Medical and dental management of Alagille syndrome: A review

Adam Berniczei-Royko, Renata Chałas, Iwona Mitura, Katalin Nagy, Elzbieta Prussak

Research output: Contribution to journalReview article

5 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)476-480
Number of pages5
JournalMedical Science Monitor
Volume20
DOIs
Publication statusPublished - Mar 24 2014

Keywords

  • Alagille Syndrome
  • Dental Care
  • Oral Manifestations

ASJC Scopus subject areas

  • Medicine(all)

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