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。