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Published on: November 29, 2013
Randomised controlled trial of essential fatty acid supplementation in phenylketonuria
M A Cleary1, F Feillet, F J White
1Department of Metabolic Medicine, Great Ormond St. Hospital for Sick Children, London, UK. Clearm@gosh.nhs.uk
Insights
Supplementing children with phenylketonuria (PKU) diets with essential fatty acids (EFA) improved docosahexaenoic acid (DHA) levels. This intervention enhanced LC-PUFA status without negatively impacting phenylalanine (Phe) control.
Area of Science:
- Biochemistry
- Nutritional Science
- Pediatrics
Background:
- Children with phenylketonuria (PKU) often have compromised long-chain polyunsaturated fatty acid (LC-PUFA) status.
- LC-PUFAs are crucial for central nervous system (CNS) development and can be synthesized from essential fatty acids (EFAs) if dietary intake is adequate.
Purpose of the Study:
- To assess the biochemical impact of a phenylalanine-free protein substitute supplemented with n-3 and n-6 EFAs on LC-PUFA status in children with PKU over 20 weeks.
Main Methods:
- A 20-week open, prospective study randomized 53 children (aged 1-10 years) with PKU to receive either a control formula or an EFA-supplemented test formula.
- Fatty acid status was assessed at baseline and 20 weeks. Dietary intake, safety, efficacy, and palatability were also evaluated.
Main Results:
- The EFA-supplemented group showed significantly higher fat and EFA intake.
- A significant increase in erythrocyte phospholipid docosahexaenoic acid (DHA) concentrations was observed in the test group (19% increase) compared to the control group (0.5% increase).
- Phenylalanine (Phe) control and growth were satisfactory in all participants.
Conclusions:
- Supplementation with a balanced blend of n-6 and n-3 EFAs effectively improves DHA status in children with PKU.
- This EFA supplementation enhances LC-PUFA status without compromising arachidonic acid (AA) status or phenylalanine control.
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.
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