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Essential polyunsaturated fatty acids in plasma and erythrocytes of children with inborn errors of amino acid metabolism

Vlaardingerbroek, H. ; Hornstra, G. ; de Koning, T. J. LU ; Smeitink, J. A.M. ; Bakker, H. D. ; de Klerk, H. B.C. and Rubio-Gozalbo, M. E. (2006) In Molecular Genetics and Metabolism 88(2). p.159-165
Abstract

Essential fatty acids (EFAs), and their longer-chain more-unsaturated derivatives (LCPUFAs) in particular, are essential for normal growth and cognitive development during childhood. Children with inborn errors of amino acid metabolism represent a risk population for a reduced LCPUFA status because their diet is low in EFAs and LCPUFAs. We have investigated the EFA and LCPUFA status of children with various amino acid metabolism disorders (not PKU) under treatment. Fatty acid profiles of plasma and erythrocyte phospholipids of 33 patients (aged 0-18 years) and 38 matched controls were determined by gas-liquid chromatography. Food-frequency questionnaires were used to assess the mean fatty acid intake. The dietary intake of the EFAs... (More)

Essential fatty acids (EFAs), and their longer-chain more-unsaturated derivatives (LCPUFAs) in particular, are essential for normal growth and cognitive development during childhood. Children with inborn errors of amino acid metabolism represent a risk population for a reduced LCPUFA status because their diet is low in EFAs and LCPUFAs. We have investigated the EFA and LCPUFA status of children with various amino acid metabolism disorders (not PKU) under treatment. Fatty acid profiles of plasma and erythrocyte phospholipids of 33 patients (aged 0-18 years) and 38 matched controls were determined by gas-liquid chromatography. Food-frequency questionnaires were used to assess the mean fatty acid intake. The dietary intake of the EFAs linoleic acid (LA) and α-linolenic acid (ALA) was comparable in both groups, while the LCPUFA intake was much lower in patients. This was associated with lower relative concentrations (% of total fatty acids) of n-3 docosahexaenoic acid (DHA) in plasma and erythrocyte phospholipids. Concentrations of arachidonic acid (AA) did not differ. The same was observed for the two EFAs LA and ALA. Thus, as compared to healthy controls, children with amino acid metabolism disorders have a lower intake of LCPUFAs and have lower concentrations of DHA but not of AA in plasma and erythrocyte phospholipids. This suggests that endogenous AA synthesis might guarantee an adequate AA status. The lower DHA status, however, warrants further investigations regarding the impact of DHA supplementation on growth and development of these children.

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author
; ; ; ; ; and
publishing date
type
Contribution to journal
publication status
published
subject
keywords
Arachidonic acid, Docosahexaenoic acid, Essential fatty acids, Inborn error of amino acid metabolism, Long-chain polyunsaturated fatty acids, Protein restriction
in
Molecular Genetics and Metabolism
volume
88
issue
2
pages
7 pages
publisher
Elsevier
external identifiers
  • pmid:16530443
  • scopus:33646402910
ISSN
1096-7192
DOI
10.1016/j.ymgme.2006.01.012
language
English
LU publication?
no
id
6739a9fd-a03d-4257-ac45-679b4152b094
date added to LUP
2020-03-03 19:02:25
date last changed
2024-04-17 06:13:04
@article{6739a9fd-a03d-4257-ac45-679b4152b094,
  abstract     = {{<p>Essential fatty acids (EFAs), and their longer-chain more-unsaturated derivatives (LCPUFAs) in particular, are essential for normal growth and cognitive development during childhood. Children with inborn errors of amino acid metabolism represent a risk population for a reduced LCPUFA status because their diet is low in EFAs and LCPUFAs. We have investigated the EFA and LCPUFA status of children with various amino acid metabolism disorders (not PKU) under treatment. Fatty acid profiles of plasma and erythrocyte phospholipids of 33 patients (aged 0-18 years) and 38 matched controls were determined by gas-liquid chromatography. Food-frequency questionnaires were used to assess the mean fatty acid intake. The dietary intake of the EFAs linoleic acid (LA) and α-linolenic acid (ALA) was comparable in both groups, while the LCPUFA intake was much lower in patients. This was associated with lower relative concentrations (% of total fatty acids) of n-3 docosahexaenoic acid (DHA) in plasma and erythrocyte phospholipids. Concentrations of arachidonic acid (AA) did not differ. The same was observed for the two EFAs LA and ALA. Thus, as compared to healthy controls, children with amino acid metabolism disorders have a lower intake of LCPUFAs and have lower concentrations of DHA but not of AA in plasma and erythrocyte phospholipids. This suggests that endogenous AA synthesis might guarantee an adequate AA status. The lower DHA status, however, warrants further investigations regarding the impact of DHA supplementation on growth and development of these children.</p>}},
  author       = {{Vlaardingerbroek, H. and Hornstra, G. and de Koning, T. J. and Smeitink, J. A.M. and Bakker, H. D. and de Klerk, H. B.C. and Rubio-Gozalbo, M. E.}},
  issn         = {{1096-7192}},
  keywords     = {{Arachidonic acid; Docosahexaenoic acid; Essential fatty acids; Inborn error of amino acid metabolism; Long-chain polyunsaturated fatty acids; Protein restriction}},
  language     = {{eng}},
  month        = {{06}},
  number       = {{2}},
  pages        = {{159--165}},
  publisher    = {{Elsevier}},
  series       = {{Molecular Genetics and Metabolism}},
  title        = {{Essential polyunsaturated fatty acids in plasma and erythrocytes of children with inborn errors of amino acid metabolism}},
  url          = {{http://dx.doi.org/10.1016/j.ymgme.2006.01.012}},
  doi          = {{10.1016/j.ymgme.2006.01.012}},
  volume       = {{88}},
  year         = {{2006}},
}