Alagille Syndrome

Definition

Alagille syndrome is an inherited disorder characterized by bile duct paucity, congenital heart disease, and characteristic facial appearance.

Symptoms

  • Bile duct deficiency
  • Peripheral pulmonary stenosis
  • Butterfly vertebra
  • Characteristic face

Reasons

JAG1 or NOTCH2 gene mutations.

Diagnosis

Clinical criteria, genetic testing.

Treatment

Symptomatic treatment, liver transplantation.

Which Polyclinic Should You Apply To?

Gastroenterology Polyclinic

What to Do in an Emergency?

In case of symptoms of liver failure, emergency services should be consulted.

This page is for general information purposes only.