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芥酸对肾上腺脑白质营养不良患者血小板的影响。

Effect of erucic acid on platelets in patients with adrenoleukodystrophy.

作者信息

Kickler T S, Zinkham W H, Moser A, Shankroff J, Borel J, Moser H

机构信息

Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.

出版信息

Biochem Mol Med. 1996 Apr;57(2):125-33. doi: 10.1006/bmme.1996.0018.

DOI:10.1006/bmme.1996.0018
PMID:8733890
Abstract

In a clinical trial for the management of adrenoleukodystrophy, we analyzed the effect of erucic acid (a component of Lorenzo's oil) on platelet number, fatty acid composition, and function. Analysis of variance was performed to compare platelet counts before starting treatment with Lorenzo's oil and at 6 and 12 months. We measured platelet fatty acid composition in subjects and control patients and correlated these values with their platelet counts using discriminant analysis. After 6 months, the mean platelet count decreased from 247,000/mm3 to 169,000/mm3 (+/- 1 standard deviation 58,000,n =39), P < 0.0001 compared to 18 subjects on a control diet having a mean baseline platelet count of 259,000/mm3 (+/- 1 standard deviation 67,000, n = 19) and at 6 months 267,000/mm3 (+/- 1 standard deviation 71,000). We found at P < 0.05 that the platelet counts showed a strong inverse relationship with erucic acid levels and other omega 9 fatty acids that form from the administration of the erucic acid component of Lorenzo's oil. Morphologic and platelet sizing measurements suggest that the physical properties of platelets may also be affected by erucic acid. Our studies show that the ingestion of erucic acid affects platelet biology. This indicates that platelet counts and properties are influenced by monounsaturated fatty acids, in addition to the well-known effects of polyunsaturated fatty acids. In areas of the world where erucic acid is widely ingested, the biology of platelets in these populations may be affected.

摘要

在一项关于肾上腺脑白质营养不良治疗的临床试验中,我们分析了芥酸(洛伦佐油的一种成分)对血小板数量、脂肪酸组成及功能的影响。进行方差分析以比较开始使用洛伦佐油治疗前、治疗6个月和12个月时的血小板计数。我们测定了受试者和对照患者的血小板脂肪酸组成,并使用判别分析将这些值与其血小板计数相关联。6个月后,平均血小板计数从247,000/mm³降至169,000/mm³(±1标准差58,000,n = 39),与18名接受对照饮食的受试者相比,P < 0.0001,这些受试者的平均基线血小板计数为259,000/mm³(±1标准差67,000,n = 19),6个月时为267,000/mm³(±1标准差71,000)。我们发现,在P < 0.05时,血小板计数与芥酸水平以及由洛伦佐油的芥酸成分给药形成的其他ω-9脂肪酸呈强烈负相关。形态学和血小板大小测量表明,血小板的物理性质也可能受到芥酸的影响。我们的研究表明,摄入芥酸会影响血小板生物学。这表明,除了多不饱和脂肪酸的众所周知的作用外,单不饱和脂肪酸也会影响血小板计数和性质。在世界上广泛摄入芥酸的地区,这些人群中血小板的生物学特性可能会受到影响。

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Effect of erucic acid on platelets in patients with adrenoleukodystrophy.芥酸对肾上腺脑白质营养不良患者血小板的影响。
Biochem Mol Med. 1996 Apr;57(2):125-33. doi: 10.1006/bmme.1996.0018.
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A two-year trial of oleic and erucic acids ("Lorenzo's oil") as treatment for adrenomyeloneuropathy.一项使用油酸和芥酸(“洛伦佐之油”)治疗肾上腺脑白质营养不良的两年试验。
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A model-based approach to assess the exposure-response relationship of Lorenzo's oil in adrenoleukodystrophy.一种基于模型的方法来评估洛伦佐油在肾上腺脑白质营养不良中的暴露-反应关系。
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Lorenzo's oil and thrombocytopenia in patients with adrenoleukodystrophy.洛伦佐油与肾上腺脑白质营养不良患者的血小板减少症
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Thrombocytopenia associated with use of Lorenzo's oil.与使用洛伦佐油相关的血小板减少症。
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Normal natural killer cell activity in a patient with adrenoleukodystrophy treated with Lorenzo's oil.接受洛伦佐油治疗的肾上腺脑白质营养不良患者的正常自然杀伤细胞活性。
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Platelets in treated adrenoleukodystrophy: a brief report.治疗肾上腺脑白质营养不良中的血小板:简要报告。
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Treatment of X-linked adrenoleukodystrophy with Lorenzo's oil.用洛伦佐油治疗X连锁肾上腺脑白质营养不良。
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Lorenzo's oil and lymphocytopenia.洛伦佐的油与淋巴细胞减少症
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