CONGENITAL INSENSITIVITY TO PAIN WITH ANHIDROSIS : CASE REPORT

Congenital Insensitivity to Pain with Anhidrosis 환아의 치험례

Congenital Insensivity to Pain with Anhidrosis(CIPA) is rare autosomal recessive disorder which is known to be hereditary sensory and autonomic neuropathies(HSAN) type IV. It is predilection for male and observed in early infant. Its clinical features include congenital analgesia, which leads to self-mutilation; inability to sweat, which leads to defective thermoregulation; and mild to moderate mental retardation. Its dental features include self-mutilation by nail peeling, which leads to gingival ulcer, tongue ulcer due to tongue biting, and enamel hypoplasia. Partial anodontia is often observed as well. Seventeen-months-old boy with CIPA was reported. This is the case in which the dental characteristics are described and the dental treatment of patient is discussed.

본 병원에 동통을 느끼지 못하는 것으로 인한 혀나 손가락 깨물기 등의 자가 손상, 무한증, 반복적 발열등의 임상증상을 보이며 유전성이 없이 CIPA로 내원한 환아에서 보호자에게 세심한 보호와 우발사고 방지에 대한 교육이 무엇보다도 중요하며, 치면세마, 불소도포, 소와열구전색등의 예방적인 처치와 주기적으로 내원시켜 치과적인 문제를 조기에 해결하는 것이 필요하다고 사료된다.