[Congenital insensitivity to pain: difficulty of management]. / L'insensibilité congénitale à la douleur : difficultés de prise en charge.
Rev Stomatol Chir Maxillofac
; 113(1): 46-9, 2012 Feb.
Article
en Fr
| MEDLINE
| ID: mdl-21924450
INTRODUCTION: Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare disorder, most often of genetic origin. CASE REPORT: The authors present the case of two siblings, 10 and 13 years old, both followed-up since the age of 2 for CIPA diagnosed after discovering insensitivity to pain during iterative falls, burns, and of severe oro-digital self-mutilating behavior. Sural nerve biopsy and an electromyogram confirmed the diagnosis. DISCUSSION: CIPA with anhidrosis is a very rare disease. It is characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self-mutilating behavior, and sometimes mental retardation. Complications of this insensitivity (non-treated fractures, burns, and oro-digital mutilation) may be lethal. Treatment remains preventive. The patient must observe a very strict hygiene. Prevention for maxillofacial involvement consists in breaking the cycle of oral self-mutilation.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Insensibilidad Congénita al Dolor
Tipo de estudio:
Diagnostic_studies
Límite:
Adolescent
/
Child
/
Female
/
Humans
/
Male
Idioma:
Fr
Revista:
Rev Stomatol Chir Maxillofac
Año:
2012
Tipo del documento:
Article
País de afiliación:
Marruecos
Pais de publicación:
Francia