Disappearance of intraglomerular lipoprotein thrombi and marked improvement of nephrotic syndrome by bezafibrate treatment in a patient with lipoprotein glomerulopathy
Disappearance of intraglomerular lipoprotein thrombi and marked improvement of nephrotic syndrome by bezafibrate treatment in a patient with lipoprotein glomerulopathy
复制标题
DOI：
10.1016/s0021-9150(03)00194-1
复制
发表时间：
2003-08-01
期刊：
影响因子：
5.3
通讯作者：
Matsuzawa, Y
中科院分区：
文献类型：
--
作者：
Arai, T;Yamashita, S;Matsuzawa, Y
脂蛋白肾小球病（LPG）是一种以肾小球内脂蛋白血栓形成和血清载脂蛋白E（apo E）升高为特征的遗传性疾病。LPG患者通常表现为肾病综合征，并在一定程度上进展为肾衰竭;然而，尚未建立针对这种疾病的有效治疗方案。我们经历了一个LPG患者，苯扎贝特治疗非常有效。这位30岁的日本女性患有肾病综合征和III型高脂蛋白血症。肾活检显示毛细血管腔明显扩张，内含大量脂蛋白血栓。血浆载脂蛋白E浓度升高至正常对照的2倍。结果表明，该患者为载脂蛋白E2京都（Arg 25 Cys）杂合子。经苯扎贝特（400 mg/d）治疗2年后，患者血浆白蛋白从2.1 mg/dl逐渐升高至4.0 mg/dl，肾小球内脂蛋白血栓几乎完全消失。苯扎贝特降低血浆载脂蛋白E和显着增加高密度脂蛋白（HDL）胆固醇。主要在前B组分中观察到载脂蛋白E降低，而在α组分中未观察到。我们的病人的血脂分析表明，她的脂蛋白血栓的起源可能主要来自前B-脂蛋白和HDL可能参与解决脂蛋白血栓。我们的病例表明，贝特类药物如苯扎贝特可能是解决LPG患者肾小球内血栓和改善肾病综合征的一种新的治疗策略。(C)2003爱思唯尔爱尔兰有限公司保留所有权利。
Lipoprotein glomerulopathy (LPG) is a hereditary disorder characterized by intraglomerular lipoprotein thrombi and increased serum apolipoprotein (apo) E. Patients with LPG usually manifest with nephrotic syndrome, and some progress to renal failure; however, no effective therapeutic regimen has been established for this disease. We experienced a patient with LPG for whom bezafibrate treatment was very effective. This 30-year-old Japanese woman had nephrotic syndrome and type III hyperlipoproteinemia. Renal biopsy showed markedly dilated capillary lumina containing massive lipoprotein thrombi. Plasma apo E concentration was elevated to twice that of normal controls. She was proved to be a heterozygote of apo E2 Kyoto (Arg25Cys). After 2 years treatment with bezafibrate (400 mg/day), her plasma albumin gradually increased from 2.1 to 4.0 mg/dl, and intraglomerular lipoprotein thrombi disappeared almost completely. Bezafibrate decreased plasma apo E and dramatically increased high density lipoprotein (HDL)-cholesterol. The decrease in apo E was observed mainly in the pre-B-fraction, not in the alpha fraction. Lipidological analyses of our patient suggest that the origin her lipoprotein thrombi may be mainly from pre-B-lipoproteins and that HDL might be involved in resolving lipoprotein thrombi. Our case suggests that administration of fibrates such as bezafibrate may be a novel therapeutic strategy for resolving intraglomerular thrombi and improving nephrotic syndrome in patients with LPG. (C) 2003 Elsevier Ireland Ltd. All rights reserved.