Biliary Atresia

Progressive shrinking of the tubes (ducts) which drain bile from the liver to the small intestine  No known causes, though there is a link to some blood markers Occurs 1 per 8,000 to 15,000 births Usually seen within the first 6 weeks of life

Yellow-colored skin, whites of eyes and inside of mouth  Pale-colored stools Enlarged liver, felt as a mass on the right side of the abdomen Itching  Decreased alertness, if prolonged

Many blood tests are necessary to eliminate other liver disorders. Bacterial and viral cultures should be done. Eye examination for congenital infections of some viruses Ultrasound of the liver and pancreas HIV test Liver sampling (biopsy) for microscopic evaluation X-ray studies for evaluation of congenital problems

Surgery is helpful but most patients will require a liver transplant, if available. Nutritional support is necessary, as food is poorly absorbed. Enzymes can be taken orally to help digestion. Best results occur when diagnosis is made within the first 2 months of life.

Poor growth Fluid retention with swelling of the legs and abdomen Fever and infection of the liver Ultimately, liver failure requiring a transplant

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