MANAGEMENT OF FAMILIAL HYPERCHOLESTEROLEMIA BY SECONDARY MEMBRANE FILTRATION

使用二级中空纤维膜装置的选择性血浆过滤 （Kuraray EVAL 4A过滤器）作为血浆替代品进行了研究 纯合子家族性白血病患者的长期治疗中的交换 高胆固醇血症过滤器的孔隙率允许较小的 血浆溶质，如高密度脂蛋白（HCL）和白蛋白，而 保留较大的分子，如LDL。患者血浆分离， 使用柠檬酸盐抗凝剂的连续流离心，保持在 在37 ° C下，并在25-30 ml/min下通过膜过滤， 再输注每2小时以这种方式处理4升血浆。 周无需更换液体。过滤器去除9.74 每次手术的胆固醇加/减0.51 g（平均过滤前 胆固醇547 ± 23 mg/dl，滤过后胆固醇134 ± 8.0 mg/dl，n=20）。以下平均下降也是 根据程序观察到：HCL胆固醇，56%;白蛋白，30%;纤维蛋白原， IgM阳性率为78%，IgG阳性率为48%。平均跨膜筛分系数 白蛋白（过滤器输出/过滤器输入）为0.87，总胆固醇为0.87， n.11，HCL胆固醇为0.44。 我们的数据表明，二级在线膜过滤是一种安全， 有效的方法来选择性地清除血浆中的低密度脂蛋白胆固醇，并增加了 与血浆置换相比， 胶体置换液和促进降低LDL/HCL血浆胆固醇 比例 这项工作是与MDB-NHLBI合作完成的，作为 方案编号76-H-74

Selective plasma filtration using a secondary hollow fiber membrane device (Kuraray EVAL 4A filter) was investigated as an alternative to plasma exchange in the long term therapy of a patient with homozygous familial hypercholesterolemia. The porosity of the filter allows passage of smaller plasma solutes such as high density lipoprotein (HCL) and albumin, while retaining larger molecules such as LDL. Patient plasma was separated by continuous-flow centrifugation using citrate anticoagulant, kept at 37degrees C, and filtered through the membrane at 25-30 ml/min prior to reinfusion. Four liters of plasma were processed in this manner every 2 weeks. No replacement fluid was necessary. The filter removed 9.74 plus/minus 0.51 gms of cholesterol per procedure (mean prefiltration cholesterol 547 plus/minus 23 mg/dl, postfiltration cholesterol 134 plus/minus 8.0 mg/dl, n=20). The following mean decreases were also observed per procedure: HCL cholesterol, 56%; albumin, 30%; fibrinogen, 74%,; IgM, 78%; and IgG, 48%. The mean transmembrane sieving coefficient (filter output/filter input) for albumin was 0.87, for total cholesterol n.11, and for HCL cholesterol 0.44. Our data suggest that secondary on-line membrane filtration is a safe and effective way to selectively clear plasma of LDL cholesterol, and has added advantages, when compared to plasma exchange, of eliminating the need for colloid replacement fluid and promoting a lower LDL/HCL plasma cholesterol ratio. This work was done in collaboration with MDB-NHLBI, as an addendum to Protocol #76-H-74.