Suppr超能文献

塌陷性肾小球病:30年回顾及单一大中心经验

Collapsing glomerulopathy: a 30-year perspective and single, large center experience.

作者信息

Nicholas Cossey L, Larsen Christopher P, Liapis Helen

机构信息

Renal Pathology Division, Arkana Laboratories, Little Rock, AR, USA.

Department of Pathology & Immunology, Washington University School of Medicine, St Louis, MO, USA.

出版信息

Clin Kidney J. 2017 Aug;10(4):443-449. doi: 10.1093/ckj/sfx029. Epub 2017 May 8.

Abstract

Collapsing glomerulopathy (CGP) is a pattern of kidney injury seen on renal biopsy with multiple associations and etiologies. It is most commonly described in African-Americans and others with recent African ancestry. The disease is rapidly progressive and often presents with abrupt onset of renal failure and nephrotic-range proteinuria. Since its description 30 years ago, this entity has transformed from a morphologic diagnosis typically seen in the setting of HIV infection to a complicated diagnosis with numerous etiologies, many of which are associated with underlying apolipoprotein L1 ()-risk variants or other genetic disorders. We review the evolution of CGP, and its history and proposed pathomechanisms. We also present the disease spectrum from our experience with emphasis on recognizing the lesion, distinguishing from mimics and linking the histopathological pattern to a specific cause. Our understanding continues to evolve as clinicians and scientists work toward a more complete understanding of the molecular pathways of injury in this disease and how these might be disrupted for therapeutic purposes. Much still remains to be discovered in CGP as the molecular underpinnings leading to disease are still not completely understood and no effective treatment exists despite the high morbidity. Based on this rapid evolution, CGP is a modern template of how we diagnose and think about kidney disease. The story of CGP represents the current shift in nephrology and nephropathology from morphology-alone-based diagnosis to a comprehensive approach including molecular diagnostics. We believe this new, holistic approach will lead to pathogenesis-centered diagnoses that will help to individualize risk stratification and treatment protocols.

摘要

塌陷性肾小球病(CGP)是肾活检中可见的一种肾损伤模式,有多种关联因素和病因。它最常出现在非裔美国人以及其他有近期非洲血统的人群中。该疾病进展迅速,常表现为肾衰竭和肾病范围蛋白尿的突然发作。自30年前被描述以来,这种疾病已从通常在HIV感染背景下出现的形态学诊断转变为一种病因众多的复杂诊断,其中许多病因与潜在的载脂蛋白L1(APOL1)风险变异或其他遗传疾病有关。我们回顾了CGP的演变、其历史及提出的发病机制。我们还根据自身经验展示了疾病谱,重点在于识别病变、与相似病症相鉴别以及将组织病理学模式与特定病因联系起来。随着临床医生和科学家努力更全面地了解该疾病损伤的分子途径以及如何为治疗目的而干扰这些途径,我们的认识也在不断发展。在CGP中仍有许多有待发现的地方,因为导致疾病的分子基础仍未完全明了,尽管发病率很高,但尚无有效的治疗方法。基于这种快速演变,CGP是我们诊断和思考肾脏疾病的一个现代范例。CGP的故事代表了肾脏病学和肾病理学目前从单纯基于形态学的诊断向包括分子诊断在内的综合方法的转变。我们相信这种新的整体方法将导致以发病机制为中心的诊断,这将有助于个性化风险分层和治疗方案。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/968f/5570123/6f0fbf9ca5dc/sfx029f1.jpg

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验