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用低密度脂蛋白(LDL)单采术治疗脑腱性黄瘤病。

Treatment of cerebrotendinous xanthomatosis with low-density lipoprotein (LDL)-apheresis.

作者信息

Mimura Y, Kuriyama M, Tokimura Y, Fujiyama J, Osame M, Takesako K, Tanaka N

机构信息

Third Department of Internal Medicine Kagoshima University School of Medicine, Japan.

出版信息

J Neurol Sci. 1993 Feb;114(2):227-30. doi: 10.1016/0022-510x(93)90303-g.

DOI:10.1016/0022-510x(93)90303-g
PMID:8445406
Abstract

We studied the effects of LDL-apheresis on the biochemical and clinical abnormalities of 5 patients with cerebrotendinous xanthomatosis (CTX). Levels of both cholestanol and cholesterol decreased to approximately 60% of those of pretreatment after one perfusion and gradually returned to their initial levels within 2 weeks. Improvement of clinical manifestations and regression of Achilles tendon xanthomas were detected after several perfusions, though dramatic changes could not be recognized. EEG abnormalities were improved immediately after LDL-apheresis in one patient. We conclude that LDL-apheresis may affect the serum cholestanol level and clinical manifestations in patients with CTX.

摘要

我们研究了低密度脂蛋白单采术对5例脑腱黄瘤病(CTX)患者生化及临床异常情况的影响。经一次灌注后,胆甾烷醇和胆固醇水平均降至治疗前水平的约60%,并在2周内逐渐恢复至初始水平。经过几次灌注后,观察到临床表现有所改善,跟腱黄瘤有所消退,尽管未见显著变化。1例患者在进行低密度脂蛋白单采术后脑电图异常立即得到改善。我们得出结论,低密度脂蛋白单采术可能会影响CTX患者的血清胆甾烷醇水平及临床表现。

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Treatment of cerebrotendinous xanthomatosis with low-density lipoprotein (LDL)-apheresis.用低密度脂蛋白(LDL)单采术治疗脑腱性黄瘤病。
J Neurol Sci. 1993 Feb;114(2):227-30. doi: 10.1016/0022-510x(93)90303-g.
2
Combined treatment with LDL-apheresis, chenodeoxycholic acid and HMG-CoA reductase inhibitor for cerebrotendinous xanthomatosis.
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[Effect of LDL-apheresis on a case of Cerebrotendinous Xanthomatosis].[低密度脂蛋白单采术对1例脑腱黄瘤病的疗效]
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Normalisation of serum cholestanol concentration in a patient with cerebrotendinous xanthomatosis by combined treatment with chenodeoxycholic acid, simvastatin and LDL apheresis.通过鹅去氧胆酸、辛伐他汀和低密度脂蛋白分离术联合治疗,使脑腱性黄瘤病患者血清胆甾烷醇浓度恢复正常。
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Combined treatment with chenodeoxycholic acid and pravastatin improves plasma cholestanol levels associated with marked regression of tendon xanthomas in cerebrotendinous xanthomatosis.鹅去氧胆酸和普伐他汀联合治疗可改善脑腱性黄瘤病患者的血浆胆甾烷醇水平,并使肌腱黄瘤明显消退。
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Treatment of cerebrotendinous xanthomatosis: effects of chenodeoxycholic acid, pravastatin, and combined use.脑腱性黄瘤病的治疗:鹅去氧胆酸、普伐他汀及联合使用的效果
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Electrophysiological studies in cerebrotendinous xanthomatosis.脑腱黄瘤病的电生理研究
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J Clin Invest. 1973 Nov;52(11):2822-35. doi: 10.1172/JCI107478.
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Monitoring of 7α-hydroxy-4-cholesten-3-one during therapy of cerebrotendinous xanthomatosis: a case report.监测 7α-羟基-4-胆甾烯-3-酮在脑腱黄瘤病治疗中的变化:一例报告。
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Low-density lipoprotein metabolism in cerebrotendinous xanthomatosis.脑腱黄瘤病中的低密度脂蛋白代谢
Metabolism. 1987 Mar;36(3):270-6. doi: 10.1016/0026-0495(87)90187-9.

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