biliary atresia

Question 1

bile ducts of an infant are progressively […] and destroyed, leading to […] […]-bilirubinaemia, liver failure

Answer 1

fibrosed, conjugated, hyper

Question 2

Features
- Prolonged jaundice (i.e., jaundice that persists beyond […] days of life)
- Signs of […] obstruction - […] urine and chalky white stool)

Answer 2

14
biliary, dark

Question 3

Invx
- Bloods: high […] bilirubin, deranged […]
- Imaging: hepatic […] (technetium-99) highlights liver (takes up the isotope) but poor excretion into the bowel (bile ducts fibrosed and bile not flowing)
- Abdo […] - fibrosis
- Definitive diagnosis –> […]

Answer 3

conjugated, LFTs
scintigraphy
USS
cholangiography

Question 4

Mx
hepatoportoenterostomy ( […]procedure) - new pathway from the liver to the gut to bypass fibrosed ducts

Answer 4

Kasai