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J Korean Acad Pediatr Dent. 2011;38(1): 95-100.
Young-Sun Kweon, Hong-Keun Hyun, Young-Jae Kim, Ki-Taeg Jang, Sang-Hoon Lee, Chong-Chul Kim,, Se-Hyun Hahn, Jung-Wook Kim
Department of Pediatric Dentistry, School of Dentistry, Seoul National University
Corresponding Author: Jung-Wook Kim ,Tel: 02-2072-2639, Email: pedoman@snu.ac.kr
Received: November 20, 2010;  Accepted: January 10, 2011.
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Fibrodysplasia ossificans progressiva(FOP) is characterized by episodes of permanent heterotopic ossifications of soft tissues throughout the body. FOP is inherited in an autosomal dominant pattern, but most cases result from new mutations in the ACVR1 gene. Even minimal trauma can cause permanent ossifications of soft tissues and give rise to complications following routine dental care. Dental block anesthesia, severe stretching of the jaw and biopsies are all contraindicated in children with FOP. There is no effective treatment. Since the prevalence of FOP is very low and most patients with FOP are misdiagnosed during childhood, they undergo dangerous and unnecessary treatment that can lead to permanent harm. For patients with FOP, early diagnosis and prevention of complications are most important. This study aims at contemplating the characteristic features and consideration factors for the dental treatment of FOP patients in relation to the case of an eight years-and-one month old boy who was referred to Pediatric Dentistry due to prolonged retention of mandibular right and left deciduous central incisors after being diagnosed with sporadic FOP at Pediatric Orthopaedics, Seoul National University Hospital, and received dental treatment without the exacerbation of the FOP symptoms.
Keywords: Fibrodysplasia ossificans progressiva | Heterotopic ossification | ACVR1 gene | Dental treatment consideration
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