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The role of a parental history of Balkan endemic nephropathy in the occurrence of BEN: a prospective study

机译:巴尔干地方性肾病的父母病史在BEN发生中的作用:一项前瞻性研究

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摘要

Balkan endemic nephropathy (BEN) is a chronic kidney disease that affects persons living in the Balkans. Despite the unique geographical specificity of this disease, its etiology has remained unclear. Even if a positive family history of BEN has been identified, it is still uncertain how the disease develops in offspring. In this paper, we examine clinical mechanisms related to the onset of BEN in individuals who have a parental history of BEN to identify early detection of the disease and formulate interventions. We conducted a 5-year prospective study, using markers in years one and three to predict new cases of BEN in year five. New cases of BEN were defined based on three criteria: parental history of BEN, reduced kidney size, and reduced kidney function. Incident cases were divided into (1) probable, (2) definite, and (3) combined labeled total incidence. We evaluated parental history in relation to BEN and tested the potentially intervening effects of kidney length, kidney cortex width, β2-microglobulin, C-reactive protein, and creatinine clearance, using path analyses. The findings of the path analyses suggested that parental history of BEN had both direct and indirect effects. The direct effect was significant for all three modes of parental history (biparental, maternal, and paternal; odds ratios 71.5, 52.3, and 50.1, respectively). The indirect effects of maternal BEN acted via kidney length and creatinine clearance. Biparental BEN was mediated by (1) kidney length and creatinine clearance, and (2) creatinine clearance alone. Paternal BEN had three indirect effects: (1) through kidney length and creatinine clearance, (2) via kidney cortex width and creatinine clearance, and (3) via kidney cortex width only. In conclusion, a family history of BEN led to reduced kidney length and cortex width, and a decline in creatinine clearance, which in turn predicted the onset of BEN.
机译:巴尔干地方性肾病(BEN)是一种慢性肾脏疾病,影响到居住在巴尔干地区的人们。尽管该疾病具有独特的地理特异性,但其病因仍不清楚。即使已鉴定出阳性的BEN家族史,仍不确定该疾病在后代中如何发展。在本文中,我们研究了具有BEN父母史的个体中与BEN发病有关的临床机制,以识别疾病的早期发现并制定干预措施。我们进行了为期5年的前瞻性研究,在第一年和第三年使用标记物预测第五年的BEN新病例。根据以下三个标准定义新的BEN病例:BEN的父母病史,肾脏大小缩小和肾功能下降。突发事件分为(1)可能,(2)确定和(3)组合标记总发生率。我们通过路径分析评估了与BEN相关的父母病史,并测试了肾脏长度,肾皮质宽度,β2-微球蛋白,C反应蛋白和肌酐清除率的潜在干预作用。路径分析的结果表明,BEN的父母病史具有直接和间接影响。对父母的所有三种模式(双亲,母亲和父亲;比值比分别为71.5、52.3和50.1),直接影响是显着的。母体BEN的间接作用是通过肾脏长度和肌酐清除率发挥作用。双亲BEN由(1)肾脏长度和肌酐清除率,以及(2)肌酐清除率单独介导。父亲BEN具有三个间接影响:(1)通过肾脏长度和肌酐清除率;(2)通过肾脏皮质宽度和肌酐清除率;以及(3)仅通过肾脏皮质宽度。总之,BEN的家族病史导致肾脏长度和皮层宽度减少,以及肌酐清除率下降,从而预示了BEN的发作。

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