Long-chain polyunsaturated fatty acid status in children, adolescents and adults with phenylketonuria

Abstract Background Patients with phenylketonuria have been reported to be deficient in long-chain polyunsaturated fatty acids (LCPUFAs). It has been postulated that good compliance with the dietary regimen negatively influences LCPUFA status. Methods In 36 patients with phenylketonuria and 18 age-m...

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Bibliographic Details
Published in:Prostaglandins, leukotrienes and essential fatty acids leukotrienes and essential fatty acids, 2016-06, Vol.109, p.52-57
Main Authors: Gramer, Gwendolyn, Haege, Gisela, Langhans, Claus-Dieter, Schuhmann, Vera, Burgard, Peter, Hoffmann, Georg F
Format: Article
Language:English
Subjects:
Adolescent
Adult
Advanced Basic Science
Child
Cholesterol Esters - blood
Docosahexaenoic acid
Docosahexaenoic Acids - blood
Eicosapentaenoic Acid - blood
Endocrinology & Metabolism
Erythrocytes - metabolism
Fatty Acids, Unsaturated - blood
Female
Humans
Long-chain polyunsaturated fatty acids
Male
Middle Aged
Nutritional Status
Phenylketonuria
Phenylketonurias - diet therapy
Phenylketonurias - metabolism
Phosphatidylcholines - blood
Phosphatidylethanolamines - blood
Phospholipids - blood
Young Adult
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Summary:Abstract Background Patients with phenylketonuria have been reported to be deficient in long-chain polyunsaturated fatty acids (LCPUFAs). It has been postulated that good compliance with the dietary regimen negatively influences LCPUFA status. Methods In 36 patients with phenylketonuria and 18 age-matched healthy control subjects LCPUFA-levels in plasma phospholipids and cholesteryl esters, erythrocyte phosphatidylcholine and phosphatidylethanolamine were evaluated. Results Eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA) levels did not differ significantly between patients and control subjects in plasma and erythrocyte fractions. There was a significant negative correlation between SDS (standard deviation) scores of DHA-levels in erythrocyte parameters from the respective age-matched control group and patients’ concurrent and long-term phenylalanine levels for erythrocyte phosphatidylethanolamine and erythrocyte phosphatidylcholine. Patients with lower (higher) phenylalanine levels had positive (negative) DHA-SDS. Conclusion In contrast to previous reports we did not find lower LCPUFA-levels in patients with phenylketonuria compared to age-matched healthy control subjects. Good dietary control was associated with better LCPUFA status.
ISSN:0952-3278
1532-2823
DOI:10.1016/j.plefa.2016.04.005