The Journal of Korea Assosiation for Disability and Oral Health (대한장애인치과학회지)

Korean Association for Disability and Oral Health (대한장애인치과학회)
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DENTAL MANAGEMENT OF A PATIENT WITH PRADER-WILLI SYNDROME : A CASE REPORT

Prader-Willi syndrome 환자의 치과 치료 : 증례보고

  • Lee, Myeong-Yeon (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Jung, Younwook (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Kim, Seong-Oh (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Choi, Hyung-Jun (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Son, Heung-Kyu (Department of Pediatric Dentistry, College of Dentistry, Yonsei University) ;
  • Lee, Hyo-Seol (Department of Pediatric Dentistry, College of Dentistry, Yonsei University)
  • 이명연 (연세대학교 치과대학 소아치과학교실) ;
  • 정연욱 (연세대학교 치과대학 소아치과학교실) ;
  • 김성오 (연세대학교 치과대학 소아치과학교실) ;
  • 최형준 (연세대학교 치과대학 소아치과학교실) ;
  • 손흥규 (연세대학교 치과대학 소아치과학교실) ;
  • 이효설 (연세대학교 치과대학 소아치과학교실)
  • Received : 2014.06.14
  • Accepted : 2014.06.25
  • Published : 2014.06.30
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Abstract

Prader-Willi syndrome (PWS) is a rare neurodevelopmental disease caused by abnormality of chromosome 15q11-13. The estimated prevalence of PWS is 1/10,000-30,000. Most common features of this disease are feeding problems characterized by poor sucking habit related with neonatal or infantile hypotonia and obesity due to early childhood hyperphagia involved with lack of satiety. In the orodental findings, enamel hypoplasia, rampant caries, delayed eruption, poor oral hygiene, hypodontia, supernumerary teeth, increased tooth wear, decreased salivary flow and change in saliva composition were reported. This case report describes the dental treatment of 3-year-9-months-old male patient with PWS. Periodic check-ups and conservative treatments were followed, however, rapid dental caries progression caused by estimating hyposalivation was observed. Because of lack of patient cooperation, dental procedures were performed under general anesthesia.

프라더 윌리 증후군과 원인을 알 수 없는 심낭 삼출을 앓고 있는 3세 남환이 치아우식을 주소로 내원하여 임상 구강 검사 결과 다발성 우식증과 구강 전반에 걸친 치아 마모증이 관찰되었다. 약 10개월간 보존적인 수복치료와 구강 위생관리를 시도하였으나, 치아 우식의 빠른 진행과 환아의 협조도 부족으로 전신마취 하 우식 치료를 시행하였다.

Keywords

References

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