started, but had to be stopped due to symptomatic bradycardia. The patient was discharged 3 days after presentation. The out-patient follow-up visit was scheduled within 2 weeks after discharge. Unfortunately after discharge the patient resumed his smoking habits and refused to take any medication.
Two days later the patient presented at the emergency department after reanimation because of collapse due to ventricular fibrillation. Time of delay from onset until arrival of the ambulance was approximately 8 min. The ambulance ECG once again showed marked ST-elevations, which had resolved completely at hospital arrival. At arrival patient also had complete recovery of spontaneous circulation. An emergency coronary angiography was performed, which showed no changes compared to several days earlier and no clear cause of the VF. At first a conservative approach was chosen and the patient was admitted to the ICU. Intracoronary imaging (IVUS) of the LMCA was postponed awaiting neurological recovery.
After arriving at the ICU, the patient developed ventricular arrhythmias with loss of cardiac output. Attempts to restore output were not successful, which eventually led to implantation of veno-arterial extra corporal life support (ECLS). Repeated ultrasonography after placement of the ECLS showed worsening of the systolic LV-function (EF 10%), with only some wall movement in the RCA-fueled area. This was attributed to stunning post-reanimation and ECLS-implantation. During the next several days the systolic LV-function gradually improved to a point where the ECLS could be removed. In an attempt to prevent further ventricular arrhythmias and possible spasm, PCI of the angiographically not significant lesion of the LMCA was performed. Unfortunately, no neurological recovery occurred and the patient died 1 month later. The permission to perform an autopsy was granted (Table ).
The fore mentioned case left us wondering: what caused this man without any significant angiographic coronary lesions to present with such dramatic clinical consequences? Did we miss a significant lesion, did he have coronary spasms or is there another explanation?
Macroscopically the coronary arteries were open and showed no significant sclerotic lesions. The stent placed in the LMCA seemed to be open as well. On the Lactate dehydrogenase macroreaction (LDH) the entire left ventricle wall showed discoloration, with exception of the posterior wall. This finding indicates scarring and atrophy of the septum, anterior and lateral wall, indicating a massive myocardial infarction after occlusion of the LMCA. As the stent was not occluded the infarction probably occurred beforehand and was most likely the cause of the ventricular fibrillation at presentation.
Microscopical examination of the heart showed extensive transmural infarction throughout the entire left ventricle, with avital cardiomyocytes, macrophages, siderophages, and fibroblast proliferation as a sign of starting fibrosis (Fig. ). These findings are in agreement with a myocardial infarction which occurred several weeks ago (due to ischemia either during reanimation or before). The right ventricle only showed some focal ischemic changes with zones of vital myocardium. Detailed examination of the LMCA in the stented region showed a stable atheromatous plaque with 30–40% occlusion of the coronary artery and indentations in the wall, attributable to the stent (Fig. ). The other coronary arteries, aorta and carotid arteries only showed minor atherosclerotic changes without significant occlusions.
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pmc-6350361-1
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Case 1 - A 50-year-old woman celebrated her birthday and consumed an unknown amount of alcohol. Her family was unable to wake her up the following morning (7 a.m.), therefore the patient was transferred to our department (Fig. ). On admission, somnolence, moderate dysarthria, horizontal gaze-directed nystagmus, moderate trunk ataxia, and in-coordination were found. Her laboratory values showed moderate alcohol intoxication (Table ). The symptoms were attributed to the effects of alcohol, therefore, after a negative CT and CT-angiography, forced diuresis was started (8:30 a.m.), and her clinical status was checked every hour. Initially, consciousness improved, the patient became alert, and dysarthria and ataxia ameliorated. However, early in the afternoon (2 p.m.), the control examination revealed worsening symptoms, she became somnolent again and developed severe horizontal nystagmus, double vision, dysarthria and dysphagia. Due to rapid progression, cerebral CT was repeated, which was negative again. Similarly, duplex ultrasound showed no stenosis of the carotid or vertebral arteries, however, transcranial Doppler (TCD) revealed high pulsatility index and low flow velocity in the basilar artery. Due to rapid progression and the sound suspicion of basilar artery occlusion, digital subtraction angiography (DSA) was performed. DSA showed basilar artery occlusion, therefore intraarterial thrombolysis was performed. After the administration of 25 mg rt-PA, the basilar artery was successfully recanalised (Fig. ) and the symptoms rapidly improved. The control CT 24 h after the treatment showed no abnormalities. At discharge, the patient was symptom-free.
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pmc-6350361-2
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Case 2 - A 62-year-old man consumed about 32 g ethanol (4 units) in the evening (Fig. ). Before going to bed, his wife noticed his slurred speech, and the patient complained of double vision and trunk ataxia that was disproportionate to the amount of alcohol he had consumed. His wife attributed the symptoms to alcohol consumption; however, the patient disagreed. Therefore, paramedics were called who found mild right-sided hemiparesis and severe dysphagia in addition to double vision, dysarthria, and trunk ataxia. On admission to our department, the clinical examination confirmed these findings (NIHSS: 6 points). Cerebral CT showed no cerebral hemorrhage or infarction, therefore thrombolysis was performed within 3 h of the onset of symptoms. The control examination showed significant improvement, and the NIHSS evaluated 24 h after thrombolysis decreased to 1 point.
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pmc-6350361-3
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Case 3 - A 55-year-old man consumed approximately 80 g etanol (10 units) during the night at a wedding ceremony and fell asleep at about 2 a.m. (Fig. ). His relatives tried to wake him up early in the morning (5 a.m.), the patient opened his eyes, but could not speak. He seemed to be drunk, therefore the relatives attributed the signs to alcohol consumption and let him sleep back. Upon awakening in the early afternoon (1 p.m.), his relatives realized that he had facial asymmetry, mild right-sided weakness and speech disturbance. On admission, right-sided homonymous hemianopsia, paresis of the lower half of right side of the face, mild right-sided hemiparesis, and severe receptive and expressive aphasia were found. Urgent CT scan (1:30 p.m.) revealed a huge infarction in the left middle cerebral artery (MCA) territory (Fig. ). Aspirin was administered and the risk factors were controlled. The neurological status did not change significantly.
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pmc-6350381-1
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A previously healthy 39-year old woman was diagnosed as having influenza A virus infection by rapid influenza diagnostic test (RIDT) in a clinic, and received oral Oseltamivir 75 mg twice daily for 5 days. The clinical course is shown in Fig. . While influenza like illness was improved once, fever and cough recurred on day 7 after the onset of flu. At this time, RIDT was performed, showing that the result was negative at the clinic. She complained of fever, cough and the left chest pain and presented to our institute on day 14 after the onset of the flu. RIDT was performed and the result was again negative. The data representing the inflammatory reactions were elevated (Table ) and the chest radiography showed encapsulated pleural effusion of the left lung (Figs and ). Pleural fluid from the initial thoracentesis was pus, and showed an increase in cell counts with neutrophil predominance. Thus, she was diagnosed as having acute empyema. Thoracic drainage with intrapleural urokinase and antibiotic therapy of ceftriaxone (CTRX) 2 g and metronidazole (MNZ) were started. Pleural fluid cultures from the initial thoracentesis grew Streptococcus pyogenes on day 4. Thus, MNZ was changed to clindamycin (CLDM) 600 mg three times a day. On day 10 after the antibiotic therapy with thoracic drainage was started, she received video-assisted thoracic debridement due to worsening of the patients’ general condition and infiltrations by chest radiography. After the operation, the patient’s condition improved and antibiotic de-escalation was performed to ampicillin 6 g daily iv. Due to patient’ good condition, antibiotic therapy was switched to oral amoxicillin 500 mg three times daily on day 28. Then, she was discharged. During this six months, recurrence of the infection was not observed.
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pmc-6350525-1
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A 51-year-old male with a history of nonischemic cardiomyopathy with a left ventricular assist device was admitted for expedited heart transplant evaluation. The evaluation included an elective colonoscopy in light of a family history of colorectal cancer in his mother who died at age 61 from the disease. The patient had his first screening colonoscopy at age 45 and was diagnosed with benign polyps, which were removed, and left-sided diverticulosis. The procedure was uncomplicated and he was advised to repeat a colonoscopy in five years.
The patient was without GI symptoms at the time of his colonoscopy. He denied tobacco, alcohol, or illicit drug use. His medications included amiodarone, aspirin, famotidine, levothyroxine, lisinopril, metoprolol, sildenafil, and intravenous heparin as well as torsemide, acetaminophen, docusate sodium, and melatonin as needed. On examination, he had a left ventricular assist device port entering at the upper abdomen, but otherwise the abdomen was soft and nontender to palpation with normal bowel sounds and no appreciable masses or ascites.
The patient underwent a standard bowel preparation which included a clear liquid diet the day prior to the procedure and 20mg of Dulcolax with 4 liters of polyethylene glycol the night prior to the procedure. Monitored anesthesia care sedation was administered with propofol. The colonoscopy was performed at night without difficulty with good bowel preparation. Abdominal pressure was briefly required to maneuver around the splenic flexure. The colonoscope was advanced to the cecum with identification of the appendiceal orifice and ileocecal valve. Findings included multiple sigmoid and descending colon diverticula and two, small (<5mm) sessile polyps that were removed using cold forceps.
The night of the procedure the patient had no pain or nausea and ate dinner and breakfast the following morning without incident. He then developed epigastric abdominal pain in the midmorning approximately 12-14 hours after the procedure and had one episode of nonbloody, nonbilious emesis following lunch. On physical examination, he was afebrile with a blood pressure of 104/89mmHg, heart rate of 68 beats per minute, and oxygen saturation of 100% on room air. Abdominal examination was notable for mild distension and moderate tenderness to palpation in the epigastric region without guarding or rebound tenderness and decreased bowel sounds. Laboratory examination revealed an elevated lipase of 2275 U/L and amylase of 1141 U/L. Additional abnormal laboratory findings included an elevated aspartate aminotransferase of 105 U/L, alanine aminotransferase of 94 U/L, and total bilirubin of 1.4 μmol/L (normal prior to the procedure). An abdominal X-ray did not reveal an obstructive bowel gas pattern or evidence of free air. A computed tomography (CT) scan of the abdomen/pelvis revealed diffuse edematous changes of the pancreas with surrounding inflammatory stranding in the bilateral paracolic gutters, extending superiorly to the perihepatic region and inferiorly to the pelvis (). The constellation of symptoms, labs, and imaging were suggestive of an episode of acute pancreatitis.
The patient was treated conservatively with bowel rest, intravenous fluids, and analgesics as needed. Over the next three days his symptoms and abdominal examination improved and his diet was advanced to a regular diet. The lipase normalized to 15 U/L.
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pmc-6350541-1
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A 60-year-old man was admitted to our department due to progressive deterioration of renal function approximately 3.5 months after initiation of immunotherapy with nivolumab. In April 2016, there was a diagnosis of stage IIIa non-small cell lung cancer located in the upper lobe of right lung was made (). Lung cancer was initially treated with combination of radiotherapy and 6 cycles of chemotherapy, including paclitaxel and carboplatin. In March 2017, a positron-emission-tomography/computed-tomography (PET/CT) scan showed malignant extension to tracheobronchial and subcarinal lymph nodes. Immunotherapy with nivolumab was initiated at a dosing regimen of 3 mg/kg every 2 weeks. Immunotherapy started with a normal renal function (serum creatinine: 79.56 μmol/l, estimated-glomerular-filtration-rate (eGFR): 92.5 ml/min/1.73m2). After the 7th infusion of nivolumab (approximately 105 days after initial exposure), laboratory examinations revealed for first time impaired renal function (serum creatinine: 176.8 μmol/L, eGFR: 35.2 ml/min/1.73m2). Treating oncologists decided the administration of 2 additional cycles of nivolumab with progressive doubling of serum creatinine and eGFR decline to 14.8 ml/min/1.73m2 before referral to the renal department ().
On admission, the patient's medical history revealed that he was a former heavy smoker over the past 35 years (20 cigarettes per day) and had no other comorbidities. He did not receive any medications with the exception of sporadic use of simple analgesics. He denied the use of nonsteroidal anti-inflammatory drugs, proton pump inhibitors, or other nephrotoxic agents, and he reported no drug or food allergies. His family history was unremarkable. The physical examination revealed a normal body temperature (36.7°C), blood pressure 135/70 mmHg, pulse rate 80 bpm, oxygen saturation 98% in the room air, and absence of abnormal clinical signs from the chest auscultation and palpation of the abdomen. Pedal edema, skin rash, joint pain, and swelling were not present. Blood tests revealed mild anemia (hemoglobin: 12.0 g/dl), severely impaired renal function (serum creatinine: 433.1 μmol/L, eGFR: 11.9 ml/min/1.73m2), and hyperkalemia (serum potassium: 5.8 mmol/L) with no other electrolyte or acid-base disturbances. Urinalysis showed sterile pyuria and absence of both proteinuria and microscopic hematuria. A 24-hour urine collection confirmed the absence of proteinuria. Renal ultrasonography excluded the presence of hydronephrosis and showed kidneys with normal size, contour, and cortical echotexture.
With respect to the diagnostic work-up of AKI, screening for hepatitis B and C viruses and HIV was negative. Immunological tests including antinuclear and anti-DNA antibodies, rheumatoid factor, anti-neutrophil cytoplasmic autoantibodies (ANCA), complement and serum immunoglobin levels were negative or within the normal range. Electrophoresis and immunofixation did not identify the presence of a monoclonal immunoglobin component in the serum. The absence of both proteinuria and microscopic hematuria and the negative immunological examination raised the clinical suspicion of AIN and a renal biopsy was performed to ascertain the cause of AKI. Light microscopy showed severe interstitial nephritis with infiltration of polymorphic inflammatory cells (). The interstitial inflammatory infiltrate was predominantly composed of T cells, monocytes, and eosinophils. Inflammatory infiltrates were also present in the tubular basement, and tubular epithelial cells exhibited diffusive degenerative lesions; interstitial granulomas were not present (). Glomeruli were normal, except for 2 out of 12, which were fully sclerotic. Immunofluorescence was negative for glomerular or tubular immune deposits.
After the biopsy-proven diagnosis of AIN, the patient received 500 mg/day intravenous methylprednisolone for 3 days, followed by oral prednisolone 1.0 mg/kg/day for the subsequent 2 weeks. Prednisolone dose was progressively tapered and the total duration of corticosteroid therapy was 8 weeks. Immunotherapy with nivolumab was permanently withdrawn. The patient was followed up closely in the Outpatient Nephrology Clinic (). At month 2 after discharge, renal function was significantly improved (serum creatinine: 194.5 μmol/L, eGFR: 31.4 ml/min/1.73m2) and then stabilized at 6-month follow-up visit to a slightly higher eGFR level, suggesting a partial, but clinically meaningful, recovery of kidney injury.
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pmc-6350569-1
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A 17-year-old female presented to the otolaryngologist complaining of rapidly enlarging neck swelling noticed three months earlier after a wisdom tooth extraction. She was given antibiotics for a presumed dental or thyroglossal duct cyst infection without improvement. She was subsequently referred for an otolaryngology consult for further evaluation. The patient denied neck pain, dysphagia, difficulty breathing, or weight loss. She has no history of significant radiation exposure or family history of thyroid malignancy. Past medical history was significant for chronic otitis media status post bilateral myringotomy tubes 9 years ago but had otherwise been healthy. Physical exam revealed a well-circumscribed “golf ball” shaped mass in the midline of the neck between the thyroid cartilage and hyoid bone that elevated with swallowing. Tympanic membranes showed mild scarring, but the remainder of the exam was unremarkable. Suspecting a thyroglossal duct cyst, the otolaryngologist planned for a Sistrunk procedure preceded by a CT neck to evaluate the extent of the mass and visualize the thyroid gland. The CT neck showed a 1.6 × 2.0 × 2.9 cm, enhancing, elliptically shaped mass located within the anterior soft tissues of the neck inferior to the hyoid bone, anterior to the hypopharynx and glottis. The mass enhanced similarly to the thyroid tissue, read by the radiologist as suspicious for ectopic thyroid (). There were no enlarged lymph nodes, and the thyroid gland appeared normal.
The patient underwent excision of the neck mass without complication. During the surgery, the mass was noted to sit atop the thyroid cartilage; no obvious tracts were seen. Grossly, the specimen was described as a 3 × 2 × 1.8 cm, irregular, pink-tan friable nodule with focally hemorrhagic surfaces. A frozen section showed papillary architecture adjacent to normal thyroid architecture with both tissue types surrounded by an epithelial capsule that was devoid of cilia (). No cystic components were seen. Cytology revealed intranuclear inclusions, nuclear grooves, and ground-glass nuclei. The stated abnormalities were determined by two collaborating pathologists to be highly suggestive of PTC arising from a focus of ectopic thyroid tissue. Margins were determined to be uninvolved. The lymph node was <1 mm and was negative for the disease.
Thyroid function tests, complete metabolic panel, and complete blood count were within normal limits, except for a thyroglobulin level of 48 ng/ml. Because of the risk for concurrent native thyroid malignancy, the patient underwent an uneventful total thyroidectomy. The specimen revealed a native thyroid with a normal architecture. A surrounding lymph node was resected and also shown to be benign.
Postoperatively, the patient developed symptomatic hypocalcemia that was successfully managed with calcium and vitamin D. She was discharged home in stable condition on thyroxine, calcium, and vitamin D. One month postoperatively, she was asymptomatic with normalized calcium levels and thyroid tests. She will return to the endocrinologist in 3 months for a thyroid uptake scan and potential radioactive iodine if residual disease is found.
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pmc-6350570-1
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A 35-year-old Caucasian female with extensive history of pelvic surgery but without prior urological history underwent robotic-assisted laparoscopic excision of endometriosis by gynecological surgery team secondary to chronic pelvic pain with suspected endometriosis. On initial laparoscopic evaluation of pelvic contents, visible vermiculation of bilateral ureters was noted as well as suspected findings of endometriosis-like lesions covering the pelvic peritoneum. The pelvic peritoneum was excised with sparing of the urinary bladder. Careful ureterolysis was performed bilaterally, during which the distal left ureter was found to be partially denuded, spanning 2 cm in length (). An intraoperative urologic consultation was requested, and denuded ureteral injury was confirmed by urology on laparoscopic evaluation. Given no evidence of ureteral laceration or obvious extravasation of urine from left ureter, no cystoscopy or contrast studies were performed. A 2 cm x 12 cm AmnioFix membrane was wrapped three times around the left ureter using laparoscopic robotic assistance (Figures and ). The procedure was completed without anesthesia complications and the patient was discharged on postoperative day one in stable condition.
The patient was seen by her gynecologist on postoperative day six after experiencing lower urinary tract symptoms and was subsequently started on PO antibiotic therapy. However, her symptoms did not improve, and she developed new left flank pain which brought her back to the hospital for further evaluation on postoperative day seven. She underwent noncontrast CT imaging of the abdomen and pelvis demonstrating moderate left hydroureteronephrosis to the level of the distal ureter. She underwent cystoscopy with left retrograde pyelogram demonstrating 1.5 cm distal ureteral stricture with moderate hydroureteronephrosis (). Continued contrast injection showed a small amount of extravasation from the vicinity of the narrowed ureteral segment (). However, the site of extravasation could not be delineated. A guidewire was passed through the left ureter and into left renal pelvis without resistance and a left ureteral stent was placed. Her pain improved, and she was discharged home.
Patient was readmitted one month later secondary to nausea, vomiting, and lower urinary tract symptoms at which time she was found to have enterococcus urinary tract infection. Cross section imaging of the abdomen and pelvis was unremarkable without fluid collections. Left ureteral stent was noted to be in appropriate position. She was discharged home with antibiotic therapy with outpatient follow-up in two weeks at which time her ureteral stent was removed.
The patient did not report renal colic or abdominal pain following ureteral stent removal. A Lasix renal scan was performed three months following ureteral injury which demonstrated normal perfusion and excretion by 20 minutes without signs of left ureteral obstruction (Figures and ). Differential renal function was 45% left kidney and 55% right kidney. Repeat CT urogram performed 4 months after injury demonstrated no obstructive uropathy or contrast extravasation. Patient was recommended repeat Lasix renal scan in 1 year. The patient reported no symptoms during the interim.
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pmc-6350572-1
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A 56-year-old man with a history of dyslipidemia, multiple transient ischemic attacks (TIAs), and a 40 pack-year smoking history presented to the local hospital with sudden left-sided weakness, slurred speech, and left facial droop. His family history was significant for stroke and diabetes in multiple family members. He was on atorvastatin for dyslipidemia. He had no history of connective tissue or autoimmune disease. He was diagnosed with acute ischemic cerebrovascular accident and was given tissue plasminogen activator (tPA). The patient received a single dose of atorvastatin 80 mg and aspirin 325 mg orally during the admission; he never received clopidogrel or ticlopidine therapy. The symptoms improved gradually, but he developed thrombocytopenia that worsened over the next few days. The team discontinued statin and aspirin therapy once they observed low platelets. The patient was managed conservatively, but his platelet counts reached a nadir at 16,000 per cubic mm. His peripheral blood smear showed no schistocytes in high-power fields. He was given a platelet transfusion with no improvement. He was then transferred to our hospital, and the inpatient hematology team was consulted. At the time of presentation, the patient complained of clumsiness in his left arm, although he was able to carry out daily activities with minimal difficulty. He was stable, the facial droop had resolved and speech slightly improved with residual dysarthria and expressive aphasia, and his motor power was better. A review of outside laboratory results revealed that 2 days before his transfer, his platelet count was 115,000 per cubic mm, BUN was 16, and his creatinine was 1.3. His initial lab values on arrival at our hospital were as follows: platelets 26,000 cells per cubic mm, hemoglobin (Hb) 10.7 g/dl, hematocrit (Hct) 31.2/L, leucocytes 16,300 cells per cubic mm, blood urea nitrogen 35, blood urea nitrogen/creatinine 27, lactic acid dehydrogenase of 794 (98–192), bilirubin 0.9, and reticulocyte count 2.8. During admission, B12 and folic acid levels, antiphospholipid panel, disseminated intravascular coagulation panel, coagulation panel, antinuclear antibodies, and rheumatoid factor were measured and found to be within normal limits. HIV, hepatitis B, and direct Coombs tests were negative. However, his peripheral blood smear showed 5-6 schistocytes/high-power field. Neither the patient nor his family members underwent any screening for connective tissue or autoimmune disorders.
The patient was immediately started on a daily total plasma exchange (TPE). On day 3, his neurological symptoms improved significantly, and his platelet count normalized to 167,000 per cubic mm. Despite the improved platelet count, TPE was continued for 2 more days. By day 5, his speech was clear and his expressive aphasia resolved. He recovered full strength in his right extremities and TPE was stopped. Blood samples were sent to the Blood Center of Wisconsin, Milwaukee, for measurement of ADAMTS13 levels, and we got the results 5 days later. ADAMTS13 activity measured using FRETS-VWF73 substrate was <5% (reference range ≥67%). ADAMTS13 inhibitor was measured using mixing studies with standard pooled plasma, and residual ADAMTS13 activity measured using FRETS-VWF73 substrate was <0.4 inhibitor units (reference range ≤0.4). Platelet count remained stable throughout the patient's stay. At the time of discharge, his counts and Hb/Hct remained stable, lactate dehydrogenase (LDH) was 249, and no schistocytes were present in peripheral blood smears. Because of his atypical presentation of TTP, he was advised to seek medical attention immediately if his neurological symptoms worsen to evaluate TTP relapse. After discharge, he was evaluated twice weekly with a CBC and LDH measurements after discharge for 2 weeks and once a week afterward. Higher levels of LDH were noticed subsequently normalized in 2 months. He complained of intermittent left facial numbness and tingling of the left hand during follow-up visits. The patient remained in remission without a relapse for 2 years after his initial presentation.
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pmc-6350573-1
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A 49-year-old male nonsmoker, with no past medical history, was working with nitric acid in an enclosed area. Upon noticing a reddish-brown sweet smelling gas emanating from the bottom of a 55-gallon drum, he turned on exhaust fans but continued to work. He did not put on any kind of protective mask or respirator on. He felt the sensation of eye and throat irritation and shortness of breath. During the course of the six-hour exposure, he, on multiple occasions, retreated to the outside area and felt an amelioration of symptoms. Approximately 12 hours later he experienced paroxysms of cough and shortness of breath and was driven to the emergency department by his wife.
He presented to the emergency department in moderate to severe respiratory distress. Physical examination revealed an oral temperature of 98 degrees Fahrenheit, respiratory rate of 34 breaths per minute, blood pressure of 118/61 mm/Hg, and pulse of 87 beats per minute, and room air oxygen saturation was 80 percent. There were no murmurs rubs or gallops. Diminished breath sounds were appreciated on lung examination. There were frequent paroxysms of cough which were exacerbated by deep inhalation; there was no use of extra inspiratory muscles and no cyanosis appreciated. The remainder of the exam was normal. He was placed on supplemental oxygen at 2 liters per minute with an increase in his oxygen saturation to 85 percent. The supplemental oxygen was increased to 4 liters per minute with an increase in his oxygen saturation to 92 percent and he was given bronchodilator treatments.
On 2 liters of supplemental oxygen by nasal cannula, his arterial blood gas showed a pH of 7.37, pCO2 44.4 mmHg, pO2 44.1 mmHg, and bicarbonate 25.3 mmol/L, and base deficit was 0.2 mmol/L. Carboxyhemoglobin and methemoglobin levels were unappreciable. Normal blood gas values are pH of 7.36 – 7.44, pO2 of 80–100 mmHg, pCO2 of 36–44 mmHg, and HCO3− of 22–26 mmol/L. All other laboratory values were within normal limits with the exception of a white blood cell count of 12.9 k/mm3 (normal range: 4.5 to 11.0 k/mm3). Chest radiography showed bilateral pulmonary infiltrates and pulmonary edema (). Electrocardiogram showed normal sinus rhythm with a ventricular rate of 87 beats per minute with a normal axis and normal intervals.
He was admitted to the intensive care unit, supplemental oxygen was continued, and bronchodilator treatments using albuterol and ipratropium (2.5 mg and 0.5 mg, respectively) were given every six hours. Pulmonary care for this patient was at the discretion of the pulmonologist on the case. Over a seven-day hospital course he had progressive improvement in his symptoms and his chest X-ray (). One month after discharge the patient presented to pulmonary re-evaluation and followup. At the time of outpatient followup the patient denied any complaints at that time. During the visit standard pulmonary function testing was performed which included forced vital capacity, forced expiratory volume in the first second, peak expiratory flow, and maximum mid-expiratory flow. These pulmonary function tests were found to be within their normal parameters. After this visit, he was lost to follow up.
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pmc-6350580-1
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A 46-year-old woman presented to the emergency department with a 2-day history of right-sided lower abdominal pain. The pain started suddenly around the central abdomen and then moved towards the right side. It was worse with movement and was associated with nausea and anorexia. There was no vomiting, diarrhea, or rectal bleeding. She had normal bowel movements. No history of urinary or gynecological symptoms elicited. She had no previous similar presentations. Her past medical history was significant for sarcoidosis and recurrent respiratory tract infections. Generally, she looked unwell. She was afebrile. Vital signs on presentation were a pulse rate of 76 beats per minute, a blood pressure of 110/70 mmHg, and a respiratory rate of 14 breaths per minute. Systemic examination was essentially normal. Examination of the abdomen revealed marked tenderness in the RIF with rebound tenderness and a localized guarding. The rest of the abdomen was soft and nontender with normal bowel sounds. Blood tests revealed a WCC of 7.1 and a CRP of 16.6. Renal and liver function tests were within the normal ranges. Urinalysis was normal. The pregnancy test was negative. Based on the given history and relevant physical and laboratory findings, a presumptive clinical diagnosis of acute appendicitis was suggested. The patient was admitted for observation. A computed tomography (CT) scan of the abdomen and pelvis was performed the next morning, which revealed an epiploic appendagitis of the caecum with a mild surrounding pericaecal fat stranding, no collection or free air noted (). The appendix looked entirely normal (Figures and ). She was managed conservatively with analgesia and antibiotics for 2 days and made a complete recovery and was sent home. In a follow-up visit after a week, she was generally well and reported no recurrence of her symptoms. She was finally discharged from the surgical care.
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pmc-6350583-1
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A 40-year-old African-American male with neurosarcoidosis involving the hypothalamus and pituitary () presented to an urban academic medical center with altered mental status. On arrival, the patient was lethargic but responsive to verbal stimuli and the physical examination was otherwise unremarkable. The patient was noted to be hypothermic (32°C) and hypernatremic (176 mEq/L). Admission ECG revealed sinus bradycardia at 41 beats per minute, first-degree AV block (PR interval 280 ms), premature atrial contractions, prolonged QRS (160 ms) and QT (QTc 584 ms) intervals, and Osborn waves most prominent in the precordial lateral leads ().
The patient was admitted to the intensive care unit where careful intravenous fluid management and administration of intranasal desmopressin were initiated. After 24 hours, with the improvement of his serum sodium, the patient's mental status improved. The patient was warmed via external warming blankets, with resolution of the above electrocardiographic findings (). The remainder of the patient's treatment included corticosteroids, testosterone, levothyroxine, and desmopressin, and he was discharged home ten days after presentation.
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pmc-6350591-1
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A 32-year-old lady with normal cognitive function has presented with few symptoms and signs relating to pituitary gland disorder at different timeline but refused to seek early medical care. Firstly, she had primary amenorrhea which she initially thought may be a constitutional delay, but over time, she eventually came to term and decided not to get married or conceive to self-mitigate this problem. She then developed blurring of vision at the age of 19 years, but she just coped with it as she claimed the onset was insidious and she was still able to perform her routines. She had no significant headache and other signs to suggest increased intracranial pressure. At the age of 23, she started working as a factory operator but often experienced lethargy even on mild exertion, forcing her to take multiple sick leaves. She also noticed occasional spontaneous milky discharge from both nipples that stained her inner wear, but she dismissed this sign. As these problems progressed, she quitted her job and stayed at home with her parents. Three years later, she started to have dripping of clear fluid through her nose upon bending down and during strenuous activity. She eventually came forward for medical assistance as the latter symptoms really affected her daily activities.
On physical examination, she was normotensive. There were no signs of Cushing syndrome. Funduscopic examination revealed left optic atrophy secondary to compressive optic neuropathy, with left temporal hemianopia and almost right temporal hemianopia seen on visual acuity assessment. Hormonal assay investigations disclosed serum prolactin of 4200 mIU/L with dilutional assay of 250,688 mIU/L. There was reduced level of estradiol (62 pmol/L), follicular stimulating hormone (0.9 IU/L), and luteinizing hormone (0.1 IU/L). Thyroid function test showed normal thyroid stimulating hormone (1.78 mIU/L), low T4 (8.4 pmol/L), and normal T3 (4.3 pmol/L). Synacthen test revealed good cortisol response with baseline 0 hour of 394.4, 616.2 at half an hour and 785 at one hour. Her ‘chronic rhinorrhea' was proven to be CSF leak confirmed by clinical test (positive halo sign) and biochemical test (nasal cavity CSF glucose level of 3.9 mmol/L related to plasma glucose of 5.6 mmol/L).
As the patient refused any surgical intervention, she was discharged home with bromocriptine 2.5 mg once daily with subsequent uptitration to twice daily and thyroxine. She was extensively counselled regarding the risk of worsening CSF leakage after DA treatment and the risk of meningitis. Nevertheless she was not started on any prophylaxis antibiotic. The patient was followed up for about a year and, within this period, serial serum prolactin and magnetic resonance imaging (MRI) of pituitary gland were performed to monitor the disease progression (refer to ). On baseline MRI, a large lobulated enhancing solid mass was seen in the sellar and suprasellar regions extending into the subfrontal lobes and in between the two frontal horns causing compression of foramen of Monroe (refer ). Inferiorly, there was extension of the mass to the sphenoid sinus and into the nasal cavity. The mass appeared to encase the cavernous sinus and the optic chiasma. On follow-up, she did not tolerate bromocriptine well and hence changed to cabergoline 0.5 mg per week (titrated accordingly). There was clinical improvement seen with cessation of CSF leak, marked reduction of serum prolactin (serum prolactin dilutional assay of 14,734 mIU/L at 12 months of treatment), and radiological evidence of tumor shrinkage on MRI (refer ). Throughout the follow-up, there were no symptoms and signs to indicate meningitis. Although she claimed that her symptoms improved with treatment; however, her visual status remained the same and her menstruation did not commence.
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pmc-6350603-1
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A 20-year-old right-hand-dominant and otherwise healthy female student presented with protrusion of the left upper back and left periscapular pain that occurred after sport activities. Ten months previously, the patient had been seated in the left rear passenger seat in a car that was hit in the left side by another car. Further details such as the posture and the arm position of the patient at the time of the accident were uncertain. At the time of the car accident, the patient visited an orthopedic clinic where a surgeon diagnosed left shoulder contusion without any abnormal radiographic findings. The left arm was kept in a sling for 2 months, as left arm elevation caused severe pain in the upper back. After sling removal, the patient returned to basketball, which generated continuous dull pain around the left scapula. She presented at our clinic because her mother had noticed the deformity of her back.
The patient had no relevant family or medical history. There was no neurological deficit in the left shoulder and arm. The left scapula was slightly higher than the contralateral scapula and exhibited atypical medial winging with the arm at the side. The distance between the spinal process and medial scapular border was shorter on the left side than the right side at the inferior angle level, but these distances were almost the same at the scapular spine level (). Contraction of the scapular stabilizing muscles was good. There was a palpable bony protuberance without tenderness on the ventral side of the ISA. The limitations of the active ranges of motion of the left shoulder compared with the right shoulder were 25° for total elevation, 15° for external rotation, and none for internal rotation and horizontal adduction; however, there were no limitations of the passive ranges of motion. The winged scapula became prominent at 0–45° of active flexion, while it disappeared when the patient flexed the left arm while consciously attempting to depress the scapula (). The winged scapula did not emerge when the patient pushed on a wall at chest level. Radiographs showed a small bony fragment in the ventral side of the ISA, with a narrow space between the fragment and the scapular body (). Computed tomography revealed a bony protrusion extending from the medial scapular border to the bony fragment, with a narrow gap between the protrusion and the fragment (Figures –).
The patient was instructed to avoid elevating the left arm for 2 months and then performed reinforcement exercises of the SA such as the scapular push-up and the bear hug using an elastic band for 2 months. At examination 4 months later, the periscapular pain and the winging of the scapula with the arm at the side and in active flexion had resolved. The push-on-the-wall test at waist level was negative, and the range of motion of the left arm was the same as the unaffected side, except for a 15° limitation in external rotation. Although the radiographic findings were the same as at the first visit, computed tomography demonstrated bony union (Figures and ). The patient was permitted to use the left arm without restrictions.
At the time of the final follow-up 10 years of postinjury, the patient reported that there was an occasional painless click and a sporadic floating feeling of the scapula with initial active flexion of the arm. However, there was no pain or any disturbance to the patient's activities of daily life and work as a physical therapist. The patient's colleague confirmed the disappearance of the winged scapula associated with shoulder movement. The DASH score was 0, and the Constant score ratio compared with the right shoulder was 100% [, ].
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pmc-6350920-1
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A 73-year-old woman was diagnosed with IgG lambda MM in November 2007. She received lenalidomide and dexamethasone as front-line treatment, then relapsed and received multiple lines of chemotherapy (Data Supplement). Her CD138+ cells were then collected and sequenced. Our pipeline revealed activation of the HDAC pathway through RNA analysis and, concordantly, identified the HDAC inhibitor vorinostat through drug repurposing. Moreover, gene expression analysis revealed a high expression of BCL2 compared with that of the other patients analyzed (Data Supplement). On the basis of these findings, she was administered venetoclax 400 mg PO once daily, the HDAC inhibitor panobinostat 20 mg Monday, Wednesday, and Friday, 2 weeks on, 1 week off, and, in addition, pomalidomide 2 mg Monday to Friday, 3 weeks on, 1 week off. Notably, the patient had been treated previously with pomalidomide. Before therapy, IgG was elevated to 2,910 mg/dL and free lambda, 141. IgG has decreased to as low as 785 mg/dL and free lambda light chains to 19.16 mg/dL (Data Supplement). The patient remains receiving treatment.
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pmc-6350920-2
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A 72-year-old man was diagnosed with IgA kappa plus kappa MM, Durie-Salmon stage IIB in April 2011. After relapsing after receiving multiple treatments including pomalidomide 2 mg (immediate preceding regimen), his CD138+ cells and PB samples were sent for sequencing (Data Supplement). The pipeline identified an NRAS G12S mutation, and the patient was administered the MEK inhibitor trametinib. Before treatment, his IgA and free kappa light chains measured 661 mg/dL and 576 mg/L, respectively (free kappa/lambda ratio, 19·32). Three months after treatment began, his IgA had reached a nadir of 94 mg/dL, whereas his free kappa light chains had decreased to 109 mg/L. The patient relapsed 5 months later, with free kappa light chains rising to 390 mg/L (IgA, 187 mg/dL; Data Supplement).
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pmc-6350920-3
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A 55-year-old man was diagnosed with IgG kappa MM in April 2008. The patient was initially administered lenalidomide and dexamethasone, which resulted in relapse and, after multiple failed regimens, his CD138+ cells and PB were sent for sequencing (Data Supplement). WES analysis identified a KRAS Q22K mutation. Concordantly, RNA analysis showed activation of the MAPK pathway. Gene expression analysis revealed a high expression of BCL2 compared with that of the other patients analyzed (Data Supplement). He was administered the BH3-mimetic venetoclax 400 mg Monday to Friday and trametinib 2 mg Monday, Wednesday, Friday. It has been shown that the combination of BH3-mimetic and MEK inhibition upregulates the proapoptotic Bcl-2 family member Bim and can have a synergistic anticancer activity. The patient’s free kappa/lambda ratio decreased from 13.2 to 0.251, and he responded well to therapy. However, he eventually developed grade 3 fatigue, and treatment was held. After relapse, the patient was challenged with venetoclax 400 mg Monday to Friday, trametinib 2 mg Monday, Wednesday, Friday, and carfilzomib 20/27 mg/m2. This showed tumor response, with an M spike decrease from 6.08 g/dL to 4.86 g/dL and an IgG decrease from 7,321 mg/dL to 4,818 mg/dL. Notably, the patient was previously refractory to carfilzomib. The patient has been continuing this regimen for 3 months (Data Supplement).
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pmc-6351004-1
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A 29-year-old male with no significant past history presented with a Glasgow Coma Score (GCS) of 10 after falling out of a three-storeyed building onto his head. Imaging showed a 19-mm-thick left epidural hematoma with a 5-mm midline shift, as well as a comminuted left temporal bone fracture (Figure ).
He was taken emergently to the operating room. Given the significant mechanism, he was presumed to be at a high risk of cerebral edema and therefore underwent a decompressive left hemicraniectomy, duraplasty, and placement of a left frontal EVD. Intra-operatively, a small subdural hematoma (SDH) was found originating from the cortical veins near the sylvian fissure. Hemostasis was achieved using bipolar cautery and Surgicel® (Johnson & Johnson, New Brunswick, NJ, USA). No aneurysms or unusual bleeding were noted. Immediate postoperative computed tomography showed a reduced mass effect with no atypical residual bleeding. He was discharged on postoperative day (POD) 17 to an in-patient rehabilitation unit with a GOS 3. Eventually, he was able to return home functionally independent and without neurologic deficits.
Three months postoperatively, he presented after being found on the ground unresponsive at home. He had a GCS 9, and there were no external signs of trauma. Imaging showed an unusual pattern of subarachnoid hemorrhage and SDH in the left frontoparietal region without mass effect (Figure ).
Given the uncertain history and atypical imaging, the patient underwent a four-vessel digital subtraction angiogram (DSA). Imaging showed distal left middle cerebral artery aneurysm (Figure ).
This discovery substantially changed management. He promptly underwent a left craniotomy for aneurysm clipping. Stealth-guided imaging was used in designing the craniotomy. Intra-operatively, the dome appeared grossly composed of adventitia. The neck was dissected and a single clip was placed. Postprocedure angiogram demonstrated no residual filling. His recovery was uneventful, and he was discharged home on POD nine at his neurologic baseline with a GOS 5.
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pmc-6351005-1
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A 62-year-old Hispanic male was admitted to the intensive care unit (ICU) with signs of septic shock. After an aggressive fluid resuscitation and administration of intravenous antibiotics including vancomycin and Zosyn, a computed tomography (CT) scan of the abdomen and pelvis was obtained revealing coloenteritis (Figure ).
His course was complicated by DIC requiring transfusion of blood products, along with ventilatory support for hypoxic respiratory failure and beta-blocker medications for controlling the atrial fibrillation rate. Blood cultures grew E.coli, and antibiotic coverage was changed to meropenem and ceftriaxone. The patient developed bilateral flank dark-red discoloration and bullous lesions with copious weeping. Skin lesions progressed rapidly to full-thickness necrosis in spite of local wound care with topical silver sulfadiazine (Figure ).
The differential diagnoses (Coumadin-induced necrosis, thrombotic thrombocytopenic purpura, meningococcemia, toxic shock syndrome, calciphylaxis, necrotizing fasciitis and meningococcemia) were all ruled out. Necrotic skin and subcutaneous tissues required multiple surgical excisions, debridement and the use of a wound vacuum (Figure ).
After a prolonged hospital stay with multidisciplinary care, local wound care and skin grafting, the patient did well and was discharged to an acute rehabilitation center (Figure ).
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pmc-6351007-1
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A 27-year-old male with a history of traumatic brain injury and quadriplegia, with chronic respiratory failure on home ventilator support, presented to the emergency department with increased work of breathing and no bowel movements for three days. The patient was bed-bound, nonverbal, and received nutrition via percutaneos endoscopic gastostromy (PEG) tube. The patient was found to have long-standing anemia with an average hemoglobin (Hb) of 9 g/dL and leukopenia for 2 years. He was hypotensive with a mean arterial pressure (MAP) of 54 mm/Hg. The rest of his physical exam was unremarkable, and there was no evidence of acute or ongoing blood loss. Chest X-ray revealed a right pleural effusion. A central venous line was placed, and the patient was started on vancomycin and cefepime for presumed sepsis. Initial lab data revealed hyperkalemia (K+ 6.1), severe anemia (Hb 1.5 g/dL), leukopenia (2.53 K/uL), neutropenia (ANC 700), and normal platelets. He was also found to be have acute kidney injury with creatinine (Cr) of 1.5 (mg/dL), and anion-gap metabolic acidosis with a lactate of 7.0 (mmol/L). The patient required norepinephrine support for septic shock. Peripheral smear revealed leukopenia with absolute neutropenia, marked anemia with anisopoikilocytosis, with rare dacrocytes but no evidence of schistocytes. He responded appropriately to blood transfusion with improvement in hemoglobin from 1.5 to 9.1 within 24 hours. He did not require further transfusion during hospitalization.
Investigation of the profound anemia
Evaluation for hemolysis failed to reveal an etiology. His vitamin levels (cobalamine and folate) were within the normal range. He had no personal or family history of hemoglobinopathy, and hemoglobin electrophoreses was normal. Ferritin and triglyceride levels were ordered to rule out hemophagocytic lymphohistiocytosis (HLH). Ferritin was elevated at 6506 ng/mL and triglycerides were 123 mg/dL. Soluble IL-2 receptor level was sent and found to be significantly elevated; however, this was felt to be more likely secondary to infection and inflammation, as the patient had no other clinical features of HLH, with the exception of cytopenias. Further questioning revealed significant consumption of zinc supplements as part of his wound care regimen. This necessitated an evaluation of micronutrients including copper. The copper levels were found to be <10 ug/dL (normal: 70-140). The patient’s hypotension improved with management of sepsis. His renal function normalized. Zinc supplements were stopped, and the patient was started on copper supplementation. At his three month follow-up clinic appointment, his anemia and leukopenia had resolved.
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pmc-6351061-1
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An 85-year-old Caucasian man, with a medical history of deep venous thrombosis, cerebrovascular disease (currently without anticoagulation) and a 4 year history of low-grade NHL (atypical SMZL), presented with progressive pancytopenia, significant weight loss and symptomatic splenomegaly (abdominal discomfort, sense of fullness and limitation of mobility due to spleen size). He was human immunodeficiency virus negative, hepatitis B (HB) antigen negative, anti-HBs antibody positive and hepatitis C virus negative. The patient refused splenectomy and, in December 2017, was referred to palliative splenic radiotherapy.
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pmc-6351107-1
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A 10-year-old female came to us with painful swelling in the medial portion of the right lower leg. Her past medical history included asthma, while hematologic and biochemical findings were normal. Radiography of the lower leg showed cortical thickening eroded by a broad-based soft tissue mass without the involvement of the underlying cortex (Figure ). In CT findings, a small juxtacortical mass with thick calcification was seen, along with a periosteal reaction on the surface of the right tibia (Figure ). Magnetic resonance imaging (MRI) revealed a mass with a hypointense inner segment and an isointense outer segment in the axial and sagittal T1-weighted images and a hypointense inner segment and a hyperintense outer segment in the axial T2-weighted images, as well as sagittal short T1 inversion recovery (STIR) (Figure ). Technetium 99m (99mTc) hydroxymethylene diphosphonate (HMDP) bone SPECT/CT findings showed a focal and intense uptake by the mass (Figure ). Based on the radiological results, the differential diagnosis included a primary surface bone tumor, such as periosteal osteosarcoma, conventional chondroblastic osteosarcoma, and chondrosarcoma, as well as a soft tissue tumor with secondary marrow invasion. An incisional biopsy specimen was obtained from the mass, which demonstrated a malignant tumor with chondrosarcomatous features. The pathological diagnosis was periosteal osteosarcoma. Two courses of NAC with methotrexate, adriamycin, and cisplatin were administered.
Following NAC, radiography, MRI, and 99mTc HMDP bone SPECT/CT examinations were performed. Radiograph images showed a broad-based soft tissue mass with intense calcification (Figure ) and MRI revealed growth of the inner section corresponding to the calcification (Figure ), while visual examination of the 99mTc HMDP bone SPECT/CT images showed nearly the same level of focal uptake as compared to before the NAC (Figure ). The sizes of the mass before and after NAC were 12 × 29 × 62 mm and 17 × 29 × 62 mm, indicating a mild growth. Next, two SPECT/CT scans were performed using an integrated SPECT/CT system (Discovery™ NM/CT 670) (GE Healthcare, Chicago, IL) equipped with a low-energy, high-resolution collimator three hours after an intravenous injection of 440 MBq (megabecquerel) of 99mTc HMDP. The data obtained were analyzed using a commercially available software package (GI-BONE) (Aze Co., Ltd., Tokyo, Japan), which presents values for various SUVs, including max, peak, and mean SUV, metabolic bone volume (MBV), and total bone uptake (TBU). SUVmax represents the single greatest point of metabolic activity within the tumor. SUVpeak is defined as average activity concentration within a 1 cm3 spherical volume of interest (VOI) centered on the “hottest focus” within the tumor. The average value of the SUV, which showed 40% or more of the SUVmax in the VOI, is defined as the SUVmean. MBV is defined as tumor volume with uptake. Total lesion glycolysis (TLG) was calculated as SUVmean × MBV. The SUVmax, SUVpeak, SUVmean, MBV, and TBU values of the mass before NAC were 13.45, 12.03, 9.32, 10.36, and 96.57, respectively, while those after NAC were decreased slightly to 10.68, 9.38, 8.15, 6.89, and 56.14, respectively, for reductions of -20.7%, -22.0%, -12.6%, -33.5%, and -41.9%, respectively (Figure ).
The patient underwent surgery and intraoperative extracorporeal radiation therapy. After making a wide excision, soft and tumor tissue on the tibial surface were removed, then irradiation was performed with a 50 Gy dose, followed by re-implantation in the original site and fixing with a plate. The excision biopsy at the surgery showed a pathological grade 1 (non-complete response) after NAC, including a more than 20% of cell necrosis section. The quantitative bone SPECT/CT was considered to reflect the treatment response in this case.
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pmc-6351109-1
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A 42-year-old Caucasian female with a known history of NF1 presented to the emergency department with an episode of palpitations, flushing, pounding headache, numbness and tingling in both arms, and shortness of breath for the past two hours. The patient reported a similar episode two weeks prior, and a complete basic cardiac workup performed with an outpatient cardiologist was unremarkable. A loop recorder was implanted to detect the possible arrhythmias. The patient complained of these episodes lasting one to two hours every two to three months for the past two years. She denied any anxiety, stress, or any situational factors.
Her past medical history was significant for an episode of apparent ST-elevation myocardial infarction (STEMI) three years prior. During this hospitalization, her blood pressure was well controlled and her QTc interval was prolonged at 483 ms. Cardiac catheterization was performed emergently showing normal coronary anatomy without significant obstruction. However, the left ventriculogram revealed systolic apical ballooning with reasonable contractility at the cardiac base (Figure ).
Her left ventricular ejection fraction (EF) was calculated at 25%, and she was diagnosed with TS. The patient was started on lisinopril and carvedilol per guideline-directed medical therapy (GDMT). Subsequently, she had complete recovery of cardiac function within three months as confirmed with the periodic follow-up echocardiography revealing EF improvement to 55%. Her blood pressure continued to be well controlled during this period.
However, five months after the episode of TS, she had an asymptomatic episode of nonsustained polymorphic ventricular tachycardia (torsades de pointes) recorded on a loop recorder. She had prolongation of QTc interval in the baseline electrocardiogram (EKG). Her electrolytes were stable during this episode, and echocardiogram revealed an EF of 50% to 55%. As her EF had normalized, an implantable cardioverter-defibrillator (ICD) was not placed. She underwent electrophysiological studies twice that failed to reveal any etiology of arrhythmia.
At the current presentation, physical examination was remarkable for a pulse rate of 101 beats per minute and blood pressure of 190/110 mmHg. She also had numerous cutaneous neurofibromas. Routine laboratory data showed a slightly elevated white blood cell count of 12.5 x 103/ul with normal hemoglobin and platelet counts. Her complete metabolic panel, electrolytes including magnesium, phosphorous, and thyroid-stimulating hormone levels were all within the normal limits. EKG showed sinus tachycardia with a QTc interval of 566 ms (Figure ).
Loop recorder interrogation did not reveal any arrhythmia. A recent echo one month prior showed left ventricular ejection fraction of 60% to 65% with no significant structural abnormalities. With clinical suspicion of CST, further testing revealed elevated serum metanephrine at 7.90 nmol/L (normal: 0 to 0.49 nmol/L) and normetanephrine at 5.14 nmol/L (normal: 0-0.89 nmol/L). Diagnosis of CST was confirmed with 24-hour urine metanephrine levels of 5346 ug/day (normal <350 ug) and normetanephrine levels of 1817 ug/day (normal <650 ug). Subsequently, a computed tomography (CT) scan of the abdomen (Figure ) was done that revealed a mass in the right adrenal gland, and the findings were confirmed with a magnetic resonance imaging of the abdomen, which revealed a 4.4 x 3.3-cm well-circumscribed heterogeneous fat-free mass in the right adrenal gland suspicious for a pheochromocytoma (Figure ).
Testing for adrenal cortical hormones was normal. The patient was started on phenoxybenzamine for blood pressure control, and her home medication of labetalol was switched to sustained-release metoprolol. Her blood pressure was well controlled preoperatively. She underwent laparoscopic adrenalectomy, and her blood pressure remained controlled intra- and postoperatively. No additional medications were required for blood pressure control postoperatively. Histopathology revealed pheochromocytoma with positive synaptophysin staining extending 8 cm in the maximum dimension (Figure ). There was no local invasion of the surrounding structures by tumor, and no distant metastases were found.
The patient recovered well from the surgery and on six months follow-up, her symptoms resolved completely with normalization of the QT interval (Figure ).
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pmc-6351112-1
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A 61-year-old Caucasian female presented to a community hospital with a history of hyponatremia, arthritis, migraines, and bipolar disorder. She was admitted after coming to the emergency department (ED) on an emergency petition after her husband called the police. Her husband stated he called due to her manic symptoms and forgetting to turn the stove off. She was previously diagnosed with bipolar disorder in September 2017 for which she took divalproex 250 mg daily and quetiapine 50 mg at bedtime. At the time of the interview, the patient displayed hyperverbal pressured speech with rambling. Her thought process was goal oriented with bouts of loosening associations. She denied suicidal thoughts and use of alcohol, and she reported difficulty sleeping for which she used medical marijuana regularly. The patient appeared hypomanic, and her cognition and sensorium appeared clear.
At the time of admission, her sodium level was 129 mEq/L, with decreased hemoglobin and hematocrit. She was given olanzapine 5 mg orally at bedtime and was discharged two days later after her sodium levels and manic symptoms normalized from fluid restriction and oral sodium chloride.
Two days after discharge, the patient presented to the hospital with manic symptoms and was found to be hyponatremic. At admission, her sodium levels were 128 mEq/L. After treating her mania and restricting her fluids, her sodium level rose to 134 mEq/L. The patient had clear thoughts, speech, and cognition the following day. She stated that she was not drinking as many fluids, but, per the nurses, she was constantly requesting fluids.
On the third day of her admission, her sodium levels fell to 128 mg/L with a urine osmolarity of 268 mOsm/kg (reference range: 275 to 300 mOsm/kg), and a suspected increase in overnight fluid consumption was noted (Table ). She had a normal mental state, cognition, and sensorium, but was in distress about her constant fluctuating levels and wanted to leave the hospital. It appeared that she was having racing thoughts at this time and continued to discuss plans of divorcing her husband of 38 years and staying in a motel. SIADH and PPD were both considered at this time. She was further switched from divalproex and olanzapine to perphenazine and added sodium chloride tablets. She was discharged the following day.
A week following her third discharge, the patient was readmitted to the hospital with similar symptoms and hyponatremia. After nephrology consultation, she was diagnosed with reset osmostat (i.e., ADH shutting down due to low sodium levels). Her continuous cycles had shown that she was unable to control her fluid intake while at home and that she was at risk for continual admittance to the ED for hyponatremia with recurrent mania.
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pmc-6351113-1
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A 63-year old male presented with a history of lumbar laminectomy and fusion seven months prior to his initial presentation. He recalls that two days following his prone lumbar operation he began experiencing severe bilateral pain along the respective anterolateral thigh. Lumbar magnetic resonance imaging (MRI) ruled out spinal nerve root-related pathology as causative and a definitive diagnosis of bilateral MP, secondary to LFCN compression during the prone spinal surgery, was rendered. Conservative measures with oral analgesics were initially recommended given the typically self-limited course of this pathology. Despite an increasing regimen, including NSAIDs, narcotics, and anti-neuropathic pain medications, the pain persisted over the course of several months. Additionally, traditional anatomically-guided local anesthetic injections were attempted without improvement in his symptoms. Thus, the patient elected to proceed with operative decompression after eight months of failed conservative therapy and worsening quality of life.
The history of failed local anesthesia to even temporarily alleviate symptoms suggested a possible non-classic nerve location and prompted preoperative ultrasound to outline the superficial course of the LFCN. The ultrasound technique utilized has been previously described in the setting of percutaneous injection guidance (Figure ) [].
Skin markings in the inguinal region and upper thigh indicating the course of the LFCNs were made by the ultrasound technician prior to operative site preparation for surgery. Indeed, anatomically variant LFCNs were identified bilaterally on the preoperative ultrasound with neither nerve passing in a classic location medial to the anterior superior iliac spine (ASIS) (Figure ). On the right, the ultrasound identified the LFCN passing directly over the ASIS, while on the left, the nerve was localized lateral to the ASIS. Oblique incisions were made centered over the marked LFCN locations identified by ultrasound. Both LFCN anatomical variants observed on preoperative ultrasound were confirmed after intraoperative dissection. Fascia overlying the nerves was opened to effect decompression. The fascial opening was confirmed adequate by ensuring the absence of entrapment along its course out of the pelvis into the thigh (Figure ). The patient experienced immediate resolution of his preoperative pain and was discharged home on the same day without complication.
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pmc-6351114-1
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A 16-year-old male presented to the orthopedic outpatient department of our hospital with a gradually increasing, globular, non-tender, non-pulsatile, firm swelling that measured approximately 8 × 5 ×3 cm3 and was situated in the left popliteal fossa since five months (Figure ).
The swelling was attached to underlying structures, but was free from skin, and there was no distal neurovascular deficit. A plain X-ray of the knee was unremarkable. Magnetic resonance imaging (MRI) of the lesion revealed an ill-defined, lobulated, soft-tissue, space-occupying lesion in the lateral aspect of the calf adjacent to the lateral head of the gastrocnemius that was hypointense on the T1-weighted image and hyperintense on the T2-weighted image, with multiple ill-defined T2-weighted signal voids within the lesion that gave an impression of hemangioma (Figures -).
Tru-cut biopsy showed spindle cells arranged in clusters and scattered singly in a hemorrhagic background with no evidence of malignancy, suggesting a benign spindle cell tumor. Histopathological examination with Hematoxylin–Eosin staining revealed plenty of fibroblasts spread against a background of collagen as well as infiltration of the adjoining healthy tissue that led to a microscopic diagnosis of desmoid fibromatosis (Figure ).
Blood investigations as part of the routine preoperative workup showed normal results. Clinically, desmoid tumor mimics other soft-tissue tumors such as leiomyoma, rhabdomyoma, nerve sheath tumor, as well as vascular and perivascular tumors. Common histological differential diagnoses include fibrosarcoma, spindle cell tumor, epithelioid tumor, and pleomorphic tumor.
Under general anesthesia, the patient was positioned prone and a J-shaped lazy posterior incision was made. The tumor was situated deep to the fascia although it involved it. On palpation, it was firm, partly smooth, and seemed to arise from the deep fascia and muscle without any clearly defined planes; it was located adjacent to the lateral head of the gastrocnemius. The mass did not engulf any major vascular structures in the popliteal fossa although the common peroneal nerve and the medial cutaneous sural nerve traversed it. The sural nerve was sacrificed and the common peroneal dissected out carefully; the mass was then removed, and the specimen in toto was sent for histopathological study (Figure ).
The wound was closed in layers. There were no immediate postoperative complications. The dorsiflexors of the ankle and toes were well preserved. The patient was discharged on tenth postoperative day and received adjuvant radiotherapy after three weeks to minimize any chance of recurrence. At the one-year follow-up, the patient had no radiological or clinical evidence of recurrence, and has been advised regular follow-ups every six months.
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pmc-6351115-1
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A 63-year-old woman with a past medical history of hyperlipidemia, diabetes mellitus, and a remote ischemic stroke presented with intractable headaches of five-day duration. Headaches were described as constant, unrelenting, and throbbing in nature. The pain was described as predominantly occipital, which radiated throughout the rest of her head. The month prior to presentation, the patient underwent a rhinoscopy with nasal polypectomy at an outside facility due to a six-month history of progressively worsening unilateral left nasal passage obstruction. The patient was unaware if histopathologic analysis was performed on the removed specimen.
Upon presentation to the emergency department, the patient underwent a CT evaluation of the head without contrast, which demonstrated a midline aggressive-appearing tumor versus infectious process centered in the bilateral nasal cavities, paranasal sinuses, and right orbit with an intracranial extension to the bilateral frontal lobes. Edema was noted with a mass effect and a midline shift of 4 mm to the left (Figure ).
Subsequent evaluation with MRI of the head demonstrated a large, hypercellular mass involving the anterior aspect of the right frontal lobe with erosion through the cribriform plate and lamina papyracea, with additional extension into the superior nasal cavity and superomedial right orbit. An extensive vasogenic edema within the right frontal lobe was also visualized, along with a significant mass effect with a 1.1 cm of right-to-left midline shift (Figures -). Pathology later confirmed the radiographic suspicion of esthesioneuroblastoma. The patient began an inpatient course of steroids, and further management was completed by an outpatient neurosurgeon.
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pmc-6351117-1
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A 68-year-old man visited our hospital due to exertional dyspnea. His past medical history revealed diabetes mellitus, hypertension, and benign prostate hyperplasia. He had worked as a building housebreaker and at a processing company for plastic and had been exposed to asbestos. Cytological examination of the pleural effusion and pleural biopsy during talc pleurodesis yielded a diagnosis of epithelial malignant pleural mesothelioma. Immunohistochemical analyses demonstrated that these cells were positive for AE1/AE3, calretinin, D2-40, WT-1, mesothelin, HEG1, CD146, EMA, MTAP, p16, and p53, and negative for CEA, TTF-1, desmin, and BAP1 and Ki67 index was 5%. FDG-PET/CT before talc pleurodesis showed left pleural effusion and no FDG uptake of the left pleura (Figures , ). He did not undergo 11C-choline PET/CT scan before pleurodesis and NAC. He underwent NAC of three courses of cisplatin and pemetrexed. FDG-PET/CT after talc pleurodesis and NAC showed intense FDG uptakes in the high attenuation areas of left pleural thickening (Figures , ), whereas 11C-choline PET/CT showed mild choline uptake of left pleural talc deposit (Figures , ). Although it is difficult to evaluate treatment response of NAC due to a false-positive result by FDG-PET/CT, choline-PET/CT did not interfere with the post-chemotherapy disease evaluation. Pleurectomy/decortication was performed. The disease was categorized as T3N1M0 and mild treatment response was observed (grade 1a). He received adjuvant chemotherapy consisting carboplatin and pemetrexed and remains well 10 months after the definite diagnosis.
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pmc-6351117-2
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A 77-year-old female visited our hospital for examination of right pleural effusion. She had undergone the operation of bilateral breast cancers and sigmoid cancer in the past. She had not been exposed to asbestos. Cytological examination of the pleural effusion and pleural biopsy during talc pleurodesis yielded a diagnosis of epithelial malignant pleural mesothelioma. Immunohistochemical analyses demonstrated that these cells were positive for AE1/AE3, calretinin, D2-40, WT-1, mesothelin, HEG1, CD146, MTAP, and p53, and negative for CEA, TTF-1, and claudin-4 and Ki67 index was 8%. She did not undergo FDG-PET/CT and 11C-choline PET/CT scans before pleurodesis and NAC. She underwent NAC of three courses of cisplatin and pemetrexed. FDG-PET/CT after talc pleurodesis and NAC showed intense FDG uptakes in the high attenuation areas of right pleural thickening (Figures , ), whereas 11C-choline PET/CT showed mild choline uptake of right pleural talc deposit (Figures , ). Although it is difficult to evaluate treatment response of NAC due to false-positive result by FDG-PET/CT, choline PET/CT did not interfere with the post-chemotherapy disease evaluation. Pleurectomy/decortication was performed. The disease was categorized as T3N1M0 and mild treatment response was observed (grade 1a). She received adjuvant chemotherapy consisting carboplatin and pemetrexed and remains well seven months after the definite diagnosis.
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pmc-6351118-1
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A 75-year-old Native American female presented to dermatology with a ‘port wine’ purple nodular rash on her nasolabial folds, of 12 months duration (Figure ). There was no associated pruritus, burning, pain, or bleeding from the area. She did report drainage of some clear fluid when pressure was applied to the area. On physical exam, there were areas of raised, papular, nodular purple growth along the bilateral nasolabial folds. There was no evidence of drainage or infection. There were no oral lesions or skin lesions elsewhere on her body upon complete dermatological exam. Her medical history was remarkable for cirrhosis, deemed cryptogenic or secondary to non-alcoholic steatohepatitis (NASH) following evaluation by gastroenterology. Her medical history also included iron deficiency anemia secondary to GAVE, type II diabetes mellitus, hypertension, asthma, and endometrioid carcinoma of the ovary.
A 3-mm punch biopsy of the right nasolabial fold lesion demonstrated an atypical vascular lesion extending to the tissue margins. Sections revealed prominent vascular dilatation with papillary fragments and associated endothelial proliferation with cytologic atypia (Figure ). A cluster of differentiation (CD)31 stain highlighted lesional cells, representing angiosarcoma.
She also underwent a surveillance gastrointestinal endoscopy due to her history of cirrhosis, and a duodenal ulcer was incidentally discovered. A biopsy was performed that revealed duodenal mucosa with ulceration and granulation tissue along with atypical, neoplastic proliferation of cells growing in sheets (Figure ). Immunostains for erythroblast transformation-specific (ETS)-related gene (ERG) and friend leukemia integration 1 transcription factor (FLI1) were positive, confirming endothelial differentiation and thus consistent with angiosarcoma involving the duodenum.
Staging evaluation was performed. Computed tomography (CT) imaging demonstrated right face superficial angiosarcoma without the invasion of deep tissues (Figure ), bilateral lower lung nodules indeterminate for malignancy, and a 1.1 cm hypo-attenuated lesion of the right liver lobe indeterminate for malignancy. Positron emission tomography (PET) CT demonstrated hyper-metabolic activity in the face, consistent with the known lesion, and additionally hypermetabolic activity in the right scapular spine, distal sternum, pulmonary nodules of the left lung, right lobe of the liver, and T12 vertebral body (Figure ). These findings suggested osseous and soft tissue metastases. A single liver lesion was biopsied and demonstrated changes consistent with chronic hepatitis and cirrhosis with no evidence of malignancy.
These findings confirmed the angiosarcoma of the face with multiple synchronous sites, including the duodenum. The patient was presented at the multidisciplinary tumor board where it was recommended that she receive palliative radiation for local control of the lesion on the face/nasolabial folds. She was also started on systemic therapy with intravenous paclitaxel as per the Phase II Trial of Weekly Paclitaxel for Unresectable Angiosarcoma (ANGIOTAX) study [].
She experienced some treatment delay in between chemotherapy cycles due to radiation-induced myelosuppression. Side effects were monitored closely, given her history of cirrhosis and baseline bicytopenia. She also experienced grade 2 cutaneous toxicity from radiation therapy including painful erythematous lesions in the mouth and over the lips as well as blepharitis. Post-radiation skin changes on the face eventually healed well, following completion of radiation. She did not experience any paresthesia with paclitaxel.
Imaging following two months of systemic chemotherapy revealed interval progression of hepatic and vertebral metastases (Figure ).
Due to disease progression, paclitaxel was discontinued and treatment was changed to second-line bevacizumab based on the available data from a phase II trial []. Following cycle 1 of bevacizumab, she experienced decompensated cirrhosis along with spontaneous bacterial peritonitis and ascites. Treatment was deferred due to multiple hospitalizations in the next few weeks due to decompensated cirrhosis. The patient and her family then elected to transition to hospice due to worsening quality of life. She died six months following her diagnosis of angiosarcoma with complications from decompensated liver cirrhosis.
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pmc-6351290-1
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An 18-year-old male worker was admitted to the emergency department due to pain, paresthesia, and coldness of the right lower limb. He has no history of cardiac disease or trauma and otherwise in good health. On physical examination, the right lower limb was cold and with cyanotic toes. The right femoral and popliteal pulses were undetectable. An urgent color-flow duplex scanning had been arranged which revealed a complete obstruction of the right external iliac artery blood flow by a rather big embolus. A trans-thoracic echocardiography showed an anechoic lesion of 36–40 mm originating from the left ventricle (LV). Since it was an endemic area of hydatid disease, a provisional diagnosis of (CHC) had been postulated. An urgent surgical embolectomy through the common femoral artery had been done. The obstruction has been opened with a Fogarty catheter. The catheter harvested a cruor thrombus with a white membrane from the common femoral artery, the histopathology of which was a hydatid tissue as it is shown in a and b. After immediate subsidence of symptoms and signs, and an uneventful post-operative night, a CT scan of thorax revealed a well-defined cystic lesion of 45 mm diameter was protruding into the LV, as shown in a, b, and c. A week later, the patient had been submitted to a standard a sternotomy and under cardiopulmonary bypass between the ascending aorta and the two-vena cava. The LV cavity showed an inside protruding mass, . The mass was incised and the cyst was removed, as shown in a and b). This has been followed by mopping of endocyst, as in , putting stay suture of LV as in , and enlarging of a cystic cavity as in . The perforated myocardium was sutured by prolene, and the cavity closure was achieved by a standard mattress suturing with gel foam, . The postoperative period was uneventful, and the patient was discharged after 9 days with no complications. The follow-up plan consisted of a standard albendazole treatment for 3 cycles each one for 28 days with a period of one-week rest to avoid drug complication. An abdominal ultrasound, and a single brain CT scan to rule out another organ involvement, all of which were negative.
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pmc-6351351-1
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A 43-year-old man with a history of chronic alcoholism presented with abdominal distension. The previous day, the patient had presented to a local hospital with anal bleeding and abdominal pain after an incidental insertion of barbecue skewer per anus in the drunken state; subsequently, he had undergone sigmoid loop colostomy for rectal perforation. However, after the operation, the patient had become hemodynamically unstable. At presentation, his systolic blood pressure was 90 mmHg and the pulse rate was 135 beats/min. Although there was no gross rectal bleeding, the digital rectal examination revealed a penny-sized anterior rectal wall defect 6 cm from the anal verge (AV). Computed tomography (CT) revealed a hematoma (12 × 10 × 15 cm) with active bleeding in the pelvic cavity and a pseudoaneurysm in the anterior wall of the rectum (). Since the patient was hemodynamically unstable, an emergency operation was performed. During the operation, a massive subperitoneal hematoma in the rectovesical pouch and large amount of blood in the peritoneal cavity were found. After evacuation of the hematoma and blood, oozing continued in the rectovesical pouch (). Thus, compression with gauze was performed for 30 min until the oozing stopped. The Hartmann procedure was performed with the suspected bleeding focus included, but the perforation site was not included.
Although the postoperative course was uneventful and there was no evidence of recurrent bleeding on the follow-up CT on the 7th postoperative day (POD), a focal enhancing lesion in the anterior wall of the rectum indicating a residual pseudoaneurysm was noted (). On the 11th day POD, his hemoglobin decreased from 11.6 g/dL to 7.9 g/dL, and the follow-up CT revealed recurrent hematoma (6.0 × 4.2 cm) in the pelvic cavity and the residual pseudoaneurysm (). Following the diagnosis of recurrent bleeding from the residual pseudoaneurysm, an angiography was performed. However, the angiography failed to localize the pseudoaneurysm, and definite signs of extravasation could not be ascertained. Thus, prophylactic gelfoam embolization at the anterior branch of both the internal iliac arteries was performed (). The subsequent hospital course was uneventful, and the patient was discharged on the 25th POD. After 3 months, the previous rectal lesion (AV: 6 cm) healed, and colostomy reversal was performed without morbidity.
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pmc-6351394-1
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A 26-year-old male, not known to have any medical illness and not on current medications, who smokes for 8 years 1 pack/day, presented to Emergency Department complaining of a sudden onset of chest pain and shortness of breath for few hours’. There were no other associated symptoms and no history of trauma or any strenuous activity. Drug, family and psychosocial history were negative. No genetic information was available. Upon arrival, he was anxious and ill looking with respiratory distress. His initial vital signs were: Pulse 78 per minute, Blood Pressure(BP) 130/80 mmHg, Temperature 37 °C and oxygen saturation on pulse oximetry 95% (at room air).Chest Auscultation revealed decrees air entry over the right hemithorax and hyper-resonant percussion noted over the same side. Chest radiograph () showed right apical pneumothorax with air fluid level and a collapsed lung. A decision was taken to insert a thoracostomy tube. A tube (size 32Fr) was inserted in the 5th intercostal space anterior to the mid-axillary line and then it was connected to underwater seal system with suction. Upon insertion of the tube, the initial drainage was more than 500 cc of blood.Routine Laboratory investigation revealed: white blood cells 20.4 × 1000/uL, haemoglobin 11.7 g/dl, hematocrits 34.6٪, Platelets 207 × 1000/uL, PT 12.5 s, PTT INR 1.0.Liver Function Test (LFT) and Renal Function Test (RFT) were within normal limits. Chest radiograph post-thoracostomy tube insertion () was still showing right pneumothorax and opacity, most likely retained hematoma.Patient condition did not improve over the following hours. He collected almost 1200 cc of blood with persistent tachypnea and respiratory distress over 3 h. A decision was taken by the consultant thoracic surgeon to proceed with operative option, u-VATS. After induction of general anesthesia and double lumen endotracheal tube was inserted, patient was positioned on left lateral decubitus position. A camera 5 mm/30-degree scope was introduced through the already existing thoracostomy tube incision. The pleural cavity explored, a large hematoma was evacuated (). After complete removal of hematoma, exploration was done and there was an active source of bleeding in a vascular adhesion around the subclavian artery which was well controlled by surgical clips. A small bulla was found in the apex of right upper lobe and grasped by endograsper from the same thoracostomy incision, then the apex was resected using stapler device (60 mm covidien™) which was also inserted through the same incision. Thoracostomy tube was inserted after that and connected to underwater seal system with continuous suction for 2 days' post-operatively. The procedure was done in accordance to surgical guide and principles which was well tolerated by the patient, he had an uneventful postoperative course without a special consedration and discharged in a stable condition. Chest radiograph at the time of discharge showed complete right lung expansion. The histopathology of the resected specimen showed consistent with bullae with emphysematous changes, inflammation, and hemorrhage. No malignancy.
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pmc-6351429-1
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A 14-year-old boy presented to the surgical consultation room because his mother was worried about a round brown skin lesion over the right scapula which enlarged in size and became deeper in color over the last year, there were no symptoms associated with this lesion but the family was worried about it. There were no relevant past medical, past surgical, or family histories for the chronic illnesses or skin diseases.
During examination the lesion was a round, brown, and slightly elevated from the skin surface. The size was about 1 cm in diameter with central projection. The location of the mass was in the region of the right shoulder, .
On the basis of this clinical appearance the condition diagnosed as a supernumerary nipple located in this site.
This is an extremely rare location, and no case has been reported before.
No specific therapeutic work up done.
The family was reassured about the diagnosis and the patient sent home to be followed if any symptom developed in the future.
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pmc-6351509-1
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A 33-year-old man had a non-enhancing heterogeneous tumor in the right frontal pole on MRI. En bloc resection was performed as described previously [] and histopathological examination showed an oligodendroglioma WHO grade II with IDH1 mutation, 1p/19q codeletion, and Ki67 < 5% (Table ). A total of 29 ROIs were selected on PET; 16 areas with highest MET uptake (ROI1), seven areas with medium uptake (ROI2), and six areas with lower uptake in the tumor periphery (ROI3) (Fig. ).
The MET uptake and rCBV values and the quantified protein counts (mean number of counts per ROI) in all 29 ROIs, as well as the statistical correlations between the various parameters are presented in Table . As shown, there was a strong correlation between MET uptake and tumor cell density (MET-IDH1: r = 0.91; p < 0.0001), MET uptake and vessel density (MET-CD34: r = 0.73; p < 0.0001), and MET uptake and proliferation (MET-Ki67: r = 0.71; p = 0.0465) in this tumor. No significant correlation was found between MET uptake and tumor perfusion (MET-rCBV: r = 0.06; p = 0.74). In addition, rCBV showed no significant correlations with histological cell markers.
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pmc-6351509-2
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A 50-year-old woman was diagnosed with a tumor in the left frontal pole with heterogeneous signal characteristics on MRI (Fig. ). The tumor was removed en bloc and histopathological examination showed oligodendroglioma WHO grade II with densely packed IDH1-labeled tumor cells located mainly in the grey matter []. Molecular analysis showed IDH1 mutation, Ki67 < 5% and 1p/19q codeletion (Table ). A total of 17 ROIs were selected of which eight representing regions with highest MET uptake (ROI1), four with medium uptake (ROI2), and five with lower uptake located in the tumor periphery (ROI3). The MET uptake, rCBV value, and protein expression of histological markers in these ROIs are presented in Table . There was a statistically significant correlation between MET uptake and IDH1 count (MET-IDH1: r = 0.51; p = 0.0345). As shown in Table , there were no significant correlations between MET uptake with tumor perfusion and with expression of Ki67 and CD34. We observed that several of the ROI3 in this tumor were located adjacent to or partially overlapping with the cortex, with inherent higher perfusion (Table ).
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pmc-6351509-3
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A 39-year-old man was examined showing a non-enhancing, slightly heterogeneous tumor and en bloc tumor resection was performed. Histopathological examination showed a WHO grade II glial tumor with exclusively oligodendrocytic differentiation. Molecular analysis showed IDH1 mutation but no 1p19q codeletion. In spite of the intact 1p19q chromosomes, the tumor was morphologically diagnosed as an oligodendroglioma based on its characteristic oligodendroglial phenotype throughout the entire resection (Table ). A total of 23 ROIs were selected, of which 14 in hot spot regions (ROI1), five in areas with medium uptake (ROI2), and four in areas with lower uptake in the tumor periphery (ROI3) (Fig. ) (Table ). There was a significant correlation between MET uptake and tumor cell count (MET-IDH1: r = 0.44; p = 0.0371), proliferation count (MET-Ki67: r = 0.69; p = 0.0095), and vessel count (MET-CD34: r = 0.67; p = 0.0005). No significant correlations were present between tumor perfusion and histological cell markers.
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pmc-6351509-4
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A 53-year-old man was diagnosed with a left frontal tumor showing minimal contrast enhancement on MRI (Fig. ). En bloc resection was performed, with some loss of white matter tissue on the medial/inferior side of the tumor. Histopathological examination showed IDH1-mutated codeleted oligodendroglioma WHO grade III, Ki67 proliferation rate was 25% (Table ) []. A total of 15 ROIs were identified on PET, of which nine in the hot spot (ROI1), five with medium uptake (ROI2), and due to loss of white matter tissue during en bloc resection only one representative ROI3 with lower MET uptake located in the tumor periphery. Statistical analysis showed a strong correlation between MET uptake and IDH1 (MET-IDH1: r = 0.85; p < 0.0001) (Table ). No significant correlation was found between MET uptake and tumor perfusion or the expression of other histological markers. Similar to patient 2, we observed that the single ROI3 in this tumor was located adjacent to the cortex, resulting in inherent increased perfusion values.
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pmc-6352039-1
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In May 2010, a 40-year-old Caucasian man with adulthood-onset epilepsy came to our clinic for worsening memory and poor concentration for 1 year. He had progressive cognitive impairment, specifically short-term memory loss, word-finding difficulties, slower processing speed, and difficulties in organizing and multitasking. There was no reported change in his mood with no signs of depression or anxiety. He was a university graduate without family history of dementia or past history of addiction. Being an avid MMA fan, he had been practicing the sport for over 10 years. He was previously in the US Marines before working as an MMA school manager and instructor for 5 years. Recurrent minor head concussions and transient asphyxiation episodes were common in his course of martial arts training and work. On physical examination, he had hand tremors with fine motor incoordination and lower limb ataxia. Laboratory investigations, lumbar puncture, and electroencephalography revealed normal results. Magnetic resonance imaging of the brain, however, showed mild asymmetry in the parahippocampus structures with the left hippocampus appearing slightly smaller and dilatation of the left temporal horn. A neuropsychological assessment conducted in 2010 showed above-average performances on most cognitive domains except timed working memory tasks (see ).
Since September 2010, he had worked as an English teacher, teaching his native language. Two years later, he could no longer stay in the job due to worsening memory and planning difficulties. He was also noted to be more irritable, with increased fatigability and distractibility. He was given methylphenidate (60 mg per day) to improve his attention. Furthermore, he developed benzodiazepine dependence but managed to undergo detoxification successfully. Repeated neuropsychological assessment in 2013 revealed worsening performance across most cognitive domains with significant decline in auditory and visual attention and memory, and further deterioration in executive function (see ). The clinical and neuropsychological findings suggested chronic traumatic encephalopathy (CTE). Memantine was subsequently added to his treatment schedule and he continued to be followed up in clinic. His cognitive state deteriorated progressively and he was eventually lost to follow-up.
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pmc-6352049-1
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A 19-year-old male was evaluated for painless hematochezia. A rectal mass was detected, measuring 3×2×1.5 cm. The histopathology of the tumor was compatible with infiltrative, ulcerative adenocarcinoma with the mucin-producing features, involving full intestinal wall thickness as well as a corresponding mesocolic lymph node (pT3N1Mx), KRAS and NRAS wild-type (). Hence, the patient received neoadjuvant chemotherapy, underwent proctocolectomy and subsequently adjuvant chemotherapy, including the FOLFOX regimen in 2014.
During the course of chemotherapy, the patient developed a painless right submandibular mass, ignored by him. Chemotherapy sessions continued until May 2016, when he had his first 18FDG-PET/CT, for evaluation of response to treatments as well as the new emerging pain in his chin. Unexpectedly, several 18FDG-avid foci were discovered in the right deltoid, left external oblique, posterior left biceps brachii muscles, as well as a hypermetabolic soft-tissue mass in the region of the right external tongue muscle accompanied by a lytic right mandibular lesion, suggesting metastatic disease.
In addition, a 9 mm pulmonary nodule revealing modest metabolic activity was detected in the apex of the right upper lobe, highly suggestive of metastasis (). Confirmatory excisional surgery was carried out on the right deltoid lesion (), the most hypermetabolic muscular metastasis, as well as the right submandibular mass (). As expected, these lesions were proved to be metastatic adenocarcinoma.
The other lesions did not undergo any further evaluation since these pathologically proven metastatic lesions were convincing enough to commence additional chemotherapy courses, the FOLFIRI regimen.
Immediately after termination of the chemotherapy, another painless lesion emerged on the scalp, which was proved to be subcutaneous metastatic adenocarcinoma on biopsy. However, this time, the patient refused to undergo any further treatment.
After three months, another lesion became apparent in the occipital scalp bringing about discomfort during sleep. Afterwards, the patient was reevaluated by 18FDG-PET/CT at the end of 2017 (). The imaging revealed foci of metabolic activity in the right rectus abdominis and left quadriceps muscles. However, the occipital lesion, measuring 1.5×1.5 cm, showed no abnormal FDG uptake. At this time an additional hypermetabolic focus consistent with metastasis was detected in the right adrenal gland.
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pmc-6352050-1
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A 28-year-old male presented to the hospital with a history of fatigue for 8 days, fever for 7 days, bleeding nose and gums for 6 days, and hematuria for 2 days. These signs and symptoms had no obvious cause. His past medical history and physical examination were unremarkable with the exception of patchy bleeding seen in both lower limbs.
On admission, the complete blood count of the patient showed severe pancytopenia (). Bone marrow biopsy demonstrated no hematopoietic cells (); furthermore, no reticular fiber was seen (MF-0) on reticular fiber staining. Bone marrow aspiration report revealed bone marrow suppression and A
In addition bone marrow smear and T cell subset showed CD4+CD3+lymphocyte of 33.24%, CD8+CD3+lymphocyte of 24.38%, and CD4:CD8 ratio of 1.36. The results of flow cytometry were as follows: 1) low proportion of CD34+ with nucleated cells of 0.08%, 2) a significant decrease in the proportion of neutrophils and monocytes, 3) a significant reduction in the proportion of nucleated red blood cells, 4) a significant elevation in the proportion of lymphocyte, and 5) no phenotypic abnormalities. Finally, the diagnosis of SAA was made based on the patient’s history, laboratory results, and marrow biopsy and puncture.
The patient was put on cyclosporine and androgen for the primary disease management and G-CSF for the stimulation of hematopoiesis at Zhongnan Hospital of Wuhan University, Wuhan, China. Bone marrow imaging was performed, through single photon emission computed tomography (SPECT; E.Cam, Siemens product, Hoffman Estates, Illinois, USA) by the intravenous injection of 370MBq of 99mTc-sodium phytate, 8 days after the initiation of cyclosporine plus G-CSF. The obtained images showed normal hematopoietic bone marrow activity in the central marrow correlating with level 2 of the standard bone marrow activity ().
presents the grading of bone marrow activity and its clinical significance in details. This marrow scan did not match the pathological and laboratory findings. The G-CSF was eventually stopped, together with the other medications. The second bone marrow scan for this patient was performed 4 months after the first marrow imaging. The result was abnormal and corresponded with level 1 of the standard bone marrow activity (). It showed a reduction in hematopoietic bone marrow activity in the central compartment of the skeleton. The second bone marrow image was indicative of AA and matched the pathological and laboratory findings.
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pmc-6352053-1
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A 51 years old man with poorly differentiated tonsillar carcinoma had well defined enhancing hypodense mass in left tonsillar fossa measuring 36×29×58 mms with neck nodes on CT scan. Biopsy from neck nodes showed metastasis from squamous cell carcinoma. Patient was treated with radiotherapy using Intensity-Modulated Radiation Therapy (IMRT) technique to administer 7000 cGy in 35 fractions. Concurrent weekly Cisplatin was administered intravenously in the dose of 40 mg/square meter body surface area.
Post treatment PET/CT was performed at 1 hour after intravenous administration of 6.8 mCi 18F-FDG on 6 hours fasting state. Images were acquired using 16 slice time of flight biograph horizon scanner from Siemens. Left tonsillar fossa-base of tongue-lateral oropharyngeal wall were free of FDG avid lesions or cervical nodes suggesting response to treatment. Axial CT images showed ‘polka dot’ appearance in 12th thoracic vertebra suggestive of hemangioma ().
However, the lesion showed intense FDG uptake with SUV max of 13.44 () raising a suspicion of metastasis. The patient was asymptomatic. In view of this a whole body bone scan was performed on another day, 3 hours after intravenous injection of 20 mCi of Tc-99m-MDP (Methylene Diphosphonate) using a single head E-cam gamma camera (Siemens) equipped with low energy high resolution collimator. The images did not reveal any osteoblastic lesion ().
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pmc-6352060-1
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Our case was a 71 years old woman suffering from rest and postural tremor in the upper limbs since 8 years ago. She also complained of bradykinesia and memory problems. In her dynamic MRI (Siemens, Germany, T2-weighted with Gadolinium enhancement), a contrast-enhanced tumor in the cerebello-pontine (CP) angle was found which was compatible with a meningioma ().
For differentiation of idiopathic Parkinson disease from essential tremor, dopamine transporter study with 99mTc-TRODAT-1 was requested. 4 hours after intravenous administration of 20 mCi (740 MBq) of 99mTc-TRODAT-1, brain SPECT was obtained using a dual head gamma camera (ADAC, USA) equipped with low energy high resolution collimator. Data acquisition was performed in matrix size of 128×128 and 360ο arc (180ο for each head) with 64 projections and 30 seconds per projection. Reconstruction was done with Butterworth filter with cut off frequency of 0.35 and order of 10. Chang method was used for attenuation correction. Reconstructed SPECT images showed decreased radiotracer uptake in the left putamen compatible with idiopathic Parkinson disease ().
There was also a focus of increased activity on the right side of the skull base (right CP-angle), which was compatible with meningioma on MRI. Tumor to cerebellum count ratio was 7.8 on reconstructed SPECT images ().
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pmc-6352216-1
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A 78-year-old man with a previous history of hypertension presented with progressive dyspnea, unexpected weight loss, Raynaud phenomenon, muscle weakness, dysphony, dysgeusia, and right hemifacial hypoesthesia, which had persisted for 3 months. A physical examination revealed bi-basal fine crackles and bilateral proximal muscle weakness in the upper and lower extremities. A dermatological examination revealed no cutaneous abnormalities. An electrocardiogram showed low voltages, sinus rhythm, first degree atrioventricular block, and QS-wave morphology in the anterior precordial leads. Bilateral interstitial infiltrates were found in the chest X-ray, and biochemical tests showed elevations in C-reactive protein (76 mg/L) and skeletal muscle and cardiac enzymes (creatine kinase: 1942 U/L, creatine kinase–muscle/brain: 50 ng/mL, and hs-troponin I: 31,125 pg/mL). The patient was admitted. An electromyogram showed signs of chronic radiculopathy L4–L5–S1 without acute axonal damage and primary affectation of muscular fiber in inferior extremities (fibrillation and positive waves in right psoas). During voluntary contraction, we observed many small polyphase complexes with an early recruitment pattern. The vastus lateralis and medial right gastrocnemius showed a big polyphasic complex with a reduced pattern without spontaneous activity. The deltoid and right biceps muscles had an interferential pattern without spontaneous activity. Capillaroscopy showed a reduced number of capillaries and avascular areas, and central nervous system magnetic resonance imaging (MRI) results were unremarkable. Thoracoabdominal computed tomography (CT) revealed nonspecific interstitial pneumonia and whole-body positron-emission tomography/CT revealed diffuse myocardial uptake. A cardiac MRI revealed mild systolic biventricular dysfunction, inferoseptal hypokinesia, biatrial dilatation, diffuse edema, and fibrosis in the atrial walls and right ventricle. These features fulfilled the Lake Louise criteria for myocarditis ().
Coronary CT angiography ruled out coronary artery disease. An endomyocardial biopsy (EMB) was performed and showed Dallas criteria for myocarditis, with lymphocytic myocardial infiltration and moderate fibrosis (A–C); microbiological tests were negative.
A comprehensive immunology study revealed a high titer of antinuclear antibodies (1/640), and indirect immunofluorescence showed a nucleoplasm with a speckled pattern. Antinuclear antibodies (ANA) were detected by indirect immunofluorescence (IFL) using ready-made slides from commercial sources of fixed HEp-2 cells (Nova Lite range of reagents, Inova Diagnostics). The study of autoantibodies related to the ANA pattern found was performed using a monospecific assay by inmunoblot (Euroline range of reagents, Euroimmun) in the patient’s serum being negative for Ro60, Ro52, La, RNP, and Sm. Nevertheless, given a high suspicion of autoimmune disease, the immunological study was extended to autoantibodies associated with scleroderma, such as: Scl70, PM-Scl75, PMScl100, Th/To, RNA pol III, CENP B, CENP A, fibrillarin, and Ku, using a monospecific assay by immunoblot (Euroline range of reagents, Euroimmun); being all negative. For the association of interstitial lung disease (ILD) and positive ANA pattern, we analyzed myositis-specific autoantibodies (MSA) specificities: Mi-2, MDA-5, TIF1-gamma, NXP and SAE-1, and SAE-2, the latter being positive for SAE1/SAE2, suggestive of a systemic autoimmune disease, probably inflammatory myopathy (IMM). Anti-SAE antibodies were determined using a monospecific assay by dot blot for the detection in human sera of IgG autoantibodies (Myositis12 SAE IgG, D-Tek).
Human leukocyte antigen (HLA) genotyping revealed the presence of the HLA-DQB1*03:02 allele (measured with a real-time polymerase chain reaction assay [GenVitSet, BDR]).
Treatment was initiated with pulse-dose corticosteroid therapy for 3 days, followed by a second line immunosuppressive therapy with intravenous cyclophosphamide (900 mg), which failed to slow disease progression. Indeed, cardiac involvement symptoms prevailed in the form of variable types of arrhythmia, including atypical atrial flutter, alternating with atrial fibrillation, and second- and third-degree atrioventricular block, with an intermittent left bundle branch block; moreover, cardiac enzymes remained persistently high. Third- and fourth-line therapies with immunoglobulins and rituximab (500mg) were started, respectively. On day 28 after admission, the patient evolved with progressive breathlessness, due to progression of the interstitial infiltrate and heart failure. He required intubation but remained hypoxemic, despite mechanical ventilation, and died on day 30. The necropsy pointed to acute respiratory distress syndrome (ARDS) as the primary cause of death. It also revealed vast pulmonary fibrosis, consistent with an evolved, nonspecific interstitial pneumonia and areas of diffuse alveolar damage, with edema and hemorrhage related to ARDS (), confirming the diagnosis of rapidly progressive interstitial lung disease (RP-ILD). The authorized thoracic limited necropsy revealed the indemnity of the intercostal muscular tissue. Cardiac histology showed diffuse myocarditis and large fibrotic areas.
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pmc-6352268-1
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A 58-year-old Caucasian man complained, since he was 54, of progressive walking difficulties and stiffness at lower limbs, more pronounced on the left side.
Previous clinical history as well as family history was unremarkable.
The first neurological examination, at age 54, disclosed proximal upper limb (Medical Research Council (MRC) 4/5 on the left side and 3/5 on the right side) and left lower limb weakness (MRC 4/5). Muscle tone was preserved while hypotrophy of the upper limb-girdle, upper limb proximal muscles and left lower limb was present. Winged scapula on the right side was observed. Spontaneous fasciculations were detected in the proximal segments of the upper limbs.
No sensory impairment was reported and cerebellar examination was unremarkable. Tendon reflexes were normal at the upper limbs while knee-jerk and ankle-jerk hyperreflexia was present. No Babinski sign was observed but exhaustible bilateral ankle clone was observed.
No bulbar involvement was present.
Routine laboratory tests (including blood cell count, blood glucose, vitamin B12 and folate, inflammatory parameters) and immunological tests were in the normal range. HBsAg and anti-HCV and anti-HIV antibodies as well as thyroid and parathyroid functions were normal.
Brain and spinal cord imaging was normal as well as cerebrospinal fluid analysis.
Neuropsychological examination showed moderate impairment of executive functions (abstraction, critique, working memory and planning). Abnormal calculation skills were also noted.
Needle electromyography (EMG) showed mild signs of chronic neurogenic damage on quadriceps femoris, tibialis anterior and hand dorsal interosseous muscles. Active denervation was detected in the left tibialis anterior, left quadriceps femoris and right biceps brachii muscles. Electroneurography (ENG) demonstrated a reduction of the compound motor action potential (cMAP) amplitude of the right peroneal nerve.
Motor evoked potentials indicated a hypovolted, unstructured and dispersed cortical motor response with central conduction values increased by derivation of the left lower limb. The contralateral findings were normal.
Somatosensory evoked potential showed bilateral increased central conduction time, more pronounced on the left side.
In a follow-up visit, about one year later, clinical findings were no significantly changed.
Three years after symptom onset, clinical evaluation remained unchanged except for a more pronounced proximal weakness and an increased frequency of fasciculations on upper limbs.
EMG-ENG confirmed a reduction in the cMAP amplitude of the right peroneal nerve (1.20 mV) and demonstrated denervation activity in all the investigated muscles of the lower limbs and in the right arm.
Spirometry showed a forced vital capacity (FVC) of 140%, while maximal inspiratory pressure (MIP) and maximal espiratory pressure (MEP) were reduced to 67.9% and 54%.
The last neurological examination, four years after symptoms onset, was scarcely changed. Patient exhibited a slightly paretic gait on the right side. He was able to stand from sitting without using his arms as a support. Strength was reduced at the right upper limb which can be abducted only up to 80 degrees (A). Mild increase of muscle tone at the lower limbs was detected while distribution of muscle hypotrophy and right winged scapula were unchanged (B,C). Sporadic fasciculations were observed at upper limbs, none at lower limbs.
ENG showed reduced amplitude of cMAP of right (1.20 mV) and left (3 mV) peroneal nerves with mild increase in distal latency (6.20 ms on the right side and 4.20 ms on the left side) and normal conduction velocity (43 m/s on both side). Upper limb nerves and both sural nerves were normal.
EMG displayed diffuse denervation activity associated to chronic neurogenic signs.
Right shoulder ultrasound indicated no rupture of muscle tendons.
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pmc-6352283-1
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A 28-year-old woman, affected by stage-4 CKD, was referred to our obstetric-nephrology outpatient clinic in August 2016 at six weeks of amenorrhea. While previous caregivers had recommended the therapeutic interruption of pregnancy, on the account of the high risk of kidney function impairment and of preeclampsia, she was determined to continue her pregnancy.
At referral to our unit, this small, thin woman was apparently healthy. She weighed 40 kg (BMI 16.3), was normotensive, had no sign of oedema, had a creatinine serum level of 2.73 mg/dL, and creatinine clearance on 24-h urine collection of 28 mL/min. Her BUN was 35 mg/dL and proteinuria was 200 mg/24 h.
The first detection of kidney disease had occurred at one year of age, following investigations for poor growth, polydipsia and polyuria. Already on that occasion, kidney function was reduced to about half of the normal level, and ultrasounds showed agenesis of the right kidney and hypoplasia of the left one.
She had also undergone a kidney biopsy, which led to the final diagnosis of glomerulocystic disease (), and had been on a regular nephrology follow-up since then. Kidney function showed a very slow decrease, with low-grade proteinuria and normotension. She had been on a moderately protein-restricted, mixed-protein diet since childhood, which she now self-managed, without recent dietary controls.
A treatment plan was made, including close monitoring of kidney function, blood pressure and proteinuria, psychological support, and switching from a conventional, mixed protein, moderately protein-restricted diet to the plant-based supplemented diet used in our centre [,,]. In addition, she began taking a low-dose of acetylsalicylate to reduce the risk of preeclampsia and continued supplementation with 400 mcg of folic acid per day, which she had started at the time of the positive pregnancy test. At the following visit, the patient reported nausea, which she attributed to ketoacid and amino-acid supplementation, and said she had resumed her previous diet after a couple of days of trying the new one.
At 22 weeks of pregnancy, creatinine reached 3.17 mg/dL and BUN 42 mg/dL. Proteinuria had only minimally increased (from 200 to 400 mg/24 h). However since she was reaching a BUN level considered by some authors as a maximum threshold, starting dialysis was discussed again, and a plant-based diet option, this time without aminoacid and ketoacid supplementation, was proposed. Of note, the patient had chronic acidosis (HCO3 between 16 and 18 mmol/L without bicarbonate supplementation) and bicarbonate supplementation was started during pregnancy, stabilizing blood bicarbonate levels between 20 and 24 mmol/L.
In spite of the stable protein intake, the switch from a mixed-protein to a plant-based diet was associated with a rapid decrease in serum urea and creatinine; this favourable effect was maintained up to the 33rd gestational week, when a new rise in urea and creatinine was observed, along with a mild increase in proteinuria (, ). At 31 weeks of pregnancy the patient developed cholestasis and itching: transaminase levels were normal, while blood levels of bile acids were slightly increased (10.7 µmol/L). Ursodeoxycholic acid (450 mg twice/day) was added, but the following week bile acids reached 29.4 µmol/L.
Foetal growth was normal throughout pregnancy (), and utero-placental Doppler flows were normal both at the umbilical and uterine arteries.
At 33 weeks + 5 days, considering the gestational age achieved, the progressive worsening of the renal picture and the patient’s desire to preserve her renal function, labour was successfully induced with the placement of a cervical ripening balloon and subsequent amniorexis and oxytocin infusion. A healthy female baby, adequate for gestational age, was born on the following day (33 weeks + 6 days, birth weight 1900 g, 39th centile according to the Italian reference charts). The Apgar score was 8 at 1 and 5 min and the baby, initially under observation in the neonatal intensive care unit on account of her prematurity, was discharged in good health 10 days later.
At the mother’s final follow-up visit, two months after delivery, she was in good clinical condition, normotensive and with low-grade proteinuria. It is significant that as soon as she resumed her usual diet, her urea levels rapidly increased, even though her overall serum creatinine remained stable (2.82 mg/dL). At the child’s most recent follow-up visit (age 4 months), routinely scheduled for pre-term babies, development was normal, with a normal weight (50th–75th centile), thus making it unnecessary to schedule additional follow-ups, according to the routine policy of the hospital.
The patient reported that she had followed a moderately protein-restricted diet since childhood, mainly based on replacing the carbohydrates most often used in Italian cooking (pasta, bread, biscuits, etc.) with protein-free food. Her pre-pregnancy energy intake had been about 2000 kcal/day, with a daily intake of 0.8–1 g of protein/actual body weight, about 0.6–0.8 g of protein per ideal body weight, with wide day-to-day variations (0.6–1 g per ideal body weight). She said she often consumed high-calorie, nutritionally poor foods, such as chips, popcorn, sugary drinks, ice-cream and sweets, but did not drink alcoholic beverages.
The initial prescription consisted in a plant-based supplemented diet (6 Ketosteril tablets per day), in keeping with the usual schemes reported in detail elsewhere [,]. Protein and energy prescriptions were not changed (energy intake: 2000 kcal/day; protein intake 0.8 g/ideal weight/day). As previously reported, Ketosteril was not tolerated as it caused nausea, and the patient therefore resumed the mixed-protein diet she had previously followed, until, due to an increase in urea, a second plant-based non-supplemented diet was prescribed, once more with the same energy and protein intake.
This latter diet included small quantities of milk in the morning, and an occasional yogurt as a snack during the day, with muesli, cereal or biscuits, and a small portion of animal-derived food three to four times per week at lunchtime. Less than 20% of the protein intake was of animal origin, thus reducing the overall acid load. The fibre intake was high, but it was not specifically evaluated, due also to the indication to vary the type of food as much as possible within the described indications.
Each main meal included cereal products (pasta, rice or other grains), beans, vegetables and fresh fruit, while dried fruit and nuts were eaten as snacks during the day.
In the absence of reference data on the reference urinary urea in pregnancy, the protein intake was regularly assessed by the dietician, who controlled the patient at least monthly.
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pmc-6352365-1
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A 34-year-old Asian woman presented to the nephrology department of Peking University First Hospital in August 2015 with an over two-week history of intermittent fever. She had been on continuous ambulatory peritoneal dialysis for 9 months before admission. The patient had type 2 diabetes mellitus and initiated insulin injection five years before. Four months before admission the hemoglobin A1c level was 6.2%. Besides, she was diagnosed as anti-neutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis three years before and treated with immunosupressive therapy of corticosteroid, cyclophosphamide and azathioprine. Except for predinisone with a dosage of 2.5 mg (mg) daily, other immunosuppressive agents had been discontinued one year before. She did not smoke, drink alcohol, or use illicit drugs. Her mother had diabetes mellitus too.
Fifteen days before admission, this patient had suffered from a fever of 37.5–38 degrees Celsius, and abdominal pain. Culture of cloudy peritoneal fluid with a high nucleated cell count of 1848/m3 (80% polymorphonuclear cells (PMNs)) grew Acinetobacter baumanni. She was diagnosed as PDAP and treated with intraperitoneal vancomycin (1 g every five day) and oral moxifloxacin, Clinically improvement was observed within 24 h. Peritoneal effluent became clear and nucleated cell count decreased to 10/m3 within five days. One week before admission, the patient presented to our emergency room with a high fever (39–40 degrees Celsius) again. She reported with nausea and anorexia, but without significant respiratory or abdominal symptoms. Initial laboratory tests showed significantly elevated C-reactive protein (CRP, 114 mg/L; reference range < 8 mg/L) and procalcitonin (PCT, 19.68 ng/mL; reference range < 0.05 ng/mL). A diagnosis of relapsing peritonitis was naturally suspected. Antibiotic therapy of intravenous meropenem and moxifloxacin were given immediately according to the antimicrobial susceptibility results of the last episode of PDAP. However, the patient did not respond to the antibiotic therapy. Clinical worsening was evident with a persistent fever (> 38 degrees Celsius) and symptoms of heart failure including dyspnea and chest distress.
Infectious causes of fever were thoroughly sought. Repeated exam of the peritoneal fluid nucleated cell count and PMNs showed no abnormal. No signs of bacteria, fungi or tuberculosis were found in the peritoneal fluid. Repeated cultures of peritoneal fluid and blood came back negative. A panel of respiratory viral antibodies were screened and no significant positive results were shown. Hypae of Candia albicans in the induced sputum was found. Chest computer tomography (CT) without contrast presented large areas of lung consolidation and ground-glass opacification. Thus, pulmonary fungal infection was suspected and oral voriconazole was added, although the bronchoscopy later found no significant inflammation and culture of bronchoalveolar lavage fluid (BALF) came back negative including fungi. Immunological tests showed negative ANCA, The levels of immunoglobulins and complement components had no obvious abnormalities. Abdominal and pelvic CT with contrast had no significant findings. The serological tumor markers were unremarkable.
On admission, the vital signs were as follows: temperature 38 degrees Celsius, respiratory rate 20 breaths/minute, blood pressure 130/80 mmHg, saturating 94% with supplementary oxygen through a nasal cannula at 4 L/min. Physical examination found no skin rash or enlarged lymph node. Breath sound was lower in both lungs. On abdominal exam, she was found abdominal distention with weak bowel sounds probably due to hypokalemia (serum potassium 2.0 mmol/L), but no hepatosplenomegaly. There was mild pitting edema bilaterally in the lower limbs. The patient switched to continuous vena-venous hemofiltration (CVVH) and the symptoms of heart failure improved. However, fever persisted without any response to antibiotic therapy. Meanwhile, we observed the pancytopenia, progressive decrease of fibrinogen, as well as elevated liver enzymes (Table ).
Pulmonary infection was initially suspected but was later ruled out. Firstly, the patient had no specific symptoms of pneumonia, including coughing and sputum. The symptoms of dyspnea and chest distress were probably due to the acute heart failure, since these symptoms improved soon after CVVH therapy. Secondly, repeated chest CT (five days after the first chest CT) showed both lung consolidation and infiltration mostly absorbed. This dramatic change of chest image was more likely caused by heart failure, rather than pulmonary infection. Drug fever was also considered since the patient had administrated variety antimicrobial agents during the period. However, drug fever could not explain the deterioration of the clinical status and lab index. Further biochemical serum tests showed highly elevated ferritin (15,043.2 ng/ml). A bone marrow aspiration showed the presence of hemophagocytosis (Fig. ). The HLH was suspected. Further molecular testing indicated low natural killer cell activity (11.16%, reference range 15.1–26.9%) and high levels of soluble interleukin-2 receptor (sIL2R, > 44,000 pg/mL, reference range < 6400 pg/mL), which added to fever, cytopenia, hypofibrinogenemia, increased ferritin and the presence of hemophagocytosis in bone marrow, met the criteria of HLH [].
Intravenous methylprednisolone (Medrol) was administrated at a dosage of 40 mg daily. The general condition of the patient improved dramatically including the disappearance of nausea and abdominal distention. Fever subsided after the first dose of Medrol and the body temperature remained normal afterwards. The levels of CRP and PCT gradual dropped to normal range (Table ). However, lab results still showed rapid decline of neutrophil count (minimum 0.5 × 109/L), PLT count (minimum 34 × 109/L), and fibrinogen (minimum 1.05 g/L) during the next week (Fig. ), whereas the other coagulation parameters (prothrombin time, activated partial thromboplastin time) were all within the normal range. The dosage of Medrol increased to 40 mg twice a day, and cyclosporine was then administrated with target plasmatic levels between 100 and 200 μg/L, together with supportive treatment of intravenous immunoglobulin, fibrinogen, and fresh frozen plasma transfusion. We observed a gradual improvement of cytopenia and coagulopathy (Fig. ). The patient was discharged and continued the maintain-phase treatment of HLH on a regimen of oral prednisolone and cyclosporine. The full course of glucocorticoid and cyclosporine was 12 weeks referring to the HLH-2004 protocol []. After discharge, the patient went on intermittent hemodialysis in our hemodialysis center. The laboratory tests were taken every 3–6 months. The latest lab results (June 2018) showed normal complete cell count (white-cell count 5830/mm3, hemoglobin 11.5 g/dL, platelets 138,000/mm3). The levels of ferritin and CRP were also in the normal range (123.5 ng/ml and 3.04 mg/L, respectively). Until now, no recurrence of HLH was observed.
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pmc-6352386-1
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Client A was a woman aged 79, living alone in an upper flat of a block. There was no elevator and she had to manage twelve steps to the entrance door. She had an upper and lower limb weakness that limited her mobility and also had difficulties getting into her bathtub. She was an owner-occupier and had applied for mandatory grants to replace the existing bathtub with a level access shower tray. Client A received her new shower with funding of £3624.32 on 21 January 2015 and the whole process took around 15 months (). She was initially referred by C&R to the social work department on 13 October 2013 and 50 days later, the case was allocated to the OT for assessment, which was completed on 30 December 2013. C&R, an adaptation agency, involved after the OT completed the assessment. It provided a range of assistance, including supporting the client to access grant funding and coordinating the installation process. Within two weeks after receiving the case, C&R visited the client on 12 March 2014 to look at the property’s condition, offer technical and architectural advice about the adaptation, and check the client’s entitlement to benefits. Eighteen days later, when the specification and appropriate technical drawings for the adaptation were produced, C&R invited contractors to visit the client with a view of providing quotes for the work. Once an estimate was received, C&R prepared all the relevant documents, including planning permission, building insurance, property deed, relevant certificates and benefits evidence, on behalf of the client for grant application. This took nearly two months from 20 March 2014 to 13 May 2014. Furthermore, the housing department took nearly five months to approve the grant application; the client had to wait for more than three months after grant approval before using the new shower tray.
Client A’s timeline was consistent with the general trend of significant delays during the two stages of funding and installation. The longest wait of 143 days remained at the funding approval stage, which was mainly caused by limited available resources in conjunction with large backlogs of grant applications. Because the client’s property is a typical flat, the adaptation work requires a building warrant. Applying for this warrant took roughly six weeks, resulting in an elapse of 58 days from grant approval to contractor instructed. Installation work took 62 days, within which the client postponed the process for two weeks in order to celebrate Christmas and New Year with her family. Likewise, the client experienced significant waiting times from referral to allocation, although assessment of need was undertaken shortly afterwards. Normally, once the assessment is completed, the OT closes off the case and soon passes it to C&R. However, in this case, it took 59 days for the case to come to C&R after assessment. This substantial delay reflected fragmented responsibilities and the lack of partnership working. In fact, C&R did not know until the case came from OTs and was not able to start the subsequent process as soon as assessment was completed. After C&R took over the case, there was another prolonged wait of 75 days and the main cause was preparation of all supporting documents for grant application, including an application form, an OT report, an approval of pension credit, a schedule of works, two priced estimates, a copy of the title deed.
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pmc-6352421-1
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A 31-year-old woman was referred to our emergency department with a shortness of breath. Mother of 3 children, she is in her 32nd week of pregnancy. At the beginning of the current pregnancy, swelling of the cervix appeared the size of which gradually increased, for which no consultation or diagnostic test was performed.
In fact, the symptoms began 7 days before admission. The patient had an increasingly aggravating dyspnea, first on exertion, then at rest, with notion of orthopnea, evolving into a context of apyrexia, which required an urgent consultation in our hospital.
On examination she was found to be severely dyspneic, an inspiratory stridor was audible, her respiratory rate was 40 breaths/min, her heart rate was 120 beats per minute, her blood pressure was normal (120–82 mmhg), and her Spo2 was 87% on room air. Inspection and cervical palpation revealed a large goiter. It was homogeneous without palpable nodules. A compression of the trachea by goiter was suspected. Given the patient’s gradual onset of dyspnea and anxiety, she was admitted to the observation room after oxygenation intranasally. Thoracic surgeons and gynecologists have been informed. Shortly after, the patient showed signs of respiratory struggle and her conscience began to deteriorate. During pulmonary auscultation, ventilation in both lungs was reduced. A diagnosis of acute respiratory obstruction secondary to enlarged goiter was very likely. An alert was triggered and she was immediately transferred to the intensive care unit (ICU).
In the ICU, after monitoring, an arterial gasometry was performed. The intubation equipment and induction drugs were ready. Arterial gasometry has revealed the following values (Pao2 = 58 mmHg; Paco2 = 81 mmHg; PH = 7, 09; Sao2 = 86%). The patient begins to show signs of respiratory exhaustion with bradypnea, progressing rapidly to respiratory arrest. The heart rate began to drop and blood pressure dropped to 79–35 mmhg. The decision was made to intubate the patient using rapid sequence induction. The patient was preoxygenated quickly without ventilation and induced. Etomidate was administered at a dose of 0.3 mg / kg followed by succinylcholine at the dose of 1.5 mg / kg. Sellick maneuver was not possible because of goiter compression. The intubation was attempted by the direct laryngoscopy and showed a central grade 1 view of the larynx (based on Cormack and Lehane classification). Intubation was performed using an intubation stylet and a lubricant gel. A size 6.5 cuffed polyvinyl chloride endotracheal armed tube (ETT) was passed after feeling a weak resistance. Correct positioning of the (ETT) was confirmed by pulmonary auscultation. After manual ventilation, respiratory distress was relieved and the hemodynamic state stabilized.
A cerebral and thoracic computed tomography (CT) scan were performed and revealed a parenchymal nidus secondary to pneumonitis by inhalation and a large goiter plunging into the upper mediastinal orifice, 2 cm below the thoracic orifice, heterogeneous, measuring 71x44x100 mm, including an intrathoracic portion of the trachea and repressing the vascular structures which remain permeable (Fig. ). The thyroid function tests were within normal range, with thyroid stimuling hormon (TSH) = 0,27μUI/ml, free triiodothyronine (FT3) = 5,6 pmol/l and free thyroxine (FT4) = 11 pmo/l. An obstetric ultrasound performed found a viable fetus and a positive cardiac activity. A thyroidectomiy was decided 48 h later, after preparation by corticotherapy (dexamethasone at 12 mg per day for two days), to accelerate fetal lung maturation.
As expected, the patient was admitted into the operating room after 48 h (Fig. ). The thyroid gland was found to extend in the intrathoracic part of trachea, causing severe concentric constriction of the airway. Vascular structures were slightly repressed. The removal was completed successfully by a combination of traction and blunt dissection. A total thyroidectomy was performed (Fig. ). Tracheomalacia was suspected because of the dysmorphic shape of the intrathoracic trachea in combination with the slightly softer tracheal cartilage on palpation after goiter resection. No intraoperative tracheostomy was necessary and the surgery was completed. Prior to reversal of residual neuromuscular blockage and after careful aspiration of the oropharynx, the tube balloon was deflated. No leaks were observed, and the diagnosis of tracheomalacia was almost confirmed. It was decided to leave the endotracheal tube in situ for 24 h. She was transferred back to the ICU where an extubation was attempted the next day and ended without incident. After a stay in the thoracic surgery department, the patient was discharged from the hospital on the 5th postoperative day without any complications. The pathology showed multinodular goiter with areas of atypical hyperplasia without no sign of malignancy, it mesured 13x9x5,5 cm. Patient was treated with levothyroxin sodium tablets 75mcg/day. Four weeks later, the patient gave birth to a healthy new born with no significant abnormalities.
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pmc-6352737-1
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LD is a right-handed, 26-year-old male, with 8 years of formal education, working as a kitchen aide. He had no medical history, but had been addicted to heroin for approximately 1 year. He suffered a sudden circulatory collapse of unknown duration due to a heroin overdose. He remained in a post-anoxic coma for 15 days. Upon awakening from the coma, LD’s behavior was characterized by significant inertia, lack of drive and complete loss of self-activation. In the absence of stimulation, LD neither talked, nor initiated any activity. He did not spontaneously complain about his state, although he acknowledged being ill and having voice, language, and memory problems. After direct questioning, he declared that he felt complete mental emptiness.
In the neurological examination LD showed a major impairment in his speech. His voice was aprosodic, hypophonic and characterized by accelerated articulation that led to an almost unintelligible speech. However, he was able to temporarily raise the volume of his voice upon request. The examination also revealed bilateral hyperreflexia. A psychiatric evaluation indicated low anxiety level, and marked flattened affect. When asked about his feelings and emotional reactions, LD declared to have none, but that he might have some if he were to encounter exceptionally intense situations. However, it was not possible to observe or provoke any sign of such reactions. LD reported non-significant depressive symptoms, and presented mild compulsive counting and checking.
One year following symptoms onset, forelimb dystonic syndrome appeared predominately in the left arm. The neurological examination showed preserved motor strength, brisk reflexes with a bilateral Babinski sign, dystonia and an intense akinetic syndrome without rigidity. Dystonia was particularly severe in the left hand, which was kept in a fist posture, and in the left foot, which exhibited hyperextended toes. He showed a pushover reaction toward his back when in a standing position. LD was very slow to initiate gait, which was slowed down and disturbed by akinesia, dystonia and freezing. His face was hypomimic with loss of spontaneous blinking and facial dystonia (so-called, sardonic smiling). Ocular movements were characterized by slow saccades.
Various treatments were attempted during the first 15 months following symptoms onset. LD was treated with an increasing dosage of carpipramine (50 – 150 mg/day), which was discontinued after 4 months due to the absence of any change in the patient’s behavior. Clonazepan (2 mg/day) led to an amelioration of dystonia. Levodopa was prescribed to treat the LPSA syndrome, and the dosage was progressively increased to a maximum of 750 mg/day, however, it was discontinued since no clinical change was observed. Fluvoxamine treatment was also used (100 mg/day) for 4 months, without noticeable change.
Three years following symptoms onset, LPSA had partially regressed. LD could carry out spontaneous motor activities, such as choosing movies to watch and going shopping regularly. He reported increased emotional response and mental activity. Notably, he also reported having dreams again. He described no longer performing compulsive behaviors, or having obsessive thoughts. Although he was able to spontaneously carry out many simple everyday living activities, he still had significant difficulties in planning and performing more complex actions, for which he depended on external control. His speech was less hypophonic, less accelerated, and more intelligible compared to previous neurological examinations. He still presented facial dystonia. Despite the persistence of asymmetric dystonic syndrome that was more accentuated in the left hand and foot, his motor performance strikingly improved, with quicker and more coordinated movements.
A CT scan was performed within the first 15 days after the cardiac arrest. Fourth months after the cardiac arrest, LD received a brain MRI and a SPECT. A second MRI with sequences for 3-D reconstruction in order to precise the localization of the striatal damage was performed 3 years following symptoms onset.
The CT scan showed diffuse cerebral swelling. The first MRI showed extensive bilateral lesions affecting the lenticular nucleus and other partial lesions in the caudate nucleus, as well as in adjacent white matter between these structures. The SPECT scan revealed bilateral prefrontal hypoperfusion. The second MRI allowed a more precise localization of subcortical lesions revealing that the lesions were larger in the putamen than in the caudate and had a clear dorsoventral gradient that was predominantly rostrally to the anterior commissure and spared most of the ventral striatum. The same pattern was found in the pallidus, in which the lesions affected more the dorsal than the ventral region. Additionally, the MRI scan showed mild lesions of the anterior and antero-superior periventricular white matter in the frontal region and moderate atrophy of both hippocampi and the left hemisphere of the cerebellum (see Figures , ).
LD completed a comprehensive neuropsychological battery 4, 15, and 36 months after the cardiac arrest, as well as neuropsychiatric assessments 15 and 36 months after the symptoms onset. More details about the assessments are presented in the section.
In the first neuropsychological assessment, LD’s lack of initiative was a considerable limitation and it was only partially completed at that time. In the second and third neuropsychological assessments, the primary disorder was a prominent dysexecutive syndrome with reduced verbal fluency, cognitive slowing and considerable inertia. LD’s performance on the California Sorting Test provided a striking illustration of his deficits, performing similarly to patients with focal damage to the frontal lobes (). Other major disturbances were a reduced global cognitive capacity, and impaired working and episodic memory. The three consecutive neuropsychological evaluations showed consistently below expected performance in global cognitive efficiency and episodic memory, as well as more severe impairments on executive functions, with a slight improvement in conceptual capacities and lexical evocation in the third evaluation (see Table ).
In contrast to the stability of the cognitive disorder, the neuropsychiatric assessment revealed a progressive recovery over time (see Figure ). The emotional indifference and loss of drive scores improved by approximately 75% between the two examinations. The apragmatism score improved by 15%. The Obsession and Compulsion Evaluation Scale scores did not change between the two examinations, with both scores reflecting the presence obsessive compulsive-like symptoms.
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pmc-6354018-1
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A 43-year-old female with left-sided mastodynia was referred to our department for magnetic resonance imaging (MRI). Her surgical history included an excisional biopsy of a fibroadenoma in the left breast.
On MRI, no explanation for the clinical complaints could be found. However, an enhancing stellate lesion (7 mm) at 9 o’clock in the contralateral right breast was noted (Figure , arrowhead). The lesion had a homogeneously low signal on T1-weighted images (Figure , arrow) and rapid early enhancement with plateau kinetics on dynamic contrast imaging. There was no diffusion restriction. Because of the discrepancy between the suspicious morphological findings and the rather reassuring dynamic characteristics, further mammography and breast ultrasound evaluation were recommended.
On mammography of the right breast, a focal asymmetric density with spiculated margins was seen in the superolateral quadrant (Figure , arrow). There were no associated microcalcifications or suspicious lesions elsewhere. A second-look ultrasound in this region demonstrated a small oval hypoechoic mass (4 mm) with partially obscured borders (Figure , arrow). This lesion was categorized as a BI-RADS 4b lesion, and core needle biopsy was advised.
Histological analysis revealed an infiltrative stromal process with interlacing bundles of fibroblast and myofibroblast, suggestive for a desmoid tumor. No nuclear atypia or mitosis were seen. Wide excision was recommended and confirmed fibromatosis.
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pmc-6354136-1
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We report the case of a 43-year-old HIV-positive transgender woman. The patient presented at her physician in December 2015 after discovering intermittent vaginal bleeding for 4 weeks. For the same period of time, she stated a foul-smelling vaginal discharge and occasional pain in the genital region that increased while walking.
The patient had undergone genital reconstruction surgery in Thailand at the age of 21 and had been on hormone therapy ever since. Unfortunately, the documents of the procedure were unavailable. However, it seems highly probable that the neovagina was formed using penile and scrotal skin since clinical examination indicated a full skin graft. In addition, combined penile and scrotal skin inversion is the most frequently performed vaginoplasty in transgender women, as mentioned above. The first 11 years after the creation of the neovagina remained uneventful. The following year, the patient was diagnosed with an HIV infection CDC-classification stadium B3. She then received antiretroviral therapy, which she interrupted twice but with negative viral load at time of presentation. Several months after the HIV diagnosis, the patient suffered from a cohabitational injury, which was revised subsequently.
With the history of vaginal bleeding, her physician referred the patient to the gynaecology department of the University Hospital Zurich. Vaginal and speculum examination revealed an ulcerative lesion, which was painful on palpation and started bleeding in contact with a swab. The patient denied recent cohabitations and vaginal trauma.
The swab of the lesion revealed a high-grade squamous intraepithelial lesion (HSIL). Subsequent MRI showed blood in the neovagina and could not exclude a neovaginal tumour. Nevertheless, no extravaginal masses or enlarged lymph nodes were detectable. PET-CT scanning was performed and revealed a distinctly progressive tumour at the apical anterior wall of the neovagina with a wall thickness of 1.3 cm (). The mass was highly FDG-active and infiltrated the neovagina in a craniocaudal extension of at least 4.6 cm. The scan also showed a slightly enlarged left inguinal lymph node compared to the MRI scan performed before. Moreover, multiple FDG-active osseous metastases were visible, located at the 5th right lateral rib, the 3th lumbar vertebral body, and the right side of the sacral bone (). Fine-needle biopsy confirmed the diagnosis of a high-grade squamous carcinoma.
In the given palliative situation, concomitant chemoradiotherapy was started with the aim of gaining locoregional tumour control. 6 cycles of cisplatin chemotherapy and percutaneous radiation therapy were conducted on the primary lesion, the pelvic lymph system, and the osseous metastases. This was followed by another 6 cycles of first-line chemotherapy. The patient already suffered from impaired general condition aside from the known comorbidities such as HIV infection. For this reason, the gynaecological tumour board discouraged a more aggressive chemotherapy usually applied in case of metastatic penile cancer. Six cycles of carboplatin/paclitaxel and concomitant bone protection (denosumab) were suggested instead. The PET-MRI scan after completion of chemotherapy showed a mixed response. It included regression of the primary lesion, a progressive hepatic metastasis, small lung, and several additional osseous metastases. Hence, selective internal radiation therapy was performed on the right hepatic lobe. A further approach was made, consisting of percutaneous radiotherapy on the osseous metastasis of the scapula. Unfortunately, continuous progression of the hepatic, osseous, and pulmonary metastases was visible in a PET-CT 2 weeks later, and the options of further treatment were profoundly discussed. Retrospectively performed PCR of the tissue sample, which was gained at the time of diagnostic biopsy, confirmed infection with high-risk HPV type 51. At this time, the patient was already in reduced general condition and did not qualify for another combination chemotherapy. Nevertheless, the patient explicitly wished to proceed with systemic therapy. A cycle of more tolerable topotecan monotherapy was therefore attempted, analogous to the treatment of SCC of the cervix. In addition, radiotherapy on the osseous metastases was continued. When the patient started to perceive dizziness shortly afterwards, a cystic cerebellar metastasis with partial compression of the 4th ventricle was detected by MRI scan. To ease her symptoms, suboccipital craniotomy was performed with macroscopically complete resection of the lesion. Histopathology confirmed the diagnosis of a keratinizing SCC metastasis. At this point, our patient was, regrettably, in terminal stage. She was transferred to a Palliative Care Center, and we made efforts to relieve her from pain as much as possible. Shortly afterwards, during the process of this report, our patient passed away, 2 years after primary tumour diagnosis.
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pmc-6354148-1
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A 34-year-old nulligravida with a remote history of follicular cyst treated by ovarian cystectomy presented with acute abdominal pain associated with emesis. She was hemodynamically stable, but her abdominal exam was remarkable for rebound tenderness. Complete blood count revealed hemoglobin of 5.4 mg/dL (hematocrit of 18.7%) and an undetectable platelet count. Computed tomography of the abdomen and pelvis revealed moderate-volume hemoperitoneum () and contrast blush surrounding the left ovary (), which was consistent with low volume active blood loss from the left ovary.
Five weeks prior to presentation, the patient experienced prolonged gingival bleeding after a dental appointment. Two weeks following this, she began to experience spontaneous bruising, epistaxis with minimal trauma or sneezing, and cravings for ice chips. This was followed by uncharacteristically long and heavy menses, during which the patient soaked 1 pad every 1-2 hours. She began to feel fatigue and shortness of breath with minimal activity. The day prior to admission, she began to feel abdominal bloating and the following day she described waxing and waning, moderate to severe abdominal pain.
She was admitted to the intensive care unit out of concern for possible spontaneous intracranial hemorrhage (ICH). She was transfused two units of platelets and two units of packed red cells; her platelet count rose only to 13 × 109/L and several hours later fell to 11 × 109/L (). After review of her peripheral blood smear, hematology began empiric treatment for immune thrombocytopenia with IV immunoglobulin and IV methylprednisolone. The patient's platelet counts began to spontaneously improve without additional transfusion, consistent with a consumptive thrombocytopenia; at discharge, platelets were 162 × 109/L. The patient's hemoglobin recovered appropriately after platelet count recovered, presumably because the spontaneous bleeding from the left ovary ceased.
A battery of hematologic, infectious, and rheumatologic testing revealed an antinuclear antibody (ANA) titer of 1:640, a negative double-stranded DNA (dsDNA), positive anti-Smith antibodies, positive anti-SSA antibodies, and positive anti-RNP antibodies. The patient met criteria for systemic lupus erythematosus (SLE), and her thrombocytopenia was attributed to this. Interestingly, the patient's direct Coomb's test was positive, which is unusual for ITP. The patient had normal bilirubin and liver function tests, but it was thought that she had an early synchronous autoimmune hemolytic anemia (AIHA), which can be associated with thrombocytopenia and can develop in the early years of diagnosis []. The patient's response to steroids and normalization of hemoglobin levels with normalization of platelet function illustrates that her AIHA was mild and responsive to steroids alone. The patient was discharged in stable condition on 1 mg/kg oral prednisone and plaquenil.
One week after discharge, the patient was without active bleeding and ecchymoses were fading. However, she was found to have a platelet count of 28 × 109/L, and was treated for steroid-refractory ITP with rituximab 375 mg/m2 weekly for four weeks. She was placed on oral imuran in hopes of better controlling the underlying SLE. The patient responded well to these interventions, with subsequent platelet recovery to normal levels. She was vaccinated for pneumococcus and meningococcus, and a discussion was held regarding H. influenzae vaccination in anticipation of possible splenectomy later in life.
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pmc-6354150-1
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A previously healthy 40-year-old man was referred to our intensive care unit from a regional hospital with aphasia, somnolence, weakness, maculopapular exanthema with palmoplantar hyperkeratosis and renal failure. He had been suffering from progressive fatigue and weakness for several weeks. On admission to the ICU the patient was somnolent, only responding with undirected movements to painful stimuli and incomprehensible sounds. Communication was not possible. Ptosis was evident, but pupils were reactive with normal accommodation to light. Severe tetraparesis (legs > arms) was present, and the patient was hardly able to move his tongue. Muscle fasciculations were apparent, reflexes on arms and legs were nearly absent, and Babinski's sign was positive. The patient had an initial heart rate of 103 bpm (sinus rhythm) and a blood pressure of 150/90 mmHg in the presence of fever.
Sepsis was unlikely due to high diastolic blood pressure and nearly normal parameters of inflammation. A parainfectious syndrome was also unlikely due to normal antibody profiling and complement activities. Ultrasound revealed hepatosplenomegaly and enlarged and swollen kidneys with compacted marrow and echogenic cortex. Renal biopsy showed nonpurulent interstitial nephritis. Skin biopsy demonstrated perivascular dermatitis. Magnetic resonance imaging and lumbar puncture showed no signs of myelitis, encephalitis, and meningitis. Electroneurography and -myography demonstrated reduced nerve conduction velocity and spontaneous activity, consistent with severe axonal polyneuropathy. Thus, we suspected axonal Guillain-Barré syndrome and performed plasma exchange and immunoglobulin therapy. However the patient's condition further deteriorated. Tetraplegia occurred, and the patient developed progressive weakness of the respiratory muscles and coma, for which intubation and mechanical ventilation had to be started.
Due to deterioration on therapy we questioned our diagnosis. The broad clinical picture involving skin, kidneys, and the nervous system could also be caused by intoxication. While levels of many other compounds tested were normal, mercury levels were exceedingly high in peripheral blood (4255 μg/l, , ). Chemical analysis confirmed predominant presence of methyl mercury in blood, suggesting intoxication with organic mercury (Supplementary Materials ()). Despite extensive history taking and investigation, also of the social and occupational environment, the definite source of intoxication remained elusive. In retrospect, clinical signs and symptoms were consistent with severe organic mercury intoxication. Intravenous administration of the chelating agent (RS)-2,3-bis(sulfanyl) propane-1-sulfonic acid (DMPS) was combined with hemodialysis to eliminate complexed mercury. This resulted in a strong reduction of mercury levels over time (). As methyl mercury is present in the gut of intoxicated patients and absorbed via an enterohepatic circuit [], we added enteral DMPS to further enhance elimination. This bimodal chelating therapy was associated with a strong decline of blood mercury levels. In parallel mercury levels in urine and stool increased, demonstrating efficient detoxification and supporting the concept of bimodal mercury elimination. Despite the challenging diagnosis and delay in detoxification as well as the exceedingly high mercury levels, elimination was associated with improvement of clinical symptoms and organ functions. The patient gradually regained vigilance as well as motoric and neural functions. He was also successfully weaned from mechanical ventilation and hemodialysis (). A detailed description of clinical recovery is provided in . Arrhythmias did not occur during hospitalization. Eight weeks after admission to our hospital, the awake patient was sent to a rehabilitation facility. After 3 months of continuous DMPS treatment concentrations of mercury in EDTA whole blood, serum, and urine were 122, 24, and 24 μg/L, respectively. Unfortunately, the patient died 7 months after discharge from our hospital from refractory status epilepticus. Autopsy revealed severe atrophy of cerebellum, pons, and medulla oblongata (), findings that are common after severe mercury intoxication [].
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pmc-6354154-1
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A 30-year-old nulligravida presented herself in our institution because of the inability to conceive for four years. She was healthy, with no history of previous surgeries. Menarche had occurred at age 14. She had used oral contraceptives for nine years, having stopped taking them four years ago, followed by regular menses. No one in her family had history of infertility or any type of gynecologic cancer.
General examination and body mass index were normal. Analytically she had normal blood count and hormonal assay [FSH, LH, TSH, free T4, testosterone, and dehydroepiandrosterone (DHEA)]. A transvaginal ultrasonography was performed revealing a normed uterus and ovaries slightly enlarged and polycystic with a small echogenic mass in the right ovary of 15 millimeters. The hysterosalpingography was normal.
Her husband was healthy and had no relevant past medical history and his semen analysis was normal.
She was proposed for a diagnostic laparoscopy and ovarian drilling. In the surgery, both ovaries were polycystic and a yellowish nodular solid and hardened formation of about two centimeters, vascular and friable to touch, was observed in the right ovary. That mass was removed, without rupture, and removed in an endoscopic bag. Tubal patency test confirmed bilateral permeability.
The removed mass was sent to histological examination. Macroscopically, it was a yellow-colored tumor measuring two centimeters in greatest diameter, with a smooth and well-limited surface. Microscopically, the ovarian parenchyma was almost entirely occupied by a tumor lesion, represented by large polyhedral cells with small nuclei and vacuolated eosinophilic cytoplasm, without mitosis and nuclear atypia. Hemorrhage and necrotic areas were absent (). Immunohistochemical study revealed diffuse marking for calretinin, vimentin, and melan-A (). These features were consistent with ovarian steroid cell tumor NOS.
In the postoperative period, the woman is followed up with serial measurements of serum testosterone levels and transvaginal ultrasound. At 24 months, she had no evidence of recurrence and began follow-up in reproductive medicine consultation. She was proposed to in vitro fertilization treatment, but despite gonadotropin therapy (in high doses, 300U) for ovarian stimulation, she had no response. At 36 months postoperatively, she is still not pregnant and no evidence of recurrence too.
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pmc-6354159-1
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The patient was a 71-year-old woman with a history of Multiple Sclerosis during the past 12 years receiving dimethyl fumarate and baclofen. She also had hypertension and hyperlipidemia. She noticed a nodule at her left thigh, thought initially to be a mosquito bite. As the nodule grew bigger, a biopsy was performed. Histological examination demonstrated a diffuse dermal infiltration by large lymphoid cells (). Immunohistochemistry revealed that these large cells were positive for CD5, CD20, CD79a, MUM1/IRF4, Bcl6, Bcl2, and cytoplasmic IgM/λ whereas CD3, CD56, CD23, CD21, CD10, CD30, cyclin D1, CD68, lysozyme, myeloperoxidase, and CD34 were not detected (Figures –). MYC immunopositivity was observed in 20% of tumor cells but our case was considered as MYC negative since the threshold for MYC immunohistochemical positivity in DLBCL is immunostaining of >40% of tumor cells []. Ki-67 immunostaining was detected in approximately 90% of large tumor cells (). Thus, the diagnosis of PCDLBCL-LT was made on the basis of clinical, histological, and immunohistochemical findings. The patient underwent Computed Tomography (CT) scans of thorax and abdomen and a bone marrow biopsy with no abnormal findings. She had four cycles of R-CHOP, with main side effect profound neutropenia. Ten months after the initial diagnosis she experienced right hemiplegia and entered the Neurology Department since worsening of Multiple Sclerosis was the primary diagnosis. A CT scan of the brain revealed lesions on basal ganglia and a biopsy was performed. On the basis of histological and immunohistochemical findings localization of DLBCL in Central Nervous system (CNS) was diagnosed. The patient was admitted to the Hematology Department and started treatment with methotrexate 3,5 mg/m2. After the first cycle, she experienced an episode of hematuria and urinary infection with Enterococcus faecalis. After completing the antibiotic treatment, a second methotrexate treatment was given followed by a third cycle on schedule. Ten days after the last treatment, the patient had epileptic seizures and was transferred to the Intensive Care Unit where she died five days later because of progressive disease.
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pmc-6354168-1
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A 58-year-old Afro-Caribbean gentleman attended routine follow-up with a long-standing history of reduced vision (right > left eye), but with no acute visual symptoms. His past ocular history included quiescent bilateral occlusive retinal vasculitis secondary to SLE, with neovascularization in the right temporal retina secondary to a branch retinal vein occlusion that required sector pan-retinal photocoagulation laser 6 years previously. His SLE was also associated with nephritis and was controlled with Mycophenolate Mofetil 1.5g BD.
Best-corrected visual acuity was stable (right eye 6/36, left eye 6/18). No intraocular inflammation was observed. Intraocular pressures were normal. No active neovascularization was observed, though a frond of partially ibrosed neovascularization was present supero-temporally in the right fundus. His maculae were featureless but for a group of faint pinkish lesions in the right temporal macula. Fundus autofluorescence (FAF, ) and spectral-domain optical coherence tomography (SD-OCT, ) were carried out using the Heidelberg Spectralis (Heidelberg Engineering, Heidelberg, Germany). FAF revealed laser scars as temporal hypoAF areas and there was a curvilinear group of hyperAF lesions nasal to these. SD-OCT revealed a thinned dry fovea with temporal macular subretinal fluid (SRF) observed over a group of pigment epithelial detachments (PED). As the patient was asymptomatic, observation was initiated and a 4-month follow-up arranged. At review, repeat imaging revealed progression, though the fovea remained dry (). Fundus fluorescein angiography (FFA) and indocyanine green angiography (ICGA) (Heidelberg Spectralis, Heidelberg Engineering, German) revealed a branching vascular network (BVN), arising within a laser scar, with terminal hyperfluorescent polyps (i.e. PCV, ). Dilated ‘pachyvessels' were obvious in the right eye of this patient on ICGA, including areas away from the focus of the BVN. The fellow eye had a normal ICGA without pachyvessels. FFA (, Topcon Corp, Tokyo, Japan) performed 4 years previously revealed macular ischemia, active retinal neovascularization and previous laser therapy, however there was no evidence of PCV on that angiogram. Over the course of follow-up (9 months), the subfoveal space and vision were not affected (). Enhanced-depth imaging OCT revealed with the ‘double-layer' sign and polyps within the temporal macular PEDs (). OCT-Angiography images (Zeiss Angioplex) demonstrated widespread macular ischaemia in the right macula, together with the BVN and polyps ().
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pmc-6354169-1
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A 61-year-old woman presented with two supernumerary nipples located along the milk line on each side of the upper abdomen. During a few months before referral, the patient had noticed a firm palpable mass in close relations to the supernumerary nipple on the right side (). She had no other symptoms. Bilateral mammogram and ultrasound revealed normal breast parenchyma. Ultrasound of the supernumerary nipple on the right side confirmed a small mass in relation to this nipple, presenting as a hypoechoic, well-defined area, measuring approximately 10 mm in diameter ().
Upon clinical examination, the supernumerary nipple on the right side presented with inversion and a palpable firm mass underneath this nipple. Ultrasound-guided needle core biopsy (16G) was performed (). The pathology report described elongated epithelial islands composed of cubic cells, with a small centrally located lumen with focally identifiable cuticles. The epithelial structures were surrounded by a dense fibrous stroma. The microscopic analysis indicated the possibility of SyT and the lesion was categorized as a B3-lesion (a lesion with uncertain malignant potential) ().
Following a Multidisciplinary Team Conference, it was decided to recommend resection with a 5 mm rim of normal tissue, which was subsequently performed ().
Macroscopically the tumour measured 7 x 5 x 5 mm and was described as a firm and grey-white tumour, in close relations to the supernumerary nipple. Microscopically the tumour measured 13 mm in diameter and was localized in the deep part of dermis and underlying subcutaneous tissue with no relation to the epidermis. The tumour was composed of solid trabecular and glandular imitating formations with focal cysts. The epithelium consisted of cells with slightly irregular nuclei and indistinct nucleoli, surrounded by an eosinophilic cytoplasm. The glandular structures were lined by cuboidal cells. The tumour was in close association with the lactiferous ducts and smooth muscle of the nipple (). Foreign body giant cell reactions, due to ruptured cysts, were identified.
Immunohistochemical analysis showed positive reaction for CK5, CK14, and P63 () in association with the epithelium presenting as solid cords, whereas the glandular luminal cells showed positive reaction for CK7 (). Thus, the immunohistochemical analysis demonstrated the complex nature of this lesion. The diagnosis was SyT based on the pathology report.
A re-excision was performed due to insufficient rim of normal tissue in the cranial direction in order to minimize the risk of recurrence.
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pmc-6354230-1
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A 70-year-old man patient with a 40-year smoking history presented with dyspnoea. Chest X-ray and a computed tomography (CT) scan showed massive pleural effusion. He was subsequently diagnosed with stage IV lung adenocarcinoma (cT3N3M1b) according to the analysis of the pleural effusion. The tumour was negative for epidermal growth factor receptor mutations and anaplastic lymphoma kinase gene rearrangement. More than 90% of the tumour cells expressed PD-L1. He had an Eastern Cooperative Oncology Group (ECOG) performance-status score of 2. First-line treatment with pembrolizumab was initiated at the standard dosage (200 mg/body, tri-weekly), following the drainage of the pleural effusion. After two cycles and four cycles of treatment with pembrolizumab, a CT scan showed a good response in the primary lesion and carcinomatous lymphangitis of the right lung, and the volume of pleural effusion was decreased. Pericardial effusion was not observed from the time of diagnosis. After six cycles of treatment with pembrolizumab, he was suddenly admitted to our emergency clinic with dyspnoea and general fatigue. A physical examination revealed the following: blood pressure, 112/85 mmHg; heart rate, 114 beats/min, respiratory rate, 20 breaths/min; O2 saturation, 97% (with 2 L/min of O2 by nasal cannula); and temperature, 36.4 °C. Chest X-ray showed cardiomegaly. Chest CT showed a newly developed massive pericardial effusion; however, the anti-tumour effect in the primary tumour and lymphangitis were maintained (Fig. A, B). Electrocardiography showed a low QRS voltage and complete right bundle branch block with left axis deviation. Echocardiography showed a large echo-free space around the heart and the collapse of the right atrium and ventricle, consistent with pericardial tamponade. Subsequent aggressive fluid resuscitation was initiated. Pericardiocentesis was performed and 480 mL of blackish-brown fluid was drained; a drainage tube was then placed. An analysis of the pericardial fluid revealed the following: lactate dehydrogenase, 269 IU/L; protein, 5.3 g/dL; glucose, 75 mg/dL; carbohydrate antigen (CA) 19-9, 2245 U/mL; pH, 7.301; and total cell count, 4625/μL with 26% mononucleocytes and 27% polymorphonucleocytes. A cytological examination of the pericardial fluid showed adenocarcinoma. Gram staining and bacterial culturing revealed no microorganisms. Chest X-ray showed the resolution of the pericardial effusion and the drainage tube was removed two days later. The patient showed progressive disease with newly emergent pericardial malignant effusion; however, the other malignant lesions improved. Finally, we decided to continue treatment with pembrolizumab. The patient showed a continuous response after 18 cycles of treatment without any further pericardial effusion (Fig. C).
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pmc-6354232-1
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A 44-year-old woman with a history of metastatic triple negative breast cancer and lung metastases presented with a six-month history of recurrent haemoptysis. She had no other significant medical history. She was initially managed for her right breast cancer with a wide local incision and adjuvant chemoradiotherapy in 2014; however, her malignancy recurred two years later. She had positive margins on subsequent right mastectomy and proceeded to excision of the right pectoralis major and overlying dermis. Six months later she was found to have bilateral pulmonary metastases and underwent initial diagnostic bronchoscopy identifying a bleeding mass in the medial segment of the right middle lobe (RB5), which was subsequently treated with topical adrenaline and biopsied – confirming metastatic disease. Her malignancy progressed despite palliative chemotherapy with epirubicin and cyclophosphamide, during which time she developed worsening haemoptysis of ~1/2 cup (~120 mL) daily. A multidisciplinary decision was then made to perform therapeutic bronchoscopy due to excessive distress caused to the patient because of haemoptysis. She underwent bronchoscopy using a therapeutic video bronchoscope (Olympus BF-TH190, Olympus Corporation, Tokyo, Japan) introduced via a rigid bronchoscope, which provided secure airway access. Endobronchial survey revealed the source of bleeding in the distal right middle lobe, although the actual bleeding source was not directly visible. A volume of 2 mL of TISSEEL was injected into the right middle lobe bronchus via a catheter followed by deployment of a size 6 Spiration (Redmond, WA, USA) IBV to add stability and prevent expectoration (Fig. ). A further 1 mL of TISSEEL was then applied over the valve (Fig. ). The procedure abolished the patient's haemoptysis instantly.
Two weeks later the patient developed recurrent haemoptysis; however, repeat bronchoscopy showed a different source of bleeding in the right lower lobe, with the existing combination TISSEEL and IBV still in place in right middle lobe and maintaining haemostasis. To control the new bleeding TISSEEL was injected in the right lower lobe bronchus distal to the opening of RB6, followed by deployment of a size 9 IBV. Further injection of TISSEEL was then applied and haemostasis was achieved. Unfortunately the patient was found to have brain metastases and died of her malignancy 10 weeks later, without recurrence of haemoptysis.
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pmc-6354232-2
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A 67-year-old woman with a history of papillary thyroid carcinoma and known bilateral lung metastases presented with several weeks of recurrent haemoptysis. Her initial thyroid carcinoma was managed with thyroidectomy and neck dissection in 1998; however, it recurred with lung metastases initially found in 2007 and managed conservatively. Her other comorbidities included grade 3 ductal carcinoma in situ managed with radiotherapy, type 2 diabetes mellitus, hypertension, gastro-oesophageal reflux disease, and osteoarthritis. She was not on any anti-platelet or anti-coagulant medication. Her volume of haemoptysis was mild with <100 mL daily. She underwent therapeutic bronchoscopy using a video bronchoscope (Olympus BF-T180) introduced via a rigid bronchoscope. The source of bleeding was identified to be originating from the lateral basal segment of the left lower lobe (LB9). A volume of 1 mL of TISSEEL was then injected into distal LB9, followed by deployment of a size 6 Spiration IBV, followed by a further 1 mL of TISSEEL to cover the valve. There were no complications during the procedure and patient's haemoptysis resolved. Eight months later she developed recurrent haemoptysis with repeat bronchoscopy showing bleeding originating from the posterior basal segment (LB10). The previous IBV was found to be in place in LB9. A volume of 2 mL of TISSEEL was injected in LB10, followed by deployment of a size 7 IBV and a further 2 mL of TISSEEL over the LB10 valve. There was excellent seal after the procedure with resolution of haemoptysis.
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pmc-6354340-1
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The patient was a 48-year-old man with a 4-year history of ureteral calculi and a 2-year history of ureteral stenosis for which he had undergone multiple surgeries. Ureteral calculi recurred many times, resulting in repeated ureteral stricture that was treated three times by ureteroscopic holmium laser lithotripsy, and twice by ureteroscopic balloon dilation. He had been admitted to our hospital for left ureteral stenosis in June 2015. Examination at that time revealed a 20-cm stenosis in the middle and lower segments of the ureter. Noncontrast computed tomography showed inflammation and adhesion around the kidney as a result of multiple ureteral surgeries, making the patient unsuitable for autologous kidney transplantation. Yang-Monti ileal ureter reconstruction was performed, and the 6-month postoperative examination revealed left ureteral patency (Fig. ), stable renal function, normal electrolyte levels, and no obvious mucus-like flocculation in the urine.
Ten months after the Yang-Monti ileal ureter reconstruction, the patient developed left lumbar pain and discomfort. Noncontrast computed tomography showed that the left kidney had a slightly smaller volume than the right, and that the left renal pelvis and renal calices were slightly expanded and hydronephrotic. Two nodule-shaped high-density shadows were present in the lower renal calyx; the diameter of the larger shadow was 4 to 5 mm. A liquid-density shadow was seen in the ileal lumen of the left ureter, with a small nodule-shaped high-density shadow (3-mm diameter) at its end. Corticomedullary development was good. During excretion, accumulation of contrast agent could be seen in the left renal pelvis, renal calices, and ureter (Figs. , and ). The serum creatinine concentration was normal.
The patient was placed in the lithotomy position under general anesthesia. The ileal ureterovesical reimplantation opening was smoothly entered under direct vision using a 9.5-Fr semirigid ureteroscope (Richard Wolf Medical Instruments, Knittlingen, Germany). Ureteroscopic examination revealed that the calculi were attached to surgical sutures located from 1 cm from the inner end of the ileal ureter to the ureteral opening, and that the sutures were embedded under the ileal ureteral mucosa in a ring shape (Fig. ). A basket extractor [NGage Nitinol Stone Extractor (NGE-017115), 1.7 Fr × 115 cm; Cook Medical, Bloomington, IN, USA] was used to hold the calculi and sutures, which were then broken up with a holmium laser (0.6 J, 30 Hz, 200-μm fiber; 60-W LISA Sphinx Holmium:Yttrium-Aluminium-Garnet Laser System, LISA Laser Products, Katlenburg-Lindau, Germany) and completely removed (Figs. , ). Ureteroscopy revealed that the Yang-Monti ileal ureter had a thick and straight lumen, smooth mucosa, and no obvious folds or anastomoses between sections (Fig. ). Using a Zebra guidewire (Boston Scientific, Marlborough, MA, USA) for guidance, a flexible ureteroscope working sheath (12/14 Fr × 115 cm, M006250226; Boston Scientific) was imbedded and inserted into the left renal pelvis under direct vision using a digital flexible ureteroscope (8.5 Fr, 11278VS; Karl Storz, Tuttlingen, Germany). Using a filling pump (26331020–1; Storz Medical, Tägerwilen, Switzerland) with an infusion pressure of 80 mmHg, the two lower calyceal calculi were identified, broken up, and removed. As a preoperative urine culture had revealed a urinary tract infection, antibiotics were administered postoperatively. Additionally, a 5-Fr ureteral catheter (Kangge Medical Instruments, Shanghai, China) and a urinary catheter were left indwelling, and were removed on postoperative day 3.
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pmc-6354351-1
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We report a male baby born in a peripheral setting at 36 weeks of gestation to a 36 year old primiparous mother whose pregnancy was complicated by breech presentation and premature rupture of membranes at 34 weeks of gestation. Delivery was by emergency caesarean section with maternal general anaesthetic for fetal distress and cord prolapse. Apgar scores were 4, 5, 5, and 8 at one, five, ten and fifteen minutes respectively. He required mask ventilation at birth for primary apnoea and was intubated at seven minutes of postnatal age for persistent apnoea. At 30 min of life he was extubated to CPAP of 5 cmH2O and FiO2 was weaned from 100 to 44%. On these settings he had a tachypnoea of 90 breaths per minute with mildly increased respiratory effort and a pre-ductal oxygen saturation of ≥97%. A capillary blood gas excluded respiratory or metabolic acidosis. Empiric antibiotics benzylpenicillin and gentamicin were commenced to cover the risk of sepsis due to the prolonged rupture of membranes. Chest x-rays at 2 and 4 h of life showed crescent shaped homogeneous opacities in both upper lung fields (Fig. ). The Neonatal and Paediatric Emergency Transport Service (NETS) was consulted. Initial paediatric radiological advice via telemedicine was that this appearance could represent bilateral pleural effusions. Accordingly the PEEP was increased to 8cmH2O; lateral decubitus x-ray (not shown) was uninformative. Due to the uncertainty regarding the cause of the respiratory distress the baby was transferred to the local neonatal intensive care unit (NICU) by NETS at 10 h of postnatal life.
A progress CXR at just under 13 h of age was still inconclusive. However a lateral decubitus film performed concurrently demonstrated air anterior to the mediastinum, consistent with a diagnosis of pneumomediastinum (Fig. ); all respiratory support was therefore ceased. Laboratory markers for infection were negative with a white cell count of 15.6 × 109/L and C-reactive protein of < 3.0 mg/L. The clinical examination of the baby was not suggestive of sepsis and chest x-ray findings were not consistent with a diagnosis of congenital pneumonia; thus antibiotics were ceased at 48 h of age. The baby’s respiratory distress resolved without active intervention and progress CXRs demonstrated gradual resolution of the pneumomediastinum. The homogeneous opacity in the upper- and mid-zones that had initially appeared bilateral and then right-sided only, was shown on chest ultrasound to be thymus rather than a mass lesion. A progress CXR at 11 weeks of age and MRI at 4 months of age demonstrated a normal thymus with no other intra-thoracic pathology. The baby was well when discharged from follow-up at 8 months of age.
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pmc-6354414-1
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The patient was a five-year-old girl who collapsed suddenly during activities at nursery school. She was healthy until that day. She had passed a regular health check one month before the event. Regional emergency workers transferred her to our hospital located 30 min away from the event and categorized as a secondary healthcare hospital without extracorporeal cardiopulmonary resuscitation (ECPR) capability. Her condition deteriorated, and she developed cardiac arrest in the ambulance. Regional emergency workers commenced Basic Life Support (BLS) while transferring her to the emergency room. Resuscitation with Advanced Life Support including intubation and repeated epinephrine was given to her after arrival at the emergency department (ED). Unfortunately, after resuscitation for a total of 81 min inclusive of four minutes pre-hospital BLS, she remained unresponsive; the resuscitation was unsuccessful. Venous blood gases on arrival to the ED showed pCO2 65.8 mmHg, and pO2 29.7 mmHg. pH, bicarbonate, and base excess were immeasurable possibly due to out of range of indication. Blood tests showed the following abnormal values: prothrombin time 20.2 s; APTT 88.7 s; D-dimer 106.3 μg/ml; FDP 249.8 μg/ml; potassium 7.3 mEq/L; creatinine 0.71 mg/dL; AST 65 U/l; LDH 821 U/l; and ammonia 477 μg/ml. Postmortem CT showed a large right abdominal mass extending through the IVC into the entry portion of the right atrium (Fig. ).
A judicial autopsy conducted at the local police department showed: [] the weight of the Wilms tumor that originated in the right kidney was 885 g, while the left kidney weighed 100 g, and no further histological examination was performed (Fig. ); [] tumor extended into the right renal vein, IVC, and entry portion of the right atrium (Fig. ); and [] greyish or dark red small multiple emboli filled the right and left peripheral pulmonary arteries (Fig. and ). Taken together, the main cause of her sudden cardiac arrest was attributed to multiple pulmonary tumor embolisms secondary to stage IV Wilms tumor.
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pmc-6354553-1
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A 23-month-old male, with left obstructive megaureter and an incomplete duplex collecting system was admitted to our Pediatric Surgery Unit for surgical treatment. The patient was born full-term by cesarean section at the 40th week of pregnancy (birth weight 3,850 g). Prenatally, at 31 weeks' gestation, a duplicated collecting system and ureter dilatation was suspected. In the first months of life, the diagnosis was made with a diethylenetriaminepentacetic acid (DPTA) renogram and confirmed by computed tomography (CT). A cystourethrogram showed no evidence of vesico-ureteric reflux.
Indications for surgery were based on a 12 month follow-up, where the following were observed: increased dilatation of the renal pelvis (34 vs. 13 mm), appearance of an obstructive curve upon DPTA diuretic renal scintigraphy, without upper kidney resolution following the administration of furosemide and the thin radiographic aspect of the cortical renal parenchyma.
Prior to admission, recurrent urinary tract infections were not documented. Preoperative (2 days pre-surgery) blood examinations and urine dipstick were normal. At admission, the patient was in good condition.
Correction of the megaureter included an open surgical approach. Through the transvescical mobilization of the megaureter, the distal narrowed common ureter with an incomplete duplex system (3–4 cm in length) was excised in order to free both ureters. No difficulties were encountered in mobilizing the ureters and extravescical ureteral exploration was not considered necessary. Both ureters measured 1 cm in diameter and ureteral plication was not performed. The ureters were reimplanted in a generous vescical submucosa tunnel, about 4 cm in length, using the Cohen Technique. The new ureteral orifices appeared large in size and ureteral stents were not necessary. A balanced electrolyte solution (5 ml/kg/h) for fluid therapy during anesthesia was infused. At the end of the operation only an urinary catheter was left in place. Resection of the ureterovesical junction of the obstructive megaureter was performed followed by common sheath vesicoreteral reimplantation (operative time: 2 h and 40 min). There were no intraoperative complications. After the procedure, no variations in diuresis were noted (3–4 ml/kg/hour).
A few hours postoperatively, the patient developed a fever (39°C), lethargy, abdominal pain, nausea and tachycardia (heart rate 170/min) with a blood pressure of 110/70 mmHg. A complete blood count showed leukocytosis (17.29 × 109/L) with an 80% neutrophil predominance, anemia (hemoglobin 7.6 g/dl) and a normal platelet count (134 × 109/L). C-reactive protein was elevated (19.52 mg/dl) and prothrombin time was prolonged (21.7 s). Acute kidney injury (serum creatinine 0.58 mg/dl) and hyponatremia (Na 130 mmol/L) were also detected. Fasting blood sugar was 56 mg/dl. The urine dipstick (measuring urine from a urinary Foley catheter) revealed leucocyturia, but his urine culture resulted negative. Escherichia coli was cultured from peripheral blood.
Abdominal ultrasound demonstrated an enlarged left kidney with grade 3 hydroureteronephrosis and highly reflective echoes consistent with the presence of gas, suggestive of EPN (Figures ). An abdominal CT scan with contrast confirmed the diagnosis of unilateral EPN and showed an enlarged, hydronephrotic left kidney with discrete amounts of gas in the pelvis, lower calyceal group, and renal parenchyma (Class 2) EPN according to the Huang and Tseng classification () (Figures ).
The patient received fluid resuscitation in the intensive care unit. Intravenous antibiotic therapy was administered: empiric antimicrobial therapy with gentamicin (4 mg/kg/die) and ceftriaxone (50 mg/kg/die) was initially started and subsequently based on the antibiogram treatment was changed to meropenem (40 mg/kg every 8 h). Blood and plasma transfusions were also required.
Considering the complex malformation including the duplex collecting system, conservative treatment was preferred. The patient's clinical status improved significantly with medical treatment. On the fifth postoperative day he was readmitted to our surgical unit. Seven days postoperatively, a follow-up sonogram showed resolution of gas in the kidney.
He was discharged 14 days postoperatively, with a normal serum creatinine, decreased inflammatory index values and clear urine. He is being followed up at our unit and the pediatric nephrology unit.
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pmc-6355184-1
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A 13-year-old male presented with a one-month history of fatigue, emesis and diffuse headache. Computed tomography (CT) and subsequent magnetic resonance imaging (MRI) revealed a 5.9 × 6.5 × 6.4 cm cortically based, heterogeneously enhancing mass involving the left frontal lobe (Figure ). He underwent a gross total resection of the tumor (Figure ) and pathology was consistent with anaplastic astroblastoma (Figure ). Molecular testing using the OncoScan microarray platform revealed a tetraploid tumor with four copies of all the autosomes, except for chromosome 1, and two copies of each X and Y. Analysis revealed a BRAFV600E mutation and a copy number loss of chromosome 9 encompassing CDKN2A/B.
Following resection, the patient underwent focal radiation therapy (59.4 Gy in 33 fractions) with concurrent temozolomide (90 mg/m2/day). Post-irradiation, the patient was started on maintenance therapy with dabrafenib (4.5 mg/kg/day divided twice daily) and trametinib (2 mg/day once daily). One month after starting maintenance therapy, he developed mild fatigue. Trametinib was discontinued six months later due to family preference. He had no other treatment-attributable toxicities. The patient remained disease free for 20 months at which time he presented with disseminated disease recurrence and died 2 months later (Figure ).
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pmc-6355184-2
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A 12-year-old female presented with a three-week history of diffuse headache and three days of diplopia and blurry vision. Initial head CT demonstrated edema in the left temporal and frontal lobes. Subsequent MRI revealed a 3.8 × 2.4 × 3.1 cm cortically based mass within the left superior temporal gyrus (Figure ).
The patient underwent a gross total resection of the lesion and histopathology was most consistent with an ependymoma. The initial plan was close observation, and MRI performed one month post-operatively demonstrated new infiltrating tumor within the resection cavity (Figure ). The patient underwent a partial re-resection (Figure ) followed by two cycles of chemotherapy (cisplatin, cyclophosphamide, etoposide, and vincristine). Post-chemotherapy MRI again demonstrated tumor progression requiring additional surgery. Histopathology and immunohistochemistry analysis at the time of the third resection (Figure ) were more consistent with HGG and OncoScan revealed a BRAFV600E mutation. OncoScan also detected numerous copy number abnormalities including homozygous copy number loss at chromosome 9 involving the CDKN2A/B locus. The patient underwent focal radiation therapy (54 Gy in 30 fractions) with concurrent temozolomide. MRI obtained one-month post chemoradiotherapy again showed tumor progression (Figure ). At that time the patient was started on BRAF inhibitor monotherapy (dabrafenib 4.5 mg/kg/day divided twice daily). MRI performed two months later demonstrated a significant decrease in tumor size (Figure ). Six months into treatment with dabrafenib, trametinib was added (2 mg/day). She has had no dose-modifying toxicities. The patient has remained on therapy with a small amount of stable disease for 32 months.
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pmc-6355184-3
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A four-year-old female presented after an episode of headache followed by loss of consciousness. On physical exam she was noted to have a left sided visual field deficit. MRI of the brain demonstrated a 6 × 4.6 × 5 cm mass centered in the hypothalamus with expansion into the suprasellar area and pons with intratumoral hemorrhage (Figure ). The patient underwent a biopsy and ventriculoperitoneal shunt placement. Pathology was consistent with anaplastic ganglioglioma (Figure ) and OncoScan revealed a BRAFV600E mutation. In addition to the BRAFV600E mutation, a deletion on the short arm of chromosome 4 and numerous copy number alterations spanning chromosome 22 were also discovered. No CDKN2A loss was identified. Given the young age of the child and desire to avoid irradiation, the decision was made to proceed with targeted therapy. The patient was started on dabrafenib (4.5 mg/kg/day divided twice daily) upfront with the addition of trametinib (0.025 mg/kg/day) one month later. Four weeks after initiation of therapy her visual deficit resolved. MRI obtained three months after initiation of therapy demonstrated an 85% decrease in tumor size (Figure ). MRI performed after eight months of therapy demonstrated a further decrease in size (Figure ).
The patient reports no side effects and has remained on therapy with stable disease for 23 months.
Dermatologic exams and echocardiograms were conducted every three months and ophthalmologic evaluations were completed every three to six months for all patients. None of the patients developed any significant dermatologic, cardiac or ophthalmologic findings.
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pmc-6355299-1
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A 63-year-old man with a history of type 2 diabetes mellitus complicated by a prior stroke, chronic foot ulcers, and end-stage-renal disease (ESRD) on hemodialysis presented with a fever and increased drainage from a right foot ulcer. A computed tomography (CT) scan of his foot showed cortical destruction and sclerosis consistent with osteomyelitis. The patient underwent a toe amputation and a six-week course of intravenous (IV) cefepime 1g every 24 hours and vancomycin 1,750mg with hemodialysis three days a week. Three days after starting cefepime, the patient became confused during hemodialysis and had difficulty grasping objects with his right hand. The head CT was negative for acute intracranial pathology and his laboratory tests were unremarkable. Brain magnetic resonance imaging (MRI), lumbar puncture, and electroencephalogram (EEG) did not reveal the cause of his encephalopathy. Despite the cessation of all sedating and psychotropic medications, the mental status failed to improve. Review of the patient’s medical records showed that he had received cefepime, with dosing unadjusted for his impaired renal function, for two days following his procedure. Cefepime was promptly discontinued which corresponded to the 12th day of hospitalization. He was then started on ertapenem. His mental status returned to baseline two days later without any neurological sequelae. He continued to be on ertapenem along with vancomycin without manifesting any further encephalopathy during the remaining part of his hospital course.
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pmc-6355299-2
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A 65-year-old female with a past medical history of lupus, hypertension, ESRD on dialysis, and recent left lower extremity graft repair presented to the hospital with complaints of fever, pain, and redness around her graft site. Upon admission, she was febrile and tachycardic with a white blood cell count of 30,000/cubic millimeter. Physical examination revealed erythema and tenderness around her left lower extremity graft site. Transplant surgery was consulted for debridement of her infected graft site, and she was started on IV vancomycin and cefepime 2g every 24 hours. On the second day of hospitalization, she underwent surgery but experienced right arm weakness, left eye deviation, and aphasia postoperatively. The patient was transferred to the neuro ICU where she required intubation for airway protection. The CT angiogram and brain MRI were both negative, and the electroencephalogram (EEG) showed diffuse triphasic waves and severe generalized slowing. Given the unremarkable workup, the infectious disease service recommended holding cefepime, which resulted in an improvement of mental status two days after stopping the drug. The patient was extubated and transferred to the medicine floor in stable condition.
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pmc-6355299-3
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A 70-year-old African-American female with a past medical history significant for non-ischemic cardiomyopathy, pulmonary hypertension, chronic kidney disease (CKD) stage III, and ankle fracture status post open reduction and internal fixation complicated by a wound infection, presented to our hospital with word-finding difficulty. Three weeks prior, the patient was hospitalized for a wound infection of her surgical site with wound cultures positive for pseudomonas and enterococcus. She subsequently underwent surgical debridement, incision and drainage, and was started on IV piperacillin/tazobactam 4.6g every six hours. She was later discharged to a subacute rehabilitation on IV vancomycin 1,250mg every 24 hours and cefepime IV 2g every 12 hours. At the rehabilitation facility, the patient’s daughter noted that the patient’s cognitive ability had continued to deteriorate since discharge from the hospital. The patient now had word-finding difficulty prompting an emergency department (ED) evaluation for a stroke. The vitals in the ED were within normal limits and the physical exam only remarkable for asterixis. Complete blood count showed anemia and mild leukocytosis and basal metabolic panel was remarkable for a blood urea nitrogen of 38mg/dL (ref range: 7-20mg/dL), bicarbonate of 19mEq/L (ref range: 23-29mEq/L), and a creatinine of 4.66mg/dL (ref range: 0.8-1.4mg/dL). CT, chest x-ray, ultrasound, and MRI did not identify any acute processes contributing to her presentation. An EEG suggested moderate diffuse cerebral dysfunction (encephalopathy) with possible structural or physiologic disturbances in the left hemisphere. Due to the high dose of antibiotic in the setting of chronic renal disease, there was a high suspicion for cefepime-induced neurotoxicity. Infectious disease switched the antibiotic regimen to IV meropenem 1g every 24 hours, and the patient experienced a drastic improvement in mentation. The patient was subsequently discharged back to subacute rehabilitation to finish her antibiotic course for wound infection.
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pmc-6355299-4
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A 63-year-old Caucasian woman with a past medical history of type 2 diabetes, neurogenic bladder, and a recent diagnosis of bilateral hydronephrosis was re-admitted due to worsening weakness and confusion. Two weeks prior to her re-admission, she had presented to an outside hospital for abdominal cramping and was found to have an obstructive urinary tract infectioin (UTI) with growth of candida glabrata on urinalysis. Urology was consulted for her complicated pyelonephritis with hydronephrosis, ultimately leading to bilateral stent placement. At this time, she had a creatinine of 1.2mg/dL. The patient was then discharged on fluconazole 200mg every 12 hours and cefepime 2g every 12 hours empirically for two weeks. One week later, she had complaints of weakness, difficultly ambulating, and confusion. At baseline, the patient was functional and alert and oriented to time, place, and person. However, upon admission, she was confused and oriented to only name and place but could not recall the name of the hospital. Vitals were unremarkable except for mild tachycardia (110/minute). Physical examination was significant for suprapubic region tenderness upon palpation. The repeat urinalysis was positive with culture growing candida glabrata. Head CT and other laboratory results were unremarkable. She was started on IV hydration and continued on cefepime and fluconazole. Urology performed a CT cystogram, which showed findings consistent with a combination of cystitis and partial disruption of the bladder dome, and the patient was subsequently continued on her Foley catheter that was started during the admission. Antibiotics were discontinued after the patient completed the two-week course. Within 24 hours of cessation of the antibiotics, the patient’s mental status improved. Due to persistent suprapubic pain along with re-growth of candida in the urine culture, the patient was restarted on fluconazole, which was subsequently changed to amphotericin deoxycholate for seven days based on sensitivities and infectious disease’s recommendations. The patient’s mental status returned to baseline during hospitalization in parallel with an improvement in her creatinine and discontinuation of cefepime, and the patient was discharged to a subacute rehabilitation facility.
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pmc-6355299-5
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A 60-year-old male with a past medical history of asthma, spastic paraplegia, hypertension, hyperlipidemia, peptic ulcer disease, and tibial osteomyelitis post-infected hardware removal was admitted for altered mental status. He was admitted to the orthopedic service for infected hardware removal one month prior to the current admission. He was subsequently started on IV cefepime 2g every eight hours for tibial osteomyelitis and then discharged to a skilled nursing facility for six weeks. Approximately 17 days later, the staff at the nursing facility reported that the patient was delirious, slurring his speech, and pulling out his peripherally inserted central catheter (PICC) line in the night. He was then hospitalized at an outside facility for four days where he had an extensive workup, including a CT head, MRI head, and EEG without any conclusive etiology for his altered mental status. The EEG showed generalized slowing and evidence of metabolic encephalopathy and he was discharged. He returned to an outside emergency department three days later for persistent neurological symptoms, where he was found to have acute kidney injury. Following administration of IV fluids, he was discharged to his skilled nursing facility. His mental status did not improve. After consultation with the infectious disease team, the patient was admitted to our hospital for further workup. Upon admission, his vitals were unremarkable and physical examination showed confusion and disorientation without any other focal neurological deficits. Laboratory results were unremarkable except for an elevated creatinine of 1.8mg/dL (baseline 1-1.2mg/dL) indicative of unresolved acute kidney injury. Cefepime was discontinued, and the patient’s mental status and speech improved over the next 72 hours. Nephrology was consulted. After extensive workup, acute kidney injury was presumed to be secondary to cefepime toxicity with a component of acute tubular necrosis given the hyaline and granular casts seen on urinalysis. The patient was later discharged back to the facility where he had no further episodes of confusion or altered mental status.
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pmc-6355300-1
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A 56-year-old male pedestrian was brought to our emergency department (ED) after being struck by a car at high speed resulting in an unstable pelvic fracture, massive retroperitoneal bleeding, pulmonary contusion, and traumatic rupture of the diaphragm which was not evident at the time of admission. He was admitted to our Level II trauma center ICU after initial resuscitation in the ED. Renal failure progressed rapidly due to rhabdomyolysis. His early post-injury course was complicated by refractory shock requiring high doses of vasopressors, hypoxic hypercapnia respiratory failure on ventilation support, ischemic colitis, septic shock, cardiogenic shock that required cardioversion on three different occasions, acute renal failure requiring continuous renal replacement therapy, and shocked liver. The patient required multiple visits to the operating room with initial resection of the terminal ileum and right colon, repair of the diaphragmatic hernia, chest tube insertion followed by washout, ileostomy, feeding gastro-jejunostomy tube, and biologic mesh closure.
Despite receiving high-dose norepinephrine, vasopressin, and epinephrine, the patient’s condition continued to deteriorate with a mean arterial pressure <60 mmHg. Angiotensin II (ATII) was given as an infusion starting with 5 ng/kg/minute increments. The max maintenance dose of 15 ng/kg/minute was achieved in three hours reaching our target blood pressure (BP) for the first 24 hours, and was tapered to 10 ng/kg/minute during the next 12 hours and to 5 ng/kg/minutes during the last 12 hours. ATII was completed with no side effects. The patient’s condition dramatically improved, and he was weaned off of vasopressors within three days of the ATII use. He survived his injuries and was referred to acute rehabilitation.
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pmc-6355301-1
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A 28-year-old, previously healthy woman presented to the emergency department (ED) with two months of abdominal distension and one week of upper abdominal pain. The distension had initially abated after two weeks, but then gradually worsened until presentation. Her upper abdominal pain worsened with movement and improved with sitting upright. She denied any nausea, vomiting, constipation, diarrhea, urinary symptoms, vaginal bleeding or discharge, or other complaints. She denied any prior history of abdominal distension or liver disease. She reported regular menstruation, and her last menstrual period was one week prior. Her past obstetric history was gravida three, para two, abortus one. She reported a family history of ovarian cancer and colon cancer in distant relatives.
Physical examination revealed a firmly distended abdomen with no fluid wave (Figure ). There was no focal tenderness, rebound, or guarding of the abdomen. There were no skin changes or extremity edema noted. Cardiovascular and pulmonary exams were unremarkable.
A point of care transabdominal ultrasound at the bedside showed several, large cystic structures and no obvious pregnancy. Laboratory studies were unremarkable and her beta-hcg returned negative. A comprehensive abdominal ultrasound showed a large cystic mass arising from the chest to the pelvic area (Figure ). As the source of the mass was unclear, a computed tomography (CT) scan of the abdomen and pelvis was performed and showed a multi-septated cystic mass, measuring 30.0 x 28.9 x 19.0 cm, arising from one of the adnexal regions (Figure ). Gynecology was consulted and performed a laparotomy with left salpingo-oophorectomy the following day. A 30 cm adnexal mass was removed. Surgical pathology revealed a mucinous cystadenoma with no cytologic malignancy found. The patient made a full recovery.
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pmc-6355302-1
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Clinical history
The patient is a 69-year-old man who presented with new-onset facial pain. He reported a decrease in libido and a history of nocturia. The patient had a history of prior trans-sphenoidal endoscopic endonasal pituitary surgery for a possible Rathke’s cleft cyst six months prior to presentation. Gadolinium-enhanced magnetic resonance imaging (MRI) of the brain revealed a 2.7 x 1.9 x 1.7 cm recurrence of the previously resected sellar mass (Figure ). The preoperative pituitary panel did not show any abnormalities. The patient was offered endoscopic endonasal surgery for the resection of the recurrent mass.
Surgical intervention
The patient underwent a resection of the lesion using purely endoscopic endonasal surgery (EES) with the two-surgeon technique []. Revision exposure was performed to expand the opening into the pituitary region. A dural opening was performed and several biopsy specimens were sent for intra-operative frozen section, which showed a pathological diagnosis of normal vasculature. The vascular mass was then resected until the exposure of the normal pituitary gland tissue. Adequate circumferential decompression was ensured. The routine skull base reconstruction consisted of multiple inlays of collagen matrix covering the entire bony defect. A fat graft was also applied as supplemental biological packing underlying the vascularized flap, which covered the entire construct.
Post-operative course
The patient sustained an intraoperative cerebrospinal fluid (CSF) leak and a lumbar drain remained in place for four days postoperatively with no evidence of leakage. The patient did not sustain any endocrinopathies or cranial neuropathies, remained stable, and was discharged five days postoperatively. The patient was followed up in clinic around one month following surgery and did not experience any additional deficits.
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pmc-6355664-1
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An 8-year-old girl for first-cousin parents, she is the second child among four girls of a Syrian family having a refugee-status at a camp in Sulaymaniyah, northern Iraq, since 2014. Our patient was born uneventfully in August 2010 and received BCG vaccine, according to the schedule at 7th day of age. Two months later, she developed ipsilateral axillary lymphadenitis followed by generalized lymphadenopathy. Meanwhile, features of disseminated BCG infection, including fever, weight loss, disseminated maculopapular rash, and hepatosplenomegaly, were manifested, and managed by a prolonged course of anti-TB medicines including isoniazid, and rifampin. According to the history taken from the mother, our patient had repeated episodes of non-specific illnesses, in form of relapsing/remitting maculopapular skin rash, oral thrush, respiratory infection, gastroenteritis, and urinary tract infections that were treated in an outpatient setting, in addition to one episode of meningitis treated at a hospital in Syria. At 4-year-old, as the family fled the war in Syria to a camp in northern Iraq, the child's condition was severely deteriorated and she became seriously ill with fever, night sweating, diarrhea, and poor appetite. Thus, she was referred to the intensive care unit at Hiwa Hospital in Sulaymaniyah, the northern province in Iraq. Upon admission she was toxic, cachexic, and feverish, with generalized lymphadenopathy including cervical, axillary, inguinal and epitrochlear lymph nodes. The lymph nodes were multiple, asymmetrical, and visibly enlarged with the biggest about (3.5 × 3 cm) at left axilla, firm in consistency, not tender, and discrete. The abdomen was distended with the presence of hepatosplenomegaly and ascites, in addition to right lung crepitation. The patients' growth parameters were below the third centile. Investigations showed an erythrocyte sedimentation rate (ESR) of 110 (normal range 3–13) millimeters/hour (mm/h), along with hypochromic microcytic anemia, leukocytosis, and high immunoglobulin-G assay. Ascetic fluid showed lymphocytic predominance with a serum-to-ascites albumin gradient of < 1.1 gm/dl, normal liver, and renal function tests. HIV and hepatitis screening were negative. Chest X-ray and computed tomography (CT) of the chest and abdomen showed a pulmonary consolidation at the right lower lung, in addition to mesenteric lymphadenitis disclosed by CT. Although microbiological and histopathological evaluations were not done, there was a high index of suspicion of mycobacterial infection, either in the form of relapsing disseminated BCG disease or active TB, based on the TB-prevalent situation at the area of the camp. Furthermore, the patient did not respond to an initial course of broad-spectrum antibiotics. Thus, she was treated empirically with 4 anti-TB medications for 12 months, including; isoniazid, rifampin, pyrazinamide, and streptomycin that was later changed to ethambutol. She showed a very good clinical and laboratory responses. Several months later, after stopping anti-TB therapy, she relapsed with generalized lymphadenopathy and maculopapular skin rash (Figure ).
She also had episodes of abdominal pain and bloody diarrhea, disturbed sleep, and weight loss. Our patient underwent several excisional biopsies from axillary, cervical, and groin lymph nodes, in Syria and in Iraq, but the results were non-conclusive. Moreover, during the periods of suspected infection with leukocytosis and lymph node neutrophilic infiltration, culture was not regularly done, mostly because of the limited laboratory facilities and being treated in an out-patient setting. There was no history of BCG disease or TB, among family members. On most occasions the patient had an ESR of ≥ 100 mm/h, hypochromic microcytic anemia, leukocytosis, neutrophilia, lymphopenia with hypercellular marrow examination, and low CD3 and CD4 by flowcytometry. Antinuclear antibody, in addition to toxoplasmosis, rubella, cytomegalovirus, herpes simplex, HIV, and syphilis, as well as the culture for TB, were all negative. Thyroid, liver, and renal function tests were normal.
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pmc-6355687-1
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A 34-year-old woman was admitted to our hospital presenting an 8-year history of progressively increasing fatigue, weakness and numbness in her limbs, especially in the distal part, and unsteady gait. Although she’d been to different hospitals several times and discontinuously got oral VitB12 and blood transfusion treatments, both hematologic and neurological symptoms presented poor improvement and even deteriorated. In the previous 20 days, the patient couldn’t walk or stand up, and she also experienced palpitations and shortness of breath. She has a history of vitiligo dating back more than 5 years. Her family history and her diet were unremarkable. A general examination revealed anemic appearance: pale palpebral conjunctivas, lips and finger nails. The neurological examination showed weakness (4/5) in the upper and lower extremities, decrease of superficial and deep sense below elbows and knees and hyperactive deep tendon reflexes in the lower extremities. The patellar clonuses, ankle clonuses, Babinski’s sign, Chaddock’s sign and Hoffmann’s sign were positive on both sides. She couldn’t complete the heel-knee-tibia test very well.
Laboratory tests disclosed macrocytic anemia: RBC (1.29∗10ˆ12/L, reference range 3.8–5.1∗10ˆ12/L), HGB (54 g/L, reference range 115–150 g/L), MCV (129.6 fL, reference range 82–100 fL), MHC (42.0 pg, reference range 27–34 pg), MCHC (324.0 g/L, reference range 316–354 g/L). The blood tests also showed decreased WBC (2.03∗10ˆ9/L, reference range 3.5–9.5∗10ˆ9/L), elevated erythrocyte sedimentation rate (ESR) (20.00 mm/h, reference range 0–18 mm/h ), normal ALT, elevated AST (70 U/L, reference range 13–35 U/L), elevated total bilirubin (30.1 μmol/L, reference range 5–21 μmol/L), elevated direct bilirubin (10.2 μmol/L, reference range < 6 μmol/L), elevated indirect bilirubin (19.9 μmol/L, reference range 2–15 μmol/L) and normal Cu (1166.2 μg/L, reference range 800–1500). Other significant laboratory results revealed a remarkably reduced level of VitB12 (<50.000 pg/ml, reference range 243–894 pg/ml), normal folate (19.26 ng/ml, reference range 3.89–19.8 ng/ml), increased intrinsic factor antibody (30.2 AU/ml, reference range < 1.53 AU/ml), elevated homocysteine (Hcy) (94.7 μmol/L, reference range < 15 μmol/L) and elevated LDH (3157U/L, reference range 120–230 U/L). Analyses of amino acids and acyl carnitine of metabolic disease in blood and organic acids in urine were unremarkable.
The pathology of the bone marrow biopsy reported image of hyperplastic anemia. Neurogenic damage can be seen in the electroneurography and electromyography, suggesting damage of peripheral nerves in her lower limbs. The cranial magnetic resonance image (MRI) scan had no positive findings, while spinal MRI scan showed extensive T2-weighted hyperintensity in the dorsal columns from the level of C3–C6 with inverted “V” sign on axial series (Figure ). A gastric polyp was found by gastroscope inspection (Figure ), located in the mucosa and submucosa by endoscopic ultrasound (EUS) observation. The polyp proved to be NET and revealed severe chronic atrophic gastritis in pathology (Figures –).
Our patient was diagnosed with SCD, PA, gastric NET, vitiligo and hyperhomocysteinemia. A multidisciplinary therapy plan was forumlated: blood transfusions in the first week, a daily intravenous injection of 1000 μg of mecobalamine, which is a form of VitB12, for 14 days followed by 1000 μg every week through intramuscular way and endoscopic submucosal dissection (ESD) of NET.
After 14 days our patient had an easy walk and the feelings of fatigue, weakness and numbness in her limbs were mostly relieved. In the 3 months’ follow up, her anemia was corrected (RBC: 4.91∗10ˆ12/L, HGB: 143 g/L, MCV: 88.2 fL). AST, VitB12, bilirubin and Hcy returned to the normal levels. The T2-weighted hyperintensity of spinal MRI almost disappeared (Figure ).
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pmc-6356213-1
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An 84-year-old woman presented to the hospital emergency department after a sudden onset of right hemiparesis, right-sided numbness and aphasia upon waking; her NIHSS was 13. MRI evaluation showed diffusion restriction in the left frontal lobe (A) with a corresponding area of decreased blood flow (B) on perfusion weighted imaging (PWI). Gradient echo (GRE) imaging was negative for hemorrhage. There was minimal change on the FLAIR sequence in the region of the stroke (C) despite bilateral WMH involving the deep white matter and periventricular regions. Although she had an unknown time of onset, she met the criteria for the MR WITNESS [] clinical trial and received IV tPA. Two hours after treatment, her NIHSS had improved to 11, and by 24 h, her NIHSS was down to 5. At discharge, her NIHSS was 3; at 30 days after the stroke, it was 2, and by 90 days, it was 0 where it remained out to one year. Her modified Rankin score was a 1 at 90 days and at 6 months, however, it had increased to a 2 by the one-year time point.
shows the same region of the brain at three time points: column 1 is 24 h after the stroke, column 2 is 30 days after the stroke, and column 3 is 90 days after the stroke. Row A shows the BBB permeability heatmap superimposed on the FLAIR scan at each time point. The amount of BBB disruption is color coded: green 0.5% to 0.8%, yellow 0.8% to 0.9%, orange 0.9% to 1%, and red >1%. The acute lesion appears bright on FLAIR at the 24-h time point in part due to gadolinium leakage into the CSF from an earlier MRI scan, however, the acute stroke does not demonstrate increased BBB disruption on the BBPI heat map at the time of the 24-h scan. This is consistent with our previous study demonstrating the reversal of BBB disruption after reperfusion in acute ischemic lesions [] which may then be followed by a biphasic re-opening of the BBB within the ischemic lesion. However, in this case, areas remote from the acute stroke, particularly in the contralateral hemisphere, demonstrate widespread BBB disruption that is most severe at the 24-h time point but persists out to the 90-day time point.
Row B shows the same BBB disruption from row A, except that now it is outlined instead of color coded. This allows visualization of the WMH on the FLAIR image with the regions of BBB disruption superimposed. Row C shows the boxed region from row B, contralateral to the side of the infarct, magnified for examination of the WMH. Note the pattern of BBB disruption, often involving the normal appearing white matter (NAWM) adjacent to the WMH. BBB disruption involving the NAWM persists out to 90 days.
The purpose of is to identify the overlap between the location of BBB disruption 1 month after stroke and the locations of new WMH 1 year after stroke. The WMH regions of interest (ROI) from 1 month and 1 year are compared to identify regions of new WMH which have developed over time. This ROI is then superimposed on the 1-month FLAIR image showing the area of NAWM that will progress to WMH. This ROI is then compared with the area of BBB disruption measured at 1 month. The lower images in outline the region of BBB disruption from the 1-month time point. This region of BBB disruption is then compared with the region of NAWM that will later progress to WMH to identify the overlap between these regions. The resulting image outlines the area of NAWM at the 1-month time point which has BBB disruption and will progress to WMH over the subsequent year.
shows the increase in white matter hyperintensities (WMH) between the 3-month time point and the 1-year time point for three regions in the brain. The 3-month scan was chosen in this case so that changes in the area of the acute stroke can be examined for chronic changes. The first row is the FLAIR MRI from the 3-month time point. The second row is the FLAIR MRI from the 1-year time point. The third row is a composite image from the two time points with the increase in WMH represented in green. Red areas represent regions of decreased WMH and appear to mostly represent atrophy. The most dramatic region of WMH progression is seen in the areas immediately adjacent to the region of the acute stroke. However, the bottom-right panel shows the progression of WMH in the contralateral hemisphere in the centrum semiovale as well. Thus, this figure demonstrates clear WMH progression over a 9-month period during a chronic phase of the cerebrovascular disease.
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pmc-6357208-1
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A 76-year-old-woman was evaluated because of general fatigue, loss of appetite, and jaundice. Laboratory test showed an elevation of total bilirubin (7.7 mg/dL) and hepatobiliary enzyme. An endoscopy showed a 10-mm tumor in Vater’s papilla (Fig. A), and endoscopic retrograde biliary drainage (ERBD) was placed for obstructive jaundice. After that, total bilirubin was decreased to 1.9 mg/dL. The pathological diagnosis of endoscopic biopsy of the tumor was a papillary adenocarcinoma. Endoscopic ultrasonography (EUS) revealed that this tumor invaded pancreatic parenchyma (Fig. B). A contrast-enhanced computed tomography (CT) revealed a hypovascular mass at Vater’s papilla (Fig. A, B). No evidence of distant metastasis was identified. Carcinoembryonic antigen (CEA) and carbohydrate antigen 19-9 (CA19-9) were not elevated (CEA 1.9 ng/ml, CA19-9 31.5 U/ml). Thus, the patient was preoperatively diagnosed with an adenocarcinoma of Vater’s papilla and underwent an operation.
A subtotal stomach-preserving pancreaticoduodenectomy (SSPPD) with D2 lymph node dissection was performed. The pancreas was soft and non-fibrotic. The operation time was 6 h 18 min, and the intraoperative blood loss was 417 g.
The patient developed postoperative pancreatic fistula (grade B) in accordance with the International Study Group for Pancreatic Fistula definition []. Appropriate persistent drainage was performed, and the patient recovered immediately and was discharged on the 30th postoperative day.
Macroscopically, a 2.0 × 1.4 cm elastic hard tumor was found at Vater’s papilla (Fig. A). The microscopic examination of the specimen showed that spindle cells that constructed sarcomatous tissue proliferated with intricate infiltration (Fig. B) and growth of tubular adenocarcinoma (Fig. C). Two components existed across the transition zone (Fig. D). Approximately 30% of the tumor was sarcoma component, and the remainder was carcinoma component. The tumor directly infiltrated into peripancreatic fatty tissue, pancreatic parenchyma, and a lymph node. Finally, pathological diagnosis was carcinosarcoma of Vater’s papilla. Resection margins were pathologically negative; thus, curative resection was achieved.
The patient received adjuvant chemotherapy using gemcitabine 1 month after the operation. It was continued without any obvious adverse events; however, an enhanced CT revealed multiple liver metastases at 3 months after the operation. The chemotherapy was changed to gemcitabine plus cisplatin. However, enhanced CT revealed the rapid progression of the metastasis at 6 months after the operation. The patient died at 7 months after the operation due to the continuous tumor progression.
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pmc-6357210-1
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A 78-year-old man with hematochezia was diagnosed with two synchronous rectal cancers 7 years prior to presentation. One tumor was located at the rectosigmoid junction (stage T3N1M0, well-differentiated tubular adenocarcinoma), and the second was in the distal rectum, (stage T3N1M0, well-differentiated tubular adenocarcinoma). The patient had a 10-year history of diabetes mellitus and hypertension treated with medication. No family history of CRC was noted. Physical examination was unremarkable. Preoperative CRT followed by a very low anterior resection with diverting ileostomy was performed. Preoperative CRT included 5 days of 5-FU/leucovorin infusion followed by radiation therapy delivered using the four-field technique with photon radiation administered five times per week with a daily fraction of 1.8 Gy, for a total of 40 Gy. The final pathological diagnosis revealed that the rectosigmoid cancer was ypT3N1M0, and the lower rectal cancer was ypT0N0M0 (no residual cancer, pathological complete response). The postoperative course was uneventful and the ileostomy was reversed 8 months later, after completion of postoperative adjuvant chemotherapy, which included 6 months of oral 5-FU/leucovorin.
During follow-up, multiple lymph node metastases in the para-aortic and supraclavicular regions were found 20 months after resection and chemotherapy was given, including 14 days of oral capecitabine, 1 day of oxizaliplatin (CAPOX), and bevacizumab. Bevacizumab (7.5 mg/kg) was administered intravenously on day 1 for 1 cycle. CAPOX+bevacizumab was continued for 3 years for a total of 33 cycles of CAPOX (combined with 23 cycles of bevacizumab). Progression of lymph node metastases was noted and the chemotherapy regimen was changed. Second-line chemotherapy included 14 days of oral capecitabine, 1 day of irinotecan (XELIRI), and bevacizumab. Three cycles of XELIRI+bevacizumab (7.5 mg/kg) were continued over 3 months. One month after the last course of chemotherapy (5 years after resection), the patient presented with pneumaturia and fecaluria and described three previous episodes of hematochezia. The patient had no other symptoms associated with altered bowel habits. Laboratory tests demonstrated signs of mild inflammation (white blood cell count 11,200 μL, C-reactive protein 2.2 mg/dL). A small amount of extraluminal air between the prostate and rectum, adjacent to the anastomotic site, was observed on computed tomography (CT) scan (Fig. ), which was not noted on the previous CT scans. Routine surveillance colonoscopy was not performed after the initial surgery as care was focused on management of multiple lymph node metastases. The latest colonoscopy revealed ulceration and fistula formation at the anastomotic site (Fig. ). Contrast radiography was consistent with a fistula at the anastomotic site (Fig. ). Only granulation tissue, including inflammatory changes, was seen in biopsies taken from the anastomotic site. Colonoscopy, contrast radiography, and cystoscopy demonstrated no recurrent tumor or obvious anastomotic-urethral fistula at the anastomosis, but an anastomotic-urethral fistula was suspected based on symptoms including pneumaturia and fecaluria. Transverse colostomy was performed and has not been reversed because of the continued need for chemotherapy. At this time, the patient has no symptoms associated with a leak and CT scan shows no air around the anastomotic site.
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pmc-6357285-1
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A 37-year-old man with 15-day history of headache was admitted to the Department of Neurosurgery of Wuhan Union Hospital in June 2015. The headache was characterized as a distending pain associated with nausea. Despite a family history of migraine, the patient had not experienced a headache previously. He did not smoke or drink alcohol. His general physical and neurological examination results were normal. Routine laboratory test results, electrocardiogram, and chest X-ray were unremarkable. Brain magnetic resonance imaging (MRI) with gadolinium enhancement showed a large mass with regular borders and surrounding edema, exerting a mass effect on the adjacent frontal lobe (Figure ). Surgery was suggested and successfully performed, with complete resection, as indicated in the postoperative MRI (Figure ). Surgical biopsy confirmed the diagnosis of a transitional meningioma, WHO grade I. After the operation, the patient's headache was completely relieved.
He remained free of all forms of headaches until November 2017, when he developed a strictly left-side periorbital pain in the absence any specific triggering factor. The attacks were accompanied by ipsilateral rhinorrhea, lacrimation, eyelid edema, ptosis, and bilateral photophobia. The attacks lasted 3–4 h and occurred daily, in the afternoon. During the attacks, the patient was restless and between the attacks, the patient was pain-free. He was treated in the first instance with carbamazepine, however, yielding poor efficacy; tramadol only provided partial relief.
The patient visited our department 4 months after the onset of attacks. A few days prior to the visit (in December 2017), he had undergone a brain MRI, which showed a softening lesion, glial hyperplasia, and localized thickening and enhancement of the dura (Figure ) in the left frontal-temporal lobe. Considering the possibility of tumor recurrence, an MRI of the eyes and a whole-body PET scan were performed; however, the results were confusing. In March 2018, the MRI of the eyes demonstrated a stronger signal of the cerebral dura (Figure ), a large area of the softening lesion, and glial hyperplasia (Figures ) in the left frontal-temporal lobe, as observed previously. However, no signs of tumor were indicated on the 18F-FDG PET scan performed in February 2018 (Figures ). We consulted the Department of Neurosurgery and Radiology. The consensus was that, as the enhanced volume of the fronto-temporal lobe had not increased significantly, recurrence of the tumor could not be determined at that stage, and thus regular follow-up was recommended. Based on a comprehensive analysis of the patient's clinical manifestations and the International Classification of Headache Disorders (ICHD-3 beta) (), we made a diagnosis of cluster-like headache.
During the attacks, oxygen therapy showed no effect. Treatment with methylprednisolone sodium succinate injection (40 mg per day) provided complete remission on the day after medication administration. The treatment was then shifted to oral administration of methylprednisolone tablets (40 mg per day), tapering to 8 mg per day. During this period, no cluster-like headache attacks occurred. However, the cortisone therapy did not result in permanent remission, as the cluster-like headache attacks recurred when methylprednisolone tablets were tapered to 4 mg per day. Thus, the dose of 8 mg per day was temporarily maintained. After 1 month of steroid therapy, acne appeared. Considering the side effects of steroids, the dosage was adjusted to one tablet every other day. During the 4 months of follow-up, there was no recurrence of headache.
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pmc-6357366-1
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A 68-year-old man was referred to our department with infraclavicular lymphadenopathy and an endobronchial tumor that was incidentally discovered on computed tomography (CT) at another hospital. An excision of the left infraclavicular nodes was performed, but no malignant findings were observed. A chest CT revealed a 10-mm endobronchial mass that was clearly visible as a high-attenuation area of contrast enhancement (Fig. a, b). An 18-fluorodeoxyglucose positron emission tomographic whole-body scan revealed no significant uptake in the lesion. Bronchoscopy revealed a submucosal tumor on the anterior wall of the entrance to the right bronchus intermedius that was constricting the airway lumen (Fig. a, b). The tumor surface was covered with numerous engorged blood vessels, and the middle and inferior pulmonary lobes were intact. Although a biopsy of the mass was performed, no definitive diagnosis was achieved.
A posterolateral thoracotomy was performed through the fifth intercostal space under general anesthesia. The bronchus intermedius was dissected, and the membranous portion was opened to expose the lumen. The distal end of the tumor was transected first followed by the proximal end, providing adequate tumor-free margins. Because the tumor had clearly defined borders, the resection line was determined by macroscopically securing the margin from the tumor. Subsequently, the tumor and bronchus intermedius were removed en bloc. The tumor measured 13 × 6 mm in size and was hemispherical in morphology. Examination of frozen tumor sections suggested angioma with no malignant findings. The presence of tumor-free margins at both the proximal and distal ends of the bronchus was also confirmed by examination of frozen sections. The excised segment of the bronchus measured 1 cm in length; thus, the bronchus was reconstructed by end-to-end anastomosis using 3–0 PDS (polydioxanone) sutures without excessive tension. The anastomosis was then wrapped in a pedicled intercostal muscle flap to isolate it from the pulmonary artery.
Histologically, a mixture of proliferating blood vessels and adipocytes were observed within the bronchus wall (Fig. a–c). Therefore, the pathological diagnosis was angiolipoma. The patient experienced no postoperative complications and was discharged on postoperative day 15. Two years and 7 months postsurgery, the patient has experienced no recurrence.
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pmc-6357368-1
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The first patient was a 15-year-old male. After 1 year of repeated gross hematuria, he was admitted to the Department of Nephrology in our hospital on November, 2002. Urinary system ultrasound, intravenous pyelography, contrast enhancement and plain CT scans of the kidney, and renal biopsy were performed. However a cause of the patient’s hematuria could not be identified. Later, the patient was transferred to pediatric surgery and cystourethroscopy was performed. The results showed urethral mucosa edema, mass and miliary bulging, and bleeding of the membranous urethra. The urethral mucosa biopsy was also performed, and the pathological report displayed submucosal vascular dilatation of the urethra which is consistent with UCH (Fig. ). Two weeks after the cystourethroscopy, pingyangmycin was injected under the cystoscope in the outpatient department of urology. In the operation, 4 mg of pingyangmycin was injected into the bulge on the urethral membrane. The urethral catheter was retained and removed after 3 days. At the follow-ups 1 year, 12 years, and 15 years after treatment, gross hematuria did not recur, and micturition and erectile function were normal.
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pmc-6357368-2
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The second patient was a 49-year-old male with repeated painless gross hematuria and discontinuous urethral bleeding after penile erection for more than 20 years, which had been aggravated for 4 months. He was admitted to the Department of Urology of our hospital on April 29, 2013. The patient had also been misdiagnosed in a local hospital over the course of 20 years with seminal vesitis, urethritis, or prostatitis. No obvious improvement was observed with treatment. Cystoscopy performed in local hospitals, revealed no obvious abnormalities. After artificial erection by tightening the root of the penis and injecting saline into the corpus cavernosum, a small amount of bloody liquid could be detected in the urethra. The penis MR showed an abnormal signal on the right side of the urethra cavernous body at the front of the penis. The range was about 1.1 × 2.4 cm. The distal portion closed to the urethral meatus. The proximal portion was at a distance of 2.4 cm from urethral meatus and invaded the right side of the glans (Fig. , and ). After artificial erection of the penis, urethroscopy examination showed that there was a 0.3 cm fissure located in the 11 o’clock urethral mucosa 2 cm away from the urethral meatus. The fissure bled and the bleeding was aggravated when the penis was squeezed (Additional file : Video S1). Pingyangmycin was injected into the lumen and basal side of the tumor under the urethroscope. We took a biopsy from the small incision on the ventral side of the penis that separated the hemangioma and continued to inject pingyangmycin. The total dose of pingyangmycin was 8 mg. The catheter inserted after injection was removed 3 days after the operation. The postoperative pathological report reported a diagnosis of UCH. At 1 year and 5 years of follow-ups, no bleeding occurred during or after penile erection, no gross hematuria recurred, and penile erection and voiding were normal.
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pmc-6357409-1
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An 88-year-old white woman with a history of vascular dementia and idiopathic pulmonary fibrosis (IPF) presented with a 4.5 cm left-sided level III anterior cervical lymph node (Fig. a and b). Prior to her onset of dementia and IPF, she was otherwise healthy. Her family history was not relevant for hematologic malignancies or cancer. She denied tobacco smoking. In addition to her neck mass, she developed night sweats and 1.8 kg (4 pound) weight loss. No lymph nodes were detected in her right supraclavicular, axillary, and inguinal regions. Auscultation of her lung bases revealed dry crackles. Hepatomegaly and splenomegaly were not observed. A computed tomography (CT)-guided core needle biopsy was done on September 16, 2014. A core, rather than excisional biopsy was considered given her severe lung disease and inability to tolerate general anesthesia. Tissue examination showed B cells of follicular origin, admixed with high-grade large cells (Fig. a and b). Flow cytometry showed clonal B cell population (36% of total cellularity) positive for CD10, CD19, and CD20 (Fig. b). Cells were Kappa-restricted associated with < 1% natural killer (NK) cells. Examination of her tumor biopsy showed a CD4:CD8 ratio of 9:1 without aberrant T cell antigen expression. EBV in situ hybridization was not performed. Positron emission tomography (PET-CT) revealed single uptake above the clavicle on the left side with standardized uptake value (SUV) of 4.9 confirming stage 1B disease. Her International Prognostic Index (IPI) was 2 points: low intermediate risk group; age > 60 years, lactate dehydrogenase (LDH) of 599, stage 1, Eastern Cooperative Oncology Group (ECOG) of 0. In addition, given her history of dry coughing and shortness of breath, a chest CT was obtained, which revealed honeycombing, bronchial wall thickening, and subpleural ground glass opacities suggesting interstitial pneumonitis (Fig. a and b). Her symptoms were controlled with inhaled β-agonists without administration of orally administered or systemic steroids. Her peripheral blood flow cytometry detected increased proportion of cells with cytotoxic potential including human leukocyte antigen-antigen D related (HLA-DR) + T cells (57%, normal 9–36%; absolute count of 884/mm3, normal 177–692/mm3) and double positive (DP) CD4/CD8 T cells (4%, normal 0–2%; absolute count of 62 mm3, normal 0–50 mm3) (Fig. a and b). After discussion of treatment options, she opted for best supportive care. Three months later, during a routine follow-up examination, it was noted that the lymph node had completely regressed. Ultrasonographic (Fig. c) and clinical remission were documented in October 2016, 25 months after her initial CT-guided biopsy. She died with progressive respiratory insufficiency attributed to IPF without evidence of lymphoma in December 2016.
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pmc-6357415-1
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A 24-year old male was seen at the Emergency Department of our hospital with acute scrotal swelling on the left side, which started 5 days earlier. The symptoms started during a trip to Japan, where the patient had multiple severe sneezes while walking outside. On examination, he had a large swelling of the left hemiscrotum. Except for a left sided varicocele (Fig. ), which was diagnosed 6 months earlier in our hospital, the patient had no medical history. Blood-results were negative. Colour Doppler-Ultrasonography (CDU) showed the known varicocele, a normal vascularized left testis, and a swelling of low echogenicity of 39x29mm without blood flow, suiting a scrotal bleeding (Fig. ). The hematoma was considered self-limiting, and spontaneous resorption was expected. However, after follow-up ultrasonography 2 months later, the swelling had increased in size (40x40mm) (Fig.). The patient was referred to an academic hospital. A CT-scan of the abdomen showed a prominent vena spermatica on the left, without suspicion of an arteriovenous malformation. A 3D replica of the CT-scan, illustrating the size of the hematoma (Fig. ).
Three months post-event, the hematoma even further increased in size to 50x37x30mm. Eventually, the patient underwent a microscopic inguinal varicocelectomy. After, the hematoma showed signs of reabsorption, decreasing in size to 38x24x21mm 4 months; 20x16x11mm 6 months; and to no residual hematoma eventually, 15 months post-event. The left testis itself did not differ in size at all follow-up points.
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pmc-6357463-1
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A 61-year-old male patient with bilateral adenomegaly in the neck showed in his peripheral blood a leukocyte count of 49.1 X109/L, with 90% of lymphocytes. Immunophenotyped cells were positive for CD20, CD5, and CD23 surface antigens; therefore, after being diagnosed with CLL (Rai IV), the hematologist administered chemotherapy consisting of cyclophosphamide, adriamycin, vincristine, and prednisone, but the patient’s disease was refractory to such treatment. Next, the patient was started on fludarabicin and rituximab but an adverse reaction was later reported. Another cycle of treatment with cyclophosphamide and prednisone was administered with no response since leukocytosis remained during the 3 years that preceded his demise.
Peripheral blood lymphocytes obtained before therapy were cultured in RPMI-1640 medium and stimulated with a mixture of phorbol-12-myristate-13-acetate plus pokeweed mitogen at concentrations previously described [, ]. After 72 h of incubation, metaphase cells were obtained from cell cultures harvested by standard methods. Chromosomes were stained following the Giemsa-trypsin banding protocol and analyzed under the microscope. Results were interpreted following the ISCN (2016) recommendations [].
Three fluorescent in situ hybridization (FISH) analyses were performed separately. In a first analysis, we used a mixture of the dual color 13q14.3-deletion probe (Cytocell, LPH 006), which covers the DLEU1 and DLEU2 genes (labeled in red) and the 13q subtelomere sequence (labeled in green), plus the RB1 (13q14) probe (labeled in green; Kreatech, KI-40001). According to information published by the providers, the red labeled probe targeted to the DLEU genes is conformed of two separated fragments of 215 and 93 kb, which together span a sequence from chr13:49962705 to 50,671,242 (hg38; ~ 700 kb). As for the RB1 (13q14) probe, it covers a continuous sequence approximately from chr13:48062708 to 48,801,516 (hg38; ~ 740 kb). A second FISH examination was performed using the MDM2 Amplification probe (Cytocell, LPS 016). We also performed a third FISH study with the dual color P53/ATM probe (Cytocell, LPH 052). In all these FISH studies, cells were counterstained with 4′,6-diamino-2-phenylindole.
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pmc-6357502-1
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A 24-year-old male with a history of hypertension, hypothyroidism, morbid obesity, and significant smoking history (both cigarette and marijuana) presented to urgent care with a two-week history of progressive leg edema and hemoptysis. He was found to have nephrotic range proteinuria and acute renal failure with a serum creatinine of 346 μmol/L (no comparison values available). Chest x-rays showed progressive worsening bilateral patchy opacities (Fig. ) and chest CT showed mild scattered patchy ground-glass parenchymal opacities bilaterally. Serological tests for anti-GBM (ALBIA), anti-neutrophilic cytoplasmic antibodies (ANCA, MPO, PR3), anti-nuclear antibodies, extractable nuclear antigens (including anti-dsDNA), anti-C1q, hepatitis B and C, HIV, and Streptolysin O were all negative. His C3, C4, and Kappa/Lambda free light chain ratio were within normal range.
Renal biopsy showed necrotizing and crescentic glomerulonephritis involving 70% of the glomeruli, diffuse endocapillary and mesangial hypercellularity and focal GBM duplication (Fig. ). Direct IF microscopy showed strong linear IgG staining along the glomerular basement membranes and focal staining along tubular basement membranes. Linear staining was also observed for both light chains (lambda>kappa), with weaker IgA staining and no IgM, C3 or C1q staining (Fig. ). Staining for IgG subtypes was positive for IgG2 and IgG4 in a linear pattern (IgG4 > IgG2) (Fig. ). No immune complex-type dense deposits type nor “powdery” linear densities along the GBM were seen by electron microscopy (EM) (not shown).
Based on the combination of strong linear IgG staining in the glomeruli and circulating anti-GBM antibodies within normal range, a diagnosis of atypical anti-GBM disease was made, and the patient was started on high dose prednisone and IV cyclophosphamide. His hemoptysis was felt secondary to anti-GBM disease rather than hypervolemia from severe GN though he did not undergo bronchoscopy during that time. During workup, he was found to have elevated serum IgM lambda monoclonal protein at 1.5 g/L. On discharge, a chest x-ray showed resolution of the patchy infiltrates, and he was prescribed continuation of corticosteroids, IV cyclophosphamide 1.2 g biweekly [] (received 4 treatments total), and trimethoprim/sulfamethoxazole for Pneumocystis jiroveci prophylaxis. Renal function also improved and at discharge his creatinine was 305 μmol/L, following a peak of 454 μmol/L.
The patient returned to Urgent Care approximately 1 month later with fever, massive hemoptysis, and anuria. Serum creatinine was 1065 μmol/L, and he was urgently started on dialysis. Chest X-ray showed worsening of bilateral patchy opacities (Fig. ). Empirical treatment for presumed relapse of his disease was initiated, with 500 mg IV methylprednisolone and plasma exchange with fresh frozen plasma as replacement fluid. He also received empiric ceftriaxone and azithromycin and continued on prednisone 60 mg daily, along with cyclophosphamide, and trimethoprim/sulfamethoxazole. He underwent bronchoscopy, and his bronchoalveolar lavage did not initially reveal an infectious agent, although the respiratory panel was positive for parainfluenza. CMV was not assayed at the time of initial bronchoscopy. Of note, his BAL was persistently bloody but did not meet criteria for diffuse alveolar hemorrhage (his hemosiderin-laden macrophage count was < 20%).
The patient was managed for 5 days as above but continued to have massive hemoptysis and worsening pulmonary infiltrates radiographically. As a result, rituximab therapy was initiated for possible cyclophosphamide-resistant atypical-anti-GBM disease. The patient’s clinical condition continued to decline as he required intubation and underwent repeat bronchoscopy. He was empirically treated with piperacillin/tazobactam and linezolid despite all of his blood/alveolar lavage bacterial cultures being negative. His echocardiogram and enhanced chest CT were unremarkable for other etiologies contributing to severe pulmonary hemorrhage. He was subsequently started on empiric ganciclovir for possible cytomegalovirus (CMV) infection. Results of the second bronchoscopy confirmed CMV and a viral load of > 106 IU/mL from his BAL fluid. Cytology showed inclusion bodies consistent with CMV infection. His serum showed a CMV load of > 2 million IU/mL. He never had CMV checked prior to this. After initiation of ganciclovir, the patient experienced clinical improvement with resolution of his hemoptysis and chest x-ray abnormalities. His renal function however never recovered, and he remained dialysis dependent.
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pmc-6357504-1
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A previously healthy 14-year-old girl presented with 3 days of fatigue, 2 days of fever and behavior changes including becoming abnormally talkative, and 1 day of limp. She had black spots on her palms and soles, and black discoloration of the left second finger and left fifth toe. She came to our emergency department because of worsening pain in the left toe and because she was developing a confused mental status. Initial vital signs were: temperature 39.8 °C, heart rate 130 beats/min, blood pressure 100/71 mmHg, respiratory rate 18 breaths/min, and 98% oxygen saturation on room air. She was oriented but oddly garrulous. There were no hemorrhages in the palpebral conjunctiva but the uvula and posterior pharynx were covered with petechial hemorrhages suggesting streptococcal pharyngitis. No cardiac murmurs were auscultated. Her left second finger and left fifth toe were black, she had petechiae on the right palm, and the dorsum of the left foot was erythematous, warm, and swollen. Initial laboratory evaluation showed signs of disseminated intravascular coagulation (DIC) with an elevated white blood cell count and an elevated C-reactive protein. We thought that the skin and soft tissue infection of the finger and toe were causing bacteremia and DIC. GAS was suspected as the causative microorganism, and she was started on intravenous (IV) ampicillin/sulbactam and clindamycin. Two of three blood cultures grew Streptococcus pyogenes (T6 M6, emm6.104). Based on susceptibility testing results, the ampicillin/sulbactam was switched to just ampicillin and she was continued on clindamycin. Her mental status had almost cleared by the end of the first hospital day and she was wholly awake and alert by hospital day 3. A new systolic ejection murmur was heard, and echocardiography showed vegetation on the mitral valve with mitral regurgitation (MR) (Fig. ). She also had findings of infarctions in the deep left temporal cortex, spleen, and kidney (Fig. ). Two weeks later, she developed an erythematous rash over her entire body. A drug rash was suspected and she was switched from ampicillin and clindamycin to cefotaxime. There was no sign of heart failure, however, the MR worsened. She was started on a diuretic and an angiotensin-converting enzyme inhibitor. The left fifth toe became necrotic and was amputated on hospital day 45. Subsequently, she remained stable and was discharged. Two months later, she underwent mitral valve repair. She has remained on angiotensin-converting enzyme inhibitor therapy, and one year later, shows no signs of neurological sequelae or heart failure.
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pmc-6357504-2
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A 17-month old boy presented to a neighboring municipal hospital secondary to fever. He had a past history of spontaneous closure of a ventricular septal defect (VSD) at 5 months of age with residual mild MR. He presented with a 2-day history of fever and a 1-day history of irritability. He was referred to our hospital because of elevated white blood cell count and elevated C-reactive protein. Initial vital signs were: temperature 40.6 °C, systolic blood pressure 100 mmHg, heart rate 157 beats/min, respiratory rate 60 breaths/min, and 97% oxygen saturation in room air. He had no conjunctival petechiae, no splinter hemorrhages of the nails, and no rash. No heart murmurs were auscultated. Initial laboratory evaluation was significant for a white blood cell count of 14,300 cells/ml and a C-reactive protein level of 30.89 mg/dL. Blood cultures were drawn at both the municipal hospital and our hospital 12 h apart, and he was empirically started on IV ampicillin and cefotaxime for possible bacteremia. Two blood cultures grew S. pyogenes (T4 M4, emm4.0). On hospital day 5, echocardiography showed vegetation on a right-sided membranous septal aneurysm (MSA) (Fig. ) and he was diagnosed with IE. Based on susceptibility testing results, the cefotaxime was stopped and ampicillin only was continued. While receiving antibiotic therapy, he did not develop any complications such as pulmonary hypertension or pulmonary embolism. IV ampicillin was continued for 4 additional weeks after follow-up blood cultures were determined to be negative, and he was ultimately discharged. The patient has shown no signs of heart failure during the 2-year follow-up period.
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pmc-6357678-1
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The proband is a 12-months-old boy with typical facial dysmorphism, hearing defect and bony abnormality (Table ). He was born after a normal pregnancy and delivered with birth weight of 2.9 kg (10th percentile) and birth length of 45 cm (3rd percentile) at 38 weeks, compared with the WHO Child Growth Standards in 2006. The facial appearance presents bulging forehead, prominent ears, widely spaced eyes, down slanted palpebral fissures, short nose with broad columella, thick alae nasi and septum, thick and everted underlip (Figures ). The deciduous teeth were erupted at 8 months old (not delayed) (Figure ). The hands are short, fleshy, and with remarkably hyperextensible fingers that taper from wide to narrow with small terminal phalanges and nails (Figures , ). But there was no deformity of his foramen magnum or spine column (Figures ). The weight at 12 months is 8.2 kg and height is 68.2 cm (<-3.17 z score, WHO). The bone metabolism and IGF-1α is disturbance (Vit D 45.2 nmol/L, IGF-1α < 25 ng/mL). He started sitting alone at 9 months and couldn't stand unaided until 12 months of age. At 12 months of age, his intelligence quotient (IQ) was 56 according to the Gesell Developmental Schedules. He had difficulty remaining seated or concentrating during task completion. His auditory threshold of auditory brainstem response (ABR) is >85 db and is diagnosed as a hearing disorder. The magnetic resonance imaging (MRI) showed the dilation of bilateral ventricles and less cerebral white matter (Figure ).
For genetic analysis, blood samples were obtained from the individual. The mother had given informed consent for her children. This research was approved by the bioethics committee for human gene analysis at the Zhejiang University.
Next Generation Sequencing (NGS) analysis was performed using Agilent Human Genome panel (Agilent Technologies, Inc, Santa Clara, CA, USA). A c.2185 C > T at chrX20173554 was detected in the proband, which can cause p.Arg729Trp mutation and RSK2 instability (Figure ). Subsequent analysis did not detect the same point mutation in other family members including mother, brother (). This indicated that the micromutation is new but not inherited from the mother.
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pmc-6358343-1
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An 11-month-old female child presented to our department with a right asymptomatic, painless labial swelling, incipient expressed over the last 3 months and progressively grown in size over the last 2 days. Ultrasonography examination revealed a 4.8 cm × 3.1 cm subcutaneous anechoic, fluid filled mass, located near right superficial inguinal ring without visible connection with the peritoneal cavity (Fig. ).
Physical examination revealed a soft, cystic, non-compressible, and non-fluctuant labial mass measuring approximately 5 cm × 3 cm (Fig. A). Overlying stretched skin was normal. Inguino-labial mass showed a positive transillumination. The remaining physical examination was unremarkable. Defecation and urination were also normal at all times including the last 2 days. Past medical history has shown that the patient has undergone surgical duodenoduodenostomy in the newborn period due to duodenal atresia type 3 and ventriculoperitoneostomy due to hydrocephalus associated with Arnold-Chiari malformation type 1.
Under general anesthesia, the child underwent surgical exploration through a right skin crease incision. The cystic lesion was sent to histopathologic evaluation and tissue dissection; it was confirmed to be a non-communicated hydrocele of canal of Nuck (Fig. B). Isolation of the cyst from the round ligament and hydrocelectomy were carried out. The wound was closed in layers using absorbable sutures. Postoperative recovery was uneventful and the child was discharged the next day.
Histopathological examination of the specimen confirmed the clinical diagnosis (Fig. A and B). The child was doing well at 1-year follow-up with no swelling or recurrence on the operated side.
The study was conducted in accordance with the ethical standards laid down in 1964 Declaration of Helsinki. The case report was shared with the local ethical committee; it is however the policy of the committee not to review case reports. Informed written consent was obtained from the patient for publication of this case report and accompanying images.
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