Alagille Syndrome: Features and Outcome among Filipino Children

Abstract

We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity of
intrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonic
stenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and one
with posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitant
hypercholesterolemia; the six other children died of liver-related complications.