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Outcome of patients with Alagille syndrome
There is no concrete number for the morbidity and mortality of people with Alagille syndrome. However, the main determinant of such numbers is the affection of the liver and the heart. Patients with heart conditions associated with the syndrome usually have a shorter lifespan compared to those without them. In addition, patients needing liver transplantation usually have a worse quality of life until they undergo the procedure, and they usually also need lifetime medications after performing it.
In raw numbers, up to 80% of Alagille syndrome patients without heart abnormalities survive past adulthood, compared to about 40% with significant heart problems.
