Propofol administration in patients with methylmalonic acidemia and intracellular cobalamin metabolism disorders: a review of theoretical concerns and clinical experiences in 28 patients

被引：10

作者：

Ktena, Yiouli P. ^{[1
]}

Ramstad, Trygg ^{[2
]}

Baker, Eva H. ^{[3
]}

Sloan, Jennifer L. ^{[1
]}

Mannes, Andrew J. ^{[2
]}

Manoli, Irini ^{[1
]}

Venditti, Charles P. ^{[1
]}

机构：

[1] NHGRI, Organ Acid Res Sect, Genet & Mol Biol Branch, NIH, Bethesda, MD 20892 USA

[2] NIH, Dept Perioperat Med, Ctr Clin, Bethesda, MD 20892 USA

[3] NIH, Dept Radiol & Imaging Sci, Bethesda, MD 20892 USA

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 2015年 / 38卷 / 05期

关键词：

D O I：

10.1007/s10545-015-9816-x

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Background 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. Methods Twenty eight patients (14 mut, seven cblC, three cblA, three cblB, one 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. Results 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 two cases; neither appeared to be related to propofol administration. Conclusions 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. Synopsis A review of the perianesthetic records of 28 patients with isolated MMA and intracellular cobalamin metabolism disorders suggests that propofol anesthesia can be administered safely to these patients, in the setting of metabolic stability.

引用

页码：847 / 853

页数：7

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