Renal Morphology, Clinical Findings, and Progression Rate in Mesoamerican Nephropathy

Renal Morphology, Clinical Findings, and Progression Rate in Mesoamerican Nephropathy
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DOI:
10.1053/j.ajkd.2016.10.036
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发表时间:
2017-05-01
影响因子:
13.2
通讯作者:
Wernerson, Annika
Wernerson, Annika
中科院分区:
医学1区
文献类型:
--
作者:
Wijkstrom, Julia;Gonzalez-Quiroz, Marvin;Wernerson, Annika

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背景:中美洲肾病(Mesoamerican nephropathy, MeN)是一种影响中美洲农村居民的慢性肾病。我们先前描述了8例萨尔瓦多患者的肾脏形态。为了确认肾脏病理,我们研究了尼加拉瓜男性患者的肾脏活检。随访收集两组活检的尿液和血液样本,以调查自然病史。研究设计:病例系列。背景和参与者:在肾活检研究中,对尼加拉瓜19名疑似男性的甘蔗工人进行了问卷调查、肾活检、血液和尿液分析。纳入标准为年龄20 - 65岁,血浆肌酐水平1.13 - 2.49 mg/dL或肾小球滤过率(eGFR) 30 - 80 mL/min/1.73 m(2)。排除标准为蛋白尿,蛋白质排泄量为3 g/24 h,未控制的高血压,糖尿病或其他已知的肾脏疾病。在随访研究中,分别在活检后1 - 1.5年和2 - 2.5年收集尼加拉瓜肾活检研究(n = 18)和我们之前在萨尔瓦多对男性病例进行的活检研究(n = 7)的血液和尿液。结果:肾脏形态学,临床和生化特征,每年eGFR的变化。测量方法:使用CKD-EPI肌酐(eGFR(cr))、胱抑素C (eGFR(cys))和肌酐-胱抑素C (eGFR(cr-cys))方程计算eGFR。结果:在肾活检研究中,参与者的平均eGFR(cr)为57(范围,33-96)mL/min/1.73 m(2)。47%的患者血浆钠含量低,21%的患者血浆钾含量低。16例肾活检具有代表性,显示肾小球硬化(平均38%)、肾小球肥大和慢性肾小球缺血的迹象。可见轻至中度的小管间质损伤和大多数轻微的血管改变。在随访研究中,中位随访时间为13个月(范围13-27)。eGFR(cr)的平均变化为-4.4 +/- 8.4(范围:-27.7至10.2)mL/min/1.73 m(2) /年。大多数患者已经停止了甘蔗种植。局限性:3例活检标本肾小球不超过4个。结论:本研究证实了男性的肾脏形态:慢性肾小球和小管间质损伤伴肾小球硬化和慢性肾小球缺血。随访数据显示,egfr平均恶化。(C) 2016年作者。由爱思唯尔公司代表国家肾脏基金会出版。这是一篇基于CC BY-NC-ND许可的开放获取文章。org/licenses/by-nc-nd/4.0/)。
Background: Mesoamerican nephropathy (MeN) is a chronic kidney disease affecting rural inhabitants in Central America. We have previously described the renal morphology in 8 patients from El Salvador. To confirm the renal pathology, we have studied kidney biopsies from patients with MeN in Nicaragua. Follow-up urine and blood samples from both biopsy studies were collected to investigate the natural history.Study Design: Case series.Settings & Participants: In the kidney biopsy study, 19 male sugarcane workers in Nicaragua with suspected MeN were investigated with questionnaires, kidney biopsies, and blood and urine analysis. Inclusion criteria were age 20 to 65 years and plasma creatinine level of 1.13 to 2.49 mg/dL or estimated glomerular filtration rate (eGFR) of 30 to 80 mL/min/1.73 m(2). Exclusion criteria were proteinuria with protein excretion >3 g/24 h, uncontrolled hypertension, diabetes mellitus, or other known kidney disease. In the follow upstudy, blood and urine from the kidney biopsy study in Nicaragua (n = 18) and our previous biopsy study of MeN cases in El Salvador (n = 7) were collected 1 to 1.5 and 2 to 2.5 years after biopsy, respectively.Outcomes: Renal morphology, clinical, and biochemical characteristics, change in eGFR per year.Measurements: eGFR was calculated using the CKD-EPI creatinine (eGFR(cr)), cystatin C (eGFR(cys)), and creatinine-cystatin C (eGFR(cr-cys)) equations.Results: In the kidney biopsy study, participants had a mean eGFR(cr) of 57 (range, 33-96) mL/min/1.73 m(2). 47% had low plasma sodium and 21% had low plasma potassium levels. 16 kidney biopsies were representative and showed glomerulosclerosis (mean, 38%), glomerular hypertrophy, and signs of chronic glomerular ischemia. Mild to moderate tubulointerstitial damage and mostly mild vascular changes were seen. In the follow up-study, median duration of follow-up was 13 (range, 13-27) months. Mean change in eGFR(cr) was -4.4 +/- 8.4 (range, -27.7 to 10.2) mL/min/1.73 m(2) per year. Most patients had stopped working with sugarcane cultivation.Limitations: 3 biopsy specimens had 4 or fewer glomeruli.Conclusions: This study confirms the renal morphology of MeN: chronic glomerular and tubulointerstitial damage with glomerulosclerosis and chronic glomerular ischemia. Follow-up data show that eGFRs, on average, deteriorated. (C) 2016 The Authors. Published by Elsevier Inc. on behalf of the National Kidney Foundation, Inc. This is an open access article under the CC BY-NC-ND license (http:// creativecommons. org/licenses/by-nc-nd/4.0/).