Abstract
Serum GGT activity was assayed at diagnosis in 186 consecutive cholestatic infants aged 12 months or less followed for at least 1 year or until death. In 157 ibiliary atresia 113, other extrahepatic obstructions 10, syndromic paucity of bile ducts 14, alpha-l-antitrypsin deficiency 6, other intrabepatic cholestasis of known origin 14) cholestasis could be classified in one of the known categories and serus GGT was raised in all (5-65 x N) but 1 with post-hemolytic conjugated hyperbilirubinemia in whom jaundice cleared within few months. In the 29 other, with intrahepatic cholestasis of unknown origin, results of serum GGT allowed to separate 2 groups: 17 infants with high SGT (2-13.7 x N) and 12 with normal GGT. Inllll but 1 in the first group jaundice resolved and results of clinical and laboratorj investigation were normal at last control 12-42 months later. In all the children of the group 2 jaundice, pruritus and hepatosplenoaegaiy persisted: 6 died of liver failure and 3 others had ascitis and prothrombin time < 40%, Repated liver biopsies in 5 others showed progression of fibrosis; 1 child with liver failure underwent a successful liver transplantation. This group of cholestatic infants with normal serum GST was further characterized as follows: parents were related in 6 instances and 6 infants had a sibling with a similar disease. Serum GGT remained normal througth the follow up period except in 1 in whom a transitory rise was associated, with intrahepatic lithiasis wich cleared spontaneously. In conclusions these results suggest that in infants with cholestasis of unknown origin, persistently normal serum GammaGT helps identifying a group with progressive liver disease of poor prognosis which may correspond to an hereditary disease akin to Byler disease and may thus allow tor early identification of those patients requiring liver trasplantation before patent signs of liver failure are present.
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Maggiore, G., Bernard, O., Hadchouel, M. et al. 64. NORMAL SERUM SAMMA-GLUTAMIL-TRANSPEPTIDASE (GGT) ACTIVITY IDENTIFIES A GROUP OF INFANTS WITH IDIOPATHIC COLESTASIS OF POOR PROGNOSIS. Pediatr Res 22, 106 (1987). https://doi.org/10.1203/00006450-198707000-00085
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DOI: https://doi.org/10.1203/00006450-198707000-00085