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先天性无痛觉伴无汗症患儿的麻醉管理

Anesthetic management of children with congenital insensitivity to pain with anhidrosis.

作者信息

Qiu Yongsheng, Zhao Liyuan, Yao Dongdong, Jia Yingping

机构信息

Department of Anesthesiology Children's Hospital Affiliated to Zhengzhou University Henan Children's Hospital Zhengzhou Children's Hospital Zhengzhou Henan China.

Department of Anesthesiology Brigham and Women's Hospital Harvard Medical School Boston USA.

出版信息

Pediatr Investig. 2019 Oct 28;4(4):296-298. doi: 10.1002/ped4.12152. eCollection 2020 Dec.

DOI:10.1002/ped4.12152
PMID:33376958
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7768302/
Abstract

INTRODUCTION

Congenital analgesia is a rare autosomal recessive hereditary disease. The primary damage of congenital analgesia is central structure damage of comprehensive pain perception.

CASE PRESENTATION

A 1-year-old Han Chinese boy was admitted to hospital because of a tongue bite. He had no response to noxious stimulation of the body surface and was diagnosed with congenital analgesia. A small dose of remifentanil was intravenously injected during anesthetic induction to reduce the stress response caused by endotracheal intubation. A certain depth of anesthesia should be guaranteed during anesthetic induction and surgery to alleviate the stress response induced by endotracheal intubation and the operation.

CONCLUSION

Opioid analgesics are not required for general anesthesia in patient with congenital insensitivity to pain. With a heat dissipation barrier in patients with congenital insensitivity to pain with anhidrosis, body temperature, end-tidal carbon dioxide and bispectral index should be monitored.

摘要

引言

先天性无痛觉是一种罕见的常染色体隐性遗传病。先天性无痛觉的主要损害是全面疼痛感知的中枢结构损伤。

病例介绍

一名1岁汉族男孩因咬伤舌头入院。他对体表的有害刺激无反应,被诊断为先天性无痛觉。麻醉诱导期间静脉注射小剂量瑞芬太尼以减轻气管插管引起的应激反应。麻醉诱导和手术期间应保证一定的麻醉深度,以减轻气管插管和手术引起的应激反应。

结论

对疼痛不敏感的患者全身麻醉不需要使用阿片类镇痛药。对于伴有无汗的疼痛不敏感患者,应监测体温、呼气末二氧化碳和脑电双频指数。

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本文引用的文献

1
Congenital Insensitivity to Pain and Anhydrosis: Diagnostic and Therapeutic Dilemmas revisited.先天性无痛觉与无汗症:再探诊断与治疗难题
Int J Clin Pediatr Dent. 2015 Jan-Apr;8(1):75-81. doi: 10.5005/jp-journals-10005-1288. Epub 2015 Apr 28.
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Norepinephrine deficiency with normal blood pressure control in congenital insensitivity to pain with anhidrosis.先天性无痛觉伴无汗症患者中去甲肾上腺素缺乏但血压控制正常。
Ann Neurol. 2015 May;77(5):743-52. doi: 10.1002/ana.24377. Epub 2015 Mar 13.
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Congenital Insensitivity to Pain with Anhidrosis (HSAN Type IV), Extremely Rare Syndrome that Can Be Easily Missed by Bone and Joint Surgeons: A Case Report.先天性无痛觉伴无汗症(IV型遗传性感觉神经病),一种极易被骨外科医生漏诊的极其罕见的综合征:一例报告
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[Anesthesia in a patient with congenital insensitivity to pain and anhidrosis].[先天性无痛觉和无汗症患者的麻醉]
Rev Bras Anestesiol. 2009 Sep-Oct;59(5):602-9. doi: 10.1016/s0034-7094(09)70085-6.
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Anaesthetic management of children with congenital insensitivity to pain with anhidrosis.先天性无痛觉伴无汗症患儿的麻醉管理
Paediatr Anaesth. 2000;10(5):545-8. doi: 10.1046/j.1460-9592.2000.00542.x.
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Not 'indifference to pain' but varieties of hereditary sensory and autonomic neuropathy.并非“对疼痛无动于衷”,而是多种遗传性感觉和自主神经病变。
Brain. 1983 Jun;106 (Pt 2):373-90. doi: 10.1093/brain/106.2.373.