在急性間歇型紫質症病人使用Sevoflurane和Rocuronium之病例報告

The Use of Rocuronium and Sevoflurane in Acute Intermittent Porphyria-- A Case Report

急性間歇型紫質症是一種遺傳性的疾病，其形成的原因跟體內的亞鐵蛋白原合成的路徑缺陷有關。許多手術及麻醉中的過程都有可能引發急性間歇型紫質症的迅速惡化，例如生理性賀爾蒙的變化、手術前禁食、脫水、術中的壓力、感染、以及所使用的藥物等等。而急性間歇型紫質症的急性發作是有可能會致命的。所以有關急性間歇型紫質症的麻醉安全用藥也一直 在不斷更新中，而這些更新大多是根據臨床實際經驗所做的建議。我們報告一位罹患此病的女性，術中使用了sevoflurane以及rocuronium，而這些藥物並沒有引發這個病人的症狀惡化。

Acute intermittent porphyria (AIP) is an inherited metabolic disorder caused by deficiency of porphobilinogen deaminase, an enzyme found in the synthetic pathway of heme. Acute attack of AIP may be precipitated by many factors during operation and anesthesia, including fasting, dehydration, stress, infection, and drugs. Acute attack of AIP is likely fatal. Therefore, the drugs recommended as being safe in anesthesia for porphyria patients are up-to-dately refreshed and renovated and the identification of whether a drug is safe or not is based on cumulative anecdotal experiences. Here, we report the safe use of rocuronium and sevoflurane for long exposure in a patient affected with acute intermittent porphyria.