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Fourteen infants aged from 2 to 5 months were admitted to hospital with acute viral hepatitis. Their clinical presentation ranged from severe disease to fulminant hepatitis. In all patients the prothrombin-time was 10% or less of normal and serum glutamic pyruvic transaminase and bilirubin were increased. In eight cases liver-biopsy specimens were obtained during liver failure and showed a widespread necrosis without inflammatory cells. Hepatitis-B-surface antigen (HBsAg) and antibody (HBsAb) were sought by several techniques, including passive hæmagglutination and radioimmunoassay. Hepatitis was associated with hepatitis-B virus in eleven out of fourteen patients as judged by the detection of HBsAg and/or a secondary rise in HBsAb. In eight cases, the infants had received blood-derivatives in the neonatal period. The mothers of five of the remaining cases were found to be chronic carriers of HBsAg. Despite intensive supportive therapy, including repeated exchange transfusions and administration of anti-HBsγ-globulins (six cases), eight patients died. These cases demonstrate that severe or fulminant type-B hepatitis can develop in infants, who are capable of completely eliminating the hepatitis-B virus. They also suggest that severe hepatitis can result from maternal contamination.

The clinical usefulness of serum ?-glutamyl transpeptidase (?GT) assay for the diagnosis of liver disease in children was assessed retrospectively in 398 children investigated from 1981 to 1986, in whom diagnosis was ascertained according to currently accepted criteria including liver histology in each case. Serum ?GT activity was within normal limits in 10 controls, in 19 children with portal vein obstruction, and in 10 of 12 children with congenital hepatic fibrosis. Serum ?GT was raised in all children with biliary atresia, sclerosing cholangitis, paucity of interlobular bile ducts, and a1-antitrypsin deficiency with jaundice. Serum ?GT was normal in spite of patent clinical signs of cholestasis in 3 patients with benign recurrent intrahepatic cholestasis, 1 infant with posthemolytic neonatal cholestasis, and in 22 of 28 patients with progressive idiopathic cholestasis akin to Byler disease. In the latter group, children with raised serum ?GT displayed extensive portal fibrosis and bile duct proliferation on liver histology, while this was not a prominent feature in children with normal serum ?GT. These results indicate (a) the value and limits of the assay for serum ?GT activity in children with liver disease, (b) that raised serum ?GT may be considered a fairly reliable index of bile duct damage, and (c) that serum ?GT may prove a useful tool in separating two forms of progressive idiopathic cholestasis, with or without bile duct involvement.

The clinical usefulness of serum γ-glutamyl transpeptidase (γGT) assay for the diagnosis of liver disease in children was assessed retrospectively in 398 children investigated from 1981 to 1986, in whom diagnosis was ascertained according to currently accepted criteria including liver histology in each case. Serum γGT activity was within normal limits in 10 controls, in 19 children with portal vein obstruction, and in 10 of 12 children with congenital hepatic fibrosis. Serum γGT was raised in all children with biliary atresia, sclerosing cholangitis, paucity of interlobular bile ducts, and α1-antitrypsin deficiency with jaundice. Serum γGT was normal in spite of patent clinical signs of cholestasis in 3 patients with benign recurrent intrahepatic cholestasis, 1 infant with posthemolytic neonatal cholestasis, and in 22 of 28 patients with progressive idiopathic cholestasis akin to Byler disease. In the latter group, children with raised serum γGT displayed extensive portal fibrosis and bile duct proliferation on liver histology, while this was not a prominent feature in children with normal serum γGT. These results indicate (a) the value and limits of the assay for serum γGT activity in children with liver disease, (b) that raised serum γGT may be considered a fairly reliable index of bile duct damage, and (c) that serum γGT may prove a useful tool in separating two forms of progressive idiopathic cholestasis, with or without bile duct involvement.