Perioperative measures in very long chain acyl-CoA dehydrogenase deficiency

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• 作者：P. Vellekoop^a ; E.F. Diekman^a ; I. van Tuijl^b ; M.M.C. de Vries^c ; P.M. van Hasselt^a ; G. Visser^a ; ^{gvisser4@umcutrecht.nl"" rel=""nofollow}
• 关键词：VLCADD ; Anesthetic measures ; Surgical Procedures
• 刊名：Molecular Genetics and Metabolism
• 出版年：2011
• 出版时间：May 2011
• 年：2011
• 卷：103
• 期：1
• 页码：96-97
• 全文大小：121 K

文摘

Surgical procedures in patients with metabolic disorders require specific anesthetic measures based on the nature of the involved metabolic disorder. Illustrated by the history of two patients, the need for a specific perioperative regimen in patients with very long chain acyl-CoA dehydrogenase deficiency (VLCADD) is discussed. One patient deteriorated, the other patient did well without any specific measurements. Although perioperative metabolic decompensation can currently not be predicted, it is a severe complication which should be avoided. We therefore advise to consider certain perioperative precautions in all VLCADD patients: 1) age and weight adapted glucose infusion, 2) stress avoiding premedication, 3) avoidance of volatile anesthetics, 4) avoidance of long chain fatty acid containing anesthetics and 5) perioperative glucose and CK monitoring.