先天性无痛无汗症患儿两次手术围术期管理

  • 陆 化梅 河南省洛阳正骨医院(河南省骨科医院)麻醉科,中国·河南 洛阳 471000
  • 于 国军 河南省洛阳正骨医院(河南省骨科医院)麻醉科,中国·河南 洛阳 471000
  • 郭 永娟 河南省洛阳正骨医院(河南省骨科医院)麻醉科,中国·河南 洛阳 471000
  • 康 定坤 河南省洛阳正骨医院(河南省骨科医院)麻醉科,中国·河南 洛阳 471000
关键词: 先天性无痛无汗症, 手术麻醉, 围术期管理

摘要

先天性无痛无汗症是以痛温觉丧失,全身无汗,皮肤干燥,反复发热为临床表现的罕见遗传性疾病,又称为遗传性感觉和自主神经障碍Ⅳ型.本文通过一例先天性无痛无汗症合并股骨骨髓炎患者两次手术麻醉过程介绍,针对此病的主要症状及临床表现,讨论围术期血流动力变化,体温管理,有效避免返流、误吸方法以及镇痛药物及抗胆碱药物的合理使用.

参考

Dyck PJ, Mellinger JF, Reagan CJ, et al. Not indifference to pain but

varieties of hereditary sensory and autonomic neuropathy. Brain, 1983,106: 373.

Kilic SS, Ozturk R, Sarisonzen B, et al. Humoral immunodeficiency in

congenital insensitivity to pain with anhidrosis. Neurogenetics,2009,10:161.

Machtei A, Levy J, Friger M, et al. Osteomyelitis of the mandible in a

group of 33 pediatric patients with congenital insensitivity to pain with anhidrosis.

Int J Pediatr Otorhinolaryngol, 2011,75:523

Yoko Takeuchi, Yoshihito Fujita, Takeshi Shimomura, et al. Anesthetic

management of a patient with congenital insensitivity to pain with anhidrosis by

coadministration of remifentanil. JA Clinical reports(2018)4:72

窦银聪,葛英辉,李国艳等.11例先天性无痛无汗症的骨关节病影像学分析.临床放射学杂志,2013,32(2):246-248。

Alexander Z, Dmitry N, Ruslan K, et al. Anesthetic Management of Pa-

tients with Congenital Insensitivity to Pain with Anhidrosis: A Retrospective Analysis

of 358 Procedures Perform Under General Anesthesia. Anesth Analg. 2015.11;121(5):1316-1320

Yuki Sugiyama,Sayako Gotoh,Masatoshi Urasawa,et al. Hemodynamic

response to massive bleeding in a patient with congenital insensitivity to pain with

anhidrosis. Anesthesiology.2018,(12)95:1-4.

已出版
2020-08-05
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