relation: http://publicatio.bibl.u-szeged.hu/15721/
title: Medical and dental management of Alagille syndrome: A review.
creator:  Berniczei-Royko Adam
creator:  Chalas Renata
creator:  Mitura Iwona
creator:  Nagy Katalin
creator:  Prussak Elżbieta
description: Abstract Alagille syndrome is a rare, autosomal, complex, dominant disorder associated with dysfunction of the liver, heart, skeleton, and eyes, as well as characteristic facial appearance. It is associated with the defect in component of the Notch signalling pathway. Here, we review the main features of Alagille syndrome with special focus on oro-facial manifestations like prominent forehead, moderate hypertelorism with deep-set eyes, a saddle or straight nose with a flattened, bulbous tip, and large ears. The article is based on the most recent and significant literature available from the Medline database. Contrary to healthy children, patients with Alagille syndrome have many problems, depending on several factors like the severity of cholestasis and scarring in the liver, heart or lung problems, presence of infections, or other problems related to poor nutrition that can manifest in their oral cavity in the dental and periodontal tissues, as well as oral mucosa. From the dentist's view, the most important elements are careful observation, accurate diagnosis, and planned management of such patients, especially during the patient's formative years, to prevent complications. Aggressive preventive oral care and consultations with medical specialists before any invasive procedure are obligatory. All this can improve quality of life in patients with Alagille syndrome.
date: 2014
type: Folyóiratcikk
type: PeerReviewed
format: text
identifier: http://publicatio.bibl.u-szeged.hu/15721/1/2571553.pdf
identifier:     Berniczei-Royko Adam;  Chalas Renata;  Mitura Iwona;  Nagy Katalin;  Prussak Elżbieta: Medical and dental management of Alagille syndrome: A review.   MEDICAL SCIENCE MONITOR, 20.  pp. 476-480.  ISSN 1234-1010 (2014)     
identifier: doi:10.12659/MSM.890577
relation: 2571553
language: eng
relation: info:eu-repo/semantics/altIdentifier/doi/10.12659/MSM.890577
rights: info:eu-repo/semantics/openAccess