Biliary atresia: A comprehensive review

TY - JOUR

T1 - Biliary atresia: A comprehensive review

AU - Lakshminarayanan, Bhanumathi

AU - Davenport, Mark

PY - 2016/6/23

Y1 - 2016/6/23

N2 - 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.

AB - 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.

KW - Biliary atresia

KW - Inflammation

KW - Kasai portoenterostomy

KW - Immune response

KW - Microchimerism

U2 - 10.1016/j.jaut.2016.06.005

DO - 10.1016/j.jaut.2016.06.005

M3 - Article

SN - 0896-8411

JO - Journal of Autoimmunity

JF - Journal of Autoimmunity

ER -