Randomised controlled trial of essential fatty acid supplementation in phenylketonuria

European Journal of Clinical Nutrition
M A ClearyP J Rutherford

Abstract

The long-chain polyunsaturated fatty acids (LC-PUFA) status of children with PKU is often compromised. LC-PUFA, which are important fatty acids in the development of the CNS, can be synthesised endogenously from the parent essential fatty acids (EFA) provided dietary intakes are adequate. This study was designed to assess the biochemical effect over a 20-week period of a phe-free protein substitute that has been supplemented with a balanced blend of n-3 and n-6 EFAs on LC-PUFA status of children with PKU. Fifty three community-living children aged 1-10 years diagnosed with PKU in the newborn period were recruited from seven tertiary centres in the UK and France and randomised to a fat-free control formula or the EFA-supplemented test-treatment formula in an open, prospective study. Forty four children completed the study (20 controls, 24 test-treatments). Fatty acid status was assessed at entry and 20-weeks follow-up. Three day dietary diaries were recorded at 20 weeks' follow-up. The safety, efficacy and palatability of the test-treatment formula were also assessed. The test-treatment group had significantly higher intakes of fat and EFA than the control group. There was a significant between group difference (P=0.04) in incre...Continue Reading

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Citations

Feb 13, 2010·Journal of Inherited Metabolic Disease·François Feillet, Carlo Agostoni
Sep 1, 2010·Nutrients·Katalin Fekete, Tamás Decsi
Jan 5, 2014·Genetics in Medicine : Official Journal of the American College of Medical Genetics·Rani H SinghSandra C Van Calcar
Dec 29, 2015·Nutrition, Metabolism, and Cardiovascular Diseases : NMCD·E VerduciM Giovannini
Jul 21, 2009·Prostaglandins, Leukotrienes, and Essential Fatty Acids·Berthold KoletzkoFabienne L Hanebutt
Apr 14, 2009·Clinical Nutrition : Official Journal of the European Society of Parenteral and Enteral Nutrition·Kirsten AhringMargreet van Rijn
Aug 4, 2010·Molecular Genetics and Metabolism·G M EnnsE Jurecki
Mar 29, 2014·Molecular Genetics and Metabolism·Kathryn M CampJustin M Young
Jan 22, 2010·The Cochrane Database of Systematic Reviews·Vanessa J Poustie, Joanne Wildgoose
Feb 28, 2015·The Cochrane Database of Systematic Reviews·Sarah H L Yi, Rani H Singh
Jul 17, 2020·The Cochrane Database of Systematic Reviews·Elisabeth Jameson, Tracey Remmington
Feb 6, 2021·Nutrients·Anne DalyAnita MacDonald
May 29, 2020·Archives of Biochemistry and Biophysics·Inês M S GuerraAna S P Moreira
Apr 6, 2021·Journal of Proteome Research·Inês M S GuerraAna S P Moreira

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