KMID : 0191119990140040460
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Journal of Korean Medical Science 1999 Volume.14 No. 4 p.460 ~ p.464
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Congenital insensitivity to pain with anhidrosis: a case report
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Kim JS
Woo YJ/Kim GM/Kim CJ/Ma JS/Hwang TJ/Lee MC
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Abstract
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Congenital insensitivity to pain with anhidrosis (CIPA) is a very rare genetic disorder of the peripheral nervous system characterized by recurrent episodes of unexplained fever, generalized anhidrosis, insensitivity to pain and temperature, and accompanied by self-mutilating behavior and mental retardation. We report on a 16 month-old boy with CIPA who exhibited these characteristic clinical features. A sural nerve biopsy revealed markedly reduced numbers of unmyelinated and small myelinated fibers, consistent with the characteristic features of CIPA.
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KEYWORD
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