Alagille syndrome
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What is Alagille syndrome?
- It is a Genetic disorder(JAG 1 gene) also known as arteriohepatic dysplasia.Children usually have a liver disease characterized by a progressive loss of the bile ducts within the liver over the first year of life and narrowing of the bile ducts outside the liver. This leads to a buildup of bile in the liver, causing damage to liver cells. Scarring may occur and lead to cirrhosis in about 30 to 50 percent of affected children.
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How common is this condition?
- The incidence rate is approximately 1 case in every 100,000 live births.
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What is the cause and risk of developing syndrome?