四氢生物蝶呤治疗苯丙酮尿症患者的长链多不饱和脂肪酸状况。
Long-chain polyunsaturated fatty acid status in phenylketonuric patients treated with tetrahydrobiopterin.
机构信息
Department of Biochemistry, Barcelona, Spain.
出版信息
Clin Biochem. 2010 Mar;43(4-5):411-5. doi: 10.1016/j.clinbiochem.2009.11.013. Epub 2009 Dec 3.
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.
目的
评估 BH（4）治疗苯丙酮尿症（PKU）患者的长链多不饱和脂肪酸（LCPUFA）组成。
设计和方法
对 13 名接受 BH（4）治疗的 PKU 患者的血浆和红细胞 LCPUFA 组成进行横断面研究，并与接受低蛋白饮食的 48 名 PKU 患者和 17 名自由饮食的轻度高苯丙氨酸血症（HPA）患者的数据进行比较。采用气相色谱法分析多不饱和脂肪酸。
结果
与轻度 HPA 患者和我们的参考值相比，BH（4）治疗的 PKU 患者的血浆和红细胞二十二碳六烯酸（DHA）和 LCPUFA 缺乏标志物无显著差异，但与接受低蛋白饮食的 PKU 患者相比差异显著（p<0.0001）。在任何研究组中，必需脂肪酸和花生四烯酸组成均无显著差异。只有接受低蛋白饮食的 PKU 患者的 DHA 值与饮食控制指数相关（p=0.002）。
结论
接受 BH（4）治疗的 PKU 患者的 LCPUFA 状况处于参考值范围内。这进一步证明了 BH（4）治疗的优势。
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