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What is Alagille Syndrome With Cholestatic Pruritus?

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Alagille syndrome (AGLS) with cholestatic pruritus is a rare genetic disorder caused by abnormalities in the liver’s bile ducts.

It is a rare disease, with only between 3,000 and 30,000 cases in the United States, according to the National Institutes of Health.

Structural abnormalities in the liver cause reduced flow of the digestive enzyme bile out of the liver (cholestasis). Bile then builds up in the liver, leading to liver damage and uncontrolled itching (pruritus).

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