Autosomal dominant polycystic kidney disease: the changing face of clinical management

被引：231

作者：

Ong, Albert C. M. ^{[1
,2
]}

Devuyst, Olivier ^{[3
,4
]}

Knebelmann, Bertrand ^{[5
]}

Walz, Gerd ^{[6
]}

机构：

[1] Univ Sheffield, Sch Med, Acad Nephrol Unit, Sheffield S10 2RX, S Yorkshire, England

[2] Sheffield Teaching Hosp NHS Fdn Trust, Sheffield Kidney Inst, Sheffield, S Yorkshire, England

[3] Univ Zurich, Inst Physiol, Zurich Ctr Integrat Human Physiol, Zurich, Switzerland

[4] Catholic Univ Louvain, Div Nephrol, B-1200 Brussels, Belgium

[5] Univ Paris 05, Hop Necker, AP HP, Ctr Reference Malad Renales Hereditaires MARHEA, Paris, France

[6] Univ Freiburg, Med Ctr, Dept Nephrol, Freiburg, Germany

来源：

LANCET | 2015年 / 385卷 / 9981期

基金：

瑞士国家科学基金会; 欧盟第七框架计划; 英国医学研究理事会;

关键词：

LEFT-VENTRICULAR MASS; PRIMARY CILIA; RENAL-FUNCTION; CYST GROWTH; ADPKD; GENE; PROGRESSION; MUTATIONS; RECEPTOR; VOLUME;

D O I：

10.1016/S0140-6736(15)60907-2

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Autosomal dominant polycystic kidney disease is the most common inherited kidney disease and accounts for 7-10% of all patients on renal replacement therapy worldwide. Although first reported 500 years ago, this disorder is still regarded as untreatable and its pathogenesis is poorly understood despite much study. During the past 40 years, however, remarkable advances have transformed our understanding of how the disease develops and have led to rapid changes in diagnosis, prognosis, and treatment, especially during the past decade. This Review will summarise the key findings, highlight recent developments, and look ahead to the changes in clinical practice that will likely arise from the adoption of a new management framework for this major kidney disease.

引用

页码：1993 / 2002

页数：10

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