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首页> 外文期刊>The Open Anesthesia Journal >Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report
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Clinical Pearls in Anesthesia for Xeroderma Pigmentosum: A Case Report

机译：干燥皮肤色素皮炎麻醉中的临床珍珠：一例报告

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摘要

Xeroderma Pigmentosum (XP) is a rare autosomal recessive (AR) disease characterized by hypersensitivity ofthe skin to ultra violet (UV) radiation, resulting in a high frequency of UV induced skin tumors and progressive neurologicalcomplications at an early age. Through the following case report we emphasize that perioperative management ofxeroderma patients entails meticulous evaluation for neurological abnormalities, shielding the skin from OT (operationtheatre) lights by using protective clothing, sunscreen and UV blocking film as well as avoidance of genotoxic drugs likevolatile anaesthetics and paracetamol. One must be prepared for the possibility of difficult mask ventilation (we used amask one size larger), difficult intubation and prolonged effect of muscle relaxants (as in our case) due to skin atrophy,neoplasia, joint contracture and neuronal dysfunction.
机译：Xerderma Pigmentosum（XP）是一种罕见的常染色体隐性遗传（AR）疾病，其特征在于皮肤对紫外线（UV）辐射过敏，导致高频率的紫外线诱发的皮肤肿瘤和小儿进行性神经系统并发症。通过以下病例报告，我们强调，对干性皮肤病患者的围手术期处理需要对神经系统异常情况进行仔细评估，通过使用防护服，防晒霜和紫外线阻挡膜来遮盖皮肤免受OT（手术室）光照射，并避免使用遗传毒性药物，例如挥发性麻醉剂和扑热息痛。必须准备好因皮肤萎缩，赘生物，关节挛缩和神经元功能障碍而导致面罩通气困难（我们使用的是大一号的面罩），插管困难和肌肉松弛剂作用延长（如本例）的可能性。

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