How Does CIPA Work?

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CIPA is 'Congenital Insensitivity to Pain with Anhidrosis.' This is an extremely 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 even extreme temperatures. 'Anhidrosis' means the body doesn't sweat. This condition is present from birth. Patients with CIPA are very likely to injure themselves in ways that would normally be prevented by feeling pain. These people suffer fever during hot weather, due to not sweating; painless injuries of the arms, legs, and oral structures, sometimes mental retardation, infection, and scarring of the tongue, lips, and gums, chronic infections of bones & joints, bone fractures, multiple scars, osteomyelitis and joint deformities, which may lead to amputation.

CIPA is caused by a genetic mutation which prevents the formation of nerve cells responsible for transmitting signals of pain, heat, and cold to the brain. Overheating kills more than half of all children with CIPA before age 3.

Source: {http://www.medical-library.net/content/view/1691/41/}

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