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家族性高胆固醇血症患儿的冠心病。采用低密度脂蛋白分离术进行最大程度治疗

[Coronary heart disease in childhood in familial hypercholesteremia. Maximum therapy with LDL apheresis].

作者信息

Mellwig K P, Schmidt H K, Brettschneider-Meyer A, Meyer H, Jaeger B R, Walli A K, Seidel D, Horstkotte D

机构信息

Kardiologische Klinik, Herz- und Diabeteszentrum Nordrhein-Westfalen, Ruhr-Universität Bochum, Bad Oeynhausen.

出版信息

Internist (Berl). 2003 Apr;44(4):476-80. doi: 10.1007/s00108-002-0832-1.

DOI:10.1007/s00108-002-0832-1
PMID:12914404
Abstract

In children with familial hypercholesterolemia, coronary heart disease requires both medical theraphy and LDL apheresis. We report a case of verified occlusion of the anterior descending branch of the left coronary artery in a 10-year-old patient. The pathological findings revealed by ergometry established the diagnosis. The goal was to achieve the greatest possible reduction of lipid parameters and fibrinogen by lowering plasma viscosity employing LDL apheresis. It is astonishing that this treatment is also well tolerated by children. The basic vascular approaches suffice and shunt operations are not absolutely necessary. The efficacy of this method became vividly apparent by the changes in the skin lesions. Additional angiographic follow-up and further clinical course wil provide information on the usefulness of this treatment strategy with maximum lipid theraphy and the expected improvement in prognosis.

摘要

在患有家族性高胆固醇血症的儿童中,冠心病需要药物治疗和低密度脂蛋白分离术。我们报告一例10岁患者左冠状动脉前降支经证实的闭塞病例。运动试验揭示的病理结果确立了诊断。目标是通过低密度脂蛋白分离术降低血浆粘度,尽可能最大程度地降低脂质参数和纤维蛋白原。令人惊讶的是,这种治疗儿童也能很好耐受。基本的血管治疗方法就足够了,分流手术并非绝对必要。这种方法的疗效通过皮肤病变的变化清晰显现。额外的血管造影随访和进一步的临床病程将提供关于这种采用最大程度脂质治疗的治疗策略的有效性以及预期预后改善情况的信息。

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Internist (Berl). 2003 Apr;44(4):476-80. doi: 10.1007/s00108-002-0832-1.
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本文引用的文献

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Improvement of coronary vasodilatation capacity through single LDL apheresis.通过单次低密度脂蛋白去除术改善冠状动脉血管舒张能力。
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LDL apheresis in a homozygous familial hypercholesterolemic child aged 4.5.一名4.5岁纯合子家族性高胆固醇血症儿童的低密度脂蛋白单采术
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HELP-LDL 单采多中心研究,一项关于 LDL 单采在冠心病二级预防中作用的血管造影评估试验。II. 定期治疗对血浆 LDL 胆固醇浓度及冠心病病程影响的最终评估。HELP 研究组。肝素诱导的体外 LDL 沉淀。
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Low-density lipoprotein apheresis as long-term treatment for children with homozygous familial hypercholesterolemia.低密度脂蛋白单采术作为纯合子家族性高胆固醇血症患儿的长期治疗方法。
J Pediatr. 1995 May;126(5 Pt 1):728-35. doi: 10.1016/s0022-3476(95)70400-0.
5
Treatment of homozygous and double heterozygous familial hypercholesterolemic children with LDL-apheresis.采用低密度脂蛋白去除法治疗纯合子和双杂合子家族性高胆固醇血症儿童。
Int J Artif Organs. 1995 Feb;18(2):103-10.
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A simple specific method for precipitation of low density lipoproteins.一种用于沉淀低密度脂蛋白的简单特异性方法。
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Cardiovascular features of homozygous familial hypercholesterolemia: analysis of 16 patients.纯合子家族性高胆固醇血症的心血管特征:16例患者分析
Am J Cardiol. 1984 Jul 1;54(1):20-30. doi: 10.1016/0002-9149(84)90298-4.
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Improved haemorheology associated with a reduction in plasma fibrinogen and LDL in patients being treated by heparin-induced extracorporeal LDL precipitation (HELP).在接受肝素诱导的体外低密度脂蛋白沉淀(HELP)治疗的患者中,血液流变学改善与血浆纤维蛋白原和低密度脂蛋白降低有关。
Eur J Clin Invest. 1989 Feb;19(1):30-7. doi: 10.1111/j.1365-2362.1989.tb00192.x.
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Receptor mediated uptake of apo B and apo E rich lipoproteins by human glomerular epithelial cells.人类肾小球上皮细胞通过受体介导摄取富含载脂蛋白B和载脂蛋白E的脂蛋白。
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