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一例载脂蛋白E京都突变型脂蛋白肾小球病的五年随访

Five-year follow-up of a case of lipoprotein glomerulopathy with APOE Kyoto mutation.

作者信息

Usui Ryosuke, Takahashi Masaki, Nitta Kosaku, Koike Minako

机构信息

Division of Nephrology, Department of Medicine, Yachiyo Medical Center, Tokyo Women's Medical University, 477-96, Yachiyo, Chiba, Japan.

Division of Nephrology, Minami-Senju Hospital, Tokyo, Japan.

出版信息

CEN Case Rep. 2016 Nov;5(2):148-153. doi: 10.1007/s13730-016-0214-5. Epub 2016 Mar 4.

Abstract

We report the case of a 34-year-old Japanese male with lipoprotein glomerulopathy (LPG). Renal biopsy showed LPG, and followed by a genetic analysis revealed a mutation in apolipoprotein E gene (APOE Kyoto; Arg25Cys). We started treatment with probucol, bezafibrate, losartan, and allopurinol. Urinary protein decreased in response to treatment but has remained at about 1.27 ± 0.71 g/gCr, and a repeat biopsy which was performed 1 year after the first biopsy showed no clear evidence of pathological remission and complication of other glomerular disease. After 5 years of follow-up after the start of treatment, renal function has almost maintained without apparent deterioration. Interestingly, the course of the urinary protein level closely paralleled his triglyceride and cholesterol levels in a long-term. This observation suggests the importance of tight control of lipid profiles as a means of renoprotection in LPG patient.

摘要

我们报告了一例34岁的日本男性脂蛋白肾小球病(LPG)患者。肾活检显示为LPG,随后的基因分析发现载脂蛋白E基因(APOE京都型;Arg25Cys)存在突变。我们开始使用普罗布考、苯扎贝特、氯沙坦和别嘌醇进行治疗。治疗后尿蛋白减少,但仍维持在约1.27±0.71 g/gCr,首次活检1年后进行的重复活检未显示明确的病理缓解证据及其他肾小球疾病并发症。治疗开始后随访5年,肾功能几乎维持稳定,无明显恶化。有趣的是,长期来看,尿蛋白水平的变化过程与他的甘油三酯和胆固醇水平密切平行。这一观察结果表明,严格控制血脂水平对于LPG患者肾脏保护具有重要意义。

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