Literature DB >> 11237478

Biochemical effects of supplemented long-chain polyunsaturated fatty acids in hyperphenylalaninemia.

C Agostoni1, S Scaglioni, M Bonvissuto, M G Bruzzese, M Giovannini, E Riva.   

Abstract

Hyperphenylalaninemic (HPA) children display low levels of long-chain polyunsaturated fatty acids (LCPUFA), particularly docosahexaenoic acid (DHA), in circulating lipids and erythrocytes. We have investigated the effects on the blood fatty acid status and lipid picture of a balanced supplementation with LCPUFA in HPA children through a double-blind, placebo-controlled trial. A total of 20 well-controlled HPA, school-age children were randomized to receive through a 12-month trial fat capsules supplying either 26% fatty acid as LCPUFA (including 4.6%gamma -linolenic acid, 7.4% arachidonic acid, AA, 5.5% eicosapentaenoic acid and 8% DHA) or placebo (olive oil). The study supplementation was administered in order to provide 0.3-0.5% of the individual daily energy requirements as LCPUFA. Reference data were obtained from healthy children of comparable age. Among HPA children (whose DHA status was poor at baseline), those supplemented with LCPUFA showed an increase of around 100% in the baseline DHA levels in plasma phospholipids and erythrocytes. No changes of AA levels were observed. Blood lipid levels did not significantly change. A balanced supplementation with LCPUFA in treated HPA children may improve the DHA status without adversely affecting the AA status.

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Year:  2001        PMID: 11237478     DOI: 10.1054/plef.2001.0249

Source DB:  PubMed          Journal:  Prostaglandins Leukot Essent Fatty Acids        ISSN: 0952-3278            Impact factor:   4.006


  7 in total

1.  Nutritional Management of Phenylketonuria.

Authors:  Erin L Macleod; Denise M Ney
Journal:  Ann Nestle Eng       Date:  2010-06

2.  Metabolomic Markers of Essential Fatty Acids, Carnitine, and Cholesterol Metabolism in Adults and Adolescents with Phenylketonuria.

Authors:  Bridget M Stroup; Nivedita Nair; Sangita G Murali; Katarzyna Broniowska; Fran Rohr; Harvey L Levy; Denise M Ney
Journal:  J Nutr       Date:  2018-02-01       Impact factor: 4.798

3.  NORMAL FATTY ACID CONCENTRATIONS IN YOUNG CHILDREN WITH PHENYLKETONURIA (PKU).

Authors:  Stacey M Lavoie; Cary O Harding; Melanie B Gillingham
Journal:  Top Clin Nutr       Date:  2009-10-01       Impact factor: 0.508

Review 4.  Long-chain polyunsaturated fatty acids in inborn errors of metabolism.

Authors:  Katalin Fekete; Tamás Decsi
Journal:  Nutrients       Date:  2010-09-15       Impact factor: 5.717

Review 5.  A Review of Recruitment, Adherence and Drop-Out Rates in Omega-3 Polyunsaturated Fatty Acid Supplementation Trials in Children and Adolescents.

Authors:  Inge S M van der Wurff; Barbara J Meyer; Renate H M de Groot
Journal:  Nutrients       Date:  2017-05-10       Impact factor: 5.717

6.  Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis.

Authors:  Gina A Montoya Parra; Rani H Singh; Aysun Cetinyurek-Yavuz; Mirjam Kuhn; Anita MacDonald
Journal:  Orphanet J Rare Dis       Date:  2018-06-26       Impact factor: 4.123

Review 7.  Phenylketonuria: dietary and therapeutic challenges.

Authors:  M Giovannini; E Verduci; E Salvatici; L Fiori; E Riva
Journal:  J Inherit Metab Dis       Date:  2007-03-08       Impact factor: 4.750

  7 in total

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