Congenital indifference to pain
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Very rare disorder which resembles congenital insensitivity to pain (or HSAN) but is distinguished by the fact that only the perception of nociception is affected. Other sensory functions (except, in some cases, the presence of anosmia or hyposmia) and autonomic functions are intact. There is therefore a loss of nociception and of thermal sensitivity; ulcers, painless trauma (burns, fracture). It is due to a mutation of the SCN9A gene on chromosome 2q24-q31. This gene codes for Nav1.7 protein of the sodium channel.
Anesthetic implications:
no published case
Updated: September 2019