Matsell D G
Department of Pediatrics, University of Western Ontario, London, Canada.
Am J Kidney Dis. 1998 Oct;32(4):535-43. doi: 10.1016/s0272-6386(98)70014-9.
Renal dysplasia is a clinically important consequence of abnormal nephrogenesis. Various forms are encountered in clinical practice; however, renal dysplasia may represent the final common end point of defects in the normal cascade of fetal kidney development. Typical histopathologic changes characterize renal dysplasia, including architectural distortion, metaplasia, and primitive glomeruli and tubules. Cystic changes are not universal but can be found in most situations. The advent of recent molecular techniques, including gene targeting and positional cloning, has expanded our knowledge of the molecular control of normal mammalian nephrogenesis and with it our understanding of the pathogenesis of renal dysplasia. A defect in the ability of the branching ureteric duct and the undifferentiated metanephric blastema to communicate appears to be the basic underlying principle for the formation of dysplasia. Mutation, defective regulation of transcription, and alteration in spatial or temporal expression of a number of classes of genes, including growth factors, have been implicated in the development of renal dysplasia. Numerous examples, both experimental and in nature, highlight this point.
肾发育异常是肾发生异常的一个临床重要后果。临床实践中会遇到多种形式；然而，肾发育异常可能代表了胎儿肾脏正常发育级联缺陷的最终共同终点。典型的组织病理学变化是肾发育异常的特征，包括结构扭曲、化生以及原始肾小球和肾小管。囊性改变并不普遍，但在大多数情况下都能发现。包括基因靶向和定位克隆在内的近期分子技术的出现，扩展了我们对正常哺乳动物肾发生分子控制的认识，也增进了我们对肾发育异常发病机制的理解。分支输尿管导管与未分化的后肾胚基之间沟通能力的缺陷似乎是发育异常形成的基本潜在原理。多种基因（包括生长因子）的突变、转录调控缺陷以及空间或时间表达改变都与肾发育异常的发生有关。实验和自然界中的众多例子都突出了这一点。
