Perioperative measures in very long chain acyl-CoA dehydrogenase deficiency
| Autor: | M.C. de Vries, I. van Tuijl, Gepke Visser, P.M. van Hasselt, P. Vellekoop, Eugene F. Diekman |
|---|---|
| Rok vydání: | 2011 |
| Předmět: |
Male
Mitochondrial Diseases Endocrinology Diabetes and Metabolism Biochemistry Lipid Metabolism Inborn Errors Very Long-Chain Acyl-CoA Dehydrogenase Deficiency Endocrinology Muscular Diseases Genetics medicine Congenital Bone Marrow Failure Syndromes Humans Lymphocytes Perioperative Period Molecular Biology biology business.industry Acyl-CoA Dehydrogenase Long-Chain Homozygote Metabolic disorder Infant Newborn Infant Acyl CoA dehydrogenase Perioperative Fibroblasts medicine.disease Regimen Treatment Outcome Anesthesia Mutation Anesthetic biology.protein Female Premedication Long chain fatty acid business Metabolism Inborn Errors medicine.drug |
| Zdroj: | Molecular Genetics and Metabolism. 103:96-97 |
| ISSN: | 1096-7192 |
| DOI: | 10.1016/j.ymgme.2011.01.010 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|
Full Text from ScienceDirect