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Obstructive nephropathy: lessons from cystic kidney disease.

机译:梗阻性肾病:来自囊性肾病的经验教训。

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Obstructive nephropathy is one of the most important causes of renal failure in infants and children, while polycystic kidney disease (PKD) is a major cause of renal failure in the adult population. This review summarizes the evidence that there may be a number of mechanisms common to the pathophysiology of both conditions. In animal models of obstructive nephropathy and PKD, the renal tubular expression of epidermal growth factor is suppressed, and expression of clusterin is increased, both of which suggest arrested maturation or dedifferentiation of the tubular cell. There is a marked increase in apoptosis of epithelial cells in dilated tubules, associated with an increase in apoptotic stimuli. The renin-angiotensin system is activated in both obstructive nephropathy and PKD, which may contribute to tubular atrophy and interstitial fibrosis, which characterize the progression of both conditions. Focal cystic dilatation of the tubule is found in obstructive nephropathy, while tubular obstruction is present in cystic kidney disease. It is therefore likely that elucidation of the effects of mechanical stretch on renal tubular epithelial cells will contribute to our understanding of both conditions. Copyright 2000 S. Karger AG, Basel
机译:阻塞性肾病是婴儿和儿童肾衰竭的最重要原因之一,而多囊肾疾病(PKD)是成年人口肾衰竭的主要原因。这篇综述总结了两种情况的病理生理共同机制。在阻塞性肾病和PKD的动物模型中,表皮生长因子的肾小管表达受到抑制,而簇蛋白的表达增加,这两者均提示肾小管细胞成熟或去分化停止。扩张的小管中上皮细胞的凋亡明显增加,与凋亡刺激的增加有关。肾素-血管紧张素系统在阻塞性肾病和PKD中均被激活,这可能导致肾小管萎缩和间质纤维化,这是两种疾病的特征。在梗阻性肾病中发现小管的局灶性囊性扩张,而在囊性肾病中则存在肾小管阻塞。因此,很可能阐明机械拉伸对肾小管上皮细胞的作用将有助于我们对这两种情况的理解。版权所有2000 S. Karger AG,巴塞尔

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