title: Alagille syndrome.
abstract: Syndromic paucity of bile ducts or "Alagille syndrome" is characterized by peculiar facies, chronic cholestasis, posterior embryotoxon, butterfly-like vertebral arch defects and peripheral pulmonary artery hypoplasia or stenosis. We present a two-year-old female child with the 'partial' or 'incomplete' Alagille syndrome. The child had three of the five major features of the syndrome. A brief review of literature of the syndrome is presented.
authors.affiliation: Department of Pediatrics, Seth G. S. Medical College and KEM Hospital, Mumbai, Maharashtra, India.
authors.forename: Harshalee
authors.initials: H
authors.lastname: Shendge
authors.forename: Milind S
authors.initials: MS
authors.lastname: Tullu
authors.forename: Asha
authors.initials: A
authors.lastname: Shenoy
authors.forename: Rachana
authors.initials: R
authors.lastname: Chaturvedi
authors.forename: Jaishree R
authors.initials: JR
authors.lastname: Kamat
authors.forename: Manisha
authors.initials: M
authors.lastname: Khare
authors.forename: Amita
authors.initials: A
authors.lastname: Joshi
mesh_terms: D016738:Alagille Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D002675:Child, Preschool; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007194:India; D008111:Liver Function Tests; D018570:Risk Assessment; D012720:Severity of Illness Index