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・ Congenital insensitivity to pain
Congenital insensitivity to pain with anhidrosis
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Congenital insensitivity to pain with anhidrosis : ウィキペディア英語版
Congenital insensitivity to pain with anhidrosis

Congenital insensitivity to pain with anhidrosis (CIPA), also called hereditary sensory and autonomic neuropathy type IV—is an extremely rare inherited disorder of the nervous system which prevents the sensation of pain, heat, cold, or ''any'' real nerve-related sensations (including feeling the need to urinate); however, patients can still feel pressure. CIPA is the fourth type of hereditary sensory and autonomic neuropathy (HSAN), known as HSAN IV. (It is also referred to as HSAN Type IV). A person with CIPA cannot feel pain or differentiate even extreme temperatures. "Anhidrosis" means the body does not sweat, and "congenital" indicates that the condition is present from birth.
==Clinical description==

People with this disorder are very likely to injure themselves in ways that would normally be prevented by feeling pain. For example, a patient could burn themselves severely and not even notice. The main features of the disorder are lack of pain sensation, painless injuries of the arms, legs and oral structures, hyperthermia during hot weather because of inability to sweat, syndromic intellectual disability as a result of hyperthermia, 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. Other common problems are eye related, such as infection due to the sufferers rubbing them too hard, too frequently or scratching them during sleep. In addition, patients typically lack unmyelinated and small myelinated nerve fibers in the dorsal root ganglion. Both are responsible for transmitting pain signals. In addition, patients' sweat glands are normal in both structure and function, though they lack innervations by small diameter neurons.

抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)
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