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There is invariably a conjugated hyperbilirubinaemia with elevated transaminases.
First line investigation is liver ultrasound, during which it is important to visualise the gallbladder; if it can be seen as normal then biliary atresia is unlikely.
Radioisotope scanning may fail to visualise the biliary tree and a failure of the isotope to reach the intestine.
Liver biopsy shows bile ductular proliferation, bile plugs, and portal or perilobular oedema and fibrosis, with the basic hepatic lobular architecture intact. This should only be undertaken in a centre with facilities to cope with the potential complications of liver biopsy.
Biliary atresia can be excluded if bile-stained fluid is found on duodenal intubation.
Laparotomy may be needed to confirm the diagnosis.