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pmc-6199146-1
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A 63-year-old woman, with a history of Boerhaave's oesophageal rupture status post-oesophagectomy with extra-thoracic transverse colon interposition 40 years ago, presented with two weeks of malaise and was found to have a non-mechanical primary bowel dysmotility. She was dehydrated and resuscitated in the emergency room with intravenous (IV) normal saline which was later switched to IV 5% dextrose normal saline. Over the next week, she became progressively more lethargic and was thought to be delirious with “sundowning”. On neurologic examination, she was drowsy but arousable to verbal stimuli with bilateral vertical and horizontal ophthalmoplegia. An MRI of the brain showed symmetrical hyperintensity in the bilateral medial thalami and dorsal midbrain, including periaqueductal grey matter, as shown in Figure . She was started on high-dose IV thiamine (500 mg three times daily for two days followed by 250 mg daily for five days followed by oral treatment with 100 mg daily) along with other B-complex vitamins and magnesium. Repeat brain MRI one week later showed significantly reduced hyperintensities in the thalamus and the dorsal midbrain region. Unfortunately, her hospital course was complicated by Pseudomonas and Klebsiella pneumonia requiring intubation, along with sepsis and progressive multiple organ failure. She passed away after the goal of her care was changed to comfort measures only.
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pmc-6199147-1
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A 32-year-old obese woman gravida five, para 3104, with a history of preterm contractions and preeclampsia, presented to the emergency department with persistent pelvic pain and a fever of 103.1 F after delivering a baby girl four days prior, via a normal spontaneous vaginal delivery at term. Per the patient report, she had been experiencing fever and chills since the delivery. On physical exam, she was found to have a normal amount of foul-smelling lochia. The labs showed an elevated white blood cell (WBC) count and anemia. A pelvic x-ray showed diastases of the pubic symphysis measuring 3.75 cm (Figure ). A magnetic resonance imaging (MRI) scan of the pelvis and lumbar spine failed to show a pelvic abscess or hematoma.
The patient was admitted for further evaluation. Over the course of the day, the patient became progressively tachycardic with a fever of 102.5 F, and sepsis secondary to the endometritis was suspected. The patient was started on intravenous (IV) ampicillin and clindamycin. The following day, blood cultures were drawn showing Streptococcus pyogenes (group A) and Proteus mirabilis. A urine culture grew 80,000 cfu/mL of Proteus mirabilis. The Proteus mirabilis was susceptible to ampicillin, cefazolin, ceftriaxone, ciprofloxacin, gentamicin, levofloxacin, and trimethoprim/sulfamethoxazole and resistant to nitrofurantoin. Susceptibility for GAS was not done, as routine susceptibility for beta-hemolytic streptococcus was not routinely performed at the institution due to its usual susceptibility to the penicillin family.
On hospital day three, the patient was noted to be febrile, tachypneic, tachycardic, and hypoxic. She was also experiencing a productive cough. Expiratory wheezes were heard in the upper lung fields bilaterally. A chest computed tomography (CT) showed bilateral patchy opacities (Figure ). Hospital-acquired pneumonia was diagnosed, based on the patient's clinical picture and CT scan. Seven days of empirical therapy for hospital-acquired pneumonia was started, which included a one-time dose of azithromycin and a course of IV piperacillin/tazobactam, vancomycin, and levofloxacin. Repeat blood cultures that same day showed no growth. On hospital day eight, vancomycin was discontinued as there was no evidence of methicillin-resistant Staphylococcus aureus (MRSA) in the blood or nares culture. The antibiotics were switched to two grams of IV ceftriaxone once a day to treat the GAS endometritis with bacteremia. Oral metronidazole, 500 mg every eight hours, was also added for empirical anaerobic coverage. Ceftriaxone was chosen over penicillin, given the convenience of once a day dosing with ceftriaxone as compared to penicillin. She improved clinically, became asymptomatic, and was discharged to a skilled nursing care facility. At her three-week outpatient follow-up visit, the patient was doing well. She had completed a four-week course of IV ceftriaxone and oral metronidazole, and an additional course of amoxicillin/clavulanate was initiated for two more weeks.
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pmc-6199376-1
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A 51-year-old Caucasian man presented to his primary care provider with left “facial pressure” and pain radiating to his nose. After two failed courses of antibiotics and steroids, CT scan 6 months after initial presentation revealed a left maxillary mass, and biopsy showed undifferentiated pleomorphic sarcoma (UPS) (stage cT2bN0M0, FNCLCC grade 3/3). PET/CT demonstrated a 5.1 × 4.7 cm infiltrative mass centered at the left maxillary sinus and nasal cavity with bony destruction extending into the left orbit, ethmoid sinuses, and inferotemporal fossa and no evidence of metastatic disease.
Neoadjuvant chemoradiation with doxorubicin and 30Gy radiation therapy (RT) in 10 fractions to the left maxillary sinus (modified Eilber regimen) was followed by radical resection 9 months after initial presentation (). Pathology showed a 3 cm tumor with multiple positive margins, which prompted the addition of post-operative boost RT to 26Gy in 13 fractions to the tumor bed. Follow-up PET/CT immediately after boost RT showed fluorodeoxyglucose (FDG) avid lesions in both the tumor bed (SUVmax = 5.1) and left submandibular neck (SUVmax = 32.1). Subsequent ultrasound-guided biopsy of a left submandibular neck mass was positive for high-grade sarcoma.
After four cycles of gemcitabine and docetaxel, five of five lymph nodes were negative for disease on surgical lymph node dissection of the neck. After completing two additional cycles of chemotherapy, surveillance imaging showed no evidence of disease for 23 months off of treatment until CT scan detected multiple new, large lymph nodes in the left level V area of the neck and supraclavicular region as well as a left apical extrapleural mass. Biopsy of the left neck mass showed UPS. Foundation One CDx (Foundation Medicine, Cambridge, MA) immunohistochemistry profiling showed low-positive PD-1 and PD-L1 expression (1+ staining intensity, 1–24% staining distribution) on tumor infiltrating lymphocytes (TILs), and high-positive PD-L1 expression (2+ staining intensity, >25% staining distribution) on tumor cells (Supplemental Figure ).
His disease continued to progress on PET/CT despite two additional cycles of gemcitabine plus docetaxel, as well as three cycles of ifosfamide (Figure ). Significant symptomatic progression of disease in the neck prompted treatment with palliative radiation therapy combined with biweekly intravenous nivolumab. The first dose of nivolumab was given 11 days prior to 30Gy RT to the left neck mass delivered in 10 fractions. Follow-up CTs demonstrated a striking decrease in tumor burden at the left lateral neck mass, supraclavicular lymph nodes, left chest wall and left axilla over the next 13 months of nivolumab treatment (Figure ). Additionally, serum lactate dehydrogenase (LDH), a prognostic factor and surrogate marker of disease burden, decreased from 1,000 U/L at the beginning of this therapy to 215 U/L () (Figure ).
CT scan of the neck 13 months after initiating palliative RT plus nivolumab showed an increase in the size of a right tracheoesophageal groove lymph node at the margin of the radiation field and redemonstration of an enlarged node left clavicular node. The patient was enrolled on a phase II randomized clinical trial combining stereotactic body radiation therapy (SBRT) with CBI (NCT02843165), and randomized to the SBRT plus CBI treatment arm. He was treated with dual agent CBI including ipilimumab and nivolumab, receiving 24Gy SBRT in 3 fractions to the right tracheoesophageal groove lymph node 1 week after the first of four intravenous ipilimumab infusions (1 mg/kg), followed by three more ipilimumab treatments Q3 weeks.
Five months after starting SBRT plus dual agent CBI, CT imaging of the neck showed resolution of the irradiated right tracheoesophageal groove lymph node (Figure ) as well as the non-irradiated left clavicular node enlargement and no new or progressive lymphadenopathy or other masses. He had previously developed hypothyroidism on nivolumab alone, but treatment with SBRT plus dual agent CBI was well-tolerated without any high-grade toxicities. Assessment by Response Evaluation Criteria in Solid Tumors (RECIST) 1.1 5 months after initiation of ipilimumab plus nivolumab plus SBRT determined complete response. The patient remains in complete response at most recent imaging over 2 years since his initial treatment with radiation combined with nivolumab and he continues to do well clinically (Figure ).
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pmc-6199550-1
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A 22-year-old male patient with a past medical history of nephrotic syndrome due to membranous nephropathy, which was diagnosed by renal biopsy 1 year ago, was admitted to the emergency room and transferred to our cardiac care unit with the complaint of chest pain along with dizziness, headache, and radiating pain to his left arm for 4 hours. The patient took no drugs in the past 6 months. On clinical examination, his pain was reported to be 5/10 according to the visual analogue pain scale, he was afebrile, with a maximum temperature of 36.6°C, and had ortho-arteriotony, with a blood pressure of 128/85 mmHg, and no tachycardia or bradycardia (pulse rate of 66 beats per minute). The patient’s face was bloated, but there was no jugular venous distention, muffled breath sounds, rhonchi or moist rale, the third or fourth heart sound gallop, pericardial rub, peritoneal irritation, hepatosplenomegaly, or leg oedema. Electrocardiogram on admission showed that ST elevated to 0.1–0.2 mv in II, III, aVF, and V4–V6 ().
Initial abnormal serum laboratory results were as follows: troponin I, 3.499 ug/L; myoglobin, 1200 ug/L; creatine kinase-MB, 69 U/L; creatine kinase, 678 U/L; lactate dehydrogenase, 343 U/L; cholesterol, 6.43 mmol/L; low-density lipoprotein cholesterol, 5.02 mmol/L; glucose, 2.44 mmol/L; high-density lipoprotein cholesterol, 0.76 mmol/L; prothrombin time, 10.3 s; activated partial thromboplastin time, 24.5 seconds; serum total protein, 39.1 g/L; albumin, 20.2 g/L; and 24-hour urine protein, 11.054 g/24 hours.
According to the results, our preliminary diagnosis was acute inferior myocardial infarction (Killip grade I) combined with nephrotic syndrome, and we performed a percutaneous intervention procedure, which was followed by 300 mg aspirin and 300 mg clopidogrel orally immediately. During the operation, we saw that the left coronary artery had thickened and the left main artery, left circumflex artery, and right coronary artery were normal. However, there was a great deal of thrombi blocking the anterior and middle anterior descending branch, resulting in 95% stricture () and leading to bloodstream classification at thrombolysis and thrombin inhibition in myocardial infarction grade II. Next, we applied the thrombus suction technique and injected 13 ml of tirofiban to dissolve blood clots. Unfortunately, there was still medium thrombi remaining and 60% stricture in the re-examination of coronary arteriography (). The bloodstream classification at thrombolysis and thrombin inhibition in myocardial infarction grade III. Because the anterior descending branch dilatation made it unsuitable for stent implantation, we had to stop further operation. Next, we gave him a therapeutic regimen of low-molecular-weight heparin for 7 days, 100 mg aspirin per day, and 90 mg ticagrelor twice a day to fight platelet aggregation, and 40 mg atorvastatin per night to remedy hyperlipidaemia persistently.
One day after the operation, his postoperative electrocardiogram showed that the elevated ST segments in II, III, aVF, and V4–V6 were down to baseline, and an inverted T wave began to appear in V2–V6 (). An echocardiography performed 3 days after admission showed a left ventricular ejection fraction of 48.2%, enlarged bilateral atrium, dilated left ventricle, segmental weak pulse of left ventricular wall, mild regurgitation of bicuspid valve or of tricuspid valve, and the estranged left ventricular cells (). Laboratory tests on the same day showed a higher troponin I of 31.833 ug/L, lower myoglobin of 27.9 ug/L, creatine kinase-MB of 21 U/L, creatine kinase of 74 U/L, and lactate dehydrogenase of 489 U/L. After 4 days, the serum laboratory results showed the following results: troponin I, 6.912 ug/L; myoglobin, 29.5 ug/L; creatine kinase-MB, 17 U/L; creatine kinase, 36 U/L; lactate dehydrogenase, 330 U/L; cholesterol, 2.86 mmol/L; low-density lipoprotein cholesterol, 1.63 mmol/L; triglyceride, 2.28 mmol/L; and high-density lipoprotein cholesterol, 0.57 mmol/L. All the related indexes were decreased. On the 14th day after the operation, the patient had a third examination of coronary arteriography, which revealed that there was barely any blockage caused by thrombi in the anterior descending branch and no stricture in the coronary artery (). The patient was discharged on the 17th day, as his vitals were stable and symptoms disappeared.
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pmc-6199716-1
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A 31-month-old infant black girl was presented to our imaging department with a recurrent history of difficulty in breathing, bluish skin discoloration, easy fatigability, failure to thrive, and on-and-off swelling of the lower limbs and face. The patient’s past medical history revealed a recurrent history of cough and fevers. She was delivered at term weighing 3.5 kg, her parents’ first-born and only child. There were no perinatal complications. The patient had an Apgar score of 9/10 and breastfed immediately. Immediately after birth, her mother started noticing that the patient was frequently inactive and weak despite breastfeeding well. The patient also started developing bluish skin discoloration a few weeks after birth, which was accompanied by easy fatigability. Milestones were delayed. She started sitting unsupported at 12 months and began crawling at 20 months. Currently, she can walk for short distances owing to the easy fatigability previously mentioned. She had several episodes of upper respiratory infections that were treated on an outpatient basis because they were not serious enough to necessitate hospital admission The patient had no family history of congenital heart disease or asthma. Her mother had no history of diabetes during the index pregnancy.
The patient’s physical examination revealed she was a girl who was small for her age, alert, and afebrile; had swollen lower limbs and puffy face; and was cyanotic with finger clubbing. Her pulse rate was 168 beats/min. Her blood pressure was 102/64 mmHg. Her respiratory examination revealed her respiratory rate was 28 breaths/min, oxygen saturation was 50%, and that she had fine bibasal crepitations. Her cardiac examination revealed S1 and S2 sounds. She demonstrated systolic murmur grade 3 at the right upper sternal border. No thrill was present. A provisional diagnosis of dextrocardia with congenital cardiac disease was made.
Hematological tests revealed normal ranges of hemoglobin, leukocytes, and platelets. The results of the sickling test, rapid plasma reagin test for syphilis, and enzyme-linked immunosorbent assay for human immunodeficiency virus were negative. A chest x-ray revealed dextroposition of the cardiac silhouette and trilobed lungs (Fig. ).
Echocardiography revealed dextroposition of the heart, complete atrioventricular canal, pulmonary atresia, and reverse patent ductus arteriosus (PDA). The child was further investigated with cardiac computed tomography (CT), which revealed multiple complex congenital anomalies. Cardiac CT confirmed dextroposition of the heart with a huge ostium primum atrial septal defect and membranous ventricular septal defect (Fig. ). Right atrial isomerism, bilateral trilobed lungs, and asplenia were seen. Both lower-lobe bronchi were severely hypoplastic (Fig. ). The liver was centrally located.
Ventricular switch was seen with a morphological right ventricle on the left side giving rise to the ascending aorta. Severely hypoplastic main pulmonary artery (MPA) was seen with no direct connection to the morphologic left ventricular outlet, consistent with pulmonary atresia (Fig. ). A left-sided PDA and multiple major aortopulmonary collateral arteries supplying the right lung were noted (Fig. ).
Abnormal venous drainage was seen, as shown by a double-sided superior vena cava (SVC) and right upper lobe anomalous venous return. The right-sided SVC emptied into the right-sided atrium, and the left-sided SVC emptied into left-sided atrium. Liver drainage was split with the intrahepatic inferior vena cava, which received tributaries from right and middle hepatic veins, draining into right-sided atrium. The left hepatic vein drained into left sided atrium (Fig. ).
A final imaging diagnosis of situs ambiguus with right isomerism, l-transposition of great vessels with atrioventricular discordance, ventriculoarterial discordance, dextrocardia, and multiple complex anomalies. The patient is currently on antifailure medications and prophylactic antibiotics. Owing to the complexity of the patient’s cardiac anomalies and unavailability of corrective pediatric cardiac surgical services in Tanzania, the patient was referred abroad for further management.
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pmc-6199719-1
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A 61 year-old Caucasian woman was referred to our colorectal clinic with an 18-year history of severe intermittent anal pain and constipation. She described experiencing intermittent anal spasms lasting around 15 minutes. These episodes were worse when sitting down for longer than 45 minutes or when lying in bed. The frequency of these anal spasms was increasing with time and occurring every hour at night at the time of presentation. Her constipation symptoms constituted experiencing difficulty in defecation and a sensation of incomplete evacuation. She had no response to amitriptyline or topical diltiazem. Her past medical history was unremarkable apart from four normal vaginal deliveries. Her sister had colorectal cancer diagnosed at the age of 49 and had previously been treated for an undiagnosed anal sphincter problem. There was no other relevant history of note.
She initially underwent a flexible sigmoidoscopy and magnetic resonance imaging (MRI) of her perineum. The endoscopy was reported as normal, whereas the MRI showed edema of the IAS. She subsequently had an endoanal ultrasound which confirmed that her IAS was abnormally thick and greater than 5 mm (Fig. ). Anal manometry revealed that although resting and squeeze pressures were within normal limits there were periods of a significant increase in anal resting pressure lasting longer than 2 minutes (Fig. ). Pressures during this period were in excess of 200 mmHg which settled spontaneously. These pressures were even higher than the maximum recorded squeeze pressure (Fig. ).
She had an examination of the anal canal under anesthetic which showed a very prominent sphincter complex. She also received Botox injections (Dysport™) at the 3 and 9 o’clock positions of the IAS which led to no subsequent resolution of her symptoms. She then underwent a lateral internal anal sphincterotomy by dividing half of the length (1 cm) of the IAS on the left lateral aspect. A biopsy of the IAS taken at the time of surgery was sent for histology which confirmed polyglucosan body myopathy of the IAS (Fig. ). At 3-month follow-up, she had complete resolution of her symptoms and has not contacted our department with any concerns for more than 1-year postoperatively.
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pmc-6199854-1
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A 63-year-old woman presented with a firm 7 cm multilobular tender mass located in the region of the right sternocleidomastoid directly above the clavicle. She had multiple comorbidities including renal failure, systemic lupus erythematosus, and protein S deficiency, a history of transient ischemic attack, deep vein thrombosis, and pulmonary embolism, and longstanding malnourishment requiring jejunostomy tube placement. The firm mass was identified 1 month after an unsuccessful attempt at placing a right internal jugular vein central line. When the central line was originally placed, she developed pain and swelling of her neck. The swelling was pulsatile at that time and enlarging. She was offered surgery as the standard of care for her condition which she refused because she did not want to undergo an invasive procedure and was aware of the risks involved with holding her anticoagulant medication. She had two thrombin injections in the mass since without success. She complained of right neck pain. She was not having any difficulty breathing and denied shortness of breath.
Soft tissue ultrasound after the two thrombin injections showed pseudoaneurysm and a 7 × 3.3 × 4 cm multilobular hematoma at the base of the right neck. CTA of the neck showed a corresponding heterogeneous mass. The pseudoaneurysm was seen measuring 1.5 cm at the internal margin of the hematoma. Serial imaging with CTA demonstrated enlargement over a 2-week interval ().
Angiogram showed blush of contrast coming off of the sternocleidomastoid branch of the superior thyroid artery identifying the location of the pseudoaneurysm (). This branch was embolized using glue. Repeat angiogram showed successful embolization and no contrast blush.
On follow-up one month later, the mass was no longer pulsatile but still present on physical exam. CTA of the neck showed a decrease in size from the previous CTA ().
On follow-up 8 months later, she was found to have a soft neck with no mass on physical exam.
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pmc-6199862-1
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A 76-year-old man was referred to our hospital in September 201X, because of right leg pain, lower back pain, and weight loss of 3 kg. Lumbar magnetic resonance imaging and computed tomography (CT) suggested the presence of lumbar spinal canal stenosis and a sacral tumor (). Laboratory testing revealed a markedly elevated serum IgG level (5,436 mg/dL, normal: 800–1,800 mg/dL) and an elevated serum beta-2 microglobulin level (4.1 µg/mL, normal: 0–3 µg/mL), although there were no signs of anemia, renal dysfunction, or proteinuria. Serum immunofixation revealed IgGκ-type M-protein, with an estimated serum-free κ and λ chain ratio of 21.5 : 1 (). Microscopic examination and flow cytometric analysis of bone marrow aspirate revealed elevated numbers of CD138-positive abnormal plasma cells. Cytogenetic analysis of the bone marrow revealed 46XY, and the patient was diagnosed as having MM (R-ISS, stage II). Chimeric p190 BCR-ABL mRNA was not detected in the bone marrow sample at this point. The patient underwent two cycles of bortezomib plus dexamethasone and two cycles of cyclophosphamide, bortezomib, and dexamethasone (CBD) but did not respond to either treatment regimen. The treatment was switched to lenalidomide (25 mg/day) plus dexamethasone (20 mg/week; Ld therapy), and there was a marked response, with a substantial decrease in the M-protein and disappearance of the sacral tumor on CT. After 24 cycles of Ld therapy, the patient achieved a partial response based on the International Myeloma Working Group criteria.
In December 201X+2, the patient developed leukocytosis (white blood cell count: 35.8 × 109/L) and thrombocytopenia (platelet count: 3 × 109/L). Bone marrow biopsy and aspiration revealed hypercellularity with a marked increase in myeloid lineage cells but without an increase in blast cells (4%). Cytogenetic analysis revealed 46XY t(9; 22) (q34; q11.2) in 20 of 20 cells, and fluorescence in situ hybridization (FISH) analysis revealed that 99.5% of the cells were positive for BCR-ABL. Peripheral blood neutrophils were also positive for BCR-ABL (98.8%) (Figures –). Chimeric p190, but not p210, BCR-ABL mRNA was detected by using polymerase chain reaction (). The diagnosis was confirmed to be CML with p190 BCR-ABL in the accelerated phase (AP), which coexisted with MM (a maintained partial response). Dasatinib treatment (100 mg/day) was started immediately. The dose was subsequently decreased to 50 mg/day, due to the persistence of thrombocytopenia. In April 201X+3, a bone marrow examination indicated that the patient had achieved a second chronic phase, with 31% of this cells being positive for BCR-ABL upon FISH analysis, and that his peripheral blood count had normalized. However, 5 months later, FISH analysis revealed that 85.8% of his bone marrow cells were positive for BCR-ABL, and subsequently, his treatment was changed from dasatinib to bosutinib. This switch appeared to be ineffective, as no decrease in the BCR-ABL-positive bone marrow cells was detected after 2 months.
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pmc-6199865-1
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Patient 1. A male patient in his late 80s with arterial hypertension underwent repeat aortic valve replacement due to a paravalvular leak and cardiac decompensation. After surgery, he developed severe nosocomial pneumonia. Bronchial aspirates grew Pseudomonas aeruginosa and the empirical antibiotic treatment with meropenem was adjusted to ceftazidime. After moderate respiratory improvement, the patient’s condition deteriorated and he developed liver failure with jaundice and renal insufficiency. Twenty-three days after surgery, additional bronchial aspirates were sent for microbiological investigations and BCC was detected after 24 hours of incubation. Despite treatment with ceftazidime and tobramycin, the patient died from multi-organ failure.
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pmc-6199865-2
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Patient 2. A male patient in his late 70s underwent replacement of the ascending aorta and repeat aortic valve replacement due to valve degeneration and cardiac decompensation. Morganella morganii was found in several blood cultures and treated with piperacillin/tazobactam. On day 27 after surgery, BCC grew in respiratory specimens after 24 hours of incubation and the antibiotic treatment was changed to ceftazidime and tobramycin. The patient developed liver and renal failure, as well as haemodynamic instability necessitating vasopressor treatment. Despite escalation of the anti-infective treatment, the patient succumbed to septic multi-organ failure 9 days after detection of BCC.
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pmc-6199886-1
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A 48-year-old Caucasian male with a past medical history of hypertension, diverticulosis, bicuspid aortic valve with mild insufficiency, and daily alcohol use presented to the emergency department (ED) with 1 week of hiccups associated with a few episodes of nonbloody and nonbilious emesis with no chest pain, abdominal pain, or dyspnea. He was not taking any medications. He had no known prior history of palpitations, syncope or seizures. There was no known significant family medical history. His triage vital signs were: temperature of 36.2°C; blood pressure of 140/90 mmHg; heart rate of 114; respiratory rate of 18; and an oxygen saturation of 96% on room air. Physical examination showed dry mucus membranes, regular heart rate without murmur, soft abdomen, and active hiccups. The patient was given a 1-liter bolus of intravenous normal saline and chlorpromazine (Thorazine) 25 mg by mouth. Approximately 15-20 minutes after receiving chlorpromazine, the patient was found in cardiac arrest, and advanced cardiac life support (ACLS) interventions were initiated. The initial rhythm was ventricular fibrillation, and he received a total of four defibrillations at 200 joules each. Torsades de pointes was also observed, and he received 2 grams of intravenous magnesium.
The post-arrest ECGs () were notable for normal sinus rhythm with a rate of 98-99, and a QTc of 495-521 ms (normal QTc is <440 ms in men). Immediate post-return of spontaneous circulation (ROSC) arterial blood gas was significant for metabolic acidosis with a pH of 7.22 (range, 7.38–7.42), a lactate of 14 mmol/L (range, 0.60–2.40), and an ionized calcium of 1.12 mmol/L (range, 1.10–1.33). A comprehensive metabolic panel was remarkable for a glucose of 206 mg/dL (range, 74–99), potassium of 3.0 mmol/L (range, 3.5–5.3), bicarbonate of 16 mmol/L (range, 21–32), an anion gap of 30 mmol/L (range, 10–20), aspartate transaminase (AST) of 466 U/L (range, 10–37), alanine transaminase of 374 U/L (range, 10–65), and serum total bilirubin of 1.9 mg/dL (range, 0.0–1.2). A complete blood count was only significant for a platelet count of 101 x 10E9/L (range, 150–450). Blood and urine drug screens, thyroid stimulating hormone level, magnesium level, and a cardiac troponin, were all found to be normal.
Chest X-ray, computed tomography of the head, chest, abdomen, and pelvis were unremarkable. Post-ROSC echocardiogram showed diffuse areas of hypokinesis and an ejection fraction (EF) of 20-25% which improved a week later to no wall motion abnormalities and an EF of 60-65%. Cardiac troponin peaked at 0.48 ng/mL (range, 0.00–0.06), and a cardiac catheterization showed no coronary artery disease. Multiple post-arrest ECGs consistently demonstrated a prolonged QTc with measurements up to 611 ms despite optimization of electrolytes (). An old ECG from a past clinic visit showed a QTc of 581 ms. Electrophysiology diagnosed him with congenital Long QT Syndrome for which he received a dual chamber implantable cardioverter defibrillator (ICD). The patient was extubated on day 6, transferred from the intensive care unit to the medical floor on day 12, and discharged from the hospital on day 19. It was not clear what precipitated the patient's hiccups.
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pmc-6200441-1
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An 82-year-old man presented with the new onset of rapidly enlarging skin lesions on his right leg. He has a history of actinic keratosis (periodically treated with cryotherapy using liquid nitrogen) and four non-melanoma skin cancers (three basal cell carcinomas and one squamous cell carcinoma that were excised without recurrence).
Cutaneous examination showed three nontender erythematous nodules that presented in a sporotrichoid pattern, with peripheral scaling and central crust, on his right leg (Figures , ). The proximal lesion was 5 x 5 mm and located on his distal thigh, the distal lesion was 1.5 x 1.5 cm and located on his right pretibial area, and the middle lesion was 1.0 x 1.0 cm and located lateral and inferior to his knee. A biopsy for pathology was performed from all of the lesions; the distal lesion was also bisected and tissue was sent for bacterial, fungal, and mycobacterial cultures.
Microscopic examination of all three lesions showed similar pathologic findings. There was parakeratosis filling a cup-shaped depression in the epithelium. The keratinocytes in the hyperplastic epidermis had glossy-appearing cytoplasm and nuclear atypia. All of the cultures were negative for infectious organisms.
Correlation of the clinical presentation, pathology findings, and laboratory results established a diagnosis of eruptive keratoacanthomas in a sporotrichoid distribution. Evaluation of the groin, axillae, and neck were negative for palpable adenopathy. The lesional sites were excised to ensure complete removal of the tumors. There is no evidence of recurrence at his follow-up examination six months later.
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pmc-6200443-1
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This is the case of a 101-year-old Caucasian female with a past medical history of hypertension, melanoma, chronic hearing loss, and macular degeneration who was hospitalized for hypertensive urgency. Her blood pressure on arrival was 205/94, without physical exam or laboratory evidence of end organ damage. On the evening of her second hospital day, she reported frightening auditory and visual hallucinations, and neurology was consulted to evaluate her. She described hearing the sound of drums beating and seeing worms in a container of candy. On further questioning, she admitted to experiencing both visual and auditory hallucinations for several weeks preceding her hospitalization, with the latter arising first. Her visual hallucinations began, and her auditory hallucinations worsened, following an episode of intense anxiety that she described as “body shakes” lasting ten minutes and resolving spontaneously. She had never experienced anything similar in the past, and she denied any associated tongue biting, urinary or fecal incontinence, and confusion. She attributed her anxiety to a pain in her right eye, which she was concerned could be cancerous. She had been recently treated for blepharitis and had no abnormalities on follow-up with her ophthalmologist. The “visions” she described lasted seconds to minutes and usually occurred in the right peripheral field of vision, although she was unsure whether they were present in one or both eyes. They initially consisted of “grey blobs” and “creatures” that stood beside her while she was watching television in the evenings and later became more human-like. Looking directly at these figures or turning on the light made them disappear. While in the hospital, she saw a young girl in a ballerina costume standing beside her and several men dressed in uniform “on the walls and hanging from the ceiling”. She also described “snow” or “dust” coming from the walls. When asked about hallucinations in the past, she said that several years ago she would occasionally see faces just before falling asleep, especially in the setting of emotional “excitement”. She believed this might have been related to the fact that she was a serious artist and frequently painted human faces. She denied hallucinations upon waking, daytime sleepiness, and falling asleep unexpectedly. She denied recent changes in sleep and said she had always slept well at night. Her auditory hallucinations came in the form of music, usually well-recognized melodies such as "America the Beautiful" and "Ave Maria” sung by a man's voice or played on a piano. She heard them in both ears and they were present constantly throughout the day. She occasionally heard voices speaking that were unintelligible except for one time when she heard “she’s a teacher, she teaches English”. She and her family both noticed that the hallucinations worsen in the setting of anxiety or other emotional disturbance. She had no known history of psychiatric illness and had never seen a psychiatrist. On review of systems, she admitted to right eye pain, gradual hearing and vision loss, anxiety, and sadness. She denied headache, dizziness, vertigo, weakness, numbness, tingling, diplopia, photophobia, tinnitus, fever, fatigue, weight change, and memory change.
General examination revealed a mildly anxious elderly woman. On neurological exam, the patient was alert and fully oriented. She scored 29/30 on a Mini Mental State Examination, and 25/30 on the Montreal Cognitive Assessment, with 0/5 points given for delayed recall, although she recalled three with category cue and the remaining two with multiple choice cue. A PHQ-9 gave a score of 2/27. Examination of the cranial nerves revealed full visual fields, decreased visual acuity, and diminished hearing bilaterally with right worse than left on finger rub test. Cranial nerves were otherwise intact. She walked with a walker, and her gait unsupported was unsteady and cautious. The remainder of the neurological exam was unremarkable.
Computed tomography (CT) scan of the brain without contrast was notable for atrophy in the parietotemporal areas bilaterally, with no evidence of acute infarct, hemorrhage, or mass effect (Figure ).
She was started on quetiapine at 12.5 mg every evening and discharged once her blood pressure was controlled. At her first follow-up appointment two weeks later, she reported partial improvement in her symptoms. She also noted additional types of hallucinations, including a checkered pattern on the walls and floor and a human arm holding a salt shaker. The arm appeared in her periphery while she was eating breakfast but disappeared when she looked directly at it. She also described intermittent headaches, which she believed to be a manifestation of anxiety, that begin in various locations and become diffuse. Her family reported that she tended to be anxious at baseline with increased anxiety surrounding these sensory experiences. She and her family reported a noticeable decrease in her sense of disturbance upon receiving reassurance that her hallucinations are benign and do not represent mental illness, as well as learning methods to minimize them.
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pmc-6200444-1
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A 50-year-old Chinese lady presented to our emergency department with right-sided chest pain. She had a history of De Quervain’s tenosynovitis of the left hand post-release seven years earlier and was a non-smoker.
She complained of right-sided chest pain on deep inspiration. She had just sought treatment at a traditional medicine practitioner for a session of acupuncture and cupping (‘ba guan’) to the back to treat her symptoms of long-standing neck pain and intermittent numbness and tingling of both hands. This pain occurred while she was sitting down after receiving treatment. She denies any trauma to her chest. She did have some difficulty taking in breaths due to the chest pain. There was no fever, cough, runny nose or sore throat.
Physical examination revealed that she was afebrile, had a blood pressure of 99/63 mmHg, heart rate of 96 beats per minute, respiratory rate of 22 per minute and an oxygen saturation of 100% on room air. She was alert, comfortable and conversant in full sentences. Her heart sounds were dual, with no clicks, rubs or murmurs. Breath sounds were slightly decreased on the right but otherwise no crepitations were heard. Inspection of her back is as shown in Figure , with multiple cupping and needling marks. Her abdomen was soft and non-tender. She had supple calves and no pedal edema on examination.
Initial investigations revealed a normal complete blood count and metabolic panel. Electrocardiogram showed normal sinus rhythm with no acute ST segment or T wave changes. The chest radiograph on admission is as shown in Figure , with a right-sided pneumothorax with an apical-cupola distance of 3.6 cm.
Subsequently, the patient was put on a non-rebreather mask with a FiO2 of 100% and a 12 French chest tube was inserted via the Seldinger’s technique into the right chest wall in the emergency department. The patient was admitted to the Respiratory service with radiographic resolution of the pneumothorax within four days and was discharged when the apex-cupola distance of the pneumothorax was 4 mm. A repeat chest radiograph a week later in the follow-up clinic showed complete resolution of the pneumothorax (Figure ).
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pmc-6200469-1
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A 29-year-old man was admitted to the hospital for chest pain, decreased exercise capacity, and hemoptysis. The otherwise healthy patient had developed chest pain in September 2015, with a dull pain in the left shoulder and back and paroxysmal cough. Computed tomography pulmonary angiography (CTPA) revealed a left inferior lobe pulmonary embolism (PE). The patient was prescribed rivaroxaban 20 mg qd, which resolved the pain symptoms.
One month later, he experienced a symptom recurrence and developed shortness of breath after some activities. Laboratory results revealed abnormally high titers of lupus anticoagulant (2.90 with a reference range of ≤1.2), anticardiolipin antibodies (>120 IgG U/mL with a reference range of < 12 IgG U/mL), and anti-beta-2-glycoprotein I (188 RU/mL with a reference range of < 20 RU/mL), indicative of higher thrombosis risk of primary antiphospholipid syndrome (PAPS). Ultrasonic cardiography (UCG) revealed an enlarged right ventricle and atrium, while his pulmonary artery (PA) systolic pressure was 98 mm Hg with an ejection fraction of 63%. He was diagnosed with CTEPH and treatment with hydroxychloroquine (HCQ) 200 mg bid and sildenafil 20 mg tid relieved the symptoms.
Three months later, the patient returned to the hospital because of hemoptysis. A computed tomography scan of the chest showed multiple ill-defined and patchy ground-glass opacities and nodules. Laboratory results showed an NT-proBNP level of 4244 pg/mL.
The patient did not exhibit a rash or experience joint pain, photosensitization, or dryness of the mouth or eyes throughout the disease duration. He had no history of hypertension, diabetes mellitus, hypercholesterolaemia, surgery, malignancy, or other thrombosis risk factors. He had smoked 15 cigarettes per day for many years but had quit 8 months earlier.
During his hospitalization, the anticoagulant therapy was suspended and he was given a continuous intravenous drip of pituitrin. Two weeks later, the hemoptysis was under control. However, CTPA demonstrated multiple PEs, complete obstruction of the left PA, and recurrent thrombosis of the right lung (Fig. ). The patient's thrombosis was initially treated with low-molecular-weight heparin at 4000 IU qd by hypodermic injection, but he again experienced hemoptysis with ∼ 200 to 300 mL of blood. Bronchial arteriography showed a pulmonary bronchial shunt of the left lung. The hemoptysis was finally successfully treated by bronchial artery embolization. The anticoagulation treatment was continued under close surveillance, and the hemoptysis and dyspnoea did not recur.
Three months later, the patient underwent PTE (Fig. ). The mean pulmonary arterial pressure (mPAP) was 30 mm Hg determined by right heart catheterization before PTE, and postoperative mPAP was 17 mm Hg. No complications were observed for the patient. After the surgery, the patient reported a markedly increased exercise capacity. Although the patient retained positive aPL titers, UCG revealed that the PA systolic pressure and cardiac structure returned to normal. Life-long warfarin and HCQ 200 mg bid were used to maintain the curative effect. The patient remained well at the 6-month follow-up.
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pmc-6200547-1
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A 65-year-old woman under tramadol was admitted in our institution because of fever and confusion after a 10 days course of ceftriaxone for an Escherichia coli-induced pyelonephritis. She was diagnosed with an acute renal injury, which had favored tramadol adverse effects since confusion receded after tramadol withdrawal. Regarding the acute renal injury, serum creatinine (SCr) was 182 μmol/L (eGFRCKD-EPI 25 mL/min/1.73 m2), the urinary protein-to-creatinine ratio was 0.43 g/mmol (with a profile suggesting nonglomerular proteinuria), and urine microscopy revealed leukocyturia with Enterococcus faecalis. Imaging studies revealed medullary sponge kidneys without an obstructive cause for renal failure and bilateral nephromegaly (right kidney 165 mm and left kidney 155 mm). A renal biopsy was performed, revealing interstitial nephritis composed of macrophages with an abundant eosinophilic cytoplasm (von Hansemann cells) and Michaelis–Gutmann bodies in keeping with renal malakoplakia (Fig. A). The patient was given prolonged antibiotic therapy with cotrimoxazole. In addition, this patient with chronic kidney disease had 25-OH vitamin D insufficiency (56 nmol/L), for which she was prescribed oral cholecalciferol (100,000 IU per month) in spite of normal serum calcium (2.21 mmol/L) and parathyroid hormone (PTH) levels (38 ng/L). At the one-month follow-up after renal biopsy, her renal function had improved (SCr 137 μmol/L), as well as her 25-OH vitamin D serum levels and calcemia (67 nmol/L and 2.49 mmol/L respectively).
Two months later, she was admitted to our unit for dehydration and hypercalcemia (3.64 mmol/L) with normal 25-OH vitamin D (113.1 nmol/L) and PTH (15 ng/L) levels and high 1,25-dihydroxyvitamin D levels (336 pmol/L), suggesting ectopic 25-hydroxyvitamin D3 1-alpha-hydroxylase activity. Extensive investigations, comprising 18fluoro-deoxy-glucose positron emission tomography, bone marrow biopsy, thoracic computed tomography scanning, sputum examination for tuberculosis and a second renal biopsy, did not reveal a superimposed granulomatous disease. We suspected the malakoplakia cells to be responsible for the abnormal 25-hydroxyvitamin D3 1-alpha-hydroxylase activity, and we performed immunohistochemistry for 25-hydroxyvitamin D3 1-alpha-hydroxylase in slides from the renal biopsies (Fig. B). The test revealed ectopic expression of this enzyme by the infiltrating macrophages, whereas the infiltrating cells of a nonhypercalcemic sarcoidosis patient did not, although the tubular cells of this same patient did (data not shown). Cholecalciferol was stopped, the patient was rehydrated with intravenous physiological saline, and prednisone was initiated to decrease the enzyme activity. She was discharged with normal serum calcium (2.25 mmol/L). Six months later, serum calcium was 2.37 mmol/L, 25-hydroxyvitamin D was 99 nmol/L, 1,25-dihydroxyvitamin D was 100 pmol/L, and PTH was 147 ng/L (Fig. ). Her renal function had stabilized at 202 μmol/L (eGFRCKD-EPI 22 mL/min/1.73 m2). Unfortunately, the patient died 3 months later from cardiac failure caused by atrial fibrillation with normal calcium level.
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pmc-6200967-1
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A 39-years-old man was compulsory admitted to our inpatient psychiatric unit because of an episode with mixed mood features and psychotic symptoms, with it being the third episode in his personal history of Bipolar Disorder with peripartum onset following the birth of his child.
He had no history of prior psychoactive substance use or encephalitis. His past medical history was free of any chronic medical disorders. Dysfunctional personality traits were described in the patient since his early adult life, such as rigidity, stubbornness, suspiciousness, hostility, and being argumentative in his interpersonal relations, indicative of premorbid paranoid personality disorder, according to DSM-5 criteria, as well as narcissistic personality disorder traits, namely lack of empathy, exploitative behavior toward his relationships, and excessive need to be admired.
He had no family psychiatric history. His mother was described as the dominant family figure with his father being withdrawn without evidence of suffering from any mood or psychotic disorder.
The patient had his first episode, being a depressive episode with onset during his wife's postpartum period, for which he received venlafaxine up to 300 mg per day which he discontinued 1 month after its initiation.
One year after his initial depressive episode, he relapsed with a manic episode, the second in his personal history, characterized by irritability, dysphoric mood, distractibility, aggressiveness, grandiosity, psychomotor agitation, increase in goal-directed activity, mood lability, decreased need of sleep, recurrent suicidal ideation, feelings of despair, and diminished pleasure in most of his activities. A mixture of mood-congruent and mood-incongruent psychotic symptoms was also present, consisting mostly of persecutory ideas, ideas of reference, grandiose ideas involving his 15 months old son, as well as regression and feelings of jealousy toward the child. The theme of his delusional ideas related to his son led the patient to inappropriate (i.e., he insisted being present during his wife's breastfeeding sessions while he demanded that she caress his hair as he leaned on her breasts near to the breastfeeding child) and dangerous behaviors toward the infant, such as bathing him in ice cold water to the point of hypothermia rationalizing the incident as an effort to “toughen up” his child. Because of this abusive and potentially dangerous behavior, compulsory psychiatric evaluation and subsequent hospitalization were ordered.
Six hundred micrograms of carbamazepine was introduced daily and the sequential add-on of olanzapine up to 20 mg per day yielded full symptomatic remission. Olanzapine was subsequently lowered to 10 mg/day and the patient was discharged with a diagnosis of Bipolar Disorder I and Paranoid Personality Disorder, returning to the premorbid level of relational and personal functioning for the following 6 months.
Six months after his first hospitalization, the patient discontinued medication and relapsed with the episode discussed, presenting with mixed mood features which resulted in a second compulsory hospitalization.
The episode was characterized by behavioral problems, aggravation of his paranoid ideation, dysphoric and irritable mood, observed distractibility, insomnia, psychomotor agitation, feelings of despair and helplessness, involvement in quarrels and aggressive behavior, withdrawal from intimate relationships, and a waste of money and assets. The physical and neurological examination, as well as the imaging and laboratory testing, did not reveal any further pathological findings since his first hospitalization.
Physical and neurological examination revealed no pathological findings while electroencephalography, thyroid, and liver function tests, electrolytes, urine and creatinine, vitamin B12 and folic acid levels, and whole blood count were all within normal levels. HIV, HBV, HCV were all negative. Chest Ro and ECG revealed no pathological findings.
A brain computerized tomography (CT) scan revealed a small hypodense wedge-shaped lesion at the left posterior parietal lobe. The patient had a history of a traumatic brain injury at the age of 15 years. The imaging finding based on its localization and features and according to neurosurgical and neurological assessments yielded no evidence of any clinical relevance to his symptoms nor had any clinical significance.
The further neurocognitive assessment did not show any alterations in the premorbid level of cognitive function.
During the patient's last hospitalization, valproate slow release was initiated up to 1,500 mg/day with the consequent add-on of olanzapine up to 15 mg/day leading to the clinical remission and discharge of the patient who recovered to his premorbid level of functioning. He also received long-term psychodynamically-oriented psychotherapy, aiming at resolving paternity issues and related conflicts.
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pmc-6201338-1
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A 58-year-old male was referred to the rheumatology clinic for evaluation of arthralgia of the hands, wrists, and elbows. The patient's symptoms started six months prior and gradually worsened. The patient endorsed swelling of the hands and wrists, difficulty making fists, as well as morning stiffness lasting more than thirty minutes. He denied any constitutional symptoms such as fevers, chills, weight loss, decreased appetite, or night sweats. Review of systems was negative for alopecia, dry eyes, dry mouth, mouth sores, and skin rash. The patient also denied any recent travel, tick bites, or sick contacts. He never smoked and consumed alcohol on an occasional basis. The patient had a past medical history of osteoarthritis, with a surgical history significant for multiple procedures, including bilateral shoulder replacement for severe osteoarthritic changes, carpal tunnel repair of the right side, and laminectomy of the cervical and lumbar spine. Clinical exam revealed normal vitals, with benign head, eye, ear, nose, throat, cardiopulmonary, and abdominal exams. No lymphadenopathy or bruises were observed. Musculoskeletal exam revealed synovitis of the second through fifth metacarpophalangeal (MCP) and proximal interphalangeal regions bilaterally, with swelling and tenderness of the wrists with warmth to touch. In addition, there were 30-degree fixed contractions of the elbows. As per the patient, there was no history of psoriasis or nail changes, which was confirmed on physical exam as well. Laboratory data showed white blood cells of 12,000/mm, hemoglobin of 9.7 g/dl, hematocrit of 30.9%, C-reactive protein of 40 mg per liter (reference value <8), and erythrocyte sedimentation rate of 50 mm per hour (reference range 0 to 15). Laboratory testing of liver function, calcium, thyroid function, uric acid, renal function, and urinalysis was normal. Other normal or negative tests included antinuclear antibody, rheumatoid factor (RF), anti-cyclic citrullinated peptide (anti-CCP) antibodies, hepatitis B panel, hepatitis C antibody, QuantiFERON-TB Gold test, and angiotensin-converting enzyme. The patient was seen by an outside rheumatologist and treated initially with prednisone 20 mg/d and methotrexate, which was quickly escalated to a dose of 20 mg/weekly, with no response after three months of treatment. He was subsequently started on antitumor necrosis factor inhibitors.
Six months later, a follow-up visit showed persistent symptoms. Alternative anti-TNF inhibitors were tried with no improvement. Further workup included X-rays of the hands and wrists, which showed mild degenerative changes only. Chest X-ray revealed no pathology. The patient underwent MRI of the right hand, which showed synovial thickening of the radiocarpal joint and MCP joints with flexor tendon tenosynovitis, compatible with inflammatory arthropathy (). Further questioning of the patient revealed a family history of primary amyloidosis in his mother. Serum calcium, uric acid, and creatinine remained normal during the time of RA treatment. As a result, serum protein electrophoresis (SPEP) and urine protein electrophoresis were sent. Interestingly, SPEP was positive for M-band. The patient was then referred to hematology for further evaluation and underwent a bone marrow biopsy, which was positive for more than 40% plasma cells, as well as being Congo red stain negative, findings consistent with MM. FISH analysis was positive for monosomy 13 in 88% of the cells. The patient then started treatment for MM with bortezomib and dexamethasone. Six-month follow-up showed complete resolution of joint swelling, with significant improvement in pain of the hands, wrists, and elbows. His MM remained quiescent with chemotherapy, and the patient did not require bone marrow transplant.
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pmc-6201379-1
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An 80-year-old woman was admitted to the hospital after a fall. Her prior history was notable for coronary artery disease status post percutaneous intervention, poorly controlled type 2 diabetes mellitus, congestive heart failure, hypothyroidism, and atrial fibrillation. She had had multiple mechanical falls in the past with cervical spine and right-sided rib fractures. She had no recent hospitalization in the last 90 days and has been living at home prior to presentation. There was no history of exposure to the farm animals. During this hospitalization, she developed progressive dyspnea and hypoxia. Computed tomography (CT) revealed a bilateral pleural effusion, right more than left, with diffuse interlobular septal thickening. Note was also made of a diffuse, mosaic-like attenuation of the lung parenchyma, likely related to air trapping or obstructive small airway disease. There was no pleural enhancement, septations, or air noted within the pleural space (). She was noted to have new fracture of right posterior seventh, eighth, and ninth ribs. Laboratory data at admission revealed a white blood cell (WBC) count of 9.7 × 1000/µL (with 83% neutrophils and 1% eosinophils). Her hemoglobin was 11.09 g/dL, hematocrit 40.6%, platelets 143 × 1000/µL, total protein 5.1 g/dL, blood urea nitrogen (BUN) 31 mg/dL, serum creatinine 1.51 mg/dL, serum sodium (Na) 145 mEq/L, chloride (Cl) 102 mEq/L, potassium (K) 3.6 mEq/L, aspartate aminotransferase (AST) 24 IU/L, and lactate dehydrogenase (LDH) 472 IU/L. Serum procalcitonin levels were elevated to 0.59 ng/mL (normal 0.00 – 0.08 ng/mL) and hemoglobin A1C (HbA1C) to 8.9%.
Blood cultures prior to initiation of antimicrobial therapy returned negative. Sputum culture was negative for bacteria, including acid-fast bacilli. Transthoracic echocardiography was unremarkable.
The patient was treated initially with levofloxacin and ampicillin/sulbactam for a pneumonia and parapneumonic pleural effusion. Despite antimicrobial therapy, her respiratory status continued to deteriorate and within 48 hours of hospitalization required endotracheal intubation and ventilatory support. A thoracentesis yielded 250 ml of pus (WBCs more than 50,000 with 95% neutrophils, elevated protein 3.6 g/dL (normal 0.0–2.4 g/dL), LDH 13461 IU/L, glucose 6 mg/dL, and pH 6.94). A right-sided thoracostomy was performed to facilitate evacuation of the empyema using a 32 French (F) thoracostomy tube. Postprocedure chest X-ray confirmed the optimal placement of the tube (). Gram stains obtained from the pleural fluid collected under sterile conditions revealed gram-positive cocci, and her antibiotics were changed to vancomycin. The final isolate on culture and sensitivity was identified as S. simulans with heavy growth, susceptible to vancomycin and clindamycin only. A repeat CT scan of the chest revealed a persistent collection of right-sided empyema (). Ultrasound imaging of the pleural collections did not demonstrate any septations or loculated pockets on either side. Second attempt was made to evacuate the pleural collection with the use of intrapleural fibrinolytic tissue plasminogen activator (t-PA) with little success. The patient was considered a high-risk candidate for surgical intervention considering her frailty and other medical comorbidities. Her course was further complicated by circulatory shock requiring vasopressor supports and atrial fibrillation with rapid ventricular response requiring multiple rate-controlling drugs. After about 3 weeks of a tenuous course, her family elected to withdraw care and she passed away very shortly thereafter.
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pmc-6201484-1
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A 65-year-old Indian man presented with complaints of chest pain along with fatigue and generalized weakness for 2 months. His chest pain was constant, dull and boring in nature, and all over his chest. He had no history of trauma.
Contrast-enhanced computed tomography of his thorax showed erosion of left seventh and eighth rib with soft tissue mass involving the right side of his chest wall (Fig. ).
We conducted SPEP of our patient as he had multiple rib erosions. SPEP showed monoclonal band in gamma globulin region (Fig. ).
Bone marrow aspiration and biopsy were performed to detect multiple myeloma. However, instead, metastatic adenocarcinoma was revealed. No evidence of plasmacytosis was noted (Fig. ).
Computed tomography-guided fine-needle aspiration cytology (FNAC) from right-sided soft tissue mass of his chest wall showed metastatic adenocarcinoma (Fig. ). On further enquiry, he also gave history of urinary obstruction with lower urinary tract symptoms. Clinical suspicion of prostate carcinoma was considered in our old male patient with metastatic deposit in ribs. A per-rectal examination done by a urologist revealed enlarged hard prostate. Ultrasonography of our patient’s abdomen showed enlarged prostate. Serum prostate-specific antigen (PSA) was 124 ng/ml. A prostate biopsy was performed which demonstrated infiltrative adenocarcinoma with perineural invasion (Gleason’s score 4) (Fig. ).
A complete hemogram showed hemoglobin 9.4 gm/dl, white blood cell count (WBC) 9900, platelets 280,000, and erythrocyte sedimentation rate (ESR) 89. Blood biochemistry showed Na+ 130, K+ 4.4, Ca+ 1.16 mmol/L, protein 7 gm/dl, albumin 3.4 gm/dl, and globulin 3.6 gm/dl. Liver and renal function tests were within normal limits.
The valuable opinions of the urologist and the oncologist were taken into account. A plan for bilateral orchiectomy was decided upon to control growth of metastatic prostate carcinoma. However, our patient had a sudden cardiac arrest in the preceding week of the planned surgery. Efforts were made to resuscitate him but he died.
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pmc-6201510-1
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We report the case of a 91-year-old woman, who developed a generalized maculopapular, exfoliative skin eruption extending to the back, torso and extremities 15 days after initiation of CAPD with the use of icodextrin dialysate solutions. The medical history of the patient included stage IV congestive heart failure according to the New York Heart Association (NYHA) classification secondary to massive tricuspid valve failure and severe mitral valve deficiency, chronic atrial fibrillation (cAF) and stage 4 chronic kidney disease (CKD) with an estimated-glomerular-filtration-rate (eGFR) of 20 ml/min/1.73m2. A Tenckhoff peritoneal catheter was surgically inserted and after a prolonged hospitalization in the Peritoneal Dialysis Unit due to leakage from the peritoneal catheter exit site, the patient was discharged and her CAPD regimen included 2 icodextrin exchanges per day (2 X 1.0 L icodextrin). The daily dose of icodextrin relative to the patient’s body weight was 41.7 ml/kg and the peritoneal ultrafiltration volume was 600 ml/day. The patient was re-evaluated 7 days later and the CAPD regimen was intensified with the addition of another exchange during the day with dialysate glucose 1.36% (2 X 1.0 L icodextrin and 1 X 1.0 L glucose 1.36%). Peritoneal ultrafiltration achieved with the intensified regimen was approximately 800 ml/day.
On Day 15 after her initial exposure to icodextrin, the patient was admitted to the Peritoneal Dialysis Unit because of a widespread maculopapular and exfoliative skin rash of abrupt onset extending over her abdomen, arms, legs and back (Fig. and ). Her physical examination on admission revealed a normal body temperature (36.7 °C), blood pressure 105/60 mmHg, pulse rate 70 bpm, oxygen saturation 95% in the room air and absence of abnormal clinical signs from the chest auscultation and palpation of the abdomen. The peritoneal effluent was macroscopically clear and the white blood cell (WBC) count in the fluid was 35 cells/mm3, indicating absence of peritonitis. As shown in Table , standard laboratory tests revealed a normal WBC count with absence of eosinophilia (WBC: 8630 cells/μL; Neutrophils: 84%; Lymphocytes: 7%; Eosinophils: 1.1%), stable renal function without significant electrolyte disturbances (serum urea: 141 mg/dl, serum creatinine: 2.19 mg/dl, serum potassium: 3.6 mEq/L, serum sodium: 135 mEq/L), whereas inflammatory biomarkers remained within the normal range (c-reactive-protein: 0.8 mg/dl, normal range: 0.1–0.8 mg/dl; erythrocyte sedimentation rate: 18 mm/hour). The levels of immunoglobulin IgE in the serum were also normal (20.8 IU/ml, normal range 10–100 IU/ml).
With respect to her medications, the patient was receiving oral therapy with digoxin (0.25 mg/d), furosemide (125 mg twice daily), eplerenone (25 mg/d), and folic acid (5 mg/d); the patient was also on darbepoetin alfa (40 μgr/week) subcutaneously for the treatment of CKD-related anemia as well as tinzaparin (3500 IU/d) as anti-coagulant therapy due to the history of cAF. The above regimen remained unchanged since her initial admittance to the hospital until the appearance of the skin rash. Notably, the medical history failed to uncover the use of any other drugs or substances that could be causally associated with the adverse skin reaction. Moreover, the patient reported no previous history of allergic reaction or known allergies.
The approximately 15-day-long period after the initial exposure to icodextrin along with the negative work-up for other drug-inducible allergic reactions set the suspicion of skin hypersensitivity to icodextrin. On this basis, we decided the discontinuation of icodextrin and modified the CAPD regimen using glucose 3.86% and glucose 1.36% dialysate solutions (2 X 1.5 L glucose 3.86% and 1 X 1.5 L glucose 1.36% per day). The replacement of icodextrin with hypertonic dialysate glucose solutions produced a similar peritoneal ultrafiltration volume of 800 ml/day. The patient was also initiated on oral therapy with methyl-prednisolone 32 mg daily with gradual tapering of the administered dose at weekly intervals. The clinical response was satisfactory and the skin rash improved within 7 days after icodextrin discontinuation. Unfortunately, 2 weeks later, the patient was admitted to our Department with clinical signs of fecal peritonitis that was attributed to colonic rupture and died after a major surgery in the Intensive Care Unit of our Hospital.
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pmc-6201569-1
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We report a case of a 75 year old woman addressed to Allergology Department of our hospital in January 2014 for recurrent episodes of angioedema since the age of 66, with progressively increased severity and frequency. It was first considered to be induced by treatment with angiotensin-converting-enzyme inhibitors (ACEI) for mild hypertension, but she continued to have angioedema attacks for the next 6 years after discontinuation of ACE, with progressive aggravation during the last year. The previous multiple evaluations by many specialists in other hospitals did not succeed to give a clear diagnosis and treatment.
The patient had no relevant medical history and took no medication, except ACEI that was stopped some months after angioedema onset. No relation with possible allergic stimuli could be identified and she had no clinical manifestations between attacks. Angioedema was painful, not accompanied by urticaria or abdominal symptoms, located variably to neck, arms or buttocks, without facial involvement during 6 years. The attacks occurred at weeks or months intervals and usually lasted between 48 and 72 h, irrespective of corticosteroids and antihistamines treatment usually administered. The frequency of attacks had progressively increased from one at 2–3 months intervals to almost weekly during the last year before presentation. The last angioedema attack, determining emergency hospitalization in September 2014, was more severe and prolonged, accompanied for the first time by laryngeal edema and respiratory symptoms. The extended medical evaluation, including complete blood tests for inflammation, allergy, autoimmunity and cancer, were all negative (Table ). Full body CT scan and bone marrow examination were normal. No criteria for lymphoproliferative, mieloproliferative or autoimmune diseases could be found. Measurement of C1 inhibitor (C1INH) in plasma showed significantly decreased level at all measurements, with low activity ranging from 58 to 4% and constantly low C4 (Fig. ). Complement fraction C1q plasma level was measured twice and had normal value. Genetic tests were not performed, given the patient advanced age and lack of family history of angioedema, which are against HAE. A spontaneous mutation in SERPING 1 gene is noticed in up to 25% cases of HAE without family history, but we considered this probability very low in our case, due to late onset of angioedema.
Treatment of angioedema attacks before hospitalization consisted of antihistamines and systemic corticosteroids, which proved to be ineffective. Since no pathogenic therapy with C1INH concentrate, antagonists of bradikinin receptors (icatibant) or selective inhibitor of plasma kallikrein (ecallantide) was available in 2014, we first initiated daily prophylactic treatment with tranexamic acid for 3 months, with no amelioration. During the more severe attack with laryngeal edema, in September 2014, we have switched to attenuated androgen danazol, given 400 mg the initial dose, reduced to 200 mg daily after 1 week and then to 100 mg daily. The clinical evolution was very good, no angioedema attack occurred since the introduction of danazol. C1INH and C4 plasma levels increased after 2 weeks of treatment and became normal after 1 month. After some months, the patient decided herself to discontinue danazol for short time, in order to check effects and to taper the minimal dose. Serum C1-INH and C4 plasma levels were measured after 2 and 4 weeks and showed significant lower levels, but no angioedema attack occurred during this period of time. She therefore restarted danazol prophylactic therapy at a minimum of 50 mg dose daily, ongoing after 3 years. The patient was closely monitored during the next 3 years, with complete clinical and laboratory control twice a year. We took into consideration the possible side effects of danazol, mainly dislipidemia, haematological and liver malignancies and any other complications or concomitant diseases. The clinical evaluation was very good, except two episodes of pulmonary cysts infection, remitted with broad spectrum antibiotherapy, which could not be related to danazol treatment. The clinical outcome of angioedema after 3 years of danazol treatment is very good, with no attacks or other related symptoms. No clinical or laboratory sign of any disease that could induce C1 INH deficiency occurred. No relevant side effects of danazol were noticed. The patient has an improved quality of life due to therapeutic compliance and general management plan.
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pmc-6201585-1
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A 72-year old man suffered from recurrent abdominal attacks beginning at age 8 (Fig. , Timeline). The attacks usually began as a colic-like periumbilical pain that spread to involve the entire abdomen, mimicking abdominal guarding, and progressing with vomiting, diarrhea, debilitation, and often syncope. During adolescence, infrequent subcutaneous angioedema episodes occurred and could not be related to any specific cause. Sometimes, an ill-defined, non-pruritic skin “rash” occurred in various locations. There was no evidence of any atopic condition such as allergic rhino-conjunctivitis, atopic dermatitis or asthma, and no specific tests for allergy were performed. Family history was positive as his father had similar symptoms; however, this evidence went ignored by the consulting physicians. Later, our patient started to experience peripheral subcutaneous swellings, often as a result of minor traumatic stimuli. Conventional anti-allergic therapy with antihistamines, with or without corticosteroids, had scarce benefit. No matter the treatment, these attacks lasted for 48–72 h, or more. He had frequent ER visits over his entire lifetime.
In 1971, at the age of 26, he underwent surgery with a diagnosis of catarrhal appendicitis. There is a limited clinical documentation for the next 30 years, during which he experienced his stereotypical, recurrent abdominal episodes and had 4–6 hospital admissions per year with no conclusive diagnosis.
Recurrent abdominal symptoms in February 2010, led to emergency colonoscopy with removal of a benign polyp. No other diagnosis was made.
One month later, in March 2010, the recurrent acute abdominal pain was diagnosed as acalculous cholecystitis. Ultrasound examination showed ascites in all abdominal compartments, and radiography showed hydro-aeric shadows in the intestinal region. Laparoscopic cholecystectomy was performed without objective evidence for gallbladder lithiasis. Diagnosis at discharge was catarrhal cholecystitis and laparoscopic cholecystectomy.
Two weeks later the patient was hospitalized for severe abdominal pain interpreted as mechanical ileus. Ascites and hydro-aeric shadows were present. Computed tomography (CT) showed 13 mm thickening of the jejunal wall. Symptoms resolved after 7 days of conservative spasmolytic and analgesic treatment.
Seven weeks later in June 2010 he was hospitalized with the identical clinical picture and given the diagnosis of “recurrent mechanical ileus.” An “urticarial rash” was documented on admission. Physical examination found extensive abdominal tympanism, with radiographic hydro-aeric shadows in the left side of the abdomen. Symptoms resolved after 5 days of conservative therapy.
In October 2010 he presented with analogous symptoms. Diagnosis this time was presumed to be an acute volvulus and he had an emergency median and inferior laparotomy. The procedure was described as “manual fixation of a volvulus without intestinal resection, plus debridement of peritoneal adhesions.” The post-operative period was complicated by severe abdominal pain that was resistant to combined analgesic therapy with opioids and nonsteroidal drugs (NSAIDs).
In 2011 and 2012, the patient’s recurrent abdominal pain attacks lead to 4 emergency colonoscopies and 1 gastroscopy. Several benign polyps were extirpated, but there was no indication they were related to the symptoms. Between 2013 and 2015, repeated emergency visits resulted in hospital admissions to Departments of Internal Medicine, Gastroenterology, and Intensive Care, with no conclusive diagnosis at discharge. Additional surgery and endoscopies were proposed, but were refused by the patient based on his previous experiences of multiple ineffective, and in fact, worsening his suffering interventions.
In 2015 one of his grandsons developed episodes of recurrent, disfiguring subcutaneous oro-facial angioedema, abdominal pain and swellings of the oro-pharyngeal mucosa (lips, epi- and hypopharynx, and uvula) that lasted up to 1 week, and were totally resistant to conventional anti-allergic therapy with epinephrine, corticosteroids and antihistamines. No association with an allergic condition could be made: no food, drug, or other triggers precipitated the episodes. They seemed to be unpredictable, or in some occasions provoked by traumatic stimuli. The child had frequent emergency visits and hospitalizations of 3–7 days’ duration. His mother noticed that similar symptoms of facial swellings and abdominal pain occurred in at least three generations of her husband’s family. She searched the internet for these symptoms and began to suspect hereditary angioedema. She requested that her son have C4 and C3 measured. The results showed low C4 and normal C3, so the presumptive diagnosis of HAE was made and the boy was referred to our tertiary center. Further evaluation confirmed the diagnosis of C1-INH-HAE type I in the child and 6 other family members (Fig. , Family tree). Specific treatment for bradykinin-mediated angioedema, C1-INH concentrate on demand: recombinant in the adults (Ruconest®, conestat alfa), and plasma-derived in the children (Berinert®) was introduced in all family members with good clinical outcomes.
|
pmc-6201596-1
|
The patient was a 39-year old female, self-presenting for treatment for severe PMDD. Upon her own request, the patient had been referred from a secondary health care center specializing in OB-GYN (i.e. obstetrics and gynecology), to the Department of Gynecology at Oslo University Hospital (OUS). The aim of the consultation at OUS was to get a second opinion regarding having bilateral oophorectomy to treat her PMDD symptoms. The patient and her husband, voluntarily child-free, both took part in the medical consultation.
When presenting for treatment, the patient reported having struggled with PMDD for nearly a decade with the most debilitating symptoms being affective lability, irritability, anger and interpersonal conflicts, as well as depressed mood and anxiety. The symptoms had increased exponentially over the course of the previous 12–18 months, and were now severely impairing daily functioning. The patient had tried a number of conservative treatments such as oral contraceptives, individual therapy and couple’s counselling, none of which had had a positive impact with regards to PMDD symptoms. Five years prior to presenting for treatment at OUS, the patient had been prescribed intermittent luteal-onset dosing of Citalopram®, an anti-depressant drug in the SSRI class. Albeit “taking the edge off”, the patient reported that the antidepressants provided far from satisfactory effects, and that she had reached a point where the symptoms were so debilitating that she was no longer functioning emotionally, socially, nor professionally. As well as listing the DSM-5 PMDD criteria, Table also gives a post hoc account of the patient’s symptomatology. Criteria met are marked with an asterisk (*), illustrating the fulfilment of all listed criteria, accompanied by the presence of all listed symptoms, an immense symptom burden by any standard.
In addition to the psychological symptoms for which the patient had sought treatment, she also reported having struggled with eating and weight problems since the onset of PMDD (no prior history of an eating disorder was reported). Over the years, these had worsened in parallel with the intensification of affective and behavioral problems. The patient described these problems as an overall, marked increase in appetite and specific food cravings which started intensifying at the time of ovulation, and then increased exponentially until the onset of the menstrual period. She described this pre-menstrual appetite as an “insatiable hunger” characterized by cravings for palatable foods high in carbohydrates (e.g. chocolate, bread, cakes/cookies and pastry) and fats (e.g. nuts and cheese). The patient further described the urge to eat as “impossible to resist”, and explained that once she had given in to the cravings, she was unable to control her food intake (i.e. stop eating). Episodes of uncontrollable food intake almost exclusively took place when the patient was home alone, where large amounts of highly palatable foods were consumed. Initially, food was consumed to satiate hunger and cravings. However, even after having consumed enough to cease hunger, the patient kept on eating, often until feeling uncomfortably full. Due to the fact that as little as one slice of bread, a hand full of nuts or one piece of chocolate could trigger over eating, the patient tried avoiding having “trigger foods” at home. However, this solution only partially helped the patient to resist over eating. Episodes of over eating had occurred almost once weekly over the past 5–6 years, and were associated with a strong sense of shame and disgust, and a feeling of having to compensate by intermittently restricting food intake. The patient, albeit with a normal weight of 65 kg (BMI = 23.9) when presenting for treatment, reported having gained 12 k since the onset of PMDD. Prior to the onset of PMDD, the patient’s weight had been stable at 53 kg (BMI = 19.5) for many years, and as such, she attributed her weight increase to the erratic eating behaviors. Overall, food, eating and weight were constantly on the patient’s mind as she tried to manage a perpetual cycle of restricting and over eating.
Bilateral salpingo-oophorectomy (BSO) in the treatment of PMDD is considered a last-resort measure [], and includes the removal of both ovaries and both fallopian tubes to induce surgical menopause. In the current case, all conservative first- and second line treatments had failed, leading to the consideration of BSO. With the aim of predicting the patient’s response to surgery (and as a prerequisite before referring the patient for BSO), a 3-month trial of a GnRH agonist injection (Procren®, 3.75 mg monthly), combined with estrogen replacement (Progynova®, 2 mg daily) was instigated. Two weeks after the first injection, the patient started noticing a number of adverse effects such as dizziness, arthropathy in hands and feet and amnesia. The discomfort associated with the side effects was severe enough to warrant discontinuation of the GnRH agonist trial. Hence, only one of the three depot injections was given. On a positive note, the patient reported complete (albeit short-term) resolution of all the above mentioned PMDD symptoms, indicating a positive, predictive response to BSO. Two months after the discontinuation of the GnRH agonist trial, laparoscopic BSO was approved. To prevent the risk of reintroducing PMDD symptoms after the surgery (which may be a result of progesterone which is prescribed to prevent endometrial hyperplasia or malignancy) [], the patient also had a (supracervical) hysterectomy.
Albeit being a part of the collective PMDD symptoms, the patient was not treated primarily for problems related to eating. As such, assessment of eating disorder symptoms and attitudes was not performed pre-surgery. However, just short of 4 weeks post-surgery, the patient was retrospectively assessed (i.e. asked to report symptoms present over the past 3 months prior to surgery) using the semi-structured, diagnostic interview the Eating Disorder Assessment for DSM-5 (EDA-5) [], and the diagnostic self-report questionnaire Eating Disorder Diagnostic Scale (EDDS) []. Both assessments yielded a subclinical binge eating disorder (BED), where the patient fulfilled all BED criteria, but reported less than one weekly objective binge eating episode the previous 3 months (the DSM-5 frequency criteria requires that binge eating occurs, on average, at least once a week the previous 3 months). Based on the pattern of food intake related cognitions and behaviors identified through the EDA-5 and the EDDS, the patient’s eating behaviors are hereafter referred to as binge eating rather than over eating.
Symptom burden was assessed four, eight and 12 weeks, as well as at 6 months-post-surgery. Already at the four-week post-op evaluation, the patient reported complete resolution of all previously reported PMDD symptoms. This was also the case eight and 12-weeks after surgery, as well as at the 6-month follow-up. The patient had stopped taking the SSRIs the day after the surgery, and had not felt the need to reintroduce them. Further, the estrogen replacement was well tolerated, with no reports of adverse effects. Paraphrasing the patient, she describing the time after the surgery as “a period of inner peace” that she had not experienced for years. As for the problems related to food, eating and weight, the patient reported a significant decrease in appetite, a complete absence of the aforementioned cravings, and zero binge eating episodes. She now described feeling full after eating “healthy sized” portions, and had successfully reintroduced some of the trigger foods that she had previously avoided (e.g. chocolates, bread, nuts and cheese). She also described a sense of relief as she was no longer constantly thinking of food, and reported eating when hungry, without experiencing loss of control. Follow-up EDA-5 and EDDS assessments were performed 12-weeks and 6 months post-operatively. None of these interviews yielded a full threshold, or a subclinical eating disorder diagnosis. An EDDS symptom composite score, indicating the patient’s overall level of eating pathology, was calculated pre-surgery, and at three and six months follow-up. The retrospective, pre-surgery assessment yielded a composite score of 25, whereas the follow-up composite scores had markedly decreased to seven at 3-month follow-up, and to five at 6-month follow-up. An overall symptom cut-off score of 16.5 has shown to accurately distinguish between patients with eating disorders and healthy controls following DSM-IV criteria [].
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pmc-6201631-1
|
A 69-year-old white Caucasian woman with right kidney tumor and IVC supradiaphragmatic thrombus with no relevant medical history was referred to our department for treatment. Based on computed tomography angiography (CTAG) the tumor was classified as level IV (Fig. ). The results of a laboratory evaluation before the procedure were: hemoglobin (Hb) 92 g/L, C-reactive protein (CRP) 63.9 mg/L, urea 4.6 mmol/L, creatinine 88.5 μmol/L, and white blood cells (WBC) 10.2 × 109/L.
A right nephrectomy was performed in a standard manner with a tumor of the size 40 × 37 × 35mm (Fig. ). TEE was used to determine the position of the IVC clamp with reference to the tumor position. Based on TEE, the pericardium was resected, and an IVC clamp was placed just below the RA through subxiphoid access. A cavotomy was performed with the extraction of tumor thrombus 85 × 35 mm. The cavotomy and laparotomy were closed in a standard manner using non-absorbable monofilament running suture.
She was discharged on the 11th postoperative day with urea 5.6 mmol/L and creatinine 67.8 μmol/L. Her postoperative period was uneventful. Tumor histology revealed clear cell renal carcinoma Patient 1 - pT3b pNO pMO, grade 3/4 (Fuhrman classification) (Fig. ). She is alive was without recurrence of RCC and/or IVC tumor thrombus at a 2-month follow-up.
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pmc-6201631-2
|
A 74-year-old white Caucasian woman with left kidney tumor and supradiaphragmatic IVC malignant thrombus reaching the RA with no relevant medical history was referred to our department for treatment. Based on CTAG the tumor was classified as level IV (Fig. ). The results of a laboratory evaluation before the procedure were: Hb 92 g/L, CRP 35 mg/L, urea 4.8 mmol/L, creatinine 98.7 μmol/L, and WBC 10.6 × 109/L.
Her left kidney and IVC were dissected, liver mobilized. Atrial thrombus was confirmed using TEE. First, a nephrectomy of the left kidney was performed in a standard manner. The tumor size was 75 × 80 × 72mm. Based on the TEE the thrombus in the RA was hardly attached to its wall. A transdiaphragmatic approach was used. A circular suture was placed on the RA where the thrombus was attached, the RA was opened, and the thrombus was flushed out. The RA was closed with primary suture. TEE confirmed the removal of the entire thrombus; therefore, there was no need to remove the thrombus with the use of extracorporeal circulation. In the end, a cavotomy was performed, and the malignant thrombus was removed. The cavotomy closure was performed with primary suture.
On the 8th postoperative day, our patient was transferred to the department of internal medicine for further treatment with urea 10 mmol/L and creatinine 129.7 μmol/L. Tumor histology revealed clear cell renal carcinoma Patient 2 - pT3b pNO pMO, grade 4 (Fuhrman classification) (Fig. ). She is alive and was without recurrence of RCC and/or IVC tumor thrombus at an 18-month follow-up.
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pmc-6201631-3
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A 58-year-old white Caucasian woman with right kidney tumor and IVC thrombus with no relevant medical history was referred to our department for treatment. Based on CTAG the tumor was classified as level II (Fig. ). The results of a laboratory evaluation before the procedure were: Hb 83 g/L, CRP 52.2 mg/L, urea 5 mmol/L, creatinine 105.4 μmol/L, and WBC 12.2 × 109/L.
The right kidney and IVC were dissected in a standard manner. IVC was dilated up to 5 cm just under the right ventricle (RV). A right nephrectomy was performed in a standard manner. The tumor dimensions were 120 × 75 × 70 mm. An IVC cross-clamp was placed just under the liver. The tumorous thrombus was removed through cavotomy in two pieces (65 × 40 × 40 mm and 42 × 30 × 32 mm) as we were unable to remove it at one attempt due to IVC wall infiltration. The IVC wall was infiltrated with a tumor; therefore, it was also resected. The cavectomy was closed with a primary suture (residual stenosis after the closure of the cavectomy was around 40%) (Fig. ).
She was discharged on the 6th postoperative day with urea 9.2 mmol/L and creatinine 111.2 μmol/L. Her postoperative period was uneventful. Tumor histology revealed clear cell renal carcinoma Patient 3 - pT3a pNO, pMO, grade 3 (Fuhrman classification) (Fig. ). She is alive and without recurrence of RCC and/or IVC tumor thrombus at a 48-month follow-up.
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pmc-6201638-1
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A 12-year-old girl presented with Ewing sarcoma of the left pelvis (PI-II). Neoadjuvant chemotherapy (4 courses of ifosfamide + etoposide and 4 courses of vincristine + doxorubicine + cyclophosphamide) was administered, and the extra-skeletal mass disappeared on MRI. Wide excision and reconstruction were performed. We planned osteotomy including the load surface of acetabulum assisted by a computer navigation system and reconstruction using a frozen bone autograft. The patient’s tracker was placed on the iliac crest; the error after registration was within 1 mm. Subsequently, by pulling the lower limbs, a gap of approximately 2 cm was made in the joint space and osteotomy was performed without dislocation of the hip joint (Fig. ). In this case, navigation was particularly helpful in osteotomy of the acetabulum. Although osteotomy under direct view is possible via dislocation of the hip joint, it involves the risk of femoral head necrosis, and this cannot be confirmed from the cartilage surface. In addition, X-ray image alone cannot provide the orientation. Reconstruction was performed using plates and an artificial bone graft and autograft (normal iliac bone) to fill the cavity for the defect of the load surface of acetabulum after tumor curettage. The resection margins were free of tumor, and this was pathologically confirmed in a small sample collected from preserved host tissue. Pathological evaluation of curettage of cancellous bone indicated almost total necrosis of the tumor, which was classified as grade III/IV according to the Rosen and Huvos evaluation system []. After the completion of postoperative chemotherapy (three courses of ifosfamide + etoposide, 1 course of vincristine + doxorubicine + cyclophosphamide, and two courses of vincristine + cyclophosphamide), the patient was free of the disease. Her X-ray did not show osteoarthritis of the hip joint; she could walk normally with some claudication, and there were no limitations in the sitting posture (no limitation of the range of motion of the hip and knee joint) at her 23-month follow-up (Fig. ). Moreover, we evaluated the outcome at the 23-month follow-up using the Musculoskeletal Tumor Society (MSTS) score [], Toronto Extremity Salvage Score (TESS) [], and 36-item Short-form Health Survey (SF-36) [, ]. Her MSTS score was 86.7 and TESS was 86.5. Additionally, her physical component summary, mental component summary, and role-social component summary scores in the SF-36 were 39.4, 59.9, and 44.1, respectively (each summary had a mean of 50 and a standard deviation of 10, according to data from 2007 for healthy Japanese individuals []).
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pmc-6201638-2
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A 71-year-old woman presented with dedifferentiated chondrosarcoma of the right pelvis (PII-III). The initial pathological diagnosis was osteosarcoma, and thus, neoadjuvant chemotherapy (3 courses of cisplatin + doxorubicine) was administered. The chemotherapy caused marked shrinkage and ossification of the tumor. Wide excision and reconstruction were performed. We planned osteotomy avoiding load surface of the acetabulum, in which there was no tumor, assisted by a computer navigation system. The patient’s tracker was placed on the iliac crest, and the error after registration was within 1 mm. In this case, navigation was also particularly useful for osteotomy of the acetabulum. The location and orientation of osteotomy were extremely important for preserving the load surface, but similar to other methods, it is currently impossible to precisely achieve this. Reconstruction was performed using plates and a frozen bone autograft (Fig. ). The resection margins were free of tumor, and this was pathologically confirmed in a small sample collected from preserved host tissue. Pathological evaluation of curettage of the tumor, which was classified as grade II/IV according to the Rosen and Huvos evaluation system. The patient did not undergo postoperative chemotherapy as the final diagnosis was dedifferentiated chondrosarcoma (resistant to chemotherapy), and her physical status was limited. However, she was free of the disease her X-ray did not show osteoarthritis of the hip joint. She could walk almost normally with a cane at her 33-month follow-up. At that follow-up, her MSTS score was 63.3 and TESS was 68.8. Additionally, her physical component summary, mental component summary, and role-social component summary scores in the SF-36 were 26.0, 58.8, and 33.1, respectively.
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pmc-6202075-1
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We present the case of a 61-year-old man with chronic hepatitis B with liver cirrhosis (Child A class) and hepatocellular carcinoma. He underwent right-lobe hepatectomy in 2009. However, the cancer recurred, and he underwent transarterial chemoembolization seven times. In February 2017, he underwent radiofrequency ablation for the recurrent hepatoma. Three weeks after the treatment, he presented with the concern about intractable cough with yellowish sputum. Chest film examination indicated right lower lung consolidation; moreover, computed tomography demonstrated right middle lobe consolidation with pleural effusion and right subphrenic fluid collection (Fig. A and B). The laboratory findings also show abnormality (CRP 28.5 mg/dL, ALP 121 U/L, r-GT 111 U/L, Bil T/D 3.18/1.79 mg/dL, AST 16 U/L, ALT 17 U/L). Right-side subphrenic abscess with empyema thoracis was the tentative diagnosis, and surgical drainage of the observed empyema was performed. During the perioperative period, the attending anaesthesiologist noted an aspiration of yellowish clear fluid from the endotracheal tube, and bronchobiliary fistula was suspected. Percutaneous echo-guided transhepatic cholangiography demonstrated an abnormal tract from the liver to the lung. Thus, this confirmed the diagnosis of bronchobiliary fistula (Fig. C).
Surgical intervention was not considered because the patient’s general condition was not adequate for surgery. We decided to perform percutaneous drainage. The patient’s biliptysis was considerably alleviated after the procedure. However, the amount of fluid drained did not decrease in 2 weeks, and we observed that occluding the drainage tube would lead to biliptysis recurrence. We decided to perform endoscopic retrograde biliary drainage combined with percutaneous drainage (Fig. D) because we assumed that a biliary tract stricture might occur after radiofrequency ablation and lead to inadequate bile drainage. The endoscopic retrograde biliary drainage tube was removed 13 days later, and the observed fistula healed at clinical presentation. Follow-up computed tomography conducted five months after the episode demonstrated satisfactory recovery of the liver and lung parenchyma.
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pmc-6202579-1
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A 73-day-old boy was brought to our hospital with the complaint of progressively increasing jaundice. This boy was conceived by in vitro fertilization, the second among twins, through cesarean section. Antenatal check-ups were unremarkable. Until 15 days of life, this boy was healthy, taking breastfeeds well and passing yellow/greenish stool. Later he developed symptoms: passing clay colored stool, yellowish discoloration of sclera and body. Initial evaluation done in other hospitals revealed cholestatic jaundice. Finally, when the patient was brought to our hospital, he was deeply icteric. The patient was underweight compared with his elder twin. Liver, with smooth margin and soft consistency, was palpable 3 cm below costal margin. Patient had elevated liver enzymes (AST–64 U/L, ALT–129 U/L, ALP–1,000 U/L) and hyperbilirubinemia (Total bilirubin–8.74 mg /dL, direct–4.9 mg/dL). Gamma glutamyl transpeptidase (GGT) was elevated (1,006 U/L). Hemogram, PT/INR, C-reactive protein, and thyroid profile were normal.
Ultrasonography of abdomen revealed dilated intrahepatic biliary radicles with dilated common bile duct (CBD) till mid part of CBD. A hyperechoic soft calculus without distal acoustic shadowing was seen in the lumen of distal intrapancreatic CBD. Gall bladder (GB) was normal in size with well-defined walls (
).
Further, Magnetic resonance cholangiopancreatographic (MRCP) imaging revealed tubular cystic dilatation of common hepatic duct and confirmed intrahepatic biliary radicles dilatation. Cystic duct was unusually draining quite distally. Abrupt narrowing was seen at the junction of common hepatic duct (CHD) and CBD which was hypothesized to be due to a possible stricture at this level. A calculus (8 mm) was evident in the lower CBD extending into intrapancreatic part of CBD. Cystic duct was draining just above the calculus (
and
). Main pancreatic duct was not dilated.
HIDA (hepatobiliary iminodiacetic acid scan) scan revealed no radioisotope excretion into gut even after 24 hours. Presumptive diagnosis of choledochal cyst was made before taking up the patient for laparotomy.
Intraoperatively, hepatomegaly normal size GB, and a 3 × 2.5 cm cyst in subhepatic region were found (
). Bile was seen on needle aspiration from cyst. Mobilization of GB was done and cystic duct was found to be opening into duodenum, without any communication with the cyst or CHD. The cyst was mobilized and looped with a feeding tube. On further dissection, cyst was found to have a blind ending distally (
). Right and left hepatic ducts were patent and opening into the cyst. Both hepatic ducts were irrigated with saline. Excision of cyst and hepaticodocho-jejunostomy was done. Distally, ligation and division of cystic duct at the entry into duodenum, and cholecystectomy completed the procedure. Postoperatively oral feeds were started after 48 hours. Patient passed normal colored stool and showed normal weight gain. Jaundice had subsided and he was discharged after 5 days.
Histopathological examination of cyst (cuboidal epithelium, stromal mononuclear infiltrates) and liver (normal lobular architecture and no evidence of fibrosis, giant cells) were consistent with choledochal cyst.
Follow-up at 6 weeks was done. The patient was healthy, anicteric, taking feeds well, passing normal colored stool, and gained weight.
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pmc-6202760-1
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The patient was an 80-year-old woman (height 155 cm, weight 52 kg) with advanced arthritis of the shoulder combined with rupture of the tendons covering the joint, motivating the implantation of a shoulder prosthesis.
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pmc-6202787-1
|
A twenty-six-year-old male presented to our emergency department after sustaining a self-inflicted gunshot wound to the abdomen thirty minutes prior to arrival. His past medical history was noted for bipolar disorder. Upon arrival by Emergency Medical Services (EMS), trauma code was activated. On primary survey, patient was hemodynamically unstable. His blood pressure was 90\65 mmHg, heart rate was 121 beats\minutes, and was maintaining his airway with a saturation of 98% on oxygen face mask with good bilateral air entry. He scored 14\15 in Glasgow Coma Scale (GCS), with bilaterally reactive three-millimeter pupils. Examination of the abdomen showed an entry wound midline, about ten centimeters above the umbilicus with no exit wound on logrolling. Focused Assessment with Sonography for Trauma (FAST) was positive for free fluid in Morrison’s pouch, spleno-renal and suprapubic regions. Patient received two liters of normal saline, two units of Packed Red Blood Cells (PRBC) class I, and one gram of Tranexamic acid. After that, the patient was intubated, with trauma line inserted, and immediately pushed to the Operating Room (OR) as first-level emergency case within fifteen minutes. A midline exploratory laparotomy revealed large amount of hemoperitoneum and active bleeding which was controlled by packing all four-quadrants of the abdomen. One bleeder was identified to be coming from the lesser curvature, most likely from the right gastric vein, which was controlled with a figure-of-eight PDS stich. After opening the lesser sac, there was another expanding hematoma posteriorly. Decision was made to kocherize the duodenum to reach the retroperitoneum, where another two bleeders were identified. One of which was from one of the supra-renal tributaries of the Inferior Vena Cava (IVC) at its lateral wall and the other coming from the SMV just inferior to the pancreas (). The site of SMV injury was in the mid-third point, around 3–5 cm from the IVC. The SMV injury was less than 1 cm in size, with a low-flow bleeding. Therefore, we cleared the bleeding vessel above and below the injury, identified the lesion, applied proximal and distal pressure, and a couple of stiches were taken in a figure-of-eight manner using PDS sutures (, ). Then, we released proximal and distal pressure, and hemostasis was effectively achieved with our stitch. Multiple pellets were found, and we were able to remove around seventy-five of them. Exploration of major vessels using Mattox and Kocher maneuvers revealed a pocket of pellets next to the aorta, without other injuries (). Therefore, we proceeded with irrigation and partial closure of the abdomen with a Bagota’ bag. A total of eight units of PRBC and three units of Fresh Frozen Plasma (FFP) were given during the operation. The patient was shifted to the Surgical Intensive Care Unit (SICU), and post-operative CT scan and CTA run-off were done, showing patent vessels and no extravasation of contrast, with only minimal irregularity at the IVC injury site. The patient was taken back to the OR in less than forty-eight hours for a second-look laparotomy, which showed no active bleeding or other injuries. There was a concern for bowel viability, however, there was good supply as there was no ischemia or dusky bowel. Therefore, the packs were removed, irrigation and permanent closure of the abdomen were performed, and patient was sent back to the SICU. Two days later, the patient was extubated and started on oral feeding. Psychiatry consultation was made, and the patient was started on Olanzipine and Valproic acid. Close observation of the patient’s safety was ensured, with suicide precautions and one-to-one monitoring. The patient was then transferred to the general surgical ward the following day where he was observed daily by both the psychiatry and surgery teams. After two weeks, the patient was transferred to a local hospital specialized in mental health. Last follow up of the patient was in the surgery clinic, after one month from his first presentation, where he was in good health with no active issue.
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pmc-6202887-1
|
A boy aged 34 days, born in the city and Metropolitan region of the state of São
Paulo, referred from the Basic Health Unit (UBS) to Hospital de Clínicas of
Universidade Estadual de Campinas (HC Unicamp), for presenting with increased
abdominal volume and progressive jaundice for 2 weeks, besides fecal hypocholia in
the past week. The following exams had been performed: hemoglobin (Hb): 8.1 g/dL,
platelets: 85,000/mm3, total bilirubin (TB): 13.3 mg/dL, direct bilirubin
(DB): 8.0 mg/dL, aspartate aminotransferase (AST): 220 U/L, alanine aminotransferase
(ALT): 119 U/L, alkaline phosphatase (ALP): 684 U/L. Abdominal ultrasound:
contracted biliary vesicle, hepatosplenomegaly, small ascites and thick-walled bowel
loops. The mother reported reddish lesions with vesicles, blisters and desquamation
at the palms and soles since birth.
Gestational history: Third pregnancy of the mother, with history of one previous
spontaneous abortion and one living healthy child. Prenatal care with six
appointments, negative serology for HIV, syphilis and hepatitis in the first
trimester, not repeated afterwards. The mother reported use of drugs (amphetamine,
alcohol and cocaine), during the pregnancy. The children was born of natural birth,
at the hospital, weighing 3,000 g, measuring 48 cm, cephalic perimeter of 35 cm,
Apgar score at 1 and 5 minutes of 9 and 10, and gestational age assessed by the
physical examination of 37 weeks and 2 days. He was discharged from the maternity
ward with 48 hours of life. In the child’s birth card, as well as in the mother’s
prenatal card, there were no data about the performance of maternal serology for
syphilis at the time of birth. Afterwards, after contacting the maternity ward, the
information of maternal nonreactive result for VDRL (Venereal Diseases Research
Laboratory) during the hospitalization for the delivery was received. A treponemal
test was not carried out at the time. Serology for syphilis was not performed in the
child.
At the physical examination for the hospitalization, the child was weighing 3,680 g
and cephalic perimeter of 35 cm. There were erythematous desquamative lesions in the
hands and feet, and macular exanthema in the inguinal region. At the physical
examination, the following were identified: ascites, ++/4+ jaundice, palpable liver
5 cm below the right costal and palpable spleen 3 cm from the left costal margin
( and ). Laboratory exams: Hb: 7.3 g/dL, Platelets:
20,000/mm3, TB: 16.7 mg/dL, DB: 14.8 mg/dL, AST: 244 U/L, ALT:
105/U/L, Gamma-glutamyltransferase (GGT): 95 U/L, ALP: 539 U/L, INR: 1,24, R: 2.23.
Syphilis serology: reactive CMIA (chemiluminescent microparticle immunoassay), VDLR:
1:1024, reactive TPHA (Treponema pallidum Hemaglutination),
non-reactive HIV, toxoplasmosis, hepatites B and C and cytomegalovirus serologies.
Cephalorachidian fluid was not collected due to low platelet cont. The long-bone
x-ray showed symmetric and disseminated periostitis, lucent metaphyseal bands and
periosteal thickening of femur, humerus, ulna and tibia (). Transfontanelar ultrasound and fundus oculi showed no
changes. Maternal serology for syphilis (collected right after the positive result
of the child): reactive CMIA and TPHA, VDRL 1:256. Other maternal serologies were
negative. Paternal serology for syphilis was also reactive, performed in a UBS right
after the diagnosis of the child. The father did not provide the result of the
staff, nor the results of other serologies. The father reported the use of
psychoactive substances.
A treatment with intravenous crystalline penicillin, 50,000 UI/kg/dose of 4/4 hours
per 10 days was started, as established for neurosyphilis by the Ministry of Health
(MH). During the treatment, the child received a concentrate of red blood cells and
supplementary offer of oxygen, since he presented with respiratory distress due to
the restrictive effect caused by large ascites. He improved, both clinically and in
laboratory terms: Hb: 10.9 g/dL, Platelets: 89,000/mm3, TB: 12.85 mg/dL,
DB: 8.1 mg/dL, AST: 244 U/L, ALT: 105 U/L, GGT: 182 U/L, ALP: 634 U/L, INR: 1.66, R:
1.09. At this moment, there was an attempt to collect cerebrospinal fluid, however,
without success due to technical difficulties. He was discharged on the 18th
hospitalization day, for outpatient clinic follow-up in the pediatric
gastroenterology and infectology medical clinics. During hospitalization, the mother
began a treatment with 3 weekly doses of 2,400,000 UI of benzathine penicillin, and
the father was referred to treatment and follow-up in the UBS.
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pmc-6202903-1
|
Male, 8-month-old patient, weighting 11 kg, living in the city of São Paulo (São
Paulo), was hospitalized in the pediatric emergency room presenting with laryngeal
inspiratory stridor associated with suprasternal notch and subdiaphragmatic
retraction, classified as severe (score 11) in the Westley’s scale (). He evolved to severe acute respiratory
failure, presenting respiratory frequency (RF) of 70 breaths per minute (bpm) and
heart rate (HR) higher than 200 beats per minute (bpm), requiring tracheal
intubation and being referred to the pediatric intensive care unit (ICU).
As personal history, the child was born in a C-section, at term, and was intubated
for 17 days in the pediatric ICU due to early respiratory distress. He presented
with tracheal extubation failure caused by high respiratory distress in that
hospitalization, evolving without intercurrences after that period.
In the pediatric ICU, dexamethasone (0.6 mg/kg/day) was introduced on the first day
of hospitalization, and the patient remained in mechanical ventilation, requiring
ventilator support: fraction of inspired oxygen (FiO2): 0.25; inspiratory
time: 0.79 seconds; RF: 30bpm; positive end expiratory pressure: 5 cmH2O;
pressure support: 14 cmH2O; pressure control: 16 cmH2O; and
current volume: 85 mL. He remained intubated, on midazolam (0.2 mg/kg/h) and
fentanyl (2 mcg/kg/h), without the need for vasoactive medication, remaining
clinically stable. Six days after hospitalization, flexible bronchoscopy was carried
out to assess the conditions of the mucosa before indicating extubation, since the
patient had been intubated for six days without signs of tracheal peri-cannula
ventilator escape. Bronchoscopy revealed “major mucosal edema, involving the
intratracheal cannula” (), so the
choice was to maintain the tracheal cannula, without progression of bronchoscopy
through the trachea. The infant was intubated, stable, on dexamethasone and
medication for sedation and analgesia. Seven days after bronchoscopy, he
demonstrated signs of escape through the tracheal cannula, shown by auscultation and
observed in the monitor of the ventilation equipment (Servo-i®, Maquet,
Rastatt, Germany). A new flexible bronchoscopy was carried out for the supervised
extubation. Bronchoscopy showed “larynx with discrete posterior wall edema, with
good mobilization of vocal folds and air escape between the tracheal cannula and the
larynx”.
For the programming of the tracheal extubation, a thoracic X-ray was performed and
did not show any abnormalities nor arterial blood gas (pH: 7.50; pO2: 120
mmHg; pCO2: 32 mmHg; sodium bicarbonate: 21 mEq/L; base excess: +1;
SatO2: 98%). Tracheal extubation was conducted and the use of nasal
oxygen catheter began, with 2 L/minute, presenting with mild laryngeal stridor, with
improvements after inhalation with one ampoule of budesonide (0.25 mg). Forty-eight
hours after the extubation, the patient evolved to severe acute respiratory failure,
with major laryngeal stridor, vacillation of the wings of the nose, and increased HR
until 167 bmp, and RF of 39 bpm, indicating a new tracheal intubation. The choice
was to start support with high-flow nasal cannula (Optiflow Junior®,
Fisher & Paykel, Auckland, New Zealand) at 15 L/minute, FiO2: 25%, with
significant improvement in the respiratory situation, as well as reduced HR and RF
(). The infant was comfortable in
the high-flow nasal cannula, preventing tracheal reintubation.
The patient remained with the high-flow nasal cannula for nine days, and showed
improvements in high respiratory discomfort. There was a transition from the
high-flow nasal cannula to nebulization with oxygen at 2 L/minute for one more day,
being discharged to pediatric nursery in good clinical conditions.
Before discharge, since the patient presented with persistent laryngeal stridor, and
because of the previous history of intubation for 17 days, and extubation failure in
the neonatal period, a new flexible bronchoscopy was performed to investigate the
after-effects in the airways, showing “hyperemia of the larynx and subglottic region
with edema and granular surface”.
|
pmc-6203193-1
|
A 45-year-old Japanese woman, an out-patient, underwent an emergency examination for left lower abdominal pain. She was in her usual state of good health until 1 day ago when she noticed the gradual onset of abdominal pain. Over the last 12–24 hours, she noticed it more in the left lower quadrant. Pain did not radiate, but stayed localized in the costovertebral angle (CVA) when her back was clapped. She had not noticed any fever, chills, or night sweats. She reported anorexia. She was not pregnant; she was not menstruating. She had no past medical history except for mammoplasty. No diseases run in her family. She drank alcohol occasionally; she did not smoke tobacco or use illegal drugs. She worked as an office worker. The physical findings were absence of the sternal head of the right pectoralis major (Fig. ) and protrusion of the right scapula (Fig. ). She reported right-sided weakness and presented symptoms of glaucoma. There were no symbrachydactyly on right hand and no signs of neurological disorders. She had been aware of an underdevelopment of one breast since infancy, lack of right underarm hair, difficulty exerting strength on the right side of her body, and feeling fatigue mainly on the right side when in a supine position.
Computed tomography (CT) images revealed a hydronephrotic left kidney and dilatation of the left ureter. No ureteral calculus, neoplasm, or obstruction was found, and no abnormal findings that would explain her left abdominal pain were identified. Other CT findings were absence of the sternal head of the right pectoralis major, asymmetrical malformation of her chest wall due to hypoplasia of the right rib cage (Fig. ), a shortened sternal body and anomaly of the xiphoid process (Fig. ), spinal curvature to the right (Fig. ), hypoplasia of the right ilium (Fig. ), advanced atrophy of her right kidney (Fig. ), hypoplasia of the right gluteus minimus (Fig. ), and cystic mass in her right ovary (Fig. ). Contrast-enhanced CT revealed a disruption of her right internal iliac artery (Fig. ). Blood examinations revealed no abnormalities. Urine analysis revealed leukocytosis but no urinary blood. Nephritis was diagnosed and antibiotics were prescribed. She is currently showing improvement and receiving out-patient treatment. She has recently been on the verge of developing glaucoma and is seeing an ophthalmologist.
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pmc-6203196-1
|
A 57-year-old woman presented to our office with complaints of wheals, arthralgias, and a severe, generalized burning sensation on the skin. The skin lesions appeared as urticarial plaques located mainly on the trunk and proximal extremities persisting for more than 24 h after the initial appearance and leaving faint residual hyperpigmentation on the skin (Fig. ). The patient reported approximately 6 months of relapsing and remitting symptoms. No angioedema or relevant past medical history were noted. On the basis of the previous findings, a possible diagnosis of urticarial vasculitis was suspected, and laboratory tests with a skin biopsy were requested. Laboratory tests were unremarkable; no abnormalities were noted on hemogram, acute phase reactants, thyroid function, or complement levels. Skin biopsy revealed leukocytoclastic vasculitis with perivascular infiltrates and neutrophil predominance (Fig. ). After a careful assessment, the patient was diagnosed with normocomplementemic urticarial vasculitis.
The patient was initially treated with a short course of oral corticosteroids (prednisone 40 mg initially for 4 days, followed by gradual tapering off for a total of 12 days), first generation H1 antihistamine (hydroxyzine 50 mg taken at night), second generation H1 antihistamine (fexofenadine 20 mg up to fourfold dosage), and hydroxychloroquine 200 mg daily for 4 months. Despite the initial treatment, the symptoms did not improve, and the UV appeared as a more active and severe disease during physical examination. In addition, the patient was disappointed with how her quality of life was markedly impaired due to her clinical condition. For this reason, Urticaria Activity Score 7 (UAS7) and Chronic Urticaria Quality of Life Questionnaire (CU-Q2oL) were assessed for the first time to have a more objective course of the disease. Her UAS7 immediately after the 4-month course of first-line therapy was 19, while CU-Q2oL showed a functioning status of 29.2, sleep of 12.5, itching/embarrassment of 25.0, mental status of 25.0, swelling/eating of 18.8, and limited appearance of 25.0. Due to the lack of response to first-line treatment and the severity of the disease, treatment with omalizumab was initiated.
Omalizumab 150 mg was administered every 4 weeks for 3 months. Second-generation antihistamines were used as needed. Both CU-Q2oL and UAS 7 improved. After three-month therapy with omalizumab, disease severity improved from moderate severity (UAS7 = 19) to well controlled (UAS7 = 6). However, due to economic limitations and insurance restrictions, the patient discontinued the treatment with omalizumab. Then, 5 months after she received her third dose, the patient complained of worsening symptoms and active disease with quality of life impairment. Thus, the fourth omalizumab (150 mg) dose was prescribed with corticosteroids. Thereafter, the patient presented a disease activity and quality of life with a fluctuating pattern that was controlled with additional doses of omalizumab (5 in total), which is depicted in Fig. . While follow-up is still ongoing, the patient is currently asymptomatic without any kind of medications and her mean UAS7 score after the last administration of omalizumab (ninth dose) is 4.88, which is considered a well-controlled urticaria (Fig. ) [].
|
pmc-6203207-1
|
In January 2017, a 76-year-old male patient was admitted to our hospital with ascites and dyspnea. In the patient’s history, a portal vein thrombosis was known for more than 10 years due to relapsing, necrotizing biliary pancreatitis. At that time a cholecystectomy with biliodigestive anastomosis was performed. Ascites was analysed after large-volume paracentesis without signs of spontaneous bacterial peritonitis or malignancy. On the second day after hospitalization, an esophagogastroduodenoscopy was performed to screen for esophageal varices. After the intervention, the patient developed fever and chills. Cholangitis was suspected due to biliodigestive anastomosis, increase of cholestasis parameters and an antibiotic treatment with ceftriaxone was started the same day (dose 4 g intravenously). Immediately after drug application the patient complained about nausea, vomited and developed dyspnea, confusion and a positive shock index (systolic RR < 100 mmHg, cardiac frequency 140 /min). The patient was referred to our intensive care unit and the antibiotic regime was escalated to piperacillin/tazobactam and ciprofloxacin for sepsis therapy. The patient received no further dose of ceftriaxone. Laboratory analysis about 1 h after application of ceftriaxone showed first signs of hemolysis with an elevated lactate dehydrogenase (LDH) (1,116 U/L (18.6 μkat/l); baseline 290 U/L (4.83 μkat/l)) and a decrease in hemoglobin (6.4 g/dl (3.97 mmol/l), baseline 8.5 g/dl (5.28 mmol/l)). Coagulation parameters were significantly disturbed indicating DIC with an international normalized ratio (INR) of 3.31 (baseline 1.29), fibrinogen not measurable, thrombocytopenia down to 56,000/μl (baseline 203,000/μl). During the next days, the patient developed an increase in leukocytes (up to 23,000/μL) and in infection parameters (peak C-reactive protein (CRP) 9.35 mg/dl (890.48 nmol/l), peak procalcitonin (PCT) 134 μg/l). Additionally, hemolysis aggravated with a nadir hemoglobin of 4.8 g/dl (2.98 mmol/l), an elevated LDH up to 1,734 U/L (28.9 μkat/l) and suppressed haptoglobin < 0.1 g/l. (course of laboratory parameters is depicted in Fig. ). Furthermore, the patient subsequently developed a severe acute kidney failure with uremia (peak creatinine 6.29 mg/dl (556.04 μmol/l), urea 192.3 mg/dl (32.11 mmol/l)) and intermittent hemodialysis was necessary for 14 days. A kidney biopsy was performed and showed a severe acute tubular damage fitting with shock-induced injury and/or tubular-toxic effects of free hemoglobin/hemin.
The massive hemolytic reaction came suddenly and was unexpected. After exclusion of hematological comorbidities, a detailed patient history with current drug exposure was performed. Before admission to our department the patient had been hospitalized in our surgical department due to pneumothorax after pacemaker implantation. During this hospitalisation (< 3 weeks before the current admission) the patient had already been treated with ceftriaxone for at least 6 days and had already developed mild hemolysis in laboratory analysis without further consequences at that time. Further detailed diagnostic showed a positive Coombs’ direct antiglobulin test (DAT) for immunoglobulin M (IgM), immunoglobulin G (IgG) and complement factor C3d. On Naranjo Scale, a probability scale for adverse drug reactions, the patient would have reached a value of 9 points (maximal score 13 points, with values ≥9 points indicating a definite adverse drug reaction) []. The suspected DIIHA was proven by reference laboratory analysis (Institute of Transfusion Medicine, Charité, Berlin), confirming the presence of a strongly agglutinating ceftriaxone-dependent antibody (Fig. ).
The patient’s situation stabilized with decrease of hemolysis parameters, stable hemoglobin levels and reconstitution of kidney function after withdrawal of hemodialysis. At the time of discharge from hospital laboratory results were stabilized or even normalized: creatinine 2.04 mg/dl (180.34 μmol/l), bilirubin 0.5 mg/dl (8.55 μmol/l), LDH 207 U/L (3.45 μkat/l), INR 1.26, hemoglobin 7.6 g/dl (4.72 mmol/l). In a follow-up visit 4 months later kidney function was also normalized and the patient had returned to normal daily life.
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pmc-6203215-1
|
A 45-year-old male was hospitalized due to bloody nasal discharge, hemoptysis and rash for 11 years, which aggravated after 2 weeks, and presented with abdominal pain and melena for 1 month. The patient was hospitalized and diagnosed with WG 11 years ago. Furthermore, he had rashes on his face, trunk, limbs and feet, and had oral ulcers, perianal ulcers, and sinusitis.
The laboratory tests revealed the following: leucocytes count of 8.5 × 109/L, hemoglobin level of 79 g/L, urine protein (+), microhematuria (+), erythrocyte sedimentation rate of 50 mm/h, c-ANCA (antineutrophil cytoplasmic autoantibodies) with a titre of 1 in 100, and PR3-ANCA (anti-neutrophil cytoplasmic antibodies proteinase 3) of > 200 RU/ml. Computed tomography (CT) revealed left frontal and ethmoid sinusitis, and bilateral maxillary sinusitis. Based on these clinical features and laboratory findings, the patient’s diagnosis of WG was accurate. In order to clarify the cause of the abdominal pain, a colonoscopy was performed with the written informed consent of the patient. Different sizes of irregular ulcerations, which were 3–4 cm and 1–2 cm in diameter, were scattered at different intervals throughout the colon (Fig. ). The larger ulcers presented raised margins and fibrin coatings on the base. Colonic biopsies revealed inflammatory granulation tissues. At approximately 40 cm from the anus, an annular black membrane, which was approximately 4 cm in diameter, adhered to the surface of the intestinal wall. The membrane could be lifted using biopsy forceps. Part of the membranous substances appeared like mucosal surface tissues, and was completely separated from the intestinal wall. The colonoscope could pass through the membrane, and erosions and ulcers were scattered at the bottom (Fig. and ). The membrane biopsy revealed degenerative colonic mucosal tissues, while the colonic biopsy revealed inflammatory granulation tissues without normal colon glands (Fig. and ). Oral prednisolone (60 mg/d) combined with intravenous cyclophosphamide (600 mg/d, once) was given. The patient was rehabilitated and discharged after 10 days of treatment. Symptoms including bloody nasal discharge, hemoptysis, skin rash and abdominal pain all disappeared.
|
pmc-6203236-1
|
A 55-year-old female presented to the Otolaryngology – Head & Neck Surgery clinic with a four-month history of a left-sided neck mass. The patient had a history of metastatic ileal NET with metastases to the liver, mesenteric nodes, and peritoneum. She underwent primary right hemicolectomy and small bowel resection in 2010. Pathology revealed two foci of well-differentiated malignant NET in the terminal ileum, with muscle and serosal invasion, and three of nine regional lymph nodes involved. There were two to three mitoses per ten high power fields (HPFs) with no necrosis and the Ki-67 index was 3%. Immunohistochemistry revealed positivity for synaptophysin and chromogranin. Pre-operative imaging revealed metastatic involvement of an aorto-caval node, thus the disease was staged as pT4(m)N1M1. Post-operatively, treatment began with long acting somatostatin analogue (SSA). Liver metastases were detected eleven months after her initial surgery. In 2014, she developed carcinoid syndrome leading to therapy with radiolabelled metaiodobenzylguanidine (MIBG). Subsequently she received telotristat etiprate due to progressive symptoms.
On presentation of the neck mass in 2015, she had no additional otorhinolaryngological symptoms. Examination of the neck revealed a smooth, mobile, painless, 2-cm mass located in the submandibular triangle. The remainder of the physical examination was unremarkable, including flexible nasopharyngoscopy which revealed no mucosal masses or lesions in the upper aerodigestive tract.
Fine needle aspiration (FNA) revealed malignant cells with mildly pleomorphic nuclei and salt and pepper chromatin. Immunohistochemistry was positive for chromogranin and synaptophysin, and consistent with metastatic NET. Computed tomography imaging of the mass revealed left submandibular gland enlargement and no cervical lymphadenopathy (). The multidisciplinary head and neck tumor board, in conjunction with her primary medical oncologist, recommended resection of the tumor to improve local control due to the low morbidity of the surgery.
An uncomplicated left level 1b neck dissection was performed and there were no permanent post-operative sequelae. Gross pathology revealed a circumscribed tumor mass within the submandibular gland that measured 2.2 × 1.5 × 1.4 cm, and appeared as a firm, smooth nodule with a grayish white color. The tumor displayed focal abutment of the margin, but did not extend outside of the gland. Immunohistochemistry was again positive for chromogranin and synaptophysin, consistent with the previous FNA and was ().
Unfortunately, the patient passed away one year later due to progressive disease in the mesentery and liver.
|
pmc-6203241-1
|
The patient is a 55-year-old white male with a history of hypertension, type II diabetes, resulting in hypertensive and diabetic nephropathy, as well as NASH cirrhosis. This diagnosis was further complicated by hepatorenal syndrome, hepatic encephalopathy, esophageal varices, and ascites requiring multiple endoscopies for variceal banding and paracenteses. Abdominal imaging demonstrated cirrhosis without evidence of hepatocellular carcinoma. He did have evidence of portal hypertension, splenomegaly, and thrombosed main portal vein with poor distal reconstitution. In October of 2017, the patient received combined liver-kidney transplantation with en bloc graft. The donor was a brain dead 42-year-old white male. His terminal creatinine was 1.1 mg/dL and liver function tests were within normal limits.
Organ recovery was performed using standard techniques for donation after brain death (DBD) as previously described using histidine-tryptophan-ketoglutarate (HTK) solution for flushing []. The liver and right kidney allografts were procured en bloc, dividing the inferior vena cava (IVC) inferior to the right renal vein. The renal allograft did have a main hilar artery and an inferior polar artery. Standard liver and kidney back-table reconstruction was performed. However, an arterioplasty was performed on the main and lower pole renal arteries followed by end-to-end anastomosis to the donor splenic artery using 7-0 polypropylene sutures ( A, B).
The recipient hepatectomy was performed in the standard fashion with ligation of the hepatic arteries, common hepatic duct, and portal vein. A portal vein thrombectomy was performed using eversion technique, extended down to the confluence of the superior mesenteric vein and splenic vein resulting in adequate portal flow. The right colon was mobilized medially to identify the proximal right ureter.
Transplantation was performed by modified piggy-back technique with a side-to-side cavo-caval anastomosis. The portal venous anastomosis was completed in a standard end-to-end fashion. Next, the arterial inflow to the allografts was performed by anastomosing the donor celiac artery to the recipient common hepatic artery. The renal arteries were positioned posterior to the portal vein and donor IVC. Portal venous and arterial flow was restored to the en bloc organs. The lower pole of the kidney did not demonstrate brisk perfusion compared to the rest of the allograft, with poor Doppler signal in the lower pole artery. Therefore, the lower pole artery was re-anastomosed to the donor left gastric artery stump anterior to the portal vein, resulting in excellent flow. Finally, an end-to-side ureteroureterostomy was performed to the recipient’s right ureter over two Double-J stents (). Total operative time was 8 h, wherein CIT was 4 h and warm ischemia time (WIT) was 40 min.
Postoperative liver and transplant kidney Doppler studies demonstrated normal portal vein flow (86.4 cm/s), normal hepatic artery flow with resistive index (RI) slightly elevated at 0.75, and normal arterial waveforms on kidney Doppler with RI ranging from 0.56 to 0.66 between upper pole and lower pole. Improvements in serum creatinine and liver function tests were noted immediately post-transplantation and continued to improve. The patient’s operative course was uncomplicated and he was discharged on postoperative day six. At 30-day follow up, the portal vein remained patent with normalization of hepatic artery RI at 0.68 and stable range of intrarenal arterial RI (0.49–0.53). The ureteral stents were removed by rigid cystoscopy and ureteroscopy with basket extraction by Urology 6 weeks post-transplantation.
|
pmc-6203242-1
|
A 17 year old horse-rider girl was referred to our clinic for 2 weeks history of moderate continuous, crampy abdominal pain, starting in the epigastric region and shifted to the left upper quadrant. This pain was associated with fatigue, loss of appetite. Patient denies any nausea, vomiting, diarrhea, fever, night sweats. She reported a remoteleft shoulder pain with negative MRI. Her physical exam was positive for splenomegaly 9 cm below costal margins, and left upper quadrant tenderness, with no rebound tenderness. Her laboratory examination showed a Hg:12.8 g/dL, platelet: 124,000/mm, WBC: 6500/mm with 62% neutrophils, 24% lymphocytes, and 7% eosinophils, platelet: 124,000/mm, a CRP:0.78 mg/L. The Liver enzymes, Bilirubin, Albumin, LDH, the Chemistry panel were all in normal range. Abdominal ultrasound showed a large splenic cyst of 15 cm containing homogenous internal debris (). An abdominal computed tomography scan showed the same 15 cm splenic cyst with parietal calcifications, compressing the stomach, most likely of hydatid origin (a, b). Abdominal MRI showed unilocular splenic cyst hypo-intense T1, hyper-intense T2 (a, b). Differential diagnosis for described findings include; Splenic abscess, Hydatid cyst, epithelial cyst and post traumatic hemorrhage in pre-existing epithelial cyst. Based on clinical picture and endemic status for hydatid cyst differential can be narrowed.
Serologic test for Hepatitis B virus (HBV), hepatitis C virus (HCV), Cytomegalovirus (CMV), HIV, Toxoplasmosis, Entamoeba histolytica, Leishmania brazilensis, donovani, and EBV IgM were all negative. EBV IgG was elevated showing prior immunization. The immune-diffusion test for Echinococus multilocularis was negative. The Indirect hemagglutination test and the Elisa test for Echinococcus granulosus were also negative.
Nevertheless, due to an elevated IgE level: 317 kU/L, the patient was considered as having splenic hydatid cyst and was treated by albendazole PO with meals in a dose of 400 mg twice daily for 28 days, and received the anti-pneumococcal vaccine.
Due to the severe continuous pain, the large size, the risk of spontaneous rupture and the patient’s wishes to resume her hobby as a horse-rider as soon as possible, she was consented for operative exploration via a laparotomy incision for splenic cyst un-roofing.
Exploration was done, abdominal cavity was protected by hypertonic saline (3%NaCl) filled pads, cyst was punctured, 2 liters of dark green fluid was aspirated. Hypertonic saline was injected in the cyst, and then aspirated after 15 min. Un-roofing and partial resection was done afterward.
The postoperative course was un-eventful and the patient was discharged home on the post-operative day 5.
The pathology report showed stratified epithelium with fibro-inflammatory reaction in the pericystic zone compatible with splenic epithelial cyst.
The patient still symptom free after 5 years of follow up and her labs showed a WBC: 7700/mm3 normalization of eosinophils (2.5%).
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pmc-6203469-1
|
A 48-year-old female was sent to the emergency department of our hospital with the chief complaint of sudden occurrence of dyspnea. The patient experienced sudden respiratory distress when she was lifting heavy cargo during farm work. She also complained orthopnea with pink bubble sputum cough. The patient was 155 cm in height and weighed 48 kg. Her heart rate was 103 per minute, and blood pressure was 99/63 mm Hg. Physical examination revealed a 4/6 systolic murmur in cardiac apex. Electrocardiogram demonstrated depression of ST segments at multiple leads: I, II, aVL, and V3 to V6. Meanwhile, blood test showed no troponin elevation. Acute pulmonary congestion was detected by chest X-ray. Echocardiogram found a prolapsed posterior leaflet of the mitral valve with massive regurgitation, with an ejection fraction (EF) of 68% and a normal sized left ventricle. The patient denied of similar symptoms in her past history.
Therefore, she was preliminarily diagnosed of acute left heart failure caused by mitral regurgitation and scheduled for valve repair surgery. Routine coronary angiography was performed to exclude coexisting coronary artery disease. However, the left main coronary trunk could not be catheterized. Meanwhile, collateral vessels could be seen flowing from right coronary artery (RCA) to fill the left coronary system (Fig. ). The patient was then suspected of left main coronary atresia or complete occlusion. She received CT coronary angiography for further diagnosis, which confirmed absence of left coronary ostium, with abnormally small LAD and left circumflex arteries (LCX), both filled by collateral vessels arising from RCA.
During operation, the patient's mitral chordae of the posterior leaflet was found ruptured, with dysplasia of papillary muscle (Fig. ). Then, a successful mitral valvuloplasty was first performed. Although with worry that the small caliber left coronary system may not be a suitable target for surgical revascularization, we performed CABG with left internal mammary artery anastomosed to proximal LAD anyway. Graft flow was satisfactory, with a flow value of 28 mL/min and pulsatile index of 1.2. By direct inspection through aortotomy, no LMCA trunk nor left aortic sinus could be identified. Therefore, the diagnosis of left main coronary atresia was confirmed.
The patient recovered uneventfully and discharged 7 days after operation. Three months later, she came back for follow-up visit and received routine examination together with CT angiography. Her heart function was normal as revealed by echocardiography, and coronary angiography showed a patent LIMA-LAD graft with a small LAD (Fig. ).
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pmc-6203572-1
|
A 68-year-old woman presented with progressively worsening dysphagia that occurred 5 months ago, with a rapid weight loss of 3 kg. She was previously healthy with no special medical history. Esophagogastroscopy showed a prominent luminal stenosis of the esophagus, which is apparently caused by an external pressure. A contrast-enhanced abdominal computed tomography (CT) revealed an inhomogeneous, soft tissue mass in the posterior mediastinum measuring 11.9 × 10.2 cm (Fig. ). Tumor markers including CEA, CA19-9, CA125, were all within the normal range.
The patient underwent a mediastinoscopy and chest CT-guided core biopsy of the mass. Biopsy was suspicious of stromal tumor. Immunohistochemical stains of tumor cells were consistent with a GIST (strong positive for CD117, CD34, and DOG-1; negative for SMA, PCK, CK18, S-100, and desmin; Ki67 index was 8%). Molecular analysis revealed a deletion mutation in exon 11 of the KIT gene.
The patient was counseled receiving preoperative imatinib therapy and the second-stage operation. She refused and insisted direct surgical resection. During the operation, we found that the mass pressed the inferior lobe of the left lung, distal esophagus, thoracic aorta and was adherent to the fundus of stomach, which was in favor of gastric origin instead of the esophagus. Curative resection including the tumor, lower part of the esophagus, and fundus of the stomach were performed. The tumor measured 13 × 10 × 10 cm and was well-encapsulated, firm mass with focal hemorrhage and cystic formation, and without obvious necrosis. The microscopic findings showed that the tumor cells were spindle in shape; the mitotic counts were increased to 18 per 50 high-power fields. Immunohistochemical analysis of tumor cells confirmed a high-risk GIST according to the National Institutes of Health (NIH) risk criteria, which was consistent with the previous report. The postoperative course was uneventful, without adjuvant imatinib therapy. Forty-eight months postoperative, the patient is alive without any evidence of tumor recurrence.
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pmc-6203695-1
|
A 69-year-old man was referred to our hospital because he developed dyspnoea and delirium two days after working with wood preservative in an enclosed space. He had been exposed to the agent for about eight hours on each of the two days. He had a current 60 pack-year smoking history. On presentation, he was tachycardic (pulse rate of 88/min) and tachypnoeic (respiration rate of 20/min), with laboured breathing. Oxygen saturation was 90% on 10 L/min oxygen via a reservoir mask. Burn injuries were apparent on his face, neck, chest, and both upper arms. Bibasal coarse crackles were present on chest auscultation. Brain computed tomography (CT) demonstrated no remarkable phenomena. He had an elevated white cell count with 90% neutrophils, and the C-reactive protein (CRP) was 23.8 mg/dL. Arterial blood gas examination results were as follows: pH 7.40, PaCO2 31.8 mmHg, PaO2 63.7 mmHg, and HCO3
− 20 mmol/dL (10 L/min oxygen via a reservoir mask). The chest X-ray on admission demonstrated increased bilateral hilar shadows (Fig. A), and thoracic CT indicated infiltration in both lower lobes (Fig. B), which was observed to have a low-density area in the infiltrative shadow using mediastinal windows (Fig. C). His ECG was 82 bpm in the normal range, and no cardiac enzyme was elevated. Because burning of the respiratory tract was expected in connection with the burn injury on his face and neck, intratracheal intubation and mechanical ventilation were performed in anticipation. Subsequently, bronchoalveolar lavage (BAL) was carried out, and the return fluid (Fig. D, white arrow) resembled the wood preservative (Fig. D, yellow arrow). The BAL differential count demonstrated a neutrophilia of 46% (the normal range; 3% or less). Bacteriological culture and acid-fast bacilli stains were negative. Kerosene was assessed to be the main component of the wood preservative. The diagnosis of hydrocarbon pneumonitis caused by inhalation of this agent containing kerosene was established based on these findings. Antimicrobials were administered because of potential infectious complications. In addition, intensive treatment, including mechanical ventilation and hydration, was provided. Although the infiltration of both lower lung fields worsened temporarily (Fig. E), the patient’s general condition and radiological findings gradually improved with persistent, intensive management (Fig. F). He was successfully weaned off mechanical ventilation and was discharged after two months. He fully recovered without any residual deficits after his discharge.
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pmc-6203696-1
|
A 74-year-old female presented to her cardiologist with a 6-week history of worsening dyspnoea on exertion. She had a past medical history of stage IIIa invasive lobular carcinoma of the left breast, positive for both oestrogen and progesterone receptors (oestrogen receptor (ER)/progesterone receptor (PR)), which was diagnosed 21 years back. At that time, she had undergone modified radical left mastectomy at age 53, followed by treatment with adjuvant chemotherapy and radiation. Due to her current symptoms of dyspnoea, a chest X-ray was taken, which showed moderate-sized left pleural effusion compared to a chest X-ray from 2 years ago (Fig. a). She was therefore referred to Interventional Pulmonology for further workup of pleural effusion. An ultrasound-guided left-sided thoracentesis demonstrated lymphocyte-predominant exudative pleural effusion (Fig. b). Cytology of the pleural fluid showed malignant cells with immunohistochemistry positive for breast tumour markers. Analysis of the pleural fluid was weakly positive for ER and PR and negative for human epidermal growth factor 2 (HER2) receptors. A post-thoracentesis chest computerized tomography scan with contrast showed a small residual left pleural effusion with a right pulmonary nodule. The patient was diagnosed with metastatic breast carcinoma and was referred to Oncology for further treatment.
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pmc-6203696-2
|
A 76-year-old female presented to her cardiologist with a 3-week history of progressive shortness of breath on exertion. Her cardiac workup was negative, including an ejection fraction of 60–65% on echocardiography. However, chest X-ray showed right-sided pleural effusion. She was therefore sent to Interventional Pulmonology for further workup. Further review of her past medical history indicated that, at age 62, she was diagnosed with invasive ductal carcinoma of the left breast as well as in-situ ductal and lobular carcinoma of the right breast. At that time, she underwent bilateral simple mastectomies with adjuvant chemotherapy. A right-sided thoracentesis was performed and demonstrated lymphocyte-predominant exudative pleural effusion. Cytology of the pleural fluid was negative for malignancy.
Over the next few weeks, the patient developed progressively worsening dyspnoea. A repeat chest X-ray 4 weeks following her initial thoracentesis showed recurrent right-sided pleural effusion. Pleuroscopy with pleural biopsy was scheduled. Pleuroscopy showed multiple masses throughout the parietal pleura (Fig. ), diaphragm, and on the right lung. Biopsy of the pleural masses showed metastatic adenocarcinoma positive for breast markers and negative for lung or gastrointestinal markers. She was ultimately diagnosed with MPE secondary to metastatic ER+/PR+/HER2—breast carcinoma. She was referred to Oncology for further treatment.
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pmc-6203708-1
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On February 2016, a 40-year-old Djiboutian man presented at Chakib Saad Hospital, Djibouti, a hospital in charge of pulmonary pathologies. The patient was living in Balbala (Bouldhouqo) in Djibouti and working as a seller in a clothing store. He reported no travel outside Djibouti, no medical, surgical or tuberculosis histories, but a three-month cough. The patient was found to be HIV-negative. A chest radiograph revealed a retractile opacity of the right upper lobe of the lung and a para-aortic opacity with micronodules of the culmen (Fig. ). Direct microscopic examination of the sputum smear after Ziehl-Neelsen staining exhibited acid-fast bacilli but the sputum was not cultured. The patient was diagnosed with pulmonary tuberculosis and received first-line antituberculosis drugs. After three months of treatment, the patient returned to the hospital with persistent symptoms. Direct examination of the sputum was positive and a rifampicin-resistant M. tuberculosis complex isolate was detected by GeneXpert® MTB/RIF lab test (Cepheid, Sunnyvale, CA). The patient was hospitalized and treated with daily kanamycin (1 g), moxifloxacin (400 mg), prothionamide (250 mg), clofazimine (100 mg), isoniazid (300 mg), ethionamide (250 mg) and pyrazinamide (400 mg), the doses being adjusted to the patient’s weight. On July 2016, a first positive MGIT (Becton Dickinson, Le Pont-de-Claix, France) culture obtained from sputum yielded strain 5175 identified as a M. tuberculosis complex isolate by SD BIOLINE TB Ag MPT64 rapid test® (Standard Diagnostics, Inc., Seoul, South Korea). In September 2016, improved clinical course contrasted with the positivity of sputum cultures in MGIT but the patient was readmitted to the hospital in November 2016 for persisting cough. In January 2017, the patient was diagnosed with treatment failure and was treated with kanamycin, levofloxacin, cycloserine, linezolid, para-aminosalicylic acid (PAS) and bedaquiline and Directly Observed Treatment (DOT) follow-up. Complementary microbiological investigations confirmed antibiotic-resistant M. tuberculosis and occasional isolation of strain FB-527 from respiratory material during follow-up.
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pmc-6203853-1
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The defendant was a 24-year-old woman, JF, who was charged with murder for smothering her newborn child to death immediately after delivery. She then wrapped the infant’s body in a towel and hid it inside a suitcase. The defendant later claimed that the newborn child was ‘born dead’ due to drug abstinence syndrome (see below).
Anamnestic information revealed that the defendant started to heavily smoke cigarettes at the age of eleven. She had a well-documented history of multidrug abuse, as well as alcohol abuse, since the age of thirteen. She had become pregnant during a party and did not interrupt her drug use during pregnancy.
The forensic psychiatric examination revealed that the defendant had a personality profile characterized by antisocial features such as a history of illegal behaviour, sensation seeking, familial conflict, lack of sensitivity, rejection of conventional standards, poor response to threatening situations and a willingness to forgo careful consideration of alternative solutions to problems. The neuropsychological evaluation revealed impulsivity, i.e., inability to inhibit the prepotent response (as measured by means of the Hayling test); a deficit in planning (as measured by means of the Tower of London); and deficits in emotional attribution and in identifying violations of social norms (as measured by the emotion attribution task and social situation task).
The structural MRI of the defendant was compared with the MRI of healthy women using voxel-based morphometry (VBM), a neuroimaging technique that highlights subtle structural anatomical abnormalities. The analysis revealed a reduced grey matter volume in the left prefrontal cortex in JF relative to the control group (Fig. ).
According to the defence consultant, the sNI data in this case were fundamental in explaining the constellation of symptoms that characterized the defendant’s history. Indeed, the site of the brain alteration (i.e., the frontal lobe) has a strong anatomo-clinical correlation with the descriptive diagnosis, which can be summarized as frontal syndrome, as the defendant manifested symptoms ascribable to frontal lobe dysfunction, particularly impulsivity, a deficit in planning, a deficit in automatic response inhibition and difficulty in emotional attribution, as well as alteration of the personality characterized by high scores on the borderline, depression and substance abuse subscales. Furthermore, according to the false-positive distribution map, the decrease in grey matter in the dorsolateral prefrontal cortex (DLPFC) in a single individual compared to the control group has only a 0.5–5% probability of being a false positive.
The presence of the brain alteration in the frontal lobe is also important from the crimino-dynamic prospective: the symptoms identified in JF showed that she had a reduced capacity to control her behaviour. This was extremely relevant for the evaluation of the prosecutor’s hypothesis, according to which JF impulsively smothered the newborn to death. Thus, the clinically identified pathological features are causally linked to the crime, providing the basis for an insanity defence. JF’s behavioural reports, accompanied by reduced grey matter in the frontal lobe, provided “hard” biological evidence for the defence’s case.
In summary, the defence consultants concluded that the constellation of behavioural and neuropsychological symptoms manifested by JF were in strong anatomo-clinical correlation with the brain abnormalities and were causally linked to the crime. According to the defence consultants, JF should be considered not responsible for her behaviour because she was unable to inhibit her impulses.
A woman was charged with murder for running over a man with her car. She underwent psychiatric and psychological evaluations to understand her state of mind at the moment of the homicide. The descriptive diagnosis gave a negative result. The defendant’s brain MRI, analysed using VBM methodology, revealed an increase in grey matter in the DLPFC, particularly in the middle frontal gyrus (Fig. , left panel). In the absence of any descriptive diagnosis, this result can be interpreted only according to the literature. Since an increase in DLPFC grey matter has been linked to binge drinking (Fig. , right panel), it would be tempting to infer that the defendant was a binge drinker and that the accident occurred because she was drunk.
This conclusion and reasoning are invalid, since inferring the presence of altered mental states (being a binge drinker) from the presence of a brain alteration (increased grey matter in the DLPFC) is a logical fallacy called reverse inference. This fallacy is particularly relevant in neuroimaging studies, where behaviours are subserved by complex brain networks and each brain region is consequently involved in more than one behaviour. Furthermore, according to the false-positive distribution map, an increase in grey matter in the DLPFC in a single individual compared to the control group has up to a 23% chance of being a false positive. Thus, this result, which has no clinical correlate (i.e., is not in anatomo-clinical correlation with the descriptive diagnosis), is likely to be a false positive result, probably reflecting normal variability in neuroanatomy rather than any brain pathology. Indeed, this case was fictional, and the VBM results reported highlighted the grey matter increase in the brain of one of the authors (CS) compared to a control group.
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pmc-6203866-1
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A 59-year-old woman was referred with the history of exertional dyspnea. She also had left-sided chest pain and BIPAP ventilation device dependence. Primary studies found a huge mass in left hemi thorax (). The CT-guided biopsy had been done for the patient and solitary fibrous tumor was the final pathologic diagnosis. Open surgical excision was planned. Except for the breathing rate of 20/minute, vital signs were normal. The pronounced disorder was seen in PFT and FEV1 that was only 20% predicted value. The patient underwent general anesthesia before connecting to ECMO machine. ECMO was used via femoral artery and vein through Seldinger technique. After positioning of the patient to lateral decubitus marked hemodynamic and oxygenation derangement occurred so operation continued by the assistance of ECMO. The huge mass was excised via posterolateral thoracotomy. Complete excision was uneventful and during operation, the patient was completely stable. ECMO was continued for about 3 hours postoperatively to assure hemodynamic and blood gas stability before weaning. The mass sized 18×16×10 cm with 1670 g weight. There was no major complication and O2 saturation was 94% immediately after the operation. She extubated 12 hours after ICU transferring. There was no major post-operative complication and liver function tests (LFT) were in normal range and except in mild anemia that managed conservatively there was no other abnormality in laboratory tests. At 10-month follow up there was no problem and the patient has no dyspnea and lungs were completely expanded and there was no signs and symptoms of tumor recurrence.
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pmc-6203866-2
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A 45-year-old man with the 2 years history of exertional dyspnea, dry cough and a huge mass in right hemi thorax was referred to our center. The mentioned mass had a fatty density at CT scan while shifted the heart to the left (). CT guided biopsy revealed the diagnosis of lipoma. FEV1was 0.65liter and respiratory rate were 20/minute but other vital signs were within normal range. Due to huge mediastinal soft tissue mass (lipoma) and pleural effusion along with the mentioned symptoms, surgical resection was planned for the patient. According to low oxygen saturation and dimension of mass, ECMO was used at the beginning of the operation. Cannulation was done from right femoral artery and vein by a cardiac surgeon after anesthesia induction to use ECMO. At the time of operation, a very large ovoid encapsulated tumor with fat consistency was excised through a posterolateral thoracotomy. He had an O2 saturation of 96% and his condition was stable postoperatively so using of ECMO discontinued at the end of surgery. After six hours, right sided re-expansion pulmonary edema developed which was managed by intubation and mechanical ventilation. Early post-operative anemia managed by packed cell transfusion although there was no major bleeding at this time. All other laboratory tests were normal. The patient discharged two weeks after surgery after successful weaning and discontinuing mechanical ventilation. The final pathologic report confirmed the diagnosis of low-grade liposarcoma. According to oncology consult the patient did not need adjuvant therapy. Nearly after two years of surgery the patient is completely symptomless and there is no tumor recurrence.
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pmc-6203866-3
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A 13-year-old boy with a one-month history of chronic cough in addition to dyspnea referred by oncology service. Vital signs and O2 saturation were rather normal except the respiratory rate of 26/minute. CT scan was done that showed a huge mass with compression effects on the heart (). CT guided biopsy was undiagnostic so we performed an incisional biopsy that was reported aneurysmal bone cyst. At echocardiography, there was observable compression of the mass on right atrium and inferior vena cava. Peripheral ECMO was applied through cannulation of left femoral artery and vein under general anesthesia. After ECMO installation a wide posterolateral thoracotomy was done. Diaphragmatic and pulmonary adhesion with pulmonary collapse were seen. Large hemorrhagic tumor was excised completely with adjacent seventh and eighth ribs as the origin of the tumor. The patient was stable after the surgery with 100% O2 saturation without the need of using ECMO in the postoperative period. According to hemorrhagic nature of tumor, bleeding was modest at the time of operation but after complete resection of the tumor, there was no major bleeding but due to low hemoglobin level (Hg=6 mg/dL) packed cell transfused. He was extubated in the operating room after ECMO removal and finally was discharged on the sixth postoperative day. The final pathologic diagnosis was compatible with telangiectatic osteosarcoma and at the present time adjuvant chemotherapy has been administered for him by oncologist.
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pmc-6203868-1
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A 24-year-old woman, following motor vehicle collision (MVC), was admitted to the Emergency Medicine Department of Imam Reza hospital, Tabriz, Iran. She was fully oriented with a GCS of 15. Her vital signs were within normal ranges. She complained of mild cervical pain. In neurologic examination, she had no focal neurological deficits. Primary and secondary trauma care was provided. Diagnostic studies including Brain CT scan, cervical and chest radiography and FAST examination were performed which were of unremarkable findings. Consequently, she was discharged after being under observation for 8 hours. Nevertheless, the patient was readmitted to the emergency department with left hemiplegia two days later. Doppler sonography of right vertebral artery reported the probability of dissection or thrombosis; however, CT angiography (-) confirmed the occlusion of the right vertebral artery. Patient was admitted to the neurology ward. Immediately, heparin (1000 unit per hour) was initiated; titration was performed to a PTT target range of 50-70 seconds. Subsequently, warfarin (5 mg per day) was initiated to reach the target INR of 2-3. Additionally, atorvastatin 40 mg was prescribed due to the unknown etiology of the stroke on admission. Cholesterol lowering agents are not considered as part of the standard treatment regimen in patients with large vessel dissection. Nevertheless, we decided to continue atorvastatin as our patient also suffered from dyslipidemia. Physiotherapy of the left extremities was initiated. Her left hemiplegia recovered after four days and she was consequently discharged with 5/5 muscle forces. Warfarin was continued for her after discharge.
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pmc-6203901-1
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A 22-year-old woman with no general health problems was referred by her dentist to the Endodontic Department, School of Dentistry, Feira de Santana State University. The patient complained of painful swelling on the mucosa over the maxillary right lateral incisor. Clinical examination revealed a sinus tract (Fig. A), and the tooth did not respond to cold thermal test (Endo-Frost; Roeko, Langenau, Germany), as well as presented hypersensitive response to percussion and palpation, although adjacent teeth all responded within normal patterns. Periodontal probing depths were normal (< 3 mm).
Radiographic examination revealed a large periradicular radiolucency, an open apex and a complex canal anatomy with type II DI (Fig. B). Cone-beam computed tomographic (CBCT) scan was requested as a complementary examination to avoid possible complications during treatment (). Sagittal and axial CBCT images showed the invagination extending beyond the cementoenamel junction, reaching the pulpal space and periapical radiolucency disrupting the bone cortical palatal (Fig. 1C,D). The patient was informed of the diagnosis and the need for root canal treatment.
Root canal treatment was performed under local anesthesia using 2% lidocaine with 1:100,000 epinephrine (Nova DFL; Taquara, Rio de Janeiro, Brazil). A rubber dam was placed, and the access cavity was performed using diamond burs in high-speed rotation. The invaginated tissue was carefully removed using an ultrasonic tip (E3D; Helse Dental Technology, São Paulo, Brazil) (Fig. A) with the aid of a dental operating microscope (Alliance; São Carlos, São Paulo, Brazil). The canal was copiously irrigated with 2.5% NaOCl and the working length (WL) was established electronically with an apex locator (Root ZX; J Morita USA Inc, Irvine, CA) using a size 50 K-file (Dentsply Maillefer, Ballaigues, Switzerland). The XP-endo Finisher instrument was placed in a contra-angle hand piece (VDW, Munich, Germany), cooled (Endo-Frost), removed from the plastic tube and inserted in the canal without rotation. Then, the rotation was initialized (800 rpm and 1 Ncm), and the instrument was activated for 1 min using a slow and gentle 7-8 mm lengthwise movements up to the WL. The instrument was brushed against the sidewalls of the canals during the instrumentation (Fig. B,C). This cycle was repeated three times. The canal was irrigated with 5 mL of 2.5% NaOCl using a 30-G NaviTip needle (Ultradent Products Inc, South Jordan, UT, USA) up to 3 mm short of the WL after each cycle. The smear layer was removed by rinsing the canal with 2 mL of 17% EDTA for 3 min followed by 5 mL of 2.5% NaOCl. Finally, a 3 mL rinse with bidistilled water was used in the final irrigation to flush out the NaOCl. After that, the canal was dried with sterile absorbent paper points (Dentsply Maillefer), filled with calcium hydroxide (Ultracal; Ultradent Products Inc), and sealed with a temporary filling material (Cavit; 3M ESPE, Seefeld, Germany).
One month later, at the second appointment, the tooth was asymptomatic and the sinus tract had disappeared. After access, the canal was copiously irrigated with 2.5% NaOCl and another cycle using the XP-endo Finisher instrument was performed to remove the calcium hydroxide. An apical plug of mineral trioxide aggregate (MTA) Repair HP (Angelus Dental Solutions; Londrina, Paraná, Brazil) with approximately 3 mm was performed. The remaining pulp space was then carefully filled with gutta-percha (VDW) and AH Plus sealer (Dentsply De Trey, Konstanz, Germany) using the continuous wave of condensation technique. The continuous wave of condensation technique was performed using a fine tip plugger of the System B, which was heated to 200°C and taken to a depth 6-mm short of the apical plug. The tip was allowed to cool for 15 s, and a single burst of heat was applied for 1 s and the tip was removed. The canal was completely backfilled with Obtura II gutta-percha (Spartan, Fenton, MO) with the unit set at 200°C.The access was sealed with a temporary filling material (Cavit), a final radiographic was performed (Fig. D) and the patient was referred to her general dentistry. The patient returned for clinical and radiographic examinations after six (Fig. A) and fourteen months (Fig. B). Another CBCT scan was performed after fourteen months (Fig. C,D). Periapical radiograph and CBCT images revealed evidence of advanced healing and periapical repair.
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pmc-6203909-1
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A 19-year-old man was referred to the Department of Oral and Maxillofacial Surgery at Nara Kasuga Hospital with a complaint of swelling at the left submandibular region. He had felt pain and swelling at the left molar region several times in the previous 2 years. He consulted his internist at the Department of Infectious Disease at Nara City Hospital and was administered antibiotics and analgesics. The patient was strongly recommended to undergo dental treatment. However, he refused to receive dental treatment due to dental anxiety. When the pain and swelling developed, he presented at otolaryngology department at Nara City Hospital and was treated with intraoral incision and drainage.
The patient was diagnosed with HIES at 1 month of age and had been followed-up by the Department of Pediatrics until the age of 18. Genetic analysis for signal transducer and activator of transcription 3 (STAT3) mutation was positive. He had multiple episodes of subcutaneous abscess on his lower legs and buttocks, perianal abscess, recurrent pneumonia, and osteomyelitis of the foot. Cultures of his abscesses revealed methicillin-resistant Staphylococcus aureus in most cases. These infections usually became severe and showed slow improvement. Delayed primary tooth shedding was noted by his dentist. The patient’s medications included minocycline hydrochloride (200 mg/day), levocetirizine dihydrochloride (5 mg/day), domperidone (30 mg/day), and amphotericin B.
On physical examination, the patient’s body size was smaller than normal for his age and sex. His face was coarse, with a prominent forehead and broad nasal bridge. Diffuse swelling was observed in the left submandibular area. Oral examination disclosed gingival swelling, candidiasis at the palatal and buccal mucosa, and multiple dental caries (Fig. ). A panoramic radiograph revealed a large periapical bone resorption involving the lower left first and second molars (Fig. A). The clinical diagnosis was perimandibular abscess due to periapical periodontitis of the left lower first and second molars. He underwent root canal treatment several times; however, he stopped visiting our hospital.
The patient felt submandibular swelling and dysphagia 5 months after his last visit to our hospital and consulted his otolaryngologist. Swelling was observed extending from the left submandibular region to the submental area. A computed tomography scan showed an area of bony destruction in the left mandible and abscess formation in the submandibular and submental spaces (Fig. B,C). Laboratory examination showed an infection, with a white blood cell count of 13,000/mm3 and C-reactive protein of 8.0 mg/dL. His previous laboratory reports were unremarkable except for significantly increased IgE levels (4000-8000 IU/mL). Under a diagnosis of submandibular and submental abscess, the patient was admitted to the Otolaryngology Department of Nara City Hospital. Drainage and necrotomy of the neck abscess was performed under general anesthesia by otolaryngologists. Microbiological culture yielded Peptostreptococcus spp., Escherichia coli, and Clostridium spp. Intravenous administration of sulbactam/ampicillin 3 g 4 times daily was provided for 3 days. C-reactive protein markedly decreased to 0.98 mg/dL 3 days after drainage and necrotomy. Four days after drainage and necrotomy, the patient was referred to our hospital. The left first and second lower molars were extracted and the periapical lesion was curetted under intravenous sedation with midazolam by the oral and maxillofacial surgeon. Additional intravenous administration of sulbactam/ampicillin 3 g 4 times daily was provided for 3 days, followed by oral administration of amoxicillin hydrate (3 g) and amoxicillin-clavulanic acid (3 g) for 30 days, with reference to the dose and duration of antibiotics for the treatment of previous subcutaneous abscesses. The patient was transferred to our hospital and admitted to the Department of Oral and Maxillofacial Surgery 7 days after drainage and necrotomy. The treatment of multiple dental caries and periodontitis was done under intravenous sedation 3 times per week. Postoperative healing was uneventful and the patient was discharged 4 weeks after surgery. He continued to undergo restorative and prosthodontic treatment. At 7 months after surgery, he showed no signs of intraoral infection. A panoramic radiograph showed bone regeneration at the left posterior mandible and no periodontal lesions of the teeth (Fig. ).
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pmc-6203924-1
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The patient was a 68 years old woman suffering from depression, hypothyroidism, arrhythmias and hypercholesterolemia, pharmacologically controlled with clomipramine 25mg (0-0-1), lormetazepan 2mg (0-0-0.5), fluoxetine 20mg (1-0-0), levotiroxin 100mg (1-0-0), bisoprolol 2.5mg (1-0-0) and simvastatin 20mg (0-0-1). She did not have toxic habits neither allergies. The patient attended the dental clinic because of pain on the right side of the upper jaw. She wore a fixed upper-arch implant supported prosthesis placed five years ago and she had not attended the control visits for the last 2 years (Fig. ). The clinical exam after removing the prosthesis showed intense accumulation of plaque (both in the prosthesis and in the intermediate abutments) and a mucosal enlargement with suppuration on palpating the vestibular area of the implant in position 1.5 and a probing depth of 8mm. The implants in position 2.4 and 2.5 also showed vestibular mucosal enlargement and a probing depth of 6mm. Periapical radiographs showed no changes on the peri-implant bone level. Therefore, it was decided to perform a surgical treatment of the implant 1.5 under local anesthesia (articaine 4% and epinephrine 1:200.000) with a full-thickness trapezoidal flap. After rising the flap, a correct bone level and the absence of exposed implant threads were observed. Hence, the thickness of the vestibular flap was reduced and the flap was repositioned with 4/0 monofilament suture. On the left side, an incisional biopsy was made in order to reduce the vestibular thickness and send the sample for the histological study (Fig. ). The presumptive diagnosis was gingival hyperplasia due to plaque accumulation.
The lesion was immersed in a 10% formaldehyde solution and sent to the Oral and Maxillofacial Pathology and Diagnosis Service (SDPOMF) for the histopathological exam.
The histopathological exam found that the lesion was mainly constituted by fibrocellular collagen connective tissue with scarce cellularity and a diffuse and mild lymphoplasmacytic chronic inflammatory infiltration. The superficial mucosal epithelium was parakeratinized and hyperplastic but without any dysplastic phenomena (Fig. A,B). Hence, the definitive diagnosis was fibro-epithelial hyperplasia with inflammation. No recurrence has been reported after a 6 month follow-up.
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pmc-6203963-1
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A 47 years old man presented to our institution after three episodes of hemoptysis. The past medical and family history and Physical examination were unremarkable. Chest X-ray showed wide mediastinum and prominent pulmonary conus. A transthoracic echocardiography showed normal LV systolic function with ejection fraction of 60%, no valve lesion, normal RV size and function and no pulmonary hypertension (PHT) (Additional file : Video). Chest computed tomography scan (CT) revealed aneurysmal dilatation with pending rupture of the pulmonary artery trunk (4.5 cm), the left pulmonary artery (6 cm) and the right pulmonary artery (2.3 cm) (Figs. and ). Cardiac catheterization showed normal coronaries with no signs of compression by the adjacent pulmonary artery aneurysms (PAAs). Acquired causes of pulmonary artery aneurysm, such as syphilis, tuberculosis, Behcet’s disease, Marfan syndrome and vasculitis of pulmonary artery, were all ruled out.
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pmc-6203970-1
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A previously healthy 48-year-old woman developed sudden and painless visual loss and optic disc edema in the right eye (OD). A tentative diagnosis of ON was made and high-dose intravenous methylprednisolone was administered for 5 days, followed by oral prednisone therapy, but to no avail. Three weeks later a similar event occurred in the left eye (OS). Oral corticosteroid treatment was maintained for the following 4 months with only slight improvement in vision. The disc edema resolved and was followed by severe optic atrophy. Neurologic examination, brain and orbits computerized tomography and magnetic resonance imaging (MRI) scans, cerebrospinal fluid (CSF) analysis and extensive laboratory investigation including anti-aquaporin 4 antibody assay were unrevealing, except for a weakly positive antinuclear antibody test (1/160, granular pattern). Because of sequential involvement and the lack of significant improvement the patient was considered to have atypical ON, presumably from NMO-spectrum disease. Oral azathioprine 150 mg/day was introduced and kept for the following years. Her visual function remained stable, and significant peripapillary RNFL loss developed on sequential spectral-domain (SD) OCT examinations, directed at quantifying peripapillary RNFL. However, high-resolution OCT scanning passing through the optic nerve were not obtained.
Five years later, the patient was seen for the first time by us for a second opinion regarding her condition and her current treatment. Her vision was stable and she had no new complaints. Upon examination, visual acuity (VA) was 20/20–2 in OD and 20/25 in OS. A mild relative afferent pupillary defect was present in OS. Extraocular motility, slit lamp findings and intraocular pressure measurements were normal. The fundus examination showed diffuse disc pallor in both eyes (OU). The visual field (VF) test revealed marked sensitivity loss in the upper and lower nasal quadrants, enlarged blind spot in OD, and marked lower nasal and temporal defect associated with diffuse VF depression in OS. Swept-source (SS) OCT showed severely reduced peripapillary RNFL thickness in OU. OCT radial B-scans passing through the disc showed ovoid areas of reduced reflectivity with some hyper-reflective areas in the retrolaminar region of the disc in both eyes (Fig. ). The findings were consistent with a diagnosis of ODD complicated by NAION. Azathioprine treatment was discontinued and she was advised to continue periodic ophthalmic examination.
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pmc-6203970-2
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A 35-year-old woman presented with a complaint of a small dark spot laterally in OS. Taking into account her history of acute visual loss in OD 10 years earlier, left optic disc edema from ON was suspected by her ophthalmologist and the patient was referred to us for neuro-ophthalmic evaluation. Ten years previously, she had acute VF loss and optic disc swelling in OD which regressed completely after treatment with intravenous high-dose methylprednisolone for 5 days. However, since her visual loss remained unchanged she was tentatively diagnosed with atypical ON by her previous physicians. After extensive laboratory testing including anti-aquaporin-4 antibody, MRI and CSF examination were normal, close neurological follow-up was prescribed justified by the suspicion of seronegative NMO-spectrum disease.
Upon ophthalmic examination, VA was 20/20 in OU and the pupils reacted to light and near stimuli with a relative afferent pupillary defect in OD. Extraocular motility, slit lamp examination and intraocular pressure measurements were normal. The fundus examination showed diffuse optic disc pallor with a peripapillary depigmented halo around the disc in OD and mildly blurred disc margins in OS. A small round vitreous opacity presumably due to vitreous detachment was found in OS. The VF examination disclosed an arcuate dense pericentral defect in OD and was within normal range in OS. Spectral-domain (SD) OCT showed severely reduced peripapillary RNFL thickness in OD and mildly increased RNFL thickness in the superior quadrant of OS. Enhanced-depth imaging (EDI) horizontal and vertical OCT B-scans passing through the disc showed large ovoid areas of reduced reflectivity with some hyper-reflective regions in the retrolaminar region of the optic disc in OD (Fig. ). The OCT scans also revealed a small prelaminar area of reduced reflectivity and some deeper areas in OS, suggesting ODD in both eyes, complicated by NAION in OD. Mildly increased RNFL thickness in OS was attributed to ODD and her recent complaint of a small dark spot in her vision was due to vitreous opacity. No evidence of NAION in OS was found and the appearance of her disc remained stable on follow-up examinations.
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pmc-6203978-1
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A 62-year-old man was diagnosed with end-stage renal disease 20 year ago, and a native AVF was created at the left wrist for hemodialysis treatments. One year later, the patient received his first kidney transplant in the right iliac fossa and took a combination of immunosuppressive medications (including azathioprine, cyclosporin A, and corticosteroids). After that, the AVF was abandoned. Fifteen years ago, physical examination found that the AVF was occluded. At 6 years after renal transplantation, the patient suffered from lower extremity edema and was diagnosed with acute kidney transplant rejection based on renal biopsy. The failed transplanted kidney was surgically removed, and the patient received his second kidney transplant in the left iliac fossa. After the transplantation, the patient continued immunosuppressive therapy, with serum creatinine levels ranging between 120 and 130 μmol/L.
Eighteen months ago, the patient presented with orthopnea, nausea, and vomiting, and was admitted to the Department of Nephrology. On physical examination, there was no thrill or pulse over the AVF and no bruit on auscultation. Vascular ultrasound examination revealed the patent brachial (Fig. , blood flow: 101 mL/min) and radial (Fig. , diameter: 1.1 mm) arteries and the totally occluded AVF with no blood flow (Fig. ), which was illustrated in the skin (Fig. ). Serum creatinine concentration was 853 μmol/L and blood urea nitrogen was 34.1 mmol/L. The patient was diagnosed with renal allograft failure, and immediate hemodialysis restart was required. To promptly prepare a vascular access, we decided to attempt percutaneous revascularization of the patient’s chronically occluded AVF. Since we already had successful experience in treating stenotic and acutely thrombosed AVFs with ultrasound-guided transluminal angioplasty, we performed the revascularization under ultrasound using the Apollo 500 system (Toshiba, Tokyo, Japan), equipped with a 9–18 MHz linear transducer probe. The AVF was retrogradely punctured with a 22-G needle, and a 6-Fr sheath (Terumo, Tokyo, Japan) was retrogradely inserted into the fistulous vein (Fig. ). At first, a 0.014-in. Hi-Torque BMW Elite guidewire (Abbott Vascular, Santa Clara, CA, USA) was advanced to the lesion, but failed to cross the chronic total occlusion lesion at the AVF after switching the wire tip direction several times (Fig. ). An additional movie file shows this in more detail (see Additional file ). With the support of a 6-Fr angiographic catheter advanced to the occlusion site, the guidewire was carefully and gradually inserted the chronic total occlusion lesion and successfully crossed the lesions at the fistula, anastomosis, and the radial artery, and parked in the radial artery (Fig. ). An additional movie file shows this in more detail (see Additional file ). Subsequently, the radial artery was dilated with a 4.0 mm × 20 mm TREK balloon (Abbott Vascular) three times at 4, 8, and 12 atm (Fig. ), and the occluded lesion was dilated at 12, 18, and 20 atm. Then, further dilation of the lesion was performed with a 6.0 mm × 40 mm TREK balloon (Abbott Vascular) three times at 12 and 20 atm (Fig. ). The procedure took 54 min. After the dilation, the diameter and blood flow of the radial artery were 3.3 mm and 138 cm/s, respectively; and the cephalic vein diameter and blood flow were 2.9 mm and 251 cm/s, respectively. The diameter of the anastomose site was 3.2 mm (Fig. ).
The revascularized AVF was used for hemodialysis after the intervention and remains patent for as long as 18 months. At the 2-, 6-, and 18-month follow-ups, the anastomose site diameter and AVF blood flow were 3.8 mm and 340 cm/s (Fig. and ), 3.8 mm and 291 cm/s (Fig. and ), and 0.8 mm and 255 cm/s (Fig. and ), respectively. Although the anastomose site was stenotic at 18-month visit, the proximal AVF segment was normal with a diameter of 4.5 mm.
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pmc-6203990-1
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A 19-year-old male was a student and presented with a reduced right-eye vision for 6 months. The male was Han Chinese nationality. He did not have any systemic disease cand family history. The result of head magnetic resonance imaging (MRI) was normal. Systemic investigations did not show any evidence of von Hippel-Lindau disease. His best-corrected visual acuity (BCVA) was 0.02 and 1.0 for the right and left eyes, respectively. The fundus examination of the right eye revealed an elevated reddish lesion measuring 3.3 mm × 3.2 mm at the optic disc, with the surrounding subretinal fluid (SRF) and exudation extended to the macular region (Fig. ). No other abnormalities were found in the retina of the other eye. Fluorescence angiography (FA) and Indocyanine green angiography (ICGA) demonstrated hyperfluorescence of the tumor vessels in the early phase and a continuous leakage in the late phase of the angiogram, thereby confirming the diagnosis of JRCH (Fig. ). Optic coherence tomography (OCT) revealed an extensive serous retinal detachment, and the central foveal thickness was increased to 830 μm (Fig. ). Thus, JRCH was diagnosed.
After discussing the treatment options with the patient, standard PDT was performed using Visudyne infusion at a dose of 6 mg/m2 body surface area for 10 min. Subsequently, after 5 min, light exposure was performed with a diode laser (5000 μm) at 600 mW/cm2 for 83 s. The spot size was adjusted to encompass the largest diameter of the hemangioma without extension into the surrounding retina.
One month after the first PDT, the BCVA of the patient was 0.1 and 1.0 for the right and left eyes, respectively. The fundus examination of the right eye showed a reduced size of the JRCH, approximately measuring 3.0 mm × 2.0 mm. In addition, a significant decrease in the SRF was observed. OCT revealed that the central foveal thickness was decreased to 277 μm (Fig. ). After 3 months of the first PDT, the subfoveal fluid was reduced but not disappeared. Subsequently, we applied a second PDT with a spot size of 2700 μm based on the same strategy as the first session.
One month after the second PDT, the BCVA in his right eye improved to 0.2. OCT revealed that the subfoveal fluid was disappeared and the central foveal thickness was 149 μm (Fig. ). The structure of the macular ellipsoid zone of the photoreceptors in the right eye was discontinued, and cystoid changes in the inner and outer nuclear layers were observed. Up to the final follow-up at 18 months, the BCVA of the patient’s right eye had stabilized to 0.8. The fundus examination of the right eye showed that the size of the JRCH was 2.0 mm × 1.5 mm (Fig. ). The results of OCT showed that the structure of the macular ellipsoid zone of the photoreceptors in the right eye was better than before; however, cystoid changes in the inner and outer nuclear layers persisted (Fig. ). The final visual field examination showed an enlarged blind spot in the right eye. Presently, the patient is under continuous follow-up.
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pmc-6204022-1
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Case 1 was a 33-year-old Caucasian male. He was a pilot and flew routes between France, Guinea and the Republic of the Congo. In September 2016 he presented with fever, headaches and vomiting, and thereafter received treatment in Paris for severe malaria (positive thick drop for P. falciparum with 5 parasites/2 μL, positive HRP2 antigen test) with hepatic impairment (SGOT/SGPT 92/105 U/L and hyperbilirubinaemia (93 µmol/L, normal range < 25 µmol/L) but no neurologic involvement or any other severity criteria. The treatment regimen included intravenous artesunate (2.4 mg/kg, 5 doses for 3 days) then atovaquone/proguanil (1000/400 mg per day for 3 days), and the patient improved quickly, both clinically and biologically (blood smear negative for P. falciparum on day 3). On day 7, he presented headaches and fever (38 °C) and on day 8 abdominal pain, nausea and vomiting. The renewed blood smear was negative. On day 10, the patient showed confusion, ataxia, tremor, and dysarthria, and his fever increased to 39 °C. On day 11, he was given ceftriaxone for presumed enteric fever. On day 12, he remained confused and started having visual hallucinations and urine incontinence. CSF analysis showed lymphocytic meningitis (Table ), MRI was normal and EEG revealed asymmetric (right) frontal slowing. Laboratory results showed no inflammation, a slight hyperbilirubinaemia that diminished over the first days and a weak positive titre of anti-nuclear factor (1/80) with no positivity for anti-DNA. Thereafter, he was treated with cefotaxime and acyclovir from day 12–21 (until a second CSF analysis showed no viral or bacterial infection), and corticosteroids from day 15–30 (methylprednisolone 500 mg/od for 3 days then prednisone 1 mg/kg/od), with clinical improvement on day 19. The patient was discharged with only a slight residual cerebellar ataxia on day 29 and had fully recovered on day 60.
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pmc-6204022-2
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Case 2 was a 29-year-old Caucasian male who worked in Ivory Coast. He presented a first episode of acute falciparum malaria without severity criteria in July 2013. That episode was treated with a 3-day course of atovaquone/proguanil (standard treatment). The patient experienced a second symptomatic episode with a positive blood smear (0.18%) 3 weeks later and received a 3-day course of artemether/lumefantrine with good outcome. He then consulted on day 37 for vomiting and a 40 °C fever for which intravenous quinine was initiated despite a negative blood smear. On day 38, he presented convulsions and a severe alteration of consciousness requiring sedation and ventilation. He was evacuated to Paris where his anti-malarial treatment was changed to artesunate despite a still negative blood smear. On day 42, sedation was stopped but the patient presented visual hallucinations and generalized convulsions and consequently had to be re-sedated. The blood test was again negative for malaria but an HRP2 antigen test was positive. T2 and T1 gadolinium-enhanced MRI sequences on day 48 showed hippocampal lesions (Fig. ), EEG diffuse slowing, and CSF analysis lymphocytic meningitis (Table a). Differential diagnoses such as infectious or inflammatory/immunological diseases were ruled out (tests for all of the following were negative or normal: HSV, EBV, VZV, HIV, VHB, VHC, HHV6, adenovirus, dengue fever, Chikungunya, Rift Valley fever virus, West-Nile virus, Borrelia, Coxiella, Brucella, Bartonella, Tropheryma whipplei, gram-negative bacilli, TPHA-VDRL, African trypanosomiasis, cysticercosis, toxocariasis, anti-nuclear factor (ANF), anti-neutrophil cytoplasmic antibodies (ANCA), complement, anti-phospholipid (APL) antibodies, anti-neuronal antibodies including NMDA-receptor in CSF, rheumatoid factor, anti-CCP, angiotensin conversion enzyme and 16S RNA on CSF). The patient improved around day 54 without specific treatment. MRI on day 63 had returned to normal. On day 73, the patient was free of neurological symptoms.
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pmc-6204022-3
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Case 3 was a 36-year-old Caucasian female who lived in France but had visited friends in Mali in November 2009. On 17 November, she complained of fever, diarrhoea and vomiting and on the 18th returned to Paris. She was diagnosed with non-severe (2% parasitaemia) falciparum malaria on 28 November and treated with a 3-day course of atovaquone/proguanil with good outcome. Thirteen days later (day 15) she became confused, aphasic and anosognosic. CSF analysis showed lymphocytic meningitis (Table ). EEG showed frontal bilateral slowing and MRI no abnormalities. Infectious investigations were all negative (including HSV, VZV and enterovirus, HIV, Chikungunya, dengue, West Nile and Rift Valley fevers, yellow fever and African trypanosomiasis). Intravenous acyclovir was stopped after 2 days following the negative result for HSV PCR in CSF. On day 19, she was discharged with partial recovery. On day 22, she was re-admitted for confusion, aphasia, ataxia, delirium, and fever (38 °C). MRI remained unremarkable but EEG showed major frontal slowing and spike-and-wave discharges. Treatment with acyclovir and levetiracetam was re-initiated for 3 weeks. The patient’s clinical status gradually improved allowing for her discharge on day 41. She had fully recovered on day 87.
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pmc-6204022-4
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Case 4 was a 43-year-old Caucasian female who presented with severe falciparum malaria (4.5% parasitaemia) in July 1999 after a trip to Ivory Coast. She was initially treated with intravenous quinine (25 mg/kg/day) and on day 5 was given an oral dose of quinine (500 mg tid) after which she developed hypoacusis. On day 6, she was treated with mefloquine (6 × 250 mg for 24 h). She was discharged after recovery but on day 18 she experienced dizziness, limb weakness, gait impairment, nausea, an increase in pre-existing headaches and an episode where she was unable to read. Tremor and ataxia were observed during a resulting physical examination. On day 21, the level of mefloquine in her blood was high (5 µg/L, HPLC method, normal value < 1.5 µg/L). On day 27, her clinical status improved spontaneously and she was discharged without any specific treatment. Follow-up blood examinations showed that the half-life of mefloquine elimination for this patient was 9 days. On day 41, the patient had a normal clinical status and no sequelae.
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pmc-6204027-1
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A 69-year-old Japanese man underwent treatment for liver dysfunction 3 years after aortic valve replacement. Later, rapid elevation in his serum alkaline phosphatase (ALP) level was recorded and he was readmitted to determine the etiology. His body temperature was 36 °C, blood pressure 164/65 mmHg, and pulse rate was 66/minute. Laboratory data revealed mild anemia and liver-renal injury: white blood cells (WBC) 4600/uL, hemoglobin 9.7 g/dL, platelet 18.9 × 104/dL, C-reactive protein (CRP) 0.29 mg/dL, ALP 1138 U/L, aspartate aminotransferase (AST) 40 U/L, alanine aminotransferase (ALT) 37 U/L, and γ glutamyl transpeptidase (γ-GTP) 298 U/L. His blood urea nitrogen (BUN) was 22.4 mg/dL, creatinine 1.14 mg/dL, activated partial thromboplastin time (APTT) 45.6 seconds, and prothrombin time-international normalized ratio (PT-INR) 2.67. He also had a past history of duodenal ulcer perforation and was currently being treated with warfarin, angiotensin receptor blocker, and proton pump inhibitor. In addition, he was taking orally administered ursodeoxycholic acid for unknown liver function disorder. He had no alcohol consumption or tobacco smoking history and no relevant family history.
A plain radiograph showed no significant findings, but computed tomography (CT) revealed an enhanced tumor within the hilar bile duct and dilatation of the left hepatic duct (Fig. ), which are typical findings for hilar cholangiocarcinoma. In addition, endoscopic retrograde cholangiopancreatography (ERCP) revealed tumor shadow in his bile duct, and the cytology confirmed malignant cells in the bile (Fig. ). As no lymph node and distant metastases were detected, we inserted endoscopic nasobiliary drainage (ENBD) to reduce jaundice as preparation for surgery. We performed extended left hepatectomy with resection of his bile duct; his postoperative course was good without severe complications. After 3 months postoperatively, he was readmitted for subacute cholangitis and obstructive jaundice. Immediately, percutaneous transhepatic cholangiography drainage (PTCD) was performed, followed by cholangiography that exhibited the intrabiliary tumor growth in the remnant liver.
Simultaneously, histological examination of resected specimens revealed tumor growth in the hilar duct across the left hepatic duct (Fig. ). Microscopic findings at the same site revealed a dilated bile duct filled with well-differentiated tubular adenocarcinoma (Fig. ). On immunohistochemical examination, tumor cells were positive for cytokeratin (CK) 20 (Fig. ) but negative for CK7 (Fig. ). Furthermore, CK18 (Fig. ) as control and CDX2 (Fig. ) were stained. Although these findings were not typical of intrahepatic cholangiocarcinoma, hepatic metastasis from another primary lesion was strongly suspected [, ]. Furthermore, CT revealed an enhanced tumor-like lesion at the descending colon, followed by diagnosis of type 2 cancer in total colonography. Then, left hemicolectomy was performed; the immunohistochemical-identified feature matched with an intrabiliary tumor. Meanwhile, the PTCD fluid turned bloody, which was considered to indicate bleeding from a residual bile duct tumor (Fig. ). Accordingly, we planned chemotherapy with orally administered capecitabine but our patient experienced a spike fever because of refractory cholangitis. Thus, we abandoned chemotherapy and initiated radiotherapy to stop the tumor bleeding around the hilar bile duct. After completing radiotherapy (total 50 Gy) for approximately 1 month, we observed an improvement in his liver function because of tumor shrinkage. Unfortunately, the effects were short-lived, intrabiliary growth and cholangitis rebooted after 1 month leading to his death due to septic liver failure (Fig. ). Autopsy revealed a remnant tumor in the bile duct (Fig. ), but no noticeable nodular metastasis was observed, except for a single small metastasis in the lower lobe of his left lung.
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pmc-6204155-1
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A 30-year-old woman, gravida 0, visited our hospital because of right lower abdominal pain. She had no medical history. She had taken oral contraceptives, and her last withdrawal bleeding started 18 days prior. She reported that the abdominal pain had a gradual onset, and her pain severity alternated between severe and mild. The strongest pain intensity experienced was graded as 8 using the numerical rating scale. Transvaginal ultrasonography revealed a 5-cm swollen right ovary, which was suspected to be a mature cystic teratoma, and the absence of ascites at the Douglas' pouch. Pelvic pain examination demonstrated slightly moderate pain for the condition. Contrast-enhanced computed tomography revealed a 44-mm cystic mass containing fat and calcified material in the right pelvis. There was no finding suggestive of torsion, such as irregular thickening of the cyst wall, whirl sign of the right ovarian vein, and deviation of the affected side of the uterus (). However, the possibility of torsion could not be ruled out; thus, an emergent laparoscopic surgery was performed. Intraoperative findings showed a swollen right ovary, but without torsion or congestion. Two small pedunculated 1- and 2-cm diameter paratubal cysts that grew from almost the same place of the ampulla of the right fallopian tube were observed. The thin stalk of the 1-cm paratubal cyst was entangled around the stalk of the 2-cm paratubal cyst, with its head congested, thereby causing the pain (Figures and ). Through a small abdominal laparoscopic incision, the tumor of the right ovary and the two paratubal cysts were excised (). The right ovarian tumor contained fat and hair, and histopathological examination showed that the cyst was a mature cystic teratoma, which was lined with keratinized stratified squamous epithelium and skin appendages, but without an immature component or malignancy. The two paratubal cysts were not malignant.
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pmc-6204162-1
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A 31-year-old gravida 3 para 1 patient presented to antenatal clinic at 19 weeks and 3 days' gestation to discuss the results of her morphology scan which had demonstrated a grade 4 placenta previa covering the cervical os. She had a medical history significant for Arnold Chiari malformation requiring craniotomy in 2006 as well as correction of a Syringomyelia in 2005. She also suffers from irritable bowel syndrome but was taking no regular medications and had a BMI of 23. Her first pregnancy resulted in a spontaneous miscarriage that did not require dilatation and curettage. Her second pregnancy resulted in a planned elective caesarean due to concerns about raised intracranial pressure during labour, as recommended by her neurologist. She had routine antenatal care this pregnancy which had been unremarkable to date.
The morphology scan demonstrated a small omphalocele but otherwise no significant structural defects and estimated foetal weight was noted to be within the normal range. During the clinic review, the fetus was found to have a heart rate well below 100 bpm. Repeat ultrasound the following day confirmed IUFD. This ultrasound also demonstrated evidence of an abnormally invasive placenta with the appearance of dysplastic vascular hypertrophy. An obstetric MRI was performed which supported the diagnosis of morbidly adherent placenta. This showed a low lying inhomogeneous placenta, dysplastic vascular hypertrophy, ill-defined placental bands, and an overall impression of some areas of increta with no overt evidence of percreta.
Options were discussed with the patient who decided for conservative management in order to optimise her chance of preserving her fertility. This was balanced against potential complications of prolonged conservative management of an IUFD, including sepsis and coagulopathy. A plan was made for serial ultrasounds as an outpatient, to be followed by induction of labour when placental blood flow was no longer detectable. Twenty-seven days following IUFD confirmation, the patient was admitted to hospital with abdominal cramping and associated small antepartum haemorrhage (APH).
Ultrasound scan at 31 days showed a minimal reduction in blood flow through the anterior placenta and to the cervix. At day 33 she suffered a further 300mL APH. Given her increasing blood loss and minimal changes to placental blood flow on ultrasound, she was administered a dose of 80mg methotrexate intramuscularly with the hope of accelerating devitalisation of the placenta. A repeat dose of methotrexate was planned for five days' time. During the subsequent two days after the administration of methotrexate, the patient continued to suffer moderate bleeds and increasingly significant contractions. 35 days following IUFD she spontaneously delivered a male fetus with only minimal bleeding during delivery.
A brisk 2 L postpartum haemorrhage (PPH) followed delivery and the patient was immediately taken to the operating theatre for examination under anaesthesia and attempted manual removal. A urinary indwelling catheter was inserted and remained in situ for the entirety of the operation. Due to only partial removal (approximately 80%) of the placenta being achieved manually, the case quickly progressed to laparotomy. Intraoperative findings revealed a full thickness increta at the previous caesarean incision just above the level of the bladder. Hysterotomy was performed with a transverse incision made above the prior caesarean incision, and the remaining placenta was removed manually, creating a 3x3cm plug-like defect anteriorly. This defect was closed with a primary closure separate to the hysterotomy incision. In addition, the placental bed was oversewn to establish haemostasis. A Foley's catheter was inserted vaginally and inflated with 60mL normal saline. Total blood loss was 4 litres (L): 2L immediately postpartum, 1L while attempting per vaginal manual removal of the placenta, and 1L intraoperatively. Massive transfusion protocol was activated with the patient receiving 10 units of packed red cells, 6 units of fresh frozen plasma and 5 units of cryoprecipitate. The patient remained stable throughout the process. A further 3 units of packed cells was given over the next two days for persistent anaemia. The fetus was found to weigh 170g. No cause for foetal demise was identified and the family decided against an autopsy. Pathological examination of the placenta was performed. This was noted to be difficult due to extensive haemorrhage and areas of necrosis commensurate with intrauterine foetal death and prolonged intrauterine retention. There was no evidence of funisitis or umbilical cord vasculitis to support a diagnosis of chorioamnionitis nor were any pathogens observed. The degree of decidual haemorrhage and necrosis made a histological diagnosis of placenta accreta impossible.
The patient recovered without significant complication over the following days and was discharged 1 week later on oral antibiotics and aperients. Six weeks after discharge the patient was seen in a postnatal follow-up clinic. She experienced minimal lochia in the postpartum period and was feeling generally well.
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pmc-6204163-1
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A 70-year-old African American gentleman with a history of CKD, pseudogout, and metastatic castration-resistant prostate cancer (to bone, nodes, and lung) was admitted to the hospital due to left knee swelling. He was incidentally found to be severely hypocalcemic to 2.7 mg/dl with EKG showing a prolonged QTC interval to 525 ms. Physical examination was negative for Chvostek and Trousseau's sign. Home medications included acetaminophen, amlodipine, bicalutamide, docusate-senna, lidocaine patches, ondansetron, and polyethylene glycol. Twenty-eight days prior, he had received his first dose of denosumab (together with leuprolide) when his calcium level was 8.8 mg/dl. He was prescribed vitamin D and calcium supplementation, but never took it. He finished a five-day course of prednisone 10 mg daily thirteen days prior for a pseudogout flare of the right foot. Other labs on admission were significant for phosphorus 5.5 mg/dl, total vitamin D3 31 IU, iPTH 93, and Mg 1.1 mg/dl. He was admitted to the intensive care unit for continuous, high-dose IV calcium gluconate and frequent electrolyte monitoring. During his ICU course, he received a total of 21 grams of IV calcium gluconate. He was also given 1 μg of oral calcitriol BID, 1000 mg of oral calcium carbonate TID, and aggressive magnesium supplementation. Unfortunately, the patient's creatinine worsened to 7.5 mg and he developed severe metabolic acidosis and hyponatremia in the setting of Clostridium difficile colitis. The decision was made to initiate hemodialysis given the poor recovery in kidney function from obstructive uropathy. For one week, he received intermittent hemodialysis with high calcium baths. Calcium, magnesium, and phosphate levels were monitored daily and supplemented as needed. Calcium levels improved to 8.5 mg/dl and magnesium levels improved to 2.0 mg/dl. He was discharged on 0.5 μg oral calcitriol daily, calcium carbonate 1000 mg BID, and continued intermittent hemodialysis.
The patient was originally diagnosed with de novo metastatic prostate cancer two years prior. He had not been on ADT or chemotherapy for approximately one year until he presented to our hospital two months prior with evidence of prostate cancer progression. His PSA was elevated to 1472 and he was found to have acute kidney injury with a Cr 12.8 mg/dl and a BUN of 131 mg/dl due to obstructive uropathy. CT of the abdomen/pelvis showed marked bilateral hydronephrosis due to a 6.3 cm pelvic mass and a 3.8 cm mass in the left ureteropelvic junction. Extensive pelvic and retroperitoneal lymphadenopathy was evident in addition to innumerable metastatic lytic skeletal lesions. Bilateral percutaneous nephrostomy tubes were placed by interventional radiology and the patient's creatinine improved to 7.3 mg/dl with a BUN of 120 mg/dl. Combined androgen deprivation was resumed and he was discharged with plans for close monitoring of renal functions with the ultimate plan to place ureteral stents depending upon renal recovery.
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pmc-6204171-1
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We present a 64-year-old Hispanic male with Rai Stage II, IgH mutated, and trisomy 12 positive CLL, diagnosed 13 years prior to admission. He was initially observed for 3 years and then received first-line fludarabine, cyclophosphamide, and rituximab (FCR) for 3 cycles with good response. He remained on observation for 4 additional years and then received an additional 4 cycles of FCR followed by 5 cycles of maintenance rituximab. Five years later, he was found to have 17p deleted recurrent disease and he was started on ibrutinib with good response. He presented to an outside facility with acute onset altered mental status after stopping ibrutinib 2 months earlier due to financial constraints. Cerebrospinal fluid (CSF) analysis was initially concerning for CLL infiltration of the CNS with neoplastic-appearing lymphocytes identified by cytomorphology; however, serological tests for West Nile virus indicated acute infection based on positive IgM and negative IgG; however, PCR could not be performed. The patient received therapy for presumed CNS involvement by CLL with intrathecal methotrexate along with intravenous rituximab and methylprednisolone before being transferred to our institution. On admission, his complete blood count revealed leukocytosis with a normal differential and thrombocytopenia, after reviewing the patients' historical trends, we could discern that the platelet count had been within normal limits in the past and had likely decreased due to the acute illness; additionally, we can appreciate the increasing trend in WBCs until the start of treatment around 10 years prior to this admission (). Peripheral blood smear showed normal platelet morphology and moderate leukocytosis with lymphocytosis with coarse, block-like chromatin pattern concerning for prolymphocytoid transformation which had not been present previously. Bone marrow biopsy revealed hypercellularity with diffuse infiltrate of the medium to large-sized mononuclear cells with irregular nuclear contours and prominent nucleoli similar to that seen in the peripheral blood smear (). Repeat CSF studies at our hospital showed lymphocytes accounting for 49% of total events per flow cytometry, positive for CD19, CD20, CD5, and CD23, with lambda light chain restriction, and negative for CD10 and FMC-7 (). The morphology of lymphocytes in CSF was consistent with neoplasia; however, given the admixture of reactive lymphocytes and the presence of known CNS infection, it was deemed that this most likely represented peripheral blood contamination and reactive infiltration of neoplastic lymphocytes.
He was treated supportively during his hospital stay and improved without any further CNS-directed therapy. Ibrutinib was later reinstated after the resolution of thrombocytopenia and BTK mutational testing proved negative. The patient continued to improve during outpatient follow-up, prolymphocitoid morphology disappeared from peripheral smear, and brain MRI showed no evidence of CNS disease.
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pmc-6204186-1
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A three-year-old boy with a rectosigmoidal Hirschsprung's disease and trisomy 21 received laparoscopically assisted Georgeson pull-through operation at the age of six months. Histology confirmed normal ganglion cells at the site of the anastomosis. The initial postoperative course was unremarkable; especially, the anastomosis healed with neither stricture nor dehiscence. Nevertheless, the boy suffered from recurrent episodes of HAEC with diarrhea, abdominal distention, pain, and alterations of the general condition classified as grade I according to the APSA criteria [].
Stool samples were taken for microbial assessment during three healthy episodes and three HAEC episodes, sampled to PSP spin stool DNA sample kits (Stratec Molecular GmbH, Berlin, Germany), and stored at −21°C until measurement. The microbiome analysis was performed in duplicates as already published []. Statistical analysis was performed using the compare_categories.py and the group_significance.py scripts of QIIME 1.8. These scripts implement several statistical methods for the analysis of strength and statistical significance of sample groupings or OTUs via the vegan and ape R packages. Category significance was calculated using the Adonis and ANOSIM tests, while OTU significance was calculated using the Kruskal–Wallis test.
Alpha diversity between healthy and HAEC episodes was not significantly different (Chao 1 Index: mean healthy episode 967, SD 94; mean HAEC episode 1,009, SD 72; p=0.432).
To assess beta diversity, a community analysis was performed by using principal coordinate analysis (PCoA) plots and Adonis and ANOSIM tests. A statistically significant difference in the composition of the fecal microbiome between healthy and HAEC episodes was found ().
Taxonomic analysis revealed a statistically significant decrease of the relative abundances of Actinobacteria and significant increases of Bacteroidetes, Proteobacteria, and Cyanobacteria on the phylum level during HAEC episodes (). A detailed overview of the statistically significant differences of the relative abundances on the remaining levels comparing healthy and HAEC episodes is given in . One of the most striking changes was seen for the genus Bifidobacterium which was reduced from 13% to 5% during HAEC episodes.
The patient was started on continuous treatment with probiotics for three months. In detail, he received one sachet of OMNi-BiOTiC® PANDA (Institut Allergosan, Graz, Austria) in the morning and one sachet of OMNI-BiOTiC® 10 AAD (Institut Allergosan, Graz, Austria) in the evening. OMNi-BiOtiC® PANDA contains Lactococcus lactis W58, Bifidobacterium bifidum W23, and Bifidobacterium lactis W52 (total of 3 × 109 CFU/sachet). OMNi-BiOTiC® 10 AAD contains Lactobacillus acidophilus W55, Lactobacillus acidophilus W37, Lactobacillus paracasei W72, Lactobacillus rhamnosus W71, Lactobacillus salivarius W24, Lactobacillus plantarum W62, Bifidobacterium bifidum W23, Bifidobacterium lactis W18, Bifidobacterium longum W51, and Enterococcus faecium W54 (total of 5 × 109 CFU/sachet). During these 3 months of treatment, fecal samples were taken weekly (as described above) adding to a total number of 14 samples.
During the observation period, the patient had episodes of diarrhea on 18% of the days (7 out of 39 days) without probiotic treatment and on 14% of the days under probiotic treatment (13 out of 90 days).
In the period of probiotic treatment, six stool samples were taken for microbiome analysis during diarrhea episodes and eight during healthy episodes. Probiotic treatment led to a significantly increased alpha diversity (Chao 1 Index) irrespective of healthy or HAEC episodes (mean healthy episode with probiotics 1,269, SD 111; mean HAEC episode with probiotics 1,274, SD 91; mean healthy episode without probiotics 967, SD 94; mean HAEC episode without probiotics 1,009, SD 72; p < 0.05 vs. their corresponding episode without probiotics).
Community analysis of the samples taken before and under probiotics is depicted in . Statistically significant differences of the composition of the fecal microbiome were found between healthy and HAEC episodes and under probiotic treatment.
Mean relative abundances on the phylum and genus levels are depicted in . On the phylum level, the most striking findings were that, during HAEC episodes under probiotic treatment, the significant increase of Bacteroidetes and the decrease of Actinobacteria were not encountered (compare ). On the genus level, Bifidobacterium and Streptococcus were significantly increased during probiotic treatment. Additionally, probiotic treatment led to significant decreases of Rikenellaceae, Pseudobutyrivibrio, Blautia, and Lachnospiraceae.
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pmc-6204204-1
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32-year-old male without significant past medical history was found to have what appeared to be a liver mass during surgical exploration for an acute appendicitis. His symptoms preoperatively included intermittent abdominal pain and profuse episodic axillary hyperhidrosis. On examination, he was normotensive and did not show evidence of pallor, anxiety, or tachycardia. 24-hr urine metanephrine was 4339 (36-190 mcg/24 hr), normetanephrine 20025 (35-482 mcg/24 hr), and total metanephrines 24364 (116-695 mcg/24 hr). MRI of the abdomen showed an 11 x 12 x 14 cm mixed cystic and solid mass centered in the right adrenal gland and 3.4 x 2.9 x 3.8 cm mass in the region of the porta hepatis.
After preoperative alpha-blockade patient underwent total right adrenalectomy. Pathology report confirmed the diagnosis of pheochromocytoma. According to the Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP) tumor's score was 9 [], indicating poorly differentiated tumor with the presence of necrosis, irregular cell nest form, high cellularity, minimal capsular invasion, and adrenergic features. Ki67 index 5% and S100 were negative.
Postoperatively plasma free metanephrines had normalized at 31 (<57 pg/mL) but plasma free normetanephrine remained elevated at 1844 (<148 pg/mL). Based on this biochemical profile presence of paraganglioma was suspected. CT abdomen showed 4.2 x 3.5 cm round soft tissue mass in the region of the porta hepatis which slightly increased in size from previous MRI.
Simultaneously, positron emission tomographic scan identified a 1.5 cm thyroid mass. Calcitonin level was normal. Fine-needle aspiration of this nodule was consistent with papillary thyroid carcinoma. Resection of the porta hepatis mass and total thyroidectomy were performed with subsequent confirmation of paraganglioma and PTC, respectively. On surgical pathology paraganglioma was S100 positive. After the resection, level of plasma free normetanephrine had decreased to 283 (<148 pg/mL), and free metanephrine remained normal. Genetic studies, which included gene sequence changes and deletion/duplications of EGLN1, FH, KIF1B, MAX, MEN1, NF1, RET, SDHAF2, SDHC, SDHB, SDHD, TMEM127, VHL, and SDHA, were negative.
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pmc-6204234-1
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A 70-year-old female presented with a two-year history of slowly growing, nonpainful maxillary sinus mass. She has a history of chronic maxillary sinusitis corresponding to presentation of the mass, with the first episode reported in 2014. Computed tomography (CT) imaging revealed an erosive right maxillary sinus mass (2.5 x 2.1 cm) with bony destruction.
Surgical excision of the right maxillary sinus mass revealed a fragmented, white, vaguely nodular, and whorled lesion. Histological examination revealed fragments of respiratory-type epithelium with focal cystic invagination and associated squamous metaplasia []. The underlying stroma consisted of a variably cellular, benign spindle cell proliferation with an associated background of hyalinization [], calcification and ossification [], and focal chondroid change [] in a vague lobule-like arrangement. Focal areas of aneurysmal and cystic changes [] were seen which would provide an explanation for the clinically noted enlargement since hamartomas by definition would be expected to have a much lower rate of growth. The intrinsic slow-growing nature is also supported by the deficit of mitotic activity even in the highly cellular/spindled regions of the lesion (less than 1/10 hpf). Areas with haphazard arrangement of nerve bundles within the collagenous stroma [] were also noted. Immunohistochemical stains were positive for SMA [Figures and ] in the spindle cells and negative for CK AE1/AE3, EMA, CD34, Stat6, ERG/FLI-1, Mucin 4, S-100, Sox-10, and desmin []; ruling out perineurioma, solitary fibrous tumor, a vascular neoplasm, Evans tumor, a benign peripheral nerve sheath tumor, or a myogenic neoplasm. The overall findings were suggestive of a hamartomatous lesion, most likely a nasal chondromesenchymal hamartoma. The absence of submucosal glandular proliferation, myxoid stroma, or mucinous metaplasia in the lining epithelium lowers the likelihood of other neoplastic hamartomatous lesions such as COREAH.
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pmc-6204236-1
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This study reported an 11-year-old male patient with recurrent fractures born to nonconsanguineous parents; both are healthy with negative fracture histories. The boy's prenatal history was not significant. He was born at full-term by vaginal delivery, with a birth weight of 3.4 kg. His cognitive and motor development was normal, and he had a normal gait pattern during childhood. The first fracture (left humerus) occurred at age 6 after a fall. One month before his referral, he suffered another fracture (left distal radius).
Subsequent physical examination of the proband revealed blue sclera. He had a normal height (146.9 cm, about 60th percentile), gait pattern, and muscle tone. His L1-L4 BMD (0.514 g/cm2, z-score −1.2) and femoral neck BMD (0.531 g/cm2, z-score −2.1) were low, and a spine X-ray showed multiple thoracic vertebral compression fractures (). However, as no paediatric reference standards for lumbar spine and femoral neck BMD were available in China at the time of this study, we used Canadian standards that may be inappropriate for our patient []. The proband's mother showed low BMD of the femoral neck (0.764 g/cm3, z-score −1.4, T-score −1.6) and no spinal compression fractures ().
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pmc-6204248-1
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An 81-year-old man presented to emergency department with the chief complaint of hematemesis which lasted for 7 days. His hematemesis started after three times none bloody emesis. He mentioned history of weight loss, recurrent epigastric pain and post prandial vomiting for one year. The vomitus occurred one to two times daily shortly after meal consumption. He had past medical history of a laparoscopic cholecystectomy five years ago, an ischemic brain stroke two years ago, and he was paraplegic due to a lumbar herniated disc about one year ago. His familial history was negative for GI disorders.
On physical examination the patient was cachectic and extremely weak with a blood pressure of 90/40 mmHg and heart rate of 110 beats/min. Respiratory rate was 22/min and oral temperature was 36.8. The sclera of patient was not icterus, lymphadenopathy, abdominal tenderness or palpable mass were not found. Some of the laboratory finding of this patient summarized in .
Treatment initiated by infusion of crystalloid fluids and intravenous pantoprazole. After the supportive care, esophagogastroduodenoscopy (EGD) was performed. EGD revealed a linear clean base ulcer in lower third of esophagus (Mallory Weiss syndrome), that has been caused by repeated vomiting, and also a Submucosal lesion which was observed in bulb area of duodenum ().
Endoscopic ultrasonography was subsequently performed and demonstrated one anechoic lesion measuring 45×55 mm that adjacent to duodenal wall although integrity of the duodenal wall layers was intact. The most possible location of the lesion was right kidney ().
Unenhanced and contrast-enhanced multi slice CT scan of the abdomen and pelvic were done. In the CT scan of abdomen and pelvic region, some evidence of multiple cortical cysts was seen in both kidney and one of these cysts with the size of 45×55 mm, pressured on duodenum ().
Conservative treatment was preferred according to the recent investigations (), patient's adverse condition, and also patient’s refusal to surgical treatment. We started the treatment with para enteric infusion of amino acids and intralipids, infusion of crystalloid fluids and intravenous metoclopramide. The patient was advised to consume small size of meals, 5 to 6 times a day, and high calorie liquids in small volumes. The treatment plan was relatively successful and general condition of the patient improved during the next two weeks. The mentioned diet was tolerated as well by the patient.
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pmc-6204273-1
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A previously healthy six-month-old girl who was born full-term following an uncomplicated pregnancy presented with persistent high fever and was found to have pyuria and bacteriuria. Prior to this, she had no significant medical history. No obvious gross malformations were observed on physical examination nor was any pertinent family history noted. Ultrasonography revealed multilocular cysts in the right kidney; of note, no abdominal abnormalities had been observed at her four-month medical examination.
Abdominal contrast computed tomography (CT) revealed a multilocular cystic mass accompanied by septal wall formation pressing on the normal kidney parenchyma (Fig. ). The tumor had not directly infiltrated the renal pelvis and there was no coexistence of hydronephrosis. Renal dysplasia was ruled out due to the presence of adjacent normal kidney parenchyma. Because the cystic septa was thickened, cystic NB was deemed the most likely entity, preoperatively. One month later, right nephrectomy was performed. Unfortunately, the cyst wall ruptured during surgery. An intraoperative cytological evaluation by rapid Papanicolaou staining of peritoneal fluid confirmed three components of blastemal, stromal, and epithelial cells. The findings were interpreted on-site as NB (Fig. ).
Cytologically, the blastemal cells were dyshesive, exhibiting naked nuclei with scant to absent cytoplasm and were the most predominant cell type. The spindle-shaped stromal cells were arranged in fascicles, showing a fibroblast-like configuration. And epithelial cells formed tubular structures. Macroscopically, the resected cystic tumor measured 80 mm in maximum diameter involving the upper pole to middle portion of the right kidney (Fig. ) and the cystic wall was predominantly thin. A portion of the cystic wall was ruptured by the surgical procedure and was associated with hemorrhage in the surrounding tissues. On the cut surface of the tumor, the cystic septum within the tumor had disappeared (Fig. ). Instead, hemorrhage and muddy, degenerative necrotic tumor tissue was found within the cystic space. Solid areas were observed within the cyst walls.
Histologically, the tumor was diagnosed as cystic NB (blastemal-predominant) displaying a triphasic pattern. The blastemal cells showed a solid growth pattern, whereas the epithelial cells showed tubular structures (Fig. ). The spindle-shaped stromal cells resembled embryonic mesenchyme, displaying differentiation into smooth muscles and fibroblasts (Fig. ). The cystic wall was lined by blastemal cells (Fig. ). Some solid areas were present within the cyst wall. There were no anaplastic cells or atypical mitoses. Degenerated hyperchromatic nuclei and apoptotic bodies were found in some areas. Immunohistochemically, the blastemal cells and epithelial cells were positive for WT1 (WT49, Leica, diluted 1:1), but the stromal cells were negative. The Ki-67 (MIB-1, BioGenex, diluted 1:30) labeling index of the tumor was as high as 90%, while the degenerated necrotic tumor cells showed decreased staining properties. Nephrogenic rest, a precursor lesion of NB, was not identified in the remaining renal parenchyma. The clinical stage classification of the Japanese Wilms Tumor Study (JWiTS) group was 3. The patient was treated with chemotherapy and radiotherapy and tumor recurrence and metastasis have not been observed in the 8 months since surgery.
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pmc-6204274-1
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A 67-year-old Asian woman with a known long history of major depressive disorder was brought to our emergency room (ER) with complaints of intentional ingestion of two tablets of AlP 5 hours prior to presentation. Except for the depressive illness for which she was taking a tablet form of escitalopram 20 mg twice daily, with which she was compliant, there was no other significant medical, surgical, or family history. She was seen by her son who found her in her room complaining of nausea and vomiting when she expressed to him about her ingesting the tablets. She had multiple episodes of vomiting containing greenish-colored particulate material which was not blood mixed. She also had three episodes of loose stools and generalized body weakness with altered sensorium. Before she was brought to our ER, she was taken to another center where she was primarily managed with gastric lavage and initial resuscitation. When she was evaluated in our ER, she was drowsy, her Glasgow Coma Scale (GCS) was 8/15, and her pupils were bilaterally 4 mm and sluggish reactive to light. Her pulse was 58/minute, she had blood pressure (BP) of 80/60 mmHg, her respiratory rate (RR) was 35/minute, and arterial oxygen saturation was 93% with supplemental oxygen via face mask. On auscultation, there were crepitations bilaterally along with decreased breath sounds. An initial arterial blood gas (ABG) done in ER revealed pH of 7.094, partial pressure of oxygen in blood (PaO2) 130 mmHg, partial pressure of carbon dioxide in blood (PaCO2) 23.5 mmHg, bicarbonate (HCO3) 8.9 mmHg, and serum lactate of 15.
Besides these clinical findings and laboratory parameters other tests were within normal range. A chest X-ray was done immediately, which showed infiltrations with bilateral opacities. A 12-lead electrocardiogram (ECG) showed sinus rhythm, non-specific ST-T changes in all leads. Cardiologists were immediately called for screening echocardiographic evaluation which showed significantly reduced systolic function of left ventricle with an ejection fraction (LVEF) of 20%. While evaluation was ongoing, a wide bore canula was opened, and dopamine was started to support the blood pressure. However, patient’s condition continued to deteriorate and an immediate plan for veno-arterial ECMO was made. After obtaining consent for ECMO from patient’s son, the intensive care unit (ICU) team was alerted and cardiac surgeons were informed accordingly. The patient was successfully connected to ECMO (Sorin SCPC Centrifugal Pump System) via right femoral vein and femoral artery. An extra 6F sheath was inserted distally into femoral artery for perfusion of distal leg.
In the immediate postoperative period the patient was kept on mechanical ventilator volume assist-control mode on low tidal volume lung protective ventilation at 40% fraction of inspired oxygen concentration (FiO2) with positive end-expiratory pressure (PEEP) of 7 cmH2O. We were able to deliver an almost constant flow of 3 L/minute/m2 on ECMO and provided oxygenation at FiO2 of 70%. She was also kept on epinephrine, norepinephrine support, magnesium sulfate, vitamin C, thiamine, hydrocortisone, heparin infusion, and sodium bicarbonate therapy. An ABG done immediately after the procedure showed improvement in acid-base status as well as decrease in lactate level to 0.31. Blood gases were repeated 4 hourly. Boluses of heparin 1000–3000 U were given along with adjustment in background infusion rate to keep the activated clotting time (ACT) between 180 and 220 seconds.
An echocardiography done the next day showed significant improvement in cardiac status with LVEF reaching 35%. Her general condition, consciousness level, hemodynamic stability, and ventilatory parameters, especially peak airway pressure, also showed significant improvement over the next 3 days and we were able to stop all inotropic supports by ECMO day 2. She was finally weaned off the ECMO on day 3 and extubated the next day. At all times during her stay in ICU, although she showed signs of myocardial dysfunction and respiratory failure along with depression of mental status, her renal and liver function tests remained within normal range. A psychiatric evaluation was done while she was in ICU. After observation in ICU for 2 more days, she was discharged to ward with stable vital signs. She stayed in wards for 4 more days and was then finally discharged to home to have follow-up later and scheduled psychiatric consultation and adjustment of medications accordingly (timeline; Fig. ).
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pmc-6204354-1
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A 4-year-old, male Staffordshire Terrier mix was presented to the Neurology Department of the Vetsuisse Faculty Zurich due to generalized seizures. The first seizure episodes were reported as a puppy. At this time, severe hypernatremia secondary to adipsia was diagnosed and the dog was successfully managed by adding water to his meals. After the hypernatremia was corrected, no seizure episodes were observed for 3 years thereafter.
Five months before presentation, seizures began to reoccur every second week despite normal serum sodium levels. Moreover, 4 days before presentation phenobarbital (2.5 mg/kg; every 12 h) was started by the referring veterinarian. The general physical examination at presentation was within normal limits and blood work showed only mild hypernatremia (sodium 156 mmol/l; reference: 145–152 mmol/l) and mildly increased serum albumin levels (albumin 42 g/l; reference: 29–37 g/l). The neurological examination revealed proprioceptive deficits in all limbs, proprioceptive ataxia and reduced menace response bilaterally. No visual impairment could be detected during the neurological examination. Therefore, the neuroanatomical localization was consistent with a forebrain dysfunction. CCA was suspected due to the history and clinical presentation.
Afterwards, an MRI of the brain was performed under general anesthesia using a 3T scanner (Philips Ingenia, Philips AG, Zurich, Switzerland). T2-weighted (T2W) sequences were acquired in sagittal (TR 6836 ms; TE 100 ms), transverse (TR 4820 ms; TE 100 ms), and dorsal (TR 3118 ms; TE 100 ms) planes. A 3D T1-weighted (T1W; TR 11.1 ms; TE 5.1 ms) sequence was obtained pre and post intravenous administration of gadodiamid (0.1 mmol/kg; Omniscan, GE Healthcare AG, Opfikon, Switzerland). Supplementary to the conventional protocol, an echo-planar DTI sequence (TR 8534.3 ms; TE 84.3 ms) with 32 diffusion directions (low b value = 0 s/mm2; maximal b value = 800 s/mm2; field of view of 160 × 169 mm and acquisition matrix of 108 × 111) was performed. Moreover, T2W, 3D-T1W, and DTI sequences from a 4-year-old, intact male healthy Beagle (same acquisition protocols, archived from an independent research study; animal permission number: ZH272/16) were used for comparison.
For the DTI analysis, corrections for drifting, motion artifacts, eddy-currents and Gibbs-ringing were performed. Subsequently, the DTI sequences were registered to the anatomical T1W images. Whole brain tractographies were calculated using the following settings using a probabilistic approach: minimal FA threshold 0.1; minimal fiber length 20 mm and a threshold angle of 60 degrees. Afterwards, regions of interest (ROIs) were placed at the CC within sagittal planes and at the cingulum within transverse planes in order to isolate the axonal tracts of these regions where they solely cross the laterolateral or rostrocaudal axis, respectively, as previously reported (). The evaluation of T1W, T2W, and the tractography analysis of DTI images were performed using free available software (Horos Project, version 2.2.0, horosproject.org; ExploreDTI, version 4.8.6, Utrecht) ().
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pmc-6205061-1
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A 27-year-old man without any significant medical history, presented to the emergency department (ED) with right lower abdominal pain and a fever of 38.7 °C A computed tomography (CT) scan of abdomen and pelvis showed a perforated appendix (). Emergency laparotomy was performed and the patient underwent appendectomy and washout. He was discharged home three days later.
On postoperative day 12, the patient was presented again to the ED with a septic shock with a pain of right flank. The vital signs at presentation were as follows: GCS 11/15; blood pressure 110/65 mmHg; pulse 110–120 beats/min; respiratory rate 25 breaths/min and body temperature 39 °C. Physical examination showed right thigh tenderness with a moderately erythematous abdomen and subcutaneous emphysema. Laboratory evaluation revealed a white blood cell count of 25000/mm³ with 90% neutrophil forms; hemoglobin 13 g/dL; Creatinine 65 μmol/L; C-reactive protein was 200 mg/dL and serum lactate level 4.9 mmol/L
A new surgical exploration was performed after appropriate resuscitation with intravenous fluids and antibiotic, revealed a purulent peritonitis with necrotizing fasciitis involving the right lower abdomen, right psoas muscle and right retroperitoneum. Culture of the necrotic tissues revealed polymicrobial infection consisting of Escherichia coli and Pseudomonas aeruginosa.
Postoperatively, the patient stayed intubated in the surgical intensive care unit and had mild hemodynamic instability requiring low-dose of noradrenaline. Antibiotics (Vancomycin 20 mg/kg/24 h, Tazobactam-Piperacillin 80 mg/kg every 6 h, Amikacin 15 mg/kg/24 h, metronidazole 40 mg/kg) were administered and the patient received repeated debridement for the unhealed abdominal wound. In addition, incision, drainage and hyperbaric treatments were performed three times for the necrotizing soft tissue infections over the retroperitoneal region of the right flank and also over the scrotal region and the external genital organ because of the extension of the necrosis. One week later, the patient developed another septic shock due to the extension of the necrosis to the right chest wall. A CT scan of the chest showed right-sided pleural effusion with erosive aspect of the ribs (). Necrotic tissues were debrided and antibiotic was changed due to wound superinfection with Acinetobacter Baumannii (Coli-mycin 100,000 UI/kg/24 h, rifampicin 30 mg/kg/24 h). After the improvement of his general health, skin flaps were putted by plastic surgeon to cover the exposed ribs ().
On the sixty postoperative day, the patient developed a mitral valve infective endocarditis with negative blood culture. Three days later, he died of septic shock and multiple organ failure.
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pmc-6205072-1
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A 14 years old female presented to OPD with complain of swelling at the nape of neck for the last 9 months. The swelling was small initially and then started increasing gradually in size in last 2 months. The swelling was painless and there was no discharge from swelling. The family history of patient was negative for any such swellings.
On examination, a firm non-tender and normothermic swelling measuring 6 * 8 cm was found in the nape of neck with normal overlying skin. The swelling was non-mobile in both(vertical and horizontal) planes. There were no dilated veins or visible pulsation over swelling and the skin over swelling was pinchable. No bruit was auscultated. The examination for cervical and axillary lymph nodes was unremarkable.
Ultrasound of neck showed a well capsulated and lobulated mass with cystic areas which contain internal echoes seen at the nape of neck ().
MRI neck gives impression of a large lobulated mass lesion approximately measuring 9.2 * 9.1 cm in size at the back of neck with main bulk on right side. Lesion is predominantly cystic with some solid component and appears hyperintense on T2W and FLAIR images, iso to hypointense on T1W images and appearance is suggestive of lymphangioma/cystic hygroma (a, b).
Tru-Cut biopsy was taken under aseptic measures from multiple areas and it showed only fibroadipose tissue without any granulomatous or neoplastic process. After detailed discussion, it was decided to go for excisional biopsy of mass under general anesthesia. Skin crease incision was given. Skin and subcutaneous tissue incised. The tumour was located muscle deep against vertebra. So, muscles were incised and tumour delivered to the surface. It was well circumcised mass (a–c). Haemostasis secured and then muscles were closed and then skin closure done.
Gross examination of specimen measured a 8.5 * 5 cm mass and histology revealed a circumscribed neoplasm comprised of hypocellular lobules of adipocytes with fibrous septa separating the lobules. Background stroma is myxoid with thin chicken wire vasculature. No atypia, mitoses or necrosis seen. The diagnosis of Lipoblastoma was made (a–c)
Post-operative course and follow up of patient was without any complication.
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pmc-6205109-1
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An 82-year-old female with a past medical history of uncontrolled hypertension, dementia, and tobacco abuse presented with complaints of blurry vision and frequent falls for the past few weeks. She also noted recent unintentional weight loss and productive cough. She was afebrile, cachectic, had decreased left-sided breath sounds, mild left-sided proptosis, chemosis, and bilateral cataracts on admission. Ophthalmology was consulted and reported that visual acuity was R 20/25 and L hand motion, with normal pupillary function and extraocular movements. Cranial nerve functions were grossly intact and intraocular pressures were within normal limits. Lab results and initial CT head were within normal limits. Chest X-ray () and CT alike showed left-sided atelectasis with ipsilateral mediastinal shift due to mucus plugging, a moderate left pleural effusion, and multiple spiculated cavitating nodules in the right lobe highly suspicious for malignancy. Two days following admission, she developed acute left orbital pain and visual loss prompting a brain MRI. MRI revealed enlargement of the L superior and inferior ophthalmic veins without contrast enhancement concerning for thrombosis (). Following initiation of heparin infusion, a CT venogram (CTV) confirmed the presence of thrombosis along with a filling defect in the L cavernous sinus (). CTV is felt to be the best diagnostic modality for detecting venous thrombosis.
In the absence of signs, symptoms, or risk factors for underlying infection, newly diagnosed malignancy remained the primary and most likely differential, and antibiotics were not initiated. Unfortunately, several barriers to confirmation of malignancy arose. Bronchoscopy was performed which, despite removal of a mucus plug, resulted in minimal improvement in the atelectasis. Pathology from brush cytology and transbronchial biopsy were inconclusive. A transthoracic lung biopsy of right lung nodules was deemed a high risk given persistent atelectasis of the left lung and presence of underlying emphysema. A thoracentesis of the left effusion was performed; however, cytology was negative for malignancy. Given the negative autoimmune and relevant hematologic work-up for hypercoagulability and the high likelihood of newly diagnosed underlying lung cancer, she was started on Lovenox with subsequent improvement in visual symptoms over the next few weeks. Serial chest CT will be required to monitor for progression.
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pmc-6205146-1
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A 23-year-old woman, asymptomatic, presented in a gynaecological examination a pelvic tumour in the left iliac fosse. Physical examination showed a 9 cm mobile mass in the left iliac fosse compatible with fibroid or adnexal mass. She was a non-alcoholic, non-smoker with no co-morbid medical or surgical ailment.
Transvaginal ultrasound shows an anteversion uterus with a proliferative endometrium, normal ovaries and a mass of about 9 cm, which does not seem to have a clear relationship with the uterus.
Pelvic magnetic resonance imaging describes a 9 cm semi-solid mass compatible with pedunculated fibroid or primary retroperitoneal lesion. Cerebral MRI shows no abnormalities.
Abdominal CT scan during the portal phase showed a pelvic mass of 6.5 x 5.5 cm, which seemed to originate from the left ovary, predominantly cystic with solid areas compatible with cystic ovarian neoplasm. Retroperitoneal or pelvic lymph nodes were not identified. There was no ascites. There were multiple bilocular masses informed as metastatic lesions in liver parenchyma. The rest was normal ( A and B).
CEA, Ca 12.5 and Ca 19.9 tumour markers were normal. Alpha-fetoprotein, testosterone, SDHEA and delta-4-androstenedione were also normal.
After discovering these surprising findings, our gynaecologic Tumours Committee decided to perform a guided needle core biopsy of the liver lesions to discart an advanced ovarian cancer. Histologically these lesions corresponded to a vascular tumour compatible with EH.
We decided to perform a diagnostic laparoscopy. During the exploration of the abdominal cavity, an enlarged liver with multiple intra-parenchymal hepatic lesions and a tumour with a large blood vessel originating from the greater omentum could be seen. Partial omentectomy resection, including the mass, was performed and was extracted in an endobag ( A and B).
Macroscopically the lump was fleshy, filled with blood and presented whitish areas. Anatomopathological study confirmed an EH. Immunohistochemical profile was CD31+, CD34+, CKAE1/AE3-, CK8/18-, C-kit-, EMA-, vimentin +, progesterone (+ weak, focal), estrogen-, mitotic activity <1 × 10 fields of large increased (, ).
After the results, liver resection was discarded due to multicentre lesions, involving pretty much the totality of the liver; making the patient a candidate for liver transplant. Six months later the patient was subjected to a successful liver transplant.
Consent was obtained for the publication of the case report and the images from the patient.
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pmc-6205147-1
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A 76-year-old male patient presented to our emergency department with a 24 h history of gradual onset left upper quadrant abdominal pain associated with nausea, vomiting, anorexia, and obstipation. Patient had no medical history of abdominal surgery. On presentation, he was afebrile, dehydrated with normal heart rate and blood pressure. On clinical examination his abdomen was distended and tender upon palpation of left upper quadrant without guarding or rebound tenderness. White blood cell count and CRP were 10,500/mm³ (3500–11,000) and 10 mg/L (<10) respectively. He had a slight renal insufficiency with a creatinine level of 2.6 mg/dl (0.72–1.17).The rest of the blood tests (Electrolytes, hepatic function) were unremarkable. Abdominal X-rays study showed a cluster of small bowel loops in left upper quadrant with multiple air-fluid levels. Abdominal computed tomography scanner (CT scan) confirmed the diagnosis of SBO with transition zone left to the Treitz ligament (A and B) and showed a sac-like appearance in left paraduodenal fossa located posterior to the mesentery of the left colon and the left portion of the mesentery of the transverse colon measuring 30 cm (A) suggesting of a left paraduodenal hernia.
Due to the high risk of strangulation, and the presence of symptoms, the patient was consented for exploratory laparotomy. At exploration, a huge hernia sac () arising from a peritoneal opening () located left to the Treitz ligament was identified. The sac contained dilated loops of small bowels.The hernia was reduced after widening of the orifice inferiorly and the defect was closed using non-resorbable sutures. The patient started oral feeding at day 2 post-operative after removing of the nasogastric tube and he was discharged home on 5th post-operative day. At 8 months follow-up, he is doing well without any complaints.
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pmc-6205148-1
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A 43-year-old woman was referred to our institution with a neck mass in the left submandibular region. She was studied in an outside hospital, where a neck ultrasound showed a multinodular goiter and a CBT on the left carotid bifurcation. A neck Computed tomography angiography (CTA) demonstrated a carotid body tumor (3.8 cm × 2.5 cm × 3.3 cm) classified as Shamblin II and an intrathoracic multinodular thyroid gland with a right lobe extension of 11.9 cm × 9.7 cm and the left lobe of 25.2 cm × 21.3 cm with caudal retrosternal growth and evidence of slightly trachea deviation ().
Thyroid function test (TFT) were normal, serum antithyroglobulin antibodies and thyroperoxidase antibody (TPO) were undetectable. Thyroid gammagram was performed reporting a multinodular goiter. Fine needle aspiration was performed demonstrating nonmalignant cells. A total thyroidectomy was performed with a transverse lower neck incision (Kocher incision), posteriorly, CBT was resected by an extension of the previous Kocher incision to the anterior border of the sternocleidomastoid muscle using the retrocarotid reported previously in our group as an effective technique, also two surrounding lymph nodes were resected to rule out malignancy (). The pathology report demonstrated a paraganglioma with negative lymph nodes invasion and a multinodular goiter ().
The patient recovered satisfactorily after the surgery and she was discharged in postoperative day number three, she currently continues her follow-up as our patient. Twelve months after the surgery, she does not present any complaints. She is currently on levothyroxine and calcium carbonate.
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pmc-6205153-1
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The patient was a 64-year-old, right-handed man. He graduated from a junior high school at 15 years of age, and worked in a supermarket. There were no notable issues in either his medical or family history. At 60 years of age, he exhibited symptoms including the inability to name products and to comprehend in-store announcements. He visited the authors’ hospital for the first time in 2016, as his symptoms gradually interfered with his work. Proper conduct was maintained, and his spontaneous speech was fluent. However, word-finding difficulty was detected. According to the Neuropsychiatric Inventory (NPI), he had mild depression, possibly caused by his inability to understand the meaning of words, but did not exhibit any behavioral disorders or other findings. Neuropsychological assessment revealed mild anomia, disturbance in word comprehension, and spared repetition (Table ). In the object-naming subtest of the Western Aphasia Battery (WAB), some tasks revealed anomia, which was not improved after the cues of the initial sound of the words. In the vocabulary subtest of the Wechsler Adult Intelligence Scale-III (WAIS-III), he was unable to provide the definitions of low-frequency words. In the Kanji (Japanese morphogram) reading task, surface dyslexia was detected. He was unable to understand the meanings of idioms and proverbs. Because auditory word recognition was preserved in the Standard Language Test of Aphasia (SLTA), semantic aphasia was considered to be mild. In the face-recognition subtests of the Visual Perception Test for Agnosia (VPTA), prosopagnosia was not detected. Additionally, there was no impairment in recognizing the faces of family members or acquaintances in daily life. No abnormalities were detected in the neurological examination. MRI revealed atrophy in the anterior and inferior portions of the left temporal lobe. Technetium-99-ethyl cysteinate dimer ([99mTc]ECD) SPECT revealed hypoperfusion in the anterior area of the left temporal lobe. The visual assessment of [11C]Pittsburgh Compound-B (PiB) PET scans, based on the Japanese Alzheimer’s Disease Neuroimaging Initiative (J-ADNI) protocol [], yielded negative results. His apolipoprotein E phenotype was E3/5 or E3/7. He was diagnosed with svPPA based on the diagnostic criteria developed by Gorno-Tempini et al. []. His naming impairment and word comprehension deficits were mild. Moreover, he presented no atrophy in the right temporal lobe. Accordingly, this patient was considered to be in an early stage of the disease. [18F]THK-5351 PET imaging revealed significant [18F]THK-5351 retention in the bilateral temporal lobes, predominantly on the left side (Fig. and Additional file : Figure S1). Structural MRI revealed brain atrophy in the left anterior temporal pole. [99mTc]ECD SPECT scan also revealed unilateral hypoperfusion in the left anterior temporal pole. For the comparison of different imaging modalities, Z-score maps of [18F]THK-5351 PET were created by the comparison of individual PET images with the mean and standard deviation of 20 normal controls, using PMOD software (PMOD Technologies, Zürich, Switzerland). Z-score maps of CBF-SPECT and voxel-based morphometry (VBM)-MRI were also created using easy Z-score imaging system (eZIS) software (Fujifilm RI Pharma., Tokyo, Japan) and voxel-based specific regional analysis system for AD (VSRAD) software (Eisai, Tokyo, Japan) [, ]. In the right temporal lobe, the Z-score of [18F]THK-5351 PET (Z = 3.70) was greater than that of CBF-SPECT (Z < 1.0) and VBM-MRI (Z < 1.0).
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pmc-6205153-2
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The patient was a 79-year-old, right-handed man with 16 years of education. There were no notable issues in either his medical or family history. When he was 77 years of age, his wife noticed that he became unable to name objects used in daily life. During the next two years, his language symptoms had gradually worsened, and he visited the authors’ hospital in 2017. Although his spontaneous speech was fluent and proper conduct was maintained, word-finding difficulty was evident, and his episodic memory in daily life was preserved. According to the NPI, there was no behavioral disorder. Neuropsychological assessment revealed mild anomia, disturbance in word comprehension, and spared repetition (Table ). In the object-naming subtest of the WAB, some tasks revealed anomia, which was not improved after the cues of the initial sound of the words. In the vocabulary subtest of the WAIS-III, he was unable to provide the definitions of low-frequency words. In the Kanji (Japanese morphogram) reading task, surface dyslexia was detected. He was unable to understand the meanings of idioms and proverbs. Because auditory word recognition was preserved in SLTA, semantic aphasia was considered to be mild. In the face-recognition subtests of the VPTA, prosopagnosia was not detected. Additionally, there was no impairment in recognizing the faces of family members and acquaintances in daily life. No abnormalities were detected on the neurological examination. MRI revealed atrophy in the anterior and inferior portions of the left temporal lobe. [99mTc]ECD SPECT scan revealed hypoperfusion in the left temporal lobe. [11C]PiB-PET scan was considered to be negative according to the J-ADNI protocol [], although very mild and focal [11C]PiB retention was observed only in the parietal lobe. His apolipoprotein E phenotype was E3/3. He was diagnosed with svPPA based on the diagnostic criteria developed by Gorno-Tempini et al. []. He was considered to be in an early stage of svPPA because of his mild anomia and word comprehension disturbance, and no remarkable atrophy in the right temporal lobe. In [18F]THK-5351 PET scan, [18F]THK-5351 retention was observed in the bilateral temporal lobes, predominantly in the left side (Fig. and Additional file : Figure S1). In an analysis similar to case 1, Z-score in the right temporal pole was greater in [18F]THK-5351 PET (Z = 1.27) than in CBF-SPECT (Z < 1.0) and VBM-MRI (Z < 1.0).
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pmc-6205155-1
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A 2-year-old male child was admitted to hospital because of worsening one month-long fatigue and loss of appetite. He was born from non-consanguineous healthy parents; pregnancy, delivery and neonatal course were uneventful. Growth, neurological and cognitive development were normal. The extended newborn screening performed in tandem-mass spectrometry was normal: plasma propionylcarnitine was 1.8 micromol/l (normal value < 3.3) and propionylcarnitine/acetylcarnitine ratio was 0.13 (normal value 0.02–0.21). Family history was negative for cardiac or metabolic diseases.
He presented with respiratory rate 60/min, saturation rate of 85% and palmo-plantar edema. Chest X-Ray showed enlargement of the heart shadow and pulmonary interstitial involvement. Soon after, clinical worsening occurred with cyanosis, O2 requirement, anasarca and systemic hypertension (140/90 mmHg).
Right systolic murmur was appreciable. ECG evidenced sinus tachycardia with right ventricular hypertrophy and overload; echocardiogram showed severe right ventricle enlargement, poor ventricular function with paradox movement of interventricular septum (Fig. ); pulmonary pressure rate, calculated on the basis of tricuspid insufficiency velocity, resulted elevated: 107 mmHg (Fig. ); left atrium volume was normal and left ventricle resulted hypertrophic. A prompt treatment for both systemic and pulmonary hypertension was started with atenolol/amlodipine and sildenafil, inducing only partial improvement of symptoms.
Blood examinations revealed hemolytic-uremic syndrome (HUS), with macrocytic anemia (hemoglobin 9.0 g/dl, normal values [nv] 10.7–13.4; MCV 86.1 ft., nv 75–85), thrombocytopenia (platelets count 40000 × 103; nv 210–590), elevated LDH (up to 3000 IU/L, nv 192–321) and creatinine (from 0.56 to 1.2 mg/dl, nv 0.2–0.43), low albumin rate (2.6 g/dl, nv 3.5–4.5), very low haptoglobin levels (< 7.5 mg/dl; nv 30–200), negative Coombs test (both direct and indirect), proteinuria and hematuria; complement serum levels resulted low (C3 69 mg/dl, nv 90–180; C4 9 mg/dl, nv 18–55). Hypersegmented neutrophils and schistocytes in the peripheral blood smear were detected.
HUS is one of the most common TMA syndromes. The pathological feature of TMA is vascular damage, manifested by arteriolar and capillary thrombosis with characteristic abnormalities in the endothelium and vessel wall. The most common form of pediatric TMA is the so-called “typical” HUS in which vascular damage is caused by an enteric infection with a Shiga toxin–secreting strain of Escherichia coli (STEC) or Shigella dysenteriae. Other common forms of TMA are atypical HUS (aHUS) and thrombotic thrombocytopenic purpura (TTP). The latter was excluded because ADAMTS13 activity was normal and autoantibody inhibition of ADAMTS13 activity was absent. Causes of aHUS were examined and excluded except for metabolic diseases. Renal histology was not performed because of severe cardiac involvement of the patient.
Workup for inborn error metabolic diseases was started, evidencing: high levels of plasma total homocysteine (tHCy) (74 micromol/l, nv < 15), plasma propionylcarnitine (5.64 micromol/l, nv 0.86), serum methylmalonic acid (138 micromol/l, nv < 1) and urinary methylmalonic acid (919 mM/mol, n.v. < 2); normal value of folic acid and vitamin B12. According to the diagnosis of methylmalonic acidemia and homocystinuria, the child was started on treatment with IM OHCbl (intramuscular hydroxocobalamin) (1 mg/day), oral betaine (250 mg/kg/day), folinic acid (3.75 mg/day) and carnitine (50 mg/kg/day). Response to therapy was dramatic: after 15 days plasma propionylcarnitine was normal (0.74 micromol/l, nv 0.86), serum methylmalonic acid was 0.92 micromol/l (nv < 1), and tHCy was 20 micromol/l (nv < 15).
Molecular analysis of MMACHC gene was performed: cblC defect was confirmed by revealing compound heterozygous c.271-272dupA (frameshift mutation)/c.347 T > C (missense mutation) usually associated with late-onset cblC defect.
Sildenafil was suspended after 10 days and only a mild anti-hypertensive therapy with enalapril was maintained and suspended after six months. After two years of follow-up, the child is in now good health conditions, showing normal growth, normal mental and neurological development, and no behavioral problems were detected. We performed ocular studies every 6 months and no ocular maculopathy was detected. The current therapy consists in IM OHCbl 1 mg/day, oral betaine (200 mg/kg/day), folinic acid (3.75 mg/day) and carnitine (50 mg/Kg/day). Blood pressure is in normal range; echocardiogram shows normal dimensions and thickness of cardiac chambers, with continent tricuspid valve. Laboratory findings at the last clinical assessment are normal, particularly plasma tHCy (11.6 micromol/l), plasma amino acids and plasma acylcarnitines. Serum methylmalonic acid is 1.5 micromol/l.
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pmc-6205165-1
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We herein describe a representative case of T790M-positive conversion. The patient was 73 years-old male with EGFR-mutant (L858R) NSCLC. He received erlotinib as the first-line therapy for 1 year. After progression, rebiopsy was performed using bronchoscopy. T790M was negative, then ABC-therapy and carboplatin plus pemetrexed were administered as second- and third-line chemotherapies. After further progression, rebiopsy was carried out again to the same lesion and procedure. T790M was converted to positive, and osimertinib was initiated. Partial response was confirmed, and TTF was 12.2 months (Figure ).
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pmc-6205312-1
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An 81-year-old man presented with asymptomatic of hyperkalemia (5.7 mEq/L). The past history included mitral valve repair and coronary bypass surgery 27 years previously, mild type II diabetes for 12 years, renal insufficiency (stage III) with a stable creatinine level of 1.5 mg/dL for 8 years and prostatic hypertrophy. An echocardiogram performed 6 months previously revealed normal left ventricular function and minimal mitral regurgitation and an ECG documented sinus rhythm and complete left bundle branch block. Medications included Avodart 0.5 mg qd, atorvastatin 80 mg qd, aspirin 325 mg qd, Januvia 25 mg qd, and metoprolol tartrate 50 mg bid. The latter had been prescribed 6 years previously for frequent symptomatic atrial premature beats with an excellent clinical result so that metoprolol at the same dose (all tartrate preparation) was continued up to the most recent evaluation. The potassium level had always been normal before the administration of metoprolol. Afterwards, routine testing with serum electrolytes every 6 or 12 months consistently revealed a potassium level of 5.4 mEq/L. Then, for the last 2 years regular routine testing revealed a potassium level fluctuating between 5.6 and 5.7 mEq/L. Finally, metoprolol was discontinued and 18 days later the potassium level normalized at 4.2 mEq/L. Carvedilol was started and then increased to 12.5 mg bid. After about 10 days on this dose, the potassium level increased to 5.6 mEq/L. The dose of carvedilol was therefore reduced to 6.25 mg bid. A follow-up potassium level 2 weeks after the start of the lower carvedilol dose was 5.0 mEq/L which is at the upper limit of normal for the testing laboratory.
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pmc-6205313-1
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A 13-year-old female presented to pediatric otolaryngology clinic for follow-up of a left-sided tongue mass, first detected in 2015, which was recently getting bigger and causing dental problems and difficulties with chewing. The patient has a past medical history of NF2, with bilateral acoustic neuromas (also known as vestibular schwannomas), diagnosed more than 10 years ago. During this period, she underwent removal of a right optic nerve glioma and several neurofibromas of the skin. She also has a strong family history of NF2, with her father and two sisters also diagnosed with NF2.
Oral cavity examination revealed a well circumscribed mass on the left anterior tip of the tongue (). The mass was approximately 2.0 x 2.0 cm, nontender, and nonerythematous. Clinically, it appeared to be a tongue neural tumor associated with NF2.
Excisional biopsy of the tongue mass was performed under general anesthesia. The mass was removed with 2.0 mm margins around the lesion (). The postoperative period was uneventful. Gross examination of the specimen revealed a 1.7 x 1.4 x 0.5 cm nodule, which was serially sectioned to reveal multiple, well circumscribed white fibrous nodules ranging from 0.1 to 0.7 cm, with the largest nodule abutting the surgical margin.
Histopathological examination revealed a spindle cell tumor with multinodular growth pattern, resulting from interlacing fascicles of Schwann cells (plexiform growth) (). Most of the tumor consists of cellular areas (Antoni type A) with Verocay bodies, formed by palisading of nuclei and separated by cell processes of Schwann cells (). Immunohistochemical analysis revealed the tumor cells to be strongly positive for S-100 protein () and negative for smooth muscle actin (SMA). EMA highlighted perineural fibroblasts surrounding tumor nodules (). These morphologic features, along with the immunoprofile, are diagnostic of plexiform schwannoma.
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pmc-6205315-1
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A 20-year-old woman (Gravida 2/Para 1) who was unaware of the date of the last menstrual period and was evaluated during routine pregnancy follow-up presented with nonspecific complaints, such as abdominal pain, bloating, dyspepsia, and occasional respiratory distress. The medical and gynecological history of the patient was unremarkable. She had regular menstrual cycles and did not use oral contraceptives or any medication for the ovulation induction. An increase in the bilateral ovarian size (left side 14x13 cm and right side 13x12 cm) and a multicystic appearance were observed (). Ultrasonographic evaluation revealed a single live intrauterine fetus of 12 weeks gestation (). Extensive fluid was seen in the abdominal cavity. On chest X-ray, the costophrenic angles were closed and an appearance of hydrothorax was observed (). On physical examination, there was abdominal distension and tenderness. The laboratory tests of the patient were as follows: quantitative hCG 117740 IU/ml, TSH 3,229 µIU/ml, and free T3 and T4 were within normal ranges; hemoglobin 16,7 g/dl, hematocrit 47,6%, E2 >5000 pg/ml, PT, PTT, and fibrinogen were within normal limits; routine biochemical tests were normal (for example, total protein, albumin, creatinine, BUN, Na, K, AST, ALT, and LDH); interestingly CA-125 (564 IU/mL) was found higher, Inhibin A 861, Ristocetin cofactor (von Willebrand factor (VWF) activity) 100% (50-100%), and VWF antigen 150% (60-150%).
Serological tests (anti-HAV IgM, HBsAg, and anti-HCV) were found to be negative. Antithrombin 3, lupus anticoagulant, protein C and S activity, antiphospholipid antibodies (IgM and IgG), and anticardiolipin antibodies (IgM and IgG) were found to be within normal ranges in thrombophilia screening. Factor V Leiden mutation was not observed. Other causes of spontaneous OHSS were ruled out. In the examination of FSHR gene mutation due to investigation of spontaneous OHSS, a mutation was identified which has been previously described and reported as a disease-related mutation. The result is shown in . Doppler ultrasonography revealed normal arterial blood flow in bilateral ovaries. The patient was hospitalized with the diagnosis of Grade 2 spontaneous OHSS, according to the Golan classification [], and conservative treatment was initiated.
Daily 75 mg of rectal indomethacin, 1500 cc/day intravenous saline infusion adjusted by considering the electrolyte balance, follow-up of fluid input and output, daily measurement of weight and waist circumference, and 3500 IU/day of low molecular weight heparin (tinzaparin sodium) for thromboembolism prophylaxis were administered. During the hospital stay, the clinical symptoms of the patient, such as abdominal pain and distention, increased. Repeated abdominal ultrasonography revealed advanced ascites in the abdomen. Approximately 2.5 to 3 L of ascites fluid was drained with a spinal needle by paracentesis. The treatment was supplemented with 20% 50cc albumin solution. Within the next three days, the laboratory parameters and clinical symptoms of the patient improved and she was discharged with suggestions of follow-up antenatal outpatient clinic visits. At 38 weeks of pregnancy, the patient gave birth of a 2,950 g healthy alive male baby, and in the first postpartum month, both ovaries were in normal appearance in transvaginal ultrasonography and in Doppler examination. The extensive abdominal ascites fluid also completely resolved. She had no complications during pregnancy and postpartum period.
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pmc-6205315-2
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A 28-year-old woman (Gravida 3/Para 1) with unremarkable medical and gynecological history had regular menstrual cycles and did not use oral contraceptives or any medication for the ovulation induction. Ultrasonographic evaluation revealed a single live intrauterine fetus of 10-week gestation (). An increase in the bilateral ovarian size (left side 12x12,5 cm and right side 11x13 cm) and a multicystic appearance were observed (). The patient's laboratory tests were similar to those of the patient in the first case. Other causes of spontaneous OHSS were ruled out. In the examination of FSHR gene mutation due to investigation of spontaneous OHSS, a mutation was identified which has been previously described and reported as a disease-related mutation. The result is shown in .
The patient was hospitalized with the diagnosis of Grade 2 spontaneous OHSS, according to the Golan classification, and conservative treatment was initiated. At 40 weeks of pregnancy, the patient gave birth of a 3840 g healthy alive female baby. She had no complications during pregnancy and postpartum period.
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pmc-6205318-1
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A 56-year-old Japanese female was referred to our hospital for vomiting, nausea, and abdominal pain after excessive drinking and eating. Abdominal pain occurred 6 hours after her last meal, followed 2 hours later by frequent vomiting. On admission, she was alert, afebrile, 160 cm in height, and 57.2 kg in weight. Her blood pressure (BP) was 158/95 mmHg, her heart rate (HR) was 80/min, and her oxygen blood saturation (SpO2) was 100% (room air). Physically, she complained of pain in the swollen right upper quadrant but without muscular defense. Her previous history included an oophorectomy for ovarian cysts at age 26 years, conservative treatment for duodenal ulcer at age 36 years, lithotripsy for ureter stones at age 48 years, and conservative treatment for gallstones and cholecystitis at age 53 years. Laboratory data on admission included a white blood cell (WBC) count of 18,500/μL, hemoglobin (Hb) concentration of 16.0 g/dL, platelet count of 308 × 103/μL, serum C-reactive protein (CRP) concentration of 1.06 mg/dL, and lactate dehydrogenase concentration of 204 U/L, with normal hepatic and renal function. Serum electrolyte levels were all within normal ranges. Abdominal computed tomography (CT) showed that most of the small intestine was located in the right upper abdominal cavity and had a sac-like appearance, without ligament of Treitz being present in the duodenum. CT of the vascular system showed a flattened inferior vena cava in association with edematous mesenterium and dilated mesenteric veins, findings suggesting a strangulated ileus (Figures and ).
Emergency laparoscopic repair was performed. Under general anesthesia, a cut was made at the umbilicus, a 12 mm port was inserted using the open method, and 5 mm ports were inserted into the right and left lateral abdomen as well as the lower abdomen. Laparoscopic observation showed that the ascending and descending colons were in their normal positions, with the cecum in the right lower abdomen. However, ascites and distention of the small intestine were observed under the mesenteric membrane of the ascending colon. The duodenum showed a leftward and then a rightward bending, with most of the small intestine, except for a 1-meter-long section of the ileum in the ileocecal region, being packed within the ligament of Ladd, which formed a hernia sac (). Although the small intestine was tightly adhesive within the hernia sac, careful peeling and incision release were performed without complications. The absence of severe ischemia within the incarcerated intestine precluded the need for intestinal resection. The patient's clinical course of postlaparoscopic repair was uneventful; she was able to drink water on day 2 and was discharged on day 9. Examination of an abdominal CT image of this patient taken at age 48 years showed a similar sac-like appearance, suggesting that this patient may have had intermittent PDH for 8 years. A follow-up CT scan after 2 months of surgical repair showed no abnormalities. At the time of writing, 3 years after surgical repair, the patient remains well without any recurrent symptoms or other complaints.
A survey of the ICHUSHI (Igaku Chuo Zasshi; the Japan Medical Abstract Society; ) of patients in Japan who underwent laparoscopic repair of PDH during the period of 2002 to 2017, using the keywords PDH, laparoscopy, and adults (>18 years old), identified 26 such patients in 22 studies, all written in Japanese [–] and two more patients in two studies written in English [, ]. Including the present patient, a total of 29 patients were analyzed. The median age of these 29 patients was 52.3 years (range, 20–80 years), and the male to female ratio was 16/13. Eight patients had right-sided PDH while 21 had left-sided PDH. Their clinical features and laparoscopic repair results are shown in . Time from initial symptoms to diagnosis did not differ significantly between right and left-sided PDH. Emergency surgery was more frequent in patients with right-sided PDH. Additional procedures during laparoscopic repair were more frequent in patients with left-sided (6/21) than right-sided (1/8) PDH. Release of the hernia sac was more frequent in patients with right-sided PDH. Overall results were excellent, but a few patients with either right-sided or left-sided PDHs required additional postoperative care.
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pmc-6205676-1
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The case was a 67-year-old male patient with type 2 diabetes mellitus, who had complaints of sudden decreased vision in the right eye since the morning of referral. Corrected distance visual acuity in the right eye was hand motion and in the left eye, 20/40. Examination of the right eye revealed grade I hyphema, normal intraocular pressure, and normal appearance of iris tissue and posterior chamber intraocular lens, although the posterior segment was not illuminated. In ocular ultrasonography, the retina was normal yet the vitreous cavity was seen to be hyperechoic with high gain and preoccupied vitreous hemorrhage. In the left eye, there were hard exudates and dot-blot hemorrhages throughout the retina. In examinations of the left eye, optical coherence tomography revealed macular edema and the fluorescein angiogram revealed macular edema, microaneurysms, and focal leakage of vessels without any neovascularization. The patient stated that he used 100 mg of sildenafil citrate (Viagra; Pfizer, Istanbul, Turkey) the previous night for sexual stimulation and visual acuity of the right eye was good until this morning. The patient was asked about his medical history and drug use, and he reported that he had type 2 diabetes mellitus only and was only using insulin. The patient also stated that he had not received any other ocular treatment, such as retinal laser or intravitreal injection. The patient had worked regularly in the same job for the last five years and there were no important stressor factors in his life. An anterior segment photograph of the right eye was taken () and informed written consent was obtained for the academic use of the photograph.
Therapy was firstly initiated with bed rest with the head in an upright position, 0.5% loteprednol etabonate ophthalmic suspension (Lotemax; Bausch & Lomb Pharmaceuticals, New Jersey, USA) four times in a day, and 1% tropicamide eye drop (Tropamid 1% Forte; Bilim İlaç Sanayi ve Ticaret AŞ, İstanbul, Turkey) two times a day. Anterior chamber clearance and pars plana vitrectomy were planned for the patient because the hyphema and vitreous hemorrhage did not resolve during the four weeks of follow-up. In the preoperative tests, the complete blood count and coagulation tests were determined to be completely normal and no disease other than type 2 diabetes mellitus was found in the internal medicine consultation. Pars plana vitrectomy was performed after one month and final corrected distance visual acuity increased to 20/40 during the first postoperative week. Intra-retinal hemorrhages, hard exudates, and laser photocoagulation scars were seen in colored fundus photograph and irregularities in retinal layers were shown in the optical coherence tomography image ().
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pmc-6205700-1
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The patient was a 33-year-old female with intermittent claudication affecting the right lower limb. She reported that she felt no pain when walking slowly, but that the pain appeared and increased in intensity as she walked at higher velocities, and that these symptoms had worsened over the previous 2 years. She had no comorbidities and was a non-smoker. On physical examination, distal pulses were palpable and symmetrical, but during dorsiflexion maneuvers pedal pulses were attenuated bilaterally, to a greater extent on the right.
Doppler ultrasonography of the arteries of the lower limbs was suggestive of extrinsic compression of an artery and magnetic resonance angiography revealed a moderate/accentuated stenosis of the proximal segment of the right anterior tibial artery at the level of the interosseous membrane during dorsiflexion of the feet ( ).
The patient underwent surgical treatment via a longitudinal incision in the anterolateral surface of the right leg, providing access between the tibialis anterior and extensor hallucis longus muscles. The interosseous membrane causing compression and obstruction of the anterior tibial artery was identified and partial resection of the membrane was performed, increasing the size of the opening at the hiatus ( ).
The patient complained of prolonged postoperative pain, due to hematoma in the anterior compartment caused by manipulation, but recovery progressed at an acceptable rate with physiotherapy. On physical examination, pedal pulses were normal, even during foot dorsiflexion maneuvers. Additionally, magnetic resonance angiography no longer showed compression of the anterior tibial artery ( ).
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pmc-6205701-1
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A 15-year-old male patient presented complaining of pain and localized swelling in the right popliteal fossa with onset 1 month previously. He reported a prior history of vascular malformation in the posterior aspect of the right thigh, which had never been diagnosed definitively. Physical examination revealed a firm mass in the right popliteal fossa associated with diffuse increase of volume in the posterior region of the distal right thigh. Doppler ultrasonography was used to diagnose a voluminous right PVA. In view of the risk of thrombosis and the patient’s symptoms, the treatment chosen was open surgical venous aneurysmectomy followed by primary reconstruction of the popliteal vein ( ). The patient was prescribed treatment with anticoagulants for 3 months and instructed to attend for clinical follow-up.
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pmc-6205701-2
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A 66-year-old female patient presented with pain and edema of the left lower limb as far as the top of the thigh. The only risk factor for thrombosis that she reported when interviewed was immobilization lasting a few hours. Doppler ultrasonography showed venous thrombosis of the left gastrocnemius and fibular veins and a left PVA ( ). She was managed with systemic anticoagulation, taking Rivaroxaban for 6 months, and instructed to care for the site with localized heat, elevation, and compression therapy with medium pressure elastic stockings.
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pmc-6205702-1
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A male, 40-year-old patient presented with sudden exacerbation of arterial hypertension that had hitherto been controlled with two drugs (amlodipine and valsartan). Renal function was preserved, with no abnormalities, and urea and creatinine levels were also normal. He had a prior history of chronic hepatitis and smoking (20 pack years). Investigation of probable secondary hypertension was initiated with Doppler ultrasonography of the renal arteries, revealing stenosis (> 80%) of the mid third of the left renal artery. The Doppler ultrasonography images were considered satisfactory and compatible with the patient’s clinical status. In addition to providing images, the Doppler ultrasonography findings enabled the degree of stenosis to be calculated in terms of anatomic criteria and velocity, and provided sufficient evidence to indicate angiography and treatment planning during a single procedure. Angiography revealed dissection of the renal artery, with double lumen and reduction in the vessel’s caliber, provoking stenosis of the segment ( ). The patient was treated by placement of two stents, one a longer self-expanding stent and the other a balloon-expanded covered stent along the zone of dissection. Clinical progress was good and it proved possible to control arterial hypertension with just one drug. A control angiotomography at 3 months showed that the dissection had been resolved and the stents were patent ( ).
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pmc-6205705-1
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A 62-year-old white male with a history of diabetes, hypertension, and chronic kidney disease with indications for renal replacement therapy presented for surgical creation of an arteriovenous fistula for hemodialysis. After assessment by the Vascular Surgery team, a radiocephalic arteriovenous fistula in the left upper extremity was deemed most appropriate.
During surgical creation of the fistula in the left wrist, dissection of the radial artery revealed a large cystic formation enmeshed in the vessel, with no clear borders between the cyst and artery ( ), but with a strong local pulse. Slight adhesions between the vessel and cyst allowed the latter to be dissected free of the artery without rupture or major leakage of cyst content. The surgical technique adopted was resection of the cystic segment ( ) and preservation of the radial artery, enabling successful fistula creation ( ).
Gross pathological examination of the surgical specimen revealed a viscous substance within the cystic mass. Histopathological analysis confirmed the presence of a cystic lesion of the arterial wall ( ). The patient is being followed by a multidisciplinary team. The fistula is currently functional, and follow-up echo-Doppler performed 1 year after the procedure revealed no signs of artery degeneration ( ).
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pmc-6205708-1
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The patient was a 44-year-old female craftswoman who had been suffering pain in the right upper limb for several months. The pain had increased progressively over the previous 3 months. She described pain at rest associated with coldness, pallor, and paresthesia of the limb. She had fallen from stairs when 9 months old, fracturing her clavicle, which was managed conservatively.
Physical examination revealed discrete deformity at the level of the mid third of the right clavicle. The right hand was cold and blue and ulnar, radial, brachial, and axillary pulses were all absent. Continuous wave Doppler detected no blood flow distally and monophasic flow in the brachial and axillary arteries. The chest X-ray showed a deformity of the mid third of the right clavicle, compatible with pseudarthrosis ( ).
Doppler ultrasonography demonstrated segmental occlusion of the subclavian artery with distal refilling via collateral vessels and very fine, threadlike flow in the axillary artery with occlusion of the brachial and radial arteries and refilling of the distal ulnar artery. Magnetic resonance angiography confirmed the Doppler ultrasonography findings ( ).
The pseudarthrosis was treated surgically, via a right supraclavicular incision, with resection of the mid segment of the clavicle ( ). The right subclavian artery was compressed and had thrombi with a chronic appearance inside. Thromboembolectomy of the subclavian artery was conducted with a 3F Fogarty catheter, followed by closure of the arteriotomy with a patch harvested from the saphenous vein in the thigh. Thromboembolectomy of the brachial, radial, and ulnar arteries was then conducted via an arteriotomy in the brachial artery, resulting in considerable improvement in terms of pain, temperature, color, and perfusion of the limb.
Throughout the examination, Doppler ultrasound of the hand showed triphasic flow in the radial and ulnar arteries. The patient was kept on clinical anticoagulant treatment with warfarin, maintaining an international normalized ratio between 2.0 and 3.0. Follow-up Doppler ultrasonography was conducted annually for 5 years and the patient attended for clinical follow-up for more than 10 years, until she died from unrelated causes.
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