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Biliary atresia

Atresia biliaris · Atrézie žlučových cest

What it is

The bile ducts scar and close in the first weeks of life; about 1 in 15,000 births in Europe, more in East Asia, and the commonest reason for liver transplant in children.

Symptoms & signs

  • Jaundice beyond two weeks of age
  • pale or white stools
  • dark urine
  • enlarging liver and spleen
  • bleeding from lack of vitamin K
  • poor growth

🚩 Get help if…

  • call 112 / 911 now Bleeding, bruising or a swollen belly with jaundice
  • see a doctor soon Pale, white or clay-coloured stools in a baby
  • see a doctor soon Yellow skin or eyes after 2 weeks of age
  • see a doctor soon Dark urine in a baby

Where it strikes

Medical treatment

  • Split bilirubin in every baby still jaundiced at two weeks
  • Kasai portoenterostomy, best before about 60 days — clears jaundice in about half
  • fat-soluble vitamins and MCT milk
  • antibiotics for cholangitis
  • liver transplant — most eventually need one

Natural & lifestyle

  • Stool colour cards for parents — Taiwan's national programme brought surgery earlier and raised survival trials support
  • breast milk with MCT supplements

Emerging & experimental

  • Steroids after Kasai — START trial: no better bile drainage not confirmed in trials
  • Bile-acid transport inhibitors (odevixibat) — in trials

Alternative, off-label & anecdotal

Approaches used outside the guidelines, with what their supporters report and what testing has found so far.

  • Herbal liver tonics (yinchen, milk thistle) — untested; delay can lose the window for surgery anecdotal / traditional
  • Sunlight for jaundice — irrelevant to blocked ducts; delays diagnosis anecdotal / traditional

ICD codes