Back to Search
Start Over
Polyunsaturated fatty acid status in treated isovaleric acidemia patients.
- Source :
-
European journal of clinical nutrition [Eur J Clin Nutr] 2016 Oct; Vol. 70 (10), pp. 1123-1126. Date of Electronic Publication: 2016 Jun 22. - Publication Year :
- 2016
-
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.<br />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.<br />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, P<0.001) and omega-6 (in particular 20:3n-6 P<0.0001 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.<br />Conclusions: This study emphasizes the potential nutritional insufficiencies that may occur because of therapeutic intervention in IVA.
- Subjects :
- Adolescent
Amino Acid Metabolism, Inborn Errors genetics
Case-Control Studies
Child
Child, Preschool
Female
Genetic Predisposition to Disease
Humans
Isovaleryl-CoA Dehydrogenase blood
Isovaleryl-CoA Dehydrogenase genetics
Male
Nutritional Status
Young Adult
Amino Acid Metabolism, Inborn Errors blood
Diet, Protein-Restricted
Fatty Acids, Unsaturated blood
Isovaleryl-CoA Dehydrogenase deficiency
Subjects
Details
- Language :
- English
- ISSN :
- 1476-5640
- Volume :
- 70
- Issue :
- 10
- Database :
- MEDLINE
- Journal :
- European journal of clinical nutrition
- Publication Type :
- Academic Journal
- Accession number :
- 27329611
- Full Text :
- https://doi.org/10.1038/ejcn.2016.100