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KMID : 1144020160120010011
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Journal of Korean Association for Disability and Oral Health
2016 Volume.12 No. 1 p.11 ~ p.15
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DENTAL TREATMENT IN A PATIENT WITH ANGELMAN SYNDROME DUE TO UNIPARENTAL DISOMY
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Park Sung-Hee
Shin Teo-Jeon
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Lee Sang-Hoon
Kim Chong-Chul
Jang Ki-Taeg
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Abstract
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Angelman syndrome is characterized by a partial deficit of paired autosomal chromosome 15, which contains a subunit of the GABA (Gamma-Amino Butyric Acid) receptor. The main clinical characteristics of this syndrome are delayed neuropsychological development, intellectual disability, and speech impairment. The syndrome has oral manifestations such as diastemas, tongue thrusting, sucking swallowing disorder, and excessive chewing behavior. A 3-year-6-month old girl with Angelman syndrome was scheduled for dental treatment. She had multiple caries, but she was poorly cooperative for treatment due to developmental delay and movement disorder, so general anesthesia was considered.
The patient with Angelman syndrome was successfully treated under general anesthesia. There were no postoperative complications related to anesthesia and dental treatment. When treating children with this syndrome, the dentist needs to manage their uncooperative behavior and medical problem.
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KEYWORD
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Angelman syndrome, Uniparental disomy, Dental treatment, General anesthesia
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