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Autoimmune Liver Disease in Children

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The goal of the Working Group is to recommend principles that both improve the nutritional quality of foods marketed to children and can be feasibly implemented by industry with sufficient time to accomplish reformulation. The Working Group recognizes that, if the proposed nutrition principles were fully implemented by industry as proposed, a large percentage of food products currently in the marketplace would not meet the principles. The Working Group also recognizes that, while it may be feasible to reformulate some food products to meet the proposed nutrition principles, in many cases reformulation would require substantial changes in the nutritional profile of the food, such as. | Distinguished Academician Lecture 2001 Autoimmune Liver Disease in Children G Mieli-Vergani PhD FRCP FRCPCH D Vergani PhD FRCPath FRCP Abstract Autoimmune liver disorders are characterised by an inflammatory liver histology circulating non-organ specific autoantibodies and increased levels of immunoglobulin G IgG in the absence of a known aetiology. They respond to immunosuppressive treatment which should be instituted as soon as diagnosis is made. Liver disorders with a likely autoimmune pathogenesis include autoimmune hepatitis AIH and autoimmune sclerosing cholangitis ASC . Two types of AIH are recognised 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 both. LKM1-positive patients tend to present more acutely at a younger age and commonly have immunoglobulin A IgA deficiency while duration of symptoms before diagnosis clinical signs family history of autoimmunity presence of associated autoimmune disorders response to treatment andlong-termprognosis are similar in both groups. The most common type of paediatric sclerosing cholangitis is ASC. The clinical biochemical immunological and histological presentation of ASC is often indistinguishable from that of AIH. In both there are high IgG non-organ specific autoantibodies and interface hepatitis. Diagnosis is made by cholangiography. Children with ASC respond to immunosuppression satisfactorily and similarly to AIH in respect to remission and relapse rates times to normalisation of biochemical parameters and decreased inflammatory activity on follow-up liver biopsies. However the cholangiopathy can progress and there may be an evolution from AIH to ASC over the years despite treatment. Whether the juvenile autoimmune form of sclerosing cholangitis and AIH are 2 distinct entities or different aspects of the same condition remains to be elucidated. Ann Acad Med Singapore 2003 32 239-43 Key .

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