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| 1 | Autoimmune hepatitis in childhood: The role of genetic and immune factors显示文摘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. | Priscila Menezes Ferri Liu Débora Marques de Miranda Eleonora Druve Tavares Fagundes Alexandre Rodrigues Ferreira Ana Cristina Simoes e Silva | 2013 | World Journal of Gastroenterology2013，19，28： | 3 |
| 2 | Juvenile autoimmune hepatitis: Spectrum of the disease显示文摘Juvenile autoimmune hepatitis(JAIH) is a progressive inflammatory liver disease, affecting mainly young girls, from infancy to late adolescence, characterized by active liver damage, as shown by high serum activity of aminotransferases, by elevated immunoglobulin G levels, high titers of serum non organ-specific andorgan-specific autoantibodies, and by interface hepatitis on liver biopsy. It is a multifactorial disease of unknown etiology in which environmental factors act as a trigger in genetically predisposed individuals. Two types of JAIH are identified according to the autoan-tibody panel detected at diagnosis: AIH-1, characterized by the presence of anti-smooth muscle antibody and/or antinuclear antibody and AIH-2, by anti-liver-kidney microsomal antibody type 1 and/or by the presence of anti-liver cytosol type 1 antibody. Epidemiological distribution, genetic markers, clinical presentation and pattern of serum cytokines differentiate the two types of AIH suggesting possible pathogenetic mechanisms. The most effective therapy for AIH is pharmacological suppression of the immune response. Treatment should be started as soon as the diagnosis is made to avoid severe liver damage and progression of fibrosis. The aim of this review is to outline the most significant and peculiar features of JAIH, based largely on our own personal database and on a review of current literature. | Giuseppe Maggiore Silvia Nastasio Marco Sciveres | 2014 | World Journal of Hepatology2014，6，7： | 2 |
| 3 | 青少年自身免疫性肝病患者的临床观察显示文摘目的分析青少年自身免疫性肝病患者的临床病理学特征及治疗转归,提高对该病的认识。方法回顾性分析2016年9月至2019年10月于上海交通大学医学院附属仁济医院消化科住院及门诊随访的32例青少年自身免疫性肝病患者的分型、临床特点、生物化学指标、肝脏病理、治疗效果及药物不良反应。结果32例患者中自身免疫性肝炎(AIH)28例(1型AIH 25例,2型AIH 3例),自身免疫性硬化性胆管炎(ASC)4例。临床表现为急性肝炎型起病7例(21.9%),慢性隐匿型起病22例(68.8%),肝硬化及其并发症型起病3例(9.4%)。有13例患者单用激素治疗,2例因治疗时间未满1年故疗效是否应答不确切,1例不完全应答,10例完全应答,其中1例已成功停药,2例在完全应答缓解数年后复发,在激素基础上加用免疫抑制剂(这2例归为联合治疗)。21例根据病情应用激素联合免疫抑制剂治疗,11例不完全应答,10例完全应答。结论青少年AIH的临床表现多样,诊断困难,但对激素单药或联合硫唑嘌呤的免疫抑制治疗反应良好。AIH可转归至ASC,应定期对AIH患儿行磁共振胰胆管造影(MRCP)或逆行胰胆管造影(ERCP)检查以排查ASC,尤其是出现胆汁淤积症状者。 | 李达之 连敏 肖潇 苗琪 王绮夏 马雄 | 2020 | 国际消化病杂志2020，40，3： | 1 |
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