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

Korean Association for Disability and Oral Health (대한장애인치과학회)
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X-LINKED DOMINANT HYPOPHOSPHATAEMIC RICKETS: 13 YEARS FOLLOW-UP STUDY

X 염색체 우성 저인산혈증성 구루병: 13년간의 치험례

  • Ha, Na (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Kim, Youngjin (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Kim, Hyunjung (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University) ;
  • Nam, Soonhyeun (Department of Pediatric Dentistry, School of Dentistry, Kyungpook National University)
  • 하나 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 김영진 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 김현정 (경북대학교 치의학전문대학원 소아치과학교실) ;
  • 남순현 (경북대학교 치의학전문대학원 소아치과학교실)
  • Received : 2016.11.30
  • Accepted : 2016.12.21
  • Published : 2017.06.30
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Abstract

X-linked dominant hypophosphataemic rickets(XLHR) is a hereditary disease generally transmitted as an X-linked dominant trait. Oral manifestations include poorly mineralized dentin, enlarged pulp chambers and root canals, early tooth loss. Also, spontaneous dental abscesses could occur in the absence of a history of trauma or caries. This report describes the clinical finding and dental treatment of a child, who came to the hospital for treatment caries but was refered to pediatrics because the child showed clinically short of status, bow-leg and radiographically enlarged pulp chamber and canal, there as diagnosed as XLHR. After the diagnosis, 13 years follow-up and treatment were performed.

본 증례에서는 본과에서 발견한 임상적인 특징을 통해 X 염색체 우성 저인산혈증성 구루병으로 진단받아 13년간 의과적 및 치과적인 처치를 받고 있는 환아에 대해 보고하였다. 의과적으로 환아는 저신장 및 휜 다리 등의 전형적인 구루병 증상을 나타내었고, 치과적으로도 넓은 치수강, 상아법랑경계까지 연장된 치수각 및 자발적 치아농양 등의 전형적인 증상을 나타내었다. 본 환아는 정기적인 검진을 통해 유구치와 영구 소구치 및 대구치 모두 치면열구전색 및 수복치료 등을 적극적으로 시행해왔다. 그러나 예방적인 처치에도 불구하고 유치열에서는 총 8개의 치아에서 치아 우식에 이환 없이 자발적 치아농양이 발생하여 치수절제술을 시행하였으며, 조기 상실 등의 합병증 없이 영구치교환 시점까지 유지할 수 있었다. 영구치열에서는 그 빈도가 낮아졌으나 하악 우측 제1, 2대구치에서 발생한 자발적 치아농양으로 치료를 완료하였다. 앞으로 치아교모 등의 원인으로 영구치에서 발생할 수 있는 치아농양을 예방하기 위해 예방적인 전장관수복치료가 필요하리라 생각된다.

Keywords

References

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