XXXX and related disorders.

@entity1 @entity97 @entity82 @entity1956 @entity409 @entity8693 @entity2360 @entity3619 @entity99 @entity792
@entity3619 in @entity1 should be suspected if clinical features and @entity409 tests persist for over one month following an episode of @entity99 , in @entity1 who present with relapsing @entity99 , or those presenting coincidentally with features of @entity409 . Specific investigation must be undertaken to define aetiology. For example, @entity1956 , @entity2360 and @entity8693 must be excluded. Early histological diagnosis by an experienced histopathologist is mandatory. @entity3619 requires no therapy, but careful follow-up is desirable, especially for @entity1956 . If the histological appearances are those of @entity3619 , distinction between @entity97 -associated and autoimmune varieties is necessary. Autoimmune CAH in @entity1 differs from that in adults in that the onset is often acute, response to immunosuppressants usually favourable, and withdrawal of therapy may be successful, especially if diagnosis is established early before serious liver damage occurs. Evidence is presented to suggest that autoimmune CAH is associated with a genetic predisposition to @entity792 , characterized by both antigen-specific and antigen-independent T suppressor cell defects. An antibody-dependent non-T cell cytotoxicity operates against liver cell surface antigens. @entity97 CAH, on the other hand, may respond poorly to immunosuppressants and appropriate therapeutic regimens are not defined. There is some evidence to suggest that early diagnosis and institution of therapy in autoimmune CAH may lessen the incidence of @entity82 on follow-up. Further studies to understand the interaction between hepatocytes, inflammatory cells and non-parenchymal cells in the process of hepatic @entity82 may provide the means of active intervention in this area in the future.
