FAMILIAL "GIANT-CELL HEPATITIS" IN INFANCY. CLINICAL, PATHOLOGIC, AND GENETIC STUDIES ON A LARGE FAMILY.

Abstract

Introduction The evaluation of prolonged obstructive jaundice in infancy, involving primarily the differential diagnosis between giant-cell and biliary atresia, remains a difficult clinical problem. In spite of reports concerning the diagnostic usefulness of cholagogue administration, duodenal intubation, SGOT determinations,1administration of corticosteroids,2and employment of radioactive rose bengal I131,3the precise diagnosis is generally obtained by liver biopsy and cholangiography. The clinician has found it necessary to balance the increased dangers of the operative diagnostic procedure in those in fants with hepatitis,4-6against the dangers of rapidly progressive and irreversible hepatic fibrosis in untreated atresias, which might otherwise be curable by surgery.7-11Regardless of the clinician's talents and skills, errors in this situation are recurrent, particularly since the coexistence of atresia and hepatitis in the same infant has now been established in several instances.12-15,17,27The difficulty of diagnosis has been increased by