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Pyknodysostosis 환아의 구강증상 : 증례보고

Other Titles
 김남혁  ;  이제호  ;  김성오  ;  최형준  ;  송제선 
 Journal of the Korean Academy of Pediatric Dentistry (대한소아치과학회지), Vol.36(4) : 619-624, 2009 
Journal Title
Journal of the Korean Academy of Pediatric Dentistry(대한소아치과학회지)
Issue Date
Pyknodysostosis ; 파골세포 ; Cathepsin K
Pyknodysostosis(PKND) is a rare sclerosing bone disorder that has an autosomal recessive trait, also known as Toulouse-Lautrec syndrome. Deficiency of the cathepsin enzyme K in the osteoclasts of PKND patients results in continuous endosteal bone deposits without osteoclastic resorption or remodeling. This causes a generalized increase in sclerosis and fragility of bones. Osteomyelitis in the mandible and recurrent fracture of the long bones are characteristic complicatons of PKND. The patients present typical features of PKND, such as short stature under 150 cm, open cranial suture and fontanelle, club-shaped phalanges, and underdevelopment of midface. This is a case of a 7-year-old girl with PKND, who visited our clinic with the chief complaint of anterior Open-bite and generalized crowding. The patient had been diagnosed as PKND by an orthopedist and manifested characteristic clinical and radiographic features, such as open cranial suture and fontanelle, obtuse madibular gonial angle, frontal and occipital bossing, grooved palate, club-shaped phalanges, and short stature. Orthodontic treatment was not considered because patients with PKND show abnormal bone resorption and remodeling. Instead, removal of deciduous teeth near exfoliation and TFA were performed, and periodic check-up is planned to maintain good oral hygiene
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Appears in Collections:
2. College of Dentistry (치과대학) > Dept. of Pediatric Dentistry (소아치과학교실) > 1. Journal Papers
Yonsei Authors
Kim, Seong Oh(김성오) ORCID logo https://orcid.org/0000-0002-8620-1377
Song, Je Seon(송제선) ORCID logo https://orcid.org/0000-0001-8620-5629
Lee, Jae Ho(이제호) ORCID logo https://orcid.org/0000-0002-1556-3485
Choi, Hyung Jun(최형준) ORCID logo https://orcid.org/0000-0002-3315-6912
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