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梗阻性肾病:来自多囊肾病的经验教训。

Obstructive nephropathy: lessons from cystic kidney disease.

作者信息

Chevalier R L

机构信息

Department of Pediatrics, University of Virginia, School of Medicine, Charlottesville, VA, USA.

出版信息

Nephron. 2000 Jan;84(1):6-12. doi: 10.1159/000045532.

DOI:10.1159/000045532
PMID:10644902
Abstract

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.

摘要

梗阻性肾病是婴幼儿肾衰竭的最重要原因之一,而多囊肾病(PKD)是成年人群肾衰竭的主要原因。本综述总结了以下证据:这两种疾病的病理生理学可能存在一些共同机制。在梗阻性肾病和PKD的动物模型中,表皮生长因子的肾小管表达受到抑制,而簇集素的表达增加,这两者均提示肾小管细胞成熟停滞或去分化。扩张肾小管中上皮细胞的凋亡显著增加,这与凋亡刺激的增加有关。梗阻性肾病和PKD中肾素-血管紧张素系统均被激活,这可能导致肾小管萎缩和间质纤维化,这是这两种疾病进展的特征。在梗阻性肾病中发现肾小管局灶性囊性扩张,而在囊性肾病中存在肾小管梗阻。因此,阐明机械牵张对肾小管上皮细胞的影响可能有助于我们对这两种疾病的理解。

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