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KMID : 1144020210170010021
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Journal of Korean Association for Disability and Oral Health
2021 Volume.17 No. 1 p.21 ~ p.26
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Orthodontic treatment with myofunctional therapy of a patient with Pompe disease : A case report
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Park Sang-Hyun
Kim Hyun-Tae
Song Ji-Soo
Hyun Hong-Keun
Kim Young-Jae
Kim Jung-Wook
Jang Ki-Taeg
Shin Teo-Jeon
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Abstract
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Pompe disease is a rare glycogen storage disease with a lysosomal metabolism defect and autosomal recessive trait. Patient with Pompe diseases shows typical craniofacial characteristics such as facial muscle weakness and altered orofacial growth. This report presents the case of a child with the infantile-onset type of Pompe disease who receive successful orthodontic treatment with myofunctional therapy to treat malocclusion. A 10-year-old boy with Pompe disease was diagnosed as anterior crossbite with hypotonic tongue and orofacial muscle in the Department of Pediatric Dentistry, Seoul National University Dental Hospital. Orthodontic treatment using 2x4 fixed appliance followed by Frankel III appliance was done. Also, myofunctional therapy was conducted to improve the position of tongue and orofacial muscle strength. The patient was well adapted to the appliance, and the malocclusion was successfully treated without relapse. Both patient and his caregivers were very satisfied with the results.
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KEYWORD
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Pompe disease, Malocclusion, Myofunctional therapy
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