Congenital insensitivity to pain with anhidrosis
From Wikipedia, the free encyclopedia
Congenital Insensitivity to Pain with Anhidrosis (CIPA) is a rare inherited disorder of the nervous system which prevents the sensation of pain, heat, and cold. A person with CIPA cannot feel pain or differentiate extreme temperatures. 'Anhidrosis' means the body does not sweat.
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[edit] Clinical description
Patients with this disorder are very likely to injure themselves in ways that would normally be prevented by feeling pain. The main features of the disorder are: lack of pain sensation, painless injuries of the arms, legs and oral structures, fever during hot weather because of inability to sweat (anhidrosis), mental retardation, infection and scarring of the tongue, lips and gums, chronic infections of bones and joints, bone fractures, multiple scars, osteomyelitis and joint deformities, which may lead to amputation.
[edit] Cause
CIPA is caused by a genetic mutation which prevents the formation of nerve cells which are responsible for transmitting signals of pain, heat, and cold to the brain. CIPA is the most severe and fatal type of the seven types of hereditary sensory and autonomic neuropathy, or HSAN. Overheating kills more than half of all children with CIPA before age 3.
[edit] Incidence
CIPA is extremely rare. There are 35 reported cases in the U.S. and more than 300 in Japan because it occurs more often in homogeneous societies. It is also found in the Swedish city of Gällivare, where nearly 40 cases have been reported.
[edit] See also
[edit] External links
- McMaster Meducator on CIPA April 2005, 6: 20-21 by Abdullah Alabousi.
- CIPA by Courtney Wood
- Life Can be Painful for Child Who Can't Feel Pain: Purple Medical Blog
- A Life Without Pain The website of a documentary about 3 children who have CIPA or similar diseases
- Doctoral thesis at Umeå University
- Mendelian Inheritance in Man (OMIM) 256800
