Oral aspects of Rubinstein‐Taybi syndrome
作者:
Raoul C. M. Hennekam,
Jaap M. Van Doorne,
期刊:
American Journal of Medical Genetics
(WILEY Available online 1990)
卷期:
Volume 37,
issue S6
页码: 42-47
ISSN:0148-7299
年代: 1990
DOI:10.1002/ajmg.1320370607
出版商: Wiley Subscription Services, Inc., A Wiley Company
关键词: oral anomalies;palato‐schisis;talon cusp
数据来源: WILEY
摘要:
AbstractOral findings in 45 patients with Rubinstein‐Taybi syndrome living in The Netherlands are compared with those from the literature. The main non‐dental findings were thin upper lip, small oral opening, pouting lower lip, retro/micrognathia, and apparently highly arched, narrow palate. Cleft uvula, cleft palate, or, rarely, cleft upper lip can be part of the syndrome. Timing of the eruption of deciduous and permanent dentition is normal. Sixty‐two percent of patients have malpositioned, crowded teeth. Marked caries was found in 36% and was possibly caused by problems in dental care due to the small opening of the mouth, malposition and malformation of the teeth, and non‐cooperation of the patients. Hypodontia, hyperdontia, and natal teeth can be manifestations of the syndrome. In 73% of all patients and in 92% of all permanent dentitions, talon cusps were found. Two or more talon cusps are rarely found in the normal population or other syndromes. Therefore, this finding strongly supports the diagnosis of Rubinstein‐Taybi syndrome in patients in whom this diagnosis is
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