Open AccessAlagille Syndrome: Features and Outcome among Filipino Children
Author(s) -
Germana Emerita V. Gregorio,
Jossie M. Rogacion
Publication year - 2020
Publication title -
acta medica philippina/acta medica philippina
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.128
H-Index - 4
eISSN - 2094-9278
pISSN - 0001-6071
DOI - 10.47895/amp.v54i5.2272
Subject(s) - alagille syndrome , medicine , tetralogy of fallot , ductus arteriosus , cholestasis , jaundice , heart disease , pediatrics , chronic liver disease , gastroenterology , surgery , cirrhosis
We report 13 children fulfilling criteria of Alagille syndrome. All had chronic cholestasis secondary to paucity ofintrahepatic bile ducts and triangular facies. Eight children had associated congenital heart disease (six pulmonicstenosis, one each tetralogy of Fallot and patent ductus arteriosus), seven with butterfly vertebrae and onewith posterior embryotoxon. Seven of the 13 children are alive and jaundice-free but three with concomitanthypercholesterolemia; the six other children died of liver-related complications.