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Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management

Identifieur interne : 005A74 ( Main/Merge ); précédent : 005A73; suivant : 005A75

Traumatic lesions from congenital insensitivity to pain with anhidrosis in a pediatric patient: dental management

Auteurs : Beatriz Gonçalves Neves [Brésil] ; Rosemere Teixeira Roza [Brésil] ; Gloria Fernanda Castro [Brésil]

Source :

RBID : ISTEX:EE65610212437D41A4CFB59BBE2CED4596E6005D

English descriptors

Abstract

Abstract –  Congenital insensitivity to pain with anhidrosis is a rare autosomal‐recessive disorder characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self‐mutilating behavior, and mental retardation. The lack of sensitivity to pain results in traumatic lesions, such as ulcers, fractures, burns, bites, scars, and digital amputations. Several methods have been suggested to treat these patients; however, appropriate management is difficult, especially when the mutilation is particularly severe. This report describes the case of a 2‐year‐old female patient who had severe self‐mutilating injuries to her tongue, hands, lips, and oral mucosa caused by biting. The patient presented digital amputation and also a premature loss of a permanent tooth germ during the treatment. The dental management is described and discussed. It is important to include the dentist on the multidisciplinary team to reduce the frequency and severity of the self‐inflicted lesions in these patients, also to prevent complications.

Url:
DOI: 10.1111/j.1600-9657.2009.00810.x

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ISTEX:EE65610212437D41A4CFB59BBE2CED4596E6005D

Le document en format XML

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<term>Anhidrosis</term>
<term>Appropriate management</term>
<term>Autonomic neuropathy</term>
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<term>Case report</term>
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<div type="abstract">Abstract –  Congenital insensitivity to pain with anhidrosis is a rare autosomal‐recessive disorder characterized by unexplained fever episodes, anhidrosis, pain insensitivity, self‐mutilating behavior, and mental retardation. The lack of sensitivity to pain results in traumatic lesions, such as ulcers, fractures, burns, bites, scars, and digital amputations. Several methods have been suggested to treat these patients; however, appropriate management is difficult, especially when the mutilation is particularly severe. This report describes the case of a 2‐year‐old female patient who had severe self‐mutilating injuries to her tongue, hands, lips, and oral mucosa caused by biting. The patient presented digital amputation and also a premature loss of a permanent tooth germ during the treatment. The dental management is described and discussed. It is important to include the dentist on the multidisciplinary team to reduce the frequency and severity of the self‐inflicted lesions in these patients, also to prevent complications.</div>
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