title: Biliary atresia: A comprehensive review.
abstract: Biliary atresia presents as an obliterative cholangiopathy with neonatal jaundice and pale stools. The disease exhibits aetiological heterogeneity with a multiplicity of potential causative factors, both developmental and environmental. A number of clinical variants making up a minority of all cases can be defined relatively precisely which match suggested aetiology better although in most it still remains speculative. These include the syndromic form (BASM), the cystic form and those associated with CMV IgM antibodies. We review not only the clinical evidence for a developmental or an immune-mediated aetiology perhaps triggered by perinatal viral exposure but also several other recently suggested concepts such as microchimerism, gene susceptibility and environmental toxins.
authors.affiliation: Department of Paediatric Surgery, King's College Hospital, London, UK.
authors.forename: Bhanumathi
authors.initials: B
authors.lastname: Lakshminarayanan
authors.affiliation: Department of Paediatric Surgery, King's College Hospital, London, UK. Electronic address: Markdav2@ntlworld.com.
authors.forename: Mark
authors.initials: M
authors.lastname: Davenport
mesh_terms: D001652:Bile Ducts / Q000254:growth & development*; D001656:Biliary Atresia / Q000175:diagnosis / Q000453:epidemiology* / Q000209:etiology* / Q000601:surgery; D001706:Biopsy; D046528:Chimerism; D003587:Cytomegalovirus / Q000276:immunology / Q000302:isolation & purification; D004781:Environmental Exposure / Q000009:adverse effects*; D020022:Genetic Predisposition to Disease; D006801:Humans; D056724:Immunity, Humoral; D007075:Immunoglobulin M / Q000276:immunology; D015994:Incidence; D007223:Infant; D008099:Liver / Q000473:pathology; D008111:Liver Function Tests; D013601:T-Lymphocytes / Q000276:immunology; D014463:Ultrasonography
chemical_list: D007075:Immunoglobulin M
keywords: Biliary atresia; Immune response; Inflammation; Kasai portoenterostomy; Microchimerism