Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).
Therapeutic modification of membrane lipid abnormalities in juvenile neuronal ceroid-lipofuscinosis (Batten disease).
复制标题
幼年神经元蜡质脂褐素沉着症（巴顿病）膜脂异常的治疗修饰。
DOI：
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发表时间：
1988
期刊：
影响因子：
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通讯作者：
R. Pullarkat
中科院分区：
文献类型：
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作者：
M. Bennett;M. Bennett;G. Hosking;R. Gayton;Georgina Thompson;J. H. Galloway;I. Cartwright;J. M. Opitz;J. Reynolds;R. Pullarkat
5例幼年神经元类脂肪褐皮病患者血清β前脂蛋白缺失或减少，红细胞膜脂质中多不饱和脂肪酸缺乏，研究人员用鱼油提取物（富含omega-3脂肪酸）补充剂治疗了一年，并鼓励他们增加玉米油脂（富含omega-6脂肪酸）的饮食摄入，以促进这些脂肪酸与细胞膜的结合。一年后，红细胞膜中omega-3脂肪酸（P < 0.05）和总多不饱和脂肪酸（P < 0.02）显著增加，但omega-6脂肪酸的掺入没有变化。在补充前后对患者进行临床、心理测量学和神经生理学评估。治疗一年后无明显变化。
Five patients with juvenile neuronal ceroid-lipofuscinosis, who were shown to have absent or reduced serum prebetalipoprotein and a deficiency of polyunsaturated fatty acids in erythrocyte membrane lipids, were treated for a period of one year with supplements of fish oil extract (rich in omega-3 fatty acids) and encouraged to increase dietary intake of corn oil lipid (rich in omega-6 fatty acids) in an attempt to promote incorporation of these fatty acids into membranes. After one year there was a significant increase in omega-3 fatty acids (P less than 0.05) and in the total polyunsaturated fatty acids (P less than 0.02) in erythrocyte membranes but no change in the incorporation of omega-6 fatty acids. The patients were assessed clinically, psychometrically and neurophysiologically before and after the supplementation. No significant changes were noted after one year of treatment.