TY - JOUR
T1 - Use of propofol for short duration procedures in children with long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiencies
AU - Martin, Julie M.
AU - Gillingham, Melanie B.
AU - Harding, Cary O.
N1 - Funding Information:
This publication was supported by a grant from the Doernbecher Children's Hospital Foundation (Eye Disease In Inborn Errors of Metabolism) , F32DK065400 (MBG) from the National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK) and the Oregon Clinical and Translational Research Institute (OCTRI), and grant number ( UL1TR000128 ) from the National Center for Advancing Translational Sciences (NCATS). The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.
PY - 2014/6
Y1 - 2014/6
N2 - 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.
AB - 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.
KW - Anesthesia
KW - Fatty acid oxidation disorders
KW - Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency
KW - Propofol
KW - Trifunctional protein deficiency
UR - http://www.scopus.com/inward/record.url?scp=84901589096&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84901589096&partnerID=8YFLogxK
U2 - 10.1016/j.ymgme.2014.03.012
DO - 10.1016/j.ymgme.2014.03.012
M3 - Article
C2 - 24780638
AN - SCOPUS:84901589096
SN - 1096-7192
VL - 112
SP - 139
EP - 142
JO - Molecular Genetics and Metabolism
JF - Molecular Genetics and Metabolism
IS - 2
ER -