Cholestyramine therapy in patients with a paucity of intrahepatic bile ducts

Sharp, Harvey L.; Carey, J. D.; White, James G.; Krivit, William; Williams, Gale C.; Peller, Janet D.

doi:10.1016/s0022-3476(67)80212-9

Cited by 74 publications

(15 citation statements)

References 25 publications

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“…Periodic estimations of BA in serum are thus useful for optimal management of therapy of these patients, since improvement in rate of growth appears to require attainment of approximately normal BA concentrations in serum. Reduction of these abnormal concentrations is also associated with cessation of the pruritus of chronic obstructive liver disease [22]. This has been correlated with a decrease in amounts of BA extractable from the skin of such patients [21].…”

Section: Discussionmentioning

confidence: 91%

“…A recent report indicates that reduction of BA concentrations may be associated with regression of cirrhotic changes in the liver and improved growth patterns when serum concentrations approach the normal range [22]. Periodic estimations of BA in serum are thus useful for optimal management of therapy of these patients, since improvement in rate of growth appears to require attainment of approximately normal BA concentrations in serum.…”

Section: Discussionmentioning

confidence: 99%

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Bile Acid Concentrations in Serum during Infancy and Childhood

Sandberg

1970

Pediatr Res

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ExtractConcentrations of free and conjugated bile acids in cord blood (30 samples) were within the adult range and were significantly higher than in serum from newborns (15 infants). Deoxycholic acid (D) was absent from sera of newborns and present in 7 of 23 cord samples. Only two of nine infants in year 1 of life showed detectable amounts of D. In 25 children, ages 1-13 years, bile acid concentrations in serum fell within the normal adult range. Small amounts of unconjugated bile acids were present in some specimens.Thirty-three children with cystic fibrosis of the pancreas (ages 2-14 years) had serum concentrations within the normal adult range. No correlation with age, sex, or severity of disease was noted.Thirty-two children with a variety of hepatobiliary disorders had significantly elevated concentrations of bile acids with the exception of a few infants with terminal cirrhosis. Determination of patterns and concentrations of bile acids in serum are of limited usefulness in differential diagnosis of these disorders. Speculation

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 99%

Bile Acid Concentrations in Serum during Infancy and Childhood

Sandberg

1970

Pediatr Res

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“…Functionally, an equimolar concentration of taurocholate prevents the decreased bile flow caused by taurolithocholate administration. Pathologic elevations of predominantly cholic acid (a trihydroxy bile acid) are associated with less than the normal number of intrahepatic bile ducts in children [14,23]. Thus it is interesting to find during bile duct development that cholic acid is not the predominant bile acid until after birth.…”

Section: Maternal Circulationmentioning

confidence: 99%

“…When these levels are lowered by cholestyramine or neomycin therapy, an improvement is noted in liver function [7]. Both primary bile acid concentrations (cholic predominating over chenodeoxycholic) are increased in the serum of infants with decreased numbers of intrahepatic bile ducts [14,23]. Interruption of the enterohepatic bile salt circulation decreases pruritus and improves liver function.…”

Section: Introductionmentioning

confidence: 99%

Primary and Secondary Bile Acids in Meconium

et al. 1971

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ExtractMeconium, newborn stool, and fetal bile were examined for bile acids by the techniques of gas liquid chromatography, thin layer chromatography and colorimetric spectrometry. Cholic, 1 chenodeoxycholic, deoxycholic, and lithocholic acid were detected in meconium. Secondary bile acids were not present in significant amounts in either stool or fetal bile from newborns. During fetal development more chenodeoxycholate than cholate is formed. The presence of secondary bile acids in meconium is suggestive of maternal-to-fetal transfer via the placenta.

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“…Among the intrahepatic disturbances that have been reported to occur are both anatomic reduction in the size of the bile secretory apparatus [1] and metabolic abnormalities in bile acid synthesis [10]. Because of the increased mortality rate associated with surgery [8] and the beneficial effect that can occur with cholestyramine therapy [13], we undertook a study of the diagnostic and therapeutic usefulness of this agent.…”

Section: Discussionmentioning

confidence: 99%