Alagille Syndrome: Features and Outcome among Filipino Children
- Author:
Germana Emerita V. Gregorio
1
;
Jossie M. Rogacion
1
Author Information
- Publication Type:Journal Article
- Keywords: Butterfly vertebrae; infantile jaundice; facial malformation; paucity of intrahepatic bile ducts
- MeSH: Butterflies; Bile Ducts, Intrahepatic; Jaundice; Musculoskeletal Abnormalities; Spine
- From: Acta Medica Philippina 2020;54(5):632-637
- CountryPhilippines
- Language:English
- 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.
- Full text:2272-Article Text-11746-1-10-20201028.pdf
