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

• Verfasst von: Gramer, Gwendolyn [VerfasserIn]
• Verfasst von: Haege, Gisela [VerfasserIn]
• Verfasst von: Langhans, Claus-Dieter [VerfasserIn]
• Verfasst von: Schuhmann, Vera [VerfasserIn]
• Verfasst von: Burgard, Peter [VerfasserIn]
• Verfasst von: Hoffmann, Georg Friedrich [VerfasserIn]
• Titel: Long-chain polyunsaturated fatty acid status in children, adolescents and adults with phenylketonuria
• Verf.angabe: Gwendolyn Gramer, Gisela Haege, Claus-Dieter Langhans, Vera Schuhmann, Peter Burgard, Georg F. Hoffmann
• E-Jahr: 2016
• Jahr: 18 April 2016
• Umfang: 6 S.
• Fussnoten: Gesehen am 30.07.2020
• Titel Quelle: Enthalten in: Prostaglandins, leukotrienes and essential fatty acids
• Ort Quelle: Burlington, Mass. : Harcourt, 1988
• Jahr Quelle: 2016
• Band/Heft Quelle: 109(2016), Seite 52-57
• ISSN Quelle: 1532-2823
• DOI: doi:10.1016/j.plefa.2016.04.005
• Datenträger: Online-Ressource
• Sprache: eng
• Sach-SW: Docosahexaenoic acid
• Sach-SW: Long-chain polyunsaturated fatty acids
• Sach-SW: Phenylketonuria
• K10plus-PPN: 1725958368

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.