title: Medical and dental management of Alagille syndrome: a review.
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.
authors.affiliation: Department of Orthodontics, University of Szeged, Szeged, Hungary.
authors.forename: Adam
authors.initials: A
authors.lastname: Berniczei-Royko
authors.affiliation: Department of Conservative Dentistry and Endodontics, Medical University of Lublin, Lublin, Poland.
authors.forename: Renata
authors.initials: R
authors.lastname: Chałas
authors.affiliation: Orthodontic Clinic, Dental Clinical Center, Medical University of Lublin, Lublin, Poland.
authors.forename: Iwona
authors.initials: I
authors.lastname: Mitura
authors.affiliation: Department of Oral Surgery, University of Szeged, Szeged, Hungary.
authors.forename: Katalin
authors.initials: K
authors.lastname: Nagy
authors.affiliation: Department of Management in Health Care, University of Medical Sciences, Poznań, Poland.
authors.forename: Elżbieta
authors.initials: E
authors.lastname: Prussak
mesh_terms: D016738:Alagille Syndrome / Q000628:therapy*; D003817:Dentition; D006801:Humans; D009909:Oral Health; D009928:Organ Specificity