publication . Article . Other literature type . 2018

Determinants of Plasma Docosahexaenoic Acid Levels and Their Relationship to Neurological and Cognitive Functions in PKU Patients: A Double Blind Randomized Supplementation Study

Hans Demmelmair; Anita MacDonald; Urania Kotzaeridou; Peter Burgard; Domingo Gonzalez-Lamuno; Elvira Verduci; Melike Ersoy; Gulden Gokcay; Behiye Alyanak; Eva Reischl; ...
Open Access English
  • Published: 01 Dec 2018 Journal: Nutrients, volume 10, issue 12 (eissn: 2072-6643, Copyright policy)
  • Publisher: MDPI
Abstract
day for six months. Before and after supplementation, we determined plasma fatty acid concentrations, latencies of visually evoked potentials, fine and gross motor behavior, and IQ. Fatty acid desaturase genotypes were also determined. DHA supplementation increased plasma glycerophospholipid DHA proportional to dose by 0.4% DHA per 1 mg intake/kg bodyweight. Functional outcomes were not associated with DHA status before and after intervention and remained unchanged by supplementation. Genotypes were associated with plasma arachidonic acid levels and, if considered together with the levels of the precursor alpha-linolenic acid, also with DHA. Functional outcomes ...
Subjects
Medical Subject Headings: congenital, hereditary, and neonatal diseases and abnormalitiesnutritional and metabolic diseaseslipids (amino acids, peptides, and proteins)food and beveragesgenetic structures
free text keywords: Medizin, Article, phenylketonuria, docosahexaenoic acid, cognitive function, motor skills, neurological function, phenylketonuria; docosahexaenoic acid; cognitive function; motor skills; neurological function, Settore MED/38 - Pediatria Generale e Specialistica, Nutrition. Foods and food supply, TX341-641, ddc:610, Fatty acid, chemistry.chemical_classification, chemistry, Diabetes mellitus, medicine.disease, medicine, Arachidonic acid, chemistry.chemical_compound, Double blind, business.industry, business, Internal medicine, medicine.medical_specialty, Fatty acid desaturase, biology.protein, biology, Endocrinology
Funded by
EC| NUTRIMENTHE
Project
NUTRIMENTHE
“Effect of diet on the mental performance of children”
  • Funder: European Commission (EC)
  • Project Code: 212652
  • Funding stream: FP7 | SP1 | KBBE
67 references, page 1 of 5

Brumm, V.L., Azen, C., Moats, R.A., Stern, A.M., Broomand, C., Nelson, M.D., Koch, R.. Neuropsychological outcome of subjects participating in the PKU adult collaborative study: A preliminary review. J. Inherit. Metab. Dis.. 2004; 27: 549-566 [OpenAIRE] [PubMed] [DOI]

Koletzko, B., Beblo, S., Demmelmair, H., Hanebutt, F.L.. Omega-3 LC-PUFA supply and neurological outcomes in children with phenylketonuria (PKU). J. Pediatr. Gastroenterol. Nutr.. 2009; 48: S2-S7 [OpenAIRE] [PubMed] [DOI]

Gonzalez-Rodriguez, L.G., Aparicio, A., Lopez-Sobaler, A.M., Ortega, R.M.. Omega 3 and omega 6 fatty acids intake and dietary sources in a representative sample of Spanish adults. Int. J. Vitam. Nutr. Res.. 2013; 83: 36-47 [OpenAIRE] [PubMed] [DOI]

Sanjurjo, P., Perteagudo, L., Soriano, J.R., Vilaseca, A., Campistol, J.. Polyunsaturated fatty acid status in patients with phenylketonuria. J. Inherit. Metab. Dis.. 1994; 17: 704-709 [PubMed] [DOI]

Galli, C., Agostoni, C., Mosconi, C., Riva, E., Salari, P.C., Giovannini, M.. Reduced plasma C-20 and C-22 polyunsaturated fatty acids in children with phenylketonuria during dietary intervention. J. Pediatr.. 1991; 119: 562-567 [PubMed] [DOI]

Giovannini, M., Biasucci, G., Agostoni, C., Luotti, D., Riva, E.. Lipid status and fatty acid metabolism in phenylketonuria. J. Inherit. Metab. Dis.. 1995; 18: 265-272 [OpenAIRE] [PubMed] [DOI]

Lohner, S., Fekete, K., Decsi, T.. Lower n-3 long-chain polyunsaturated fatty acid values in patients with phenylketonuria: A systematic review and meta-analysis. Nutr. Res.. 2013; 33: 513-520 [OpenAIRE] [PubMed] [DOI]

Demmelmair, H., Koletzko, B.. Importance of fatty acids in the perinatal period. World Rev. Nutr. Diet. 2015; 112: 31-47 [PubMed]

Stonehouse, W.. Does consumption of LC omega-3 PUFA enhance cognitive performance in healthy school-aged children and throughout adulthood? Evidence from clinical trials. Nutrients. 2014; 6: 2730-2758 [OpenAIRE] [PubMed] [DOI]

Kuratko, C.N., Barrett, E.C., Nelson, E.B., Salem, N.. The relationship of docosahexaenoic acid (DHA) with learning and behavior in healthy children: A review. Nutrients. 2013; 5: 2777-2810 [OpenAIRE] [PubMed] [DOI]

Calder, P.C.. Docosahexaenoic Acid. Ann. Nutr. Metab.. 2016; 69: 7-21 [PubMed] [DOI]

Schmitz, G., Ecker, J.. The opposing effects of n-3 and n-6 fatty acids. Prog. Lipid Res.. 2008; 47: 147-155 [OpenAIRE] [PubMed] [DOI]

Beblo, S., Reinhardt, H., Demmelmair, H., Muntau, A.C., Koletzko, B.. Effect of fish oil supplementation on fatty acid status, coordination, and fine motor skills in children with phenylketonuria. J. Pediatr.. 2007; 150: 479-484 [PubMed] [DOI]

Beblo, S., Reinhardt, H., Muntau, A.C., Muller-Felber, W., Roscher, A.A., Koletzko, B.. Fish oil supplementation improves visual evoked potentials in children with phenyketonuria. Neurology. 2001; 57: 1488-1491 [OpenAIRE] [PubMed] [DOI]

Agostoni, C., Massetto, N., Biasucci, G., Rottoli, A., Bonvissuto, M., Bruzzese, M.G., Giovannini, M., Riva, E.. Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated children with hyperphenylalaninemia. J. Pediatr.. 2000; 137: 504-509 [OpenAIRE] [PubMed] [DOI]

67 references, page 1 of 5
Abstract
day for six months. Before and after supplementation, we determined plasma fatty acid concentrations, latencies of visually evoked potentials, fine and gross motor behavior, and IQ. Fatty acid desaturase genotypes were also determined. DHA supplementation increased plasma glycerophospholipid DHA proportional to dose by 0.4% DHA per 1 mg intake/kg bodyweight. Functional outcomes were not associated with DHA status before and after intervention and remained unchanged by supplementation. Genotypes were associated with plasma arachidonic acid levels and, if considered together with the levels of the precursor alpha-linolenic acid, also with DHA. Functional outcomes ...
Subjects
Medical Subject Headings: congenital, hereditary, and neonatal diseases and abnormalitiesnutritional and metabolic diseaseslipids (amino acids, peptides, and proteins)food and beveragesgenetic structures
free text keywords: Medizin, Article, phenylketonuria, docosahexaenoic acid, cognitive function, motor skills, neurological function, phenylketonuria; docosahexaenoic acid; cognitive function; motor skills; neurological function, Settore MED/38 - Pediatria Generale e Specialistica, Nutrition. Foods and food supply, TX341-641, ddc:610, Fatty acid, chemistry.chemical_classification, chemistry, Diabetes mellitus, medicine.disease, medicine, Arachidonic acid, chemistry.chemical_compound, Double blind, business.industry, business, Internal medicine, medicine.medical_specialty, Fatty acid desaturase, biology.protein, biology, Endocrinology
Funded by
EC| NUTRIMENTHE
Project
NUTRIMENTHE
“Effect of diet on the mental performance of children”
  • Funder: European Commission (EC)
  • Project Code: 212652
  • Funding stream: FP7 | SP1 | KBBE
67 references, page 1 of 5

Brumm, V.L., Azen, C., Moats, R.A., Stern, A.M., Broomand, C., Nelson, M.D., Koch, R.. Neuropsychological outcome of subjects participating in the PKU adult collaborative study: A preliminary review. J. Inherit. Metab. Dis.. 2004; 27: 549-566 [OpenAIRE] [PubMed] [DOI]

Koletzko, B., Beblo, S., Demmelmair, H., Hanebutt, F.L.. Omega-3 LC-PUFA supply and neurological outcomes in children with phenylketonuria (PKU). J. Pediatr. Gastroenterol. Nutr.. 2009; 48: S2-S7 [OpenAIRE] [PubMed] [DOI]

Gonzalez-Rodriguez, L.G., Aparicio, A., Lopez-Sobaler, A.M., Ortega, R.M.. Omega 3 and omega 6 fatty acids intake and dietary sources in a representative sample of Spanish adults. Int. J. Vitam. Nutr. Res.. 2013; 83: 36-47 [OpenAIRE] [PubMed] [DOI]

Sanjurjo, P., Perteagudo, L., Soriano, J.R., Vilaseca, A., Campistol, J.. Polyunsaturated fatty acid status in patients with phenylketonuria. J. Inherit. Metab. Dis.. 1994; 17: 704-709 [PubMed] [DOI]

Galli, C., Agostoni, C., Mosconi, C., Riva, E., Salari, P.C., Giovannini, M.. Reduced plasma C-20 and C-22 polyunsaturated fatty acids in children with phenylketonuria during dietary intervention. J. Pediatr.. 1991; 119: 562-567 [PubMed] [DOI]

Giovannini, M., Biasucci, G., Agostoni, C., Luotti, D., Riva, E.. Lipid status and fatty acid metabolism in phenylketonuria. J. Inherit. Metab. Dis.. 1995; 18: 265-272 [OpenAIRE] [PubMed] [DOI]

Lohner, S., Fekete, K., Decsi, T.. Lower n-3 long-chain polyunsaturated fatty acid values in patients with phenylketonuria: A systematic review and meta-analysis. Nutr. Res.. 2013; 33: 513-520 [OpenAIRE] [PubMed] [DOI]

Demmelmair, H., Koletzko, B.. Importance of fatty acids in the perinatal period. World Rev. Nutr. Diet. 2015; 112: 31-47 [PubMed]

Stonehouse, W.. Does consumption of LC omega-3 PUFA enhance cognitive performance in healthy school-aged children and throughout adulthood? Evidence from clinical trials. Nutrients. 2014; 6: 2730-2758 [OpenAIRE] [PubMed] [DOI]

Kuratko, C.N., Barrett, E.C., Nelson, E.B., Salem, N.. The relationship of docosahexaenoic acid (DHA) with learning and behavior in healthy children: A review. Nutrients. 2013; 5: 2777-2810 [OpenAIRE] [PubMed] [DOI]

Calder, P.C.. Docosahexaenoic Acid. Ann. Nutr. Metab.. 2016; 69: 7-21 [PubMed] [DOI]

Schmitz, G., Ecker, J.. The opposing effects of n-3 and n-6 fatty acids. Prog. Lipid Res.. 2008; 47: 147-155 [OpenAIRE] [PubMed] [DOI]

Beblo, S., Reinhardt, H., Demmelmair, H., Muntau, A.C., Koletzko, B.. Effect of fish oil supplementation on fatty acid status, coordination, and fine motor skills in children with phenylketonuria. J. Pediatr.. 2007; 150: 479-484 [PubMed] [DOI]

Beblo, S., Reinhardt, H., Muntau, A.C., Muller-Felber, W., Roscher, A.A., Koletzko, B.. Fish oil supplementation improves visual evoked potentials in children with phenyketonuria. Neurology. 2001; 57: 1488-1491 [OpenAIRE] [PubMed] [DOI]

Agostoni, C., Massetto, N., Biasucci, G., Rottoli, A., Bonvissuto, M., Bruzzese, M.G., Giovannini, M., Riva, E.. Effects of long-chain polyunsaturated fatty acid supplementation on fatty acid status and visual function in treated children with hyperphenylalaninemia. J. Pediatr.. 2000; 137: 504-509 [OpenAIRE] [PubMed] [DOI]

67 references, page 1 of 5
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publication . Article . Other literature type . 2018

Determinants of Plasma Docosahexaenoic Acid Levels and Their Relationship to Neurological and Cognitive Functions in PKU Patients: A Double Blind Randomized Supplementation Study

Hans Demmelmair; Anita MacDonald; Urania Kotzaeridou; Peter Burgard; Domingo Gonzalez-Lamuno; Elvira Verduci; Melike Ersoy; Gulden Gokcay; Behiye Alyanak; Eva Reischl; ...