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中美洲肾病的临床和病理特征:中美洲的一种新的肾脏疾病。

Clinical and pathological characterization of Mesoamerican nephropathy: a new kidney disease in Central America.

机构信息

Division of Renal Medicine, Department of Clinical Sciences, Intervention and Technology, Karolinska Institutet, Stockholm, Sweden.

出版信息

Am J Kidney Dis. 2013 Nov;62(5):908-18. doi: 10.1053/j.ajkd.2013.05.019. Epub 2013 Jul 10.

Abstract

BACKGROUND

An endemic of chronic kidney disease (CKD) of unknown cause among rural inhabitants in Central America has been identified. Young and otherwise healthy men working in plantations are frequently affected. The name Mesoamerican nephropathy (MeN) has been suggested. Clinically, MeN presents with low-grade proteinuria and progressive kidney failure. The renal pathology of this disease has not yet been described.

STUDY DESIGN

Case series.

SETTING & PARTICIPANTS: 8 male patients with CKD of unknown cause and clinically suspected MeN were recruited from a nephrology unit in El Salvador. All recruited patients had been working on plantations. Kidney biopsies, blood, and urine samples were collected.

OUTCOMES & MEASUREMENTS: Renal morphology examined with light microscopy, immunofluorescence, and electron microscopy; clinical and biochemical characteristics.

RESULTS

A similar pattern was seen in all 8 biopsy specimens, with extensive glomerulosclerosis (29%-78%) and signs of chronic glomerular ischemia in combination with tubular atrophy and interstitial fibrosis, but only mild vascular lesions. Electron microscopy indicates podocytic injury. Biochemical workup showed reduced estimated glomerular filtration rate (27-79 mL/min/1.73 m(2) with the CKD Epidemiology Collaboration [CKD-EPI] creatinine equation), low-grade albuminuria, and increased levels of tubular injury biomarkers. Hypokalemia was found in 6 of 8 patients.

LIMITATIONS

Small number of patients from one country.

CONCLUSIONS

This study is the first report of the biochemical and morphologic findings in patients with MeN. Our findings indicate that MeN constitutes a previously unrecognized kidney disease with damage to both glomerular and tubulointerstitial compartments.

摘要

背景

中美洲农村居民中一种原因不明的慢性肾脏病(CKD)流行已被确定。经常受到影响的是在种植园工作的年轻且健康的男性。已经提出了中美洲肾病(MeN)这个名称。临床上,MeN 表现为低水平蛋白尿和进行性肾衰竭。这种疾病的肾脏病理学尚未描述。

研究设计

病例系列。

设置和参与者

从萨尔瓦多的一个肾病科招募了 8 名患有原因不明的 CKD 且临床疑似 MeN 的男性患者。所有被招募的患者都曾在种植园工作。收集了肾脏活检、血液和尿液样本。

结果

所有 8 例活检标本均表现出相似的模式,广泛的肾小球硬化症(29%-78%)和慢性肾小球缺血的迹象,同时伴有肾小管萎缩和间质纤维化,但仅有轻度的血管病变。电子显微镜显示足细胞损伤。生化检查显示估算的肾小球滤过率降低(根据 CKD 流行病学协作组 [CKD-EPI] 肌酐方程为 27-79 mL/min/1.73 m2),低水平白蛋白尿和肾小管损伤生物标志物水平升高。6 例患者中发现低钾血症。

局限性

来自一个国家的患者数量较少。

结论

本研究是首例报道 MeN 患者的生化和形态学发现。我们的研究结果表明,MeN 构成了一种以前未被认识的肾脏疾病,涉及肾小球和肾小管间质两个部分的损伤。

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