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Integrative Genomics Identifies Novel Associations with APOL1 Risk Genotypes in Black NEPTUNE Subjects.整合基因组学揭示黑人NEPTUNE研究对象中与APOL1风险基因型的新关联。
J Am Soc Nephrol. 2016 Mar;27(3):814-23. doi: 10.1681/ASN.2014111131. Epub 2015 Jul 6.
2
Clinical Features and Histology of Apolipoprotein L1-Associated Nephropathy in the FSGS Clinical Trial.FSGS临床试验中载脂蛋白L1相关肾病的临床特征与组织学
J Am Soc Nephrol. 2015 Jun;26(6):1443-8. doi: 10.1681/ASN.2013111242. Epub 2015 Jan 8.
3
Patient age and the prognosis of idiopathic membranous nephropathy.患者年龄与特发性膜性肾病的预后
PLoS One. 2014 Oct 20;9(10):e110376. doi: 10.1371/journal.pone.0110376. eCollection 2014.
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Pattern of glomerular disease in the Saudi population: a single-center, five-year retrospective study.沙特人群肾小球疾病模式:一项单中心、五年回顾性研究。
Saudi J Kidney Dis Transpl. 2013 Nov;24(6):1265-70. doi: 10.4103/1319-2442.121288.
5
Urinary IgG and α2-macroglobulin are powerful predictors of outcome and responsiveness to steroids and cyclophosphamide in idiopathic focal segmental glomerulosclerosis with nephrotic syndrome.尿 IgG 和α2-巨球蛋白是特发性局灶节段性肾小球硬化伴肾病综合征对激素和环磷酰胺反应和预后的强有力预测指标。
Biomed Res Int. 2013;2013:941831. doi: 10.1155/2013/941831. Epub 2013 Sep 4.
6
Digital pathology evaluation in the multicenter Nephrotic Syndrome Study Network (NEPTUNE).多中心肾病综合征研究网络(NEPTUNE)中的数字病理学评估。
Clin J Am Soc Nephrol. 2013 Aug;8(8):1449-59. doi: 10.2215/CJN.08370812. Epub 2013 Feb 7.
7
Japan Renal Biopsy Registry and Japan Kidney Disease Registry: Committee Report for 2009 and 2010.日本肾活检登记处和日本肾脏病登记处:2009 年和 2010 年委员会报告。
Clin Exp Nephrol. 2013 Apr;17(2):155-73. doi: 10.1007/s10157-012-0746-8. Epub 2013 Feb 6.
8
Design of the Nephrotic Syndrome Study Network (NEPTUNE) to evaluate primary glomerular nephropathy by a multidisciplinary approach.采用多学科方法评估原发性肾小球疾病的肾病综合征研究网络(NEPTUNE)的设计。
Kidney Int. 2013 Apr;83(4):749-56. doi: 10.1038/ki.2012.428. Epub 2013 Jan 16.
9
Missense mutations in the APOL1 gene are highly associated with end stage kidney disease risk previously attributed to the MYH9 gene.APOL1 基因中的错义突变与之前归因于 MYH9 基因的终末期肾病风险高度相关。
Hum Genet. 2010 Sep;128(3):345-50. doi: 10.1007/s00439-010-0861-0. Epub 2010 Jul 16.
10
A new equation to estimate glomerular filtration rate.一种估算肾小球滤过率的新公式。
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肾病综合征研究网络中的完全缓解

Complete Remission in the Nephrotic Syndrome Study Network.

作者信息

Gipson Debbie S, Troost Jonathan P, Lafayette Richard A, Hladunewich Michelle A, Trachtman Howard, Gadegbeku Crystal A, Sedor John R, Holzman Lawrence B, Moxey-Mims Marva M, Perumal Kalyani, Kaskel Frederick J, Nelson Peter J, Tuttle Katherine R, Bagnasco Serena M, Hogan Marie C, Dell Katherine M, Appel Gerald B, Lieske John C, Ilori Titilayo O, Sethna Christine B, Fervenza Fernando C, Hogan Susan L, Nachman Patrick H, Rosenberg Avi Z, Greenbaum Larry A, Meyers Kevin E C, Hewitt Stephen M, Choi Michael J, Kopp Jeffrey B, Zhdanova Olga, Hodgin Jeffrey B, Johnstone Duncan B, Adler Sharon G, Avila-Casado Carmen, Neu Alicia M, Hingorani Sangeeta R, Lemley Kevin V, Nast Cynthia C, Brady Tammy M, Barisoni-Thomas Laura, Fornoni Alessia, Jennette J Charles, Cattran Daniel C, Palmer Matthew B, Gibson Keisha L, Reich Heather N, Mokrzycki Michele H, Sambandam Kamalanathan K, Zilleruelo Gaston E, Licht Christoph, Sampson Matthew G, Song Peter, Mariani Laura H, Kretzler Matthias

机构信息

Due to the number of contributing authors, the affiliations are provided in the Supplemental Material.

出版信息

Clin J Am Soc Nephrol. 2016 Jan 7;11(1):81-9. doi: 10.2215/CJN.02560315. Epub 2015 Dec 10.

DOI:10.2215/CJN.02560315
PMID:26656320
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4702222/
Abstract

BACKGROUND AND OBJECTIVES

This analysis from the Nephrotic Syndrome Study Network (NEPTUNE) assessed the phenotypic and pathology characteristics of proteinuric patients undergoing kidney biopsy and defined the frequency and factors associated with complete proteinuria remission (CRever).

DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We enrolled adults and children with proteinuria ≥0.5 g/d at the time of first clinically indicated renal biopsy at 21 sites in North America from April 2010 to June 2014 into a prospective cohort study. NEPTUNE central pathologists assigned participants to minimal-change disease (MCD), FSGS, membranous nephropathy, or other glomerulopathy cohorts. Outcome measures for this analysis were (1) CRever with urine protein-to-creatinine ratio (UPC) <0.3 g/g with preserved native kidney function and (2) ESRD. Continuous variables are reported as median and interquartile range (IQR; 25th, 75th percentile). Cox proportional hazards modeling was used to assess factors associated with CRever.

RESULTS

We enrolled 441 patients: 116 (27%) had MCD, 142 (32%) had FSGS, 66 (15%) had membranous nephropathy, and 117 (27%) had other glomerulopathy. The baseline UPC was 4.1 g/g (IQR, 1.9, 7.7) and the eGFR was 81 ml/min per 1.73 m(2) (IQR, 50, 105). Median duration of observation was 19 months (IQR, 11, 30). CRever occurred in 46% of patients, and 4.6% progressed to ESRD. Multivariate analysis demonstrated that higher prebiopsy proteinuria (hazard ratio, 0.3; 95% confidence interval, 0.2 to 0.5) and pathology diagnosis (FSGS versus MCD; hazard ratio, 0.2; 95% confidence interval, 0.1 to 0.5) were inversely associated with CRever. The effect of immunosuppressive therapy on remission varied by pathology diagnosis.

CONCLUSIONS

In NEPTUNE, the high frequency of other pathology in proteinuric patients affirms the value of the diagnostic kidney biopsy. Clinical factors, including level of proteinuria before biopsy, pathology diagnosis, and immunosuppression, are associated with complete remission.

摘要

背景与目的

来自肾病综合征研究网络(NEPTUNE)的这项分析评估了接受肾活检的蛋白尿患者的表型和病理特征,并确定了与蛋白尿完全缓解(CRever)相关的频率及因素。

设计、地点、参与者及测量指标:2010年4月至2014年6月期间,我们将北美21个地点首次临床指征肾活检时蛋白尿≥0.5g/d的成人和儿童纳入一项前瞻性队列研究。NEPTUNE中心病理学家将参与者分配至微小病变病(MCD)、局灶节段性肾小球硬化(FSGS)、膜性肾病或其他肾小球病队列。该分析的结局指标为:(1)尿蛋白肌酐比(UPC)<0.3g/g且保留天然肾功能的蛋白尿完全缓解(CRever);(2)终末期肾病(ESRD)。连续变量以中位数和四分位数间距(IQR;第25、75百分位数)报告。采用Cox比例风险模型评估与CRever相关的因素。

结果

我们纳入了441例患者:116例(27%)患有MCD,142例(32%)患有FSGS,66例(15%)患有膜性肾病,117例(27%)患有其他肾小球病。基线UPC为4.1g/g(IQR,1.9,7.7),估算肾小球滤过率(eGFR)为81ml/(min·1.73m²)(IQR,50,105)。中位观察期为19个月(IQR,11,30)。46%的患者出现CRever,4.6%进展为ESRD。多因素分析表明,活检前蛋白尿水平较高(风险比,0.3;95%置信区间,0.2至0.5)和病理诊断(FSGS与MCD相比;风险比,0.2;95%置信区间,0.1至0.5)与CRever呈负相关。免疫抑制治疗对缓解的影响因病理诊断而异。

结论

在NEPTUNE研究中,蛋白尿患者中其他病理类型的高频率证实了诊断性肾活检的价值。临床因素,包括活检前蛋白尿水平、病理诊断和免疫抑制,与完全缓解相关。