Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients.
Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients.
复制标题
DOI：
10.1007/s10545-015-9816-x
复制
发表时间：
2015-09
影响因子：
4.2
通讯作者：
Venditti CP
中科院分区：
文献类型：
--
作者：
Ktena YP;Ramstad T;Baker EH;Sloan JL;Mannes AJ;Manoli I;Venditti CP
甲基丙二酸血症和细胞内钴胺素代谢紊乱代表一组异质性先天性代谢缺陷。由于神经和神经认知障碍，大多数患者将需要经常需要镇静或麻醉管理的诊断和/或治疗程序。据称，该人群禁用异丙酚。我们报告了在大量患者中使用异丙酚的经验。 28 名年龄为 2-35.6 岁的患者（14 mut、7 cblC、3 cblA、3 cblB、1 cblG）参加了自然史研究（ClinicalTrials.gov 标识符：NCT00078078），并需要进行 39 项诊断或治疗程序的麻醉。收集麻醉技术、围麻醉过程以及与异丙酚相关的不良事件的数据。在大多数情况下（36/39），异丙酚被用作唯一的诱导剂，并且在所有情况下被用作主要的维持剂。输注速率为 100–400 mcg.kg−1.min−1（平均值=214）。输注持续时间为 60–325 分钟（平均值 = 158），总剂量范围为 270–3610 mg（平均值 = 1217）。记录不良事件2例；两者似乎都与异丙酚给药无关。异丙酚是一种有效、安全的诱导和维持剂，适用于患有 MMA 和钴胺素代谢紊乱的患者需要麻醉的选择性短期手术。尽管存在多种合并症和不稳定倾向，但如果在事件发生前代谢和血流动力学稳定，受影响的患者仍可以接受安全性可接受的麻醉。
Methylmalonic acidemia and intracellular cobalamin metabolism disorders represent a heterogeneous group of inborn errors of metabolism. Most patients will require diagnostic and/or therapeutic procedures frequently requiring sedation or anesthetic management due to neurological and neurocognitive impairments. It has been stated that propofol is contraindicated in this population. We report our experience with propofol administration in a large series of patients. 28 patients (14 mut, 7 cblC, 3 cblA, 3 cblB, 1 cblG) aged 2–35.6 years enrolled in a natural history study (ClinicalTrials.gov identifier: NCT00078078) and required anesthetics for 39 diagnostic or therapeutic procedures. Data were collected on the anesthetic technique, perianesthetic course and adverse events related to propofol. Propofol was used as the sole induction agent in most cases (36/39) and as the primary maintenance agent in all cases. Infusion rates were 100–400 mcg.kg−1.min−1 (mean=214). Infusion duration was 60–325 min (mean=158) and total doses ranged between 270–3610 mg (mean=1217). Adverse events were recorded in 2 cases; neither appeared to be related to propofol administration. Propofol is an effective, safe induction and maintenance agent for elective short procedures requiring anesthesia in patients with MMA and cobalamin metabolism disorders. Despite multiple comorbidities and propensity toward instability, those affected can receive anesthesia with an acceptable safety profile, if metabolically and hemodynamically stabilized prior to the event.