Use of propofol for short duration procedures in children with long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiencies.
The medication propofol, commonly used for anesthesia, has been avoided in patients with mitochondrial fatty acid oxidation disorders (FAODs) due to concerns that it contains long-chain fatty acids (LCFAs), and because of reports of severe side effects in some critically ill patients receiving high-dose propofol infusions that mimic some of the symptoms regularly found in FAOD patients. In this secondary analysis, we examined the outcomes of 8 children with long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency or trifunctional protein (TFP) deficiency who were repeatedly sedated for an electroretinogram (ERG) as part of a longitudinal study of the progression of chorioretinopathy commonly found in this population. A total of 39 sedated ERG procedures were completed using propofol for sedation. The propofol dosing, estimated total energy needs of the subject, and inpatient dietary intake recording were completed in 32 of these procedures. The LCFAs in the propofol provided approximately 1.0% of the average total daily energy needs. The sedation with propofol resulted in no adverse side effects and was safely used in this short duration procedure.
Department of Molecular & Medical Genetics, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA.
Julie M
JM
Martin
Department of Molecular & Medical Genetics, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA; Graduate Programs in Human Nutrition, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA.
Melanie B
MB
Gillingham
Department of Molecular & Medical Genetics, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA. Electronic address: hardingc@ohsu.edu.
Cary O
CO
Harding
D002648:Child; D004334:Drug Administration Schedule; D004596:Electroretinography; D004734:Energy Metabolism / Q000187:drug effects; D006801:Humans; D006993:Hypnotics and Sedatives / Q000008:administration & dosage* / Q000009:adverse effects; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase / Q000172:deficiency* / Q000378:metabolism; D008137:Longitudinal Studies; D063991:Mitochondrial Trifunctional Protein / Q000172:deficiency* / Q000378:metabolism; D015742:Propofol / Q000008:administration & dosage* / Q000009:adverse effects; D012164:Retinal Diseases / Q000178:diet therapy / Q000503:physiopathology*; D016896:Treatment Outcome
D006993:Hypnotics and Sedatives; D063989:Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase; D063991:Mitochondrial Trifunctional Protein; D015742:Propofol
Anesthesia; Fatty acid oxidation disorders; Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency; Propofol; Trifunctional protein deficiency