Biliary diversion for progressive familial intrahepatic cholestasis: Improved liver morphology and bile acid profile

Gastroenterology

Volumn 125, Issue 4, 2003, Pages 1227-1234

  Kurbegov, Amethyst C ^a   Setchell, Kenneth D R ^c   Haas, Joel E ^b   Mierau, Gary W ^b   Narkewicz, Michael ^a   Bancroft, John D ^d   Karrer, Frederick ^b   Sokol, Ronald J ^a,b,e  

^a Children's Hospital Colorado   (United States)

^b UNIVERSITY OF COLORADO SCHOOL OF MEDICINE   (United States)

^c CINCINNATI CHILDREN'S HOSPITAL MEDICAL CENTER   (United States)

^d Maine Pediatric Specialty Group   (United States)

^e CHILDREN S HOSPITAL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BILE ACID; CARRIER PROTEIN; CHENODEOXYCHOLIC ACID; CHOLIC ACID; GAMMA GLUTAMYLTRANSFERASE;

ARTICLE; BILE; BILE COMPOSITION; BILIARY DIVERSION; BILIARY TRACT SURGERY; CASE REPORT; CELL TRANSFORMATION; CENTRAL VENOUS MURAL SCLEROSIS; CHILD; CHOLESTASIS; CHRONIC INFLAMMATION; FEMALE; GAMMA GLUTAMYL TRANSFERASE BLOOD LEVEL; GIANT CELL; HUMAN; HUMAN TISSUE; INTRAHEPATIC BILE DUCT; INTRAHEPATIC CHOLESTASIS; LIVER; LIVER BIOPSY; LIVER FIBROSIS; LIVER PORTAL FIBROSIS; LIVER TRANSPLANTATION; MALE; PARTIAL EXTERNAL BILIARY DIVERSION; PRIORITY JOURNAL; PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS; PRURITUS; SCORING SYSTEM; ULTRASTRUCTURE;

EID: 0141706384     PISSN: 00165085     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0016-5085(03)01199-5     Document Type: Article

References (23)

1. Schneider BL. Genetic cholestasis syndromes. J Pediatr Gastroenterol Nutr 1999;28:124-131.
2. Emond JC, Whitington PF. Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease). J Pediatr Surg 1995;30:1635-1641.
3. Jacquemin E. Progressive familial intrahepatic cholestasis. Genetic basis and treatment. Clin Liver Dis 2000;4:753-763.
4. Colombo C, Okolicsanyi L, Strazzabosco M. Advances in familial and congenital cholestatic diseases. Clinical and diagnostic implications. Dig Liver Dis 2000;32:152-159.
5. Jansen PLM, Muller MM. Progressive familial intrahepatic cholestasis types 1, 2, and 3. Gut 1998;42:766-767.
6. Jacquemin E, Hadchouel M. Genetic basis of progressive familial intrahepatic cholestasis. J Hepatol 1999;31:377-381.
7. Trauner M, Meier PJ, Boyer JL. Molecular pathogenesis of cholestasis. N Engl J Med 1998;339:1217-1227.
8. Bull LN, van Eijk MJ, Pawlikowska L, DeYoung JA, Juijn JA, Liao M, Klomp LW, Lomri N, Berger R, Scharschmidt BF, Knisely AS, Houwen RH, Freimer NB. A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis. Nat Genet 1998;18:219-224.
9. Jansen PL, Strautnieks SS, Jacquemin E, Hadchouel M, Sokal EM, Hooiveld GJ, Koning JH, De Jager-Krikken A, Kuipers F, Stellaard F, Bijleveid CM, Gouw A, Van Goor H, Thompson RJ, Muller M. Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis. Gastroenterology 1999;117:1370-1379.
10. de Vree JM, Jacqumin E, Sturm E, Cresteil D, Bosma PJ, Aten J, Deleuze JF, Desrochers M, Burdelski M, Bernanrd O, Oude Elferink RP, Hadchouel M. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. Proc Natl Acad Sci U S A 1998;95:282-287.
11. Bull LN, Carlton VEH, Stricker NL, Baharloo S, DeYoung JA, Freimer NB, Magid NS, Kahn E, Markowitz J, DiCarlo FJ, McLoughlin L, Boyle JT, Dahms BB, Faught PR, Fitzgerald JF, Piccoli DA, Witzleben CL, O'Connell NC, Setchell KDR, Agostini RM Jr, Kocoshis SA, Reyes J, Knisely AS. Genetic and morphological findings in progressive familial intrahepatic cholestasis (Byler disease [PFIC-1] and Byler syndrome): evidence for heterogeneity. Hepatology 1997;26:155-164.
12. Setchell KDR, O'Connell NC. Disorders of bile acid synthesis and metabolism: a metabolic basis for liver disease. In: Suchy FJ, Sokol RJ, Balistreri WF, eds. Liver disease in children. Philadelphia: Lippincott Williams & Wilkins, 2001:701-734.
13. Rebhandl W, Felberbauer FX, Turnbull J, Paya K, Barcik U, Huber WD, Whitington PF, Horcher E. Biliary diversion by use of the appendix (cholescystoappendicostomy) in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1999;28:217-219.
14. Melter M, Rodeck B, Kardorff R, Hoyer PF, Petersen C, Ballauff A, Brodehl J. Progressive familial intrahepatic cholestasis: partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients. Am J Gastroenterol 2000;95:3522-3528.
15. Ng VL, Ryckman FC, Porta G, Miura IK, de Carvalho E, Servidoni MF, Bezerra JA, Balistreri WF. Long-term outcome after partial external biliary diversion for intractable pruritus in patients with intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 2000;30:152-156.
16. Ismail H, Kalicinski P, Markiewicz M, Jankowska I, Pawlowska J, Kluge P, Eliadou E, Kaminski A, Szymczak M, Drewniak T, Revillon Y. Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. Pediatr Transplant 1999;3:219-224.
17. Emerick KM, Whitington PF. Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome. Hepatology 2002;35:1501-1506.
18. Alonso EM, Snover DC, Montag A, Freese DK, Whitington PF. Histologic pathology of the liver in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994;19:128-133.
19. Setchell KD, Schwarz M, O'Connell NC, Lund EG, Davis DL, Lathe R, Thompson HR, Weslie Tyson R, Sokol RJ, Russell DW. Identification of a new inborn error in bile acid synthesis: mutation of the oxysterol 7alpha-hydroxylase gene causes severe neonatal liver disease. J Clin Invest 1998;102:1690-1703.
20. Felberbauer FX, Amann G, Rebhandl W, Huber W-D. Follow-up after partial external biliary diversion in familial cholestasis of infancy, J Pediatr Gastroenterol Nutr 2000;3:322.
21. 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.
22. Roda A, Hofmann AF, Mysels KJ. The influence of bile salt structure on self-association in aqueous solutions. J Biol Chem 1983;258:6362-6370.
23. Hollands CM, Rivera-Pedrogo FJ, Gonzalez-Vallina R, Loret-de-Mola O, Nahmad M, Burnweit CA, Ileal exclusion for Byler's disease: an alternative surgical approach with promising early results for pruritus. J Pediatr Surg 1998;33:220-224.