Follow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversionfollow-up in children with progressive familial intrahepatic cholestasis after partial external biliary diversion

Journal of Pediatric Gastroenterology and Nutrition

Volumn 51, Issue 4, 2010, Pages 494-499

  Arnell, Henrik ^a,b   Papadogiannakis, Nikos ^a   Zemack, Helen ^a   Knisely, Alex S ^a   Németh, Antal ^a   Fischler, Björn ^a  

^a KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

^b KING S COLLEGE HOSPITAL   (United Kingdom)

Author keywords

Chronic cholestasis; Genotype; Hepatic fibrosis; Long term follow up; Partial external biliary diversion; Postoperative outcome; Progressive familial intrahepatic cholestasis

Indexed keywords

MULTIDRUG RESISTANCE PROTEIN 1;

ADOLESCENT; ADULT; ANTHROPOMETRIC PARAMETERS; ARTICLE; BILIOPANCREATIC BYPASS; CHEMICAL ANALYSIS; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; FEMALE; FOLLOW UP; GENE MUTATION; GENETIC ANALYSIS; GENOTYPE; HISTOLOGY; HUMAN; INTRAHEPATIC CHOLESTASIS; LIVER CIRRHOSIS; LIVER FIBROSIS; MALE; MISSENSE MUTATION; OUTCOME ASSESSMENT; PARTIAL EXTERNAL BILIARY DIVERSION; PRESCHOOL CHILD; PRIORITY JOURNAL; PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS; SCHOOL CHILD;

ADOLESCENT; ADULT; BILE DUCTS, INTRAHEPATIC; BILIARY TRACT SURGICAL PROCEDURES; CHILD; CHILD, PRESCHOOL; CHOLESTASIS, INTRAHEPATIC; FEMALE; FIBROSIS; FOLLOW-UP STUDIES; HUMANS; INFANT; INFANT, NEWBORN; MALE; SEVERITY OF ILLNESS INDEX; TREATMENT OUTCOME; YOUNG ADULT;

EID: 78449240513     PISSN: 02772116     EISSN: None     Source Type: Journal    
DOI: 10.1097/MPG.0b013e3181df99d5     Document Type: Article

References (27)

1. Van Mil SWC, Houwen RHJ, Klomp LWJ. Genetics of familial intrahepatic cholestasis syndromes. J Med Genet 2005;42:449-63.
2. Starzl TE, Koep LJ, Schroter GP, et al. Liver replacement for pediatric patients. Pediatrics 1979;63:825-9.
3. Clayton RJ, Iber FL, Ruebner BH, et al. Byler disease. Fatal familial intrahepatic cholestasis in an Amish kindred. Am J Dis Child 1969;117:112-24.
4. Riely CA. Familial intrahepatic cholestatic syndromes. Semin Liver Dis 1987;7:119-33.
5. Whitington PF, Whitington GL. Partial external diversion of bile for the treatment of intractable pruritus associated with intrahepatic cholestasis. Gastroenterology 1988;95:130-6.
6. Emond JC, Whitington PF. Selective surgical management of progressive familial intrahepatic cholestasis (Byler's disease). J Pediatr Surg 1995;30:1635-41.
7. Kurbegov AC, Setchell KD, Haas JE, et al. Biliary diversion for progressive familial intrahepatic cholestasis: Improved liver morphology and bile acid profile. Gastroenterology 2003;125:1227-34.
8. Arnell H, Bergdahl S, Papadogiannakis N, et al. Preoperative observations and short-term outcome after partial external biliary diversion in 13 patients with progressive familial intrahepatic cholestasis. J Pediatr Surg 2008;43:1312-20.
9. Whitington PF, Freese DK, Alonso EM, et al. Clinical and biochemical findings in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994;18:134-41.
10. Painter JN, Savander M, Ropponen A, et al. Sequence variation in the ATP8B1 gene and intrahepatic cholestasis of pregnancy. Eur J Hum Genet 2005;13:435-9.
11. Batts KP, Ludwig J. Chronic hepatitis. An update on terminology and reporting. Am J Surg Pathol 1995;19:1409-17.
12. Balistreri WF. Intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 2002;35(Suppl 1):S17-23.
13. Noe J, Stieger B, Meier PJ. Functional expression of the canalicular bile salt export pump of human liver. Gastroenterology 2002;123: 1659-66.
14. Knisely AS, Strautnieks SS, Meier Y, et al. Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency. Hepatology 2006;44:478-86.
15. Colloredo G, Guido M, Sonzogni A, et al. Impact of liver biopsy size on histological evaluation of chronic viral hepatitis: The smaller the sample, the milder the disease. J Hepatol 2003;39:239-44.
16. Englert C, Grabhorn E, Richter A, et al. Liver transplantation in children with progressive familial intrahepatic cholestasis. Transplantation 2007;84:1361-3.
17. Alonso EM, Snover DC, Montag A, et al. Histologic pathology of the liver in progressive familial intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 1994;18:128-33.
18. Strautnieks SS, Byrne JA, Pawlikowska L, et al. Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families. Gastroenterology 2008;134:1203-14.
19. Carlton VEH, Harris BZ, Puffenberger EG, et al. Complex inheritance of familial hypercholanemia with associated mutations in TJP2 and BAAT. Nat Genet 2003;34:91-6.
20. Van Mil SW, Van Der Woerd WL, Van Der Brugge G, et al. Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11. Gastroenterology 2004;127:379-84.
21. D'Odorico A, Melis A, Baragiotta A, et al. Oxygen-derived free radical production by peripheral blood neutrophils in chronic cholestatic liver diseases. Hepatogastroenterology 1999;46:1831-5.
22. Seksik P, Sokol H, Lepage P, et al. Review article: The role of bacteria in onset and perpetuation of inflammatory bowel disease. Aliment Pharmacol Ther 2006;24 (Suppl 3):11-8.
23. Dobbins JW. Nephrolithiasis and intestinal disease. J Clin Gastroenterol 1985;7:21-4.
24. Melter M, Rodeck B, Kardorff R, et al. Progressive familial intrahepatic cholestasis: Partial biliary diversion normalizes serum lipids and improves growth in noncirrhotic patients. Am J Gastroenterol 2000;95: 3522-8.
25. Ng VL, Ryckman FC, Porta G, et al. Long-term outcome after partial external biliary diversion for intractable pruritus in patients with intrahepatic cholestasis. J Pediatr Gastroenterol Nutr 2000;30:152-6.
26. Kalicinski PJ, Ismail H, Jankowska I, et al. Surgical treatment of progressive familial intrahepatic cholestasis: Comparison of partial external biliary diversion and ileal bypass. Eur J Pediatr Surg 2003;13:307-11.
27. Ramaccioni V, Soriano HE, Arumugam R, et al. Nutritional aspects of chronic liver disease and liver transplantation in children. J Pediatr Gastroenterol Nutr 2000;30:361-7.