What Is Alagille Syndrome
Alagille syndrome, also known as congenital intrahepatic bile duct dysplasia syndrome, arterial-hepatic dysplasia syndrome or Watson-Alagille syndrome.
What Is The Cause Of 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.
What Are The Symptoms Of Alagille Syndrome
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.
How To Prevent Alagille Syndrome
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.
How To Treat Alagille Syndrome
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.
How To Differentiate Alagille Syndrome
Congenital intrahepatic bile duct dysplasia syndrome should be distinguished from other cholestatic diseases in infants and young children.