Rare, but leading indication for liver transplantation in children
Exact etiology unclear, but inflammation leads to destruction of intra/extrahepatic bile ducts
Prognosis significantly improved by early diagnosis
Two forms:
Peri/postnatal (90% of cases), possibly related to infection
Fetal/embryonic (10%)
Often with other associated malformations (e.g congenital heart disease, polysplenia or asplenia, malrotation, situs inversus, portal vein or hepatic artery malformations)
Peri/postnatal (90% of cases), possibly related to infection
Fetal/embryonic (10%)
Often with other associated malformations (e.g congenital heart disease, polysplenia or asplenia, malrotation, situs inversus, portal vein or hepatic artery malformations)
Often with other associated malformations (e.g congenital heart disease, polysplenia or asplenia, malrotation, situs inversus, portal vein or hepatic artery malformations)
Biliary atresia
More common causes are listed first, followed by less common causes
Neonatal jaundice
Breast milk jaundice
Breast feeding jaundice
Due to substances in milk that inhibits glucuronyl transferase. It may start as early as 3rd day and reaches peak by 3rd week of life. It is unlikely to cause kernicterus
Duodenal atresia
Patient does not receive adequate oral intake which then causes reduced bowel movement/bilirubin excretion. Best diagnosed by looking for signs of dehydration and comparing weight to birth weight.