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IgA肾病:有哪些新进展?

IgA nephropathy: what's new?

作者信息

Hogg Ronald J

出版信息

Pediatr Nephrol. 2007 Nov;22(11):1809-14. doi: 10.1007/s00467-007-0515-1. Epub 2007 Jul 10.

Abstract

Although IgA nephropathy has only been recognized as a definitive entity for fewer than 40 years, its place in the world as a prominent cause for progressive kidney disease is well established. The extent to which we understand the role of genetically derived abnormal forms of the IgA molecule in the disease is evolving, and this will, hopefully, translate into more specific modes of treatment for patients in the future. In the meantime, we have few specific therapeutic options, most of which have not been well studied in large numbers of patients. The extent to which we can define which patients are likely to progress--and hence should be considered candidates for treatment--will be discussed in this Commentary. In addition, the notion that some patients may have reached "the point of no return" will also be addressed. Unfortunately, most of the comments will be based on results obtained in studies conducted in adults--a situation that is very familiar to pediatric nephrologists.

摘要

尽管IgA肾病被确认为一种明确的疾病实体还不到40年,但它作为进行性肾病的一个主要病因,在世界范围内的地位已得到充分确立。我们对IgA分子的基因衍生异常形式在该疾病中所起作用的理解程度正在不断发展,有望在未来转化为针对患者的更具特异性的治疗方式。与此同时,我们几乎没有特定的治疗选择,其中大多数尚未在大量患者中得到充分研究。本述评将讨论我们能够确定哪些患者可能会病情进展——因此应被视为治疗候选者——的程度。此外,还将探讨一些患者可能已到“无可挽回地步”的观点。不幸的是,大多数评论将基于在成人中进行的研究结果——这是儿科肾脏病学家非常熟悉的情况。

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