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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
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- 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
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- 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
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- 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.
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- 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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