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陈宇,陈若如,黄蔚霞.临床表现为肾病综合征的特发性膜性肾病的临床和病理特点以及预后不良的因素分析[J].浙江中西医结合杂志,2022,32(11):
临床表现为肾病综合征的特发性膜性肾病的临床和病理特点以及预后不良的因素分析
Clinical and pathological features of idiopathic membranous nephropathy presenting as nephrotic syndrome and the factors of poor prognosis
投稿时间:2021-11-30  修订日期:2022-10-19
DOI:
中文关键词:  特发性膜性肾病  肾病综合征  临床  病理  预后
英文关键词:Idiopathic membranous nephropathy  Nephrotic syndrome  clinical  pathology  prognosis
基金项目:
作者单位E-mail
陈宇 温州市中医院 friendcy@139.com 
陈若如 温州市中医院  
黄蔚霞* 温州市中医院 13587662899@139.com 
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中文摘要:
      目的 通过比较有无临床表现为肾病综合征的特发性膜性肾病(IMN)患者肾穿刺基线时的临床病理资料,探讨预后相关的因素。方法 回顾性分析2006年9月至2019年11月浙江省温州市中医院经肾活检确诊为IMN并有完整随访资料的患者共345例,根据患者有无临床表现为肾病综合征分为有肾病综合征组202例和无肾病综合征组143例,比较两组间肾穿刺基线时的临床、病理资料,并对两组的预后进行分析。主要结局:血肌酐翻倍或全因死亡或进展至终末期肾病(ESRD);次要结局:完全缓解(CR)和部分缓解(PR)。结果 有肾病综合征组男性居多,更多合并浮肿(91.1%)和血尿(45.0%),收缩压和舒张压偏高,病程更短,白蛋白、血IgG更低,尿蛋白更多,纤维蛋白原、血肌酐、LDL更高,病理程度更重,系膜基质增生、间质炎性细胞浸润、肾小管萎缩、肾脏C3沉积都更严重,与无肾病综合征组相比有统计学差异(P<0.05)。Cox多因素回归分析发现年龄、血补体C3、BUN、Scr、UA、TG、eGFR是肾脏预后不良的危险因素,而高血压、尿蛋白、血沉、肾脏IgA沉积等是临床缓解的预后因素。采用 Kaplan-Meier 法绘制肾脏生存曲线,比较有无肾病综合征两组间主要终点事件和次要终点事件的差异。发现有肾病综合征组较无肾脏综合征组累积肾脏存活率更低,更容易发生不良结局(P=0.0018),而次要终点事件的发生无统计学差异(P=0.9104)。结论 肾穿刺基线时临床表现为肾病综合征的IMN患者临床病情和病理程度均较无肾病综合征组更重,年龄、血尿素氮、血肌酐、血尿酸、eGFR、甘油三酯是肾脏不良结局的独立危险因素,临床表现为肾病综合征的IMN患者预后更差,更容易进展至ESRD。
英文摘要:
      Objective: To compare the clinicopathological data at baseline of renal biopsy in patients with idiopathic membranous nephropathy (IMN) with or without nephrotic syndrome and to explore prognostic factors. Methods A total of 345 patients with IMN confirmed by renal biopsy and have complete follow-up data from September 2006 to November 2019 in Wenzhou Traditional Chinese Medicine Hospital in Zhejiang Province were retrospectively analyzed. According to the presence or absence of nephrotic syndrome, the patients were divided into two groups: 202 cases with nephrotic syndrome group and 143 cases without nephrotic syndrome group. The clinical and pathological data at baseline of renal biopsy were compared between the two groups, and the prognosis of the two groups was analyzed. Main Outcomes: Serum creatinine doubling or all-cause death or progression to end-stage renal disease (ESRD); Secondary outcomes: complete remission (CR) and partial remission (PR). Results With nephrotic syndrome group have more male, more presenting as edema (91.1%) and hematuria (45.0%), higher systolic and diastolic blood pressure, shorter course of disease, lower albumin and serum IgG, more urinary protein, higher fibrinogen, serum creatinine and LDL, and more serious pathological degree. The mesangial matrix hyperplasia, interstitial inflammatory cell infiltration, renal tubule atrophy, and renal C3 deposition were more severe than those in the group without nephrotic syndrome (P<0.05). Cox multivariate regression analysis showed that age, blood complement C3, BUN, Scr, UA, TG and eGFR were risk factors for poor renal prognosis, while hypertension, urinary protein, ESR and renal IgA deposition were prognostic factors for clinical remission. Kidney survival curves were plotted by Kaplan-Meier method to compare the differences in primary and secondary end events between the two groups with and without nephrotic syndrome. It was found that the nephrotic syndrome group had a lower cumulative renal survival rate and was more prone to adverse outcomes than the non-nephrotic syndrome group (P=0.0018), while there was no statistically significant difference in the occurrence of secondary end events (P=0.9104). Conclusion The clinical condition and pathological degree of IMN patients with nephrotic syndrome at baseline of renal puncture were more severe than those without nephrotic syndrome. Age, blood urea nitrogen, blood creatinine, blood uric acid, eGFR and triglyceride were independent risk factors for adverse renal outcomes. IMN patients presenting as nephrotic syndrome had a worse prognosis and were more likely to progress to ESRD.
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