Evidence for defective primary bile acid secretion in children with progressive familial intrahepatic cholestasis (Byler disease)
作者： Emmanuel Jacquemin , Micheline Dumont , Olivier Bernard , Serge Erlinger , Michelle Hadchouel
DOI: 10.1007/BF01983406
关键词:
摘要: To clarify the relationship of progressive familial intrahepatic cholestasis (Byler disease) to bile acid metabolism, we analysed, by high performance liquid chromatography, composition serum and in seven children with Byler disease eight control other cholestatic diseases. In serum, total concentration was increased patients (0.30 +/- 0.05 mmol/l) (0.21 0.08 mmol/l). Cholate (C) chenodeoxycholate (CDC) comprised major proportion acids as patients. Hyocholate (HC) only detected lithocholate present children. bile, very low (1.1 1.4 compared (88.9 83.2 C CDC were patients, whereas HC disease. These results suggest existence a defect primary secretion characterized presence absence or bile.
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sci-hub.se 参考文章(28)
B Stieger, B O'Neill, P J Meier, ATP-dependent bile-salt transport in canalicular rat liver plasma-membrane vesicles. Biochemical Journal. ,vol. 284, pp. 67- 74 ,(1992) , 10.1042/BJ2840067
Etsuko HIRATA, Masayasu INOUE, Yoshimasa MORINO, Mechanism of biliary secretion of membranous enzymes: bile acids are important factors for biliary occurrence of gamma-glutamyltransferase and other hydrolases. Journal of Biochemistry. ,vol. 96, pp. 289- 297 ,(1984) , 10.1093/OXFORDJOURNALS.JBCHEM.A134837
K D Setchell, R Dumaswala, C Colombo, M Ronchi, Hepatic bile acid metabolism during early development revealed from the analysis of human fetal gallbladder bile. Journal of Biological Chemistry. ,vol. 263, pp. 16637- 16644 ,(1988) , 10.1016/S0021-9258(18)37438-6
S Ruetz, G Fricker, G Hugentobler, K Winterhalter, G Kurz, P J Meier, Isolation and characterization of the putative canalicular bile salt transport system of rat liver. Journal of Biological Chemistry. ,vol. 262, pp. 11324- 11330 ,(1987) , 10.1016/S0021-9258(18)60962-7
Andrée M. Weber, Beatriz Tuchweber, Ibrahim Yousef, Pierre Brochu, Claire Turgeon, Giulio Gabbiani, Claude L. Morin, Claude C. Roy, Severe familial cholestasis in North American Indian children: A clinical model of microfilament dysfunction? Gastroenterology. ,vol. 81, pp. 653- 662 ,(1981) , 10.1016/0016-5085(81)90487-X
J Shoda, R Mahara, T Osuga, M Tohma, S Ohnishi, H Miyazaki, N Tanaka, Y Matsuzaki, Similarity of unusual bile acids in human umbilical cord blood and amniotic fluid from newborns and in sera and urine from adult patients with cholestatic liver diseases. Journal of Lipid Research. ,vol. 29, pp. 847- 858 ,(1988) , 10.1016/S0022-2275(20)38479-0
Giuseppe Maggiore, Olivier Bernard, Caroline A. Riely, Michelle Hadchouel, Alain Lemonnier, Daniel Alagille, Normal serum γ-glutamyl-transpeptidase activity identifies groups of infants with idiopathic cholestasis with poor prognosis The Journal of Pediatrics. ,vol. 111, pp. 251- 252 ,(1987) , 10.1016/S0022-3476(87)80079-3
Peter Meier, Transport polarity of hepatocytes Seminars in Liver Disease. ,vol. 8, pp. 293- 307 ,(1988) , 10.1055/S-2008-1040551
Albert Tangerman, Annie van Schaik, Egbert W. van der Hoek, Analysis of conjugated and unconjugated bile acids in serum and jejunal fluid of normal subjects Clinica Chimica Acta. ,vol. 159, pp. 123- 132 ,(1986) , 10.1016/0009-8981(86)90044-6
Marie-Noelle Chobert, Olivier Bernard, Frédérique Bulle, Alain Lemonnier, Georges Guellaen, Daniel Alagille, High hepatic γ-glutamyltransferase (γ-GT) activity with normal serum γ-GT in children with progressive idiopathic cholestasis Journal of Hepatology. ,vol. 8, pp. 22- 25 ,(1989) , 10.1016/0168-8278(89)90157-8