Treatment of progressive familial intrahepatic cholestasis: Liver transplantation or partial external biliary diversion

Pediatric Transplantation

Volumn 3, Issue 3, 1999, Pages 219-224

  Ismail, Hor ^a   Kaliciński, Piotr ^a   Markiewicz, Małgorzata ^a   Jankowska, Irena ^a   Pawłowska, Joanna ^a   Kluge, Przemysław ^a   Eliadou, Eliana ^a   Kamiński, Andrzej ^a   Szymczak, Marek ^a   Drewniak, Tomasz ^a   Revillon, Yann ^b  

^a CHILDREN'S MEMORIAL HEALTH INSTITUTE   (Poland)

^b HÔPITAL NECKER ENFANTS MALADES   (France)

Author keywords

Byler's disease; Intrahepatic cholestasis; Liver transplantation; Partial external biliary diversion; PFIC

Indexed keywords

ALANINE AMINOTRANSFERASE; ASPARTATE AMINOTRANSFERASE; BILE ACID; BILIRUBIN; URSODEOXYCHOLIC ACID;

ARTICLE; CHILD; CLINICAL ARTICLE; CLINICAL FEATURE; DISEASE ACTIVITY; DISEASE COURSE; FAMILIAL DISEASE; FEMALE; HISTOPATHOLOGY; HUMAN; INFANT; INTRAHEPATIC CHOLESTASIS; LABORATORY TEST; LIVER TRANSPLANTATION; MALE; SURGICAL APPROACH; TREATMENT INDICATION; TREATMENT OUTCOME;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; CHOLAGOGUES AND CHOLERETICS; CHOLESTASIS, INTRAHEPATIC; DATA INTERPRETATION, STATISTICAL; FEMALE; FOLLOW-UP STUDIES; GALLBLADDER; HUMANS; INFANT; JEJUNUM; LIVER TRANSPLANTATION; MALE; SKIN; STOMAS; TIME FACTORS; TREATMENT OUTCOME; URSODEOXYCHOLIC ACID;

EID: 0344563417     PISSN: 13973142     EISSN: None     Source Type: Journal    
DOI: 10.1034/j.1399-3046.1999.00046.x     Document Type: Article

References (15)

1. CLAYTON RJ, IBER FL, RUCHNER BH, MCKUSICK VA. Byler's disease - fatal intrahepatic cholestasis in an Amish kindred. Am Pediatr Soc 1965: 67: 1025-1028(abstracts).
2. CLAYTON RJ, IBER FL, RUCHNER BH, MCKUSICK VA. Byler's disease fatal intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969: 117: 112-124.
3. KNISLEY AS, BOYLE JT, NAYLOR EW, et al. Pancreatic dysfunction in Byler's disease. J Pediatr Gastroenterol Nutr 1995: 21: 3(NASPGN abstracts).
4. CHORBERT MN, BERNARD O, BULLE F, LEMONNIER A, GUELLAEN G, ALAGILLE D. High hepatic gamma-glutamyl-transferase (GGTP) activity with normal serum GGTP in children with progressive idiopathic cholestasis. J Hepatol 1989: 8: 22-25.
5. MAGGIORE G, BERNARD O, HANDCHOUEL M, LEMONNIER A, ALAGILLE D. Diagnostic value of serum gamma-glutamyl transpeptidase activity in liver diseases in children. J Pediatr Gastroenterol Nutr 1991: 12: 1-21.
6. MAGGIORE G, BERNARD O, RIELY C, et al. Normal serum g-glutamyl-transpeptidase activity identifies groups of infants with idiopathic cholestasis with poor diagnosis. J Pediatr 1987: 111: 251-252.
7. JACQUEMIN E, DUMONT M, BERNARD O, et al. Evidence for defective primary bile acid secretion in children with progressive familial intrahepatic cholestasis (Byler disease). Eur J Pediatr 1994: 153: 424-428.
8. ALONSO EM, SNOVER DC, MONTAG A, FREESE D, WHITINGTON PF. Histologic pathology of the liver in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994: 18: 128-133.
9. WHITINGTON PF, WHITINGTON GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988: 95: 130-136.
10. WHITINGTON PF. Chronic cholestasis of infancy. Pediatr Clin N Am 1996: 43: 1-25.
11. JACQUEMIN E, HERMANS D, MYARA A, et al. Ursodeoxycholic acid therapy in pediatric patients with progressive familial intrahepatic cholestasis. Hepatology 1997: 25: 519.
12. SOUBRANE O, GAUTHIER F, DEVICTOR D, et al. Orthotopic liver transplantation for Byler disease. Transplantation 1990: 50: 804-806.
13. LEE H, VACANTI J. Liver transplantation and its long term management in children. Pediatr Clin N Am 1996: 43: 99-124.
14. EMOND JC, WHITINGTON PF. Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease). J Pediatr Surg 1995: 30: 1635-1641.
15. HOLLANDS CM, RIVERA-PEDROGO FJ, GONZALEZ-VALLINA R, et al. Ileal exclusion for Byler's disease: an alternative surgical approach with promising early results for pruritus. J Pediatr Surg 1998: 33: 220-224.