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[I型黏多糖贮积症(Hurler综合征)产妇剖宫产的有效硬膜外麻醉]

[Effective epidural anesthesia for cesarean section in parturient woman with type I mucopolysaccharidosis (Hurler's syndrome)].

出版信息

Anesteziol Reanimatol. 2011 Nov-Dec(6):29-31.

Abstract

Mucopolysaccharidosis is genetically determined disease caused by the deficiency of one of the lysosomal enzymes involved in the glycosaminoglycan (GAG) breakdown pathway. This metabolic block leads to the accumulation of GAG in various organs and tissues and affects nerve system, eyes, internal organs and musculoskeletal system. The reviewer of various articles on pubmed showed only one article about epidural anesthesia in patient with type I mucopolysaccharidosis. Our observation allows to consider that epidural anesthesia with ropivacaine is possible and safe in patients with type I mucopolysaccharidosis.

摘要

黏多糖贮积症是一种由参与糖胺聚糖(GAG)分解途径的溶酶体酶之一缺乏所导致的遗传性疾病。这种代谢障碍会导致GAG在各个器官和组织中蓄积,并影响神经系统、眼睛、内脏器官和肌肉骨骼系统。对PubMed上各类文章的检索显示,仅有一篇关于I型黏多糖贮积症患者硬膜外麻醉的文章。我们的观察结果表明,对于I型黏多糖贮积症患者,使用罗哌卡因进行硬膜外麻醉是可行且安全的。

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