[Giant cell hepatitis in adults].
Differential diagnosis of progressive cholestatic liver disease includes postinfantile giant-cell hepatitis, particularly in cases with negative serology for hepatitis viruses and in the absence of an intake of potentially hepatotoxic drugs. Diagnosis is made by the histologic demonstration of syncytial giant cells in liver. To the knowledge of the authors, some 70 cases have been published so far; however, the true prevalence of the disease appears to be much higher than commonly assumed. Hepatocellular giant cells, the only diagnostic marker, may be expressed in low copy numbers, and many cases thus easily escape recognition. Some 50% of the published cases are positive for autoantibodies in serum or combined with other features of autoimmunity. A correct diagnosis is important in view of the unfavorable prognosis with a rapidly progressive course in about half the cases. Fulminant liver failure and late cirrhotic stages are candidates for liver transplantation. After transplantation no recurrence of giant-cell hepatitis has been observed. Cases positive for autoantibodies have been successfully treated with corticosteroids; cholestatic forms appear to respond to ursodeoxycholic acid. Etiology of the disease is not settled. Some authors claim aberrant immunoreactivity from various causes to be responsible for the disease, others have presented evidence in favor of a paramyxovirus infection. In any case of cholestasis of unknown origin, serology for paramyxoviruses and a liver biopsy are recommended.
Institut für Pathologie der Universität, Basel.
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Bianchi
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Terracciano
D001323:Autoantibodies / Q000302:isolation & purification; D001327:Autoimmune Diseases / Q000175:diagnosis; D003937:Diagnosis, Differential; D015726:Giant Cells / Q000648:ultrastructure; D006501:Hepatic Encephalopathy / Q000209:etiology; D006505:Hepatitis / Q000150:complications / Q000175:diagnosis* / Q000473:pathology; D006521:Hepatitis, Chronic / Q000175:diagnosis; D006801:Humans; D008854:Microscopy, Electron
D001323:Autoantibodies
Die adulte Riesenzellhepatitis.