Abstract
The unconjugated hyperbilirubinemia noted in infants with pyloric stenosis (PS) Is believed secondary to a reduction in hepatic bilirubin UDP-glucuronyl transferase (GT) activity. In order to determine if a circulating inhibitor to GT is present and related to the reduced activity and the jaundice, 60 infants with PS were studied immediately prior to surgery. Bilirubins were determined in all infants and 42 sera, 43 gastric aspirate specimens and 29 urine samples were assayed for a possible inhibitor of GT. The mean total serum bilirubin concentration was 1.6mg/dl and the unconjugated fraction was 1.2mg/dl with 35 patients having values in excess of 1.1mg/dl. The 72 urine and gastric aspirate specimens revealed no inhibitory activity while 28 of the 42 sera demonstrated significant (> 20%) inhibitory activity with a mean of 39% inhibition of GT using a standardized in vitro assay. 12 age matched healthy infants served as controls and demonstrated no serum inhibitory activity. Of the 28 infants with significant serum inhibition only 10 had elevated serum bilirubin concentrations while 14 patients had hyperbilirubinemia without serum inhibitory activity present. These data confirm the frequent appearance of jaundice, establish the presence of significant serum inhibitory activity against GT but show no relationship between a circulating inhibitor of GT and hyper-bilirubinemia in infants with PS.
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