Aim In chronic hepatitis C, iron might play an important role as a hepatotoxic co-factor. Therefore, venesection, a standard treatment for hemochromatosis, has been proposed as an alternative for patients who respond poorly to anti-viral therapy. To improve our understanding of iron-induced hepatotoxicity, we compared the responses to venesection between patients with chronic hepatitis C and those with HFE-hemochromatosis.
Methods Fourteen Japanese patients with chronic hepatitis C and eight Italian patients with HFE-hemochromatosis underwent repeated venesection with a serum ferritin endpoint of 20 and 50 ng/mL, respectively. Serum iron indices and liver function tests were measured in pre- and post treatment blood samples from each patient. Body iron stores were calculated using the removed blood volume.
Results In both patients with hepatitis and hemochromatosis, serum ferritin, aminotransferase and hepcidin 25 were reduced after venesection. The serum aminotransferase activity, but not the serum ferritin level, was predictive of effective iron removal treatment. Hepcidin regulation was set at an inappropriately low level in hemochromatosis patients (11.1 ± 9.2 ng/mL), but not so in hepatitis patients (30.7 ± 14.5 ng/mL). Inversely, the estimated body iron stores of hemochromatosis patients were 5,960 ± 2,750 mg, while those of hepatitis patients were 730 ± 560 mg. Judging from the liver enzyme reduction ratio, patients with hepatitis seemed to be more sensitive to iron hepatotoxicity than hemochromatosis patients.
Conclusion Even though the threshold of iron hepatotoxicity and benefit of its removal differ between patients with chronic hepatitis C and those with HFE-hemochromatosis, venesection is a valid choice of treatment to reduce liver disease activity in both diseases.

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Objective A drug eluting stent is often used for high-risk patients with complications such as diabetes mellitus (DM) and hemodialysis (HD), however the factors to predict restenosis after paclitaxel-eluting stent (PES) placement have not been reported to date.
Methods Between May 2007 and August 2009, 165 consecutive patients (231 stents) received PES in our hospital. Stent diameter and length were determined by the use of intravascular ultrasound (IVUS). All patients continued to take 2 types of anti-platelet agents (aspirin and Clopidogrel or Ticlopidine). Ninety percent of the subjects received a follow-up coronary angiogram 6 months later.
Results Underlying diseases were hypertension in 75%, hyperlipidemia in 78% and DM in 60% (15% on insulin), and 14% of the subjects received HD. Eighty-three percent of the patients had orally taken Statin, 85% ACE/ARB and 68% had beta blockers. Mean length and diameter of PES were 21.6 ± 7.2 mm and 2.9 ± 0.3 mm, respectively. Target lesion revascularization (TLR) rate 6 months after PES placement was 14.6% overall. In HD patients TLR was 43%, hypertension 15.0%, hyperlipemia 12.4%, DM with oral medication 12.5%, DM with insulin 12.0%, respectively. In multivariate analysis, HD was an independent risk factor for TLR (p=0.0001, OR: 6.61, 95% C.I.: 2.34-18.6).
Conclusion HD had the greatest influence on TLR after PES even though risk factors were well controlled. It is necessary to develop new PCI techniques and stents that are useful for HD patients.

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Objective Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia associated with substantial morbidity and significant mortality. The familial aggregation of AF elsewhere in the world has been documented. This investigation sought to evaluate familial aggregation of lone AF in the Chinese population.
Methods The study population included 382 unrelated patients with lone AF, and their 6,856 relatives. The controls were 15,507 age-sex-matched individuals from the general population. The prevalence of AF in each class of relatives was compared to that in each subgroup of the age- and sex- comparable control individuals.
Results The relatives of patients with lone AF had a significantly increased risk of the arrhythmia as compared to the general population. The relative risk (95% confidence intervals) of AF for relatives compared to the general population was: 37.36 (12.71-109.9) for sons, 166.6 (22.06-1258) for daughters, 27.39 (14.63-51.26) for brothers, 24.49 (14.01-42.83) for sisters, 4.87 (2.84-8.35) for mothers, and 4.78 (3.00-7.59) for fathers.
Conclusion These findings provide evidence suggesting that there is a significant familial aggregation of lone AF among Chinese families and a Mendelian genetic component involved in the pathogenesis of this prevalent disorder.

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Objective Oxidant stress is thought to be involved in the establishment of idiopathic interstitial pneumonia (IIP). Thioredoxin 1 (TRX1) plays a role as a strong antioxidant in vivo, suggesting that TRX1 may be involved in the pathogenesis of IIPs. However, there is no report on TRX1 levels in the sera of IIPs. In addition, TRX1 expression in the lungs of non-specific interstitial pneumonia (NSIP) and cryptogenic organizing pneumonia (COP) patients also has not been reported. Here, we investigated whether or not TRX1 levels are altered in the lungs and sera of patients with idiopathic pulmonary fibrosis (IPF), NSIP, and COP.
Methods Immunohistochemical analysis was performed to examine the expression of TRX1. TRX1 levels in sera were measured using an ELISA kit.
Results TRX1 was expressed in the bronchiole-alveolar epithelium, especially with regenerative or metaplastic feature, and in alveolar macrophages in usual interstitial pneumonia (UIP) and fibrotic NSIP. TRX1 was weakly expressed in the lungs of cellular NSIP and COP. TRX1 producing cells in UIP (n=16), fibrotic NSIP (n=15), cellular NSIP (n=4), and COP (n=5) were significantly increased when compared to nonsmokers (n=7). TRX1 producing cells in UIP and fibrotic NSIP were significantly increased when compared to cellular NSIP and COP. TRX1 levels in the sera of the patients with IPF (n=32; 74.2 ± 7.5 ng/mL), fibrotic NSIP (n=7; 82.5 ± 18.4 ng/mL), cellular NSIP (n=3; 62.2 ± 3.2 ng/mL) and COP (n=17; 88.8 ± 19.7 ng/mL) were significantly higher than those of control subjects (n=74; 35.3 ± 2.7 ng/mL). Furthermore, TRX1 levels in the sera of IPF patients who later showed acute exacerbation (n=7; 106.6 ± 16.3 ng/mL) were significantly higher than those of IPF patients without acute exacerbation (n=25; 65.1 ± 7.6 ng/mL).
Conclusion Overproduction of TRX1 in the lungs and sera may play an important role in the pathogenesis of IIPs.

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Objective In the advanced stage of Parkinson's disease (PD), motor fluctuation is a frequent and a disabling problem. Despite its importance, motor fluctuation has received little scientific analysis probably due to limitation in objective assessment. Here, we focused on gait disorders to estimate motor fluctuation in daily activities.
Patients and Methods Using a new device, the portable gait rhythmogram, we recorded gait rhythm continuously over 24 hours in 22 patients with PD and in 11 normal controls, for quantitative evaluation of motor fluctuation. The duration of one gait cycle was measured.
Results Continuous 24-hour recording identified changes in gait rhythm, which correlated with fluctuation of PD symptoms. Different motor fluctuations were observed; a shift to a faster gait cycle was noted in patients with short-step walking, festination or freezing of gait, whereas a shift to a slower gait cycle was observed in patients with bradykinesia or instability.
Conclusion Characterization of motor fluctuation using this device could help in the selection of appropriate anti-PD medications.

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Objective The frequency of autosomal dominant cerebellar ataxia (ADCA) varies between different regions of Japan. This is the first report on the prevalence of ADCA subtypes in Aomori, Japan.
Methods and Patients Sixty-five familial spinocerebellar ataxia (SCA) patients and 15 sporadic SCA patients were genetically examined. For only the SCA2 patients (n = 8), the magnetic resonance imaging (MRI) data were analyzed in detail.
Results Spinocerebellar ataxia (SCA) type 6 was often observed (77.7% of cases), with SCA2 (10.6% of cases) being the next most common form. In contrast, only one of the eighty patients had SCA1. Among the 15 sporadic SCA patients, genetic mutations for SCA2, SCA6, SCA17, and SCA31 were identified, indicating that ADCAs should be considered in sporadic cases of ataxia. Furthermore, in SCA2 cases, brainstem atrophy, pontine midline linear hyperintensity, and atrophy of the frontal lobes were frequently observed using MRI.
Conclusion The present data indicate that the prevalence of ADCA in Aomori differs from other prefectures in the Tohoku District. MRI findings are very similar between SCA2 and multiple system atrophy (MSA), and thus care must be taken to prevent the misdiagnosis of sporadic SCA2 as MSA.

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Background Part of Aeromonas bacteremia is polymicrobial infection. However, a clinical comparison of monomicrobial and polymicrobial Aeromonas bacteremia has not hitherto been reported.
Methods A retrospective analysis of medical records of patients with Aeromonas bacteremia at three large referral hospitals in Taiwan for an 8-year period (2001-2008) was conducted.
Results There were 154 patients with monomicrobial Aeromonas bacteremia and 62 patients with polymicrobial Aeromonas bacteremia. In the polymicrobial infections, E. coli was the most common combined pathogen (42%), followed by Klebsiella spp. (24%) and Enterobacter spp. (16%). Multivariate logistic regression analysis revealed solid cancer as the risk factor for polymicrobial Aeromonas bacteremia, with male gender and cirrhosis as risk factors for monomicrobial Aeromonas bacteremia. However, of all types of solid cancer, hepatoma was associated with monomicrobial Aeromonas bacteremia. APACHE II score was the most important prognostic factor in both groups.
Conclusion Aeromonas bacteremia in patients with cirrhosis or male gender tended to be monomicrobial. Polymicrobial Aeromonas bacteremia was associated with solid cancers. In either polymicrobial or monomicrobial Aeromonas bacteremia, prognosis could be predicted according to disease severity measured by APACHE II score.

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Introduction Sivelestat is neutrophil elastase inhibitor, which is widely used in Japan for the treatment of acute lung injury. However, the clinical efficacy of the medication has not been convincingly demonstrated.
Methods We conducted a systematic review and meta-analysis of randomized controlled trials on sivelestat for the treatment of acute lung injury and acute respiratory distress syndrome. Studies were identified using MEDLINE, EMBASE, Cochrane library, conference proceedings, and references of included studies. Authors were contacted if necessary. ICHUSHI, the Japanese database for medical literature and conference proceedings was also used for the search, since many studies on sivelestat were published in Japanese language and not registered in major databases such as MEDLINE. The primary outcome was mortality within 28-30 days after randomization. Relative risks were pooled with the random effect model.
Results 8 trials were included in the analysis. There was no difference in mortality within 28-30 days after randomization (relative risk 0.95, 95% confidence interval 0.72 to 1.26). Subgroup analysis conducted only on studies conducted in Japan showed the same result (0.59, 0.28 to 1.28). There was no difference in mechanical ventilation days (standardized mean difference -0.43, -1.12 to 0.27), but sivelestat was associated with a better short term PaO₂/FiO₂ ratio (0.30, 0.05 to 0.56). Heterogeneity was not significant for the main analysis and funnel plot did not suggest publication bias.
Conclusion Sivelestat was not associated with decreased mortality, even when including studies published in Japanese language.

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Objective Clostridial sepsis has a very poor prognosis, owing to the life-threatening combination of shock and acute massive hemolysis. No papers have described the clinical features of clostridial sepsis cases in Japan. Therefore, we retrospectively examined the clinical features of patients with systemic inflammatory response syndrome (SIRS) from whose blood cultures Clostridium perfringens was isolated.
Subjects and Materials Blood samples were obtained from SIRS patients and cultured between January 1, 2001 and June 30, 2009. The samples were retrospectively reviewed, and 18 samples were positive for C. perfringens. The medical records of these 18 patients were reviewed for age, gender, underlying disease, past illnesses, results of physical and laboratory testing, and radiographic data.
Results All patients were diagnosed with SIRS. Fifteen patients (83.3%) were >65 years old -mean age, 75±2 years (range, 59-88 years). There were more men (13) than women (5). The blood cultures were obtained from patients in various wards: tertiary care center (8), emergency room (5), surgical ward (4), and medical ward (1). Hepatobiliary tract diseases such as gallbladder stones and hepatic carcinoma were the most frequent underlying diseases (8). Five patients died, resulting in an overall mortality rate at 30 days of 27%. In the non-survival group, patients presented with septic shock (4) and gas-forming infection (2), and with significantly lower fibrinogen levels than those in the survival group. Septic shock at initial presentation was significantly associated with 30-day mortality for C. perfringens infection.
Discussion and Conclusion There were no specific characteristics among clinical features of C. perfringens infection accompanied with SIRS. This may indicate that, in emergency rooms, diagnosing and initiating appropriate treatment for C. perfringens infection may be considerably difficult. It is important to be especially vigilant in identifying patients with C. perfringens infection underlying SIRS, and accompanied by shock.

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Objective In our study, in addition to evaluating the relation between Pro-Brain natriuretic peptide (Pro-BNP), myoglobin and creatinine kinase (CK) levels and morbidity and mortality, we aimed at identifying the demographic characteristics of patients admited to emergency service after exposure to high electrical voltage.
Methods In this prospective study, 48 emergency service patients exposed to high electric voltage were included; 19 healthy individuals were included as the control group. Their blood samples and electrocardiographies (ECG) were taken at the time of recourse upon their written approval. Demographic data and laboratory data were checked and compared among the patient group. We investigated the correlation between inpatients that had special clinical manifestations (escaratomy, fasciotomy, exitus, myoglobulinuria, third-degree burn, arrhythmia and etc.) and serum Pro-BNP, myoglobin and CK levels.
Results When serum Pro-BNP, myoglobulin and CK levels were compared for the special clinical manifestations; the pro-BNP levels were statistically significantly higher in patients who had arrhythmia than in those without arrhythmia, and significantly higher in patients who died than in those who healed (respectively p=0.002 and p=0.007). In contrast, serum CK and myoglobin levels were not statistically significant. The serum CK and myoglobin levels were statistically significantly higher in patients who had third-degree burn than the others (p<0.001).
Conclusion Serum pro-BNP level is a marker that can be used for mortality and morbidity with patients exposed to high voltage electrical injuries.

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Some drugs including streptokinase have been reported to precipitate Guillain-Barré syndrome. We report a 70-year-old man with acute anterior myocardial infarction who developed Guillain-Barré syndrome seven days after thrombolytic therapy with streptokinase.

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We herein report a case of early stage ampullary cancer, treated by endoscopic papillectomy, in which tumor extension was confined to the mucosa and adjacent epithelium of the glands in Oddi's sphincter. A 77-year-old man underwent screening esophagogastroduodenoscopy, which revealed a mass in the papilla of Vater, which was well-differentiated adenocarcinoma as proven by biopsy. The tumor was diagnosed as T1 and endoscopic papillectomy was performed. Histological examination showed adenocarcinoma limited to the mucosa of the common channel and continuative epithelium of the neighboring glands in Oddi's sphincter. No signs of recurrence have been observed during a follow-up of 23 months.

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Retroperitoneal fibrosis is an uncommon collagen vascular disease of unknown etiology, characterized by the replacement of normal retroperitoneal tissue with fibrosis and/or chronic inflammation usually surrounding the abdominal aorta and the iliac arteries and extending into adjacent anatomic structures. No cases of acute coronary syndrome in the setting of retroperitoneal disorder have been published as yet. We report a 37-year-old man with a 14-year history of type I diabetes mellitus who was admitted to the endocrinology department for a routine check up and glycemic re-equilibration and who was later diagnosed to have an idiopathic retroperitoneal fibrosis. The patient presented during his hospitalisation with a non ST elevation myocardial infarction caused by an isolated thrombus located inside the left main coronary artery successfully treated with manual thrombectomy.

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A 29-year-old woman with refractory hypertension who previously suffered from subarchnoid hemorrhage visited our facility. The diagnosis of renovascular hypertension due to fibromuscular dysplasia was made based on a high level of plasma renin activity (PRA) and aldosterone concentration (AC), and computed tomographic image of bilateral renal artery stenosis/obstruction. Angioplasty, which could be performed only to the left renal artery, failed to regain suffcient BP control. The addition of eplerenone, an aldosterone receptor blocker, to the conventional antihypertensive drugs successfully and safely lowered BP and preserved the renal function despite the persistence of high PRA and AC values.

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A 60-year-old man presented with chest discomfort with fever and high C-reactive protein (CRP). Chest computed tomography (CT) disclosed a mediastinal soft tissue swelling originating from the aortic arch, and gallium-67 single-photon emission CT revealed intense uptake in the same region. We initially suspected mediastinitis and/or a thoracic aortic infection. Antibiotics improved his symptoms and CRP levels. However, a follow-up CT scan 33 days later, revealed an aortic arch aneurysm and the patient was diagnosed with infective aortic aneurysm. Here, we report a rare case of a rapidly progressing aneurysm of infected aorta aortic infection with pseudoaneurysm formation.

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Lipoprotein lipase (LPL) and hepatic triglyceride lipase (HTGL) enhance the hydrolysis of triglycerides (TG) transported by chylomicron (CM) and very-low-density lipoprotein (VLDL). We report a case of severe hyperchylomicronemia with high levels of remnant lipoprotein and total cholesterol (T-Chol) in a 15-year-old boy. Precise examination of the lipid profile showed decreased activities of both LPL and HTGL, although the protein mass for LPL and HTGL were maintained. In addition, bezafibrate treatment effectively ameliorated hypertriglyceridemia in this case. This is the first case of hyperchylomicronemia with decreased activities and unaffected protein masses for both LPL and HTGL, without overt immuno-dysfunction.

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Although reduced intestinal blood flow causing barrier dysfunction and endotoxemia is well documented in the pathogenesis of heat stroke (HS), complications of the gastrointestinal tract are less appreciated in HS patients. Herein, we report the case of a young man with exertional HS complicated with colon perforation. Acute abdomen, bloody diarrhea, dilated bowel loop, and unexplained shock should be considered as warning signs of colonic ischemia and consequent perforation. Early recognition is the key factor for improving the outcome of HS patients complicated with colon perforation.

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In addition to displaying geographic variation, focal segmental glomerulosclerosis (FSGS) has become the commonest cause of the nephrotic syndrome seen in adults in recent years. Secondary FSGS in particular, is observed when glomerular workload is increased. Polycythemia vera (PV) is a hematological disease characterized by abnormal proliferation in the erythroid series. The number of case reports belonging to glomerulonephritis secondary to PV is limited. In the literature, there are few reports of FSGS. One study pointed out that the presence of normoalbuminemia was detected in patients with FSGS secondary to hyperfiltration when there was nephrotic proteinuria. Here, we report a case of FSGS following a course with normoalbuminemia despite nephrotic range proteinuria developing secondary to PV. Our case is the first report in the literature with thes characteristics.

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A 73-year-old female hemodialysis patient experienced fever, shortness of breath on effort, and chest discomfort. A decrease in breath sounds in the right lung field, leukocytosis, elevated CRP level, and a right massive pleural effusion were observed. The patient was diagnosed with bacterial pleuritis based on leukocyte-predominant exudative pleural effusion, and treated with ceftriaxone. Her symptoms, however, were not improved, so thoracic drainage was attempted. Campylobacter species were isolated from cultured pleural fluid samples, and Campylobacter jejuni subspecies jejuni was detected on the multiplex PCR assay. The antibiotic was therefore changed to minocycline following pazufloxacin, and her symptoms were improved.

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A 20-year-old female diagnosed as idiopathic pulmonary arterial hypertension at 7 years of age was referred with worsening dyspnea and chest pain. Several imaging studies and right cardiac catheterization showed multiple stenoses in the peripheral pulmonary arteries with severe pulmonary hypertension and multiple systemic arterial stenoses lacking in systemic hypertension. No evidence of inflammatory or autoimmune disease was detected. Fibromuscular dysplasia was clinically diagnosed because of the narrowed systemic and pulmonary arterial stenoses which included dilatation and aneurysms that appeared similar to a string of beads. Treatment with sildenafil yielded a temporary improvement in her disease state.

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Myeloid sarcoma, formerly termed granulocytic sarcoma or chloroma, consists of neoplastic granulocytic precursors and myeloblasts. Isolated chloromas (granulocytic sarcomas) are rare tumors. Spinal complications of chloromas, such as cord compression secondary to epidural tumor or cauda equine syndrome have been described but are rare. We herein report two cases with spinal granulocytic sarcomas in non-leukemic patients. The case of a previously healthy 22-year-old man diagnosed with multiple spinal granulocytic sarcomas with no evidence of bone marrow or other hematological involvement is described. And, a 43-year-old woman diagnosed cervical spinal granulocytic sarcoma with no evidence of bone marrow or other hematological involvement is described. The tumor was totally removed by microsurgery. The histopathological examination was consistent with granulocytic sarcoma. Granulocytic sarcoma should be considered in the differential diagnosis of an epidural mass in patients with or without acute leukemia, because early diagnosis followed by appropriate combined chemotherapy and radiation may obviate surgical intervention and eventually prevent leukemic transformation.

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Severe systemic Geotrichum capitatum (G. capitatum) infection is rare, especially in Japan. G. capitatum infection has been reported mainly in immunocompromised patients and the prognosis is poor with a mortality rate of approximately 50-75%. Here, we report a Japanese case of systemic G. capitatum infection in a severe neutropenic patient who was receiving chemotherapy for acute myelogeneous leukemia with multilineage dysplasia. G. capitatum was isolated from blood cultures, and also formed multiple nodular lesions in lung fields. The infection was successfully cured with a combination of amphotericin B, itraconazole, and voriconazole.

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We present a patient with Epstein-Barr virus (EBV)-positive cytotoxic peripheral T-cell lymphoma, not otherwise specified (PTCL-NOS) who was successfully treated using only L-asparaginase. A 46-year-old Japanese man was diagnosed with EBV-positive cytotoxic PTCL-NOS. Although he underwent chemotherapy using multiple agents, he relapsed with hemophagocytic syndrome. L-asparaginase treatment was initiated at 6,000 U/m² on days 1, 3, 5, 10, and 12 together with prednisolone at 1 mg/kg. Although he developed grade 2 liver dysfunction and grade 2 coagulopathy, the patient achieved complete response status. Finally, he underwent allogeneic bone marrow stem cell transplantation, and he is currently still alive without disease at 24 months after the start of L-asparaginase therapy.

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We report herein the case of a 37-year-old man who developed probable progressive multifocal leukoencephalopathy (PML) following an umbilical cord blood transplant. The patient showed favorable clinical, neuroradiological and virological responses after treatment with mefloquine, an anti-malarial drug. Mefloquine may offer some benefits as a treatment for PML in patients with or without human immunodeficiency virus type-1 infection. This report highlights the need to gather sufficient data to confirm the efficacy of mefloquine against this devastating viral disease of the central nervous system.

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Diphenylhydantoin (DPH) therapy, often used in treating epileptic seizures, can cause anemia in some patients. A 26-year-old female suffered from convulsions due to encephalitis and was placed on DPH therapy. About two months after the initiation of DPH therapy, her hemoglobin level was 3.8 g/dL. Her anemia improved after the discontinuation of DPH, confirming that the anemia was caused by DPH. Pure red-cell aplasia (PRCA) combined with hemolytic anemia was indicated by results such as erythroid aplasia, an increased LDH level, and a decreased haptoglobin level. PRCA complicated by hemolytic anemia could be responsible for anemia associated with DPH.

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