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.

journal_name

Med Sci Monit

authors

Berniczei-Royko A,Chałas R,Mitura I,Nagy K,Prussak E

doi

10.12659/MSM.890577

subject

Has Abstract

pub_date

2014-03-24 00:00:00

pages

476-80

eissn

1234-1010

issn

1643-3750

pii

890577

journal_volume

20

pub_type

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