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May 1964

Familial "Giant-Cell Hepatitis" in Infancy: Clinical, Pathologic, and Genetic Studies on a Large Family

Author Affiliations

George Cassady, MD, Boston Lying-in Hospital, 221 Longwood Ave, Boston, Mass 02215.; Duke University Medical Center, Department of Pediatrics; Medical Investigations Branch, National Institute of Dental Research, National Institutes of Health; presently at Children's Medical Center, Boston (Dr. Cassady); University of Rochester, School of Medicine, Department of Pathology (Dr. Morrison); and Human Genetics Section, National Institute of Dental Research, National Institutes of Health (Dr. Cohen).

Am J Dis Child. 1964;107(5):456-469. doi:10.1001/archpedi.1964.02080060458005

Introduction  The evaluation of prolonged obstructive jaundice in infancy, involving primarily the differential diagnosis between "giant-cell hepatitis" and biliary atresia, remains a difficult clinical problem. In spite of reports concerning the diagnostic usefulness of cholagogue administration, duodenal intubation, SGOT determinations,1 administration of corticosteroids,2 and employment of radioactive rose bengal I131,3 the 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 infants with hepatitis,4-6 against the dangers of rapidly progressive and irreversible hepatic fibrosis in untreated atresias, which might otherwise be curable by surgery.7-11 Regardless 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.12-15,17,27 The difficulty of diagnosis has been increased by