Yokoyama S, Yamamoto A, Hayashi R, Satani M
Department of Etiology and Pathophysiology, National Cardiovascular Center, Osaka, Japan.
Jpn Circ J. 1987 Sep;51(9):1116-22. doi: 10.1253/jcj.51.1116.
Nine patients with familial hypercholesterolemia (FH), 6 with homozygotes and 3 with heterozygotes, were treated with long term repetitive LDL-apheresis. The techniques are simple plasma exchange with human albumin solution, double membrane filtration, and selective LDL-adsorption by dextran sulfate-cellulose gel. The average term was 3.5 years except for the two homozygotes for whom the treatment was only initiated in our facility. Plasma total cholesterol levels were controlled between pretreating level, 320 to 500 mg/dl, and posttreating level, 100 to 160 mg/dl, by biweekly treatments. All patients showed remarkable improvement of cutaneous and tendinous xanthomas. One homozygous patient died at 31 years old of myocardial infarction after 2 years of treatment. A homozygous patient who has been treated since 5 years old for 6 years was reexamined by angiography and was shown to have atheromatous lesions regressed in the aortic valve region and in the left renal artery.
9例家族性高胆固醇血症（FH）患者，其中6例为纯合子，3例为杂合子，接受了长期重复性低密度脂蛋白清除术治疗。技术包括用人白蛋白溶液进行简单的血浆置换、双重膜过滤以及用硫酸葡聚糖 - 纤维素凝胶选择性吸附低密度脂蛋白。除了在我们机构才开始治疗的两名纯合子患者外，平均治疗时间为3.5年。通过每两周一次的治疗，血浆总胆固醇水平控制在治疗前水平（320至500mg/dl）和治疗后水平（100至160mg/dl）之间。所有患者的皮肤和肌腱黄色瘤均有显著改善。一名纯合子患者在治疗2年后，31岁时死于心肌梗死。一名自5岁起接受治疗6年的纯合子患者经血管造影复查，显示主动脉瓣区域和左肾动脉的动脉粥样硬化病变有所消退。
