Journal of the korean academy of Pediatric Dentistry (대한소아치과학회지)

Korean Academy of Pediatric Dentistry (대한소아치과학회)
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Dental Management in a Patient with Congenital Insensitivity to Pain with Anhidrosis : A Case Report

선천성 무통 무한증 환자의 치과적 관리 증례보고

  • Bae, Cheolhyeon (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital) ;
  • Lee, Daewoo (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital) ;
  • Kim, Jaegon (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital) ;
  • Yang, Yeonmi (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital)
  • 배철현 (전북대학교 치과병원 소아치과) ;
  • 이대우 (전북대학교 치과병원 소아치과) ;
  • 김재곤 (전북대학교 치과병원 소아치과) ;
  • 양연미 (전북대학교 치과병원 소아치과)
  • Received : 2019.06.20
  • Accepted : 2019.08.05
  • Published : 2019.11.30
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Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal recessive disorder; affected patients are characterized by inability to feel pain and to sweat over the entire body, as well as by mental retardation. Because, in the oral examination, no specific findings on soft or hard tissue may be found except possible lesions due to self-mutilation, early recognition and diagnosis are essential for these patients. Pediatric dentists must be aware of the clinical manifestations and treatment considerations related to uncontrolled body temperature, tactile hyperesthesia and lack of pain reflex. In this case report, dental management of CIPA was suggested by presenting a 6-year follow-up of young patient.

선천성 무통무한증(Congenital insensitivity to pain with anhidrosis, CIPA)은 NTRK1 유전자의 돌연변이에 의해 발생하는 드문 유전질환으로 통증에 대한 무감각 및 땀을 흘리지 못하는 것이 특징이다. 지적장애를 동반하는 경우가 많으며, 구강내 임상검사에서는 자해로 인한 병소를 제외하고 특징적인 병소가 발견되지 않으므로 초기 감별진단이 중요하다. 소아치과의사는 이 질환과 동반되는 조절되지 않는 체온, 지각 과민, 통증 반사의 부재와 같은 다양한 임상증상 등을 인지하고 있어야 하며 이를 치과 치료에서 고려해야만 한다. 이 연구는 본원에 첫 내원 이후 6년여간 경과 관찰 중인 선천성 무통무한증 환아의 치험례를 통하여 이 질환의 감별진단 및 치과치료에서의 주의사항 등을 알아보고자 하였다.

Keywords

References

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