Autoimmune paediatric liver disease

作     者：Giorgina Mieli-Vergani Diego Vergani 

作者机构：Institute of Liver StudiesKing's College London School of Medicine at King's College HospitalLondon SE5 9RSUnited Kingdom 

出 版 物：《World Journal of Gastroenterology》 (世界胃肠病学杂志（英文版）)

年 卷 期：2008年第14卷第21期

页      面：3360-3367页

核心收录：

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

主　　题：Autoimmune hepatitis Autoimmunesclerosing cholangitis Liver transplant Children 

摘      要：Liver disorders with a likely autoimmune pathogenesis in childhood include autoimmune hepatitis(AIH),autoimmune sclerosing cholangitis(ASC),and de novo AIH after liver *** is divided into two subtypes according to seropositivity for smooth muscle and/or antinuclear antibody(SMA/ANA,type 1) or liver kidney microsomal antibody(LKM1,type 2).There is a female predominance in ***1 positive patients tend to present more acutely,at a younger age,and commonly have partial IgA deficiency,while duration of symptoms before diagnosis,clinical signs,family history of autoimmunity,presence of associated autoimmune disorders,response to treatment,and long-term prognosis are similar in both *** most common type of paediatric sclerosing cholangitis is *** clinical,biochemical,immunological,and histological presentation of ASC is often indistinguishable from that of AIH type *** both,there are high IgG,non-organ specific autoantibodies,and interface *** is made by *** with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates,times to normalization of biochemical parameters,and decreased inflammatory activity on follow up liver ***,the cholangiopathy can *** may be evolution from AIH to ASC over the years,despite *** novo AIH after liver transplantation affects patients not transplanted for autoimmune disorders and is strikingly reminiscent of classical AIH,including elevated titres of serum antibodies,hypergammaglobulinaemia,and histological findings of interface hepatitis,bridging fibrosis,and *** classical AIH,it responds to treatment with prednisolone and *** novo AIH postliver transplantation may derive from interference by calcineurin inhibitors with the intrathymic physiological mechanisms of T-cell maturation and *** this condition is a distinct entity or a form of atypical rejection