ETIOLOGY, PATHOGENESIS AND MORPHOLOGY OF BILIARY ATRESIA
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 heterogein...
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| Main Authors: | , , , |
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| Format: | Article |
| Language: | Russian |
| Published: |
Federal Research Center of Transplantology and Artificial Organs named after V.I.Shumakov
2012-09-01
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| Series: | Вестник трансплантологии и искусственных органов |
| Subjects: | |
| Online Access: | https://journal.transpl.ru/vtio/article/view/193 |
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| Summary: | 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. |
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| ISSN: | 1995-1191 |