Alagille syndrome

This code identifies Alagille syndrome, a rare genetic disorder characterized by abnormalities in the liver, heart, eyes, face, and skeleton. It primarily involves a paucity of intrahepatic bile ducts, leading to cholestasis and liver disease.

Assign this code for patients diagnosed with Alagille syndrome based on clinical findings and/or genetic testing. Documentation should clearly indicate the presence of the multi-systemic features characteristic of this inherited condition.

• Ensure documentation specifies "Alagille syndrome" rather than just general cholestasis or liver disease.
• Code any associated congenital anomalies (e.g., congenital heart defects, butterfly vertebrae) separately.
• Do not confuse with other forms of familial intrahepatic cholestasis.