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肾脏病与透析肾移植杂志

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双重血浆滤过治疗脂蛋白肾病的疗效及安全性

  

  • 出版日期:2020-04-28 发布日期:2020-05-08

Efficacy of double filtration plasmapheresis therapy on patients with lipoprotein glomerulopathy

  • Online:2020-04-28 Published:2020-05-08

摘要: 目的:观察双重血浆滤过(DFPP)治疗脂蛋白肾病(LPG)患者的临床疗效及安全性。
方法:回顾性分析2010年1月至2016年4月于东部战区总医院国家肾脏疾病临床医学研究中心经肾活检病理确诊为LPG,并行DFPP治疗的患者共17例,比较治疗前后的血清肌酐(SCr)、血浆载脂蛋白E(ApoE)及尿蛋白定量的变化及蛋白尿缓解率,DFPP治疗相关并发症,以及随访中主要临床指标变化。
结果:15例肾活检证实的LPG患者接受了3~10次DFPP治疗。所有患者均接受降脂、降压药物等对症支持治疗。DFPP治疗后,SCr(15558±7691 μmol/L vs 13702±7337  μmol/L,P<001)、ApoE(794±187 mg/dl vs 358±132 mg/dl,P<001)及尿蛋白定量(468±250 g/24h vs 270±220 g/24h,P<001)均较显著下降。其中1例(67%)患者蛋白尿完全缓解,5例(333%)患者蛋白尿部分缓解;4例患者DFPP后行重复肾活检,2例DFPP治疗10次者肾组织脂蛋白栓子几乎完全消失,ApoE染色转阴,分别接受3次及4次DFPP治疗的2例患者肾组织栓子无明显减少。2例(133%)患者因变态反应无法耐受DFPP治疗,部分患者在治疗过程中出现血压下降(10~20 mmHg)及心率增快。中位随访时间为7月(IQR 1~32),随访中患者ApoE水平较DFPP后均有升高。尿蛋白达到部分缓解及完全缓解并随访>12月的4例患者,3例蛋白尿稳定。
结论:DFPP治疗LPG短期疗效明确,总体安全性及患者耐受性较好。

关键词: 脂蛋白肾病, 双重血浆滤过, 载脂蛋白E, 肾活检

Abstract: Objective:To retrospectively observe the clinical efficacy of double fltration plasmapheresis(DFPP) in patients with lipoprotein glomerulopathy(LPG).
Methodology:17 Patients with biopsyproven LPG  who received DFPP treatment in our center  from 2010 to 2016 were involved,clinical parameters before and after DFPP treatment were recorded and analyzed.
Results:Fifteen of the patients underwent 310 courses of DFPP,and 2 patients were unable to tolerate DFPP due to allergic reactions.All patients received fenofibrate or statins and other relevant symptomatic treatments.After shortterm DFPP treatments,serum creatinine(SCr 15558±7691  μmol/L vs 13702±7337  μmol/L,P<001),plasma apolipoprotein E(794±187 mg/dl vs 358±132 mg/dl,P<001)and proteinuria (468±250 g/24h vs 270±220 g/24h,P<001),were relieved significantly.1 patient (67%) achieved complete remission of proteinuria,and 5 patients (333%) achieved partial remission of proteinuria.Four patients underwent repeated renal biopsy after DFPP treatment,and 2 of them who received 10 courses of DFPP were observed disappearment of intraglomerular lipoprotein thrombi,and negative ApoE staining.While 2 patients who  underwent only 3 and 4 courses of DFPP respectively showed no significant pathological improvement.After a median followup of 7 month(IQR 1~32),all patients had elevated ApoE level,4 patients showed an elevation trend of SCr.Three of the four patients who had got partially or completely remission of proteinuria after DFPP tend to be stable after 12 months followup.
Conclusion:Shortterm efficacy of DFPP in patients with LPG was definite.However,Scr and ApoE levels rebounded in some patients during longterm followup.