Original Article
European Journal of Clinical Nutrition (2006) 60, 915–920. doi:10.1038/sj.ejcn.1602401; published online 8 March 2006
Randomised controlled trial of essential fatty acid supplementation in phenylketonuria
Sources of financial support: SHS International Ltd, 100 Wavertree Boulevard, Wavertree Technology Park, Liverpool, L7 9PT, UK.
Guarantor: MA Cleary.
Contributors: All contributors are clinicians or dietitians from the seven recruiting centres. All contributors were involved in the development of the study protocol. The principal investigator (MC) produced the initial draft of the manuscript and the dietitians (FW, AM, AG, PR) collected and analysed the diet diaries. The other contributors provided additional information and comments on the drafts of the manuscript.
M A Cleary1, F Feillet2, F J White3, M Vidailhet2, A MacDonald4, A Grimsley5, N Maurin6, H Ogier de Baulny7 and P J Rutherford8
- 1Department of Metabolic Medicine, Great Ormond St. Hospital for Sick Children, London, UK
- 2Department of Pediatrics, Hôpital D'enfants Brabois, Nancy, France
- 3Willink Biochemical Genetics Unit, Royal Manchester Children's Hospital, Manchester, UK
- 4Birmingham Children's Hospital, Birmingham, UK
- 5Royal Belfast Hospital for Sick Children, Belfast, UK
- 6Hôpital Timone Enfants, Marseille, France
- 7Hôpital Robert Debré, Paris, France
- 8Alder Hey Children's Hospital, Liverpool, UK
Correspondence: Dr MA Cleary, Consultant Paediatrician, Department of Metabolic Medicine, 7th Floor Southwood Building, Great Ormond St Hospital for Sick Children, London WCIN 3JH, UK. E-mail: Clearm@gosh.nhs.uk
Received 9 July 2005; Revised 8 December 2005; Accepted 19 December 2005; Published online 8 March 2006.
Abstract
Objective:
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.
Design, setting and subjects:
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.
Results:
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 increases in median docosahexaenoic acid (DHA) concentrations in erythrocyte phospholipids, which increased by 19% in the test-treatment group and by 0.5% in the control group over the study period. Growth and phe control were satisfactory in all subjects.
Conclusions:
Supplementing the diets of children with PKU with a balanced blend of n-6 and n-3 EFA improves DHA status without compromising AA status.
Keywords:
phenylketonuria, docosahexaenoic acid, arachidonic acid, long-chain polyunsaturated fatty acids
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