Eitaro Hiejima

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Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of immune dysregulation and is classified as primary or secondary according to the underlying aetiology. The treatment strategies recommended for these two groups differ substantially; however, it is thought to be impossible to predict the underlying causes of HLH using conventional(More)
ited by fibrous bands in which we noted significant ductular proliferation and a lymphohistiocytic inflammatory infiltrate with some eosinophils and neutrophils, as well as plasma cells and hepatocytic changes such as focal foamy degeneration, parenchymal cytolysis and multinucleation. We identified a medium-calibre artery with transmural neutrophilic(More)
We describe two previously healthy children who had multiple ecchymoses several days after acute infection. In both cases, the prothrombin time (PT) and the activated partial thromboplastin time (APTT) were prolonged. Further examinations revealed the presence of lupus anticoagulant (LA), phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT), and(More)
AIM In order to clarify the sources of chronic HBV (hepatitis B virus) infection in children after the implementation of an "at-risk" strategy in Japan, chronically infected children were assessed. In addition, chronically infected children born to HBsAg-negative mothers and their family members were assessed to identify the sources of HBV transmission. (More)
BACKGROUND The administration of hepatitis B immunoglobulin followed by hepatitis B vaccine can result in a protective efficacy of almost 90% in mother-to-child transmission of hepatitis B virus (HBV). However, little is known about immunity against HBV infection in children after immunoprophylactic treatment. We tried to assess the association between(More)
Recently, the prevalence of genotype A in patients with acute hepatitis B virus (HBV) infection has markedly increased in Japan. We encountered a 1-year-old infant who was infected with HBV genotype A and became an HBV carrier. His grandfather was identified as an HBV carrier; however, the grandfather was not aware of chronic HBV infection. This was a case(More)
BACKGROUND AND AIM Although the diagnostic scoring system of autoimmune hepatitis (AIH) has been used, these criteria are intended mainly as research tools and are complicated to apply. To resolve these difficulties and allow quick diagnosis, a simplified scoring system was proposed in 2007. We retrospectively compared the simplified AIH scoring system with(More)
BACKGROUND The mechanisms of liver damage and steatosis in Wilson's disease (WD) presenting accumulation of copper generating oxidants remain unclear. Recent studies have shown that peroxisome proliferator-activated receptors (PPARs), in particular PPARs α and γ, regulate fat content of the liver together with the anti-oxidant and anti-inflammation systems.(More)
Familial hemophagocytic lymphohistiocytosis type 3 (FHL3) is a genetic disorder that results in immune dysregulation. It requires prompt and accurate diagnosis. A natural killer (NK) cell degranulation assay is often used to screen for FHL3 patients. However, we recently encountered two cases of late-onset FHL3 carrying novel UNC13D missense mutations: in(More)
BACKGROUND AND AIM Acute gastrointestinal graft-versus-host disease (GI-GVHD) is a major cause of morbidity and mortality after hematopoietic stem cell transplantation (HSCT). There are very few studies on specific endoscopic findings in pediatric acute GI-GVHD. The aim of this retrospective case-control study was to elucidate the characteristic endoscopic(More)