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Trihydroxycoprostanic acid in the duodenal fluid of two children with intrahepatic bile duct anomalies

1972; Elsevier BV; Volume: 273; Issue: 1 Linguagem: Inglês

10.1016/0304-4165(72)90209-7

1872-8006

H. Eyssen, G. Parmentier, Frans Compernolle, J BOON, E. Eggermont,

Liver Diseases and Immunity

Using thin-layer chromatography, gas-liquid chromatography and mass spectrometry, trihydroxycoprostanic acid (3α, 7α, 12α-trihydroxy-5β-cholestan-26-oic acid) was identified in the duodenal fluid of 2 subjects with anomalies of the intrahepatic bile ducts. Sugject I was a case of intrahepatic cholestasis due to atresia of the interlobular bile ducts with familial incidence. The bile acid spectrum in the duodenal fluid of this patient was: 23% chenodeoxycholic acid, 58% cholic acid and 19% trihydroxycoprostanic acid. Subject 2 had a Zellweger-like syndrome with cholestasis and with scarely developed intrahepatic bile ductuli. The bile acid spectrum in the duodenal fluid of this patient was: 18% chenodeoxycholic acid, 37% cholic acid and 45% trihydroxycoprostanic acid. No trihydroxycoprostanic acid was found in seven healthy subjects, in three cases of cholestasis of infancy, or in ten cases of various disorders of the small intestine. Obviously, the excretion of trihydroxycoprostanic acid with the bile of Patients 1 and 2 was due to a reduced capacity of the hepatocytes to split off the 3 terminal carbon atoms of the side chain of trihydroxyprostanic acid. The cause of the impaired function of the hepatocytes remains to be established.