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pmc-6343160-6
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Case 6 is a 17-year-old female with negative family history. Her first sign was presented at one year of age with: fever and chills, nausea, and body pain with a high score. Her MEFV gene mutations were M694V (homozygous).
She started taking colchicine 0.25 mg daily 16 years ago then increased the dose to 2.5 mg daily; however, attacks persists to one episode every 1-2 months with similar severity.
From 7 months ago, we added dapsone 100 mg daily. Dapsone has reduced the frequency and severity of attacks (one attack in 5 months with a very low severity score). There have been no known side effects of this treatment in this case.
shows these patients' data and summarized their findings.
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pmc-6343163-1
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A 30-year-old Filipino woman with no known comorbidities and good baseline functional capacity was admitted in our institution for a one-year history of intermittent high-grade fever (Tmax 40°C), malaise, generalized weakness, anorexia, unintentional weight loss, alopecia, throat discomfort, exertional dyspnea, easy fatigability, and additive arthritis of bilateral knees, elbows, and small joints of both hands. Two months prior to admission, she consulted at a private clinic where a battery of tests showed negative Salmonella and dengue IgM and IgG, normal C-reactive protein, and normal rheumatoid factor. The patient does not smoke, drinks alcoholic beverages once a month, and denies illicit drug use. She previously worked as an adult entertainer in Japan during the early 2000s, during which she had more than 50 heterosexual partners. Her obstetric score is G2P2 (2002), with no previous fetomaternal complications. Family history is unremarkable.
On admission, she was noted to have tachycardia, fever (38.9°C), hyperemic conjunctivae, facial flushing, bilateral cervical lymphadenopathies, and arthritis of both knee joints. The initial working impression was fever of unknown origin with infection (tuberculosis and HIV), connective tissue disorder, and occult malignancy as major differential diagnoses.
Initial laboratory studies showed normocytic, normochromic anemia (hemoglobin 116 g/L), trace result on direct antiglobulin testing, and a 3.9 times elevated AST (145 U/L; reference range 15–37 U/L). Stool analysis was unremarkable, and fecal occult blood test was negative. Chest X-ray showed nonsignificant chest findings. Holoabdominal ultrasound revealed hepatomegaly with a liver span of 17.4 cm with smooth borders and a normal echo pattern but with a normal spleen. Multiple sets of sputum, stool, and urine acid-fast bacilli smears were all negative. Urinalysis showed minimal bacteriuria. ESR and CRP were both elevated. HIV antigen/antibody testing was negative. RPR, anti-HBs, anti-HCV, and HBsAg were all nonreactive. Three sets of blood cultures also turned out negative. Work-ups for infection and malignancy were unremarkable. She was initially started on piperacillin/tazobactam, omeprazole, tramadol, paracetamol, and naproxen. Naproxen was discontinued because of increase in facial flushing and periorbital edema.
On the 10th hospital day, the patient was noted to have pancytopenia (hemoglobin 108 g/L, platelet count of 139 × 109/L, WBC 3.18 × 109/L) with leukoerythroblastosis. Laboratory testing during this time revealed an increase in ESR (from 47 to 92 mm/h), increase in AST (from 145 to 553 U/L), and an increase in ALT (from 32 to 189 U/L). LDH was noted to be markedly elevated (9680 U/L; reference range 100–190 U/L). Triglycerides (3.26 mmol/L) and serum ferritin (>4500 pmol/L) were also markedly elevated. 24-hour urine collection showed total protein of 900 mg with a total volume of 1500 mL. Repeat direct and indirect antiglobulin tests were negative. Anti–cyclic citrullinated peptide was negative. Antinuclear antibody was positive but anti-double-stranded DNA was negative, and C3 was normal. Bone marrow aspiration (BMA), and biopsy was done. The BMA smear revealed a normocellular marrow with trilineage hematopoiesis and the presence of hemophagocytosis (see ). The bone marrow trephine biopsy revealed a normocellular marrow with megakaryocytic hyperplasia with moderate myelofibrosis (WHO MF Grade 2) (see ). The bone marrow acid-fast bacilli smear was negative.
Having satisfied the 2012 SLICC revised diagnostic criteria, she was then diagnosed as a case of systemic lupus erythematosus with concomitant autoimmune-associated hemophagocytic syndrome (HScore of 229 with 97.8% probability of HLH) and autoimmune myelofibrosis. She was then started on intravenous hydrocortisone (prednisone 1 mkd equivalent), hydroxychloroquine, and calcium plus vitamin D supplements. During treatment, all clinical findings (e.g., malaise, generalized weakness, throat discomfort, fever, facial flushing, lymphadenopathies, and arthritis) and laboratory data (e.g., blood counts, LDH, and liver enzymes) gradually improved. No significant treatment-related side effects were noted.
She was discharged on the 19th hospital day on oral prednisone (1 mkd), hydroxychloroquine, and calcium plus vitamin D. She was closely followed at the outpatient clinics with note of continuous improvement in symptoms and laboratory findings. Prednisone was slowly tapered down. Hydroxychloroquine and calcium plus vitamin D were continued. Good adherence to treatment plan was noted. No significant therapy-related side effects were reported by the patient. A repeat bone marrow biopsy was done four months after discharge, which revealed normocellular marrow with trilineage hematopoiesis with complete resolution of myelofibrosis and hemophagocytosis ().
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pmc-6343168-1
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A 72-year-old man with a history of hypertension and cerebrovascular accident (CVA) 20 years ago without significant residual weakness presented to the emergency department with a 3-day history of right-sided facial droop, slurred speech, and clumsiness of the right hand. He had a 20 pack-year smoking history. His family history revealed a father who died suddenly at the age of 47 from an unknown cause, a brother with acute leukemia, and a sister with myocardial infarction at the age of 37 years.
On examination, he had mild right facial droop, mild dysarthria, right pronator drift, and 4/5 motor strength in the right upper and lower extremity. The remainder of the neurological examination was unremarkable. Other significant findings in physical examination were mild hepatosplenomegaly with axillary and cervical lymphadenopathy. Hematological investigations on admission showed severe anemia with a hemoglobin (Hb) level of 44 g/L, leukocytes 42.8 × 109/L, lymphocytes 35.95 × 109/L, neutrophils 4.28 × 109/L, and a platelet count of 120 × 109/L. Further investigations were most consistent with AIHA with an unconjugated hyperbilirubinemia (2.7 mg/dL), elevated LDH (444 IU/L), low haptoglobulin (<15 mg/dL), an elevated reticulocyte count 83.62 × 109/L (7.4%), and a positive direct antiglobulin test (DAT) with both IgG and anti-C3d.
Peripheral blood flow cytometry showed a monoclonal B-cell population with surface lambda-positive population and positive for CD 45, CD 19, CD 20 (weak), CD 22, CD 23, CD 5, and CD 38 (partial). This immunophenotype pattern was consistent with diagnosis of CLL. The clinical picture was consistent with modified Rai stage III CLL. The patient had multiple autoantibodies on cross match.
Computed tomography (CT) of the head was negative for acute bleed or mass effect but was suspicious for evolving stroke. It also showed an area of encephalomalacia in the left basal ganglia related to an old infarct or hemorrhage. The patient could not receive thrombolytic therapy due to severe anemia. Brain magnetic resonance imaging (MRI) showed multiple small areas of diffusion restriction with corresponding mild T2 hypersensitivity in the bilateral corona radiata and centrum semiovale. These findings were consistent with subcortical WI in a characteristic “string of pearls” pattern (). Magnetic resonance angiogram (MRA) of the head and neck was negative for stenosis in any of the major vessels. Transthoracic echocardiogram showed normal left and right ventricle systolic function with normal ejection fraction. Subsequent transesophageal echocardiogram did not show any left atrial thrombus or atrial mass. Interatrial septum was also intact. No arrhythmias were noted on telemetry during the admission; however, on a subsequent admission the patient was noted to have paroxysmal atrial fibrillation.
The patient was transfused to a goal Hb of 8 g/dL with least incompatible packed red cell (PRBC) transfusions. A total of 7 units of PRBC were transfused during the hospital stay, which the patient tolerated without any significant reactions. AIHA was treated with intravenous immunoglobulin (IVIG) 1 g/kg body weight daily for five days followed by 1 mg/kg of prednisone daily. At the time of discharge, the patient's Hb was 8.8 g/dL and LDH had normalized. There was no significant change in the platelet count with steroids. At discharge, oral prednisone was continued for four months and gradually tapered off over the next two months.
His blood counts stabilized with steroids and IVIG. About 1 year after initial presentation, he was found to have worsening diffuse adenopathy in the posterior cervical and axillary areas. PET/CT done to evaluate the possibility of Richter's transformation showed extensive bulky hypermetabolic lymphadenopathy in the head and neck region, bilateral axilla, bilateral hila, mediastinum, and throughout the mesentery and retroperitoneal distribution of the abdomen and pelvis. Maximum standardized uptake value (SUV) of 4.64 was noted in the right posterior cervical region. At this time, the patient's LDH was 538 IU/L. Repeat flow cytometry showed a monoclonal surface lambda population which was positive for CD 20 (weak), CD 19, CD 5, CD 23, CD45, and CD 22. This pattern was again consistent with CLL.
The patient's clinical status declined rapidly. Plan for excision lymph node biopsy to rule out Richter's phenomenon had to be deferred due to severe debilitation and refractory AIHA. He was started on obinutuzumab with chlorambucil but had an anaphylactic reaction to obinutuzumab which led to discontinuation after 2 doses. His diffuse adenopathy did respond to the two doses of obinutuzumab with near-complete resolution over the next several days. The patient however remained very debilitated and confined to bed and further consideration of therapy was not appropriate and further staging of his lymphoma was deferred until his condition improved. His hospital course was complicated with recurrence of autoimmune hemolytic anemia, which was managed with blood product transfusions, steroids, and IVIG as before. He was eventually started on rituximab for refractory AIHA. He was in the middle of his second course of rituximab before his counts began to improve.
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pmc-6343172-1
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At first medical contact (FMC), a 70-year-old Caucasian woman who admitted to be a heavy smoker and was slightly overweight, presented to the emergency room (ER) with severe dyspnea. Her past medical history, medications, and background information are summarized in .
Hypoxia with pulse oxygen saturation < 85%, tachypnea, tachycardia, and hypertension were present. Signs of infection, cyanosis, and peripheral edema were absent. Clinical examination revealed expiratory wheezes and prolonged expiration. Chest pain and electrocardiogram (ECG) abnormalities were absent. Chest radiography exhibited bilateral flattening of the diaphragm but no pulmonary infiltrates or pneumothorax. White blood cell count (WBC) and C-reactive protein (CRP) were mainly normal, but troponin T (TPNT) level was elevated (53 ng/L: normal level, <15 ng/L). The patient was admitted to the hospital, and standard treatment for acute exacerbation of COPD was initiated.
Soon after admission, increased dyspnea and vague chest discomfort were observed despite normal pulse oxygen saturation. ECG revealed T-wave inversion in several leads, normal QT interval (, FMC), and an increased TPNT level of 108 ng/L. Pulmonary embolism and aortic dissection were outruled via computed tomography (CT). Echocardiography (ECHO) revealed a normal left ventricular ejection factor (LVEF) without dyskinesia, at the time (see ). Dual antiplatelet therapy (DAPT), in concordance with the existing guidelines of the European Society of Cardiology (ESC) for ACS, was initiated, and coronary angiography was performed. The latter showed no signs of significant stenosis or other pathologies that could explain the patient's symptoms. During the following days, no other episodes of dyspnea or chest pain were registered, ECG and TPNT level returned to normal, and the patient was discharged from the hospital with prescriptions for standard treatment for COPD. Upon the initially assumed absence of pathology in ECHO, the patient was diagnosed with myocarditis and followed up 3 months after FMC with a new ECHO, which was without abnormalities. During the follow-up period, the patient managed to quit smoking.
Almost 32 months after FMC, the patient was again admitted to the hospital. This time she was suffering from exhaustion, dyspnea, and leg swelling for 2 weeks. Bilateral crackles and pitting edema of legs were present. ECG revealed T-wave inversions on several leads without QT interval prolongation (). TPNT level was elevated (58 ng/L). Likewise, NT-probrain natriuretic peptide (NT-pro-BNP) level was also abnormal (2088; normal age-adjusted level, <450 pg/mL). CT scan showed no signs of pulmonary embolism, aortic dissection, or pleural effusion. ECHO, at the time, revealed severe left ventricular hypokinesia, most prominent in the inferolateral wall and the septum. LVEF was approximately 30%. Subsequent analysis revealed severe midventricular hypokinesia instead of inferolateral (see ). DAPT and heparin were given and acute coronary angiography was performed. Once again, no significant pathology was found despite the patient's severe acute heart failure. Also, as during FMC and upon treatment with diuretics and standard COPD medications, dyspnea and chest pain disappeared, ECG returned to normal, and crackles and pitting edema were gone. TPNT levels decreased as well (). The patient was then diagnosed with non-Q-wave myocardial infarction, and she was prescribed metoprolol, ramipril, and eplerenone. Cardiac magnetic resonance imaging (CMR) was performed 3 weeks after discharge from the hospital. Oddly, no signs of previous myocardial infarction or myocarditis were found, LVEF was calculated to be 55%, and hypokinesia was absent. Subsequent follow-ups confirmed that the patient was feeling well without symptoms of heart failure or angina pectoris. Moreover, NT-pro-BNP level was normal and a new ECHO was completely normal. Treatment with eplerenone was discontinued due to adverse side effects, and after some months, follow-ups were discontinued on the patient's initiative.
Eight months after the second episode (4 months after the last follow-up), the patient was once again hospitalized due to acute COPD exacerbation. Chest pain was denied, but a burning feeling over the back was present. Clinical examination was concomitant with previous episodes, but pitting edema and crackles were absent. The initial ECG at ER was normal except for sinus tachycardia, and cardiac troponin, NT-pro-BNP, CRP, and WBC levels were normal.
During the first hours after admission, severe dyspnea was observed and TPNT levels increased to 244 ng/L. ECG now revealed T-wave inversions similar to those found at previous hospitalizations (). A small quantity of pleural effusion was found at CT scan, but pulmonary embolism and aortic dissection were absent. ECHO showed severe left ventricular hypokinesia, most manifest in the apical and midventricular segments. LVEF was estimated at 20%. NT-pro-BNP level increased from the previous normal level to 1000 pg/mL. As chest pain was still absent, treatment with intravenous diuretics was started; the patient's status improved rapidly, but the TPNT level remained unchanged. On day 4, severe chest pain started and the TPNT level increased to 300 ng/L. ECG was still abnormal with unspecific T-wave inversions. DAPT was given, and coronary angiography was once again normal. Astonishingly, soon after coronary angiography, chest pain ceased, ECG and NT-pro-BNP were normal, TPNT levels decreased considerably, and ECHO revealed an LVEF of 50-60% without hypokinesia. She was again diagnosed with myocarditis, and furosemide was prescribed. A follow-up within a month was planned.
Almost 2 weeks after being sent home, the patient returned to the ER with dyspnea and acute chest pain. Crackles were present, but chest radiography was normal. Pitting edema was absent. TPNT level was 107 pg/mL. The patient was again admitted to the hospital, and ECHO performed on the next day was evaluated as normal. At this point, the patient was completely recovered and stated that she had had a rough week with high stress levels. The TPNT level decreased, and since a follow-up was previously planned, she was discharged without new prescriptions. She was diagnosed with unspecific chest pain.
During the succeeding follow-ups, a grade 3 COPD was confirmed via spirometry (FEV1/FVC0.39, FEV1 39% of normal) and her COPD treatment was adjusted. Treatment with metoprolol was substituted with amlodipine, and a new ECHO was normal with an LVEF of 60%.
Six months after past hospitalization (4 months after the last follow-up), the patient was again admitted to the hospital due to severe dyspnea. Chest pain was negated, ECG showed T-wave inversion on leads AVL and I, and TPNT was slightly elevated. ECHO at ER revealed severe generalized left ventricular hypokinesia with midventricular akinesia, but the basal and apical segments were less affected. LVEF was estimated to be less than 20%. On day 2, TPNT level increased to 431 ng/L, NT-pro-BNP level was normal, and T-wave inversions on several leads were observed (). During the following days, the patient recovered, ECG returned to normal, and TPNT level decreased considerably. Importantly, chest pain was never observed. ECHO previous to discharging from the hospital showed normal global function with an LVEF of 50% with remaining left basal hypokinesia. Treatment with carvedilol was started since the patient did not tolerated amlodipine, and verapamil was considered contraindicated due to a potential interaction with lithium (see ). During this hospitalization, the patient was given the diagnosis of TTS for the first time. Additionally, a comprehensive review of the patient's previous medical history and saved ECHO images exposed several episodes of TTS (see ).
Finally, 2 months after her first diagnosed TTS, she was again admitted to the hospital due to dyspnea and elevated TPNT (107 ng/L). ECG was mainly normal. For unknown reasons, an ECHO wasn't performed and the patient was discharged on the first day. Cardiac computed tomography angiography (CCTA) was performed after two months, and no significant stenosis was observed. A previously planned ECHO showed normal LVEF and minor left basal hypokinesia. To date, no new TTS episodes have been observed and concurrently, no new acute COPD exacerbations have been noted.
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pmc-6343178-1
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A 64-year-old woman was admitted to the Neurological Unit of our hospital for recent recurrent episodes of loss of consciousness and seizures. Glycemia and ECG were normal, while hypocalcemia was present.
She has a normal brother and both her parents died in old age; her mother had cognitive impairment. Her clinical history evidenced carpo-pedal spasm since the age of 30 years, cognitive impairment, hypothyroidism diagnosed in early adulthood, spontaneous menarche, and oligomenorrhea followed by amenorrhea at the age of 30, which was diagnosed as precocious menopause. She was unmarried and had no pregnancy. She underwent bilateral hip arthroprosthesis at 45 and 50 years of age.
She was taking oral calcium (600 mg daily) and cholecalciferol (400 IU daily) for chronic hypocalcemia, diagnosed about 30 years earlier. She was also on therapy with perindopril for hypertension, atorvastatin for hypercholesterolemia, and L-thyroxine.
Physical examination revealed short stature (145 cm), slight overweight: 52 Kg (BMI: 25 Kg/m2), round facies, enlarged base of the nose, and brachydactyly. Her blood chemistry evidenced hypocalcemia (7.7 mg/dl, n.v. 8.2-10.2) with increased PTH levels (169 pg/ml, n.v. 15-65 pg/ml, intact PTH immunoassay), moderate 25OH vitamin D deficiency (22 ng/ml; n.v. ≥ 30), normal creatinine (1 mg/dl), and albumin (3.9 g/dl).
Brain computed tomography (CT) revealed calcifications of the basal ganglia, the cortical and subcortical white matter, and the cerebellum (dentate nuclei); subcutaneous pericranial ectopic calcifications were also present (Figures , , and ). Hand radiography confirmed shortness of the metacarpal bones and scapho-trapezoidal fusion (not shown). Bone mineral density of the spine and femoral bone was normal. Abdominal ultrasonography did not reveal kidney stones. The clinical picture was suggestive of PHP1A. The patient was therefore promptly switched to 1,25 (OH)2 vitamin D (calcitriol 0.75 mcg daily, in 3 split doses) associated with oral calcium supplementation (1000 mg daily in 2 split doses, after lunch and dinner), with normalization of calcemia (9.2 mg/dl) and a decrease in PTH level (36 pg/ml). Serum phosphate was normal on therapy (4.3 mg/dl), while 24-hour urinary calcium on therapy was above the normal range (321 mg/24 h, n.v. <4 mg/kg/body weight); we do not have a clear explanation for this, since urinary calcium excretion is not normally increased in this condition. However, a laboratory assay pitfall or an incorrect urine collection by the patient cannot be ruled out. A further urinary calcium control one month later, however, proved normal (200 mg/24 h). In addition, therapy with levetiracetam (1000 mg daily in 2 split doses) was started, and no further seizures or muscle spasms occurred.
The patient and her family underwent a genetic counseling session and gave informed consent to genetic analysis. Mutation analysis of the GNAS gene was performed on DNA extracted from a blood sample by means of Sanger sequencing and MLPA according to standard methods. The analyses revealed a heterozygous c.568_571del (p.Asp190Metfs∗13) frameshift mutation. The combination of physical, biochemical, and genetic findings led to the diagnosis of PHP1A.
FT4 and TSH levels were normal during substitutive treatment with L-tiroxine (75 mcg daily). Anti-thyroperoxidase and anti-thyroglobulin antibodies were negative and thyroid ultrasonography was normal, suggesting that TSH resistance was the cause of her hypothyroidism.
LH and FSH levels were in the menopausal range (FSH: 47 mU/ml, LH: 26 mU/ml); the history of premature menopause, however, was suggestive of the development of progressive LH and FSH resistance. Prolactin, basal cortisol, and ACTH levels were in the normal range. Basal growth hormone was 0.63 ng/ml, with IGF-1 levels (129 ng/ml) in the normal range for age. Calcitonin levels were increased (range: 44-92 pg/ml, n.v. <10 pg/ml) (she was not taking any proton-pump inhibitor), while carcinoembryonic antigen (CEA) was normal. Abdominal ultrasonography was normal, as were chest-X-ray and mammography. Our interpretation is that calcitonin resistance was also present, as part of the patient's multi-hormonal resistance due to impairment of the cAMP activation pathway [, ].
Six months later, during follow-up evaluation, the patient's calcium level was seen to have increased to 10.6 mg/dl and PTH had decreased to 32 pg/ml; oral calcium supplementation was gradually reduced and then discontinued, and calcitriol was reduced to 0.25 mcg twice daily; subsequent controls revealed normal calcium (10 mg/dl) and slightly increased PTH (72 pg/ml) levels. She had no further loss of consciousness or seizures; on neurological examinations, levetiracetam treatment was confirmed, also on account of the frailty of the patient.
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pmc-6343228-1
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An HIV-infected 32-year-old male presented to Mulago National Referral Hospital, Uganda with a 2-week history of headache with fevers and a 1-day history of confusion (
). He had been on ART (zidovudine, lamivudine, efavirenz) and co-trimoxazole prophylaxis for 5 years. 5 months prior, he was diagnosed with pulmonary TB by positive sputum Xpert MTB/RIF (Cepheid, Sunnyvale, CA, USA). He had completed 2 months of induction TB therapy (rifampicin, isoniazid, ethambutol, pyrazinamide) and was 3 months into continuation phase (rifampicin, isoniazid). He endorsed poor adherence to both ART and anti-tuberculous medications.
On examination, the patient was febrile (38.6°C). His blood pressure was 112/71 mmHg, pulse 94 beats/minute, respiratory rate 48, and oxygen saturation 98%. He was wasted, dehydrated, and had overt rigors. His Glasgow Coma Scale was 14/15 with nuchal rigidity and positive Kernig’s sign. Cranial nerves were intact. He had normal tone and power in all limbs. A clinical diagnosis of HIV-associated meningitis was suspected and he was recruited into the ‘Improving Diagnostics and Neurocognitive Outcomes in HIV/AIDS-related Meningitis’ study (registration:
). Whilst awaiting further investigations, he received empiric therapy of ceftriaxone 2 g twice daily for possible bacterial meningitis.
A finger stick cryptococcal antigen lateral flow assay (CrAg LFA) (IMMY, Norman, Oklahoma, USA) was negative. Liver and renal function tests were normal. Cerebrospinal fluid (CSF) opening pressure was elevated to 33 cm CSF (normal <20 cm CSF), CSF white cells 590 /µl, protein 419 mg/dl (normal range 15–45 mg/dl), CSF lactate 9.5 mmol/L (normal range <2.5 mmol/l). CSF glucose was unavailable.
Mycobacterium tuberculosis in CSF was confirmed on Xpert MTB/RIF Ultra; there was no evidence of rifampicin resistance. On day 2, he was initiated on dexamethasone at 0.4 mg/kg/day and induction TB-medications were re-commenced (rifampicin, isoniazid, ethambutol, pyrazinamide) for TBM. The IV ceftriaxone was stopped, his ART was continued. He continued to spike high-grade fevers (39.6°C.) with tachycardia (pulse 118 beats/min). A peripheral blood smear showed
P. falciparum parasites (1+ trophozoites), despite a negative malaria histidine rich protein-2 (PfHPR2)-based rapid diagnostic test (Malaria
Plasmodium falciparum Rapid Test Cassette, Vaxpert, Florida, USA). Given his ongoing neurological symptoms, which could be compatible with cerebral malaria, the decision was made to treat for severe malaria. Drug-drug interactions (DDIs) between rifampicin and artemisinin compounds, and rifampicin and quinine are recognized (
); a decision was made to treat with IV artesunate as the most efficacious anti-malarial for severe malaria
. He received three doses of IV artesunate (3 mg/kg), after which a repeat peripheral blood smear showed no malaria parasites. He then completed 3 days of oral artemether/lumefantrine. His fevers subsided on day 6. He was discharged on day 8; medication adherence counselling was provided for the patient and his guardian and outpatient follow-up was arranged for the following week.
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pmc-6343237-1
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A 74 year-old man presented with visual acuity deterioration in the right eye (RE). He had history of treated systemic hypertension. Best-corrected visual acuity (BCVA) was 20/80 in the RE and 20/20 in the LE. Fundus examination revealed a subretinal haemorrhage in the RE. Multimodal retinal imaging of the RE showed a type 2 (predominantly classic) CNV complicated by a spontaneous retinal pigment epithelial (RPE) tear (Fig. ). Patient had not received any prior treatments. A course of 3 monthly intravitreal injections of Ranibizumab (0.5 mg × 0.05 mL) was administered. 4 weeks after the third injection OCT scan showed splitting and restoration of the hyperreflective line attributable to the RPE (Fig. ). 9 months after initiation of treatment patient had received six intravitreal injections of Ranibizumab and BCVA improved to 20/32 in the RE.
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pmc-6343241-1
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A 65-year-old woman was referred to our endocrinology center for evaluation of diabetes mellitus, hyperlipidemia, and bilateral adrenal masses, which were detected for the first time prior to a scheduled operation for sigmoid-colon cancer. She previously underwent total hysterectomy for uterine fibroids at the age of 44. She was on anti-hypertensive medication from approximately 40 years of age, and had experienced aortic dissection at the age of 56. She showed normal stature and a body mass index of 24.7 kg/m2. She did not show any Cushingoid signs. Fasting morning serum cortisol and urinary free cortisol levels (measured by immune radio metric assay method, SRL, Tokyo) were normal (Table ). However, midnight levels of serum cortisol were high, and both overnight dexamethasone suppression tests, using 1 mg and 8 mg dexamethasone, did not suppress serum cortisol or dehydroepiandrosterone (DHEA) -sulfate levels. Plasma ACTH levels were low and did not respond to 100 μg of intravenous corticotropin-releasing hormone. Furthermore, a dexamethasone suppression test using Liddle’s method [] showed a paradoxical increase in the levels of urinary cortisol (Table ). The ratio of plasma aldosterone concentration (PAC) to plasma renin activity (PRA) was significantly high, although PAC was within the normal range. Based on the results of endocrinological examinations, the patient was diagnosed with idiopathic hyperaldosteronism [] (Tables and ). Adrenal venous sampling indicated bilateral aldosterone hypersecretion (Table ). Bilateral adrenal tumors, 25 × 13 mm and 18 × 15 mm, in the right and left gland respectively, had the appearance of adrenocortical adenoma on computed tomography (Fig. a, b) and magnetic resonance imaging (Fig. c-f). Accumulations of 131I-adosterol in adrenal tumors were observed on both sides, though predominantly on the left (Fig. g). Various extra-adrenal masses were detected in several imaging modalities, and patchy brown skin pigmentations were observed systemically (Fig. ).
Thus, the patient was diagnosed with SCS [] due to bilateral functioning autonomous cortisol secreting adrenal tumors []. Although serum cortisol and urinary free cortisol levels decreased after left unilateral laparoscopic adrenalectomy, the paradoxical response persisted (Table ). Pathological examination revealed adrenocortical adenoma. The tumor consisted of round to polygonal-shaped cells with microvascular or eosinophilic cytoplasm, proliferating in an alveolar fashion, accompanied by hemorrhage, inflammatory infiltrate and lipochrome deposit, leading to the diagnosis of adrenal adenoma. Immunohistochemical analysis showed positive expression for cytochrome P450 (CYP) 17A1, HSD3B type-1, HSD3B type-2, dehydroepiandrosterone sulfotransferase, and CYP11B1, but not for CYP11B2 (Fig. ). Genetic examination of the adrenal tumor revealed the somatic GNAS mutation p.R201H, which is known to be responsible for McCune-Albright syndrome, although sporadic GNAS mutations have also been reported []. No PRKAR1A mutation was detected in either the adrenal adenoma or the peripheral blood. The patient was treated with eplerenone, which had successfully ameliorated persistent hypertension and hypokalemia at her one year follow up visit.
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pmc-6343249-1
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A 23-year-old European woman who was working as a secretary, presented at an out-patient clinic with a 9-month history of a painless swelling on the right posterolateral side of her chest wall. She did not recall any trauma that may be associated with this condition neither did she have a history of genetic disease or cancer. A physical examination revealed a hard, painless mass at the posteroinferior and lateral thoracic region. The results of her laboratory tests were all within normal limits.
On a plain chest radiograph, an area of calcified opacity was observed at the ninth rib, with no destruction of the underlying bone. Computed tomography (CT) demonstrated a mass of 6 cm × 5 cm × 2.5 cm in size arising from the ninth rib (Fig. ). There was no evidence of cortical destruction or medullary involvement of the rib. Whole-body scintigraphy and CT did not show any skip or lung metastases.
After these examinations, an incisional biopsy was performed. Histopathologic examination revealed fibroblastic and osteoblastic cells with mild nuclear atypia and pleomorphism which was consistent with parosteal OS.
She was informed and a wide segmental resection was applied to her eighth, ninth, and tenth ribs with the involvement of parietal pleura (Fig. ). Afterward, chest wall reconstruction was made using collagen mesh and low-profile locked plate for the prevention of flail chest (Fig. ).
On gross examination, the lesion was found to be attached to the outer surface of her ninth rib measuring 7 cm × 3 cm × 5 cm. Histopathological evaluation of the resected specimen confirmed it to be parosteal OS. Our patient had no chemotherapy and throughout a 1-year follow-up, there was no evidence of local recurrence or distal metastasis. Informed consent was obtained from our patient before this report.
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pmc-6343251-1
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A female, 36 years old, had symptoms of pulmonary TB and intracranial TB which were alleviated after using anti-TB drugs, but the swelling and pain of the left ankle lasted for 1 year. Ankle TB was confirmed with the pathological examination of focal tissue after arthroscopy. It showed hyperplasia of synovial tissue, pale areas, regional congestion, scattered fibrous protein, and necrotic tissue, but the residual cartilage remained stable. Arthroscopic debridement was performed to remove the ankle scarring tissue and improve the motor function of the ankle. Re-examination showed she was cured with an AOFAS score of 93 points (Fig. , Additional file 1: Video S1).
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pmc-6343251-2
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The right ankle of a 64-year-old male gradually became swollen and painful. The results of venous blood test for rheumatoid factor (RF), anti-cyclic citrullinated peptide (CCP) antibody, anti-keratin antibody (AKA), and HLA-B27 were all negative. The patient received treatment for rheumatoid arthritis and was given analgesic drugs orally; no hormone was taken orally. The curative effect was poor. After 6 months of treatment, the ankle joint pain and swelling were aggravated and claudication occurred. Venous blood test was performed again, and the results of T-SPOT.TB test and TB antibody were positive; chest radiograph showed pulmonary TB. Ankle arthrocentesis was conducted; the result of bacterial culture was negative and suspected TB for pathological examination. Quadruple anti-TB therapy (isoniazid, rifampicin, pyrazinamide, ethambutol) was given orally. After 3 weeks of treatment, the swelling of the right ankle joint was relieved. ESR was improved from 42 mm/h before treatment to 25 mm/h. Then ankle arthroscopy was performed. Under arthroscope, a small area of defect in the ankle cartilage was seen, but the cartilage remained stable without looseness; the ankle joint had a large amount of fibrous protein as well as hyperplasia and hyperemia of the synovial tissue. Samples were taken for TB culture and pathological examination, and then ankle debridement was performed. Postoperative pathological examination confirmed ankle TB. Anti-TB treatment was continued for 18 months. At the last follow-up, the symptoms of the ankle joint disappeared, the ESR was 8 mm/h, and the AOFAS score improved from 49 points before treatment to 94 (Fig. ).
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pmc-6343251-3
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A male, 11 years old, suffered from pain in the left ankle joint after trauma and limitation of motion for 10 years, and the symptoms aggravated with claudication for 1 year. Physical examination showed swelling of the left ankle joint, extensive tenderness, normal skin temperature, and limited range of motion for ankle plantar flexion and dorsi flexion. Venous blood test showed no abnormalities in ESR, CRP, and blood routine. Radiograph and MRI suggested hyperplasia of synovial tissue in the articular cavity and epiphyseal injury of the distal tibia. Ankle arthrocentesis was conducted, and a small amount of turbid liquid was drained. No diagnosis of ankle TB was suggested. Preoperative diagnoses were (1) traumatic ankle synovitis and (2) epiphyseal injury of the left distal tibia. We initially planned to perform articular cavity debridement; however, when we conducted the surgery, we saw obvious hyperplasia of synovial tissue and the cartilage was obviously damaged; TB was highly suspected. Considering that the patient was still a child, ankle arthrodesis was not suitable in this case. Therefore, we only performed articular cavity debridement after obtaining the tissue sample for biopsy. Pathological examination confirmed ankle TB, and the result was also positive for TB-PCR. The patient was prevented from weight bearing for 6 weeks postoperatively; anti-TB treatment of rifampicin, isoniazid, and pyrazinamide were given orally; his nutrition was strengthened. Regular follow-ups were conducted. The swelling of the posterior malleolus was gradually relieved. After the reexamination at 6 months postoperatively, his anti-TB therapy was adjusted and only rifampicin and isoniazid were continued for maintenance treatment of 12 months. At the last follow-up at 5 years postoperatively, the patient’s left ankle swelling and pain disappeared, and the range of motion for ankle plantar flexion and dorsi flexion was basically normal. The AOFAS score improved from 57 points preoperatively to 97, with ESR 1 mm/h. Radiograph and MRI suggested that the ankle joint space was slightly narrow, the surface of tibiotalar joint was not smooth, and the lesion of synovial hyperplasia disappears. The patient was satisfied with the results (Fig. , Additional file 2: Video S2 and Additional file 3: Video S3).
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pmc-6343319-1
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A 67-year-old postmenopausal woman with hirsutism of increased hair around the upper lip and armpit and clitoromegaly for five months was referred to the endocrinology clinic of our hospital. She had normal physiological development during her infancy and childhood, and also has a normal sexual life with no other medical history. Her menarche was at 18 years old, and her menopause at age of 56. She had a normal menstrual history before menopause and had no postmenopausal bleeding. She had two healthy children and no miscarriages. She denied taking estrogen, progesterone or health care products. There are no similar patients in her family.
On physical examination, she was 153 cm tall and weighed 53 kg with body mass index of 22.6 kg/m2. Increased hair was observed in her upper lip and armpit (Ferriman- Gallwey score of 8), and a physical examination of genital revealed clitoromegaly. There was no acne, deepening of the voice or other virilization signs. Findings on examination of the head and neck, breasts and abdomen were unremarkable. She had no signs of Cushing syndrome, or acanthosis nigricans syndrome.
The hormonal test showed high total testosterone levels (714.8 ng/dL, reference value 14–56). Serum DHEAS (145.8 ng/mL, reference value 25.9–460.2), androstenedione (2.4 ng/mL, reference value 0.3–3.3) and 17-hydroxyprogesterone (1.7 nmol/l, reference value 0–11.5) levels were within normal range. The serum values of follicle-stimulating hormone, luteinizing hormone, and prolactin were also within the normal range for the menopause. The levels of anti-mullerian hormone, human chorionic gonadotropin (hCG), thyroid- stimulating hormone (TSH), plasma renin activity and aldosterone, adrenocorticotropic hormone (ACTH), serum cortisol, 24-h urinary free cortisol, and 1 mg dexamethasone suppression test were in normal range. The ovarian tumor markers (Ca 125, CEA, Ca 199) were in normal reference range. The repeated samples confirmed that her high testosterone levels were within the tumor range. We excluded overt Cushing Syndrome on the basis of normal cortisol suppression after 1 mg dexamethasone and normal urinary free cortisol levels, as recently proposed by Ceccato F []. Then a middle dosage dexamethasone test (0.75 mg, 4 times a day for 5 consecutive days) without testosterone inhibition strongly suggested the potential androgen-producing tumor, further examinations were needed to distinguish ovarian or adrenal origin of hyperandrogenemia.
Initially, the lack of co-secretion of DHEAS and androstenedione indicated that her elevated testosterone might be of ovarian origin. However, pelvic ultrasound disclosed that there was no ovarian mass, while adrenal ultrasound showed a hypoechoic nodule in the left adrenal gland. Further pelvic magnetic resonance image (MRI) showed submucous myoma of uterus, but no abnormal of ovarian, and adrenal CT scan was also performed and a left adrenal mass of about 1.5 cm in diameter was revealed (Fig. a and b). PET-CT confirmed a round nodule in the external branch of the left adrenal gland with slight increase in FDG metabolism (the SUV max of the nodule was 2.56), considering the possibility of benign adenoma. No ovarian abnormalities or other ectopic tumors were found by PET-CT.
Based on the clinical characteristics, hormone detection and imaging appearances of the case, pure testosterone-secreting adrenal tumor was suspected. Subsequently, the patient underwent a laparoscopic resection of left adrenal tumor. Histological examination (Fig. a) and immunohistochemistry also confirmed the diagnosis of benign adrenocortical adenoma with immunohistochemistry positive for inhibin α, melan A, β-captenin (Fig. b-d), SYN (focal), Ki-67 (< 3%), and negative for chromogranin (CgA), cytokeratin (CK), S-100, P53. The level of testosterone decreased to 15.8 ng/dl on the 3rd day after operation, and the symptoms of virilization were alleviated during the follow-up, which further confirms the adrenal etiology of the testosterone production.
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pmc-6343326-1
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A 58 years old man presented to dermatology clinic with 2 years history of recurrent painful mouth sores and cutaneous blisters on his extremities and genital area. A review of symptoms was notable for eye irritation, redness and foreign body sensation in both eyes. The patient was not known to have any medical illnesses and was not taking any medications. Physical examination found confluent erosions on the hard and soft palates, buccal mucosa, and on the lateral sides of his tongue (Fig. ). Skin examination revealed atrophic and hyperpigmented scars on the anterior side of both thighs. We also noticed a small atrophic scar on the penile shaft. His left middle finger showed periungal erythema and swelling that was tender to palpation. Ophthalmologic evaluation revealed chronic conjunctivitis on both eyes with fornix shortening in the right eye (Fig. ). Nasal scope examination showed few erosions. Laryngoscopy showed erythematous mucosa over the arytenoids. Gastrointestinal evaluation was normal. Histopathological examination of an oral mucosal biopsy showed sub-epithelial blister with underlying chronic inflammation. Immunofluorescence studies were negative. On the basis of the clinical assessment and histopathological results we retained the diagnosis of MMP. The patient was initially treated with 1 mg/kg of prednisone which resulted in a rapid control of his symptoms but when the dose was tapered to 0.5 mg/kg the patient showed signs of disease recurrence. 2 g/kg/cycle of intravenous immunoglobulin therapy IVIG was added. The patient received three cycles on a monthly interval and showed remarkable improvement. Prednisone dose was tapered to 0.25 mg/kg with no signs of disease activity. The left middle finger periungual inflammation had subsided but onychomadesis was noted on the same nail (Fig. ).
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pmc-6343343-1
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A 32-year-old female patient presented to the gastroenterology department of the First Affiliated Hospital, Jinan University, Guangzhou, China, in 2017 with complaints of gradually severe bloating, epigastric and left flank ache, nausea and occasional vomiting of 1 month’s duration. The epigastric and left flank ache was aggravated when the patient was supine and relieved in a prone or left lateral decubitus. The abdominal bloating was associated with early satiety. The vomiting always began 40 min after meal. The patient provided a history of urine stone with oral drotaverine hydrochloride tablets treatment 40 mg three times a day (tid) for two weeks before the gastrointestinal symptoms arising. The patient had no significant surgical history, but had a rapid weight loss of approximately 10 kg with a body mass index from 21 kg/m2 to 18 kg/m2 over the last two months. An abdominal examination revealed upper abdominal tenderness and distention. The urine routine examination showed no significant abnormality (no hematuria and proteinuria). There were no remarkable abnormalities during the initial blood tests and other laboratory investigations.
On performing a physical examination, her epigastric region was distended and tender to palpation. Contrast-enhanced abdominal computed tomography (CT) demonstrated gastroduodenal dilatation (Fig. a). There was narrowing of the third portion of the duodenum compressed by SMA and AA, with a decreased aortomesenteric distance of 3.7 mm and a narrower aortomesenteric angle of less than 15 degrees, which suggested a diagnosis of SMA syndrome (Fig. b, arrow). In addition, the LRV was compressed to 2 mm between SMA and AA (Fig. c, arrow), with a 12 mm dilatation in diameter (Fig. c, star), which formed a “bird beak sign” (Fig. c, arrow). Upper gastrointestinal double-contrast radiograph showed a vertical band of extrinsic compression (Fig. , arrow) on the mid transverse part of duodenum caused by SMA with proximal duodenal dilatation (Fig. , star). Gastroptosis was also observed by fluoroscopy. Colour Doppler indicated that the inside diameter of compressed stenosis of LRV was 1.4 mm on the left edge of AA, with 61 cm/sec of the maximum blood flow velocity. The inside diameter of proximal dilatation site was 6.1 mm, with 20 cm/sec of the maximum blood flow velocity. Additionally, the angle between SMA and AA was approximately 13 degrees, and bilateral iliac vein flow was slow. Therefore, a diagnosis of SMA syndrome and Nutcracker syndrome was confirmed.
Nasogastric tube was placed for decompression. Fluid resuscitation with parenteral and enteral nutritional support was managed conservatively to improve weight gain. Considering the neuromuscular and motility function could be impaired by the disease, pharmacotherapy (Mosapride citrate dispersible tablets 5 mg po tid) and physiotherapy (Functional gastrointestinal treatment apparatus) were treated to modulate the gastrointestinal motor function. Patient underwent a gastroduodenoscopy after the condition was relieved, revealing no intrinsic obstructions. Considering no significant abnormalities in renal function parameters and blood pressure, no special medical interventions were performed for the Nutcracker syndrome. One week later, the patient was discharged and subsequently received family nutrition support treatment for six months.
BMI data were collected each week during the family nutrition support treatment (Fig. a), and BMI values gradually increased (Fig. b). However, in the fourth month, she suspended the family nutrition support and began to work. BMI quickly reduced, and the symptoms recurred including bloating, epigastric pain, left flank ache and nausea. During her second hospitalization, an abdominal examination revealed upper abdominal distention, and the urine routine examination showed hematuria. Nasal jejunal tube was placed and Enteral nutrition was provided for one week. After weight gain and hematuria disappeared, the patient discharged. Subsequently, the patient received family nutrition support treatment for six months, and BMI values fluctuated within the normal range (18.8 ± 0.13 kg/m2 to 19.1 ± 0.74 kg/m2).
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pmc-6343460-1
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A healthy 13-years-old boy presented with a tonic-clonic seizure, electroencephalography (EEG) demonstrated generalized spike-wave discharges, suggesting generalized epilepsy and sodium valproate was initiated as therapy. His anthropometric parameters at presentation were: weight 45 kg and height 160 cm (Z-score −0.6 and −0.4, respectively) with a body mass index (BMI) of 17.6 kg/m2 (Z-score 0.5). The clinical exam and the cognitive development were normal at time of presentation. By 6 months, the patient was on triple therapy (sodium valproate, perampanel, clonazepam) for increasing seizures and myoclonus. Six months following the diagnosis of epilepsy, he was found to have fasting (6.8 mmo/L) and varying postprandial (11.1–13.8 mmo/L) hyperglycemia, and glycosuria without ketonuria (Table ). His past medical history was unremarkable, and he did not take other medications (apart from the antiepileptic medications) and had a negative family history for diabetes mellitus. His myoclonus worsened with progressive severe neurological sequelae (gait ataxia, loss of autonome ambulation, dysarthria, cognitive deterioration with extreme speech difficulties).
Evaluation of glucose metabolism showed fasting hyperglycemia (6.8 mmol/L), glycosuria, negative ketonemia and ketonuria, and glycated hemoglobin (HbA1c) of 7.5%. The insulin secretion was preserved (15.7 mU/L, C peptide 0.93 nmol/L, fasting levels) with a HOMA-IR index (homeostasis model assessment insulin resistance) {calculated as [fasting glucose (mg/dl) x fasting insulin (lU/ml)/405]} at 4.76, suggestive of insulin resistance. The child did not display clinical features of insulin resistance (acanthosis nigricans, abnormal adipose tissue distribution, or lipodystrophy) and his lipid profile and hepatic function were normal at presentation and remained so during the follow-up.
The pancreatic autoimmune markers (ICA, GAD65, IAA, ZnT8) were negative. Testing for monogenic diabetes revealed no mutations in any of the known genes (GCK, HNF1A, HNF4A, HNF1B, ABCC8, KCNJ11, and INS). The diagnosis of diabetes was made, a basal-prandial insulin regimen started, and a normal glycemic profile was quickly obtained with a very low total daily dose of insulin (0.25 μ/kg/d). The diagnosis of type 1 diabetes mellitus (T1DM) was not the right one, but at this point of diagnosis approach we were unable to define more precisely the association between the progressive myoclonic epilepsy and the hyperglycemia. Mitochondrial disease was also excluded.
Six months following the diagnosis of diabetes mellitus, the patient was under 0.2 μ/kg/d of long-acting insulin analog and showed an excellent glycemic profile (HbA1c 6%). Testing for autoimmune markers remained negative. A decision on continuing the same insulin regimen (only long-acting insulin analog) was encouraged.
Twenty four months following the first presentation and eighteen months after the diagnosis of diabetes, the patient's neurological status continued to worsen with a significant cognitive deterioration despite being under four antiepileptic drugs. His metabolic profile remained uncontrolled with persistent hyperglycemia (HbA1c 8.2%) and hyperinsulinemia (insulin 29.4 mUI/L, C peptide 1.62 nmol/L, fasting levels).
The complex nature of the metabolic and progressive neurological disease (uncontrolled seizures and unexplained insulin resistance) mandated high suspicion and testing for LD. PAS positive LBs are typically found in the eccrine duct and apocrine myoepithelial cells of sweat glands (). An axillary skin biopsy was taken accordingly and revealing LBs within apocrine myoepithelium. Genetic testing displayed a homozygous mutation NHLRC1 c.386C > A, p.Pro129His, confirming the diagnosis of LD (Figure ).
At the time of LD diagnosis, in the absence of any insulin regimen, a reevaluation of pancreatic insulin secretion demonstrated an increasing insulin resistance (insulin 29.4 mUI/L, C peptide 1.62–1.8 nmol/L, HOMA-IR 15.97). Based on the biochemistry results displaying high levels of endogenous insulin, metformin (starting a daily dose of 500 mg, increased to 1.5 g daily) was started with good tolerance and response (Table ). As the progressive subsequent increase of HbA1c levels was noted, metformin was increased to 1,000 mg/day, which resulted in intestinal side-effects, and he was switched to long-acting insulin analog, without clear glycemic improvement (HbA1c at 8.3%). T1DM antibodies remained negative, with residual insulin secretion (C peptide at 1.8 nmol/l), confirming ongoing insulin resistance. The child did not develop clinical features of insulin resistance (acanthosis nigricans, abnormal adipose tissue distribution, or lipodystrophy). Currently, the patient remains under 1,500 mg metformin daily, with favorable glycemic control (Table ). Due to the continuous neurological and clinical degradation, metformin administration was discontinued multiple times for several weeks resulting in fasting (13.8 mmol/L) and postprandial (16.6 mmol/l) hyperglycemia (Table ).
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pmc-6343859-1
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An 80-year-old African-American man, with the history of myocardial infarction in November 2000 with two-vessel CABG (coronary artery bypass grafting) performed at that time, now presented to the outpatient radiology suite for a chest X-ray, to follow up on recent pneumonia. An X-ray revealed an abnormal contour of the right heart border (Figure ).
In order to further investigate the abnormal contouring of the right heart border, a CAT (computed tomography) scan of the chest was performed. Upon checking CAT scan results, the patient was found to have a partially thrombosed aneurysm arising from the native right coronary artery (RCA) measuring approximately 6.9 cm that was also compressing the right atrium (Figure ).
The patient did not report any symptoms of chest pain, shortness of breath and dizziness. He stated that he was in his usual state of health. He presented with baseline bradycardia with heart rate in the 40s, however, since he was asymptomatic, no further treatment was performed for the bradycardia. Electrocardiogram revealed a bi-fascicular block with Wenckebach. Other labs were normal except for the leucocytosis due to pneumonia (Figure ).
Upon initial evaluation and the echocardiography (Figure ), it was uncertain whether the aneurysm was from the venous graft or the native coronary artery. Thus, it was decided to proceed with the cardiac catheterization to further evaluate the nature of the aneurysm. Catheterization revealed patent LIMA (left internal mammary artery) and RCA grafts along with a native RCA aneurysm (Figure ).
The case was discussed with cardiothoracic surgeons for interdepartmental consultation and the consensus was that the patient will need a cardiac MRI (magnetic resonance imaging) for the imaging of RCA anatomy prior to intervention. They planned to consult with interventional cardiology for possible covered stent vs. ostial coiling. During the entire eight-day hospital stay, the patient remained completely asymptomatic. He was performing his activities of daily life without any difficulty. Upon discharge, the patient was counseled about the follow-up for outpatient cardiac MRI and to report to the ER if he feels any kind of symptoms including chest pain, dizziness or shortness of breath.
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pmc-6343860-1
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We present a case of a 53-year-old female who presented with a long-standing history of right flank pain. Computed tomography (CT) scan showed bilateral renal artery aneurysms measuring less than 1 cm on the left, and 2.1 x 1.5 cm and 1.7 cm on the right (Figure ). As is a typical case, these RAAs were asymptomatic and because of their position in the hilum, these were anatomically suitable for an in situ repair.
It was decided that an ex vivo laparoscopic reconstruction of the renal artery using a graft from the internal iliac artery was the best approach, as shown in Figures -.
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pmc-6343862-1
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A 46-year-old man with past medical history significant for epidermolysis bullosa acquisita, prediabetes, and dyslipidemia presented to our emergency department (ED) with fatigue, polyuria, and weakness. The patient had seen his dermatologist eight weeks prior to presentation for a flare-up of epidermolysis bullosa acquisita, and was started on 60 mg of oral prednisone daily with a prolonged taper. His dose at the time of presentation was 30 mg. On the day of presentation, he reported two weeks of increased urinary frequency, dry mouth, diffuse muscle cramps, three days of weakness and fatigue, and one day of dizziness. He had been treated with prednisone several times since his diagnosis at age 14, but had never experienced these symptoms. A hemoglobin A1C obtained five months prior to admission was 6.4%, but the patient was not aware of a prediabetes diagnosis. He admitted to drinking sugary drinks regularly.
On examination, vitals revealed a temperature 98.1°F, pulse 96 beats per minute, respiratory rate 18 breaths per minute, and blood pressure 142/91 mmHg. Dry mucous membranes were present. Bullae were noted on the tongue, soft palate, and dorsal hands and elbows bilaterally. Figure demonstrated the tongue bullae. Otherwise, physical exam was unremarkable.
Labs revealed blood glucose of 786 mg/dL and negative urine ketones. Anion gap was normal, so this presentation was consistent with hyperglycemic hyperosmolar state. The patient was also found to have an acute kidney injury with a serum creatinine of 2.8 mg/dL, up from his baseline of 1.2 mg/dL. This was suspected to be prerenal in the setting of dehydration, which was supported by a fractional excretion of sodium of 0.8%. The patient’s glucose decreased to 413 mg/dL with a one liter normal saline bolus and 8 units of intravenous regular insulin in the ED, and he was admitted to the medicine service for further management of hyperglycemia and acute kidney injury. Intravenous fluids were continued, and his creatinine gradually normalized. Dermatology was consulted and recommended an adjusted prednisone taper with an immediate change from 30 to 20 mg daily. On admission, the patient was initially started on only a sliding scale insulin regimen by the night float resident physician, but had multiple subsequent blood glucose readings in the 300-400 mg/dL range. The next morning when the patient was staffed with an attending physician glargine insulin was added. A repeat hemoglobin A1C was 11.9%. Endocrinology was consulted. The patient ultimately required 30 units of glargine insulin and 10 units of aspart insulin with meals. He was discharged on this regimen after receiving diabetic supplies and insulin administration education.
When the patient followed up one week after discharge, his average blood glucose reading was 300 mg/dL despite taking insulins as prescribed. Aspart insulin was increased to 12 units, with a good response. The patient’s outpatient dermatologist then noted that his epidermolysis bullosa acquisita was active despite adherence to the adjusted prednisone taper, and he was bridged to a steroid-sparing therapy (rituximab).
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pmc-6343940-1
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A 55-year-old man with a history of hypertension, hyperlipidemia, coronary artery disease, status post CABG six months ago with SVGs to the obtuse marginal and right coronary arteries (RCAs), and left internal mammary artery graft to the left anterior descending coronary artery, presented to the emergency department with two episodes of cough with hemoptysis associated with some chest discomfort. Both episodes resolved spontaneously. The patient was hemodynamically stable. Laboratory evaluation included hemoglobin of 12.2 g/dL and normal troponin. Electrocardiogram did not show any changes suggestive of cardiac ischemia. A computed tomography (CT) scan of the chest with contrast to rule out pulmonary embolism showed pseudoaneurysm in SVG graft to RCA, 2 cm from its origin, measuring 1.2 cm in size with adjacent fluid possibly representing hemorrhagic debris (Figure ). The patient was hospitalized for further management.
The decision was made to repair the pseudoaneurysm through percutaneous approach with polytetrafluoroethylene (PTFE)-covered Jostent GraftMaster after a multi-disciplinary meeting. Appropriate permission was obtained for GraftMaster use. The patient was brought to the catheterization laboratory and left femoral access was obtained using modified Seldinger technique. FR4 7 Fr guiding catheter was advanced to aorta and positioned at the aortic anastomosis of the graft under fluoroscopic guidance. Angiography was performed in multiple locations using hand-injection of contrast. The SVG graft to RCA revealed pseudoaneurysm measuring 2 cm in size and 70% stenosis in the proximal third of the graft (Figure , Video ). A BMW 0.014” 190CM J-Tip wire was used to cross the lesion. Balloon angioplasty was performed using NC Emerge 4.0 mm × 15 mm balloon with single inflation and a maximum inflation pressure of 15 atm (Figure , Video ). Intracoronary stenting was performed with 4.0 mm × 26 mm GraftMaster and deployed at a maximum inflation pressure of 55 atm. Stent covered 99% of the lesion in the SVG and there was 0% residual stenosis (Figure , Video ). There was thrombolysis in myocardial infarction (TIMI) three flow before and after the procedure. There were no procedural complications, and the patient was continued on dual antiplatelet therapy. One day postoperatively, the patient was discharged home in stable condition, with a follow-up angiogram scheduled in six months. The patient continued to do well without any new complaints.
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pmc-6343969-1
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We describe the case of a 62 years old woman underwent breast cancer surgery and axillar dissection in 2010 due to a ductal breast carcinoma (pT2N1M0). Patients received postoperative chemotherapy (Taxotere-Epirrubicine) and radiotherapy (50Gy) Intravenous bisphosphonates (Zometa ® 4mg monthly 4 per month) were also used to prevent bone metastasis, following this line.
A bone scintigraphy was performed two years after surgery as a routine control. Interestingly, hypermetabolic focus on right shoulder and left mandible were observed by this test. The increase in contrast uptake on the shoulder area was attributed to a chronic arthralgia. On the other hand, the evaluation of the focus involving left mandible was more difficult (Figs. ,). Intraoral examination did not show any significant finding, as well as the orthopantomography. However, an ulceration of the oral mucosa with clinical suppuration and bone exposition was observed three months later. A CT-scan showed radiological findings of BRONJ such as osteosclerosis and bone sequestration (Fig. ). A biopsy finally confirmed the diagnosis of BRONJ. Patients was treated with surgical curettage of the area, soft tissue remodelling and medical treatment (oral amoxicillin/clavulanic acid 875/125 mg, 3 times a day, 15 days, associated with chlorhexidine 0.12% mouthwash 2-3 times a day).
Clinical evolution was favourable and patients and no recurrences were observed during follow up.
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pmc-6343978-1
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The patient, a 14-year-old black male was referred for diagnosis of a painless lesion located in the anterior mandibular region. His family could not determine the duration of the lesion. The patient had good general health and absence of extraoral changes. Intraoral examination revealed a painless swelling in the mandibular incisor region, which was covered by intact mucosa with normal color (Fig. ).
Radiographically, the lesion appeared as an unilocular, radiolucent image, with well-defined borders and sclerotic margins. The lesion also caused divergence of the roots of the mandibular left lateral incisor and canine, which was non-erupted (Fig. ). OKC and central giant cell lesion were the main diagnostic hypotheses.
Under local anesthesia, an excision was performed, due to the notable plane of cleavage when the whole lesion was detached from mandibular bone by means of vigorous curettage. During the surgical procedure, a white-colored material, similar to keratin, was noted, strongly suggestive of OKC. The left mandibular canine was also removed. Histopathological examination revealed a cystic lesion, lined with parakeratinised, stratified, squamous epithelium. The parakeratin appeared corrugated and the basal cell layer showed a palisade arrangement. The fibrous capsule did not present any inflammatory reaction. Additionally, a sparse, brownish, intracytoplasmic pigmentation was observed in the epithelial cells, mainly in the basal layer (Fig. ). The histopathological diagnosis was OKC. However, the intracytoplasmic pigmentation was further investigated.
The intracytoplasmic pigment was positive for Fontana-Masson staining. Immunohistochemistry reactions showed dendritic cells positive for S-100 protein (polyclonal, dilution 1:10,000), HMB45 (clone HMB45, dilution 1:200), and Melan A (clone A103, dilution 1:800), all localized in the basal cell layer. These findings confirmed the presence of melanocytes and melanin in the cystic epithelial lining. Thus, the final diagnosis was pigmented OKC.
Currently, the patient is under periodic follow up, and no clinical and imaging signs of recurrence have been observed 24 months after the surgical procedure, with complete bone repair.
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pmc-6343985-1
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A five-year-old girl was referred to the School of Odonto-Stomatology, Hanoi Medical University due to the appearance of a swelling on the left upper jaw since one and a half months ago. According to the patient’s mother, this abnormal enlargement was slightly growing in size and no evidence of neither pain nor pus discharge. The patient was taken to a dentist and prescribed with antibiotics but this lesion did not disappear. Based on the patient’s dental history, she had undergone a root canal therapy on the left maxillary primary first molar at a dental clinic one year before. The extra-oral observation denoted a diffused swelling on the left zygomatic region, which obliterated the left nasolabial fold and was larger than 3 cm in diameter. The lesion was firm in consistency and no tenderness to palpation.
The intra-oral examination indicated a hard bony mass on the labial surface spreading from the left maxillary primary canine to the second molar. T64 was restored with a large glass-ionomer cement filling and had slight mobility. The mucosa covering the swelling was pink in color and soft in consistency without any purulent drainage.
The panoramic radiograph illustrated a well-defined uniocular and oval-shaped radiolucency in the left maxillary region with a regular hyperostotic margin which extended from the distal surface of the root of T63 to the distal surface of T65, and surrounding totally the root of T64 without absorption of the root of T63 and T65 (Fig. A). Consequently, these features suggested a cystic lesion. The abnormal radiolucency also displaced upwards the bud of the left maxillary first premolar. After thorough analysis, we found both an absorption of the root of T64 and a radiolucent material in two root canals of this tooth. It was suspicious that T64 had been treated with gutta-percha filling.
Based on both of the patient’s symptoms and findings, we made a provisional diagnosis of RC correlated to the T64 which had been treated endodontically, and then indicated a cystic marsupialization treatment. The patient’s parents were explained thoroughly about their daughter’s condition and the pros and cons of the operation. Prior to the surgery, a written parental permission was made, routine blood tests were also performed and all indices were within normal limits. The operation was carried out under general anesthesia at the Hanoi Medical University Hospital. The antibiotics were preoperatively taken for prevention of infection.
The treatment plan included removal of the upper left primary first molar tooth and marsupialization of the RC in order to conserve the upper left first premolar tooth. Following T64 removal, the extraction socket was widened to make a cyst window and a biopsy sample was incised from the wall of the lesion for histological examination (Fig. A). The underlying cyst lining was thick, soft and looked like granulation tissue. It was clearly seen that there was gutta-percha sealer in both root canals of T64 (Fig. B). 500mg of Augmentin (Amoxicillin Clavulanate) and a Chlorhexidine gluconate 0.12% mouthwash were daily prescribed for one day before and ten days after surgery in order to avoid the postoperative contagiousness.
The tissue sample was conserved in 10% formalin solution then stained with Hematoxyline and Eosin for digital histomorphometry analysis (Fig. A-C). The histological examination of the lesion illustrated a cystic lumen, which was lined with completely non-keratinized stratified squamous epithelium with some degenerated or ulcerated regions. The epithelium contained a multiple-cell layer thickness and the outer wall comprised proliferative fibro-vascular connective tissues with a significant infiltration of inflammatory cells consisting of the lymphocytes, plasma cells, macrophages and neutrophils (also called polymorphonuclear neutrophilic leukocytes). The needle-like cholesterol crystals were seen in the connective tissue wall. In summary, the histologic findings confirmed the final diagnosis as an RC in relation to an infectious primary tooth.
Subsequently, a customized acrylic obturator was prepared and put into the cyst window 1 week after the procedure (Fig. C-E). Patient and her parents were carefully notified how to wear and remove the obturator, and how to rinse the cyst cavity with saline daily. The patient had monthly appointments for follow-up and adjusting the denture corresponding to the size of the cyst opening. The resolution of the cystic lesion, as well as the eruption of the permanent successor, were assessed with a periapical radiograph in each recall. At 3 months after the procedure, periapical radiograph showed a reduction in the size of the radiolucent lesion and a spontaneous eruption of T24 (Fig. B). Moreover, the crown of T24 could be seen through the cyst window (Fig. C).
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pmc-6343996-1
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In July 2008, a 65-year-old caucasian man completely edentulous came to the Oral Surgery and Implantology Department of the University of Barcelona (Barcelona, Spain) to evaluate his possibilities of oral rehabilitation.
Patient pathological background included: smoke habit of 60 cigarettes per day for 30 years until 1999 and alcohol consumption of 150 gr/day (cessation in 1999), Oral Squamous Cell Carcinoma (OSCC) in the right side of the floor of the mouth (pTis pN0 M0) diagnosed in July 1999 and surgically treated with tumor exeresis, functional bilateral supramilohid lymphadenectomy, reconstruction with microvascular free radial flap and tracheostomy. In the postoperative period a cervical hematoma appeared, which had to be surgically debrided. Furthermore, the patient received internal radiation with brachytherapy (total dose 50 Gy).
In 2000, a second OSCC arising in the soft palate and latero-cervical area (pT1 pN2b M0) was detected and treated by local excision with direct repair and a radical lymphadenectomy. No complications appeared during the postoperative period. A second radiotherapy with external radiation with a total dose in the tumor site of 60 Gy (2 Gy per fraction), 50 Gy (2 Gy per fraction) in the supraclavicular field and 60 Gy in the spinal lymphatic right chains and 50 Gy in the left (2 Gy per fraction) were applied between the years of 2000 and 2001. Mucositis and epithelitis GII grade appeared as toxicity consequences of the radiation.
In 2017, a third OSCC was located on the left buccal mucosa (T2 N0 M0) which was treated with tumor resection and without radiotherapy. The defect was reconstructed with a radial microsurgical graft. A vein thrombosis of the pedicle occurred in the postoperative period, this complication was solved with a new vein anastomosis.
The patient is currently being treated for prostate cancer with external radiotherapy.
In addition, the patient was diagnosed with hypothyroidism caused by the radiation therapy, hypercholesterolemia, hiatal hernia, phlebitis, anxiety disorder, cervical stenosis and angor pectoris. These conditions were controlled with: Simvastatin, Omeprazole, Pentoxifylline, Acetylsalicylic acid and Levothyroxine.
On the first visit consultation in our Department, a panoramic radiography and a computed tomography were taken for treatment planning due to the impossibility of wearing a full prosthesis. Residual alveolar ridge was classified as class III according to the Cadwood and Howell classification. In accordance with the patient, it was decided to insert 4 dental implants in each jaw to support two overdentures. In July 2008, the implant surgery was done (Nobel Speedy groovy® 4.0 mm × 13.0 mm) under local anesthesia with Articaine 4%, 1:100.000 (Ultracain; Normon, Madrid, Spain) and antibiotic prophylaxis (Amoxicillin [GlaxoSmithKline, Madrid, Spain] 2 grams/ 1 hour before) (Figure -A). One of the lower dental implants was placed with angulation in order to avoid the emergence of the mental nerve and improve the distance between implants. One week after the surgery, a wound dehiscence with bone exposure occurred on the right side of the mandible. This complication was treated with amoxicillin/clavulanate 875/125 mg [Augmentine 875/125 mg; GlaxoSmithKline, Madrid, Spain] every 8 hours for 10 days, as well as a strict oral hygiene program with 0.12% chlorhexidine digluconate [Clorhexidina Lacer; Lacer, Barcelona, Spain] every 12 hours for 15 days. Two months later an osteoradionecrosis was diagnosed because no total healing was obtained, a surgery with local anesthetic to curette the area was performed but after all the wound kept failing to heal normally.
In March 2009, another curettage with primary closure and a bone biopsy were done under local anesthesia and intravenous sedation. The histopathological study revealed marrow fibrosis without confirmation of bone remodeling or evidence of malignancy. From then on, the wound started to heal normally in the post-operatory period.
In August 2009, the patient was hospitalized due to severe pain and mobility on the right side of the mandible without history of trauma. The clinical and radiological examination revealed a cutaneous fistula in the lower jaw and a mandibular fracture on the right side with 12 mm of distance between bone fragments (Figs. B, A, A). The fistula was debrided twice to remove the purulent content and a soft diet was prescribed along with amoxicillin/clavulanate 875/125 mg (Augmentine ® 875/125 mg; GlaxoSmithKline, Madrid, Spain) every 8 hours for 15 days. The sensibility of the lips and chin were lost as a result of the fracture.
Although several options were suggested in order to repair the fracture (pseudoarthrosis), the patient decided not to be treated. Even though an antibiotic therapy was used immediately after the diagnosis of the fracture, it has not been required since.
A second-stage implant surgery was made after 6 months post-mandibular fracture with Laser Er,Cr:YSGG 1.5 W, 30 pps (Waterlase MD®;Biolase Technology, California, EE.UU.) without complications. Although the implant near to the line of fracture was osseointegrated, it was decided not to use it and an inferior bar supported by the remaining implants and Locator abutments (Ancladen SL, Barcelona, Spain) on the superior ones were used for retaining two overdentures (Fig. C). Up to 2017, during more than 8 years of follow-up, the prothesis were functionally useful without significant problems, but since the patient received the radial microsurgical graft his mouth-opening was very limited, and that prevented wearing the prosthesis. Although after 1 year of follow-up, the dentals implants in the upper jaw presented periimplantitis-disease while lower implants exhibited mucositis. Intraoral tissues and fistula on the fracture area were entirely healed at that time period (Figs. B, C, B).
The patient was included in a maintenance program to be controlled radiographically and clinically every 6 months for periodontal maintenance treatment to manage the peri-implant disease but currently did not follow a good fulfillment (Fig. D,E,F,G,H).
shows the timeline which summarizes the diagnostics and treatments carried out in our patient.
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pmc-6344064-1
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A 41-year-old female with a past medical history significant for asthma presented to an outside hospital emergency department in pulseless electrical activity (PEA) arrest. Her husband found her earlier in the morning on the couch with nebulizer treatment in hand. She quickly progressed to being unresponsive and cardiopulmonary resuscitation (CPR) was started. When emergency medical service (EMS) arrived (approximately 10 minutes from the onset of CPR), she was in PEA arrest. She was given two doses of epinephrine. Return of spontaneous circulation (ROSC) was achieved. She was taken to an outside hospital (OSH).
She was hypotensive (50/41 mmHg) requiring vasopressor infusion and had diffuse expiratory wheezing requiring continuous nebulizer treatment. Her initial arterial blood gas (ABG) was significant for a pH of < 6.8, carbon dioxide (CO2) of 130 mmHg, and oxygen (O2) of 331 mmHg. After continuous albuterol treatments and adjustments to the ventilator, a repeat ABG had the following values: pH of 6.81, PaCO2 of 138 mmHg, and PaO2 of 262 mmHg. On examination, she remained comatose with fixed and dilated pupils (6 mm, nonreactive) with a Glasgow Coma Scale (GCS) score of 3T. She was transferred to our facility for consideration of extracorporeal membrane oxygenation (ECMO).
On arrival, she was sedated and paralyzed to optimize ventilation/oxygenation prior to ECMO. Computed tomography (CT) of the head showed diffuse cerebral edema concerning for severe anoxic brain injury (Figure ). She was evaluated by the neurologic intensive care unit (NICU) team. She was given mannitol (100 g) and 23.4% (30 cc) without a change in the neurological examination. Veno-venous (V-V) ECMO was started (flow 4 LPM, speed 3215 RPM, FiO2 of 100%, sweep 9 L/min, with ventilator FiO2 of 40%). Her ABG improved to pH of 7.29 and PaCO2 of 36. She was then transitioned from epinephrine to norepinephrine.
Over the next 12 hours, she developed polyuria (7.2 L) with increasing sodium (from 147 meq/L to 172 meq/L). She was given desmopressin and started on a D5W infusion. Her sodium improved to 149 meq/L over the next 12 hours. Her ABG now had a pH of 7.37, PaCO2 of 39 mmHg, and PaO2 of 90 mmHg. Continuous electroencephalography (CEEG) showed background suppression. Despite the correction of severe acid-base disorder and electrolyte disorders, she remained in a comatose state with 6 mm, nonreactive pupils. She was noted to have absent oculocephalic, oculovestibular, cough, gag, and not triggering ventilator. There was no change in heart rate with noxious stimulation. Apnea test was attempted. She was pre-oxygenated. She was then disconnected from respirator with six liters of oxygen (O2) delivered to carina via red rubber suction tubing. The sweep on the ECMO was adjusted to 1 L/min. However, she quickly desaturated to 82% for >30 seconds. The test was aborted. She was reconnected to the ventilator and ECMO adjusted. A second attempt occurred after pre-oxygenation. The sweep this time was reduced to 4 L/min from 9 L/min. She again did not tolerate, and the test was aborted. She was reconnected to the respirator. ECMO was changed back to sweep of 9 L/min. Her temperature was 36.2°C at the time of testing. Transcranial Doppler (TCD) was then ordered.
The following morning while awaiting TCD to be performed, repeat neurological examination again found her to be in a coma with brainstem areflexia, except for performing apnea test. On nailbed pressure to her fourth finger, she had flexion of her third finger – similar to the finding of a Hoffman’s sign in an upper motor neuron injury (Figure ). This flexion occurred over one second. Her lower extremities remained areflexic and flaccid. She was noted to have reduced ECMO requirements. The decision was made to repeat the apnea test. Her ABG had a pH of 7.39, PaCO2 of 37 mmHg, and PaO2 of 138 mmHg. Again, she was prepared by pre-oxygenating and then disconnected from the respirator. A cannula was placed in the endotracheal tube (ETT) and delivered six liters of O2 to the carina. The sweep on the ECMO was systematically decreased over the five-minute period down to 500 cc/min. She tolerated this adjustment. Her O2 saturation remained 95–100% during repeat apnea test. After 37 minutes of testing, her ABG showed a pH of 7.2, PaCO2 of 60 mmHg, and PaO2 of 147 mmHg. She was declared brain dead. TCD was eventually completed and interpreted as systolic spikes (Figure ).
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pmc-6344065-1
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A 19-year-old Hispanic female presented with complaints of an eruption of the hands and forearms that had started one year prior. She reported mild associated pruritus, which increased with exposure to natural sunlight. She denied the involvement of the head, trunk, lower extremities, or genitalia. She took no daily medications and had no chronic medical problems. There was no evidence of conditions associated with syringomas on history, physical exam, or workup. The physical exam revealed numerous, light brown, ovoid papules on the dorsal hands and fingers and on the dorsal and ventral surfaces of the forearms, with some areas of confluence on the lateral dorsal hands (Figures -). Similar lesions were not observed elsewhere, including the groin, on the patient. A punch biopsy was obtained from the right forearm to establish the diagnosis and revealed a proliferation of small eccrine ductal structures lined by cuboidal cells within a fibrous stroma with an unremarkable epidermis, consistent with a diagnosis of syringoma. No cytologic atypia or significant infiltration of the deeper dermis by these ductal structures was appreciated (Figure ). A limited laboratory analysis was conducted and revealed a normal complete blood count, as well as a glycated hemoglobin test of 5.2% (normal < 5.7%).
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pmc-6344067-1
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An 80-year-old female was referred with a six-month history of medically refractory seizures and evidence of a right tegmen dehiscence and encephalocele. Her medical history was significant for chronic otitis media, with a history of a right-sided tympanomastoidectomy 20 years prior and ongoing follow-up for chronic eustachian tube dysfunction. Her symptoms included daily episodic paresthesias with phantosmia and a right-sided severe to profound mixed hearing loss. She denied CSF rhinorrhea, otorrhea, or other symptoms. Seizure workup included video EEG confirming right temporal lobe epilepsy as a cause of her paresthesias and phantosmia. Computed tomography (CT) and magnetic resonance imaging (MRI) demonstrated a right temporal lobe encephalocele. In addition to the TLE, MRI demonstrated increased edema and flair signal within the right mesial temporal lobe (Figures -).
She was discussed at a multidisciplinary skull base conference with the decision to undergo a combined mastoid-middle cranial fossa encephalocele repair. Informed consent was obtained prior to proceeding with surgery. Intraoperatively, a 1 x 1 cm tegmen defect with the herniation of glial tissue into the mastoid was repaired with partial resection and an Onlay dural substitute (Lyoplant, Aesculap, Tuttlingen, Germany). Her postoperative course was uncomplicated. Immediately, she noted an improvement in her seizure frequency and duration; however, her seizures did not fully resolve. A repeat 3T epilepsy protocol MRI demonstrated further hippocampal atrophy, increased flair within the right hippocampus, and the loss of gray-white differentiation in the anterior temporal lobe, diagnosing mesial temporal lobe sclerosis (Figures -). On retrospective neuroradiology review, the progression of decreased hippocampal volume was noted on MRIs leading up to surgery. She is currently undergoing workup for resection of the temporal epileptically focal lesion.
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pmc-6344156-1
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A 49-year-old female with a medical history significant for left-sided breast cancer underwent lumpectomy in 2017, for which the pathology was ductal carcinoma, HER2 positive. Then, she was treated with trastuzumab (8 mg/kg loading dose, then 6 mg/kg every 3 weeks intravenously) and oral capecitabine (1000 mg/m2 twice a day on days 1–14 every 3 weeks) for 7 cycles from September 2017 to February 2018. During chemotherapy, a course of radiotherapy was performed, delivering 50 Gy in 25 fractions to left chest wall and supraclavicular fossa (2 Gy every fraction and 5 fractions per week) from November 17 to December 22, 2017. Two months after radiation treatment, the patient complained of productive cough and progressive breathlessness, occasional wheezing, and left pectoralgia. Her chest X-ray showed infiltrates in the left apical segment and was prescribed ipratropium inhalers and antibiotics. With no improvement in her symptoms, the computed tomography (CT) scan of the chest (Fig. A) revealed a left upper lobe consolidation. Half a month later, the range and density of the consolidation increased, and the left pleural effusion was newly seen (Fig. B).
On review of systems, the patient reported suffering from nocturnal sweats but no fevers, no change in appetite, and no weight loss. She had a full-time job as an office worker and denied any significant environmental exposure history. She is a never smoker with a 6-year past medical history of well-controlled asthma.
Laboratory studies revealed 56% eosinophils (6.16 × 109/L) in peripheral blood, IgE 154.0 kU/L. Her blood biochemical profiles as well as serum immunoglobulins were all unremarkable. Infectious disease etiologies workup including serologies for aspergillus, filarial worms, lungworms, cysticercosis, and trichinella spiralis was negative. Stool examinations for ova and parasites were negative. Vasculitides and connective tissue diseases workup including antinuclear antibody, anti-double-stranded DNA, rheumatoid factor, and anti-neutrophil cytoplasmic antibody was negative. Bone marrow biopsy and FIP1L1-PDGFR alpha test excluded hematological diseases such as myeloproliferative neoplasms and hypereosinophilic syndrome. Pulmonary function tests revealed forced expiratory volume in the first second of 2.88 L (97.0% predicted), FEV1/FVC ratio of 73.73%, total lung capacity of 4.68 L (96.9% predicted), and diffusing capacity for carbon monoxide of 6.41 mmol/min/kPa (79.8% predicted). She underwent bronchoscopy, and bronchoalveolar lavage (BAL) fluid demonstrated eosinophil count of 85%. A transbronchial biopsy performed from the left upper lobe was suggestive of eosinophilic pneumonia with marked increased eosinophils without evidence of vasculitis, malignancy, or infection (Fig. ). The diagnosis of chronic eosinophilic pneumonia (CEP) was made based on peripheral eosinophilia, pulmonary consolidation with pleural effusion, high percentage of BAL eosinophils, the finding of eosinophilic pneumonia on transbronchial biopsy, and the absence of other causes of eosinophilia. Our patient was given oral prednisone at 0.5 mg/kg/day and all antibiotics were discontinued. She had an excellent rapid clinical improvement. A CT scan of the chest (Fig. C) obtained 2 weeks after steroid treatment showed diminishment of the consolidations. Her prednisone was tapered slowly over 1 month and consequently stopped. At 6-month follow-up, the patient had no complaints of discomfort with no relapse of pulmonary lesions (Fig. D).
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pmc-6344168-1
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A 65-year-old female came to our hospital on May 6, 2016 due to “cough and pain and lack of movement in the left leg”. The diagnosis was adenocarcinoma of the left upper lobe of the lung with involvement of both lungs, mediastinal lymph nodes, liver, and multiple bone metastases (cT4N2M1b, stage IV).Since the biopsy specimens were too small, a liquid biopsy was carried out and no gene mutation was found. According to the National Comprehensive Cancer Network (NCCN) Treatment Summary for NSCLC, anti-angiogenesis drugs combined with chemotherapy was recommended for advanced wild gene-type NSCLC when the PS score was 0 to 2. The PS of this patient at the time of diagnosis was 2 to 3 points which precluded the use of chemotherapy and apatinib was recommended. Because of the potential of liquid biopsies to give false negative results and because the patient belonged to the high-risk EGFR mutation group, she was also administered erlotinib with monthly infusions of zoledronic acid to prevent bone destruction and accelerate bone regeneration.
In further evaluating the patient, we found no active bleeding, no enterobrosis, no ileus, no cardiac insufficiency, and no anaphylaxis. Her hepatorenal function was normal and her hypertension was controlled. After signing a consent form on May 26, 2016, 250 mg po qd of apatinib (supported by Heng Rui Pharmaceutical Co., Ltd.) was given with a 28-day cycle. On July 2, 2017, 150 mg po qd erlotinib was administered. Due to severe diarrhea, the erlotinib dose was reduced to 75 mg qd 1 month later. After treatment, the pain in the left leg was significantly improved and she was able to walk normally.
The tumor markers, carcinoembryonic antigen (CEA), and OC125 antigen (CA125) were rechecked and levels had dropped from 396.4 ng/mL to 67.90 ng/mL and from 20.63 U/mL to 8.28 U/mL respectively (Fig. ). Her computed tomography (CT) showed that the displacement of the left lung was significantly smaller than before (Figs. and ) and that the left iliac bone mass was reduced (Fig. ). Subsequently, the partial response (PR) efficacy was rechecked regularly and stable disease (SD) condition evaluated. The patient was hospitalized again for fainting and urinary incontinence on September 7, 2017. We found that the tumor marker levels had increased (Fig. ), and an intracranial magnetic resonance imaging (MRI) showed multiple abnormally strengthened intracranial nodular shadows, suggesting metastasis (Figs. and ). In view of the apparent tumor progression, it was proposed to repeat the genetic test again. We still had to use a liquid biopsy because of the difficulty of sampling and the results again showed no gene mutation. Local radiotherapy was recommended but the patients and her family refused, opting instead for continuing with the self-treatment of apatinib combined with erlotinib.
In December 2017, the patient came to our hospital again for fainting. An MRI showed that the number of intracranial nodules was slightly increased and they were larger than before. On December 7, 2017, the patient agreed to radiotherapy and the Gamma Knife was adopted for treatment. Her current condition is stable. The entire therapy regimen is shown in Figure .
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pmc-6344173-1
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A 43-year-old right-handed woman presented with a progressive flexion of the small finger of her left hand. Six weeks prior, she had received a puncture wound to the skin of the volar aspect of the metacarpophalangeal joint of her left small finger from a piece of glass while she was working. She did not receive any treatment for that injury, and she had not had any trouble in daily life or work. Two weeks later; however, she noticed a painful triggering of her small finger with a progressive lack of extension.
On physical examination, she exhibited direct tenderness in the volar aspect of the metacarpophalangeal joint of the finger. Triggering with small finger flexion was observed, and she could not extend her finger because of the pain. The diagnosis of triggering caused by a neglected partial rupture of the flexor tendon was suspected.
The patient underwent surgical exploration 6 weeks after the injury. A zigzag incision was used at the level of the A1 pulley, and there was a small mass-like lesion at the proximal edge of the A1 pulley. The sheath was opened and the synovial tissue was removed, and it was obvious that there was a proximal stump of the ruptured ulnar slip of the FDS tendon (Fig. A and B). In addition, the FDP tendon was partially injured, and we found two small retained fragments of glass.
The A1 pulley was excised and the injured tendon was sutured at the original position after trimming (Fig. C). At 7-month follow-up, the patient was completely asymptomatic and had full ROM in her left small finger (Fig. D and E).
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pmc-6344173-2
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A 28-year-old right-handed man visited the clinic because of painful triggering in the PIP joint of his left long finger. Four weeks prior he had experienced laceration wound to the skin of the volar flexion crease of the PIP joint of his left long finger by a hacksaw. His wound had been sutured by an orthopedic surgeon without exploration. One week later, the stitches were removed, and then he noticed intermittent catching and triggering, associated with a dull pain, in the injured finger. The symptoms progressively worsened.
On physical examination, there was a 5 mm healed wound on the radial side of the flexion crease in the PIP joint of the left long finger, with localized tenderness and swelling. Although passive motion was full, active flexion was limited to 0° to 80° at the PIP joint, and 0° to 30° at the distal interphalangeal joint (Fig. A).
The patient underwent surgical exploration 4 weeks after the injury. A zigzag incision was used at the level of the A2 and A3 pulleys (Fig. B). A partial laceration was found of the radial aspect of the FDP tendon, that formed a tag that impinged on the C2 and A3 pulleys, (Fig. C). The FDS tendon was not involved.
The C2 and A3 pulleys were excised and the tag was then sutured at the original position after trimming (Fig. D). At 16-month follow-up, the patient had regained full ROM, and there has been no recurrence of triggering (Fig. E and F).
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pmc-6344186-1
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The patient's mother was 36 years old, with a total of 5 pregnancies and 1 live birth. Eleven years ago, she gave birth to a healthy boy via cesarean section. She previously had 2 artificial abortions and 1 spontaneous abortion, with no family history of hereditary diseases. Ultrasound test result was normal at gestational week 6. At gestational week 12, nuchal translucency of 1.3 mm was observed, with no positive findings. Non-invasive DNA analysis showed low trisomy 21 risk, low trisomy 18 risk, and low trisomy 13 risk. At gestational week 24+5, ultrasound examination showed abnormal development of the long bones of the limbs (the length of the long bones was shorter than 1% controlled to the same gestational week of normal fetus), thick metaphysis in the right lower limb, irregular vertebral arrangement, and a narrow and small thorax (Fig. A–D). The patient's parents decided to terminate the pregnancy at 27 weeks of gestation considering this as a lethal skeletal dysplasia.
After the termination of pregnancy at week 27, the gross examination of the fetus showed a flat face and nose bridge, short limbs, asymmetric short lower limbs, and bilateral clubfeet with bilateral ankle joint contracture (Fig. A, B). The autopsy report indicated that the left humerus bone was 2.5 cm long; the right humerus bone, 2.0 cm; the left femur, 3.0 cm; and the right femur, 2.2 cm; all were significantly shorter than 4SD measured at the same gestational age. In addition, the shapes of T3 to L3 were abnormal, the vertebral bodies appeared fused, the thorax was small with a maximum circumference of 16 cm, and the abdominal circumference was 26 cm. The autopsy also found subcutaneous edema in the head and face and thick metaphysis in the lower limbs. X-ray imaging showed short femur and humerus bones, a narrow and small thorax, thick metaphysis with a thick “splashed paint”’ pattern, and asymmetric short lower limbs (Fig. A–C).
The karyotype of the fetus was 46, XX. No unusual single-nucleotide polymorphisms was detected. Whole exome analysis showed that the fetus was heterozygous for the EBP mutation (NM_006579.2; C.440G>A p.Arg147His), which must have occurred de novo because the parents were non-carriers (Fig. ). Thus, CDPX2 was confirmed.
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pmc-6344642-1
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A 45-year-old male, with a known history of psoriatic arthritis, was referred to our hospital by his physician, with fever, nausea, headache, asthenia, and visual disturbances. He had competed in a triathlon four days earlier and had been kayaking, cycling, and running in the forest for weeks before.
General clinical and neurological examination were normal. Blood analyses on admission showed increased C-reactive protein (247.9 mg/l) and mild thrombocytopenia (74,000 platelets per microliter), then high creatinine (2.02 mg/dl), high urea (66 mg/dl), and eosinophilia (1600/μl) four days later. Due to increasing headaches, MRI of the brain was performed (Figure ) to rule out cerebral vein thrombosis. Axial diffusion-weighted image showed a high signal intensity in the splenium of corpus callosum at high b-value (b = 1000 s/mm2) with low apparent diffusion coefficient values. Axial T2-weighted and fluid-attenuated inversion-recovery images showed a slight hyperintense signal on the same location. There was no abnormal contrast enhancement, nor cerebral venous thrombosis. Anti-Puumala virus IgM antibodies were detected using enzyme immunoassay, confirming diagnosis of acute PUUV infection []. Patient was discharged eight days after admission. The MRI findings had resolved completely in follow-up study three weeks later (not shown). Diagnosis of cytotoxic lesion of the corpus callosum (CLOCC) was made.
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pmc-6344988-1
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A 72-year-old woman presented with poor appetite and was initially drowsy at home; the symptoms progressed to loss of consciousness accompanied by mild incontinence. Therefore, the patient was admitted to the emergency department with an initial glucose level of 44 mg/dL, and no nausea, vomiting, fever, or cold sweating was reported. After intravenous glucose supplementation, she partially recovered consciousness (Glasgow Coma Scale [GCS]: E2V2M3), and her serum glucose level increased to 242 mg/dL. Physical examination revealed the absence of focal neurological signs, facial palsy, and tongue or eye deviations; however, mildly increased deep tendon reflexes were noted at the bilateral lower limbs. The images obtained 24 h after symptoms onset revealed symmetrical hyperintensities on DWI (b-value: 1000) associated with hypointensities on ADC map along the corticospinal tract, from the levels of the cerebral peduncle and the posterior limbs of the internal capsule to the level of the corona radiata, but there was no abnormal signal on T2-fluid attenuated inversion recovery (FLAIR) images (Fig. ), which may mimic the imaging findings of acute ischemic infarction or amyotrophic lateral sclerosis. In-hospital electroencephalography indicated only generalized cortical dysfunction without evidence of focal seizure. The patient received sliding-scale insulin therapy and rehabilitation and recovered consciousness. A comprehensive neurological examination performed 1 month since the initial event of loss of consciousness revealed total recovery without motor function deficits. Moreover, repeat DWI (b-value: 1000) and ADC map showed the complete disappearance of the lesions (Fig. , Additional file ).
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pmc-6345031-1
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A 51-year-old woman was referred because of a 20-year history of intermittent headaches and dizziness that had been accompanied by blurred vision in both eyes for 10 years. The patient did not have a family history of glaucoma. An ophthalmic examination of the patient revealed that although the best corrected visual acuity (BCVA) values were 0.8 and 0.5 in her eyes and that the intraocular pressure (IOP) was normal in both eyes, the superior visual field was narrowed in both eyes, likely due to drooping upper eyelids (Fig. -a). A neurological examination revealed that the muscle strength and muscle tension of the limbs were normal, and Babinski’s sign, Kernig’s sign, and Brudzinski’s sign were negative. A CT scan (Fig. -b) and an MRI (Fig. -c) of the sella revealed a mass in the sellar-suparsellar-parasellar region; this mass was likely to be a meningioma, which compressed the left optic nerve. We then performed a craniotomy using the anterior cranial base approach. During the operation, the frontal sinus apex was opened, and the tumor was found to be surrounding the left optic nerve, which is also close to the internal carotid artery and the oculomotor nerve. We removed as much of the tumor as possible, sealed the top of the frontal sinus with bone wax, and sutured the epidural.
The operation was successful. A postoperative CT showed that the lesion in the saddle area had been removed, and a small amount of blood and effusion accumulated under the dural membrane of the left frontotemporal region. On the third day after operation, the patient complained of swelling and pain in her left eye, accompanied by difficulty opening the left eyelid. An examination revealed proptosis with ptosis in the left eye, eyelid swelling, and increases in intraorbital pressure and IOP (Fig. -a). The left eye movement, especially upward movement, was limited, and the BCVA was limited to 0.1, yet the RAPD was negative. A B-scan (Fig. -b) and an MRI (Fig. -c) revealed a regularly shaped, hypodense, oval mass in the upper nasal side of the orbit; the left optic nerve, superior rectus muscle and left eyeball were significantly compressed, likely due to a foreign body, such as intraoperatively placed bone wax. Given the patient’s recent craniotomy and given the risks associated with orbital surgery, she refused to undergo a surgery to remove the bone wax. Thus, to reduce IOP, the patient was administered mannitol intravenously (125 ml q8h) daily, accompanied by Timolol topically. This treatment led to decreased IOP and intraorbital pressure, and the parameters remained stable after treatment. Three weeks after the craniotomy, the BCVA improved to 0.2, and the patient was discharged.
At 1 month of follow-up, although the BCVA of the patient’s left eye improved to 0.6 and ptosis and restricted eye movements improved significantly, there was still proptosis with respect to the orbit. Ophthalmic examination revealed that the thicknesses of the retinal nerve fiber layer in the superior and temporal left eye were decreased with a narrowed inferior visual field. A B-scan showed that the retrobulbar mass was still not absorbed (Fig. ). Removal of the bone wax seemed necessary for improving the patient’s ophthalmic and neurologic symptoms. However, the patient refused to undergo a surgery due to the associated risks. At a 6 month follow-up, the retrobulbar mass did not change significantly (Fig. ), the ophthalmic symptoms of the patient remained stable, and the visual field of the left eye did not appear to improve.
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pmc-6345050-1
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A 52-year-old Caucasian woman with SS secondary to AIH/primary sclerosing cholangitis overlap was admitted to our emergency department owing to bilateral keratolysis and corneal perforation in the left eye. The patient had had filiform keratitis and recurrent erosions for the previous 3 years. The dramatic worsening of her dry eye disease followed corneal exposure in an artificially induced coma during her stay on an intensive care unit owing to sigmoid colon perforation and sepsis. At the time of admission, the patient’s right eye had deep corneal melting, and best corrected visual acuity (BCVA) was 0.2 decimal. In the left eye, there was a corneal perforation, and BCVA was hand motions. Table documents the surgical therapies performed in the right and left eyes owing to fulminant relapses of keratolysis and corneal perforations in the subsequent 10 months. Postoperative topical therapy consisted of dexamethasone disodium phosphate 1 mg/ml six times per day, cyclosporine 0.1% twice per day, ofloxacin eye drops four times per day, and hourly application of artificial tears and human albumin. Additionally, mycophenolate mofetil (2 g/day) was administered systemically. An enhancement of the systemic immunosuppression by corticosteroids or azathioprine was contraindicated because the patient had a history of sepsis []. Intravitreal injection of the FAc implant was performed off-label in her left eye 2 weeks after the second penetrating keratoplasty (PKP) because of new signs of corneal melting (Fig. a) and was followed by the third PKP and amniotic membrane transplant (AMT) 2 weeks later.
In the 6 months of follow-up after the third PKP, no more surgical interventions were needed in the left eye that had been treated with the FAc implant. In this eye, there was a closed epithelium, BCVA was 0.16, intraocular pressure was normal without any intraocular pressure-lowering medication (Fig. b). However, during this period, two further PKPs, one vitrectomy, five AMTs, and three tarsorrhaphies were performed in the right eye owing to recurrent keratolysis and perforations (Table ).
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pmc-6345076-1
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A 68-year-old woman referred to the haematology outpatients department of our centre (Virgen del Puerto Hospital, Plasencia) in August 2015 with progressive anaemia detected 4 months earlier with asthenia, anorexia, profuse sweating and a weight loss of 6 kg. She was treated with dicumarinics for atrial fibrillation with no relevant history. On physical examination: performance status 1, skin pallor and a small axillary lymph node. The analytical and peripheral blood morphology data are shown in .
Imaging studies: Abdominal ultrasound: liver with a slight increase in overall size, with homogenous parenchyma of normal echogenicity and with no focal lesions. Homogeneous splenomegaly of 14 cm. Portal vein slightly enlarged. Chest X-ray: no changes. Computerised tomography (CT scan): absence of mediastinal adenopathies. Liver slightly increased in size with heterogeneous densitometry without demonstrating focal lesions. Spleen at the upper limit of normality. Small retroperitoneal adenopathies measuring 11 mm. Mammogram: normal.
Bone marrow aspiration: reactive and with no morphological evidence of tumour infiltration. Increased iron deposits, no sideroblasts.
Other studies: gastroscopy and colonoscopy: normal. Core-needle biopsy-aspiration of axillary ganglion cyst: could not be assessed.
The patient was periodically checked in the outpatients clinic and 2 months after the start of the investigation, and in view of the persistence of asthenia and anaemia, we decided to perform a splenectomy, removing a spleen measuring 12 × 11.5 × 6.5 cm and weighing 317 g. Five nodular formations were detected, the largest measuring 0.5 cm in white pulp, composed of germinal centre-type cells compatible with non-Hodgkin follicular lymphoma (NHFL). Therapeutic abstention was decided given the good tolerance to anaemia and the biological characteristics of the lymphoma.
At 6 months, the anaemia progressed and hepatomegaly was detected 6 cm below the costal margin. During magnetic nuclear resonance (MNR), we observed severe hepatomegaly suggestive of infiltration, with increased retroperitoneal adenopathies and compromise of the right renal excretory duct (). Positron emission tomography/computerised axial tomography (PET/CT scans) were performed as a staging evaluation: multiple hypermetabolic ganglion lesions in the retroperitoneum (SUVmax 7) in blocks, the largest measuring 2.4 × 3.0 cm, the hepatomegaly being better evaluated by other techniques such as the MNR. The liver biopsy shows slight portal fibrosis, marked dilation of the sinusoids that appear filled with blood material and with a tendency to form cystic cavities, compatible with peliosis hepatis ().
After confirming the progression of NHFL with clinical deterioration, treatment was started with immuno-chemotherapy type R-CHOP with good tolerance (six cycles of R-CHOP with maintenance rituximab every 2 months for 2 years). After the third cycle, an improvement in anaemia and of the inflammatory patterns, including those of iron metabolism, was observed. In the evaluation after six cycles of R-CHOP, no foci of uptake were detected on the PET/CT scan, no adenopathies were observed and the liver was normal in size and appearance.
Serial analytical determinations of hepcidin and cytokines (IL-6, VEGF) were performed from the moment of diagnosis, at the start of treatment, and before and at the end of each cycle of chemotherapy. shows the evolution of these parameters, demonstrating their decrease from the first cycle, highlighting the decrease in IL-6 in parallel to the decrease in hepcidin and VEGF. Likewise, the decrease in liver enzymes occurred in parallel with the reduction in hepatomegaly. Radiological data (CT and MRI scans) confirmed this regression of peliosis (). Currently, the patient has completed 2 years of maintenance therapy with a good general condition and complete remission of her disease.
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pmc-6345097-1
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A 64-year-old woman, with an uneventful past medical history, was diagnosed with stage IIIB (cT3pN3M0), epithelial-growth-factor-receptor (EGFR) wild type, KRAS mutated lung adenocarcinoma. The patient underwent 6 cycles of first line chemotherapy with cisplatin/gemcitabine obtaining a stable disease as best response. After 6 months, tumor progression was identified, as assessed by whole-body 18-Fluorodeoxyglucose positron emission tomography (FDG-PET) scan showing liver, bone, pleural and node metastasis. Nivolumab, 3 mg/kg every 2 weeks, was started. For dyspnea, the patient was also addressed to 3D conformational mediastinal radiotherapy for a total of 30 Gy in 12 fractions. During radiotherapy, nivolumab was temporally stopped for 1 month. While pre-nivolumab thyroid function was normal, 3 months after starting the therapy a low serum TSH level was found (TSH < 0.01 mU/L), associated with an FT4 level in the mid normal range (1.3 ng/dl: n.r. 0.89–1.76). Thyroid antibody (Ab) tests, including TSH-receptor Ab, were negative. At ultrasound examination, the estimated thyroid volume was in the upper normal range (18 ml) and gland parenchyma was normo-echoic. Due to these unclear findings, a laboratory assessment of other pituitary axes was requested, which showed low levels of serum cortisol (1.8 mcg/dl; n. r. 6.02–18.4) and ACTH (< 5.0 pg/ml; n. r. 7.2–63.3), and inappropriately low for a menopausal state serum levels of LH (0.46 mUI/ml; n. r. 1.7–8.6) and FSH (7.14 mUI/ml; n.r. 1.5–12.4). The serum levels of GH (5.3 ng/mL; n. r. < 10 ng/ml) and IGF-1 (162 ng/ml; 75th centile for sex and age) were in the normal range. Drug history indicated that the patient had not received corticosteroid therapy for the last 6 months. The patient was apparently symptomless regarding adrenocortical deficiency, and her blood pressure and serum electrolytes were normal. Adrenal stimulation with 1-24 ACTH (250 mcg i.v.) yielded a partial increase in serum cortisol levels (basal = 1.7 mcg/dl; 30 min = 8.1 mcg/dl; 60 min = 10.4 mcg/dl). These data suggested a condition of partial hypopituitarism with impairment of at least the adreno-cortical and gonadal axes, possibly due to a nivolumab-induced hypophysitis, which however was not evident at magnetic resonance imaging (MRI) of the pituitary gland. The patient was started on a replacement dose of cortisone acetate (12.5 mg at 8:00 a.m.; 5 mg at 2:00 p.m.; and 5 mg at 6:00 p.m.) while thyroid function was monitored with no specific treatment. One month later, serum TSH was slightly below the normal range (0.29 mU/L; n. r. = 0.35–4.2) in spite of a subnormal level of serum FT4 (0.74 ng/dl: n. r. 0.89–1.76). A 99 mTc-pertechnetate scintigraphy was performed showing normal and reduced areas of radionuclide uptake. The pituitary-thyroid axis was further checked with a TRH test. This provocative test was performed 5 months after the initiation of nivolumab and showed a blunted TSH response (basal = 0.7 mU/L, 20 min = 5.13 mU/L, 60 min = 3.44 mU/L). Thyroid replacement was not started and 1 month later serum TSH (1.0 mU/L n. r. = 0.35–4.2) and FT4 (0.97 ng/dl; n. r. = 0.89–1.76) were both in the normal range. Nine months later, the patient was still receiving glucocorticoids replacement therapy, while thyroid function remained normal with no specific treatment. The thyroid hormone profile of the patient is shown in Figure . Nivolumab therapy was continued since she achieved a response, which was partial for bone, pleural and node metastasis and complete for liver ones. No other irAEs occurred during treatment.
Taken together the above clinical, laboratory, and instrumental data indicate that the patient had both a painless thyroiditis and an autoimmune hypophysits. Painless thyroiditis presented with an early phase of subclinical thyrotoxicosis, which was followed by a later phase of hypothyroidism (low FT4) and by a subsequent complete recovery of thyroid function. During the hypothyroid phase, TSH was inappropriately low-normal and its response to TRH was blunted. It is reasonable to believe that the failed raise of serum TSH can be attributed the concomitant presence of autoimmune hypophysitis.
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pmc-6345097-2
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A 66-year-old male patient presented with a diagnosis of left lung adenocarcinoma for which he underwent superior-left lobectomy and local lymphadenectomy. Thereafter, he received adjuvant chemotherapy with cisplatin and vinorelbine, as well as local radiation therapy. Twelve months later, the patient experienced a relapsing disease, as assessed by whole-body FDG-PET, which showed disseminated metastatic disease involving lung, liver and bone. Docetaxel plus nintedanib therapy was performed for 8 months till liver and lung progression was observed. At this point, nivolumab (3 mg/kg i.v. every 2 weeks) was started. Pre-treatment serum levels of TSH, FT4 and FT3 were in the normal range; tests for anti-thyroglobulin (TgAb) and anti-thyroid-peroxidase (TPO-Ab) antibodies were negative. After the second administrations of nivolumab, the patient complained of palpitations and tremors. Biochemical assessment showed an undetectable serum TSH (< 0.01 mU/L) associated with elevated levels of FT3 (5.71 pg/ml; n.r. = 2.0–4.4). The serum level of FT4 was in the upper-normal range (FT4 1.36 ng/dl; n. r. = 0.89–1.76). Tests for TRAb, TPO-Ab and Tg-Ab were negative. In the month before, the patient did not receive any iodinated contrast media nor corticosteroid therapy. In basal conditions, other pituitary and peripheral hormones (ACTH, cortisol, GH, IGF-1, PRL, FSH, LH, testosterone) were normal. Adrenal stimulation with 1-24 ACTH (250 mcg i.v.) yielded a normal increase in serum cortisol levels (basal = 6.1 mcg/dl; 30 min = 16.4 mcg/d; 60 min = 21.3 mcg/dl). Thyroid ultrasound showed a multinodular goiter (estimated volume = 34 ml) with a normo-echoic pattern of the parenchyma and a normal pattern of vascularization. Fine-needle aspiration was performed on the two dominant nodules which yielded cytological benign findings.
The patient was initially treated with beta-blocker drugs only, but in the subsequent follow-up a worsening T3-toxicosis was evident. At this time, a 99 mTc scintigraphy revealed a diffuse thyroid uptake of the radionuclide suggesting Graves'-like hyperthyroidism. Methimazole (MMI) therapy was started at a dose of 15 mg/day. In the subsequent 3 months, the MMI dose was tapered and the patient is currently euthyroid under a maintenance dose of 7.5 mg/day of the drug. TRAb tests remained persistently negative. The thyroid hormone profiles of the patient are shown in Figure . Nivolumab therapy was continued and is still ongoing with no further progression of the neoplastic disease.
Written informed consent was obtained from both patients for the publication of this case reports.
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pmc-6345152-1
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Diana (a pseudonym) is a young woman of 25 years old that reached the Eating Disorders Centre, Division of Endocrine and Metabolic Diseases, San Luca Hospital in Milan, following a dramatic weight loss. Diana reached the Centre with a BMI of 16.06 kg/m2 reporting several disruptions in her eating patterns and several distressful alterations in her body image perception. As reported in the clinical history, Diana’s first eating related crisis was dated back 2 years before her current admission, with a subtle episode when she started a diet to lose some weight after health issues related to her thyroid. During that period, Diana was located abroad for work and – under moderate stress – she began a restrictive diet with a low caloric intake that brought her to lose 10 kg in 6 months. Diana’s weight remained constant in the following months but she developed an obsessive attention to the caloric intake along with intrusive thoughts regarding her weight and regarding specific types of foods. Diana also reported body-related image distortions such as overvaluation of her weight, mirror and body checking, and avoidance of body exposure. Moreover, during the crises, she reported frequent crying spells observing her body in front of a mirror.
In the last year, Diana reported a stressful situation at the University that heightened her psychological symptoms. Following these new difficulties, Diana re-enacted the restrictive conducts, reducing the caloric intake with a consequent weight loss of 4 kg in a month. In the period before the admission, the restrictive conduits were accompanied by self-induced vomit and daily binge episodes.
Diana matched all the DSM-5 criteria for a diagnosis of AN, binge-purge subtype. Compatible with the diagnosis, Diana presented distortions in her body perception and obsessive thoughts regarding her weight and her body image; for these reasons, she was considered as an optimal candidate for the interoceptive assessment.
At the begin of the rehabilitative protocol, Diana’s blood panels showed no signs of metabolic distress, with values in normal ranges. At her admission, Diana’s thyroid levels were within normal range and they remained within the normal range during the curse of the treatment. Endocrinologist suggested a chronic autoimmune normal-functioning condition. The psychiatric assessment indicated mood alterations toward a depressive condition accompanied by severe sleep difficulties and insomnia.
The rehabilitative program was composed of a multidisciplinary approach that included several experts in different fields: endocrinology, psychiatry, psychology, and nutrition. The specialists collaborated in an outpatients service tailored to the specific users’ needs. The rehabilitative program could extend from two to four cycles of treatment. Diana followed a two-cycle rehabilitative protocol with a frequency of 3 sessions a week for a total of 37 sessions. The protocol was composed of psychological intervention with group psychotherapy and individual sessions focused upon a psyco-corporal therapy approach (body-oriented psychotherapy). Psychological intervention was accompanied by psychiatric and pharmacological support (citalopram and mirtazapine), to moderate Diana’s mood alterations. The rehabilitative protocol was integrated with alimentary education sessions provided by the nutritionist. Additionally, the protocol was also accompanied by a nutritional program with fixed meals (both in quantity and composition) that Diana consumed under supervision. Scheduled assessment sessions ensured an adequate monitoring of the progress.
From the beginning, Diana showed a deep insight regarding her condition. Nonetheless, from her first session, Diana showed severe difficulties in following the assigned rehabilitative diet due to obsessive thoughts connected to her body weight and to certain types of food. In the following weeks, Diana improved her adherence to the recovery protocol with a better ability to follow the changes in the diet both on quantitative both on the qualitative level (e.g., types of foods consumed). Diana reached a BMI of 19.00 kg/m2 at the end of her second cycle of rehabilitative treatment. Considering the noticeable improvements regarding her eating behaviors and her general clinical condition, Diana was dismissed from the Centre and continued her program following only monthly assessments accompanied by individual psychotherapy.
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pmc-6345354-1
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In our case, we present a 28-year-old gentleman with a remote medical history of seizure disorder five years ago, who was transferred to our facility for further evaluation after presenting with tonic-clonic seizures and new-onset right-sided hemiparesis. The patient was afebrile, and did not report a history of recent infections, injuries or injection drug use. Neurological examination revealed an awake and oriented middle-aged male, with a paucity of speech, motor strength 1 of 5 in the right upper and lower extremities. Further examination of the oral cavity, oro- and naso-pharynx revealed poor dentition with no signs of localized infection.
Biochemical and hematological investigations revealed a normal leukocyte count (4,500/µL, normal 3,500-10,600), normal C-reactive protein level (4.22 mg/L, normal <9.10) and normal erythrocyte sedimentation rate (8 mm/hr, normal 0-13). Computed tomography (CT) of the head showed three adjacent ring-enhancing lesions in the left frontal lobe with 5 mm midline shift to the right, brain magnetic resonance imaging (MRI) T1 Axial sections showed a multi-loculated enhancing lesion with restricted focal diffusion surrounded by perilesional vasogenic edema with mass effect on the frontal horn of the left lateral ventricle (Figure ).
Intravenous (IV) corticosteroids were administered for their anti-inflammatory effect to hinder further edema, and empiric antibiotics were initiated with vancomycin, ceftriaxone, and metronidazole. Human immunodeficiency virus (HIV) infection was excluded. In an attempt to localize the primary source, CT of the sinuses did not reveal any evidence of sinusitis. CT of the abdomen and pelvis did not reveal any intra-abdominal abscesses. Trans-esophageal echocardiography revealed no evidence of valvular vegetations, however, it revealed a large sinus venosus atrial septal defect. CT angiography revealed contrast extravasation from the superior vena cava to the right superior pulmonary vein, suggestive of a right-to-left shunt.
The abscess was drained via a frontal craniotomy with stereotactic assistance; purulent fluid was evacuated and sent for culturing. Anaerobic cultures grew G morbillorum and Peptostreptococcus species. We used an initial antibiotic regimen consisting of a combination of ceftriaxone, metronidazole, and vancomycin. Antimicrobial susceptibility testing was not done, given the lack of standardized testing and no interpretation criteria. The patient started to develop fevers on post-operative day 7; the decision was made to discontinue vancomycin given the lack of methicillin resistant staphylococcus aureus (MRSA) growth in tissue cultures. Antibiotics were de-escalated to penicillin G and metronidazole, for a six-week course. Fever defervesced 24 hours after switching to penicillin, suggesting adequate sensitivity. Decision to use penicillin G was made after results for syphilis testing returned positive. On discharge, 17 days from presentation, the patient had a dramatic improvement in muscular strength, with bilateral motor function at baseline.
Our patient was discharged home to complete a six-week course of penicillin G and metronidazole. Antibiotics were continued for a course of six weeks. The patient was followed up with further imaging to ensure complete neurological resolution of the brain abscess. Brain MRI was done four months after presentation showing near-complete resolution of the abscess with significant improvement of brain edema (Figure ).
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pmc-6345719-1
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Patient 1 was a 38 years-old male. In May 2010, this patient was diagnosed with glioma soon after an episode of seizures. MRI showed intra-axial expansive and infiltrative lesions that were cortical and subcortical, and which affected the anterior half of the right temporal lobe and extending from the pole to the Sylvian fissure superiorly and to the right parahippocampal gyrus, posteriorly, and medially. Partial surgical resection was performed in August 2010 and the first pathologic diagnosis was astrocytoma grade II. He underwent chemotherapy with TMZ at a dose of 2,000 mg with cycles every 28 days for 5 days in the years 2011–2013, with no tumor regrowth until the beginning of 2015. At this time, he underwent MRI, which was used to compare the discrete extension of the signal alteration areas, especially the subinsular regions. In March 2015, he resumed chemotherapy with TMZ at a dose of 100 mg/day and the patient then lost 12 kg of body weight, which was associated with anorexia, insomnia, and depression. In May 2015, he suffered a seizure requiring hospitalization. In June 2015, the patient resumed the old chemotherapy regimen with TMZ (2,000 mg every 28 days for 5 days), and a follow-up with MRI; however, the tumor size continued to increase. In January 2016, the neuro-oncology team decided to discontinue treatment with TMZ considering the risk/benefit and planned a surgical re-approach. This was followed by chemoradiation and lasting 6 cycles of PCV associated with CBD. The CBD dosage was ranging from 300 to 450 mg/day.
During chemoradiation, the patient had an excellent clinical performance, practiced sports and had few symptoms of fatigue and/or nausea.
At 1 month after the end of chemoradiation, control MRI (Figure ) was characterized by exacerbation and the ultra-precocious phenomenon of PSD with increased edema and inflammatory disease characterized by extensive areas of contrast enhancement associated with tissue hypoperfusion (not shown). MRI controls demonstrated the progressive reduction of these findings.
The result of a pathological study after the first surgery was astrocytoma grade II with Ki67 staining of 5%. After the second surgery, he progressed to GBM grade IV (Figure ), related to increased cellularity, frequent mitosis, presence of micronecrosis, microvascular proliferation/endothelial, Ki67 staining of 30%, and loss of ATRX expression. Biomolecular marker analysis indicated IDH-1 mutated and MGMT methylated.
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pmc-6345719-2
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Patient 2 was a 38 years-old male diagnosed as left temporal glial neoplasia in May 2014 after a seizure. MRI showed an expansive infiltrative lesion predominantly in the subcortical region, with poorly defined contours located in the left temporal lobe, involvement of the upper, middle, and lower temporal gyrus, and an increase in the left temporal gyrus cortex. The lesion compromised a large part of the temporal lobe and extended to the temporal isthmus, the posterior aspect of the insula, and was deep in the trigeminal effigy of the left lateral ventricle. There was diffuse erasure of the regional cortical sulci and the Sylvian fissure, as well as a slight compression over the atrium of the left lateral ventricle. Stereotactic biopsy on April 2014 indicated a diagnosis of oligodendroglioma grade II. He received TMZ 1,875 mg with cycles every 23 days (during the 5 days of use he received a dose of 375 mg/day) from September 2014 to July 2015, with no tumor growth until the beginning of 2016. After checking the evolution of the tumor by MRI in February 2016, there was an increase in the dimensions of the remaining lesion, notably in the temporal isthmus, which had a similar expansive effect on the adjacent encephalic structures. The patient was submitted to a partial surgical resection followed by chemoradiation and lasting 6 cycles of PCV associated with CBD. The CBD dosage was ranging from 100 to 200 mg/day.
During the chemoradiation he had an excellent clinical performance, practiced sports, and had few symptoms of fatigue and/or nausea.
MRI control immediately after chemoradiation (Figure ) was used to characterize post-operative changes and showed a significant reduction of the infiltrative components of the tumor. The result of the pathological study after the first surgery (Figure ) was oligodendroglioma grade II. After the second surgery, he was diagnosed as oligodendroglioma grade III characterized by an increase in Ki67 staining of 9% and increased cellularity. Biomolecular marker analysis indicated IDH-1 mutated and MGMT methylated.
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pmc-6346143-1
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A CVID-diagnosed, 25-year-old, non-smoker woman was admitted to our center with LIP progression: CVID diagnosis was consistent with ESID criteria. The restrictive, granulomatous lung disease developed: open lung biopsy and histological examination showed lymphocytic infiltration of interstitial tissue: LIP diagnosis was confirmed by the histologic examination as well as T and B cells repertoire analysis as described previously (). Before the therapy spleen extended to the iliac crest (27 cm, see Figure bottom panel): subileus was observed due to the pressure on intestines.
Progressive LPD was observed with hyperviscosity, paraproteinemia, high β2M and IgM level (Figure upper panel). Respiratory functional study showed: reduced forced vital capacity (FVC-60% of the predicted volume, i.e., 23 dl) () and low diffusion capacity (for carbon monoxide DLCO-5,18 mmol/min/Kpa, i.e., 49%). BAL, blood, urine, bone marrow, sputum cultures, and MALDI analyses were all free of bacteria, mycobacteria, actinobacteria, and fungi. Analysis of fluid obtained by BAL showed an increase in the total cell count, predominantly in neutrophils and lymphocytes but without significant predominance of NKT cells as observed in hypersensitivity pneumonitis or pulmonary sarcoidosis (; ) (data not shown). Initial immunoparameters and cytometric analysis are shown in the first column of Table .
Contrary to previous data (), after intravenous immunoglobulin (IVIG) dosage adjustment (from 0.3 to 0.5 g/kg every 21 days, accordingly to the decrease of IgG before replacement) was ineffective as well as glucocorticoids (topical, then systemic with high-dose methylprednisolone up to 50 mg/daily) (Figure ).
Due to serum sickness with high IgM-paraproteinemia and high risk opportunistic infections, especially EBV reactivation (see pentamer analysis in Table ) lower rituximab dose (150 mg/m2 every week) was used. The patient did not receive other concomitant medication. The sixth dose (Figures , ) was delayed due to spontaneously resolved temporary neurological sign.
After temporary (6-month) remission and relapse the standard dose (375 mg/m2) infusion every 21 days) was used with the same remission interval (about 6 months). Such optimal schedule for RTx dosage every 3 weeks was described elsewhere (). In the third course patients could not receive regular regimen because of the increased incidence of infectious processes (especially pneumococcal, herpes zoster reactivation).
Relevant data from episode of care is shown as a timeline (Figure ) and in Table .
Unexpectedly, after rituximab therapy the LPD was stopped (Figure , as shown in PET). Restrictive pulmonary disease and FVC systematically improved and finally normalized (Figure ). The decrease of spleen size and leading biochemical and serological parameters were observed (i.e., β2M and IgM level, see Figures , ) in line with LIP regression and FVC increase. Fortunately, rituximab therapy was a glaring alternative for IVIG escalation: It also reduced IgG requirements by up to 40% of adjustment dose (Figure ). The serological and clinical effectiveness (IgM, FVC and PET finding) of both schedules were comparable, but more significant reduction of β2M was observed after 150 mg/m2 of rituximab (Figure ).
Parallel cytometric lymphocyte analysis is organized as a timeline (Figure ). A low dose does not affect the incidence of infections, but surprisingly it shows more pleiotropic immunomodulatory effects. There was observed high increase in low absolute number of CD16+56+ NK cells. Interestingly, T cell (CD4 and CD8) level also decreased, but it increased after the standard dose without the considerable increase of very low level of regulatory FoxP3 positive T lymphocytes (Treg) (Table ). Absolute number of natural killer T cells (NKT, CD3+56+) increased temporarily after low and standard dose (Figure ). Therefore, initial NK/NKT ratio (2.7) decreased during LPD progression, but it increased after the initial low dose of rituximab (Table ). Rituximab therapy shows a significant inhibitory effect on CMV-specific CD8+ cytotoxic T lymphocyte (CTL) levels and interferon gamma release, but RTx-induced CD38 expression on T cells was higher after the low dose (Table ). Interestingly, a low dose causes increase of post-mitogenic (PHA) interferon release.
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pmc-6346226-1
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A 62-year-old man visited our hospital due to a persistent fever of up to 38°C for one week. He had no significant medical history. He worked as a teacher at the university, and was an ex-smoker with a 14 pack-year history. He appeared to be well, and his vital signs were normal except for a low-grade fever of 37.5°C. However, chest radiography showed infiltrates in the upper lung fields bilaterally, and in the right middle to lower lung fields (Fig. A). The infiltrates were confirmed on the thoracic CT, which showed multiple large nodules up to 7 cm in diameter (Fig. B, arrow), which were in turn composed of numerous discrete small nodules like fireworks (Fig. B–D, arrows), the so-called “cluster” signs. Some of the large nodules had a hyper-dense portion centrally as a result of the coalescence of smaller nodules surrounded by partially discrete small nodules, not as densely assembled, suggestive of the “galaxy” sign (Fig. B, C, arrow heads). Thoracic CT showed no apparent mediastinal lymphadenopathy.
Based on a suspicion of TB or sarcoidosis, three samples of sputum or gastric contents were obtained on three separate days, tested using smears and cultures, and the results were negative for acid-fast bacilli in the outpatient setting. Additionally, the first bronchial brushings obtained from the right upper lobe (S1) and left upper lobe (S1 + 2) showed no evidence of Mycobacterium tuberculosis on cytology, polymerase chain reaction, and acid-fast culture. Therefore, 10 days after his first visit to our hospital, he was admitted to the respiratory department.
On the day of admission (Day 1), right thoracentesis was performed, which showed elevated lactate dehydrogenase levels of 1254 IU/L, total protein level of 5.1 g/dL, and glucose levels of 100 mg/dL, consistent with an exudative pleural effusion. The pleural fluid showed an increased total cell count of 1250 cells/μL, with a lymphocytic predominance (neutrophils 15%, lymphocytes 73%, monocytes 11%, and other cells 1%). Furthermore, marked elevation of adenosine deaminase levels in the pleural fluid (108 U/L) was noted. Serum laboratory test for TB, T-SPOT, was positive. Those results lead to the tentative diagnosis of bilateral pulmonary TB pleuritis on the right side, and anti-TB medication was commenced on Day 8.
The second bronchoscopy was performed on Day 4. The bronchoalveolar lavage (BAL) fluid obtained from the right middle lobe (B4) demonstrated an elevated total cell count (5.5 × 105 cells/mL), with lymphocytic predominance (lymphocytes 41%, macrophages 58%, and eosinophils 1%). The CD4-to-CD8 ratio of the lymphocytes in the BAL fluid was elevated at 5.87. Additionally, acid-fast culture of the BAL fluid was negative. Furthermore, on haematoxylin and eosin staining, the transbronchial biopsy specimens obtained from the right upper lobe (B3) and middle lobe (B4) showed non-caseating granulomas (Fig. ). Taken together, these results favour the diagnosis of pulmonary sarcoidosis, rather than TB.
However, at Day 21, acid-fast culture from the pleural effusion sample proved to be positive for M. tuberculosis. At Day 42, acid-fast culture of sputum taken soon after the first bronchoscopy was positive for M. tuberculosis, but was negative in all materials derived from both bronchoscopic procedures.
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pmc-6346500-1
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Our patient is a 64-year-old white man, height 176 cm, weight 90 kg, who developed a sudden-onset confusional state with perseverations and repetition of the same questions during a funeral for his brother-in-law to whom he had a close emotional relation. He had a previous history of arterial hypertension, myocarditis due to borreliosis with systolic dysfunction that was diagnosed 13 years prior to the current admission, and an allergy to penicillin. He was regularly taking candesartan and bisoprolol. A clinical neurologic examination on admission revealed disorientation in all qualities, retrograde amnesia, and reduced tendon reflexes but was otherwise normal. Blood pressure on admission was 140/77 mmHg. An electrocardiogram (ECG) showed left anterior hemiblock and negative T-waves in V2–V6. Blood tests revealed moderate renal insufficiency, high-sensitive troponin-T of 243 ng/L (normal, < 14 ng/L), and an N-terminal prohormone of brain natriuretic peptide (NT-proBNP) of 588 ng/L (normal, < 241 ng/L). MRI of his cerebrum was normal. Transthoracic echocardiography revealed dyskinesia of the left ventricular posterior, posterolateral, and apical parts of the left ventricular myocardium and apical ballooning (Fig. ). Clinical cardiologic examination was normal. On hospital day (hd) 2 his troponin-T fell to 77 ng/L. An electroencephalogram (EEG) was normal. Coronary angiography on hd4 was normal but ventriculography still showed mild apical ballooning. The neurological manifestations of the stress syndrome resolved except for mild memory disturbances for some words within a few hours after onset. Echocardiography and ECG normalized under medication with candesartan, bisoprolol, acetyl-salicylic acid, and atorvastatin within a few days after onset. Cardiologic and neurologic follow-up investigations 6 weeks after onset of the clinical manifestations were normal.
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pmc-6346580-1
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A 50- year old mentally disabled white male with a history of epilepsy was admitted to our hospital with fever and a painless red macule on his right anterior forearm (2x2cm) (Fig. ) The macule had first appeared 2 days prior to presentation followed by fever since one day. Physical examination was otherwise normal. Laboratory tests showed pancytopenia (Hb 7.0 g/dl, leukocytes 1,5/mm3 with an absolute neutrophil count of 0.09/mm3 and thrombocytes 31/mm3) and elevated CRP (60 mg/l). Patient was admitted and treated empirically for erysipelas with flucloxacillin. Within 4 days the arm lesion evolved from a painless red macule into a papule, haemorrhagic bullae and ultimately into a painful ulcer suggestive of ecthyma gangrenosum (Fig. .). Blood and lesion cultures revealed Pseudomonas Aeruginosa (wild type), confirming the diagnosis. The initial empirical treatment was switched to ceftazidime.
Microscopical examination of a peripheral-blood smear revealed abnormal lymphocytes (lambda positive, monocolonal B-cell population, 4% of peripheral blood leukocytes) and immunophenotyping using the immunofluorescence with flow cytometry was positive for CD45, CD19, CD20, CD22, CD79b, CD200, CD10, CD11c,CD103, CD305 and CD25 (Fig. ), and a diagnosis of hairy cell leukemia (HCL) was made. BRAF mutation analysis was not performed.
Despite adequate antibiotic treatment our patient continued to have high fever and elevated CRP. Clindamycin and single dose of gentamicin were empirically added to ceftazidim, but no clinical improvement ensued. We decided to start treatment of hairy cell leukaemia with cladribine (0.12 mg/kg during 5 days). After initiation of treatment, the ectyhma gangrenosum resolved completely within 3 months and the patient achieved a complete remission of HCL.
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pmc-6346691-1
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A 62-year-old man had obstructive jaundice and pancreatitis due to locally advanced pancreatic head cancer (T3, N1, M0, stage IIB, TNM classification on UICC). Endoscopic placement of a biliary and pancreatic stent (plastic stent, 5Fr. 9 cm) was performed, after which chemoradiotherapy (20 Gy, gemcitabine 1353 mg/body+S-1 120 mg/body) had been performed for 5 weeks. Four months later, he suddenly developed severe abdominal pain with symptoms of peritoneal irritation and presented to our hospital. His blood pressure was 91/67 mmHg, pulse rate 113/min, and temperature 37.0 °C. His abdomen was hard with some tenderness.
Laboratory data showed elevation of leukocytes (10,100/μl; reference values 4300 to 8000/μl) and C-reactive protein (13.92 mg/dl; reference values 0 to 0.40 mg/dl). Computed tomography (CT) revealed the tip of a pancreatic stent protruding from the pancreatic body, and there was fluid collection around the pancreas, omental bursa, and Douglas cavum (Fig. ). A diagnosis of panperitonitis due to perforation of the pancreatic duct was confirmed, and emergency operation was performed. The onset time was unclear, but he had experience slight epigastric pain 1 week before visiting, and the possibility that this event had occurred approximately 1 week prior to presentation was thus considered.
There was a large amount of cloudy ascites, and the tip of the pancreatic stent protruded from the pancreatic body (Fig. a). We deemed pancreatectomy and anastomosis to be risky with regard to postoperative complications. Therefore, we inserted pancreatic tubes into both sides of the perforated site, sutured between the posterior wall of the stomach and pancreas, and thereafter performed percutaneous transgastric drainage (Fig. b). The operation time was 173 min. The postoperative course was uneventful, and we changed to internal drainage by cutting the tubes in the stomach. The patient was discharged on postoperative day 87 and has undergone chemotherapy.
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pmc-6346692-1
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A 67-year-old female with NF1 presented with lumbago, cold sweats, and sudden onset weakness, which necessitated an emergency transfer to our institution. She had no significant past medical history, was negative for hypertension, and was on no regular medications. She was hypotensive (systolic blood pressure = 60 mmHg) and tachycardic on arrival. An abdominal contrast computed tomography (CT) scan showed a rupture of an abdominal aortic aneurysm (AAA) with communication between the aneurysm and the inferior vena cava (IVC) (Fig. a, b). The maximum dimensions of the ruptured AAA and IVC were 34 mm and 37 mm, respectively. The aortic rupture was located 7.5 cm distal to the renal artery and 2.5 cm proximal to the bifurcation of the aorta (Fig. c). Due to the difficulty of primarily closing the ruptured IVC, we planned an endovascular treatment to control the bleeding from the IVC by exclusion of the ruptured AAA. The diameter of normal proximal aorta was 16 mm, which was too narrow to place a normal Y-shaped graft. Moreover, there was insufficient time to prepare another stent in emergency. Therefore, we instead deployed an ENDURANTII (Medtronic Vascular, Santa Rosa, CA, USA) iliac extension proximal to the terminal aorta that was long enough to insert three or more stents (Fig. d). As the bleeding from the AAA and the communication between the aneurysm and the IVC were not well controlled, we placed an EXCLUDER (W.L. Gore & Associates, Flagstaff, AZ, USA) cuff in the ENDURANTII iliac extension. Although a type IV endoleak was detected on angiography, the patient’s hemodynamics stabilized. We therefore decided to conclude the operation at this point and re-assess the endoleak in a few days.
An abdominal contrast CT performed 3 days after the operation showed a type Ib endoleak and injury to the distal abdominal aorta (Fig. a). This led to a redo-EVAR 4 days after operation. This included deploying an AFX (Endologix, Inc., Irvine, CA, USA) graft, as well as an infrarenal cuff, as the AFX head did not fit exactly within the stent placed in the primary operation. We also performed coil embolization treatment of the left internal iliac artery aneurysm (Fig. b), which was identified initially (Fig. b). We confirmed no endoleak at the final angiography. An abdominal contrast CT performed 18 days after the primary operation showed two new sequential aneurysms of the LCA, which were not previously detected. Coil embolization was planned to address these new aneurysms.
Twenty-two days after primary operation, however, the patient presented with new onset nausea, left abdominal pain, and hypotension. Her systolic blood pressure was 50 mmHg. She was resuscitated and required vasopressor support. Laboratory tests, including leukocyte count and electrolytes, were normal. The serum C-reactive protein and D-dimer were elevated to 0.37 mg/dl (normal range 0.01–0.30) and 6.8 μg/ml (normal range 0.0–1.0), respectively. We considered potential bleeding from nearby arteries, including the inferior mesenteric artery (IMA) and lumbar artery [, ]. An abdominal contrast CT showed enlargement of LCA aneurysms and surrounding hematoma (Fig. a–c), establishing the diagnosis of rupture of the LCA aneurysms. Emergency exploratory surgery was performed.
We gained access through a midline abdominal incision. Active bleeding was encountered intraperitoneally and controlled with compression. As a result of the patient’s initial AAA rupture, residual hematoma was present, which extended along the entire left abdomen and retroperitoneum (Fig. a). In addition, the AAA stent endograft had resulted in a left laterally displaced aorta, narrowing our operative field. Due to her distorted anatomy, active bleeding, and clinical condition, we elected to perform a left hemicolectomy. We divided the inferior mesenteric artery (IMA) 3 cm distal to the IMA root to not to injure the recently repaired aorta. We divided the colon at the rectosigmoid junction. Proximally, we divided the colon at the middle of the transverse colon (Fig. b). The mesentery was divided to include the LCA aneurysms. Given her clinical condition, we elected to not perform a colorectal anastomosis, but instead performed a Hartmann’s procedure with an end transverse colon colostomy. The surgery took 145 min, and the estimated blood loss was 4.5 L. Packed red blood cell transfusion volume was approximately 1.96 L, and fresh frozen plasma volume was 1.44 L.
In reviewing the specimens, a 2-cm aneurysmal sac and 1-cm aneurysmal sac were identified in the LCA. No thrombus was identified (Fig. a, b). No ischemic changes and no tumor were observed in the excised colon (Fig. c). Interestingly, fibrous tissue around the left colic artery stained positive for S-100 protein, suggesting that neurofibroma from NF1 might have been associated with the rupture of these aneurysms due to weakened integrity of the vascular walls (Fig. a–e). A follow-up abdominal CT showed no recurrence of the endoleak. The patient was discharged 37 days after the last operation without any other post-operative complications.
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pmc-6346695-1
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An 11-year-old boy with pulmonary arterial hypertension underwent living-donor lung transplantation, with tacrolimus, mycophenolate mofetil (MMF), and prednisolone (PSL) given as immunosuppressive agents. At 76 months after transplantation, M. gordonae was isolated from a broncho-alveolar lavage (BAL) sample obtained during a surveillance examination. Contamination was suspected, because even though there were no symptoms, chest computed tomography (CT) showed a slight amount of consolidation in the left upper lesion (Fig. a). PSL as therapeutic and diagnostic treatment was decreased. Sputum culture findings were negative after 5 months, and chest CT images were clear. There was no further NTM detection during the following 15 years.
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pmc-6346695-2
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A 38-year-old female with bronchial ectasia underwent living-donor lung transplantation, with ciclosporin, MMF, and PSL given as immunosuppressive agents. At 82 months after transplantation, the patient developed a fever with purulent sputum and chest CT showed consolidation in the left lower lesion (Fig. b). M. abscessus complex (MABSC) was isolated from a BAL sample. Following administrations of tazobactam/piperacillin and azithromycin (AZM), as well as a decrease in ciclosporin from 120 to 50 mg for 1 month, the sputum cultures became negative. AZM administration and decreased ciclosporin were continued for 22 months, with no recurrence noted.
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pmc-6346695-3
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A 39-year-old female with lymphangioleiomyomatosis underwent single deceased donor lung transplantation, with ciclosporin, MMF, and PSL given as immunosuppressive agents. At 58 months after transplantation, a fever developed and chest CT showed consolidation in the transplanted lung (Fig. c). MABSC was isolated in a cultured sputum sample. Following administrations of imipenem, amikacin, and AZM for 4 months, sputum culture findings became negative. Maintenance therapy with imipenem and amikacin was given once a week, along with daily AZM and a decrease in MMF, with no recurrence seen during the following 1-year period.
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pmc-6346695-4
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A 41-year-old male with interstitial pneumonia underwent single deceased donor lung transplantation, with ciclosporin, MMF, and PSL given as immunosuppressive agents. At 12 months after transplantation, a fever developed and chest CT showed consolidation in the native lung (Fig. d), which was suspected to be pneumonia caused by general bacteria. Broad-spectrum antibiotic therapy was started, though was not effective. After a period of time, M. intracellulare was isolated from a cultured sputum sample. Thereafter, rifampicin (RFP), ethambutol, and clarithromycin (REC) treatment was administered for 3 months, after which sputum findings were negative. Rifampicin was continued at 800 mg, the standard level, while ciclosporin was adjusted according to trough level and finally administered at 550–600 mg, three times the normal dose, and PSL was gradually reduced. The patient died after 21 months because of respiratory failure due to chronic rejection, though sputum culture findings remained negative with REC treatment These cases are summarized in Table .
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pmc-6347147-1
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A male patient in his 80s who was diagnosed with HCC was admitted to our hospital. He suffered from type C hepatitis and his ICGR15 was 15%. The very large tumor necessitated right lobectomy of the liver to achieve curative resection. Although a left lobectomy or segmentectomy were recommended by the Makuuchi criteria, 3D simulation using VINCENT showed that remnant liver volume after right lobectomy was almost 50% because tumor was large (). Therefore, right lobectomy was performed. Unfortunately, this patient died due to liver failure 8 days postoperatively. The safety limit and maximum limit in this case were 35% and 58%, respectively; thus, this case was located in risky area. The advanced age of the patient and the relatively large (826 ml) intraoperative blood loss might be additional factors that contributed to his death.
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pmc-6347147-2
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A male patient in his 60s was admitted to our hospital with intrahepatic chorangiocarcinoma. Because the tumor was located in liver segments S2 and S3, lateral segmentectmy of the liver was necessary to achieve curative resection. The patient suffered from alcoholic liver cirrhosis; his ICGR15 value was 21.9%. Based on the Makuuchi criteria, a subsegmentectomy was recommended. However, 3D simulation using VINCENT showed that the volume of the lateral segment was 16.4% () as the safety limit was 30%. Lateral segmentectomy of the liver was safely performed without postoperative complications.
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pmc-6347445-1
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A previously healthy, 52-year-old Caucasian man presented to his family physician a week after having a tonic-clonic seizure. A magnetic resonance imaging (MRI) scan showed a 10 cm left frontal tumor, which was confirmed as an atypical meningioma following craniotomy and resection (Figure ).
Postoperatively, he took 400 mg of phenytoin PO once a day. He had no seizures postoperatively or afterward. The patient uneventfully received 60 Gy of adjuvant radiation therapy to the postoperative bed in 30 fractions. Three months after the resection of the tumor, the patient began a trial of phenytoin but nine days later, he developed symptoms consistent with a generalized seizure. He resumed his daily phenytoin prophylaxis with good effect.
Two months later, he complained of blood in the stool and after an evaluation was diagnosed with a locally advanced nonmetastatic adenocarcinoma of the low rectum (Figure ). A curative-intent dose of 50.4 Gy in 28 fractions of neoadjuvant radiation therapy was prescribed, with 2000 mg PO BID of concurrent radiosensitizing capecitabine []. After 20 of the planned 28 fractions, he began to feel unwell and experienced new, right-sided upper and lower limb dysfunction and an unsteady gait. A contrast-enhanced computed tomography (CT) scan of the brain showed no suspicious findings but his phenytoin level was dramatically elevated at 138 µmol/L, compared to 49 µmol/L just prior to neoadjuvant therapy (normal range: 40-80 µmol/L). His albumin level from a few weeks prior to these symptoms had also been normal at 39 g/L (normal range: 34-46 g/L), and he was taking no other medications other than an occasional stimulant laxative. Capecitabine was discontinued, and the patient was treated with charcoal and admitted for observation. Phenytoin was temporarily discontinued and then reintroduced at the previous dose of 400 mg PO per day once levels began to normalize. His symptoms quickly resolved and he showed no further toxicity.
He resumed radiation therapy a few days later without concurrent capecitabine. It was believed that he had developed phenytoin toxicity secondary to impaired clearance as a result of his capecitabine. His phenytoin levels were monitored during the following weeks and his phenytoin dose was bridged with lacosamide and titrated down gradually and then discontinued, with no further symptoms of toxicity. The patient remained on 200 mg PO per day of lacosamide. He underwent a surgical resection with clear margins followed by adjuvant capecitabine and showed no signs of a recurrence of rectal adenocarcinoma thereafter. Three years later, the patient passed away from recurrent meningioma.
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pmc-6347751-1
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A 56-year-old Chinese man was hospitalized 2 months after the discovery of a pancreatic mass and a 1-month history of abdominal pain. He had taken no medication before hospitalization.
A physical examination after the hospitalization did not reveal any obvious abnormalities. He had a body temperature of 36.6 °C, heart rate of 99 beats per minute, blood pressure of 141/83 mmHg, respiratory rate of 20 breaths per minute, and oxygen saturation of 100%. His neurological status was normal. His family history was noncontributory. He smoked cigarettes for 20 years, but it is unknown how many cigarettes he smoked per day. He never consumed alcohol. Occupationally, he worked as an office manager.
Laboratory test results are shown in Table . Blood tests revealed a high level of the CA19-9 tumor marker (1525.84 U/mL). An abdominal computed tomography scan with enhancement and vascular reconstruction revealed a space-occupying lesion in the pancreatic head and neck; the findings suggested the presence of pancreatic cancer with invasion of the hepatic artery, splenic artery, mesenteric vein, and origin of the portal vein (Fig. ). Pathological examination of a specimen from an endoscopic ultrasound puncture biopsy revealed abnormal cells, with a morphology consistent with that of adenocarcinoma. Positron emission tomography-computed tomography revealed abnormally high fluorodeoxyglucose metabolism that was limited to the space-occupying lesion, suggesting a malignant pancreatic lesion, with atrophy of the pancreatic tail and many small nodules in the space surrounding the pancreas. He was diagnosed as having a T4N2M0 local advanced pancreatic cancer.
Six days after admission, he underwent distal pancreatectomy and splenectomy as well as intraoperative radiotherapy (described below) under general anesthesia with tracheal intubation. A subcostal incision was made to expose his abdominal cavity, and the Kocher maneuver was subsequently performed to dissect the gastrocolic ligament and duodenal lateral peritoneum, which exposed the pancreatic tumor. The lesion was approximately 8 cm × 5 cm and had a hard texture and the tumor activity is poor (Fig. ). Dissection and pancreatic isolation (starting from the tail and moving to the right) revealed that the tumor had invaded the superior mesenteric vein, middle colic artery, and celiac trunk artery. After isolating his spleen, the pancreatic retroperitoneum was opened, and his spleen and pancreatic tail were turned upward and isolated left to right from the posterior aspect to the superior mesenteric artery. A harmonic scalpel was used at this site to sever his pancreas, and the pancreatic duct was closed using a 4–0 Dexon suture. Local tumor remnants were observed in the posterior pancreas. The upper boundary of the remaining tumor bed reached the trunk of his abdominal cavity, the lower boundary reached his middle colic artery starting from the superior mesenteric artery, and the right boundary reached his superior mesenteric artery.
The INTRABEAM system was moved into the surgical field, and a 6-cm flatbed source applicator was selected based on the appearance of the tumor bed (Fig. ). After applying an aseptic protective cover, the source applicator was placed close to the tumor bed, and our patient’s surrounding bowel and organs were protectively insulated using two layers of surgical gauze (approximate thickness 2 cm). After all personnel had left the operating room, radiotherapy was started using the following parameters: a radiotherapy dose of 10 Gy, an irradiation time of 27.4 minutes, an acceleration voltage of 50 kV, and an acceleration current of 40 μA. A drainage tube was subsequently placed at the cut edge of his pancreas, and his abdominal cavity was closed after confirming that there was no bleeding or pancreatic leakage. The total intraoperative blood loss was 200 mL.
He passed gas on postoperative day 2, and the gastric feeding tube was subsequently removed. On postoperative day 8, < 30 mL of fluid (amylase 21 U/L) had been lost via the abdominal drainage tube, which was removed before our patient was discharged on postoperative day 9. No surgery-related complications were observed, his postoperative CA19-9 level was 924.73 U/L, and his abdominal pain completely disappeared based on the numerical rating scale for cancer pain []. He has been followed for 6 months after surgery, and no obvious tumor recurrence has been observed.
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pmc-6347754-1
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A 66-year-old woman with a significant past medical history of well-controlled hypertension was admitted with complaints of microscopic hematuria and mild proteinuria for the past 3 years. Serum creatinine level was within normal range at that time and therefore the anti-GBM antibody was not tested. The first renal biopsy revealed mesangial proliferative glomerulonephritis with fibro-cellular crescents in one out of 18 glomeruli, excluding one global sclerotic glomerulus (Fig. ), and deposition of IgA and C3 in mesangial areas by immunofluorescence microscopy (Fig. ). Weak but significant IgG deposition was also observed in glomeruli in the distribution somewhat different from IgA or C3 (Fig. ). The electron-dense deposits were observed in mesangial areas by electron microscopy. Therefore, the diagnosis was IgA nephropathy. Antihypertensive therapy was initiated, mainly with an RAS inhibitor. Eight months later, the patient’s serum creatinine suddenly rose to 4.53 mg/dL (it was 1.04 mg/dL from the routine blood test 1 month before). Urinalysis showed 100 red blood cells per high power field and urinary protein excretion of 12.3 g/gCr (Fig. ). The serological tests that were performed to differentiate the cause of rapidly progressive glomerulonephritis revealed the presence of anti-GBM antibody at the titer of 116 IU/mL and the absence of anti-nuclear antibody and anti-neutrophil cytoplasmic antibody. Laboratory findings on admission are summarized in the Table .
After admission, treatments with hemodialysis, plasma exchange, and intravenous methylprednisolone pulse therapy followed by oral prednisolone at the dose of 50 mg/day were initiated. The second renal biopsy was performed at 4 weeks after admission in order to assess the probability of renal recovery and to make the final diagnosis. It revealed cellular to fibrocellular crescents in 18 of 25 glomeruli, excluding six global sclerotic glomeruli by light microscopy. By immunofluorescence study, linear IgG deposition along the glomerular capillary walls and mesangial staining for IgA were observed. On the other hand, C3 deposition was observed in the mesangium as well as in the glomerular capillary walls (Fig. ). Electron-dense deposits were observed in mesangial areas, similarly as in the first biopsy, by electron microscopy (Fig. ). Based on the aforementioned findings, the diagnosis of anti-GBM glomerulonephritis and IgA nephropathy was confirmed. Plasmapheresis was performed eight times, anti-GBM antibody gradually decreased, and alveolar hemorrhage was prevented. However, her renal function could not be restored and she underwent maintenance hemodialysis (Fig. ).
Additional immunosuppressant was not given because the patient did not show any sign of pulmonary involvement and because the renal recovery was quite unlikely from clinical (continuous oliguria and hemodialysis dependence) as well as histological (crescent formation in most of non-sclerotic glomeruli) point of view.
Clinical and histological presentations from IgA nephropathy (at the time of first renal biopsy) and from anti-GBM disease (at the time of second renal biopsy) were summarized in the Table .
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pmc-6347841-1
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A 63-year-old male patient with large anterior mediastinal mass was referred to our hospital for treatment. The patient was pathologically diagnosed as cervical schwannoma and underwent surgical resection twelve years ago. He had re-operation because of the recurrent neck tumor four years ago. No specific neural, cardiovascular and respiratory disfunction and neoplasms his history contained as well as his family history. The patient suffered from chest oppression and shortness of breath for four months, and these symptoms gradually became worse. The Preoperative CT confirmed that the patient was diagnosed as TM and large anterior mediastinal mass (Fig. ) Due to occasion of severe airway overreaction during the process of his endoscopy, fiber bronchoscopy was not finished.
Consideration of potential risk from serious TM, the patient was intubated with guidance of fibreoptic bronchoscopy in the supine position, then underwent median sternotomy and tumor resection followed by tracheal suspension. The prime procedures of this surgery were briefly depicted by hand drawings (Fig. a: tumor site exposure; Fig. b: further sculpture of removed autogenous rib cartilage; Fig. c: anchoring malacial tracheal rings and membrane by fresh graft) and details of surgical procedure were as follows:
Step 1: Tumorectomy. After medisection of sternum followed by opening pretracheal fascia, upper principal bronchus and frontage of cervical schwannoma were revealed. Along the line between the tumor and its adjacent tissue,the tumor was underwent entire resected.
Step 2: Fabrication of scaffold. Partial autogenous rib cartilage was removed from 5th rib, and its top and bottom parts were penetrated with flexible steel needle to form two channels available for thread, which could manufactured a scaffold to anchor the extensive malacial tracheal rings and membrane (Fig. ).
Step 3: Tracheal suspension. Free rib cartilage graft, fixed with bilateral tracheal rings, were deposited in front of malacial trachea by silk thread across the channels to cover the collapsed tracheal wall, so the malactic tracheal rings and membrane were elevated and pulled for enlarging the diameter of cartilaginous ring (Fig. a).
After living a short period of mechanical ventilation with positive airway pressure, the patient was successfully extubated within 12 h after surgery. During his hospital stay, major postoperative complications didn’t occur, but mild pneumonia happened. The patient was discharged on the 16th day postoperatively. In the follow-up,the images showed that either cross section of intraluminal stenosis or collapsed segment of airway was remarkably relieved, and scaffold made by autogenous rib cartilage clinged to extratrachea stably. It was surprising that the graft finally integrated with tracheal wall (Fig. b).
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pmc-6348079-1
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A 73-year-old Caucasian woman with a medical history significant only for hypertension, presented to our emergency department complaining of intermittent subjective fever, anorexia, weakness, and fatigue for 2 weeks. Her subjective fevers were occurring almost nightly, and she had associated night sweats. Her weight was stable. She had a persistent non-productive cough. There was no sore throat or rashes. Her review of systems was negative for any other current symptoms. Her only medication was enalapril. Her family history was non-contributory.
She had been previously assessed by her family doctor for the same symptoms 2 weeks prior to this presentation. Routine investigations were unrevealing. At that time, she had left knee pain that developed after a hike the previous month. X-rays of her knee and femur were unremarkable. Her pain resolved within a week. No therapeutic interventions were undertaken at that time.
She had no sick contacts, no sexual partners, and no insect or tick bites. She had no known exposure to tuberculosis. She travelled to the Channel Islands 3 months before presentation. She had no animal exposures. She denied any history of injection drug use.
On initial examination, she appeared non-toxic. Her vital signs included a temperature of 38.6 °C, a heart rate of 96 beats/minute, blood pressure of 130/65 mmHg, and oxygen saturation of 99% on room air. There were no rashes and no lymphadenopathy was present. There were no signs of hyperthyroidism and the thyroid itself was normal in size without any nodules. Her jugular venous pulse was 2 cm above the sternal angle. She had normal heart sounds with no extra sounds or murmurs. There were no stigmata of endocarditis. Her lungs were clear with equal breath sounds bilaterally. An abdominal examination revealed a soft and non-tender abdomen. There was no hepatosplenomegaly, jaundice, or asterixis. Examination of her knees did not reveal any redness, warmth, effusions, or pain. A screening neurologic examination demonstrated grossly normal cranial nerves, full strength bilaterally, and normal reflexes, tone, and coordination. She was admitted for further investigation for her fever of unclear cause. Empiric piperacillin-tazobactam and intravenously administered saline were started on admission as acute bacterial infection was in the differential diagnosis.
Table displays the results of her laboratory investigations. A peripheral smear was unremarkable. Serum free light chains were normal. No monoclone was found on serum protein electrophoresis. Urine analysis was bland. Five sets of blood cultures, a urine culture, and Lyme serology were negative. A chest X-ray was normal. Computed tomography (CT) scans of her head, neck, chest, abdomen, and pelvis were all unremarkable. A transthoracic echocardiogram revealed a normal heart with no vegetations.
She had one further temperature of 39.4 °C while in hospital, without any clear infectious source. Once the blood cultures were known to be negative, piperacillin-tazobactam was stopped. There was an impression that her workup could be continued on an out-patient basis as immediately life-threatening causes of fever had been ruled out. She was discharged home after an 8-day admission in hospital with plan for out-patient follow up.
She was seen 1 month after discharge. She had no improvement in her symptoms and noted a recurrence of her left leg pain. Her C-reactive protein (CRP) was 207 mg/L. On examination, she had a large, warm, left thigh mass. An urgent ultrasound revealed a 4.5 × 6.8 × 11.6 cm spindle-shaped, well-defined soft tissue mass with internal vascularity (Fig. ). Magnetic resonance imaging (MRI) found that the mass met the femur but was not invading (Fig. ). An initial biopsy revealed a poorly differentiated malignant neoplasm.
She underwent a distal femur excision with distal Global Modular Replacement System (GMRS) reconstruction. Final pathology revealed a grade 3, pT2bN0M0 undifferentiated sarcoma with epithelioid morphology. She had no nodal involvement or distant metastases at this time. Her CRP fell to 28.42 mg/L within 8 days of surgical excision. She recovered well from her surgery with resolution of her constitutional symptoms. She subsequently was planned to receive radiation therapy.
Prior to receiving radiation therapy, a follow-up CT scan was done a couple months after her surgery. This revealed the presence of a new 4 mm pulmonary nodule in the lower lobe of her left lung that was not felt to be a metastasis. There was no other evidence of distant metastases. Given these results, adjuvant radiation treatment was begun. She received 6600 cGy given in 33 fractions to her leg.
Roughly 1 month following the end of her radiation therapy course, she re-presented to our emergency room with painless hematuria and a month-long history of non-productive cough associated with decreased energy. CT scans of her chest revealed 16 pulmonary masses, measuring up to 6.2 cm. A CT scan of her abdomen and pelvis revealed a solitary nonobstructive renal calculus, as well as a new 3.2 × 6.5 cm pelvic mass.
She was subsequently referred to radiation and medical oncology where a shared decision was made to pursue palliative management.
Figure provides a timeline of the above described case.
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pmc-6348206-1
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In Mar 2016, a 10-yr-old Italian Segugio breed neutered female dog from Iasi County-Moldova Region, northeastern Romania, was presented at a local veterinary clinic. The dog was born in Torino, Italy and adopted from an animal shelter as a puppy by a Romanian owner and transferred to Romania. The female canine had a history of multiple backs and forward traveling from Romania to Italy. Symptoms at presentation were: progressive weight loss, skin wounds over the muzzle, foot pads and dermal lesions over the right and left tarsal joints. According to the owner, these lesions gradually appeared and progressed at least 30 d before the visit. On physical examination, multifocal alopecia and crusting dermatitis were seen () together with polyarthritis (), lymphadenopathies, fatigue, and weight loss. No symptoms of fever or diarrhea were observed.
A direct radiography was performed. Furthermore, a blood sample was collected for serology and for complete blood count and serum biochemistry panel. The puncture of a popliteal lymph node was performed, for histological examination.
The enlargement of spleen and liver were seen, with no other changes. Considering the traveling history we followed the presumptive diagnosis of leishmaniasis. Serology for Leishmania spp. was submitted to the Synevovet Laboratory, Bucharest and performed by ELISA assay, providing a positive result, since the laboratory reference was considered negative. The blood count reported by the laboratory revealed mild leukopenia WBC-6.3×103/mm3 (reference 6.9–12×103), anemia – low hemoglobin level – 7.8 g/dl (reference15–29 g/dl) with low red blood cell count −3.9×106 /mm3 (reference 5.50–8.50×106 /mm3) and low hematocrit level −18.8% (reference 44%–57%), thrombocytopenia −117 ×103/mm3 (reference 200–450 ×103/mm3) and lymphopenia 0.5×103/mm3 (reference 1–3.6 ×103/mm3). Serum biochemistry showed hyperproteinemia 10.7 g/dL (reference 5.4–7.4 g/dL), low ala-nine transaminase level 6 U/L (8–57 U/L), low triglyceride levels 28 mg/dL (reference 37–39 mg/dL) and elevated creatine phosphokinase – 213 U/L (reference 14–120 U/L).
The puncture sample was submitted to the Department of Animal Pathology, Faculty of Veterinary Medicine of Iasi. Light microscopy of the stained smears prepared from popliteal lymph node puncture, failed to identify the amastigotes. However, a massive inflammatory reaction was seen, accompanied by lymphocytic and neutrophilic infiltration.
In this case, the diagnosis of CaL was based on the clinical symptoms, history of traveling to endemic area and on laboratory findings. The dog was treated using N-methylglucamine antimoniate (Glucantime) (50 mg/kg/BID subcutaneous) for eight weeks, and Allopurinol (10 mg/kg/BID per os) for eight months. After 9 months follow-up, the dog showed an improved general body condition, with no signs of recurrence. At this time, the blood count reported by the laboratory was in range.
The Ethics Committee of the university approved the study.
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pmc-6348210-1
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A 6-yr-old girl who was traveling to rural area in the suburb area of Sari (Mazandaran Province, northern Iran) as tourist from Mashhad (Khorasan Razavi Province, eastern Iran) with vulvar bleeding, referred to the Emergency unit of the Booali Sina Hospital in Sari, northern Iran in Sep 2015,
She had such complaint for one week before admission and suffered from continuous moderate vulvar bleeding and dizziness. She denied having a history of trauma. The patient did not complain of any other symptoms. Through obtain history; her mother explained that who swim in a pond for about one hour. Clinical examination was achieved after her parents gave oral informed consent. Surprisingly, one leech about 5 cm in length was found in the minor labia of the vulva ().
No abnormality and trauma were seen and her hymen was intact. The leech was sent to the parasitology laboratory section and documented as leech belonging to Erpobdellidae family.
To improve the symptoms associated with vulvar bleeding, the leech was removed using forceps and washed twice the vulvar and vaginal cavity with normal saline and antibacterial solutions. The bleeding stopped 1 h later and the patient was discharged on the next day. She was followed up 2 d after removal of the leech; there was no symptom of infestation and bleeding.
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pmc-6348214-1
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A 53-yr-old man from Golpayegan, Isfahan Province, central Iran, with past history of cholecystectomy referred with complaint of vague colicky epigastric pain begun 2 yr from presentation and had fluctuation during the time. The pain became severe and constant gradually, with the peak about 4 months ago, led him to seek medical attention. There was no relationship between food consumption, especially high fat-containing meals, and pain severity. At the beginning of the disease course, the pain had been relieved by anti-acids but eventually, none of the outpatient medications made him free of pain. He did not mention any urticarial reaction, jaundice, nausea, vomiting, night sweats or pruritus during these two years, but he had history of occasional fever without any specific pattern accompanied by chills within this period. He did not have anorexia, but had increased appetite instead; constipation was a cardinal symptom along with malaise. Significant weight loss (about 10 kg) occurred late in the course of his disease (i.e. the last 2 months).
The patient agreed to be presented in this report and a written consent was signed by him. He was formerly a farmer and husband-man and from one year before the onset of disease (i.e. 3 yr from now), he was a trucker and had history of traveling around the country and taking unsafe and insanitary food and water. He denied consumption of any illicit drugs, alcohol, and opium at all, but he used to smoke cigarettes. No special medication he was taking.
On physical examination, he was afebrile and moderately ill, with stable vital signs and normal sclera without icterus. There was no rash or signs of excoriation, no conjunctivitis, no lymphadenopathy, no gynecomastia and abdominal distention. There was not any abnormality in cardiovascular and lung examination. Right upper quadrant tenderness existed on abdominal palpation, without rebound tenderness and organomegaly. Musculoskeletal and neurologic examinations were normal. The results of initial laboratory investigations are presented in . The first colonoscopy survey was normal and initial evaluation by upper gastrointestinal endoscopy revealed mild antral gastritis. Abdominal ultrasonography revealed dilated CBD without any visible lesion at its end. A hypo-echo mass lesion suggesting pathologic lymph node was seen lateral to aortic bifurcation, at left side. Abdominal CT-scan suggested dilated intra- and extrahepatic biliary ducts and CBD with 10 mm diameter and also some large intestinal lymph nodes with compressive effect on adjacent structures.
The second upper gastrointestinal endoscopic survey had the same result as the first.
During Endoscopic retrograde cholangiopancreatography (ERCP), dilated common bile duct (CBD) about 12 millimeter and large ampulla were seen. After sphincterotomy, multiple flat whitish parasites were incarcerated behind Oddi’s sphincter and within common bile duct, which extracted. There was no stone in CBD and ampulla did not contain any mass. Serologic exam, using ELISA method was performed by somatic antigen of Fasciola and yielded positive according to the reference laboratory cut-off.
The patient was treated with triclabendazole 250 mg, three tablets daily, for two executive days (six tablets totally) successfully and he pains relieved after ERCP and full course of treatment. He advised not to use unsanitary food and water. On follow up, he was well and did not mention any similar abdominal pain as before.
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pmc-6348215-1
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In 2009, a 47 yr-old man, who lived and worked in the city of Pereira Barreto, state of São Paulo, Brazil, located 650 km from the state capital, received an initial diagnosis of mucocutaneous leishmaniasis (ML) affecting his face and oral mucosa. His hometown belongs to an area in which there is a high transmission of cutaneous (CL) and visceral leishmaniasis (VL) in dogs and humans (, ). The patient was successfully treated with meglumine antimoniate for a month (1200 mg/day, intramuscular). Two yr later, in 2011, he attended the same outpatient clinic presenting a significant weight loss and mucocutaneous lesions located at the same region of the ones diagnosed in 2009. Once again he was successfully treated with meglumine antimoniate for a month (same therapeutic regimen). The patient was also diagnosed with HIV and hepatitis C (high viral loads) and had a CD4+ cell count of 198/mm3. An antiretroviral treatment (HAART) was started, with significant viral load reduction, but soon after, the medical monitoring was abandoned again, when he was discharged from hospital. Patient’s last hospital admission occurred in Jul 2013. He was attended at the Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo (HCFMUSP), a tertiary reference center located in the city of São Paulo (SP). A severe hoarseness, discomfort swallowing, and whitish oropharyngeal lesions, aside from episodes of cough, fever, sweating and a marked weight loss referred to in the last two months were presented by the patient. In addition, the abdominal ultrasonography revealed the presence of a marked splenomegaly. The CD4+ cell count was 40/mm3 and the HIV and hepatitis C viral loads were extremely high. Due to the severe hoarseness, a larynx biopsy was performed and the histological findings () confirmed the diagnosis of ML. In this time, a Visceralization of the ML had occurred because of a pancytopenia and the presence of Leishmania amastigotes in stained smears from a bone marrow aspirate (). Regarding the leishmaniasis, the patient received 200 mg/day of intravenous liposomal amphotericin B for 10 d. Before another myelogram cytomegalo-virus-infected cells were evidenced and treated with ganciclovir (5 mg/kg, twice a day, 21 d). As the oropharyngeal lesions did not improve, liposomal amphotericin B was reintroduced (same therapeutic regimen). The patient evolved with temporary hemodynamic stability and a Glasgow coma scale of 14 and developed respiratory distress and tachypnea. Although being treated with broad-spectrum antibiotics, ganciclovir, and liposomal amphotericin B, the patient progressed with clinical deterioration and died after 75 d of hospitalization. According to the medical chart, the final diagnosis was a ML visceralized due to the presence of numerous comorbidities such as the HIV and the hepatitis C infection.
Serological techniques performed at the end of the patient life were positive (rK39 immunochromatographic test - Kalazar DetectTM -InBios, Inc., Seattle, WA, USA; Indirect Immunofluorescence-IFA -Biomanguinhos® and an in-house ELISA) (). After the analysis of the patient’s medical chart, the following things have caught our attention: the patient’s hometown is a known endemic area for both, CL and VL, as well as, the initially unknown HIV coinfection and the positivity of serological tests (mainly rK39). Therefore, we decided to investigate the etiological agent responsible for these clinical manifestations
This study was approved by the institutional Ethics Committee (CAPPesq) process number 0006/11.
An extremely tiny stored paraffin-embedded larynx biopsy was retrieved from Pathology Laboratory. After DNA extraction it was submitted to 3 conventional PCR assays: kDNA (K20/K22) () and (RV1/RV2) () and ITS1 (LITSR/L5.8S) (, ). Amplifications have followed previously described protocols (, ), with strict measures to minimize the risk of carry-over contamination (, , ). Reference strains, that occurs in Brazil, obtained from cultures, were used as positive controls: L. (L.) chagasi (MHOM/BR/81/M6445), L. (L.) amazonensis (MHOM/BR/1973/M2269), L. (Viannia) guyanensis (MHOM/BR/1975/M4147) and L.(V.) braziliensis (MHOM/BR/75/M2903).
Leishmania genus was defined by kDNAK20/K22 (120 bp) and ITS1 (320bp), and subgenus by RV1/RV2 (145 bp). L. (L.) infantum chagasi specie was identified by the ITS1-RFLP (180, 70 and 50 bp) ().
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pmc-6348217-1
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A 3-yr-old female Flamingo (P. ruber) with a specific wound in left wing was referred by the environmental department of East Azerbaijan Province to the private clinic of Tabriz City Environment Protection Department, in Northwest Iran in September 2016 ().
At the initial examination, clinical signs were extended with a wound upper the left wing.
The wound was infested with the numerous white maggots. The maggots were carefully removed from her wing using sterile forceps and placed in 10% neutral-buffered formalin. The numerous cylindrical vermiform maggot measuring 4-6 mm in length and 3 mm in diameter was observed under the dissecting microscope. The specimen was gently washed in phosphate-buffered saline, pH 7.4, and cleared in graded solutions of glycerol (up to 80%).
According to key diagnostic features for maggots in birds (), the larvae were identified as second and third instars of Calliphora spp. has the posterior spiracles (). The cephaloskeleton was also large and darkly colored ().
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pmc-6348320-1
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A 50 year old, previously healthy man was transferred to the emergency department of Shinshu University Hospital following resuscitation from ventricular fibrillation in September 2013. One morning in early summer of 2008, he woke up to find that he had experienced enuresis. Since then he experienced two to three additional episodes of nocturnal enuresis, which always occurred in the spring or summer. He did not consult with anyone about enuresis. At 0340 hours (h) on the day of admission, he awoke and found the bed was wet. He slept again after he changed his underwear. At 0416 h, his wife noticed that he suddenly groaned and became unresponsive. His wife immediately started basic life support. At 0436 h when emergency medical technicians arrived, the patient was found to be in ventricular fibrillation (). At 0447 h, he was successfully resuscitated after two defibrillations and administration of epinephrine. At 0511 h, following hospital admission, he was unresponsive. Physical examination and laboratory test results were otherwise unremarkable. Coronary angiography showed normal coronary arteries, while an echocardiogram showed no structural heart disease and normal ventricular function. He underwent therapeutic hypothermia and was extubated on the fourth hospital day without neurological deficit.
The electrocardiogram showed sinus rhythm with transient coved-type ST elevation in the V2 lead, which is characteristic of Brugada syndrome type I. On a different day, his electrocardiogram showed saddleback type ST elevation. The patient did not have a history of syncope, seizures, chest discomfort, or nocturnal agonal respiration, nor was there a family history of sudden cardiac death. The diagnosis of Brugada syndrome was made. An implantable cardioverter defibrillator (ICD) was inserted.
When last followed up in July 2016, the patient was in good condition. His bed was dry even during an episode when an ICD shock was delivered to treat ventricular fibrillation during the night-time in May 2015.
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pmc-6348452-1
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A 62-year-old male was referred for 18 months of left forehead numbness, 9 months of horizontal binocular diplopia, and 3 months of left cheek numbness. He had a history of Mohs micrographic surgery (MMS) 9 years prior for a left eyebrow BCC. Though he lacked clinical neuropathies at that time, the BCC was infiltrative, ulcerated, and demonstrated histologic PNI. Therefore, Mohs excision extended into the frontalis muscle to obtain 3-mm tumor-free margins. On examination in our office 9 years later, the patient demonstrated a left cranial nerve VI palsy and hypoesthesia along the left V1 and V2 dermatomes. There were no suspicious skin lesions or lymphadenopathy ().
An extensive workup had been performed over the preceding 12 months prior to referral. Cholesterol, blood pressure, blood glucose, complete blood count, acetylcholine receptor binding and blocking antibodies, erythrocyte sedimentation rate, and C-reactive protein were unremarkable. Serial MRIs over the preceding seven months identified progressive atrophy of the left lateral rectus muscle without abnormality specific to the ophthalmic (V1) or maxillary (V2) branches of the left trigeminal nerve. Review by multiple neuro-radiologists and clinicians suggested that the left superior orbital fissure and left lateral cavernous sinus had either normal appearance or subtle fullness, lacking a clear consensus (). Imaging also revealed chronic opacification of the left sphenoid sinus. Endonasal biopsies of the sphenoid sinus showed chronic fungal sinusitis without invasive disease or necrosis. Cerebrospinal fluid cytology and whole-body PET/CT were negative for malignancy.
PNI was suspected based on the patient's history of an ipsilateral BCC, and the patient underwent biopsy of the left supraorbital (V1) and infraorbital (V2) nerves via superior and inferior orbitotomies. Pathologic examination revealed normal infraorbital nerve tissue and PNI of the supraorbital nerve by an epithelial neoplasm with basaloid morphology (). Immunohistochemistry was consistent with BCC (pancytokeratin+, p63+, Ber-EP4+, EMA-). This pattern suggested retrograde spread of BCC along V1 into the cavernous sinus, followed by anterograde spread into cranial nerves V2 and VI, presumably with V2 involvement isolated to the region proximal of the negative biopsy site.
The patient was initiated on treatment for American Joint Committee on Cancer stage IV BCC, beginning with external beam radiation to the left orbit and cavernous sinus. He was placed on a continual course of vismodegib, a small molecule inhibitor of the hedgehog signaling pathway indicated for advanced basal cell carcinoma. After 6 months of treatment, he unfortunately required temporary cessation of his vismodegib due to adverse effects including weight loss, muscle spasm, loss of taste, and pruritic rash; however, at 10 months of follow-up, repeat magnetic resonance imaging showed no progressive disease. He recovered a small measure of abduction in the left eye, but ultimately required strabismus surgery to achieve orthotropia in primary gaze. His V2 paresthesia evolved into a persistent dysesthesia during treatment, while his V1 paresthesia remained static.
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pmc-6348607-1
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A 37-year-old male presented with masticatory disturbance and aesthetic complaints. His facial profile was straight, and the frontal view was almost symmetrical, with long lower facial height. When the patient smiled, upper incisors could not be seen below the upper lip. He exhibited no phenotypes and medical and family histories about osteogenesis imperfecta and bone diseases.
Molar relationships were Angle Class I on both sides. All erupted teeth showed severe AI without loss of congenital teeth (Fig. ). Anterior open bite of − 10.0 mm was observed between the edges of the upper and lower central incisors. The upper dental midline shifted 2.0 mm to the right relative to the facial midline, and the lower dental midline shifted 0.5 mm to the left relative to the facial midline.
Clinical and radiographic examinations revealed a stump lesion on the patient’s lower right second molar and caries lesions on his upper right first premolar, upper and lower left first, second and third molars.
Cephalometric analysis revealed a skeletal Class I jaw-base relationship (Table ). The mandibular plane and gonial angles were larger than historical values for Japanese control subjects [], indicating a high mandibular plane angle. The maxillary incisors showed an average degree of inclination, but the mandibular incisors were inclined lingually.
The patient was diagnosed as a skeletal open bite with severe AI of all teeth erupted, a skeletal Class I jaw-base relationship, and high mandibular plane angle. The treatment objectives were: (1) to correct the anterior open bite and establish ideal overjet and overbite; (2) to achieve acceptable occlusion with good functional Class I occlusion; and (3) to recover the shape of the collapsed teeth with AI by prosthodontic treatment in order to prevent further wear and sensitivity. The treatment was planned as follows:3.5 mm impaction of the posterior maxillary segments (bilateral second premolar, first and second molars) by compression osteogenesis Minimal extrusion of the anterior teeth to correct severe open bite Establishment of an ideal occlusal relationship through prosthetic restoration.
After the upper and lower left third molars and the lower second molar were extracted, 0.018″-slot pre-adjusted edgewise appliances were placed on both arches. After leveling, corticotomy was performed under local anesthesia with intravenous sedation. Surgery was performed on an outpatient basis (in two stages to avoid bone necrosis) []. In the first stage, corticotomy was initiated at the palatal surface of the first and second upper premolars with a mucoperiosteum incision on the alveolar ridge, 3 mm above the tooth root apices (Fig. ). A fissure half the width of the desired amount was made with a round bar of 4 mm in diameter through the cortical plate of bone surrounding the teeth. The anchor plate was placed onto the center of the hard palate. The second corticotomy at the buccal site was performed 3 weeks after the first corticotomy (Fig. ). The mucoperiosteal flap was abraded beforehand to visualize the area of the corticotomy and to ensure that the procedure was carried out in accordance with previously corticotomized regions. The anchor plates were bilaterally fixed to the zygomatic buttress. Then, elastomeric chains were added to move the corticotomized bone/teeth segments 3.5 mm superiorly. After 1 month, posterior maxillary segments were moved superiorly, which ultimately resulted in correction of the skeletal open bite.
After 8 months of postoperative orthodontic treatment, overbite was improved by − 2.0 mm, and molar relationships were maintained as a Class I relationship (Fig. ). Prosthodontic treatment was initiated to protect dentin and establish stable occlusion. After 5 months of prosthodontic preparation, all edgewise appliances were removed, and provisional crowns were set on all teeth with AI. The total active treatment period was 16 months.
On facial photographs obtained after treatment, anterior lower facial height was reduced, resulting in a balanced facial profile (Fig. ). The anterior open bite was corrected, and the occlusion was much more stable and acceptable, with Class I canine and molar relationships. The overjet and overbite were + 2.0 mm and + 1.5 mm, respectively. When the patient smiled, approximately one-third of the maxillary central incisors were properly exposed. Panoramic radiograph showed that root parallelism was achieved (Fig. ).
Post-treatment cephalometric evaluation showed a skeletal Class I jaw base relationship (ANB 3.6°) (Table , Fig. ). The mandible was rotated 3.0° counter-clockwisely (MP-FH plane 37.3°). The upper first molars were intruded 3.5 mm toward the palatal plane. The maxillary and mandibular central incisors were extruded 3.0 mm and 0.5 mm, respectively. These factors contributed to the maintenance of an acceptable interincisal relationship.
After 8-year retention, the occlusion was stable, and a good facial profile was maintained (Fig. ). Panoramic radiograph revealed no or less changes in alveolar bone level and root parallelism (Fig. ). Cephalometric analysis showed only minor changes in maxillary and mandibular position, which did not result in relapse (Fig. ).
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pmc-6348653-1
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A 30-year-old African-American male presented to his primary care physician with a chief complaint of a several-year history of unhealing wounds on the right side of his penile shaft after his penis was caught in his zipper several years ago. Patient became concerned after noting white penile discharge 2 weeks prior. He denies any anal or oral lesions as well as exposure to any sexually transmitted diseases (STDs). Following outpatient specialty referral, the dermatologist reported excess skin tissue with underlying edema circumferentially on the distal penile shaft with overlying multiple firm skin-colored papules, some with exophytic crusting [Fig. ]. Chlamydia trachomatis, human immunodeficiency virus (HIV), Neisseria gonorrhea and syphilis were negative. Subsequent biopsy found dilated vascular channels consistent with benign acquired lymphangioma of the penis (Fig. ), and the patient was referred to urology for evaluation and management. With the urologist, the patient elected for surgical intervention due to cosmetic concerns despite the asymptomatic nature of the lymphangioma.
The patient underwent circumcision for redundant prepuce, excision of the skin lesion and penile foreskin reconstruction. A circumferential incision was made on the mucosa 0.5 cm proximal to the glans, distal to the lymphangioma. The foreskin was then retracted and another circumferential incision was made around the mucosal skin. The foreskin was then dissected using Bovie cautery and blunt dissection while the foreskin with lymphangioma tissue was excised. Intraoperative and postoperative courses were unremarkable. At 1-month follow-up, the patient reported no pain, erythema or discharge from the wound.
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pmc-6348665-1
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A 51-year-old male painter with a 40 pack/years history of cigarette smoking and a diagnosis of chronic respiratory failure due to post ischemic cervical myelopathy was trained in bi-level PAP which he used up to 16 h per day since 2003 at 25 inspiratory cm H2O (IPAP) and 7 cm H2O expiratory (EPAP) pressures and rate 15/min. However, during the last year he experienced daytime dyspnoea, tachypnoea, orthopnea, and deterioration of gas exchange when not using it and complained of the interface causing discomfort and interfering with his daytime employment. His arterial blood gases (ABG) breathing unassisted in ambient air 4 h after discontinuing nocturnal bi-level PAP was PaO2 62 and PaCO2 58 mmHg. On admission, he was placed on IAPV ventilation. He wore the IAPV corset’s horizontal upper border two fingers below the costophrenic junction.
His spontaneous tidal volumes of 172–180 ml increased to 771–908 ml using the IAPV with the LUNA ventilator set at 24 cm H2O pressure, rate 15/min (Table ). Arterial blood gases were monitored after the second hour of IAPV use. Table demonstrates normalization of the diurnal breathing pattern and gas exchange. After 3 months his PaO2 breathing unassisted in ambient air was 75 and PaCO2 44 mmHg (Table ).
Quality of life parameters were measured and at discharge thanks to the EuroQoL (EQ-5D) [] and the World Health Organization Quality of Life Questionnaire (WHOQOL-Bref) [], the patient used the IAPV 8 h/day with improved mood (assessed by the Hospital Anxiety and Depression Scale (HADS) []) and cognition (as assessed by the Mini Mental Status Examination [] and the Addenbrooke’s Cognitive Examination Revised (ACE-R) [] (Table ). Moreover, three months later he reported that the IAPV was still effectively relieving his former daytime dyspnoea but that he had achieved up to 6 h/d of autonomous breathing without dyspnoea or tachypnea.
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pmc-6348674-1
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A 49-year-old Thai woman with relapsed/refractory ITP was diagnosed in December 2016 with petechiae on her legs. She was a shop owner in Nonthaburi Province, Thailand. Her complete blood count (CBC) showed hemoglobin of 13 g/dl, a white blood cell count of 7 × 109/L, and a platelet count of 4 × 109/L. The results of her urinalysis and renal and liver function tests were normal. The results of all of her other blood tests (including viral hepatitis, anti-human immunodeficiency virus, and antiphospholipid profiles) were negative. She also had underlying diseases of poorly controlled diabetes mellitus type 2, hypertension, and hyperthyroidism. She denied having any other medical illness or a history of surgery. Her first-degree family members were healthy and had no history of hematological disorders. She had no history of smoking or alcohol consumption. Her current medications were losartan 100 mg/day, metformin 2000 mg/day, glipizide 20 mg/day, pioglitazone 30 mg/day, atorvastatin 40 mg/day, and methimazole 5 mg/day.
Her platelet count responded well to the normal range with oral prednisolone, and the prednisolone was tapered in January 2017. The first relapse episode happened in August 2017. She presented with bleeding from the gums, and treatment was reinitiated with steroids. Once her CBC was normal, the treatment was gradually tapered. The last event occurred in October 2017, when her platelet count dropped to 36 × 109/L without clinical bleeding. After treatment with high-dose prednisolone for 1 month, her platelet count recovered to the normal range. Although the prednisolone dosage was decreased gradually by 10 mg per week, she could not maintain her platelet count with prednisolone 0.5 mg/kg/day. She was therefore treated with 50 mg/day of azathioprine and 200 mg/day of danazol, combined with a high-dose prednisolone, to increase her platelet count.
In January 2018, she came to our hospital with a large hematoma on her right buttock. Her initial vital signs showed a temperature of 37.2 °C, pulse rate of 87/minute, respiratory rate of 14/minute, and blood pressure of 125/82 mmHg. The results of her physical examinations (cardiovascular, respiratory, gastrointestinal, and neurological) were normal, except for the presence of a large hematoma about 10 cm in diameter on her right buttock. Her CBC showed hemoglobin of 11.5 g/dl, a white blood cell count of 10.4 × 109/L, and a decreased platelet count of 3 × 109/L. Other initial laboratory findings (including a renal function test, liver function test, and urinalysis) were found to be within normal limits. She was admitted to the hospital for IVIg administration. The timelines of her treatments and her platelet counts are illustrated in Fig. . A bone marrow study was performed, which revealed an increase in the number of megakaryocytes, compatible with peripheral destruction (Fig. ). Intravenous dexamethasone (40 mg/day) and IVIg 60 g/day (1 g/kg/day) were initiated. The infusion rate of IVIg was 40 ml/hour for 1 hour and then 60 ml/hour. The patient was also given premedication (4 mg of intravenous chlorpheniramine).
One hour after completion of the IVIg infusion, the patient’s sister complained that the patient was unconscious and had not been able to move both legs and arms. A neurological examination showed a Glasgow Coma Scale score of E3V3M5 and motor power of grade 2 on both sides; both pupils were 5 mm and semireactive to lights. Emergency computed tomography (CT) of the brain showed no abnormal findings, such as brain edema, intracranial hemorrhage, or infarction. One day later, repeat CT of the brain displayed extensive acute ischemic changes and loss of gray-white differentiation of bilateral cerebral hemispheres (Fig. ). The patient’s consciousness was deteriorating. The decision was made to forgo intubation at the request of the family and in accordance with the patient’s advance care directive. Consequently, her blood pressure dropped rapidly, and she died within a few hours. Her family members declined an autopsy. A timeline of the long-term treatment of the patient is provided in Additional file .
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pmc-6348684-1
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A 65 year old woman was referred for a second respiratory opinion for persistent MRC grade 4 dyspnoea on a background of chronic obstructive pulmonary disease (COPD). Relevant past history included 33 pack years smoking history with smoking cessation 12 months prior, childhood history of mild asthma, and mild diastolic dysfunction. She required two hospital admissions for exacerbations in the past 12 months, in addition to multiple courses of oral corticosteroids. She had been adherent to her medications, which included total daily doses of budesonide/eformoterol 800/24mcg, ciclesonide 320mcg, aclidinium 322mcg, theophylline slow release 600 mg, and doxycycline 50 mg. In addition, she nebulised salbutamol 5 mg each morning, and took 6-8 additional puffs of salbutamol during the day.
On examination, her body mass index was 25, with normal vital signs and oxygen saturation 95% breathing room air. There was no finger clubbing. The chest was hyperinflated and there were no adventitial sounds.
Spirometry revealed severe airflow obstruction with a forced expiratory ratio of 43% and forced expiratory volume in 1 s (FEV1) of 47% predicted (0.86 L), with a partial bronchodilator response (130 ml and 15.6%). Gas trapping was evident with an elevated residual volume (RV) of 189% predicted, and a Residual Volume to Total Lung Capacity ratio of 55%. The single breath Diffusing Capacity for Carbon Monoxide was measured at 8.2 ml/mmHg/l or 45% predicted. Arterial blood gases were not measured as SpO2 was greater than 90%, and serum standard bicarbonate was 26 mmol/L. The fractional exhaled nitric oxide was 25 ppb. The blood eosinophil count was 200 cells/μl and the IgE was 164 IU [0-200], with elevated serum specific IgE to Aspergillus fumigatus, and non-reactive results to other allergens including grasses and dust mite. The haemoglobin level was 145 g/l. Computed tomography pulmonary angiogram (CTPA) did not detect pulmonary emboli, and the lungs were noted to be hyperinflated, but without marked emphysematous changes.
The patient was referred for pulmonary rehabilitation, which led to modest improvement, though she remained limited by exertional dyspnoea. At re-evaluation, consideration was given to whether the patient could benefit from an interventional approach such as endobronchial lung volume reduction surgery. A Quantitative CT for emphysema distribution and fissure integrity was requested. This demonstrated a relatively small lung fraction with <− 950 Hounsfield units (9.35%), although more prominent changes were observed in the left lower lobe. As this result did not unequivocally support a diagnosis of COPD, we undertook flexible bronchoscopy to obtain endobronchial biopsies from the left lower lobe. The 2.8 mm channel bronchoscope, Olympus BF-ITH190 (Olympus Australia, Victoria, Australia) was used with the 2.3 mm forceps, and four biopsies up to 5 mm in size were obtained from the subsegmental carina at LB8/9. Histopathology revealed very marked smooth muscle hypertrophy (Fig. ) and significant thickening of the basement membrane typical of asthma. Additionally, squamous metaplasia due to cigarette smoking was evident.
As a direct result of the endobronchial biopsy, the treatment approach shifted to advanced therapies for severe asthma. The patient did not meet Australian funding criteria for anti-IgE or anti-interleukin 5 monoclonal antibodies, but she was however a suitable candidate for BT. The patient was treated over three sessions without complication and in keeping with the standard technique [].
Six months after BT, the Asthma Control Questionnaire score had reduced from a baseline value of 3.0 to 1.6, where a score of 1.5 indicates well controlled asthma, and a change of 0.5 is regarded as clinically significant. The daily salbutamol use had decreased substantively to 0.5puffs/day. There had been no instances of exacerbations requiring antibiotics or corticosteroids. The prebronchodilator FEV1 improved slightly from 47 to 52% predicted and the RV improved markedly from 189 to 152% predicted.
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pmc-6348794-1
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Case 1 was a 66-year-old Japanese woman who had presented at another hospital with acute abdominal pain which she experienced when she took a bath before she was rushed to the hospital. Her vital signs indicated shock (systolic blood pressure was 70 mmHg), and her CT scan showed intraperitoneal bleeding. She was transferred to our hospital. She had no notable medical history. On admission, her body temperature was 37.4°C and her pulse was 118 bpm/min.
After fluid resuscitation, the patient's blood pressure was 129/94. Laboratory findings showed slight leukocytosis (15,600/μl) and anemia (9.9 g/dl). A CT scan revealed a great volume of ascites and an aneurysm (). The angioarchitectonic examination showed the aneurysm of the inferior pancreaticoduodenal artery (IPDA) and stricture of a root of the celiac artery (). We then performed angiography (), which also showed the aneurysm of the IPDA. We attempted coil embolization, but because of the difficulty inserting the catheter, we aborted the embolization and decided to perform surgery instead. We had no prior experience with MAL syndrome, and in light of the emergency, we chose an abdominal operation.
We conducted an abdominal median section and could see the intra-abdominal hemorrhage. Excessive bleeding was observed in the retroperitoneum around the duodenum, pancreas, and transverse colon. The IPDA, which is the vascular arcade of the inferior margin of the pancreas, had a 10 mm aneurysm. We confirmed the aneurysm's existence by perioperative sonography, ligated the feeder, and removed the aneurysm.
For the prevention of a rerupture of the aneurysm, we attempted to resect the MAL. After taping the left gastric artery, we observed that the celiac artery was covered by the MAL. We cut the MAL away little by little and confirmed the increase of the beating of the left gastric artery. After placing drainage tubes in the left subphrenic area and the inferior side of pancreas, we closed the wound of the abdominal incision. The operation time was 4 hours 27 minutes, and the blood loss was 1800 ml. The patient began to take hospital meals on postoperative day 8. The postoperative course was uneventful, and she was discharged on the 22nd hospital day. CT scans have shown no recurrence of the aneurysm for 3 years.
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pmc-6348794-2
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Case 2 was a 75-year-old Japanese man who presented at our hospital with acute abdominal pain, nausea, and cold sweat. His CT scan showed retroperitoneal bleeding (around the pancreas and the dorsal side of the ascending colon). His general condition was stable, but he was admitted to our hospital as a conservative measure. His angiography (6 days after admission) showed an aneurysm of the pancreaticoduodenal artery without active bleeding.
Our experience treating MAL syndrome in case 1 enabled us to diagnose the disease accurately in case 2. MAL syndrome was the cause of the aneurysm in this patient too (). We selected laparoscopic surgery based on the MAL syndrome and the benefits of this surgery. The patient's posture for the surgery was the lithotomy position. Intra-abdominal pressure of 12 mmHg was maintained. The points of the trocars were as follows: a 12 mm trocar at the navel for the camera, two 12 mm trocars at the right upper abdomen, and a 12 mm trocar and a 5 mm trocar at the left upper abdomen ().
First, we lifted the liver umbilical ligament by surgical sutures and put in an organ retractor to the crus of the diaphragm in order to improve the field of vision. After opening the omental bursa, we lifted the stomach with a snake retractor and observed the dorsal side of the stomach. We confirmed the left gastric artery and tied it with tape. The tape was taken out from the right outside trocar, and an assistant pulled it to provide traction of the surgical field. Following the celiac artery to the root, the artery was fastened by the MAL. We cut the MAL away little by little with a vessel-sealing system until the running direction of the celiac artery was clearly confirmed. Using a blood flow meter, we confirmed the improvement of blood flow of the left gastric artery (from 5 mm/min to 69 mm/min). A drainage tube was placed in the left subphrenic area, and the wound of the abdominal incision was closed. The operation time was 3 hours 35 minutes, and the blood loss was minimal at 15 ml. The patient's CT scans have shown no recurrence of the aneurysm for 2 years.
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pmc-6348808-1
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The patient was a 10-year-old girl referred to our genetic department because of developmental delay, microcephaly, epilepsy, severe short stature, impaired speech, facial dysmorphism, congenital deafness, and skeletal abnormalities. She was the second of two born children of healthy nonconsanguineous parents and was born at 36 weeks due to intrauterine growth restriction and oligohydramnios, with a birth weight of 1,800 g and length of 42 cm (both bellow 2SD). She presented her first epileptic crisis at five months of age; it was characterized by generalized tonic-clonic seizures accompanied by eye deviation. By the age of 8 months, absent seizures began to present, and the generalized tonic-clonic seizures disappeared, although she has had careful follow-up by the neurologist, the pharmacological control of absence seizures has been partial. Her development has been severely delayed. She could sit at the age of 7 months; no crawling, independent standing was achieved at 30 months of age, and until the present, she persists with severe language development delay since she only recognizes names of few ordinary objects without having a fluent language. Facial dysmorphism was noted at one year of age; it consisted of a triangular face, blepharophimosis, telecanthus, epicanthus, palpebral ptosis, sparse eyebrows, low-set ears, flat malar region, thin upper lip vermillion, and down-turned corners of the mouth. At one year of age, she was diagnosed with bilateral hip dysplasia, conductive left hearing loss, and right mixed hearing loss. Currently, at the age of 10, she persists with the same facial dysmorphisms, severe short stature (110 cm -2SD), microcephaly (OFC 46 cm –2SD), small hands, short thumbs, bilateral fifth finger clinodactyly, severe cognitive impairment, absent of fluent speech, easy distractibility, and inadequate attention. shows the phenotypic findings described in this case.
Sleep electroencephalogram shows only nonrapid eye movement (NREM) sleep associated with epileptogenic activity characterized by generalized high-voltage biphasic waves followed by slow and sharp waves (spikes) with right-sided predominance. She has a normal cerebral MRI, screening for inborn errors of metabolism was negative, and high-resolution conventional karyotype was normal 46, XX. According to the genome-wide array, CGH (Agilent Human Genome CGH Microarray 44K), a minimal/maximal deletion of 5.238 Mb and 5.374Mb, respectively, at 8q22.2q22.3 was diagnosed. The region contains 33 genes in a minimal interval of 99,509,806 to 104,747,354 and maximal interval of 99,509,429 to 104,883,122 (based on the March 2006 Human Genome Sequence Assembly [hg18]). The deletion was excluded in the mother's blood sample; the father was not available for investigation.
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pmc-6348809-1
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A 65-year-old male known to have chronic viral hepatitis C presented to accident and emergency department with 1-year history of shortness of breath and blue discoloration of his fingers.
His shortness of breath worsened with the time; it was exertional initially, but recently it was noticed even at rest. It was aggravated by walking and setting, but relieved by lying flat and oxygen therapy. It limited his activities of daily living, sexual intercourse, and self-care (modified medical research council MMRC grade = 4). In addition, he reported that his shortness of breath was associated with generalized fatigue, blue discoloration of his hands, feet, mouth, and nose, and bilateral hand tremor that occurred mainly during exercises. He also had intermittent generalized nonradiating, moderately severe pressure like headache but no sensory or motor symptoms were noticed. Furthermore, this shortness of breath was not associated with chest pain, cough, sputum production, palpitation, or loss of consciousness. There was no leg swelling, abdominal distension, abdominal pain, vomiting, jaundice, or change in bowel or urine habits.
Our patient was known to have chronic hepatitis C infection, which was not treated because of nonadherence issues. His social history revealed that he smoked cigarettes and consumed alcohol for more than forty years but quitted one year back. He also stopped using illicit drugs and shared needles after more than thirty years of consumption.
On physical examination he had peripheral and central cyanosis (), grade 4 clubbing (), muscle wasting, needle marks, palmar erythema, and bilateral resting tremor but no asterixis. The patient had scattered telangiectasia over his body. The abdominal, cardiovascular, respiratory, and neurological examinations were normal.
Initial laboratory investigations demonstrated a low platelet count 104 × 109 per L (reference range (RR) 150 –400 × 109/L), normal white and red cell counts, and normal haemoglobin level. In addition, his coagulation profile, renal function, and electrolytes were almost within normal limits. Liver function test revealed elevated total bilirubin 34 μmol/L (RR 5-21 μmol/L) and direct bilirubin 17 μmol/L (RR 0-5 μmol/L). Total protein 82 g/L including albumin (39 g/L), globulin 39 g/L (RR 15-30 g/L), G-Glutamyl transferase, and alkaline phosphatase was within normal ranges. Alanine transaminase level was 108 U/L (RR < 41 U/L) and his alpha fetoprotein was 13.3 μg/L (RR < 9 μg /L). Electrocardiogram was normal, but chest X-ray showed bibasal nodular opacities. Hepatitis profile revealed a positive anti-HCV Antibodies, HCV genotype 3, and HCV viral load of 251188.640 IU/mL.
At this point, pneumonia and hepatic hydrothorax were extremely less likely based on the mentioned clinical and basic laboratory investigations.
Arterial blood gases (ABG) showed severe hypoxemia with improvement on lying down as follows: While setting, ABG readings were PH= 7.43, SO2= 57%, PO2=33.5 mmHg, PCO2= 31.9 mmHg, and HCO3=21 mmol/L. However, while lying down, his ABG results were PH= 7.438, SO2= 71.6%, PO2= 37.6 mmHg, PCO2= 29.3 mmHg, and HCO3= 21 mmol/L). A-a gradient increased in supine position from 76 mmHg to 78 mmHg. Abdominal Ultrasound showed liver surface nodularity and an incidental renal cyst. Gastroscopy confirmed the prescience of oesophageal varices hepatic gastropathy. As a result, his Child-Pugh score was 5 points (Class A) and MELD score (9 points)
His pulmonary function test showed normal forced expiratory volume to forced vital capacity ratio (FEV1/FVC) but reduced FEV1 and FVC. Chest computed tomography scan showed bibasal prominent pulmonary arterial dilatation and irregular borders of the liver, but no emphysematous or fibrotic changes. Contrast-enhanced transthoracic echocardiography was performed. Within 5 heart cycles from appearing in the right atrium, contrasts' microbubbles appeared in the left atrium and left ventricle (Figures and ).
Based on the history, physical examination, and laboratory investigations, our patient was diagnosed with hepatopulmonary syndrome ()
We treated him for his underling liver disease with Sofosbuvir and Daclatasvir, and for HPS he was managed symptomatically with long term oxygen therapy (LTOT). Subsequently, he was closely followed up in the outpatient department by hepatologists and pulmonologists every 3 months, advised to receive vaccination for hepatitis A and hepatitis B, and assessed for liver transplant. After the completion of his medical therapy, the laboratory results confirmed the resolution of hepatitis C infection.
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pmc-6348813-1
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A 66-year-old female with a history of mucinous adenocarcinoma of the cervix presented to the neurosurgery outpatient clinic for evaluation of a sellar mass found during workup of adrenal insufficiency and hypothyroidism. The patient did not have unusual headaches or vision problems. Three months prior to discovery of the sellar mass, she was diagnosed with stage IIb mucinous adenocarcinoma of the cervix and was treated with chemotherapy. At the time of neurosurgery clinic presentation, she was neurologically intact, including full visual fields. Laboratory work-up demonstrated pituitary insufficiency with central hypothyroidism.
The initial magnetic resonance imaging (MRI) revealed a 1.8 × 1.1 cm contrast-enhancing mass within the sella, with extension to the suprasellar cistern and optic chiasm abutment. Preoperative imaging obtained the following month in preparation for surgery demonstrated that the mass had grown to 2.2 × 1.5 cm ().
The patient underwent an endoscopic endonasal approach for resection of the intradural sellar mass. Intraoperative findings demonstrated a very firm, infiltrative, vascular mass with dense adherence to surrounding structures, including the dura, medical cavernous walls, and diaphragma. Intraoperative frozen section pathology was consistent with metastatic carcinoma. The tumor was debulked until normal appearing pituitary tissue was identified and the margin of tumor adherence to the diaphragma was reached. Postoperatively, the patient did well without new hormonal deficiencies or vision problems. A subtotal resection (>80%) was achieved (). Gross histology and immunohistochemical staining ultimately confirmed the diagnosis of metastatic mucinous adenocarcinoma of the cervix (Figures and ).
The patient's immediate postoperative course was unremarkable. Given the diagnosis and intraoperative/postoperative findings of subtotal resection, adjuvant chemoradiation therapy was encouraged but the patient refused additional treatment. She was discharged home two days after surgery. She developed decreased left eye visual acuity and ptosis one week after surgery. A CT of the head at this time did not show any intracranial hemorrhage and a repeat MRI showed new enhancement suggestive of tumor recurrence within the sellar and suprasellar regions. The patient was started on steroids but declined any further treatment, including repeat surgery. Her ophthalmic symptoms ultimately progressed to a complete left cranial nerve III palsy four weeks after surgery. Follow-up MR imaging at five weeks after surgery revealed significant progression of the tumor to 2.9 × 2.4 cm with significant suprasellar extension (). Though a computed tomography scan of the chest, abdomen, and pelvis at this time demonstrated no new neoplastic burden, a radiotracer bone scan demonstrated likely new metastatic lesions in the skull, bilateral humeri, bilateral acetabula, bilateral femurs, and the lumbosacral vertebrae. After further discussion with her gynecologic oncologist and radiation oncology, the patient again refused pursuing any further treatment, including palliative radiation or systemic therapies and elected to pursue home hospice. The patient died approximately two months after surgery.
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pmc-6348819-1
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A 35-year-old woman, impregnated via intracytoplasmic sperm injection (ICSI), visited our hospital at 9 weeks of gestation. She had a history of one pregnancy with a normal delivery. The patient also had a history of asthma and no history of blood cell transfusion or medication except for the use of the antibiotic cephem during ICSI to prevent infection. A blood test administered at her first visit revealed that she was D-antigen-positive and irregular antibody-negative and her hemoglobin concentration was 14.4 g/dl.
At 28 weeks of gestation, a blood test revealed acute macrocytic anemia (hemoglobin concentration, 7.9 g/dl; mean corpuscular volume, 108.1 fl; and mean corpuscular hemoglobin, 35.3 pg; ). A detailed examination was performed to determine the reason for these results (). Hemolysis, elevated liver enzymes, low platelet count (HELLP) syndrome; hemolytic uremic syndrome (HUS); and thrombotic thrombocytopenic purpura (TTP) were unlikely. Systemic lupus erythematosus is reported as a disease that causes anemia [] but was also unlikely because a test for anti-nuclear antibody was negative. Her C3, C4, and erythrocyte-binding IgG, IgA, and IgM levels were also normal. We then suspected the presence of hemolytic anemia and performed several additional examinations.
As shown in , an increase of reticulocyte and lactate dehydrogenase (LDH) and a marked decrease of haptoglobin (<10 mg/dl) were found. We further examined the LDH fractions and found that LDH1 and LDH2 were markedly increased. Her urine was negative for hemoglobin. These results strongly suggested the presence of hemolysis. In addition, the direct antiglobulin test (DAT) was positive for anti-IgG and negative for anti-C3d. The indirect antiglobulin test was negative. There was no corresponding medical history or symptoms of infection that could have contributed to the observed hemolytic anemia. A blood test for cold agglutinins was negative. Hill et al. have reported that they can diagnose AIHA when there is evidence of hemolytic anemia, the DAT is positive for IgG, and there is no evidence of an alternative cause of hemolytic anemia when the DAT is positive []. Accordingly, warm AIHA was diagnosed as the cause of anemia in this case.
Maternal blood was regularly tested. We had started iron preparation from onset of anemia empirically, and her hemoglobin level recovered to 10.1 g/dl by 31 weeks of gestation (). Although her iron level in the blood was normal, we assumed that iron deficiency might have coexisted and kept iron supplementation. The DAT remained positive at 30 and 34 weeks of gestation. Fetal estimated weight and middle cerebral artery peak systolic velocity (MCA-PSV) were assessed every 2 weeks via ultrasound examination to monitor effects of the anemia (Figures and ), and these factors remained in the normal range.
Labor started spontaneously at 40+1 weeks of gestation, and a normal female newborn was delivered. Her Apgar score was 9/9 (1/5 min), and her body weight was 3575 g. The total bleeding amount was 330 g, and the duration of labor was 380 minutes. No notable event occurred during delivery or the postpartum period. On the days following delivery, the patient's hemoglobin concentration was 10.7 g/dl. The neonatal hemoglobin concentration was 13.6 g/dl. At 2 days of age, the newborn was treated with 24-hour phototherapy because of neonatal jaundice. Both the mother and neonate were discharged on postdelivery day 5.
After discharge, the patient's DAT and hemoglobin concentrations were regularly assessed on an outpatient basis. Her DAT remained positive at 32, 95, and 203 days after delivery. Her hemoglobin level and blood platelet count were normal at 100 days after delivery. From approximately 150 days after delivery, the patient frequently observed nose bleeding and subcutaneous hemorrhage. A blood test at 203 days revealed an extremely low platelet count at 8000/μl (). The patient was admitted to the Department of Hematology, and bone marrow aspiration was performed. The form of megakaryocytes was normal, and no malignant cells were detected. A diagnosis of ITP was made. Because it occurred after the development of AIHA, Evans syndrome was considered.
Treatment with corticosteroids was initiated (3 days of methylprednisolone 500 mg) on the day after hospitalization, and the patient's platelet count recovered to 88,000/μl. Notably, after completion of the corticosteroids treatment, the platelet count decreased again, and oral administration of prednisolone 60 mg was initiated. Progress was satisfactory, and the prednisolone dosage was gradually decreased to 0 mg ().
At 1 year after completing corticosteroid treatment, hemoglobin and platelet counts remained in the normal range.
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pmc-6348844-1
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Patient is a 70-year-old female with a past medical history of diabetes mellitus, hyperlipidemia, fibromyalgia, rheumatoid arthritis, and depression, who was referred to our institution's gastroenterology clinic for dysphagia to solids for 1 month. This was accompanied by 2-3 episodes of vomiting daily and a 30-pound weight loss. Due to her alarm symptoms an upper endoscopy was scheduled. The upper endoscopy revealed luminal narrowing in the lower 1/3 of the esophagus without any discernable esophageal web or ring. During the procedure, intubation of the stomach was difficult, but showed nonbleeding erosive antral gastropathy. On follow-up appointment 6 days later, the patient reported progression of symptoms, now complaining of dysphagia to liquids. The patient was then referred to the emergency department due to inability to tolerate oral intake. On admission workup included a barium swallow showing abrupt tapering of the gastroesophageal junction with a bird beak configuration consistent with achalasia (). These radiologic findings, coupled with her symptoms, raised our suspicion of intrinsic achalasia as the culprit. The patient was then taken to the endoscopy suite to undergo palliative treatment with a botulinum injection to the lower esophageal sphincter. One day later, however, the patient's symptoms showed no improvement. Given her lack of clinical improvement, the differential diagnosis now included pseudoachalasia as a possible cause. A CT scan of the chest and abdomen was then done to rule out extrinsic compression of the esophagus. This showed a 12 x 12 soft tissue mass in the gastrohepatic omentum compressing the distal esophagus and gastric fundus (Figures and ). Due to the size of the mass and the small sample size that would have been obtained with FNA, EUS was not done. Instead a CT guided biopsy was done, the results of which showed a classic “starry sky” appearance consistent with Burkitt's lymphoma (). After tissue diagnosis, the patient was transferred to an outside institution to undergo chemotherapy. During her course at our institution, her nutritional needs were met through total parenteral nutrition. 3 months later she presented to our gastroenterology clinic for follow-up with complete resolution of symptoms. Repeat barium swallow was done showing resolution of the previously seen birds beak appearance with complete esophagogastric emptying ().
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pmc-6348857-1
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A 23-year-old male who suffered from acroparesthesias, decreased sweating, exercise intolerance, and frequent episodes of diarrhea and abdominal discomfort was diagnosed with FD. Physical examination: angiokeratomas on palms and genitals were found (). The αGalA test in dried blood spot disclosed decreased enzyme activity, 0.1 nmol/hour/liter (normal > than 4 nmol/hour/liter). A mutation [c.317T>G (p.L106R)] was identified in the GLA gene, by sequential analysis. The laboratory results were unremarkable, with a GFR of 104.4 mL/min/m2 estimated by the CKD-EPI equation, 24-hour urinary albumin excretion 6.00 mg/day. A renal ultrasound and DOPPLER echocardiogram were normal. A plasma Lyso-Gb3 value of 124.5 nmol/L was found, determined by tandem mass spectrometry method. To detect the relative excretion urinary levels of miR-21, miR-29, miR-192, miR-200, and miR-433, reverse transcription reaction with a stem-loop primer was used. The resulting cDNA was amplified using a miRNA-specific forward primer and the universal reverse primer []. Relative miRNAs expression levels were calculated using the 2-ΔΔCt method as previously described [] ().
After the FD diagnostic confirmation enzyme replacement therapy with agalsidase-beta at a dose of 1 mg/Kg/every other week was indicated.
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pmc-6348877-1
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The patient is a 25-year-old Caucasian female who presented to the emergency department after a witnessed event described as a period of rapid eye blinking and unresponsiveness. Her episode ceased after 10 minutes and she complained of numbness and weakness. According to her parents, this was the first event of this description and she was “talking slowly” after the event.
She described incontinence, clumsiness, and difficulty “regulating her body temperature” since middle school. Her past medical history included loss of consciousness after head trauma at age 14 and neuropathic pain in her left arm since a motor vehicle accident at age 20. Later that year, she felt like she “was burning up inside” and collapsed causing a basilar skull fracture. She stated that, prior to losing consciousness, she felt extremely hot but denied diaphoresis. She has had headaches and several similar “burning” episodes without trauma since that time. She then developed dysphagia and dysarthria prompting referrals to ENT, neurology, and psychiatry, leading to diagnoses of anxiety, depression, and sleep apnea. Despite treatment for these diseases, she continued to have episodic hypothermia, weakness, dysphagia, dysarthria, and spasticity. She later went to a traumatic brain injury clinic where she was treated with hyperbaric oxygen and physical therapy. She felt that she had some improvement in that time. At the time of admission, she had worsening symptoms in addition to urinary incontinence, coughing, and spells of unresponsiveness. Social history included avoidance of western medicine and use of several herbal supplements and essential oils daily for health, including colloidal silver. Her family and surgical histories were not significant.
Vital signs on admission included a temperature of 98.7°F, 18 respirations per minute, heart rate of 50 beats per minute, blood pressure of 89/52 mmHg, and oxygen saturation of 96%. Other significant physical exam findings included HEENT exam within normal limits and neurological exam with central lower extremity weakness (3/5) and central upper extremity weakness (4/5). There were bilateral hyperreflexia in the lower extremities and some rigidity with passive movement in the RLE.
Initial laboratory testing was significant for pancytopenia (). Due to the presenting complaint of seizure, an EEG and MRI were also performed. Initial and extended electroencephalography (EEG) tests were positive for diffuse slowing, indicative of the moderate to severe encephalopathic state of nonspecific etiology. Brain MRI was notable for the diffuse symmetric high T2/FLAIR signal within and along the ependymal lining, involving the subependymal periventricular tissue, raising the possibility of ventriculitis. Other abnormalities included marked wasting and narrowing of upper cervical spinal cord and medullary atrophy as well as increased signal in the pons and cerebellar peduncles. A lumbar puncture was then performed to rule out infection and toxins, but cerebral spinal fluid showed normal cell counts and was negative for herpes simplex virus (HSV), enterovirus, VDRL, cryptococcal antigen, and West Nile virus. Hematologic abnormalities were assessed and ruled out by bone marrow biopsy and flow cytometry.
Lab values, including vitamin levels, serologic studies, thyroid studies, adrenal function, general toxicology screens, and most heavy metal concentrations, were normal. Her blood silver level was elevated to 9.6 mcg/dL (normal is < 5 mcg/dL). This was initially thought to be the cause of the patient's pancytopenia and abnormal MRI findings. Treatment involved maintaining appropriate blood glucose and temperature, initiating a low residue diet and antibiotics, and requesting the family to stop colloidal silver treatment. Her blood counts reached a nadir and recovered during her admission.
Though the pancytopenia resolved with discontinuation of oral silver supplementation, the patient continued to have dysautonomia, dysarthria, and ataxia after discharge. This prompted an investigation into the alternative etiologies that matched her symptoms—specifically the dysautonomia, dysarthria, and ataxia—and unique MRI findings. AxD has been known to cause these symptoms and was consistent with her imaging findings. She was followed up in our resident continuity clinic as an outpatient, where a genetic test for AxD was ordered revealing a missense variation in the glial fibrillation acidic protein (GFAP) gene of p.Lys260Gln (c.778 A > C in exon 4 of the GFAP gene; K260Q). This was classified as a “likely pathologic variant”; in silico analyses including evolutionary conservation and protein predictors supported a deleterious effect. Her parents were also tested revealing the same mutation in her mother. On further discussion with the mother, she reported decreased balance and coordination that were out of proportion to her age and “difficulty regulating her temperature.” No objective data exist for these complaints, and she has not yet had an MRI performed. There are no other family members with these complaints, although the parents say the patient's younger sister is beginning to show symptoms of dysautonomia. No one else in the family has been officially diagnosed with this disease. She and her family were referred to a local university-affiliated genetic disease clinic for assistance with management.
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pmc-6348878-1
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A 56-year-old male presented with a 3-day history of altered mental status and weakness. His past medical history was significant for long-standing IVDU, chronic purulent cellulitis of bilateral lower extremities, osteomyelitis of bilateral tibiae, latent tuberculosis treated eleven years prior to presentation, and previously treated hepatitis C infection. The current hospitalization was his second within eight months, as he was previously hospitalized for methicillin-sensitive Staphylococcus aureus (MSSA) bacteremia due to cellulitis and osteomyelitis attributed to extensive ongoing intravenous drug injections through lower extremity veins. A transthoracic echocardiogram (TTE) performed during that hospitalization was negative for endocarditis.
During the current admission, the patient's Glasgow coma scale was 13 on presentation. Physical examination was limited by the patient's inability to cooperate, but the patient was noted to have left lower quadrant abdominal tenderness, bilateral lower extremity and right upper extremity wounds, and a large tender sacrocoxal erythematous ulcerated lesion. Presenting vital signs included a blood pressure of 140/79 mm Hg, temperature of 36.4 Celsius, heart rate of 114 beats per minute, respiratory rate of 28 breaths per minute, and oxygen saturation of 95% on 3 liters of supplemental oxygen. Laboratory studies were concerning for leukocytosis of 25.6 K/μL (4.5–11.0 K/μL), hemoglobin of 5.7 gm/dL (13.5–16.5 gm/dL), and platelet count of 129 K/μL (150–400 K/μL). Iron studies were suggestive of anemia of chronic inflammation. Other laboratory abnormalities included serum creatinine of 1.25 mg/dL (0.4–1.24 mg/dL), serum sodium of 127 mmol/L (137–147 mmol/L), and albumin of 2.0 g/dL (3.5–5.0 g/dL). Creatine kinase was 1288 U/L (35–232 U/L), lactic acid was 3.1 mmol/L (0.5–2.0 mmol/L), and troponin was 0.18 ng/mL (0–0.05 ng/mL). Blood as well as urine cultures were positive for MSSA. Furthermore, urine drug screening returned positive for cocaine and opioids. Soon after presentation, the patient developed acute hypoxic respiratory failure, hemodynamic shock, and worsening encephalopathy. He was admitted to the medical intensive care unit (MICU) for pressor support and mechanical ventilation.
Pan-computed tomography (CT) scans revealed bilateral multiple pulmonary nodular opacities, some of which were cavitary in nature concerning for multifocal pneumonia, acute hematomas in the abdominal wall musculature, and multiple subacute to chronic left cerebellar and left occipital infarcts, all concerning for septic emboli. These brain lesions were confirmed on subsequent brain MRI. Cultures from the bronchoalveolar lavage were positive for MSSA, negative fungal culture, and acid-fast stain. Further laboratory testing showed negative results in a fourth generation HIV1/2 immunoassay and in T-spot tuberculosis screening.
A transthoracic echocardiogram (TTE) revealed a 0.5 cm mobile mass, consistent with vegetation, in the atrial aspect of the septal leaflet of the tricuspid valve without any valvular dysfunction (). Although the other valves were not well visualized on this study, the patient's left ventricular ejection function was noted to be normal. Given concerns for left-sided endocarditis, a transesophageal echocardiogram (TEE) was pursued. TEE showed vegetations on the tricuspid, mitral, and aortic valves, as well as in the right ventricular outflow tract. The tricuspid valve had a 1.0 × 1.0 cm vegetation on the anterior leaflet and a 0.5 × 0.5 cm vegetation on the septal leaflet. The mitral valve had a 1.2 × 1.1 cm vegetation on the P3 segment. There was also a 0.8 cm vegetation on the noncoronary cusp of the aortic valve with only mild aortic insufficiency. The pulmonic valve itself was without vegetations, but there was a 1.1 × 1.1 cm vegetation in the right ventricle outflow tract (RVOT) ().
The patient continued to receive medical care in the MICU for 2 weeks with a progressive decline in his condition. The patient was deemed to be a poor unstable surgical candidate by the cardiothoracic surgery team, and hence, the patient was transitioned to comfort care measures only after detailed discussions with the family. The patient passed away shortly thereafter from multiorgan failure. An autopsy was declined by the family.
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pmc-6348891-1
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A 63-year-old female presented with epigastric pain, loss of appetite, abdominal bloating, regurgitation, and episodic projectile vomiting of five-year duration. These symptoms were aggravated particularly after meals. Her bowel opening was normal. The patient had lost 20 kilograms over five years. The patient had a background history of hypothyroidism for which she was on thyroxine replacement therapy. She was clinically euthyroid. She had undergone a vaginal hysterectomy for uterovaginal prolapse at the age of 39 years. There was no significant family history for bowel disorders.
On physical examination, she had a body mass index of 13. She was pale. There were peripheral stigmata of chronic malnutrition and vitamin B12 deficiency. She had a distended abdomen, visible peristalsis, and hyperacute bowel sounds. There was no clinically demonstrable free fluid in the abdomen. She had anaemia (haemoglobin-8.9 g/dl, haematocrit-27.3%, mean corpuscular volume-97.4 fl, mean corpuscular haemoglobin-31.6 pg, mean corpuscular haemoglobin concentration-325 g/l, and red cell distribution width-58.4 fl), with normal platelet (402 × 103/μl) and leucocyte (8.07 × 103/μl) counts. Blood picture showed macrocytic red cells and hypersegmented neutrophils. Abnormal chemical pathological investigations comprised of elevated C-reactive protein (20.1 mg/l), hypoproteinaemia (59 g/dl), hypoalbuminaemia (25.3 g/l), hypovitaminosis B12 (160 pg/ml), and hypocholesterolaemia (total cholesterol-125.5 mg/dl, HDL-32 mg/dl, LDL-66.3 mg/dl, and triglycerides-136.4% with normal VLDL-27.2 mg/dl). Serum ionized calcium was 2.41 mmol/l. Serum iron studies favoured anaemia of chronic disease (serum iron-95 μg/dl, total iron binding capacity-138 μg/dl, iron saturation-40.8%, and ferritin-238 μg/l). She was biochemically euthyroid (TSH-4.65 mIU/l). Ultrasonography was suggestive of subacute small intestinal obstruction with distended first part of the duodenum filled with fluid. Multiple tortuous small bowel loops were noted around the pancreas with increased peristalsis. Large bowel was distended with gas. There was no bowel wall thickening, mass lesions, or free fluid in the abdomen. Computed tomography (CT) of dual slice contiguous axial sections of the abdomen obtained after intravenous, oral, and rectal contrast administrations demonstrated mild dilatation of the first and second parts of the duodenum with no evidence of significant obstruction to distal passage of oral contrast. The stomach was not distended. A focal calcification of the segment VII of the liver was noted, which was likely to be an incidental finding. There was no CT evidence of an annular pancreas or superior mesenteric artery syndrome. A spiral CT was repeated after one week. It showed markedly distended proximal bowel loops involving the duodenum and the proximal jejunum. No definite transition point was identified. There was a whirl appearance seen in the mesentery and superior mesenteric venous branches around the superior mesenteric artery raising the suspicion of a possible midgut volvulus. Mild mesenteric engorgement was also seen. No definite CT evidence of diverticuli was seen. Upper gastrointestinal (UGI) endoscopy revealed multiple duodenal diverticuli with a small hiatus hernia. Barium meal and follow-through study revealed a slightly distended duodenum without evidence of obstruction or persistent narrowing. Magnetic resonance enterography revealed multiple dilated small bowel loops with loss of valvulae in the right side of the abdomen (). There were numerous outpouchings arising from the small bowel.
However, with unexplained weight loss, we wanted to exclude a gastrointestinal malignancy and intestinal tuberculosis. We opted for laparotomy out of diagnostic laparoscopy and laparotomy. Multiple large diverticuli were noted extending from the first part of the duodenum to the proximal ileum (). Diverticuli were measuring from 0.5-12.0 cm. There was macroscopic evidence of diverticulitis. There was gross gastric dilatation. Proximal small bowel was dilated without a definitive transition point. The rest of the terminal ileum and colon were normal macroscopically. Small bowel was not surgically resected because she was not a suitable candidate for a primary anastomosis as she had nutritional deprivation and the risk of short gut syndrome. We closed the abdomen without any surgical interventions. Because of macroscopic evidence of diverticulitis, intravenous cefuroxime 750 mg 8 hourly and intravenous metronidazole 500 mg 8 hourly were administered for 7 days and were converted to oral cefuroxime 500 mg 12 hourly and oral metronidazole 400 mg 8 hourly for another 21 days. She had an uneventful postoperative period. She received complementary parenteral nutrition with amino acids, electrolytes, dextrose, and lipid injectable emulsions followed by standard polymeric formulae containing whole proteins. Soluble fibres were gradually introduced to her diet. She received a high-calorie diet, initially 125% of the daily calorie requirement followed by 150% of the daily calorie requirement after one month. Thousand international units of vitamin B12 was administered intramuscularly every other day for five days, and oral vitamin B complex 1 mg three times a day was continued for six months, with folate and micronutrient replacement. Iron-rich food and standard formulae were used to supplement micronutrients such as selenium and zinc for six months. She had a remarkable recovery with no recurrence of symptoms following 10 months follow-up.
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pmc-6348892-1
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Our patient is a 68-year-old male with a past medical history of hyperlipidemia, hypertension, and smoking, who presented with an incidental pancreatic cyst on lung cancer screening helical CT. His CT had shown a 23 × 18 mm fluid density lesion in the distal pancreatic body, without pancreatic ductal dilation. He underwent an EUS-FNA which revealed an anechoic and septated cyst. Needle aspiration with a 19 G Boston Sci. needle was performed for amylase, tumor marker (CEA), and cytology. Cyst fluid analysis showed amylase of 1532 and a CEA of less than 200. FNA cytology revealed a moderately cellular aspirate with no identifiable malignant cells (). These findings were consistent with a pseudocyst or a benign cyst.
On follow-up CT abdomen and pelvis with IV contrast in six months, the cyst persisted and the size was unchanged ().
This prompted a repeat EUS-FNA using 19G Boston Scientific needle combined with nCLE (using AQ-Flex 19; Mauna Kea Technologies). The tip of the AQ-Flex probe was advanced with the needle under EUS guidance until there was contact with the cyst wall without putting pressure. Fluorescein (2.5 to 5 mL of 10% Fluorescein) was injected intravenously immediately prior to CLE imaging. Around-3-minute-long video was acquired with permissible needle angulation. nCLE revealed thick cord like and dark nest like structures (Figures and ).
There was no evidence for dark rings, vasculature network, or papillary projections to suggest intraductal papillary mucinous neoplasm. These findings were consistent with cystic neuroendocrine tumor of the pancreas []. These findings prompted us to send the patient for surgical evaluation. Final histopathology (Figures and ) confirmed the preoperative nCLE based diagnosis of the cystic neuroendocrine tumor of the pancreas.
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pmc-6348928-1
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A three-year-old, 14.5 kg boy, without medical history and prescribed no medications, presented to the emergency department (ED) within an hour of ingesting between seven and sixteen oral disintegrating 25 mg lamotrigine tablets and one to six 0.5 mg clonazepam tablets from his brother's pill organizer.
Emergency Medical Services arrived at the home within 20 minutes of ingestion and found the patient minimally responsive and arousable only to painful stimuli with poor respiratory effort. Bag-valve-mask ventilation was provided. An intraosseous (IO) line was established, and the patient was transported to the ED.
In the ED, the child had a Glasgow Coma Scale of 3 and minimal independent respiratory effort. Vital signs included heart rate of 100 beats per minute, pulse oximetry 100% (100% oxygen via bag-valve mask), temperature 35.7°C (96.2°F), and blood pressure 92/43 mmHg. The child then developed a tonic-clonic seizure which was treated with 1 mg IO lorazepam. The patient was endotracheally intubated for airway protection and admitted to the pediatric intensive care unit (PICU) for further monitoring and care. An electrocardiogram (QRS interval of 86ms), complete blood count, serum chemistries, serum acetaminophen, salicylate and ethanol concentrations were all unremarkable.
Serum lamotrigine concentrations measured 23.2 mcg/mL and 18.5 mcg/mL approximately three and 24 hours after ingestion, respectively. Serum liquid chromatography-mass spectrometry detected the following: acetaminophen, lamotrigine, 7-aminoclonazepam, midazolam, alpha-hydroxymidazolam, and lorazepam. This result is consistent with both ingestion of lamotrigine and clonazepam, as well as iatrogenic administration of lorazepam, midazolam, and acetaminophen.
While in the PICU, the child demonstrated mild hyperkinesia and periods of agitation. He was extubated on hospital day (HD) one, and these symptoms resolved over the subsequent 36 hours. Ultimately, the patient was discharged in normal condition on HD five.
|
pmc-6349438-1
|
A 50 year-old woman presented to her primary care physician for evaluation of a 2 week history of right upper quadrant abdominal pain and weight loss. CT of the abdomen and pelvis revealed multifocal hepatic disease and a dominant 7 cm lesion in the right lobe of the liver (Figure ). CT scans of chest and brain showed no evidence of extrahepatic disease.
Percutaneous liver biopsy showed an extensively hemorrhagic and necrotic tumor composed of irregular, anastomosing vascular channels lined with atypical cuboidal to flattened endothelial cells with irregular hyperchromatic nuclei (Figure ). Occasional mitoses were identified in vascular lining. The neoplastic cells were positive for vascular endothelial markers CD31 and CD34, but negative for cytokeratin E1/AE3, cytokeratins 7 and 20, hep-par1, AFP, and CA19.9. Proliferation index as detected by Ki-67 immunostaining was variable, ranging from <10% to focal areas of 40%. These morphologic features and immunophenotype were consistent with diagnosis of HAS.
Before starting therapy, patient had an acute decrease in hemoglobin (Hgb) from 8 to 6.5 and repeated CT scan that showed progression of dominant lesion to 12 cm, ascites and a small area of subcapsular hemorrhage compared to scan one month prior. The patient underwent hepatic artery embolization with post-procedural stabilization of Hgb and received chemotherapy with paclitaxel. Shortly thereafter, the patient experienced gradual deterioration of performance status with progressive abdominal pain, ascites and lower extremity edema. She chose to not receive additional cancer-directed therapy and pursued hospice care. The patient expired 2 months after initial diagnosis.
Comprehensive genomic profiling (CGP) performed on liver biopsy specimen revealed a ROS1 rearrangement involving GOPC that had not been previously described in HAS. []. CGP of the liver biopsy specimen was performed in a Clinical Laboratory Improvement Amendments (CLIA)-certified pathology laboratory (Foundation Medicine, Cambridge, MA), as previously described [, ]. In brief, ≥50 ng DNA was extracted from 40 microns of tumor sample in formalin-fixed, paraffin-embedded tissue blocks. Next generation sequencing was performed and targeted all coding exons in 405 cancer-related genes plus select introns within 31 genes that often display rearrangements in malignancy (FoundationOne) using Illumina HiSeq (Illumina, San Diego, CA) technology [].
Additional abnormalities included mutations in MLL2 and PRDM1 genes, CDKN2A loss, and a low overall tumor mutational burden (4 mutations per megabase). Variants of unknown significance were noted in the following genes BRSK1, JARID2, KIT, MLH1, PPP2R1A, and ZNF217. Since these results became available only after the patient's decline and subsequent transition to hospice care, she was unable to receive targeted therapy against ROS1.
|
pmc-6349568-1
|
A 34-year-old female presented with intractable left leg radiculopathy. She reported moderate pain in her lower back with pain and numbness radiating down the left S1 distribution. She had no motor weakness or altered bowel or bladder function. Magnetic resonance imaging (MRI) demonstrated L5-S1 disc herniation compressing the S1 nerve root (Figure ). The patient underwent an L5-S1 tubular hemilaminectomy and microdiscectomy. An intraoperative CSF leak was repaired with onlay autologous fat graft and dural spray sealant. Postoperatively, she developed positional headaches attributable to the CSF leak. She was treated with two EBPs at the L5/S1 interlaminar space on postoperative day one and two. The headaches resolved, and on outpatient follow-up two weeks postoperatively, she continued to deny headaches, and reported a complete resolution of her radiculopathy and resumption of daily activities pain-free. After her two-week follow-up, she continues to deny headaches and does not complain of pain.
|
pmc-6349568-2
|
A 49-year-old male presented with intractable and progressive pain in the lower back and left leg. Additionally, he had weakness of left plantar flexion. MRI revealed a large disc herniation at L5-S1 with compression of the left S1 nerve root (Figure ). The patient subsequently underwent L5-S1 tubular hemilaminectomy and discectomy. An intraoperative CSF leak was repaired with onlay autologous fat graft and dural spray sealant. The patient developed positional headaches and received an EBP at the level of L5/S1 interlaminar space on postoperative day one with complete symptom relief. On outpatient follow-up two weeks postoperatively, he reported resolution of radiculopathy and denied headaches. Upon further follow-up, he continues to deny headaches and reports he is physically active.
|
pmc-6349569-1
|
Case 1: contact burn to left hip
A 77-year-old male was admitted for contact burns to the bilateral lower extremities and over the left hip, involving not only the skin but a burn injury down to the fascia and muscle. The patient had multiple medical comorbidities, including diabetes mellitus and hyperlipidemia. After debridement and dressing changes (Figure ), attempts at grafting the site with split-thickness skin grafts (meshed 2:1, 150 sq cm) resulted in graft loss within days, secondary to an inadequate depth of debridement and the lack of appropriate granulation tissue presence. Therefore, the patient was transitioned into VVCC NPWT to assist in irrigation debridement, granulation tissue formation, and wound contraction with the addition of Vashe® instillation at 30 ml for 20 minutes duration every three hours before returning to suction for over two weeks. NPWT suction was maintained at 125 mmHg while on suction. Rapid improvement in wound granulation tissue formation was noted (“comedones” or discrete and increased granulation tissue within the ROCF hole boundaries, Figure ). The patient continues to follow up in our clinic with planned skin grafting at the time of this manuscript’s submission.
|
pmc-6349569-2
|
Case 2: contact burn to left buttock
An 87-year-old male was admitted for contact burns following a syncopal episode. The wounds were full thickness burns requiring excision down to the subcutaneous tissue (Figure ). A significant soft tissue defect over the left buttock was noted and VVCC NPWT was placed over the wound. Vashe instillation was started at 30 ml for 20 minutes every three hours before returning to NPWT suction at 125 mmHg, which was applied to assist in the debridement and granulation of the wound. Short-term therapy with the VVCC resulted in a healthy granulation bed demonstrating near-skin-level comedone granulation tissue formation (Figure ). The patient received an autologous skin graft (meshed 1:1, 200 sq cm) after the completion of VVCC therapy with 100% skin graft take (Figure ).
|
pmc-6349569-3
|
Case 3: contact burns to bilateral buttocks
A 61-year-old female was admitted with full thickness contact burns to the bilateral buttocks (Figure ). Initially, this was debrided down to the soft tissues. After a week of dressing changes, an autologous skin graft was applied (meshed 1:1, 100 sq cm) but the skin graft failed and necessitated a return to the operating room for further debridement. A VVCC NPWT was placed with improved granulation tissue formation (Figure ). Vashe solution was used to irrigate the wound with 30 ml for a 20 minute dwell time every three hours with a NPWT suction of 125 mmHg. Eventual split-thickness skin grafting to the bilateral buttocks had a 100% graft take (Figure ).
|
pmc-6349569-4
|
Case 4: contact burns to the anterior torso and bilateral lower extremities
A 76-year-old male admitted with 21% total body surface area (TBSA) full thickness contact burns to his anterior torso and bilateral lower extremities following a ground level fall. The patient was debrided multiple times, which included a right, above-knee amputation, and autologous skin grafting to the anterior upper torso and right thigh. Deeper tissue defects of the lower abdomen (Figure ) and left lower extremity (Figure ) required the placement of the VVCC NPWT system with HOCl instillation of approximately 30 ml to the lower abdomen and 30 ml to the left lower extremity for 30 minutes every three hours with an NPWT suction of 125 mmHg. After two weeks of therapy (Figures -), the patient had an autologous skin graft applied to the anterior torso and left lower extremity (meshed 2:1, 1400 sq cm). There was a 100% skin graft take to the lower abdominal torso and 90% skin graft take of the left lower extremity (Figures -).
|
pmc-6349569-5
|
Case 5: necrotizing fasciitis of the lower abdominal wall
A 55-year-old female was admitted with a past medical history of multiple medical comorbidities, including diabetes mellitus (type 1), rheumatoid arthritis requiring immunosuppressant therapy, and baseline liver dysfunction. She had an abdominal wall necrotizing fasciitis due to an infected subcutaneous insulin pump. The patient underwent extensive debridement down to the rectus fascia and was left with a large soft tissue defect (Figure ). The patient had placement of the VVCC NPET once the wound was debrided to viable tissue and the initial infection was controlled. The wound has been granulating and contracting with V.A.C. VERAFLO CLEANSE CHOICE™ also utilizing HOCl instillation of 30 ml with a dwell time of 20 minutes every three hours and then returning to a negative pressure of 125 mmHg. Because of the patient’s multiple medical issues and compromised immune system that would normally impair wound healing, the patient is weeks away from wound closure (Figure ). The patient's therapy was completed with the closure of the wound following split-thickness skin grafting (Figure ).
|
pmc-6349571-1
|
A 64-year-old Caucasian male with a medical history of type 2 diabetes mellitus, hypertension, and hyperlipidemia presented to an eye clinic for a diabetic eye exam. He had no ocular complaints aside from slightly blurred vision, which he attributed to “scarring on his retina.” He stated that he had developed “smoky vision” several years ago, which had been treated with oral and topical medications. He denied any current ocular discomfort or pain.
On exam, his visual acuity was 20/25+2 in the right eye and 20/20-2 in the left with correction. Anterior segment examination did not reveal any abnormalities. Intraocular pressures were within normal limits. Dilated fundus examination found optic nerve head drusen, extramacular healed chorioretinal scars, and mild non-proliferative diabetic retinopathy in both eyes.
Diagnostic work-up included laboratory testing and magnetic resonance imaging (MRI) of the brain and orbits with and without contrast. This work-up was prompted by the history of chorioretinal scarring and blurry vision in the past requiring treatment. Laboratory workup was negative aside from positive toxoplasma IgG antibodies. MRI of the brain and orbits with and without contrast revealed varices of the bilateral inferior ophthalmic veins, bilateral pterygoid plexuses, and the infratemporal veins. There was no dilation or thrombosis of the superior ophthalmic veins, and no abnormal enhancing mass lesions within the orbits or brain parenchyma. No intracranial arteriovenous malformations, dural fistulas, or carotid cavernous fistulas were identified (Figures -).
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pmc-6349695-1
|
A 65-year-old man with a 68 pack-year smoking history consulted his primary care physician with the chief complaint of a productive cough. Subsequently, a large mass lesion of his right lung was detected on chest X-ray, and he was referred to our hospital. He was further examined through contrast-enhanced computed tomography (CT), which revealed a mass lesion with a 92-mm diameter, extending from the middle lobe of his right lung to the upper mediastinum, lymphadenopathy of the mediastinum and bilateral neck, swelling of bilateral adrenal grands, intraperitoneal dissemination, and slight pericardial effusion. After further examination, he was diagnosed with adenocarcinoma of the lung, cT4N3M1c, stage IVB (8th edition of the TNM classification for lung cancer). Neither epidermal growth factor receptor (EGFR) mutations nor an anaplastic lymphoma kinase (ALK) gene rearrangement were detected. The patient was treated with four cycles of carboplatin and pemetrexed. Nearly all lesions diminished in size; however, intraperitoneal dissemination worsened.
Nivolumab therapy was then initiated for the patient (3 mg/kg every 2 weeks) as a second-line therapy. His serum carcinoembryonic antigen (CEA) level before initiation of nivolumab therapy was 143.7 ng/ml; his chest X-ray and CT are presented as Figures , respectively. After two cycles of nivolumab administration, the tumor size decreased (Figures , respectively). After four cycles of nivolumab administration, he returned to our hospital with the complaint of dyspnea. His blood pressure was 141/85 mmHg, pulse rate was 111/min, and oxygen saturation was 96% on room air. A chest X-ray revealed cardiomegaly, and echocardiography indicated massive pericardial effusion (Figures , respectively). He was further diagnosed as having cardiac tamponade. Other irAEs, including myocarditis, were not detected. His serum CEA level was decreased (22.5 ng/ml). He then received pericardiocentesis, and 1,000 ml of bloody effusion was removed. Immediately following this procedure, his condition improved. The pericardial effusion contained 3,025 white blood cells per microliter, and 84% of these cells were lymphocytes. Moreover, cytology revealed adenocarcinoma cells. Despite the fact that nivolumab therapy had not had a positive impact on the pericardial effusion, it had been effective for decreasing the tumor lesions; therefore, the therapy was continued. Corticosteroid treatment was not administered. After five cycles of nivolumab administration following the pericardiocentesis, the pericardial effusion did not recur (Figures , respectively); however, intraperitoneal dissemination worsened again, and nivolumab therapy was discontinued. Subsequently, he was treated with several chemotherapies, such as pemetrexed and bevacizumab, gemcitabine, and bevacizumab, as well as nab-paclitaxel monotherapy; however, the efficacy of these treatment regimens was limited. Eighteen months after pericardiocentesis, the patient died of lung cancer progression; however, pericardial effusion had not increased.
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pmc-6349695-2
|
A 71-year-old man with a 25 pack-year smoking history visited our hospital with the chief complaints of productive cough and dyspnea. Subsequently, a massive left pleural effusion was detected on chest X-ray. He was then examined through contrast-enhanced CT, which revealed a massive left pleural effusion, a mass lesion with a 36-mm diameter, in the lower lobe of his left lung, and slight pericardial effusion. After further examination, the patient was diagnosed with adenocarcinoma of the lung, cT4N3M1a, stage IVA. Neither EGFR mutations nor an ALK gene rearrangement were detected. He was treated with four cycles of carboplatin and nab-paclitaxel, and the treatment was effective for all previously detected lesions; however, multiple brain metastases arose. He then received whole brain irradiation, and these new lesions showed reduction. Subsequently, he was treated with three cycles of pemetrexed as a second-line chemotherapy; however, the primary lesion showed regrowth.
Nivolumab therapy was then initiated for the patient (3 mg/kg every 2 weeks) as a third-line therapy. The chest X-ray and CT before initiation of nivolumab therapy are presented as Figures –, respectively, (circle: primary lesion), and the serum cytokeratin 19-fragment (CYFRA 21-1) level was 20.7 ng/ml. After two cycles of nivolumab administration, he returned to our hospital with complaints of chest pain and dyspnea. His blood pressure was 95/60 mmHg, pulse rate was 133/min, and oxygen saturation was 89% on 1 L of oxygen delivered by nasal cannula. A chest X-ray revealed cardiomegaly (Figure ). Massive pericardial effusion was detected by echocardiography as well as by chest CT (Figure ). In addition, the chest CT detected enlargement of the primary lesion (Figure , circle). Other irAEs including myocarditis were not detected. The serum CYFRA 21-1 level was increased (40.7 ng/ml). After he was diagnosed as having cardiac tamponade, he received pericardiocentesis, and 1,400 ml of bloody effusion was removed. The pericardial effusion contained 756 white blood cells per microliter, and 2% of these cells were lymphocytes. Cytology of the effusion detected adenocarcinoma cells. One month after the first pericardiocentesis, pericardial effusion had increased again. Therefore, pericardiocentesis was re-conducted, and 450 ml of pericardial effusion was removed. At this time, the serum CYFRA 21-1 level was decreased (8.8 ng/ml). All anticancer therapy, including nivolumab therapy, was discontinued. Corticosteroid treatment was not administered. Two months after the second pericardiocentesis, a chest X-ray showed no cardiomegaly (Figure ), and chest CT showed decreasing pericardial effusion (Figure ). Despite the fact that the primary lesion was reduced (Figure , circle), a few new intrapulmonary lesions appeared (Figure , arrows); therefore, the disease was deemed to be progressing. Ten months following the second pericardiocentesis, the patient died of lung cancer progression, without any increase in pericardial effusion.
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pmc-6349767-1
|
An otherwise healthy 5-year-old boy presented with an acute papulovesicular rash of both legs (Figure ) and intermittent abdominal pain. The patient did not have fever. Differential blood count (white blood cell count of 12,900/μl with 60% granulocytes and 29% lymphocytes), C-reactive protein (0.45 mg/dl), erythrocyte sedimentation rate (20 mm/h) and global blood clotting tests (INR 0.98, PTT 31.5 s) were normal. Serum IgA (147 mg/dl) and IgM (66 mg/dl) levels were within age matched reference ranges whereas IgG levels were slightly decreased (557 mg/dl, reference range 640-1420). No hematuria or fecal occult blood could be detected. Abdominal ultrasound could exclude intussusception but revealed thickened bowel wall at the ileocecal junction. The abdominal symptoms resolved spontaneously within 2 days but arthralgia appeared thereafter.
Nine days after the onset of disease the skin lesions at the arms, legs, feet and ankles rapidly evolved into palpable purpura and hemorrhagic-bullous lesions of variable size ranging from 5 to 40 mm (Figures ). Some of the blisters spontaneously ruptured and disclosed hemorrhagic fluid which remained sterile in the microbiological work-up. The patient was given cefuroxime as antibiotic prophylaxis. Severe hemorrhagic-bullous HSP was suspected but differential diagnoses included septicemia/septic emboli and autoimmune blistering disease. Absence of fever and leukocytosis and sterile blood cultures argued against an infectious etiology. Neither circulating antibodies directed against structural proteins of the basement-membrane zone nor ANAs or ANCAs could be detected in the patient‘s serum. C4 levels were in the normal range while C3c levels were slightly elevated (152 mg/dl, reference range 80-120). A skin biopsy was performed and histological examination showed signs of a small vessel leukocytoclastic vasculitis limited to the upper dermis (Figure ), and direct immunofluorescence analysis revealed IgA and C3 deposits in vessel walls, compatible with HSP.
The patient was treated with oral corticosteroids (prednisolone 1 mg/kg/day) for 7 days, then subsequently tapered over 39 additional days. Although fading of inflammation paralleled healing of most erosions, a deep necrosis resulting from a large blister at the dorsum of the right foot persisted (Figures ) so that autologous skin transplantation was performed. Re-examination 11 months after disease onset showed complete clinical remission of disease with re-epithelialization but also scarring of some affected areas (Figures ).
|
pmc-6350115-1
|
A 72-year-old male with a history of bioprosthetic aortic valve replacement was
admitted for generalized weakness and fatigue. He was found to have anemia with
positive fecal occult blood and subsequently received upper
esophagogastroduodenoscopy revealing an obstructive esophageal cancer. Given the
presence of the prosthetic aortic valve and an episode of bradycardia that occurred
during colonoscopy, the cardiology team was involved in the patient’s care.
During his hospital stay, the patient had intermittent fever and leukocytosis. Blood
cultures were positive for Staphylococcal species. Appropriate
antibiotics failed to improve his fever. A transthoracic echocardiogram (TTE)
revealed no abnormalities of bioprosthetic aortic valve except mild aortic
regurgitation.
The TEE was not feasible due to the advanced obstructive nature of esophageal cancer.
Intracardiac echo (ICE) was attempted in this patient to establish a prompt
diagnosis and institute an appropriate treatment. The AcuNav, 8 Fr intracardiac
echography probe (Siemens AG, Munich, Germany) was introduced into the right femoral
vein and then advanced into the right atrium and subsequently into the right
ventricle. The bioprosthetic aortic valve was visualized in both short axis and
longitudinal views ( and ).
The images disclosed the perivalvular aortic root abscess as well as a rocking motion
of the bioprosthesis with moderate aortic regurgitation. All these findings were
consistent with complicated IE. Periaortic root abscess was drained by an urgent
surgical intervention and the infected bioprosthetic valve was also replaced. The
patient was referred for urgent surgery, where the perivalvular aortic root abscess
was drained, and subsequently the infected bioprosthetic valve was replaced.
|
pmc-6350132-1
|
A 47-year-old man presented to our university-based internal medicine clinic with
complaints of dark urine, pruritus, subjective fevers, and fatigue for several days
duration. He described subjective fevers with objective measurements ranging from
100°F to 101°F for 2 days with subsequent symptoms of dysuria, urinary frequency,
urinary urgency, and darkening of his urine despite large volumes of oral intake.
The patient developed generalized malaise, a reduction in appetite, and diffuse
pruritus without an associated rash or change in skin color. He reported one episode
of nonbloody, nonbilious emesis. He endorsed sick contacts noting his 2 children
suffered upper respiratory infection symptoms of cough, rhinorrhea, and sore throat.
He denied any recent travel, hospitalizations, or antibiotic use. He took
acetaminophen for symptom control but restricted its use to the recommended 3000 mg
per day limit. He denied any new or over-the- counter medications including herbal
supplements. His previous medical history was notable for obesity (body mass index
of 32.68 kg/m2), hypertension, prediabetes (previous A1C 6.2%), anxiety,
major depressive disorder, and untreated hypertriglyceridemia. His current
medications entailed valsartan, metoprolol tartrate, escitalopram, clonazepam, and
fexofenadine. His vitals on presentation included a temperature of 36.7°C, heart
rate of 53 beats/min, blood pressure of 127/84 mm Hg, and oxygen saturation of 96%
on room air. His physical examination revealed nonicteric sclera and sublingual
jaundice. He possessed no lymphadenopathy or hepatomegaly. Initial laboratory
testing included a point of care urinalysis notable for the presence of urobilinogen
and no leukocyte esterase or nitrites. Additional blood work revealed an elevated
total bilirubin of 5.8 mg/dL with a direct bilirubin of 4.3 mg/dL, elevated
aspartate aminotransferase (AST), alanine aminotransferase (ALT), and alkaline
phosphatase (ALP) of 108 U/L, 265 U/L, and 170 U/L, respectively, and an albumin of
3.5 g/dL. His serum creatinine was 1.1 mg/dL with a blood urea nitrogen level of 15
mg/dL. A urinalysis with microscopy noted 30 mg/dL protein, moderate bilirubin, 4.0
mg/dL urobilinogen, and 2 red blood cells per high-power field. The patient was
contacted via phone with the laboratory results with an emphasis on the
hyperbilirubinemia and elevated aminotransferases. Further history was solicited,
and the patient reported a trip to Seattle 3 weeks prior to presentation where he
received kratom from a friend. The patient reported that he ingested kratom capsules
in an effort to manage his low back pain. Initially, he admitted to only using
kratom once. On further questioning, he reported using the substance on multiple
occasions, but not daily, at the time of presentation. He again denied alcohol use.
His medical stability with normal mentation and robust support system allowed for
further evaluation in the outpatient setting.
Further testing revealed a normal prothrombin time and international normalized
ratio. Repeat liver tests revealed an uptrend in total bilirubin to 6.1 mg/dL, with
a direct bilirubin of 5.1 mg/dL, elevated AST, ALT, and ALP of 114 U/L, 324 U/L, and
148 U/L, respectively, and an albumin 3.5 g/dL. A right upper quadrant ultrasound
identified hepatic steatosis without cholelithiasis, cholecystitis, or duct
dilation. Additional laboratory tests included an undetectable acetaminophen level,
negative Epstein-Barr virus polymerase chain reaction, negative acute hepatitis
panel (testing for viral hepatitis A, B, and C), normal α-1 antitrypsin level, and
negative antinuclear antibody. He also had a normal thyroid-stimulating hormone of
2.168 U/mL and ceruloplasmin level of 35 mg/dL. The ferritin was elevated at 818
ng/mL with otherwise normal iron studies. Notably, his cytomegalovirus (CMV) IgM
antibody index returned positive at 1.7. Laboratory values both 1 week and 2 weeks
post index showed improving, but persistent, abnormalities (). The patient remained out of the
hospital during the entire clinical course without complications.
Nine months after the resolution of his symptoms and liver test abnormalities, the
patient again presented with 2 days of fatigue, loss of appetite, and intense
pruritus without rash. A laboratory evaluation revealed a total bilirubin of 3.2
mg/dL, an AST of 185 IU/L, an ALT of 566 IU/L, and an ALP of 211 U/L. After intense
questioning, the patient reluctantly admitted to using kratom again, this time in a
powder form. This was his first use of kratom since his initial presentation. Given
the similar symptoms, biochemical profile, and shortened latency, this constituted a
positive rechallenge and further validated the diagnosis of drug-induced liver
injury (DILI) caused by kratom. Fortunately, he suffered no impairment of his
liver’s synthetic function, and his liver chemistries trended toward normal 3 weeks
following rechallenge.
|
pmc-6350155-1
|
A 68-year-old white female presented to emergency department with complaints of
nausea, vomiting, and altered mental status.
One day prior, she presented to an outpatient clinic with left lower quadrant pain
and diagnosed with clinical diverticulosis. She was started on oral ciprofloxacin
and metronidazole from the clinic. She started taking these medications that
afternoon, both on an empty stomach. She began to have dizziness and vomiting since
4 am on the morning of presentation. She was brought by Emergency Medical
Service to the emergency department that afternoon.
The patient was afebrile (97.3°F) but had elevated blood pressure at presentation
(176/107 mm Hg). Physical examination revealed that she had dry oral mucosa, her
abdomen was soft, with no organomegaly, and she did not have any neurological
deficits. Laboratory findings on admission are shown in . Having nausea and vomiting,
physical examination with findings of dry mucus membranes and laboratory tests with
elevated blood urea nitrogen and creatinine suggested dehydration. She was also
noted to have hypercalcemia (). Hypercalcemia by itself could likely cause volume depletion,
since hypercalcemia leads to diuresis and vasoconstriction and contributes to acute
kidney injury. However, in this patient, given her recent history of vomiting, it
would be difficult to attribute volume depletion solely to hypercalcemia. There was
no history of primary HPT, thyroid disease, or thyroid cancer in her family. She was
treated with intravenous hydration with normal saline. Serum calcium reduced from
15.8 to 13.6 mg/dL with hydration alone. Prior to this hospitalization, she was on
over-the-counter calcium 600 mg, vitamin D3 800 IU, vitamin A 11 000 IU
daily, for history of macular degeneration, and valsartan/HCTZ 320 to 12.5 mg daily
for her hypertension; all medications had been taken for 3 years. These medications
were stopped during hospitalization.
She was subsequently referred for endocrinology evaluation. One week later,
laboratory findings in endocrinology clinic showed elevated serum calcium and
parathyroid hormone (PTH; ). 25-Hydroxy (25-OH) vitamin D; 1,25-OH vitamin D; and PTH-RP were
within normal limits. Serum immunofixation electrophoresis showed poorly defined
area of monoclonal protein. She underwent oncology evaluation to rule out multiple
myeloma.
Laboratory findings on oncology evaluation showed that urine Kappa light chains were
increased, as was the urine free Kappa/Lambda ratio, but no monoclonal spike was
detected in urine electrophoresis. On quantitative immunoglobulin (Ig) evaluation,
IgG (1327 mg/dL) and IgA (258 mg/dL) were normal, and IgM minimally elevated (289
mg/dL). Immunofixation revealed IgG Lambda monoclonal protein. She thus had
monoclonal gammopathy of undetermined significance but no myeloma; therefore,
myeloma was not contributing to hypercalcemia. Normal 1,25-dihydroxyvitamin D level
and angiotensin converting enzyme level ruled out the possibility of granulomatous
diseases like sarcoidosis and lymphoma.
She came for review in endocrinology clinic, a few weeks after her initial visit. She
had been off HCTZ for a few weeks at the time of this visit. Laboratory findings in
this visit ( and
) revealed elevated
calcium, with non-suppressed PTH, and normal 24-hour urine calcium. The diagnosis of
primary HPT was thus confirmed.
She was subsequently treated surgically for primary HPT. The right and left superior
parathyroid showed hypercellular parathyroid on pathology. The patient was
normocalcemic after surgery. The calcium on follow-up visit about a week after
surgery was 9.8 mg/dL (8.5-12.1).
|
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