Prolonged obstructive jaundice in infancy.

Abstract

A PREVIOUS report,1 we have reviewed the case histories of 156 infants admitted to the Children's Medical Center because of prolonged obstructive jaundice beginning early in life. Although at the time of admission these patients presented a uniform clinical picture and were admitted with a tentative diagnosis of atresia of the bile ducts, further studies revealed that approximately 60% of the cases were due to biliary atresia, 15% of the cases were due to inspissated bile associated with erythroblastosis fetalis and 25% of the cases were considered to be due to inspissated bile of unknown etiology. The last group was characterized by early onset of jaundice during the first few weeks of life, acholic stools, dark urine, and moderate hepatomegaly and splenomegaly. Laboratory studies on these infants 2 showed their urine to contain bile and their stools to be free of both bile and urobilinogen ; the flocculation tests of liver function were usually negative. The one\x=req- minute and total serum bilirubin were, however, always elevated and showed either a slowly falling or a variable trend. Follow-up studies revealed that about 60% of these infants recovered without sequelae, some developed cirrhosis despite treat¬ ment, and a small group died as a result of either cirrhosis or the complications of surgery. It was felt that this condition resulted from several different etiologies, none of which was fully understood, and for this reason, the group was labelled the inspissated bile syndrome of unknown etiology. In 1952, Craig and Landing,3 in a study of the pathology material available in this group of infants and additional cases, came to the conclusion that these patients suffered from a viral hepatitis. They found that there was gross distortion of the liver cell cord pattern with great variation in liver cell size. Multinucleate giant parenchymal cells were diffusely distributed throughout the lobules. The findings varied from the classic postmortem lesions described by Lucke 4 and Lucke and Mallory 5 only in the lack of widespread central necrosis and complete destruction of many of the liver cells of the lobule. They concluded that the changes were diagnostic of viral hepatitis during early life. Earlier, Stokes and his associates 6 had reported a series of cases of 12 infants in whom hepatitis developed during the first two months of life. The pathologic From the Departments of Pediatrics and Pathology, Harvard Medical School, and the Children's Medical Center.