ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA
https://doi.org/10.15825/1995-1191-2012-3-69-76
Abstract
Biliary atresia is an inflammatory fibrosing process, which invades both extrahepatic and intrahepatic bile ducts. There are two forms of biliary atresia: embryonic form, associated with another visceral malformations, and perinatal form, which is an isolated disease. Biliary atresia is a heterogeinc disease which may appear as a result of different etiological factors, the most important of them are genetics, viral infection, vascular factors, toxins and disregulation of immune system. The characteristic morphological features of biliaryatresia are: intracellular and ductular cholestasis, ductular proliferation, giant cell transformation, portal and perilobular oedema and/or fibrosis. As the patalogical process progresses, ductopenia appears, and fibrosis turns to cirrhosis. Embryonic form mophologically manifests with ductal plate malformation is, what is an important diagnostic criterion. Pro- gnosis of biliary atresia depends on form of disease, age of patient and type of surgical operation. Today surgical treatment includes two steps: palliative portoenterostomia, performed in neonatal period and further liver trans- plantation. However liver transplantation is more preferable as first and single-step treatment.
About the Authors
O. E. Iryshkin
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
I. M. Ilyinsky
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
O. M. Tsirulnikova
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
S. V. Gautier
Academician V.I. Shumakov Federal Research Center of Transplantology and Artificial Organs, Moscow
References
1. Готье С.В., Константинов Б.А., Цирульникова О. М. Трансплантация печени. М.: Мед. информационное агентство, 2008. 246 с.
2. Дегтярева А.В. Дифференциальная диагностика и принципы этиопатогенетического лечения заболева- ний печени и желчных путей у новорожденных и де- тей раннего возраста: Автореферат дис. ... докт. мед. наук. М., 2008. 48 с.
3. Цирульникова О.М., Готье С.В. Трансплантация пе- чени / Под ред. Н.А. Мухина // Практическая гепато- логия. М.: Проект «МЫ», 2004. С. 237–244.
4. Alexopoulos S.P., Merrill M., Kin C. et al. The impact of hepatic portoenterostomy on liver transplantation for the treatment of biliary atresia: Early failure adversely affects outcome // Pediatr. Transplant. 2012. Vol. 16. P. 373–378.
5. Awasthi A., Das A., Srinivasan R., Joshi K. Morpholo- gical and immunohistochemical analysis of ductal plate malformation: correlation with fetal liver // Histopatho- logy. 2004. Vol. 45. P. 260–267.
6. Azar G., Benecck D., Lane B. et al. Atypical morpholo- gic presentation of biliary atresia and value of serial liver biopsies // J. Pediatr. Gastroenterol. Nutr. 2002. Vol. 34. P. 212–215.
7. Balistreri W.F., Grand R., Hoofnagle J.H. et al. Bilia- ry Atresia: Current Concepts and Research Directions Summary of a Symposium // Hepatology. 1996. Vol. 23. P. 1682–1692.
8. Baumann U., Ure B. Biliary atresia // Clin. Res. Hepatol. Gastroenterol. 2012.
9. Broniszczak D., Apanasiewicz A., Czubkowski P. et al. Liver transplantation in children with biliary atresia and polysplenia syndrome // Ann. Transplant. 2011. Vol. 16. P. 14–17.
10. Caudle S.E., Katzenstein J.M., Karpen S., Valérie M. De- velopmental Assessment of Infants with Biliary Atresia: Differences between Males and Females // J. Pediatr. Gastroenterol. Nutr. 2012.
11. Chardot C. Biliary atresia // Orphanet Journal of Rare Diseases. 2006. Vol. 1. P. 1–28.
12. Chardot C., Carton M., Spire-Bendelac N. et al. Prog- nosis of biliary atresia in the era of liver transplantation: French national study from 1986 to 1996 // Hepatology. 1999. Vol. 30. P. 606–611.
13. Chardot C., Debray D. Biliary atresia: a condition re- quiring urgent diagnosis and treatment // Arch. Pediatr. 2011. Vol. 18. P. 476–481.
14. Davenport M. Biliary atresia // Semin. Pediatr. Surg. 2005. Vol. 14. P. 42–48.
15. Davenport M., Gonde C., Redkar R. et al. Immunohis- tochemistry of the liver and biliary tree in extrahepatic biliary atresia // J. Pediatr. Surg. 2001. Vol. 36. P. 1017– 1025.
16. Dehghani S.M., Haghighat M., Imanieh M.H., Geramiz- adeh B. Comparison of different diagnostic methods in infants with Cholestasis // World J. Gastroenterol. 2006. Vol. 12. P. 5893–5896.
17. Desmet V.J., Rosai J. From Liver, Non-neoplastic Disea- ses // Rosai J., ed. Ackerman’s Surgical Pathology. 2004. P. 949–950.
18. Drut R., Drut R.M., Gomez M.A. et al. Presence of human papillomavirus in extrahepatic biliary atresia // J. Pedia- tr. Gastroenterol. Nutr. 1998. Vol. 27. P. 530–535.
19. Fischler B., Ehrnst A., Forsgren M. et al. The viral as- sociation of neonatal cholestasis in Sweden: a possible link between cytomegalovirus infection and extrahepa- tic biliary atresia // J. Pediatr. Gastroenterol. Nutr. 1998. Vol. 27. P. 57–64.
20. Friedman S.L., Roll F.J., Boyles J., Bissel D.M. Hepatic lipocytes: the principal collagen-producing cells of nor- mal rat liver // Proc. Natl. Acad. Sci USA. 1985. Vol. 82. P. 8681–8685.
21. Garcia A.V., Cowles R.A., Kato T., Hardy M.A. Morio Kasai: a remarkable impact beyond the Kasai procedu- re // J. Pediatr. Surg. 2012. Vol. 47. P.1023–1027.
22. Glaser J.H., Balistreri W.F., Morecki R. Role of reovi- rus type 3 in persistent infantile cholestasis // J. Pediatr. 1984. Vol. 105. P. 912–915.
23. Ho C.W., Shioda K., Shirasaki K. et al. The pathogenesis of biliary atresia: a morphological study of the hepatobi- liary system and the hepatic artery // J. Pediatr. Gastroen- terol. Nutr. 1993. Vol. 16. P. 53–60.
24. Hofmann A.F., Popper H. Ursodeoxycholic acid for pri- mary biliary cirrhosis // Lancet. 1987. Vol. 2. P. 398– 399.
25. Houwen R.H., Kerremans I., van Steensel-Moll H.A. et al. Time-space distribution of extrahepatic biliary atresia in The Netherlands and West Germany // Z. Kinderchir. 1988. Vol. 43. P. 68–71.
26. Howard E.N., Stringer M.D., Colombani P.M. Surgery of the Liver, Bile duct and Pancreas in Children. London: Arnold, 2002. 572 p.
27. Jung E., Park W.H., Choi S.O. Late complications and current status of long-term survivals over 10 years after Kasai portoenterostomy // J. Korean. Surg. Soc. 2011. Vol. 81. P. 271–275.
28. Kalayoglu M., Stratta R.J., Sollinger H.W. Liver trans- plantation in infants and children // J. Pediatr Surg. 1989. Vol. 24. P. 70–76.
29. Karrer F.M., Bensard D.D. Neonatal cholestasis // Se- min. Pediatr. Surg. 2000. Vol. 9. P. 166–169.
30. Kumagi T., Drenth J.P., Guttman O. et al. Biliary atresia and survival into adulthood without transplantation: a collaborative multicentre clinic review // Liver Int. 2012. Vol. 32. P. 510–518.
31. Lai H.S., Chen W.J., Chen C.C. et al. Long-term prog- nosis and factors affecting biliary atresia from experi- ence over a 25 year period // Chang. Gung. Med. J. 2006. Vol. 29. P. 234–239.
32. Mack C.L. The pathogenesis of biliary atresia: evidence for a virus-induced autoimmune disease // Semin. Liver Dis. 2007. Vol. 27. P. 233–242.
33. Mack L.M., Sokol R.J. Unraveling the Pathogenesis and Etiology of Biliary Atresia // Pediatric Research. 2005. Vol. 57. P. 87–94.
34. Matsuo S., Suita S., Kubota M., Shono K. Long-term results and clinical problems after portoenterostomy in patients with biliary atresia // Eur. J. Pediatr. Surg. 1998. Vol. 8. P. 142–145.
35. McKiernan P.J., Baker A.J., Kelly D.A. The frequency and outcome of biliary atresia in the UK and Ireland // Lancet. 2000. Vol. 355. P. 25–29.
36. Oetzmann von Sochaczewski C., Petersen C., Ure B.M. et al. Laparoscopic versus conventional kasai portoen- terostomy does not facilitate subsequent liver transplan- tation in infants with biliary atresia // J. Laparoendosc. Adv. Surg. 2012. Vol. 22. P. 408–411.
37. Pakarinen M.P., Rintala R.J. Surgery of biliary atresia // Scand. J. Surg. 2011. Vol. 100. P. 49–53.
38. Riepenhoff-Talty M., Gouvea V., Evans M.J. et al. De- tection of group C rotavirus in infants with extrahepatic biliary atresia // J. Infect. Dis. 1996. Vol. 174. P. 8–15.
39. Roy P., Chatterjee U., Ganguli M. et al. A histopatho- logical study of liver and biliary remnants with clinical outcome in cases of extrahepatic biliary atresia // Indian J. Pathol. Microbiol. 2010. Vol. 53. P. 101–105.
40. Schweizer P. Extrahepatic bile duct atresia: how efficient is the hepatoporto-enterostomy? // Eur. J. Pediatr. Surg. 1998. Vol. 8. P. 150–154.
41. Shalaby A., Makin E., Davenport M. Classification of the biliary atresia phenotype // Pediatr. Int. 2010. Vol. 52.
42. Silveira T.R., Salzano F.M., Donaldson P.T. et al. Asso- ciation between HLA and extrahepatic biliary atresia // J. Pediatr. Gastroenterol. Nutr. 1993. Vol. 16. P. 114–117.
43. Smith B.M., Laberge J.M., Schreiber R. et al. Familial bi- liary atresia in three siblings including twins // J. Pediatr. Surg. 1991. Vol. 26. P. 1331–1333.
44. Soomro G.B., Abbas Z., Hassan M. et al. Is there any as- sociation of extra hepatic biliary atresia with cytomega- lovirus or other infections? // J. Pak. Med. Assoc. 2011. Vol. 61. P. 281–283.
45. Steele M.I., Marshall C.M., Lloyd R.E., Randolph V.E. Reovirus 3 not detected by reverse transcriptase-medi- ated polymerase chain reaction analysis of preserved tissue from infants with cholestatic liver disease // Hepa- tology. 1995. Vol. 21. P. 697–702.
46. Vries W., Langen Z.J., Groen H. et al. Biliary atresia in the Netherlands: outcome of patients diagnosed between 1987 and 2008 // J. Pediatr. 2012. Vol. 160. P. 638–644.
47. Xu Y., Yu J., Zhang R. The perinatal infection of cytome- galovirus is an important etiology for biliary atresia in China // Clin. Pediatr. 2012. Vol. 51. P.109–113.
48. Yamaquti D.C., Patricio F.R. Morphometrical and im- munohistochemical study of intrahepatic bile ducts in biliary atresia // Eur. J. Gastroenterol. Hepatol. 2011. Vol. 23. P. 759–765.
49. Yoon P.W., Bresee J.S., Olney R.S. et al. Epidemiology of biliary atresia: a population-based study // Pediatrics. 1997. Vol. 99. P. 376–382.
Review
For citations:
Iryshkin O.E., Ilyinsky I.M., Tsirulnikova O.M., Gautier S.V. ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA. Russian Journal of Transplantology and Artificial Organs. 2012;14(3):69-76. (In Russ.) https://doi.org/10.15825/1995-1191-2012-3-69-76
Views:
1073
Email this article 