Pediatric and Developmental Pathology

, Volume 7, Issue 2, pp 109–124

Biliary Atresia Revisited

Perspectives in Pediatric Pathology


Extrahepatic biliary atresia (EHBA) is an inflammatory fibrosing process affecting the extrahepatic and intrahepatic biliary tree resulting in fibrous obliteration of the extrahepatic biliary tract, ductopenia of intrahepatic bile ducts, and biliary cirrhosis. EHBA is divided into a correctable and a noncorrectable type with focal patency of the otherwise atretic biliary tree in the former and no patency of the biliary tree in the noncorrectable type. EHBA is divided in a fetal, prenatal or embryonic, and a more common, perinatal, acquired form. The symptoms of the former start shortly after birth and there is frequently an association with a variety of congenital anomalies. Children with the perinatal form become jaundiced several weeks after birth; no associated congenital anomalies are present. Morphologically, an inflammatory and fibrosing process of the extrahepatic biliary tree leads to complete lumenal obliteration. The liver is characterized by a nonspecific giant cell transformation, and portal expansion by fibrous connective tissue with marked ductular proliferation. With time, ductopenia and biliary cirrhosis develop. The diffential diagnosis with other conditions with similar microscopic patterns such as as alpha-1 antitrypsin deficiency, total parental nutrition, obstruction by a choledochal cyst, arteriohepatic dysplasia, familial progressive intrahepatic cholestasis, and alteration of the bile acid metabolism is discussed. In the fetal group, abnormalities in different genes seem to play a role; ductal plate malformation is another possibility. Different etiologies have been postulated in the perinatal form of EHBA: genetic susceptilibility, vascular factors, toxins, and infections mainly by rotavirus and reovirus. The pathogenesis is complex. EHBA is a heterogenous disease, resulting from a combination of genetic factors, insults, and activition of different genetic and immunologic pathways. The treatment of EHBA is surgical, with anastomosis between the biliary tree and the intestine in the correctable type and a hepatic portoenterostomy (HPE) for the noncorrectable group. HPE is a temporizing treatment allowing the infant to develop and grow, followed in the majority of the patients by liver transplantation.


cholestasis conjugated hyperbilirubinemia etiology extrahepatic biliary atresia pathogenesis pediatric diseases of the liver 


  1. 1.
    Movat, AP 1996Biliary atresia into the 21st century: a historical perspective.Hepatology2316931695PubMedGoogle Scholar
  2. 2.
    Witzleben, CL 1979Extrahepatic biliary atresia: concepts of cause, diagnosis, and management.Perspect Pediatr Pathol54162PubMedGoogle Scholar
  3. 3.
    Kasai, M 1974Treatment of biliary atresia with special reference to hepatic portoenterostomy and its modifications.Prog Pediatr Surg6552PubMedGoogle Scholar
  4. 4.
    Howard, ER, Mowat, A 1977Extrahepatic biliary atresia. Recent developments in management.Arch Dis Child52825827PubMedGoogle Scholar
  5. 5.
    McKieman, PJ, Baker, AJ, Kelly, DA 2000The frequency and outcome of biliary atresia in the UK and Ireland.Lancet3552529CrossRefPubMedGoogle Scholar
  6. 6.
    Balistreri, WF, Grand, R, Hoofnagle, JH,  et al. 1996Biliary atresia: current concepts and research directions.Hepatology2316821692PubMedGoogle Scholar
  7. 7.
    Ohi, R 2001Surgery for biliary atresia.Liver21175182CrossRefPubMedGoogle Scholar
  8. 8.
    Perlmutter, DH, Shepherd, RW 2002Extrahepatic biliary atresia: a disease or a phenotype?Hepatology3512971304PubMedGoogle Scholar
  9. 9.
    Smith, BM, Laberge, JM, Schreiber, R, Weber, AM, Blanchard, H 1991Familial biliary atresia in three siblings including twins.J Pediatr Surg2613311333PubMedGoogle Scholar
  10. 10.
    Hyams, JS, Glaser, JH, Leichtner, AM, Morechi, R 1985Discordance for biliary atresia in two sets of monozygotic twins.J Pediatr107420422PubMedGoogle Scholar
  11. 11.
    Hart, MH, Kaufman, SS, Vanderhoof, JA,  et al. 1991Neonatal hepatitis and extrahepatic biliary atresia associated with cytomegalovirus infection in twins.Am J Dis Child145302305PubMedGoogle Scholar
  12. 12.
    Fischler, B, Haglund, B, Hjern, A 2002A population-based study on the incidence and possible pre- and perinatal etiologic risk factors of biliary atresia.J Pediatr141217222CrossRefPubMedGoogle Scholar
  13. 13.
    Chardot, C, Carton, M, Spire-Bendelac, N, Le Pommelet, C, Goldmard, JL, Auvert, B 1999Epidemiology of biliary atresia in France: a national study 1986–96.J Hepatol3110061013CrossRefPubMedGoogle Scholar
  14. 14.
    Lai, MW, Chang, MH, Hsu, SC,  et al. 1994Differential diagnosis of extrahepatic biliary atresia from neonatal hepatitis: a prospective study.J Pediatr Gastroenterol Nutr18121127PubMedGoogle Scholar
  15. 15.
    Kahn, E, Daum, F, Markowitz, J,  et al. 1983Arteriohepatic dysplasia. II—Hepatobiliary morphology.Hepatology37784PubMedGoogle Scholar
  16. 16.
    Guelrud, M, Jaen, D, Mendoza, S, Plaz, J, Torres, P 1991ERCP in the diagnosis of extrahepatic biliary atresia.Gastrointest Endosc37522526PubMedGoogle Scholar
  17. 17.
    Silveira, TR, Salzano, FM, Howard, ER, Movat, AP 1991Congenital structural abnormalities in biliary atresia: evidence for etiopathogenetic heterogeneity and therapeutic implications.Acta Paed Scand8011921199Google Scholar
  18. 18.
    Silveira, TR, Salzano, FM, Donaldson, PT, Mieli-Vergani, G, Howard, ER, Movat, AP 1993Association between HLA and extrahepatic biliary atresia.J Pediatr Gastroenterol Nutr1611141117Google Scholar
  19. 19.
    Chandra, RS 1974Biliary atresia and other structural anomalies in the congenital polysplenia syndrome.J Pediatr85649653PubMedGoogle Scholar
  20. 20.
    Schreiber, RA, Kleinman, RE 1993Genetics, immunology, and biliary atresia: an opening or a diversion?J Pediatr Gastroenterol Nutr16111113PubMedGoogle Scholar
  21. 21.
    Davenport, M, Savage, M, Mowat, AP, Howard, ER 1993Biliary atresia splenic malformation syndrome: an etiologic and prognostic subgroup.Surgery113662668PubMedGoogle Scholar
  22. 22.
    Chandra, RS, Altman, RP 1978Ductal remnants in extrahepatic biliary atresia: a histopathological study with clinical correlation.J Pediatr3196200Google Scholar
  23. 23.
    Tan, CEL, Davenport, M, Driver, M, Howard, ER 1994Does the morphology of the extrahepatic biliary remnants in biliary atresia influence survival? A review of 205 cases.J Pediatr Surg2914591464PubMedGoogle Scholar
  24. 24.
    Nietgen, GW, Vacanti, JP, Perez-Atayde, AR 1992Intrahepatic bile duct loss in biliary atresia despite portoenterostomy: a consequence of ongoing obstruction?Gastroenterology10221262133PubMedGoogle Scholar
  25. 25.
    Desmet, VJ 1998Ludwig symposium on biliary disorders—part I. Pathogenesis of ductal plate abnormalities.Mayo Clin Proc738089PubMedGoogle Scholar
  26. 26.
    Low, Y, Vijayan, V, Tan, CEL 2001The prognostic value of ductal plate malformation and other histologic parameters in biliary atresia: an immunohistochemical study.J Pediatr139320322CrossRefPubMedGoogle Scholar
  27. 27.
    Cocjin, J, Rosenthal, P, Buslon, V,  et al. 1996Bile ductule formation in fetal, neonatal, and infant livers compared with extrahepatic biliary atresia.Hepatology24568574PubMedGoogle Scholar
  28. 28.
    Okamoto, S, Oike, F, Egawa, H, Inomata, Y, Tanaka, K 1996Heterogeneity of the explanted liver at the time of transplantation after failed Kasai procedure for biliary atresia: an immunohistochemical study of biliary system.Hepatology24556ACrossRefGoogle Scholar
  29. 29.
    Shirahase, I, Ooshima, A, Tanaka, K, Ozawa, K, Yamabe, H, Yamamoto, Y 1993Increased deposition and serum level of type IV collagen in patients with extrahepatic biliary atresia.J Pediatr Surg28847850PubMedGoogle Scholar
  30. 30.
    Shirahase, I, Ooshima, A, Tanaka, K,  et al. 1994Immunohistochemical demonstration of collagen types III and IV and myofibroblasts in the liver of patients with biliary atresia.J Pediatr Surg29639644PubMedGoogle Scholar
  31. 31.
    Ho, C-W, Shioda, K, Shirasaki, K, Takahashi, S, Tokimatsu, S, Maeda, K 1993The pathogenesis of biliary atresia: a morphological study of the hepatobiliary system and the hepatic artery.J Pediatr Gastroenterol Nutr165360PubMedGoogle Scholar
  32. 32.
    Deutsch, GH, Sokol, RJ, Stathos, TH, Knisely, AS 2002Proliferation to paucity: evolution of bile duct abnormalities in a case of Alagille syndrome.Pediatr Dev Pathol4559563CrossRefGoogle Scholar
  33. 33.
    Quiros-Tejeira, RE, Ament, ME, Heyman, MB,  et al. 1999Variable morbidity in Alagille syndrome: a review of 43 cases.J Pediatr Gastroenterol Nutr29431437CrossRefPubMedGoogle Scholar
  34. 34.
    Azar, G, Beneck, D, Lane, B, Markowitz, J, Daum, F, Kahn, E 2002Atypical morphologic presentation of biliary atresia and value of serial liver biopsies.J Pediatr Gastroenterol Nutr34212215CrossRefPubMedGoogle Scholar
  35. 35.
    Landing, BH 1974Considerations of the pathogenesis of neonatal hepatitis, biliary atresia and choledochal cyst. The concept of infantile obstructive cholangiopathy.Progr Pediatr Surg6113139Google Scholar
  36. 36.
    Kahn E, Vadmal M, Markowitz J (1998) Ontogeny of the bile duct to portal space ratio (BD/PS) revisited. Impact of AE1/AE3 staining (Abstract). Mod Pathol 11:3PGoogle Scholar
  37. 37.
    Yokoyama, T, Copeland, NG, Jenkins, NA, Montgomery, CA, Elder, FFB, Overbeek, PA 1993Reversal of left-right asymmetry: a situs inversus mutation.Science260679682PubMedGoogle Scholar
  38. 38.
    Jacquemin, E, Setchell, KDR, O’Connell, NC,  et al. 1994A new cause of progressive intrahepatic cholestasis: 3beta-hydroxy-C27-steroid dehydrogenase/isomerase deficiency.J Pediatr125379384PubMedGoogle Scholar
  39. 39.
    Willis, LK, Mazziotti, MV, LaRegina, MC, Swanson, PE, Overbeek, PA, Perlmutter, DH 1998The inv mouse resembles a type of extrahepatic biliary atresia associated with situs inversus.Hepatology28645AGoogle Scholar
  40. 40.
    Mazziotti, MV, Willis, LK, Heuckeroth, RO,  et al. 1999Anomalous development of the hepatobiliary system in the inv mouse.Hepatology30372378PubMedGoogle Scholar
  41. 41.
    Teichberg, S, Markowitz, J, Silverberg, M,  et al. 1982Abnormal cilia in a child with polysplenia syndrome, extrahepatic biliary atresia and abdominal situs inversus.J Pediatr100399401PubMedGoogle Scholar
  42. 42.
    Jacquemin, E, Cresteil, D, Raynaud, N, Hadchouel, M 2002CFC1 gene mutation and biliary atresia with polysplenia syndrome.J Pediatr Gastroenterol Nutr34325326CrossRefPubMedGoogle Scholar
  43. 43.
    Bamford, RN, Roessler, E, Burdine, RD,  et al. 2000Loss-of-function mutations in the EGF-CFC gene CFC1 are associated with human left-right laterality defects.Nat Genet26365369PubMedGoogle Scholar
  44. 44.
    Louis, AA, Van Eyken, P, Haber, BA,  et al. 1999Hepatic jagged1 expression studies.Hepatology302691275Google Scholar
  45. 45.
    Kohsaka, T, Yuan, ZR, Guo, SX,  et al. 2002The significance of human jagged 1 mutations detected in severe cases of extrahepatic biliary atresia.Hepatology36904912PubMedGoogle Scholar
  46. 46.
    Gunasekaran, TS, Hassall, EG, Steinbrecher, UP, Yong, SL 1992Recurrence of extrahepatic biliary atresia in two half sibs.Am J Med Genet43592594PubMedGoogle Scholar
  47. 47.
    A-Kader, HH, El-Ayyouti, M, Hawas, S,  et al. 2002HLA in Egyptian children with biliary atresia.J Pediatr141432434CrossRefPubMedGoogle Scholar
  48. 48.
    Nakada, M, Nakada, K, Kawaguchi, F,  et al. 1997Immunologic reaction and genetic factors in biliary atresia.Tohoku J Exp Med1814147PubMedGoogle Scholar
  49. 49.
    Clare, M, Constantini, PK, Randall, RJ,  et al. 1998Immunogenetic assessment of biliary atresia: a candidate gene search.Hepatology28241AGoogle Scholar
  50. 50.
    Sokol, RJ, Mack, C 2001Etiopathogenesis of biliary atresia.Semin Liver Dis21517524CrossRefPubMedGoogle Scholar
  51. 51.
    Donaldson, PT, Clare, M, Constantini, PK,  et al. 2002HLA and cytokine gene polymorphisms in biliary atresia.Liver22213219PubMedGoogle Scholar
  52. 52.
    Harper, P, Plant, JW, Linger, DB 1990Congenital biliary atresia and jaundice in lambs and calves.Aust Vet J671822PubMedGoogle Scholar
  53. 53.
    Riepenhoff-Talty, M, Gouvea, V, Evans, MJ,  et al. 1996Detection of group C rotavirus in infants with extrahepatic biliary atresia.J Infect Dis174815PubMedGoogle Scholar
  54. 54.
    Drut, R, Gomez, MA, Drut, RM, Lojo, MM 1996Human papillomavirus (HPV)-associated neonatal giant cell hepatitis.Pediatr Pathol Lab Med16403412PubMedGoogle Scholar
  55. 55.
    Fischler, B, Ehrnst, A, Forsgren, M, Orvell, C, Nemeth, A 1998The viral association of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepatic biliary atresia.J Pediatr Gastroenterol Nutr275764CrossRefPubMedGoogle Scholar
  56. 56.
    Domiati-Saad, R, Dawson, DB, Margraf, LR, Finegold, MJ, Weinberg, AG, Rogers, BB 2000Cytomegalovirus and human herpesvirus 6, but not human papillomavirus, are present in neonatal giant cell hepatitis and extrahepatic biliary atresia.Pediatr Dev Pathol3367373CrossRefPubMedGoogle Scholar
  57. 57.
    Jevon, GP, Dimmick, JE 1999Biliary atresia and cytomegalovirus infection: a DNA study.Pediatr Dev Pathol21114CrossRefPubMedGoogle Scholar
  58. 58.
    Bangaru, B, Morecki, R, Glaser, JH, Gartner, LM, Horwitz, MS 1980Comparative studies of biliary atresia in the human newborn and reovirus-induced cholangitis in weanling mice.Lab Invest43456462PubMedGoogle Scholar
  59. 59.
    Matsui A, Sasaki N, Tanaka, Kobita K (1992) Reovirus type 3 and biliary atresia (Abstract). Hepatology 1992;16:192AGoogle Scholar
  60. 60.
    Morecki, R, Glaser, JH, Cho, S,  et al. 1982Biliary atresia and reovirus type 3 infection.N Engl J Med307481484PubMedGoogle Scholar
  61. 61.
    Morecki, R, Glaser, JH, Johnson, AB, Kress, Y 1984Detection of reovirus type 3 in the porta hepatis of an infant with extrahepatic biliary atresia: ultrastructural and immunocytochemical study.Hepatology411371142PubMedGoogle Scholar
  62. 62.
    Rosenberg, DP, Morecki, R, Lollini, LO, Glaser, J, Cornelius, CE 1983Extrahepatic biliary atresia in a rhesus monkey (Macaca mulatta).Hepatology3577581PubMedGoogle Scholar
  63. 63.
    Petersen, C, Bruns, E, Kuske, M, von Wussow, P 1997Treatment of extrahepatic biliary atresia with interferon-alpha in a murine infectious model.Pediatr Res42623628PubMedGoogle Scholar
  64. 64.
    Petersen, C, Grasshoff, S, Luciano, L 1998Diverse morphology of biliary atresia in an animal model.J Hepatol28603607CrossRefPubMedGoogle Scholar
  65. 65.
    Tyler, KL, Sokol, RJ, Oberhaus, SM,  et al. 1998Detection of reovirus DNA in hepatobiliary tissues from patients with extrahepatic biliary atresia and choledochal cysts.Hepatology2714751482PubMedGoogle Scholar
  66. 66.
    Brown, WR, Sokol, RJ, Levin, MJ,  et al. 1988Lack of correlation between infection with reovirus 3 and extrahepatic biliary atresia or neonatal hepatitis.J Pediatr113670676PubMedGoogle Scholar
  67. 67.
    Parashar, K, Tarlow, MJ, McCrae, MA 1992Experimental reovirus type 3-induced murine biliary tract disease.J Pediatr Surg27843847PubMedGoogle Scholar
  68. 68.
    Sokol RJ, Endo N, Ohara JI, et al (1991) Reovirus 3 RNA not detected by polymerase chain reaction in tissues from infants with extrahepatic biliary atresia (Abstract). Hepatology 14:125AGoogle Scholar
  69. 69.
    Steele, MI, Marshall, CM, Lloyd, R, Randolph, VE 1995Reovirus 3 not detected by reverse transcriptase-mediated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease.Hepatology21697702PubMedGoogle Scholar
  70. 70.
    Riepenhoff-Talty, M, Schaekel, K, Clark, HF,  et al. 1993Group A rotaviruses produce extrahepatic biliary obstruction in orally inoculated newborn mice.Pediatr Res33394399PubMedGoogle Scholar
  71. 71.
    Tan, CEL, Driver, M, Howard, ER, Moscoso, GJ 1994Extrahepatic biliary atresia: a first-trimester event? Clues from light microscopy and immunohistochemistry.J Pediatr Surg29808814PubMedGoogle Scholar
  72. 72.
    Tan, CEL, Moscoso, GJ 1994The developing human biliary system at the porta hepatis level between 11 and 25 weeks of gestation: a way to understanding biliary atresia. Part 2.Pathol Intern44600610Google Scholar
  73. 73.
    Rosen, EM, Nigam, SK, Goldberg, ID 1994Scatter factor and the c-Met receptor: a paradigm for mesenchymal/epithelial interaction.J Cell Biol12717831787PubMedGoogle Scholar
  74. 74.
    Dillon, P, Belchis, D, Tracy, T, Cilley, R, Hafer, L, Krummel, T 1994Increased expression of intercellular adhesion molecules in biliary atresia.Am J Pathol145263267PubMedGoogle Scholar
  75. 75.
    Fabris, L, Stazzabosco, M, Crobsy, HA,  et al. 2000Characterization and isolation of ductular cells coexpressing neural cell adhesion molecule and bcl-2 from primary cholangiopathies and ductal plate malformations.Am J Pathol15615991612PubMedGoogle Scholar
  76. 76.
    Davenport, M, Gonde, C, Redkar, R,  et al. 2001Immunohistochemistry of the liver and biliary tree in extrahepatic biliary atresia.J Pediatr Surg3610171025CrossRefPubMedGoogle Scholar
  77. 77.
    Ramm, GA, Nair, VG, Bridle, KR, Shepherd, RW, Crawford, DHG 1998Contribution of hepatic parenchymal and nonparenchymal cells to hepatic fibrogenesis in biliary atresia.Am J Pathol153527535PubMedGoogle Scholar
  78. 78.
    Liu, C, Jen-Chiu, H, Chin, T,  et al. 2000Expression of Fas ligand on bile ductule epithelium in biliary atresia—a poor prognostic factor.J Pediatr Surg3515911596CrossRefPubMedGoogle Scholar
  79. 79.
    Bezerra, JA, Tiao, G, Ryckman, FC, Alonso, M,  et al. 2002Genetic induction of proinflammatory immunity in children with biliary atresia.Lancet36016531659CrossRefPubMedGoogle Scholar
  80. 80.
    Kobayashi, H, Narumi, S, Tamatani, T, Lane, GJ, Miyano, T 1999Serum IFN-inducible protein-10: a new clinical prognostic predictor of hepatocyte death in biliary atresia.J Pediatr Surg34308311PubMedGoogle Scholar
  81. 81.
    Urushihara, N, Iwagaki, H, Yagi, T,  et al. 2000Elevation of serum interleukin-18 levels and activation of Kupffer cells in biliary atresia.J Pediatr Surg35446449PubMedGoogle Scholar
  82. 82.
    Ahmed, AFKU, Ohtani, H, Nio, M,  et al. 2000In situ expression of fibrogenic growth factors and their receptors in biliary atresia: comparison between early and late stages.J Pathol1927380CrossRefPubMedGoogle Scholar
  83. 83.
    Tan, CEL, Chan, VSW, Yong, RYY,  et al. 1995Distortion of TGFb1 peptide immunolocalisation in biliary atresia: comparison with the normal pattern in the developing human intrahepatic bile duct system.Pathol Int45815824PubMedGoogle Scholar
  84. 84.
    Dillon, PW, Owings, E, Cilley, R, Field, D, Curnow, A, Hershey, KG 2001Immunosupppression as adjuvant therapy for biliary atresia.J Pediatr Surg368085CrossRefPubMedGoogle Scholar
  85. 85.
    Kasai, M, Kimura, S, Asakura, Y 1968Surgical treatment of biliary atresia.J Pediatr Surg3665675Google Scholar
  86. 86.
    Alagille, D 1984Extrahepatic biliary atresia.Hepatology47S10SPubMedGoogle Scholar
  87. 87.
    Engelskirchen, R, Holschneider, AM, Gharib, M, Vente, C 1990Biliary atresia—a 25 year survey.Eur J Pediatr Surg1154160Google Scholar
  88. 88.
    Rudoph, JA, Balistreri, WF 1999Optimal treatment of biliary atresia—“halfway” there!Hepatology30808810PubMedGoogle Scholar
  89. 89.
    Karrer, FM, Price, MR, Bensard, DD,  et al. 1996Long-term results with the Kasai operation for biliary atresia.Arch Surg131493496Google Scholar
  90. 90.
    Laurent, J, Gauthier, F, Bernard, O,  et al. 1990Long-term outcome after surgery for biliary atresia. Study of 40 patients surviving for more than 10 years.Gastroenterology9917931797PubMedGoogle Scholar
  91. 91.
    Starzl, TE 1983

    Liver transplantation for biliary atresia.

    Daum, F eds. Gastroenterology, vol. 1: Extrahepatic Biliary Atresia.Marcel DekkerNew York7990
    Google Scholar
  92. 92.
    Whitington, PF, Balistreri, WF 1991Liver transplantation in pediatrics: indications, contra-indications, and pretransplant management.J Pediatr118169177PubMedGoogle Scholar
  93. 93.
    Otte, JB, de Ville de Goyet, J, Reding, R,  et al. 1994Sequential treatment of biliary atresia with Kasai portoenterostomy and liver transplantation: a review.Hepatology2041S48SPubMedGoogle Scholar
  94. 94.
    Martinez-Ibanez, V, Boix-Ochoa, J, Lloret, J, Broto, J 1992Pediatric liver transplantation: life after portoenterostomy in biliary atresia.J Pediatr Surg27830832PubMedGoogle Scholar
  95. 95.
    Fujimoto, T, Ohya, T, Miyano, T 1994A new clinical prognostic predictor for patients with biliary atresia.J Pediatr Surg29757760PubMedGoogle Scholar
  96. 96.
    National Institute of Health Consensus Development Conference Statement: Liver transplantation—June 20–23, 1983. Hepatopathology 1984;4(Suppl):107S–110S.Google Scholar
  97. 97.
    Broelsch, CE, Emond, JC, Whitington, PF, Thislethwaite, JR, Baker, AL, Lichtor, JJ 1990Application of reduced-size liver transplants as split grafts, auxiliary orthotopic grafts and living related segmental transplants.Ann Surg212368372PubMedGoogle Scholar
  98. 98.
    Beath, S, Pearmain, G, Kelly, D, McMaster, P, Mayer, A, Buckels, J 1993Liver transplantation in babies and children with extrahepatic biliary atresia.J Pediatr Surg2810441047PubMedGoogle Scholar
  99. 99.
    Ryckmann, F, Fisher, R, Pedersen, S,  et al. 1993Improved survival in biliary atresia patients in the present era of liver transplantation.J Pediatr Surg28382386PubMedGoogle Scholar
  100. 100.
    Losty, P, Guiney, E 1992Biliary atresia—lessons from the Dublin experience.Irish Med J85144147Google Scholar
  101. 101.
    Kasai M, Susuki H, Ohasi, Ohi R, Chiba T, Okamoto A (1978) Technique and results of operative management of biliary atresia. World J Surg 2:571–580.PubMedGoogle Scholar
  102. 102.
    Balestreri, WF 1985Neonatal cholestasis.J Pediatr106171184PubMedGoogle Scholar
  103. 103.
    Suruga, K, Tsunoda, S, Deguchi, E, Miyano, T 1992The future role of hepatic portoenterostomy as treatment of biliary atresia.J Pediatr Surg7707709Google Scholar
  104. 104.
    Oh, M, Hobeldin, M, Chen, T, Thomas, DW, Atkinson, JB 1995The Kasai procedure in the treatment of biliary atresia.J Pediatr Surg3010771081PubMedGoogle Scholar
  105. 105.
    Ohi, R, Ibrahim, M 1992Biliary atresia.Semin Pediatr Surg1115124PubMedGoogle Scholar
  106. 106.
    Oike, F, Oshima, A, Okamoto, S,  et al. 1996Differences in fibrogenic process between early failure and late failure of Kasai operation in biliary atresia.Hepatology24556ACrossRefGoogle Scholar
  107. 107.
    Segawa, O, Miyano, T, Fujimoto, T, Watanabe, S, Hirose, M, Fujiwara, T 1993Actin and myosin deposition around bile canaliculi: a predictor of clinical outcome in biliary atresia.J Pediatr Surg28851856PubMedGoogle Scholar

Copyright information

© Society for Pediatric Pathology 2004

Authors and Affiliations

  1. 1.Department of PathologyNorth Shore University Hospital, NYU School of Medicine, 300 Community Drive, Manhasset, NY 11030USA

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