What's the diagnosis?

Split (conjugated) bilirubin. Worry: biliary atresia

What's going on

The bile ducts outside the liver become inflamed and blocked, so bile can't drain. Without treatment this leads to scarring of the liver (cirrhosis).

Spot it

Jaundice beyond 14 days (21 days if preterm), pale stools and dark urine. The baby often looks well and feeds normally.

Test

Split bilirubin. A conjugated bilirubin over 25 micromol/L may mean serious liver disease, so get expert advice from a liver team.

Treatment

Kasai portoenterostomy: the scarred ducts are removed and a loop of small bowel is joined to the underside of the liver so bile can drain. It works best early, ideally before about 60 days.

Why it matters

It is the most common reason for a liver transplant in children. In England and Wales, every baby is treated in one of just 3 centres.

Go further

Tap a question to check your answer.

1. When is jaundice “prolonged”?

   Beyond 14 days in a term baby, or 21 days if preterm.

2. What do pale stools tell you?

   Bile isn't reaching the gut.

3. Which bilirubin do you measure?

   Split (conjugated). Over 25 micromol/L may mean serious liver disease, so get expert advice.

4. Why does timing matter?

   The Kasai operation works best early, ideally before about 60 days.

5. Harder: what if the Kasai doesn't work?

   A liver transplant. Biliary atresia is the most common reason children need one.

Source: NICE CG98 · Patient.info Professional