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脂蛋白肾小球病可能为解开肾脂质沉积症的谜团提供关键线索。

Lipoprotein glomerulopathy may provide a key to unlock the puzzles of renal lipidosis.

机构信息

General Medical Research Center, Fukuoka University School of Medicine, Fukuoka, Japan.

Department of Laboratory Medicine, Fukuoka University School of Medicine, Fukuoka, Japan.

出版信息

Kidney Int. 2014 Feb;85(2):243-5. doi: 10.1038/ki.2013.404.

Abstract

Lipoprotein glomerulopathy is an inherited renal disease characterized by unique lipoprotein thrombi in the glomerulus and is associated with the APOE mutation. Hu and colleagues investigated the genetic and clinical features of a large group of patients with lipoprotein glomerulopathy who carried APOE Kyoto, a major APOE variant. Their findings suggest its descent through a founder effect. Fibrate therapy in this group showed favorable results in the patient and renal survival rates.

摘要

脂蛋白肾小球病是一种遗传性肾脏疾病,其特征是肾小球中存在独特的脂蛋白血栓,与 APOE 突变有关。胡和同事研究了携带 APOE Kyoto(一种主要的 APOE 变体)的一大组脂蛋白肾小球病患者的遗传和临床特征。他们的发现表明,它是通过一个奠基者效应而传承下来的。在该组患者中,贝特类药物治疗显示出良好的治疗效果和肾脏存活率。

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