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Autoimmune hepatitis in childhood: The role of genetic and immune factors

World Journal of Gastroenterology 2013年第28期19卷 4455-4463页

作者： priscila menezes ferri liu Débora Marques de Miranda Eleonora Druve Tavares Fagundes Alexandre Rodrigues Ferreira Ana Cristina Simoes e Silva Department of Pediatrics Interdisciplinary Laboratory of Medical Investigation Federal University of Minas Gerais Belo Horizonte Minas Gerais 30130-100 Brazil National Institute of Science and Technology of Molecular Medicine (INCT-MM) CNPq-FAPEMIG Federal University of Minas Gerais Belo Horizonte Minas Gerais 30130-100 Brazil

Autoimmune hepatitis (AIH) is a rare chronic inflammatory disease of the liver, which affects a group of patients who lost their immunological tolerance to antigens of the liver. It is clinically characterized by hypergammaglobulinemia, elevated liver enzymes, presence of autoantibodies and histological changes. Although being rare in children, it represents a serious cause of chronic hepatic disease that can lead to cirrhosis and hepatic failure. Clinical findings, exclusion of more common liver disorders and the detection of antibodies antinuclear antibodies, smooth muscle antibodies and anti-LKM1 are usually enough for diagnosis on clinical practice. The pathogenic mechanisms that lead to AIH remain obscure, but some research findings suggest the participation of immunologic and genetic factors. It is not yet knew the triggering factor or factors that stimulate inflammatory response. Several mechanisms proposed partially explain the immunologic findings of AIH. The knowledge of immune factors evolved might result in better markers of prognosis and response to treatment. In this review, we aim to evaluate the findings of research about genetic and immune markers and their perspectives of application in clinical practice especially in pediatric population.

关键词： Autoimmune hepatitis Genetics Clinical practice Immunophenotype

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Diagnostic criteria for autoimmune hepatitis in children: A challenge for pediatric hepatologists

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World Journal of Gastroenterology 2012年第33期18卷 4470-4473页

作者： priscila menezes ferri Alexandre Rodrigues Ferreira Débora Marques Miranda Ana Cristina Sim■es e Silva Division of Pediatric Gastroenterology and Hepatology Department of Pediatrics School Medicine Federal University of Minas Gerais Minas Gerais 30130-100 Brazil National Institute of Science and Technology of Molecular Medicine Faculty of Medicine Federal University of Minas Gerais Minas Gerais 30130-100 Brazil

Autoimmune hepatitis (AIH) is a progressive inflammatory liver disorder that is rare in children and adolescents. AIH has a broad clinical spectrum and a quick response to treatment with corticosteroids and immunosuppressive medication. The available diagnosis criteria have limitations and should be evaluated in pediatric populations. Recently, some studies reported that the 2008 simplified diagnostic criteria for AIH could be used in children with high sensibility and specificity. In addition, the authors reported that globulin and immunoglobulin G levels can be used interchangeably for diagnostic purposes. They also demonstrated that the 2008 simplified criteria fail in identifying patients with fulminant hepatic failure. Here, we discuss the limitations of the use of these criteria in pediatric patients and the requirement of more studies to improve the diagnosis of AIH in children.

关键词：自身免疫性儿童标准诊断肝病肝炎小儿免疫球蛋白G

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