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Xanthomas in the Watson-Alagille syndrome
Journal of the American Academy of Dermatology
|May 1, 1987
Summary
Watson-Alagille syndrome, a congenital cholestasis, presents with liver, eye, bone, and heart issues. This case shows a good prognosis, with symptoms resolving in adulthood.
Area of Science:
- Hepatology
- Pediatric Gastroenterology
- Medical Genetics
Background:
- Watson-Alagille syndrome is a rare genetic disorder characterized by congenital cholestasis.
- It involves hepatic ductular hypoplasia, ocular, bony, and cardiac anomalies, and a distinctive facial appearance.
- The long-term prognosis and symptom progression are not always well-documented.