Cameron J S
Ulster Med J. 1985 Aug;54 Suppl(Suppl):S5-19.
The nephrotic syndrome has emerged over several centuries as the consequence of continued profuse proteinuria, arising in turn from a variety of lesions affecting the glomerulus which impair glomerular ability to retain plasma proteins, in particular, albumin. As a syndrome, it has its own complications and requires its own management irrespective of the underlying lesions. Dissection of these by renal biopsy and by clinical investigation reveals a variety of systemic diseases which affect the kidney, but a majority of primary immune-based diseases appear unique to the glomerulus. Whether the lesion called by Müller and Munk 'nephrosis', and now called minimal change disease and focal segmental glomerulosclerosis is one disease or many, is the subject of intense debate at the moment, as is the relationship between two types of lesion. Only a better understanding of their pathogenesis, and of how the glomerulus normally retains plasma protein, will solve this knotty problem.
肾病综合征在几个世纪以来逐渐形成,是持续大量蛋白尿的结果,而蛋白尿又是由多种影响肾小球的病变引起的,这些病变损害了肾小球保留血浆蛋白(特别是白蛋白)的能力。作为一种综合征,它有其自身的并发症,无论潜在病变如何,都需要进行相应的治疗。通过肾活检和临床研究对这些病变进行剖析,发现有多种全身性疾病会影响肾脏,但大多数原发性免疫性疾病似乎是肾小球特有的。目前,穆勒和蒙克所称的“肾病”(现在称为微小病变病和局灶节段性肾小球硬化症)究竟是一种疾病还是多种疾病,以及这两种病变之间的关系,都在激烈争论中。只有更好地了解它们的发病机制,以及肾小球正常保留血浆蛋白的方式,才能解决这个棘手的问题。