甲基丙二酸血症患者的麻醉管理：一例报告
Anesthetic management of a patient with methylmalonic acidemia: a case report.
作者信息
Uemura Yuta, Kakuta Nami, Tanaka Katsuya, Tsutsumi Yasuo M
机构信息
Department of Anesthesiology, Tokushima University, 3-18-15, Kuramoto, Tokushima, 770-8503, Japan.
出版信息
JA Clin Rep. 2018 Oct 1;4(1):71. doi: 10.1186/s40981-018-0209-7.
BACKGROUND
Methylmalonic acidemia (MMA) is a metabolic disorder of organic acids and is characterized by the accumulation of methylmalonic acids.
CASE PRESENTATION
The patient was a 19-year-old female diagnosed with severe MMA at 3 days of age, who was scheduled for renal replacement therapy. Preoperatively, there was no evidence of metabolic acidosis or electrolyte abnormalities. Glucose was administered preoperatively following a 6-h fast. Anesthesia was administered using thiamylal, remifentanil, rocuronium, and sevoflurane. After tracheal intubation, the patient underwent an ultrasound-guided bilateral rectus sheath block with ropivacaine. A drop in blood sugar level was treated with 5% glucose. Extubation was performed after intravenous administration of sugammadex.
CONCLUSIONS
We report the anesthetic management of a patient with MMA using a combination of general anesthesia and rectus sheath block.
背景
甲基丙二酸血症（MMA）是一种有机酸代谢紊乱疾病，其特征是甲基丙二酸积累。
病例介绍
该患者为19岁女性，3日龄时被诊断为重度MMA，计划接受肾脏替代治疗。术前，无代谢性酸中毒或电解质异常证据。术前禁食6小时后给予葡萄糖。使用硫喷妥钠、瑞芬太尼、罗库溴铵和七氟醚进行麻醉。气管插管后，患者接受了超声引导下的双侧腹直肌鞘阻滞，使用罗哌卡因。血糖水平下降用5%葡萄糖治疗。静脉注射舒更葡糖后进行拔管。
结论
我们报告了一名MMA患者采用全身麻醉和腹直肌鞘阻滞联合的麻醉管理情况。
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