TREATMENT OF FAMILIAL HYPERCHOLESTEROL. BY DEXTRAN SULFATE ADSORPTION/APHERESIS

硫酸葡聚糖选择性去除低密度脂蛋白的研究 亲和力吸附在家族性疾病患者中的评估 高胆固醇血症(FH)。既有纯合子又有杂合子的患者 只要他们的血浆低密度脂蛋白-胆固醇符合治疗条件 浓度超过180毫克/分升，尽管最大限度的药物和饮食 管理层。原型葡聚糖硫酸盐分离系统(Liposorber La-15，Kanegafuchi，日本)去除低密度脂蛋白，而不影响HCL胆固醇或 白蛋白浓度，从而允许自体耗尽的低密度脂蛋白返回 并避免使用含白蛋白的替代溶液。 每两周处理一次半血浆体积。二 纯合子患者完成了为期18周的研究。在这两个人中 在个体中，这些程序使总胆固醇降低了75% 低密度脂蛋白胆固醇水平降低86%。平均术前 低密度脂蛋白-胆固醇水平为237加/减31毫克/分升和手术后 值为34+/-4 mg/dl(n=30)。Lp(A)水平下降82% (47加/减27至8加/减4毫克/分升)。盐酸-胆固醇和白蛋白 浓度不受程序的显著影响。 时间平均的低密度脂蛋白-胆固醇浓度为160毫克/分升，通过 将连续的低密度脂蛋白分离程序之间的间隔保持在两个 几周。手术耐受性很好，没有明显的不良反应。 反应发生了。 我们的数据表明，硫酸葡聚糖吸附是安全有效的 选择性地清除血浆中的低密度脂蛋白-胆固醇的方法，并具有优势， 与简单的血浆交换相比，消除了 胶体替代方案和促进更有益的低密度脂蛋白-HCL 胆固醇比率。

Selective removal of low density lipoprotein (LDL) by dextran sulfate affinity adsorption was evaluated in patients with familial hypercholesterolemia (FH). Patients with both homozygous and heterozygous disease were eligible for treatment as long as their plasma LDL-cholesterol concentrations exceeded 180 mg/dl despite maximal drug and dietary management. The prototype dextran sulfate apheresis system (Liposorber LA-15, Kanegafuchi, Japan) removed LDL without affecting HCL cholesterol or albumin concentrations, thus allowing return of autologous LDL-depleted plasma and avoiding the need for albumin-containing replacement solutions. One and one-half plasma volumes were processed every two weeks. Two homozygous patients completed an 18-week period of study. In these two individuals, the procedures effected a 75% reduction in total cholesterol and an 86% reduction in LDL cholesterol levels. Mean pre-procedure LDL-cholesterol levels were 237 plus/minus 31 mg/dl and post-procedure values were 34 plus/minus 4 mg/dl (n = 30). Lp(a) levels declined by 82% (47 plus/minus 27 to 8 plus/minus 4 mg/dl). HCL-cholesterol and albumin concentrations were not significantly affected by the procedures. Time-averaged LDL-cholesterol concentrations of <160 mg/dl were attained by keeping the interval between successive LDL-apheresis procedures at two weeks. The procedures were very well tolerated and no significant adverse reactions occurred. Our data suggest that dextran sulfate adsorption is a safe and effective way to selectively clear plasma of LDL-cholesterol, and has advantages, when compared to simple plasma exchange, of eliminating the need for colloid replacement solutions and promoting a more beneficial LDL-HCL cholesterol ratio.