Autoimmune Liver Diseases in Children. Part I

Česká verzia

Authors: P. Dědek
Authors place of work: Dětská klinika Fakultní nemocnice, Hradec Králové, přednosta prof. MUDr. M. Bayer, CSc.
Published in the journal: Čes-slov Pediat 2010; 65 (12): 694-705.
Category: Přehledový článek

Summary

Autoimmune liver diseases form a group of chronic inflammatory illnesses associated with serious prognosis. Autoimmune hepatitis, autoimmune sclerosing cholangitis, and primary sclerosing cholangitis are the most common entities seen during childhood. Clinical findings of liver disorder are not typical, even the disease course may be asymptomatic. We can frequently found other illnesses with autoimmune pathogenesis in patients or their first-degree relatives. Laboratory diagnostics is based on detection of different types of autoantibodies, hypergammaglobulinemia, and elevated IgG. The definitive diagnosis is provided by liver biopsy and imaging of biliary tree.

Early and proper diagnosis followed by prompt therapy affects the patient’s prognosis significantly. Corticosteroids alone or paired with azathioprine have proven efficacy, administration of ursodeoxycholic acid is still a matter of debate. Chronic liver failure requiring liver transplant occurs despite treatment in many patients, especially those with sclerosing cholangitis.

Key words:
autoimmune liver disease, autoimmune hepatitis, primary sclerosing cholangitis, autoimmune sclerosing cholangitis, primary biliary cirrhosis, childhood, overlap syndrome

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