Chapman Arlene B, Devuyst Olivier, Eckardt Kai-Uwe, Gansevoort Ron T, Harris Tess, Horie Shigeo, Kasiske Bertram L, Odland Dwight, Pei York, Perrone Ronald D, Pirson Yves, Schrier Robert W, Torra Roser, Torres Vicente E, Watnick Terry, Wheeler David C
Emory University School of Medicine, Atlanta, Georgia, USA.
Institute of Physiology, Zurich Center for Integrative Human Physiology, University of Zurich, Zurich, Switzerland.
Kidney Int. 2015 Jul;88(1):17-27. doi: 10.1038/ki.2015.59. Epub 2015 Mar 18.
Autosomal-dominant polycystic kidney disease (ADPKD) affects up to 12 million individuals and is the fourth most common cause for renal replacement therapy worldwide. There have been many recent advances in the understanding of its molecular genetics and biology, and in the diagnosis and management of its manifestations. Yet, diagnosis, evaluation, prevention, and treatment vary widely and there are no broadly accepted practice guidelines. Barriers to translation of basic science breakthroughs to clinical care exist, with considerable heterogeneity across countries. The Kidney Disease: Improving Global Outcomes Controversies Conference on ADPKD brought together a panel of multidisciplinary clinical expertise and engaged patients to identify areas of consensus, gaps in knowledge, and research and health-care priorities related to diagnosis; monitoring of kidney disease progression; management of hypertension, renal function decline and complications; end-stage renal disease; extrarenal complications; and practical integrated patient support. These are summarized in this review.
常染色体显性多囊肾病(ADPKD)影响着多达1200万人,是全球范围内肾替代治疗的第四大常见病因。近年来,在对其分子遗传学和生物学的理解以及对其临床表现的诊断和管理方面取得了许多进展。然而,诊断、评估、预防和治疗方法差异很大,且没有广泛认可的实践指南。基础科学突破转化为临床护理存在障碍,各国之间存在相当大的异质性。“改善全球肾脏病预后争议会议:ADPKD”汇聚了多学科临床专家小组并邀请患者参与,以确定共识领域、知识差距以及与诊断、肾脏疾病进展监测、高血压管理、肾功能下降和并发症、终末期肾病、肾外并发症以及实际的综合患者支持相关的研究和医疗保健重点。本综述对这些内容进行了总结。