dc.contributor.author | Dercksen, M. | |
dc.contributor.author | Mienie, L.J. | |
dc.contributor.author | Reinecke, C.J. | |
dc.contributor.author | Kulik, W. | |
dc.contributor.author | Wanders, R.J.A. | |
dc.date.accessioned | 2016-09-27T07:51:35Z | |
dc.date.available | 2016-09-27T07:51:35Z | |
dc.date.issued | 2016 | |
dc.identifier.citation | Dercksen, M. et al. 2016. Polyunsaturated fatty acid status in treated isovaleric acidemia patients. European journal of clinical nutrition, 70:1123-1126. [https://doi.org/10.1038/ejcn.2016.100] | en_US |
dc.identifier.issn | 0954-3007 | |
dc.identifier.issn | 1476-5640 (Online) | |
dc.identifier.uri | http://hdl.handle.net/10394/18884 | |
dc.identifier.uri | https://doi.org/10.1038/ejcn.2016.100 | |
dc.identifier.uri | https://www.nature.com/ejcn/journal/v70/n10/full/ejcn2016100a.html | |
dc.description.abstract | BACKGROUND/OBJECTIVES: Nutritional deficiencies are frequently observed when treating patients with inborn errors of
metabolism due to an unbalanced diet. Thus far, patients with isovaleric acidemia (IVA) who adhere to a restricted protein diet have
not been investigated in this respect. We hypothesize that these patients may have a polyunsaturated fatty acid (PUFA) deficiency,
leading to potential clinical complications.
SUBJECTS/METHODS: We examined the nutritional status by reporting on potential deficiencies in PUFAs in treated IVA patients.
A general clinical chemistry work-up as well as gas chromatography flame ionization detector analysis was performed to determine
PUFAs in the plasma of 10 IVA patients.
RESULTS: The general clinical chemistry tests did not indicate severe hematological abnormalities or nutritional insufficiencies.
We identified a significant reduction in plasma PUFA levels, especially in omega-3 (all acids, Po0.001) and omega-6 (in particular
20:3n-6 Po0.0001 and 20:4n-6 P = 0.0005) fatty acids. In addition, an elevation in omega-9 fatty acids, with the exception of 20:3n-9
and C22:1n-9, was not suggestive of complete essential fatty acid deficiency but rather indicative of isolated and/or combined
omega-3 and omega-6 fatty acid depletion.
CONCLUSIONS: This study emphasizes the potential nutritional insufficiencies that may occur because of therapeutic intervention
in IVA | en_US |
dc.language.iso | en | en_US |
dc.publisher | Springer Nature | en_US |
dc.subject | Biomarkers | |
dc.subject | Metabolomics | |
dc.subject | Risk factors | |
dc.title | Polyunsaturated fatty acid status in treated isovaleric acidemia patients | en_US |
dc.type | Article | en_US |
dc.contributor.researchID | 11998938 - Dercksen, Marli | |
dc.contributor.researchID | 10061533 - Mienie, Lodewyk Jacobus | |
dc.contributor.researchID | 10055037 - Reinecke, Carolus Johannes | |