General and stomatologic aspects of bardet-biedl syndrome.

Autor: Ferreira do Amaral CO; From the Departments of *Special Care Dentistry, †Pediatric Dentistry, and ‡Prosthodontics and Geriatic Dentistry, Dental School, University of Western Sao Paulo, Presidente Prudente, Sao Paulo, Brazil., Logar Gde A, Parisi AG, Takahashi K, Straioto FG
Jazyk: angličtina
Zdroj: The Journal of craniofacial surgery [J Craniofac Surg] 2014 Nov; Vol. 25 (6), pp. e575-8.
DOI: 10.1097/SCS.0000000000001169
Abstrakt: Objective: The purpose of this study is to present a clinical report of a patient with Bardet-Biedl syndrome, aiming to help the dentist to identify the general aspects, systemic changes, alterations.
Clinical Report: Bardet-Biedl syndrome is defined as a genetic disorder of autosomal recessive condition; this case is a male patient, 20 years old. The general features presented by the patient were strabismus, polydactyly, hypogonadism, obesity, cognitive impairment, and autistic behavior. Dentally, this patient presents with hypotonic face, high-arched palate, dental crowding, unilateral crossbite, and difficulty in cleaning but with good-quality oral health.
Conclusions: Many patients with this syndrome have compromised oral hygiene, difficulty in addressing dental cases, developmental delay, and autistic behavior. Thus, it is important to pay attention to these early children from an early age and encourage preventive approach.
Databáze: MEDLINE