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KMID : 1144020220180010025
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Journal of Korean Association for Disability and Oral Health
2022 Volume.18 No. 1 p.25 ~ p.31
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Dental Management of a Patient with Congenital heart defects, dysmorphic facial features and intellectual developmental disorders (CHDFIDD) under General Anesthesia: A Case Report
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Chun Se-Jin
Kim Hyun-Tae
Song Ji-Soo
Shin Teo-Jeon
Hyun Hong-Keun
Kim Jung-Wook
Jang Ki-Taeg
Kim Young-Jae
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Abstract
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Congenital heart defects, dysmorphic facial features and intellectual developmental disorders (CHDFIDD) is an autosomal dominant hereditary disease caused by mutation in cyclin-dependent kinase 13 (CDK13) gene. It was first discovered in 2016 by exome sequencing of patients with congenital heart defects and now, more than 50 patients have been diagnosed for CHDFIDD since the initial discovery of the disease and the number is increasing every year. Dental manifestation of wide-spaced or peg-shaped teeth are reported in patients with CHDFIDD. This case report describes the dental treatment of a 5-year-girl with CHDFIDD under general anesthesia. Through clinical and radiographic examination, interproximal caries lesion on primary molars and severe attrition on primary incisors with deep bite were detected. Fibrous gingival hyperplasia on buccal interproximal area between lower right lateral incisor and canine was also observed. Considering the extensive treatment need and patient’s uncooperative behavior, we planned to do dental treatment under general anesthesia. Conservative resin restoration, stainless steel crown restoration and excisional biopsy were done. Dental treatment of patients with CHDFIDD is challenging due to poor cooperation. Preventive dental care including oral hygiene instruction, plaque control, dietary consultation and fluoride application are necessary for maintaining oral health and preventing the development and progression of dental caries.
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KEYWORD
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Congenital heart defects, dysmorphic facial features and intellectual developmental disorders, Dental caries, Attrition, Dental treatment, General anesthesia
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