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先天性无痛觉伴无汗症患者的脊髓麻醉。

Spinal anesthesia in a patient with congenital insensitivity to pain with anhidrosis.

作者信息

Oliveira Carlos R D, dos Santos Fúlvio A, Nogueira Celso S, Mainardes Emerson J

机构信息

Department of Anesthesiology, Irmandade da Santa Casa de Misericórdia de Santos, CET em Anestesiologia da Santa Casa de Santos, Santos, São Paulo, Brazil.

出版信息

Anesth Analg. 2007 Jun;104(6):1561-2, table of contents. doi: 10.1213/01.ane.0000261247.53075.dd.

Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, hereditary, autonomic recessive disorder. The inability to perceive pain results from loss of nociceptive afferents, while anhidrosis is caused by loss of innervation to the sweat glands. Insensitivity to pain and mental retardation lead to self-inflicted injuries, corneal lacerations, painless bony fractures, joint deformities with consequent chronic osteomyelitis, and septic arthritis. There are only a few reports on the anesthetic management for patients with CIPA. We describe the anesthetic management of a young woman with CIPA receiving bilateral arthrodesis of the ankle.

摘要

先天性无痛无汗症(CIPA)是一种罕见的遗传性自主神经隐性疾病。无法感知疼痛是由于伤害性传入神经丧失所致,而无汗则是由汗腺神经支配丧失引起的。对疼痛不敏感和智力迟钝会导致自我伤害、角膜撕裂、无痛性骨折、关节畸形并随之引发慢性骨髓炎和化脓性关节炎。关于CIPA患者麻醉管理的报道很少。我们描述了一名接受双侧踝关节融合术的CIPA年轻女性的麻醉管理情况。

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