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[通过全血浆置换和选择性去除低密度脂蛋白(低密度脂蛋白单采术)治疗重度家族性高胆固醇血症的疗效比较]

[Comparison between treatments of severe forms of familial hypercholesterolemia by total plasma exchange and selective removal of low density lipoproteins (LDLapheresis)].

作者信息

Dairou F, Rottembourg J, de Gennes J L, Assogba U, Bruckert E, Jacobs C, Truffert J

机构信息

Service d'Endocrinologie, Hôpital de la Pitié, Paris.

出版信息

Presse Med. 1988 Oct 1;17(33):1679-82.

PMID:2973030
Abstract

A plasma exchange program for familial hypercholesterolaemia was started in 1982. Ten patients aged from 7 to 58 years were progressively included: 3 had an heterozygous form of the disease with ischaemic heart disease; 3 had an homozygous form with defective low density lipoprotein receptor activity, 4 had a receptor-negative homozygous familial hypercholesterolaemia and had previously undergone portacaval shunt. During total plasma exchange against human albumin (470 sessions in 9 patients) low density lipoprotein cholesterol values, but also high density lipoprotein cholesterol values, decreased by 40 per cent. More recently, 5 patients had selective low density lipoprotein absorption on dextran sulfate column (Liposorber); 90 exchanges were performed. High density lipoprotein cholesterol values decreased by 55 per cent and high density lipoprotein cholesterol values by only 27 per cent. The patients' attitude to treatment was excellent, with less fatigue and better compliance.

摘要

1982年启动了一项针对家族性高胆固醇血症的血浆置换计划。逐渐纳入了10名年龄在7至58岁之间的患者:3名患有杂合子形式的疾病并伴有缺血性心脏病;3名患有纯合子形式且低密度脂蛋白受体活性有缺陷;4名患有受体阴性的纯合子家族性高胆固醇血症,且此前接受过门腔分流术。在用人白蛋白进行全血浆置换期间(9名患者共进行了470次置换),低密度脂蛋白胆固醇值以及高密度脂蛋白胆固醇值均下降了40%。最近,5名患者在硫酸葡聚糖柱(Liposorber)上进行了选择性低密度脂蛋白吸附;共进行了90次置换。高密度脂蛋白胆固醇值下降了55%,而低密度脂蛋白胆固醇值仅下降了27%。患者对治疗的态度很好,疲劳感减轻且依从性更佳。

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