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KMID : 1144020210170010010
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Journal of Korean Association for Disability and Oral Health
2021 Volume.17 No. 1 p.10 ~ p.14
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Dental considerations for patients with Hunter syndrome
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Cho Da-Young
Cho Kyung-Hyun
Song Je-Seon
Kim Seong-Oh
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Abstract
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Hunter syndrome, or mucopolysaccharidosis type II, is a rare X-linked disorder caused by a deficiency in the lysosomal enzyme iduronate-2-sulfatase. This results in accumulation of glycosaminoglycans (GAGs) in the lysosomes of various tissues. It causes physical and mental degeneration, in severe cases, death during the teenage years. Dental complications include enamel hypoplasia, microdontia, dentigerous cyst-like follicles, unerupted dentition, malocclusions, gingival hyperplasia, macroglossia, and condylar defects. This is a case report of a 9-year-old boy who visited the Dentistry Department of Pediatric Dentistry, Yonsei University Dental Hospital with a chief complaint of over-all dental treatment and bruxism. This report discusses oral manifestations of Hunter syndrome and its dental management methods
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KEYWORD
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Hunter syndrome, Mucopolysaccharidosis type II, Iduronate-2-sulfatase
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