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Biochemical effects of supplemented long-chain polyunsaturated fatty acids in hyperphenylalaninemia
C Agostoni1, S Scaglioni, M Bonvissuto
1Department of Pediatrics, San Paolo Hospital, Milan, 20142, Italy. agostoc@tin.it
Insights
Supplementing hyperphenylalaninemic (HPA) children with long-chain polyunsaturated fatty acids (LCPUFA) significantly improved docosahexaenoic acid (DHA) levels. This balanced LCPUFA supplementation enhanced DHA status without negatively impacting arachidonic acid (AA) levels in HPA children.
Area of Science:
- Biochemistry
- Pediatric Nutrition
- Metabolic Disorders
Background:
- Children with hyperphenylalaninemia (HPA) often exhibit deficiencies in long-chain polyunsaturated fatty acids (LCPUFA), especially docosahexaenoic acid (DHA).
- Low LCPUFA levels can impact various physiological functions, necessitating nutritional interventions.
Purpose of the Study:
- To evaluate the impact of balanced LCPUFA supplementation on the blood fatty acid profile and lipid status in HPA children.
- To determine if LCPUFA supplementation can correct DHA deficiencies in HPA children.
Main Methods:
- A 12-month, double-blind, placebo-controlled trial involving 20 well-controlled HPA school-age children.
- Participants received either LCPUFA-rich fat capsules (including DHA and arachidonic acid, AA) or a placebo (olive oil).
- Supplementation provided 0.3-0.5% of daily energy requirements as LCPUFA, with comparisons to healthy children.
Main Results:
- HPA children receiving LCPUFA supplementation showed a significant increase (approximately 100%) in plasma phospholipid and erythrocyte DHA levels.
- Arachidonic acid (AA) levels remained unchanged in the supplemented group.
- No significant alterations in overall blood lipid levels were observed.
Conclusions:
- Balanced LCPUFA supplementation is effective in improving DHA status in HPA children.
- This intervention appears safe, as it does not adversely affect AA status or blood lipid profiles.
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.