Alagille Syndrome (Watson-Alagille Syndrome,Congenital Dysplasia Of Intrahepatic Bile Duct,Arterial-Hepatic Dysplasia Syndrome,)

Alagille syndrome, also known as congenital intrahepatic bile duct dysplasia syndrome, arterial-hepatic dysplasia syndrome or Watson-Alagille syndrome.

Congenital dysplasia of intrahepatic bile duct, characterized by obvious enlargement of the liver, no bile duct in most portal areas under microscope, sometimes dysplasia of bile duct, no obvious lumen, obvious cholestasis, mild fibrosis in portal areas and interstitial fibrosis in testicles.

The diagnosis can be made according to typical symptoms and liver biopsy. Patients with the following three or more items can be diagnosed as congenital intrahepatic bile duct dysplasia:

1. Intrahepatic bile duct hypoplasia.

2. Stenosis of peripheral pulmonary artery.

3. Typical facial features.

4. The anterior arch of spine is split.

5. More than one of the immediate family members suffers from Alagille syndrome.

It can be judged by the results of liver biopsy.

Congenital intrahepatic bile duct dysplasia syndrome is a genetic disease. If there is a clear family history, it is best to go to a regular hospital or medical institution for genetic consultation before giving birth, and decide whether to give birth according to the doctor's advice, and choose the appropriate birth mode. Prenatal diagnosis can be made by genetic testing during pregnancy.

There is no special treatment for congenital intrahepatic bile duct dysplasia syndrome. Cholecysteamine and other drugs can be given to treat cholestasis and supplement fat-soluble vitamins.

Congenital intrahepatic bile duct dysplasia syndrome should be distinguished from other cholestatic diseases in infants and young children.

AGS children with neonatal cholestatic jaundice have poor prognosis of liver diseases. However, serious liver complications may not appear until the late stage of liver disease, which requires lifelong follow-up.