During the past few years increasing attention has been directed toward a group of infants with prolonged obstructive jaundice not associated with erythroblastosis fetalis or biliary atresia. This syndrome is characterized by onset of jaundice during the first weeks of life, acholic stools, dark urine, and moderate hepatomegaly and splenomegaly. Laboratory studies on these infants show their urine to contain bile and their stools to be free of both bile and urobilinogen, the flocculation tests of liver function being usually negative. The one-minute and total serum bilirubin are, however, always elevated and show either a slowly falling or variable trend. Histological examination of the liver reveals focal or widespread hepatocellular degeneration, often with the formation of multinucleated liver cells or liver "giant cells." Follow-up studies have revealed that about 60% of these infants recover without sequelae, some develop cirrhosis despite treatment, and a small group die as a result of
HSIA DY, BOGGS JD, DRISCOLL SG, GELLIS SS. Prolonged Obstructive Jaundice in Infancy: V. The Genetic Components in Neonatal Hepatitis. AMA Am J Dis Child. 1958;95(5):485–491. doi:10.1001/archpedi.1958.02060050489005
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