Isolated biliary atresia
ORPHA:30391· ICD-10 Q44.2
Definition
A rare, biliary tract disease characterized by progressive obliterative cholangiopathy of the intra- and extrahepatic bile ducts, occuring in the embryonic/ perinatal period, leading to severe and persistent neonatal jaundice and acholic stool.
- Prevalence
- 1-5 / 10 000
- Inheritance
- Multigenic/multifactorial
- Age of onset
- Antenatal, Infancy, Neonatal