AI Article Synopsis
- The study aimed to assess the levels of long-chain polyunsaturated fatty acids (LCPUFA) in patients with phenylketonuria (PKU) who were treated with BH(4) compared to other PKU patients on a restricted diet and mild hyperphenylalaninemia (HPA) patients.
- Results showed no significant differences in LCPUFA status, particularly docosahexaenoic acid (DHA), between PKU patients on BH(4) and other groups, but notable differences were found when compared to PKU patients on a protein-restricted diet.
- The findings suggest that PKU patients receiving BH(4) maintain LCPUFA levels within healthy reference ranges,
Article Abstract
Objectives: To evaluate LCPUFA composition in PKU patients treated with BH(4).
Design And Methods: Cross-sectional study of plasma and erythrocyte LCPUFA composition of 13 PKU patients treated with BH(4) compared with data from 48 PKU patients on protein-restricted diet, and 17 mild HPA patients on free diet. PUFA were analysed by gas chromatography.
Results: Plasma and erythrocyte docosahexaenoic acid (DHA), and LCPUFA deficiency markers did not show significant differences in PKU patients on BH(4) compared with those with mild HPA and our reference values, but they did in comparison with PKU on protein-restricted diet (p<0.0001). Essential fatty acids and arachidonic acid composition were not significantly different in any of the studied groups. DHA values correlate with the index of dietary control only in PKU patients on protein-restricted diet (p=0.002).
Conclusion: LCPUFA status is within the reference values in PKU patients treated with BH(4). This translates to a further advantage of BH(4) therapy.
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| http://dx.doi.org/10.1016/j.clinbiochem.2009.11.013 | DOI Listing |
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