title: [Syndromal paucity of the interlobular bile ducts (Alagille's syndrome). A report of 2 Sicilian siblings].
abstract: The authors describe a sister and brother [correction of two brothers] affected by syndromic paucity of interlobular bile ducts in which a marked heterogeneity in the beginning of symptoms puts serious difficulties in differential diagnosis. The evident similarity to the mother and the presence of hypertelorism support the hypothesis of a genetic inherited autosomal dominant disorder at variable penetration and expressivity.
authors.affiliation: Cattedra di Pediatria Preventiva e Sociale-Università di Catania, Italia.
authors.forename: S
authors.initials: S
authors.lastname: Leonardi
authors.forename: G
authors.initials: G
authors.lastname: Arcidiacono
authors.forename: G
authors.initials: G
authors.lastname: Ferlito
authors.forename: S
authors.initials: S
authors.lastname: Musumeci
mesh_terms: D016738:Alagille Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology; D002648:Child; D005260:Female; D006801:Humans; D007223:Infant; D008099:Liver / Q000473:pathology; D008297:Male; D012802:Sicily
vernacular_title: Paucità sindromica delle vie biliari interlobulari (sindrome di Alagille). Descrizione di due fratelli siciliani.