Biliary atresia: A comprehensive review

Intrahepatic bile duct primary cilia in biliary atresia

“…At present, the most discussed theories for the etiopathogenesis of isolated BA include viral infections and aberrant immune‐mediated processes. Factors of both innate and adaptive immunity, including early aberrant expression of HLA class II antigens, intercellular adhesion molecules, and vascular cell adhesion molecules on the surface of BECs and the vascular endothelium are associated with intense mononuclear cell infiltrates of mainly Th1 immune response in the livers of infants with isolated BA …”

“…Biliary atresia is not a uniform disease and at least four different variants are identifiable from clinical observation alone, including cystic BA (10%), cytomegalovirus immunoglobulin M (CMV IgM)‐positive BA, and syndromic BA, often called the BA splenic malformation syndrome (BASM), which constitutes approximately 10% of BA cases. In BASM, BA is associated with “laterality defects” including asplenia or polysplenia, situs inversus, cardiac defects, preduodenal portal vein, and/or malrotation .…”

Update on investigations pertaining to the pathogenesis of biliary atresia

“…The incidence ranges from 1:5000 to 1:19,000 births, and is dependent on geographic location [1–4]. Biliary atresia has been found to occur more frequently in Asian countries such as Taiwan and Japan in comparison to North America and Europe [4].…”

“…Biliary atresia has been found to occur more frequently in Asian countries such as Taiwan and Japan in comparison to North America and Europe [4]. According to the National Birth Defects Prevention Study on BA risk factors (1997–2002), infants with BA were more likely to be preterm, born to non-Hispanic Black mothers, and were more likely to have been conceived in the Spring than the Winter [1].…”

Increased expression of senescence‐associated cell cycle regulators in the progression of biliary atresia: an immunohistochemical study

“…As a result, biliary atresia is the most common indication for liver transplantation (LT) in children. A combination of viral, toxic, genetic and immunological aetiologies have been implied, but the pathophysiology of biliary atresia remains unknown …”