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KMID : 0358920010280010166
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Journal of the Korean Academy of Pedodontics
2001 Volume.28 No. 1 p.166 ~ p.170
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CASE REPORT OF RUBINSTEIN-TABYI SYNDROME
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Jung Sung-Ho
Yang Kyu-Ho
Lee Tae-Hee
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Abstract
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Rubinstein-Tabyi syndrome(RTS) is a congenital multisystem dysmorphic syndrome with many non-specific features, making diagnosis occasionally difficult. The major features of this syndrome include mental retardation, characteristic facial appearance, short stature, microcephaly, and broad thumbs and halluces. This syndrome was first described by Rubinstein and Tabyi in 1963, and many studies have been continued about this syndrome, but specific pathogenesis of the Rubinstein-Tabyi syndrome phenotype is still not clear. High arched palate, micrognathia and multiple caries etc have been reported in Rubinstein-Tabyi syndrome. In this report, a 6-year and 5-month-old boy visited at our department due to multiple dental caries, who showed broad thumbs, mental and physical development retardation, and characteristic facial appearance including both ptosis and ear deformity. This patient was diagnosed as a Rubinstein-Tabyi syndrome, and treated the multiple dental caries under general anesthesia. This study was aimed to observe the relationship between medical and dental characteristics.
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KEYWORD
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Rubinstein-Tabyi syndrome, Congenital multisystem dysmorphic syndrome, Multiple dental caries, General anesthesia
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