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.
How common is this condition?
- The incidence rate is approximately 1 case in every 100,000 live births.
What is the cause and risk of developing syndrome?