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Review
. 2020 Mar 17;6(5):FSO466.
doi: 10.2144/fsoa-2019-0153.

Biliary atresia: pathology, etiology and pathogenesis

Mukul Vij  1 , Mohamed Rela  2   3
Affiliations
Review

Biliary atresia: pathology, etiology and pathogenesis

Mukul Vij et al. Future Sci OA. .

Abstract

Biliary atresia is a progressive fibrosing obstructive cholangiopathy of the intrahepatic and extrahepatic biliary system, resulting in obstruction of bile flow and neonatal jaundice. Histopathological findings in liver biopsies include the expansion of the portal tracts, with edematous fibroplasia and bile ductular proliferation, with bile plugs in duct lumen. Lobular morphological features may include variable multinucleate giant cells, bilirubinostasis and hemopoiesis. The etiopathogenesis of biliary atresia is multifactorial and multiple pathomechanisms have been proposed. Experimental and clinical studies have suggested that viral infection initiates biliary epithelium destruction and release of antigens that trigger a Th1 immune response, which leads to further injury of the bile duct, resulting in inflammation and obstructive scarring of the biliary tree. It has also been postulated that biliary atresia is caused by a defect in the normal remodelling process. Genetic predisposition has also been proposed as a factor for the development of biliary atresia.

Keywords: biliary atresia; etiology; genetics; immunology; morphogenesis; pathogenesis; pathology; toxins; viruses.

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Conflict of interest statement

Financial & competing interests disclosure The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript.

Figures

Figure 1.
Figure 1.. Liver biopsy displaying portal fibrous expansion with ductular proliferation (hematoxylin and eosin, x10).
Figure 2.
Figure 2.. Ductal plate malformation like arrays in atresia biopsy (hematoxylin and eosin, x10).
See this image and copyright information in PMC

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