Dental treatment of a patient with GM1 gangliosidosis under general anesthesia: A case report
김인기, 송지수, 신터전, 현홍근, 김영재, 김정욱, 장기택,
소속 상세정보
김인기 ( Kim In-Ki ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
송지수 ( Song Ji-Soo ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
신터전 ( Shin Teo-Jeon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
현홍근 ( Hyun Hong-Keun ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김영재 ( Kim Young-Jae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김정욱 ( Kim Jung-Wook ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
장기택 ( Jang Ki-Taeg ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
Abstract
GM1 gangliosidosis is a lysosomal storage disease caused by various mutations in the GLB1 gene leading to deficiency in ßgalactosidase activity. Reported oral manifestations of GM1 gangliosidosis are gingival hyperplasia, enlarged tongue, disturbance in tooth eruption, dystonic movements of jaw and tongue, and mastication impairment. This report presents the dental treatment of a 4-year-old girl with GM1 gangliosidosis under general anesthesia. Caries lesions were detected on the primary second molars, and calculus was deposited around anterior teeth. Red granular spots around the marginal gingiva were observed, and gingival bleeding was easily triggered by simple stimulation. Such bleeding tendency might have been related to the use of valproic acid. The patient was treated by stainless steel crown restorations on the primary molars and scaling. Intensive oral hygiene care is a crucial duty of the oral health providers to reduce the risk of aspiration pneumonia in tube-feeding patients. To understand the medical status of patients and make appropriate modifications to the treatment plan, dental professionals should properly communicate with pediatricians.
키워드
GM1 gangliosidosis; Valproic acid; Aspiration pneumonia; Tube-feeding patients
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