Title: Extrahepatic biliary atresia: A disease or a phenotype?
Abstract: HepatologyVolume 35, Issue 6 p. 1297-1304 Concise Review In Mechanisms Of DiseaseFree Access Extrahepatic biliary atresia: A disease or a phenotype? David H. Perlmutter 3705 Fifth Ave., Corresponding Author David H. Perlmutter 3705 Fifth Ave. [email protected] Department of Pediatrics, Washington University School of Medicine; Division of Gastroenterology and Nutrition, St. Louis Children's Hospital; and Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, PAPittsburgh, PA 15213-2583. fax: 412-692-5946.===Search for more papers by this authorRoss W. Shepherd, Ross W. Shepherd Department of Pediatrics, Washington University School of Medicine; Division of Gastroenterology and Nutrition, St. Louis Children's Hospital; and Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, PASearch for more papers by this author David H. Perlmutter 3705 Fifth Ave., Corresponding Author David H. Perlmutter 3705 Fifth Ave. [email protected] Department of Pediatrics, Washington University School of Medicine; Division of Gastroenterology and Nutrition, St. Louis Children's Hospital; and Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, PAPittsburgh, PA 15213-2583. fax: 412-692-5946.===Search for more papers by this authorRoss W. Shepherd, Ross W. Shepherd Department of Pediatrics, Washington University School of Medicine; Division of Gastroenterology and Nutrition, St. Louis Children's Hospital; and Department of Pediatrics, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, Pittsburgh, PASearch for more papers by this author First published: 30 December 2003 https://doi.org/10.1053/jhep.2002.34170Citations: 113AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL References 1 Carmi R, Magee CA, Neill CA, Karrer FM. Extrahepatic biliary atresia and associated anomalies: etiologic heterogeneity suggested by distinctive patterns of associations. Am J Med Genet 1993; 45: 683– 693. MEDLINE 2 Desmet VJ. Congenital diseases of intrahepatic bile ducts: Variations on the theme “Ductal plate malformation.” Hepatology 1992; 15: 1069– 1083. 3 Schweizer P. Treatment of extrahepatic bile duct atresia: results and long-term prognosis after hepatic portoenterostomy. Pediatr Surg Int 1986; 1: 30– 36. 4 Silveira TR, Salzano FM, Howard ER, Mowat AP. Congenital structural abnormalities in biliary atresia: evidence for etiopathogenic heterogeneity and therapeutic implications. Acta Pediatr Scand 1991; 80: 1192– 1199. 5 Ghishan FK, LaBrecque DR, Mitros FA, Younoszai MK. The evolving nature of “infantile obstructive cholangiopathy.” J Pediatr 1980; 97: 27– 32. MEDLINE 6 Markowitz J, Daum F, Kahn EL, Schneider KM, So HB, Altman RP, Aiges HW, et al. Arteriohepatic dysplasia. I. Pitfalls in diagnosis and management. Hepatology 1983; 3: 74– 76. MEDLINE 7 Schwartz MZ. An alternate method for intraoperative cholangiography in infants with severe obstructive jaundice. J Pediatr Surg 1985; 20: 440– 442. MEDLINE 8 Amedee-Manesme O, Bernard O, Brunelle F, Hadchouel M, Polonovski C, Baudon JJ, Beguet P, et al. Sclerosing cholangitis with neonatal onset. J Pediatr 1987; 111: 225– 229. MEDLINE 9 de Vree JML, Jacquemin E, Sturm E, Cresteil D, Bosma PJ, Aten J, Deleuze J-F, et al. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. Proc Natl Acad Sci U S A 1998; 95: 282– 287. MEDLINE 10 Kasai M, Suzuki S. A new operation for noncorrectable biliary atresia—hepatic portoenterostomy. Shujutsu 1959; 13: 733– 739. 11 Karrer FM, Price MR, Bensard DD, Sokol RJ, Narkewicz MR, Smith DJ, Lilly JR. Long-term results with the Kasai operation for biliary atresia. Arch Surg 1996; 131: 493– 496. MEDLINE 12 Laurent J, Gauthier F, Bernard O, Hadchouel M, Odievre M, Valayer J, Alagille D. Long-term outcome after surgery for biliary atresia: study of 40 patients surviving for more than 10 years. Gastroenterology 1990; 99: 1793– 1797. MEDLINE 13 Vajro P, Couturier M, Lemonnier F, Odievre M. Effects of postoperative cholestyramine and phenobarbital administration on bile flow restoration in infants with extrahepatic biliary atresia. J Pediatr Surg 1986; 21: 362– 365. MEDLINE 14 Ohi R. Biliary atresia. A surgical perspective. Clin Liver Dis 2000; 4: 779– 804. MEDLINE 15 Ohi R, Hanamatou M, Mochizuki I, Chiba T, Kasai M. Progress in the treatment of biliary atresia. World J Surg 1985; 9: 285– 293. MEDLINE 16 Volpert D, White F, Finegold MJ, Molleston J, Perlmutter DH. Outcome of early hepatic portoenterostomy for biliary atresia. J Pediatr Gastro Nutr 2001; 32: 265– 269. 17 Davenport M, Kerkar N, Mieli-Vergani G, Mowat AP, Howard ER. Biliary atresia: the King's College Hospital experience (1974-1995). J Pediatr Surg 1997; 32: 479– 485. MEDLINE 18 Chardot C, Carton M, Spire-Benedelac N, Le Pommelet C, Golmard JL, Reding R, Auvert B. Is the Kasai operation still indicated in children older than 3 months diagnosed with biliary atresia. J Pediatr 2001; 138: 224– 228. MEDLINE 19 Kasai M, Mochizuki I, Ohkohchi N, Chiba T, Ohi R. Surgical limitations for biliary atresia: indication for liver transplantation. J Pediatr Surg 1989; 24: 851– 854. MEDLINE 20 Otte JB, de Ville de Goyet J, Reding R, Hausleithner V, Sokal E, Chardot C, Debande B. Sequential treatment of biliary atresia with Kasai portoenterostomy and liver transplantation: a review. Hepatology 1994; 20: 418– 488. 21 Ryckman F, Fisher R, Pedersen S, Dittrich V, Heubi J, Farrell M, Balistreri W, et al. Improved survival in biliary atresia patients in the present era of liver transplantation. J Pediatr Surg 1993; 28: 382– 386. MEDLINE 22 Cuervas-Mons V, Rinola A, Van Thiel DH, Gavaler JS, Schade RR, Starzl TE. Does previous abdominal surgery alter the outcome of pediatric patients subjected to orthotopic liver transplantation? Gastroenterology 1986; 90: 853– 857. MEDLINE 23 Sandler AD, Azarow KS, Superina RA. The impact of a previous Kasai procedure on liver transplantation for biliary atresia. J Pediatr Surg 1997; 32: 416– 419. MEDLINE 24 Chardot C, Carton M, Spire-Bendelac N, Le Pommelet C, Golmard J-L, Auvert B. Prognosis of biliary atresia in the era of liver transplantation: French national study from 1986-1996. Hepatology 1999; 30: 606– 611. MEDLINE 25 Amedee-Manesme O, Bernard O, Brunelle F, Hadchouel M, Polonovski C, Baudon JJ, Beguet P, Alagille D. Sclerosing cholangitis with neonatal onset. J Pediatr 1987; 111: 225– 229. MEDLINE 26 de Vree JML, Jacquemin E, Sturm E, Cresteil D, Bosma PJ, Aten J, Deleuze J-F, et al. Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis. Proc Natl Acad Sci U S A 1998; 95: 282– 287. MEDLINE 27 Strickland AD, Shannon K. Studies in the etiology of extrahepatic biliary atresia: time-space clustering. J Pediatr 1982; 100: 749– 753. MEDLINE 28 Morecki R, Glaster JH, Cho S, Balistreri WF, Horwitz MS. Biliary atresia and reovirus type 3 infection. N Engl J Med 1982; 207– 481. 29 Morecki R, Glasere JH, Johnson AB, Kress Y. Detection of reovirus type 3 in the porta hepatis of an infant with extrahepatic biliary atresia: ultrastructural and immunocytochemical study. Hepatology 1984; 4: 1137. MEDLINE 30 Bangaru B, Morecki R, Glaser JH, Gartner LM, Horwitz MS. Comparative studies of biliary atresia in the human newborn and reovirus-induced cholangitis in weanling mice. Lab Invest 1980; 43: 456– 462. MEDLINE 31 Glaser JH, Balistreri WF, Morecki R. Role of reovirus type 3 in persistent infantile cholestasis. J Pediatr 1984; 105: 912– 917. MEDLINE 32 Brown WR, Sokol RJ, Levin MJ, Silverman A, Tamaru T, Lilly JR, Hall RJ, et al. Lack of correlation between infection with reovirus 3 and extrahepatic biliary atresia or neonatal hepatitis. J Pediatr 1988; 113: 670– 676. MEDLINE 33 Steele MI, Marshall CM, Lloyd RE, Randolph VE. Reovirus 3 not detected by reverse transcriptase-mediated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease. Hepatology 1995; 21: 697– 702. MEDLINE 34 Tyler KL, Sokol RJ, Oberhaus SM, Le M, Karrer FM, Narkewicz MR, Tyson RW, Murphy JR, Low R, Brown WR. Detection of reovirus RNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts. Hepatology 1998; 27: 1475– 1482. MEDLINE 35 Taterka J, Sutcliffe M, Rubin DH. Selective reovirus infection of murine hepatocarcinoma cells during cell division: a model of viral disease. J Clin Invest 1994; 94: 353– 360. MEDLINE 36 Riepenhoff-Talty M, Shaekel K, Clark HF, Mueller W, Uhnoo I, Rossi T, Fisher J, et al. Group A rotaviruses produce extrahepatic biliary obstruction in orally enoculated newborn mice. Pediatr Res 1993; 33: 394– 399. MEDLINE 37 Bobo L, Ojeh C, Chiu D, Machado A, Colombani P, Schwarz K. Lack of evidence for rotavirus by polymerase chain reaction/enzyme immunoassay of hepatobiliary samples from children with biliary atresia. Pediatr Res 1997; 41: 229– 234. MEDLINE 38 Harper P, Plant JW, Linger DB. Congenital biliary atresia and jaundice in lambs and calves. Aust Vet J 1990; 67: 18– 22. MEDLINE 39 Yokoyama T, Copeland NG, Jenkins NA, Montgomery CA, Elder FFB, Overbeek PA. Reversal of left-right asymmetry: a situs inversus mutation. Science 1993; 260: 679– 682. MEDLINE 40 Morgan D, Turnpenny L, Goodship J, Dai W, Majumder K, Matthews L, Gardner A. Inversin, a novel gene in the vertebrate left-right axis pathway, is partially deleted in the inv mouse. Nat Genet 1998; 20: 149– 156. MEDLINE 41 Mazziotti MV, Willis LK, Heuckeroth RO, LaRegina MC, Swanson PE, Overbeek PA, Perlmutter DH. Anomalous development of the hepatobiliary system in the Inv mouse. Hepatology 1999; 30: 372– 378. MEDLINE 42 Roberts CWM, Shutter R, Korsmeyer SJ. Hox 11 controls the genesis of the spleen. Nature 1994; 368: 747– 749. MEDLINE 43 Lettice LA, Purdie LA, carlson GJ, Kilanowski F, Dorin J, Hill RE. The mouse bagpipe gene controls development of axial skeleton, skull, and spleen. Proc Natl Acad Sci U S A 1999; 96: 9695– 9700. MEDLINE 44 Lu J, Chang P, Richardson JA, Gan L, Weiler H, Olson EN. The basic helix-loop-helix-transcription factor capsulin controls spleen organogenesis. Proc Natl Acad Sci U S A 2000; 97: 9525– 9530. MEDLINE 45 Nagy P, Bisgaard HC, Torgeirsson SS. Expression of hepatic transcription factors during liver development and oval cell differentiation. J Cell Biol 1994; 126: 223– 233. MEDLINE 46 Stamatoglou SC, Enrich C, Manson MM, Hughes RC. Temporal changes in the expression and distribution of adhesion molecules during liver development and regeneration. J Cell Biol 1992; 116: 1507– 1515. MEDLINE 47 Terada T. Nakanuma Y. Profiles of expression of carbohydrate chain structures during human intrahepatic bile duct development and maturation: a lectin-histochemical and immunohistochemical study. Hepatology 1994; 20: 388– 397. MEDLINE 48 Li L, Krantz ID, Deng Y, Genin A, Banta AB, Collins CC, Qi M, et al. Alagille syndrome is caused by mutations in human Jagged1, which encodes a ligand for Notch1. Nat Genet 1997; 16: 243– 251. MEDLINE 49 Louis AA, Eyken PV, Haber BA, Hicks C, Weinmaster G, Taub R, Rand EB. Hepatic Jagged1 expression studies. Hepatology 1999; 30: 1269– 1275. MEDLINE 50 Rosen EM, Nigam SK, Goldberg ID. Scatter factor and the c-Met receptor: a paradigm for mesenchymal/epithelial interaction. J Cell Biol 1994; 127: 1783– 1787. MEDLINE 51 Huff JL, Jelinek MA, Borgman CA, Lansing TJ, Parsons JT. The proto-oncogene c-Sea encodes a transmembrane protein-tyrosine kinase related to the Met/hepatocyte growth factor/scatter factor receptor. Proc Natl Acad Sci U S A 1993; 90: 6140– 6144. MEDLINE 52 Silveira TR, Salzano FM, Donaldson PT, Mieli-Vergani G, Howard ER, Mowat AP. Association between HLA and extrahepatic biliary atresia. J Pediatr Gastroenterol Nutr 1993; 16: 114– 117. MEDLINE 53 Schreiber RA, Kleinman RE, Barksdale EM, Maganaro TF, Donahue PR. Rejection of murine congenic bile ducts: model for immune-mediated bile duct disease. Gastroenterology 1992; 102: 924– 930. MEDLINE 54 Seidman SL, Duquesnoy RJ, Zeevi A, Fung JJ, Starzl TE, Demetris AJ. Recognition of major histocompatibility complex antigens on cultured human biliary epithelial cells by alloreactive lymphocytes. Hepatology 1991; 13: 239– 246. MEDLINE 55 Dillon P, Belchis D, Tracy T, Cilley R, Hafey I, Krummel T. Increased expression of intracellular adhesion molecules in biliary atresia. Am J Pathol 1994; 145: 263– 267. MEDLINE 56 Smith BM, Laberge J-M, Schreiber R, Weber AM, Blanchard H. Familial biliary atresia in three siblings including twins. J Ped Surg 1991; 26: 1331– 1333. 57 Gunasekaran TS, Hassall EG, Steinbrecher UP, Yong S-L. Recurrence of extrahepatic biliary atresia in two half sibs. Am J Med Genet 1992; 43: 592– 594. MEDLINE 58 Strickland AD, Shannon K, Coln CD. Biliary atresia in two sets of twins. J Pediatr 1985; 107: 418– 419. MEDLINE 59 Moore TC, Hyman PE. Extrahepatic biliary atresia in one human leukocyte antigen identical twin. Pediatrics 1985; 76: 604– 605. MEDLINE Citing Literature Volume35, Issue6June 2002Pages 1297-1304 ReferencesRelatedInformation