Abstract
In view of the abnormal bile acid metabolism and of the increased focidence of gailstones in CF, biliary lipId composition was examined in 12 CF patients (X̄ age: 8.4 yrs) who were off therapy. A microgallbladder was present in 2, cholelithiasis in 1. In 8 cases the gallbladder was normal, it was not opacified in 1. Fasting bile vas obtained after I.V. cholecystokinin-pancreozymin (1.5 U./Kg) VR the CF group as well as in 13 controls (X̄ age: 7.0). In 7 children (X̄ age: 13.8) with cholesterol galistones, bile was sampled at the time of cholecyitectony. The % molar lip id composition (X̄ ± SE) accounted by cholesterol vas 16.3 ± 2.3 in CF and 16.8 ± 1.9 in the cholelithiasis group as compared to 7.4 ± .8 in controls (P<.01). The ratios of cholesterol actually present to the maximal amount experimentally soluble (Hegardt and Dam, Holzbach et al) at the phospholipid-bile acid concentration of the samples (Thomas and Hofman Gastroenterology 65: 698, 1973) in CF (2.8 ± .4) end in the cholelithiasis group (2.6 ± .3) were much higher (P<.01) than in controls (1.2 ± .1). This first study of bile lithogenicity in children may have important implications because of the prevalence and type of hepatoblllary disease observed in CF.
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Roy, C., Chartrand, L., Combes, J. et al. LITHOGENIC BILE IN CYSTIC FIBROSIS (CF). Pediatr Res 11, 449 (1977). https://doi.org/10.1203/00006450-197704000-00477
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DOI: https://doi.org/10.1203/00006450-197704000-00477
