title: Biliary atresia: a new immunological insight into etiopathogenesis.
abstract: Biliary atresia is an idiopathic neonatal cholestatic disease characterized by the destruction of both the intra- and extra-hepatic biliary ducts. There are two clinical manifestations of the disease: an embryonal subtype, which often presents at birth and is associated with congenital malformations, and a 'perinatal' subtype, which is probably an acquired disease due to unknown etiology. Over the last two decades, researchers have focused on activation of the cell-mediated immunity as the mechanism for biliary epithelial cell destruction for the latter subtype. A proposed trigger of this immune response is an initial viral infection, inducing biliary epithelial cells to become antigen-presenting cells and thus instigating immune-mediated destruction of the biliary tract. However, putative viruses have never been confirmed. More recently, a novel hypothesis - that maternal microchimerism may initiate a host immunologic response towards the bile duct epithelia - has been proposed. This paper discusses the etiology of biliary atresia in the context of the current research.
authors.affiliation: Department of Pediatric Surgery, Ibaraki Children's Hospital, Mito, 311-4145, Japan. t-muraji@ibaraki-kodomo.com
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Muraji
authors.forename: David L
authors.initials: DL
authors.lastname: Suskind
authors.forename: Naoki
authors.initials: N
authors.lastname: Irie
mesh_terms: D000938:Antigen-Presenting Cells / Q000276:immunology / Q000473:pathology; D001656:Biliary Atresia / Q000208:ethnology / Q000209:etiology* / Q000276:immunology*; D046528:Chimerism; D004847:Epithelial Cells / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D007111:Immunity, Cellular / Q000276:immunology; D015810:Linkage Disequilibrium; D008431:Maternal-Fetal Exchange / Q000276:immunology; D011247:Pregnancy