Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis

Hepatology

Volumn 25, Issue 3, 1997, Pages 519-523

  Jacquemin, Emmanuel ^a   Hermans, Dominique ^c   Myara, Anne ^b   Hases, Dalila ^a   Debray, Dominique ^a   Hadchouel, Michelle ^a   Sokal, Etienne M ^c   Bernard, Olivier ^a  

^a BICÊTRE HOSPITAL   (France)

^b SAINT JOSEPH HOSPITAL   (France)

^c CLINIQUES UNIVERSITAIRES SAINT LUC   (Belgium)

Author keywords

[No Author keywords available]

Indexed keywords

GAMMA GLUTAMYLTRANSFERASE; URSODEOXYCHOLIC ACID;

ARTICLE; CLINICAL ARTICLE; CONTROLLED STUDY; DRUG EFFICACY; ENZYME ACTIVITY; FAMILIAL DISEASE; FEMALE; HISTOPATHOLOGY; HUMAN; INTRAHEPATIC CHOLESTASIS; LIVER BIOPSY; MALE; ORAL DRUG ADMINISTRATION; PRIORITY JOURNAL;

ALANINE TRANSAMINASE; ASPARTATE AMINOTRANSFERASES; CHILD; CHILD, PRESCHOOL; CHOLAGOGUES AND CHOLERETICS; CHOLESTASIS, INTRAHEPATIC; FAMILY; FEMALE; HUMANS; MALE; URSODEOXYCHOLIC ACID;

EID: 0031049399     PISSN: 02709139     EISSN: None     Source Type: Journal    
DOI: 10.1002/hep.510250303     Document Type: Article

References (18)

1. Clayton RJ, Iber FL, Ruebner BH, Me Kusick VA. Byler disease: fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969;117:112-124.
2. Linarelli LG, Williams CN, Phillips MJ. Byler's disease: fatal intrahepatic cholestasis. J Pediatr 1972;81:484-492.
3. Riely CA. Familial intrahepatic cholestatic syndromes. Semin Liver Dis 1987;7:119-133.
4. Maggiore G, Bernard O, Riely CA, Hadchouel M, Lemonnier A, Alagille D. Normal γ-glutamyl-transpeptidase activity identifies groups of infants with idiopathic cholestasis with poor prognosis. J Pediatr 1987;111:251-252.
5. Jacquemin E, Dumont M, Bernard O, Erlinger S, Hadchouel M. Evidence for defective primary bile acid secretion in children with progressive familial intrahepatic cholestasis (Byler disease). Eur J Pediatr 1994;153:424-428.
6. Jacquemin E, Setchell KDR, O'Connell NC, Estrada A, Maggiore G, Schmitz J, Hadchouel M, et al. A new cause of progressive intrahepatic cholestasis: 3β-hydroxy-C27-steroid dehydrogenase/isomerase deficiency. J Pediatr 1994;125:379-384.
7. Deleuze JF, Jacquemin E, Dubuisson C, Cresteil D, Dumont M, Erlinger S, Bernard O, et al. Defect of multidrug resistance 3 (MDR3) gene expression in a subtype of progressive familial intrahepatic cholestasis. HEPATOLOGY 1996;23:904-908.
8. Maggiore G, Bernard O, Hadchouel M, Lemonnier A, Alagille D. Diagnostic value of serum γ-glutamyl transpeptidase activity in liver diseases in children. J Pediatr Gastroenterol Nutr 1991;12:21-26.
9. Whitington PF, Freese DK, Alonso EM, Swarzenberg SJ, Sharp HL. Clinical and biochemical findings in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994;18:134-141.
10. Carlton VEH, Knisely AS, Freimer MB. Mapping of a locus for progressive familial intrahepatic cholestasis (Byler disease) to 18q21-q22, the benign recurrent intrahepatic cholestasis region. Hum Mol Genet 1995;4:1049-1053.
11. Smit JJM, Schinkel AH, Oude Elferink RPJ, Groen AK, Wagenaar E, van Deemter L, Mol CAAM, et al. Homozygous disruption of the murine mdr2 P-glycoprotein gene leads to a complete absence of phospholipid from bile and to liver disease. Cell 1993;75:451-462.
12. Mauad TH, van Nieuwkerk CMJ, Dingemans KP, Smit JJM, Schinkel AH, Notenboom RGE, van den Bergh Weeman MA, et al. Mice with homozygous disruption of the mdr2 P-glycoprotein gene. A novel animal model for studies of nonsuppurative inflammatory cholangitis and hepatocarcino-genesis. Am J Pathol 1994;145:1237-1245.
13. Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988;95:130-136.
14. Emond JC, Whitington PF. Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease). J Pediatr Surg 1995;30:1635-1641.
15. Soubrane O, Gauthier F, Devictor D, Bernard O, Valayer J, Houssin D, Chapuis Y. Orthotopic liver transplantation for Byler disease. Transplantation 1990;50:804-806.
16. Poupon RE, Baikau B, Eschwege E, Poupon R, and the UDCA-PBC study group. A multicenter, controlled trial of ursodiol for the treatment of primary biliary cirrhosis. N Engl J Med 1991;324:1548-1554.
17. Poupon RE, Chrétien Y, Poupon R, Paumgartner G. Serum bile acids in primary biliary cirrhosis: effect of ursodeoxycholic acid therapy. HEPATOLOGY 1993;17:599-604.
18. Beuers U, Spengler U, Kruis W, Aydemir U, Wiebecke B, Heldwein W, Weinzierl M, et al. Ursodeoxycholic acid for treatment of primary sclerosing cholangitis: a placebo-controlled trial. HEPATOLOGY 1992;16:707-714.