biliary atresia
| Rare. | |
|---|---|
| Etiology | Variable genetic polymorphisms. Viral immune response?? Inflammatory fibrous obstruction of the extra-hepatic biliary ducts → bile goes to intestines. Onset: ~week 3. Associations: situs inversus. |
| Epidemiology | |
| Clinical presentation | prolonged jaundice after week 1 (week 3 if preterm), ↓ appetite, obstructive jaundice = dark urine, pale stools Ddx: |
| Pathogenesis | |
| Diagnostic investigations | Bloods: ↑ conjugated bilirubin (↑ unconjugated bili characteristic of breast milk jaundice), ↑ LFTs (GGT>>ALP, ALT), hepatomegaly, palpable spleen USS/liver biopsy: bile duct proliferation, bile plugs |
| Management | Surgical management - Kasai hepatoportoenterostomy, liver transplant |