Congenital insensitivity to pain with anhidrosis: a case report
Journal of Korean Medical Science
;
: 460-464, 1999.
Article
in English
| WPRIM
| ID: wpr-221953
ABSTRACT
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.
Full text:
Available
Index:
WPRIM (Western Pacific)
Main subject:
Atrophy
/
Self Mutilation
/
Sural Nerve
/
Tongue
/
Microscopy, Electron
/
Pain Insensitivity, Congenital
/
Fingers
/
Hypohidrosis
/
Korea
/
Intellectual Disability
Limits:
Humans
/
Infant
/
Male
Country/Region as subject:
Asia
Language:
English
Journal:
Journal of Korean Medical Science
Year:
1999
Type:
Article
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