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

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-matc...

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Hauptverfasser: Gramer, Gwendolyn (VerfasserIn) , Haege, Gisela (VerfasserIn) , Langhans, Claus-Dieter (VerfasserIn) , Schuhmann, Vera (VerfasserIn) , Burgard, Peter (VerfasserIn) , Hoffmann, Georg F. (VerfasserIn)
Dokumenttyp: Article (Journal)
Sprache:Englisch
Veröffentlicht: 18 April 2016
In: Prostaglandins, leukotrienes and essential fatty acids
Year: 2016, Jahrgang: 109, Pages: 52-57
ISSN:1532-2823
DOI:10.1016/j.plefa.2016.04.005
Online-Zugang:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.plefa.2016.04.005
Verlag, lizenzpflichtig, Volltext: http://www.sciencedirect.com/science/article/pii/S0952327816300059
Volltext
Verfasserangaben:Gwendolyn Gramer, Gisela Haege, Claus-Dieter Langhans, Vera Schuhmann, Peter Burgard, Georg F. Hoffmann

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520 |a 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. 
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