Torra Roser
Inherited Renal Disorders, Nephrology Department, Fundació Puigvert, REDINREN, IIB Sant Pau, Universitat Autònoma de Barcelona, Barcelona, 08025, Spain.
F1000Res. 2019 Jan 29;8. doi: 10.12688/f1000research.17109.1. eCollection 2019.
Autosomal dominant polycystic kidney disease (ADPKD) is a genetic systemic disorder causing the development of renal and hepatic cysts and decline in renal function. It affects around 1 in 1,000 live births. Early hypertension and progressive renal failure due to massive enlargement of cysts and fibrosis are hallmarks of the disease. This article reviews recent advances in ADPKD and focuses mainly on diagnosis, management, and prediction of the course of the disease.
常染色体显性多囊肾病(ADPKD)是一种遗传性全身性疾病,可导致肾囊肿和肝囊肿形成以及肾功能下降。该病在活产婴儿中的发病率约为千分之一。囊肿大量增大和纤维化导致的早期高血压和进行性肾衰竭是该病的特征。本文综述了ADPKD的最新进展,主要聚焦于疾病的诊断、管理及病程预测。