Objective The aim of this study was to investigate whether remedial hydration (RH) reduces the incidence of contrast-induced nephropathy (CIN) and short-term adverse events in ST-elevation myocardial infarction (STEMI) patients undergoing primary percutaneous coronary intervention (PCI).
Methods A total of 216 consecutive STEMI patients were prospectively and randomly assigned into two groups: 108 patients in the RH group and 108 patients in the no RH (control) group. The serum creatinine (SCr) and creatinine clearance (CCr) levels were measured on admission and at 24, 48 and 72 hours after primary PCI. The rates of CIN and short-term adverse events were analyzed for each group. After surgery, the patients were categorized into four groups according to the Mehran risk score: low (≤5, n =98), moderate (6-10, n=56), high (11-15, n=40) or very high (≥16, n=22).
Results The incidence of CIN in the RH group was lower than that observed in the control group (22/108; 20.4% vs. 38/108; 35.2%, p<0.05). The subgroup analysis showed that the rate of CIN was lower in the moderate (6/29; 20.7% vs. 13/30; 43.3%, p<0.10) and significantly lower in both the high (5/21; 23.8% vs. 10/18; 55.6%, p<0.05) and very high score groups (3/12; 25.0% vs. 8/12; 66.7%, p<0.05) among the RH patients compared to the controls. At 24, 48 and 72 hours after PCI, the patients in the RH group exhibited lower SCr levels and higher CCr levels than the patients in the control group (both p<0.05). A lower incidence of in-hospital clinical events was also observed in the RH group.
Conclusion Remedial hydration decreases the occurrence of CIN and improves the short-term prognosis of STEMI patients undergoing primary PCI.

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Objective We aimed to investigate the incidence and clinical characteristics of nontuberculous mycobacterial (NTM) pulmonary disease as a complication of chronic thromboembolic pulmonary hypertension (CTEPH).
Methods We conducted a retrospective study of 10 cases (5.6%) complicated by NTM pulmonary disease among 180 CTEPH patients.
Results Isolated species of avium (n=5), kansasii (n=2), intracellulare (n=1), abscessus (n=1) and fortuitum (n=1) were detected. NTM-infected lesions were observed in 33 of 180 (18.3%) lung segments obtained from the 10 patients, and complete obstruction due to chronic pulmonary thromboembolism was detected in 65 of the 180 segmental pulmonary arteries (36.1%). The NTM-infected segments in the CTEPH patients were significantly associated with obstructed rather than unobstructed pulmonary artery segments [25 of 65 (38.5%) vs. 8 of 115 (6.9%), p<0.01]. Cavitary, nodular, ectatic and ground-glass lesions were seen in 14, 22, seven and four of the 180 segments, respectively. Thirteen of the 14 cavitary (92.9%) lesions were located in non-perfused segments. Five patients with NTM disease underwent pulmonary endarterectomy (PEA). Of the 18 assessable NTM-infected segments in six NTM-treated patients, 17 were located in non-perfused segments and one was located in a previously perfused segment. All NTM-infected segments improved among three segments reperfused with PEA. In contrast, only eight (57.1%) NTM-infected segments improved among 14 continuously non-perfused segments. A lower body mass index was found to be a significant risk factor for NTM disease in the CTEPH patients.
Conclusion This is the first report to document NTM-disease complications in patients with CTEPH. Reperfusion in cases of NTM lesions may improve the response to NTM drug therapy.

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Objective Cognitive and affective dysfunctions are important aspects for patients with multiple sclerosis (MS) and neuromyelitis optica (NMO).
Methods We herein examined the cognitive and affective ability in MS (n=35) and NMO (n=10) patients using computerized touch panel-type screening tests.
Results While MS patients and normal controls (NC1, n=40) did not significantly differ in their scores from the Hasegawa dementia scale-revised (HDS-R) or the frontal assessment battery, MS patients did score significantly lower on the mini-mental state examination (MMSE). In contrast, NMO patients did not differ from the normal control group 2 (NC2, n=15) in any of the three cognitive assessments. We also examined the affective ability and found that MS patients scored significantly higher on the apathy scale (AS) compared with the NC1 group, while NMO patients scored significantly higher on the geriatric depression scale (GDS) compared with the NC2 group. Although the GDS and AS scores did not correlate with any of the cognitive assessments among MS patients, the AS scores did correlate with the MMSE and HDS-R among NMO patients. Compared with normal controls, the times to complete the flipping cards and arranging pictures games were significantly longer for MS patients but not for NMO patients.
Conclusion These results indicate differences between some features of cognitive and affective dysfunctions between MS and NMO patients. Computerized touch panel-type screening tests may be a more useful and sensitive tool for the cognitive assessment of MS patients than NMO patients.

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Objective In 2009, the European Vasculitis Study Group reported the results of CYCLOPS, a randomized controlled trial, in which pulse cyclophosphamide (CYC) was found to be similar to a daily CYC regimen in inducing remission in patients with antineutrophil cytoplasmic antibody-associated vasculitis (AAV). We herein conducted an observational study to describe our experience with Japanese AAV patients treated with pulse CYC according to the CYCLOPS protocol.
Methods The inclusion criteria were as follows: 1) granulomatosis with polyangiitis, of either the limited or generalized type; 2) microscopic polyangiitis or renal limited glomerulonephritis with at least one poor prognostic factor; or 3) microscopic polyangiitis with a motor disturbance due to vasculitic neuropathy. The patients were treated with pulse cyclophosphamide and prednisolone according to the CYCLOPS protocol.
Results Seven patients were included, all of whom (100%) achieved remission with a median time to remission of three months. After the first remission, two patients experienced relapse during the follow-up period of 14.8 months and both were successfully treated with additional pulse CYC therapy. Regarding safety, infection was observed in all patients, including three patients with respiratory tract infections, although all infectious episodes were successfully treated. Bronchoalveolar carcinoma was diagnosed in one patient.
Conclusion The administration of pulse CYC according to the CYCLOPS protocol was found to be effective as remission induction therapy in our seven patients with AAV. With regard to safety, treating Japanese patients with AAV according to this protocol may require close attention for signs of respiratory infection. Our findings suggest that this protocol is therefore a viable option for Japanese patients with AAV.

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A 59-year-old man was orally administered rabeprazole, a proton pump inhibitor (PPI), for gastroesophageal reflux disease, after which he gradually developed iron-deficiency anemia. The anemia did not improve following the administration of ferrous fumarate, and endoscopic screening of the entire gastrointestinal tract, including the small intestine, did not reveal any findings indicating the cause of the anemia. The patient was then switched from rabeprazole to famotidine and the anemia was cured within three months. There is much debate as to whether the long-term use of PPIs causes iron-deficiency. However, this case strongly suggests that PPIs can induce iron-deficiency anemia.

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A 28-year-old woman previously diagnosed to have Down syndrome presented with a one-month history of severe hypoalbuminemia, lower extremity edema, and diarrhea. Her urine was negative for protein. She was diagnosed with immune-mediated protein-losing enteropathy (PLE) based on clinical findings, protein loss evident on ^99mTechnetium-labeled human serum albumin scintigraphy, and IgM and complement C3 deposition in the duodenum. She did not exhibit any manifestations of collagen diseases. A dramatic remission was achieved and maintained with corticosteroid administration. This is the first report of immune-mediated PLE in a patient with Down syndrome.

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A previously healthy 89-year-old man was admitted to our hospital with right upper quadrant pain and mild fever. A diagnosis of cholangitis was suspected based on the patient's physical findings and imaging features. Although he received treatment typical for cholangitis, he suddenly died of shock for unknown reasons two months after disease onset. An autopsy revealed a ruptured hepatic artery aneurysm, which had caused lethal intra-abdominal bleeding. In addition, systemic necrotizing vasculitis of small- and medium-sized arteries was detected, and polyarteritis nodosa (PAN) was diagnosed after the autopsy. Biliary symptoms as the initial manifestation of PAN are extremely rare.

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We herein report a case of intracholecystic papillary-tubular neoplasm (ICPN) of the gallbladder in which jaundice developed. A 58-year-old woman with jaundice was referred to our hospital. Computed tomography revealed a papillary tumor in the body of the gallbladder protruding into the bile duct. A transpapillary biopsy of the bile duct verified adenocarcinoma, and pancreatoduodenectomy with extended cholecystectomy was performed. The tumor spread macroscopically from the gallbladder body to the cystic duct, thus forming a polypoid mass protruding into the bile duct. This is a rare case of invasive carcinoma from ICPN leading to mechanical obstruction of the bile duct.

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A 39-year-old man presented with diarrhea and abdominal pain. At 26 years of age, he was found to have eosinophilia and abnormal liver function parameters, for which prednisolone therapy was started. He subsequently underwent a liver biopsy and endoscopic retrograde cholangiopancreatography, and received a diagnosis of primary sclerosing cholangitis (PSC). On presentation to our hospital, he was further diagnosed with eosinophilic colitis based on aggravation of diarrhea and severe eosinophilic infiltration in the colonic mucosa. We herein report a rare case of concurrent PSC and eosinophilic colitis.

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We herein report two cases of pregnant women who had chronic hypertension caused by renovascular hypertension due to multiple intrarenal microaneurysms from unknown causes, who had similar clinical courses. During the first pregnancy, both women developed uncontrollable severe hypertension that finally led to superimposed preeclampsia; however, during the second pregnancy, the blood pressure was controlled well, and the clinical courses were uneventful. These cases suggest that an uneventful term delivery may be achieved with adequate blood pressure control in pregnant women with chronic hypertension caused by renovascular hypertension, and a prior eventful clinical course of delivery does not affect the subsequent clinical course.

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QT prolongation and Torsades de Pointes were observed in a 44-year-old woman who had adrenal insufficiency caused by isolated adrenocorticotropic hormone deficiency. Although she had several risk factors for QT prolongation, we concluded that the adrenal insufficiency contributed to the QT prolongation, because the electrocardiographic changes were improved after steroid replacement therapy. It is known that the QT interval in a patient with adrenal insufficiency tends to be extended. However, reports on adrenal insufficiency in which the QT interval was sufficiently prolonged to cause Torsades de Pointes are rare. Clinicians should consider the possibility of adrenal insufficiency in patients with QT prolongation.

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We herein report the case of a 23-year-old man who presented with recurrent pancreatitis and was diagnosed with primary pancreatic extranodal natural killer/T-cell lymphoma, nasal type, involving the right ventricle. The cardiac involvement was screened and confirmed by transthoracic echocardiography (TTE), cardiac magnetic resonance imaging and fluorodeoxyglucose positron emission tomography. Although the patient did not have any cardiac symptoms or evidence of arrhythmia before chemotherapy, he presented with fatal newly developed ventricular tachycardia during the early stages of chemotherapy. The follow-up TTE after his chemotherapy demonstrated markedly decreased thickness of the invaded myocardium, thus suggesting that the myocardium infiltrated by lymphoma cells might become vulnerable to fatal arrhythmia with tumor regression.

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We herein present the first reported case of severe proptosis caused by ocular and periocular hemorrhages in a continuous ambulatory peritoneal dilaysis patient without previous history of trauma. The bleeding tendency caused by uremia and the use of warfarin during uncontrolled high blood pressure were most likely responsible for her ocular and periocular hemorrhages. Appropriate control of blood pressure and adequate self-care education are important for the prevention and treatment of any bleeding complications in uremic patients receiving both maintenance anticoagulation therapy and peritoneal dialysis.

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We herein report an extremely rare adult case presenting with life-threatening pulmonary lymphedema secondary to generalized lymphedema. A 47-year-old woman with generalized lymphedema from her feet to below her chest, had undergone surgical ligation of the thoracic duct and bilateral pleurodesis for the treatment of intractable idiopathic chylothorax three years earlier. Chest computed tomography demonstrated bilateral ground-glass opacities, air-space consolidation and interlobular septal wall thickening, presenting as a crazy-paving appearance predominantly on the gravity side. Bronchoalveolar lavage revealed marked lymphocytosis. She was treated with long-term oxygen therapy with noninvasive positive-pressure ventilation, followed by lymphovenous anastomoses of the lower extremities.

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Malignant pleural mesothelioma (MPM) is associated with a poor prognosis. The combination of cisplatin and pemetrexed has been established as a standard chemotherapy that confers a survival benefit. Because the regimen is sometimes hampered by the renal toxicity of cisplatin and no second-line chemotherapy has yet been established, the strategy of administering a higher total dose of pemetrexed to optimize the regimen could be promising. We herein describe the case of a 69-year-old man with MPM who underwent five cycles of cisplatin plus pemetrexed and exhibited a partial response. Because his serum creatinine increased, pemetrexed maintenance therapy (PMT) was adopted, and 18 cycles were successfully delivered and the patient achieved a complete response. This case suggests that PMT could thus be useful for treating MPM.

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A patient with severe cardiac dysfunction similar to dilated cardiomyopathy expired because of lung squamous cell carcinoma. He was admitted with respiratory failure and was diagnosed with congestive heart failure due to dilated cardiomyopathy based on the chest X-ray, electrocardiography, echocardiography, and coronary angiography. Chest computed tomography showed a mass shadow in the right lower lobe, and the patient was diagnosed with lung squamous cell carcinoma by bronchoscopy. The patient expired, and the autopsy revealed that a myocardial metastasis disrupted the cardiac-conduction system without dilated cardiomyopathy in myocytes. Left bundle branch block caused by myocardial metastasis presumably induced left cardiac dysfunction.

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A 17-year-old Japanese girl visited our hospital for an evaluation of exertional dyspnea. A diagnosis of pulmonary arterial hypertension (PAH) was confirmed based on the findings of right heart catheterization. Detailed questioning revealed a family history of hereditary hemorrhagic telangiectasia (HHT), and a genetic mutation analysis disclosed a mutation in the activin receptor-like kinase 1 gene (ACVRL1). The patient was finally diagnosed with HHT according to the Curaçao diagnostic criteria eight years after the diagnosis of PAH. This case supports previous reports indicating that signs of PAH can be the first manifestation of disease in ACVRL1 mutation carriers.

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Hemophagocytic lymphohistiocytosis (HLH), which is associated with various underlying conditions, is characterized by hypercytokinemia. Because it is frequently lethal, immediate mitigation of the hypercytokinemia is vital to save patients, particularly when treatments for the patient's underlying condition are ineffective on HLH. We herein present a case of Hodgkin lymphoma associated with HLH in which the HLH did not improve even after chemotherapy. We attempted to save the patient using hemoperfusion with a polymyxin B-immobilized fiber column to remove cytokines; following this treatment, the patient rapidly recovered. Hemoperfusion may be a strategic method to rescue intractable HLH patients.

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A 44-year-old man whose platelet count had been at the lower limit of the normal range for years visited the urgent care department of our hospital for treatment of a high fever and severe fatigue. The influenza A virus was detected, and the patient therefore received the intravenous antiviral agent, peramivir. One week later, he developed systemic petechial rashes. A peripheral blood examination showed a markedly decreased platelet count (3.0×10⁹ cells/L), and the bone marrow findings were compatible with a diagnosis of immune thrombocytopenia (ITP). Furthermore, a drug-induced lymphocyte-stimulating test was positive for peramivir. The thrombocytopenia slowly responded to treatment with oral prednisolone. This case suggests that neuraminidase inhibitors, including peramivir, can elicit or worsen ITP.

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A 76-year-old man came to our hospital complaining of hiccups and vomiting lasting for five days. A neurological examination showed dysfunction of cranial nerves V, VII, VIII, IX and X on the left side. Cerebrospinal fluid polymerase chain reaction for varicella zoster virus-DNA was positive. The patient responded well to treatment with intravenous acyclovir and steroids. To the best of our knowledge, this is the first case report of zoster sine herpete presenting with persistent hiccups and vomiting. It is important to keep in mind that herpes zoster can present with symptoms that closely resemble those of intractable hiccups and nausea of neuromyelitis optica. Early detection of the virus is critical for making appropriate treatment decisions.

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Abstract Tacrolimus, a novel immunosuppressant agent, has been widely used in organ transplantation and autoimmune diseases. We herein present a case of neuromyelitis optica spectrum disorder (NMOSD) combined with Sjögren's syndrome (SS) successfully treated with tacrolimus. This patient repeatedly presented with recurrent longitudinally extensive transverse myelitis. Her NMO-IgG and anti-SSA and anti-SSB antibodies were seropositive. Considering the frequency of relapses and severe disability, tacrolimus was initiated after failure of intravenous cyclophosphamide. Her status was steady for over 36 months after tacrolimus treatment. This report indicates that tacrolimus may be a potentially effective immunosuppressant for NMOSD with systemic autoimmune diseases.

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A 57-year-old Japanese woman who had been diagnosed as having entero-Behçet's disease nine years earlier was admitted with a persistent high-grade fever. An Mediterranean fever (MEFV) gene analysis revealed the compound heterozygous P369S-R408Q variant. She was treated with colchicine, and her symptoms immediately improved. Prednisolone (PSL) was added to treat the punched-out ulcers in the terminal ileum, leading to remission. There has been no relapse since the PSL was discontinued. In Behçet's disease patients with MEFV variants, the use of colchicine should therefore be considered in such patients as well as immunosuppressive therapy.

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We herein describe a 71-year-old woman with adult-onset Still's disease (AOSD) who developed fever, myalgia, and pancytopenia. The bone marrow aspiration and muscle biopsy revealed hemophagocytic syndrome (HPS) and inflammatory myopathy with abundant macrophages (IMAM). Immunostained specimens were positive for expression of retinoic acid-inducible gene-I (RIG-I), which recognizes viral RNA in infiltrated mononuclear cells as well as muscle tissues. These findings suggest that RIG-I may be involved in induction of HPS and IMAM in AOSD.

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This study aimed to determine the outcomes of Epstein-Barr virus (EBV)-associated smooth muscle tumors (SMTs) in AIDS patients at King Chulalongkorn Memorial Hospital, Bangkok, Thailand, treated from 2001-2011. Of the 17 patients, there were five men with a median CD4 count of 26 cells/μL. Eight and nine patients had single and multiple sites, respectively. The most common site was the cranial epidura (58.8%). All patients had EBV within the tumor. Seven patients underwent surgery. The median follow-up was one year. The mortality rate was 41.2%. All patients with undetectable HIV viremia survived. This is the largest case series regarding EBV-associated SMTs in AIDS patients with a long follow-up period.

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This report provides information on the clinical characteristics and treatment of three patients with avian influenza A (H7N9) virus treated in Zhejiang Province, China. The infection was characterized by respiratory symptoms, fever, rapid progression, and significant hypoxemia. Laboratory tests showed a low level or decrease in leukocytes. It is recommended that neuraminidase inhibitors be administered at early stage of the disease.

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Some infectious organisms may give rise to acute pancreatitis; brucellosis, however, extremely rarely leads to acute pancreatitis. A 40-year-old man was diagnosed with acute pancreatitis, the etiology of which was determined to be acute brucellosis. The patient was discharged without complications approximately 15 days after the initiation of trimethoprim-sulfamethoxazole and doxycycline treatment. Brucella infections may rarely be complicated by acute pancreatitis. Thus, brucellosis should be remembered in the etiology of acute pancreatitis in regions such as Turkey, where Brucella infections are endemic.

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