首页 | 本学科首页   官方微博 | 高级检索  
     


The PROPKD Score: A New Algorithm to Predict Renal Survival in Autosomal Dominant Polycystic Kidney Disease
Authors:Emilie Cornec-Le Gall  Marie-Pierre Audrézet  Annick Rousseau  Maryvonne Hourmant  Eric Renaudineau  Christophe Charasse  Marie-Pascale Morin  Marie-Christine Moal  Jacques Dantal  Bassem Wehbe  Régine Perrichot  Thierry Frouget  Cécile Vigneau  Jér?me Potier  Philippe Jousset  Marie-Paule Guillodo  Pascale Siohan  Nazim Terki  Théophile Sawadogo  Didier Legrand  Victorio Menoyo-Calonge  Seddik Benarbia  Dominique Besnier  Hélène Longuet  Claude Férec  Yannick Le Meur
Abstract:
The course of autosomal dominant polycystic kidney disease (ADPKD) varies among individuals, with some reaching ESRD before 40 years of age and others never requiring RRT. In this study, we developed a prognostic model to predict renal outcomes in patients with ADPKD on the basis of genetic and clinical data. We conducted a cross-sectional study of 1341 patients from the Genkyst cohort and evaluated the influence of clinical and genetic factors on renal survival. Multivariate survival analysis identified four variables that were significantly associated with age at ESRD onset, and a scoring system from 0 to 9 was developed as follows: being male: 1 point; hypertension before 35 years of age: 2 points; first urologic event before 35 years of age: 2 points; PKD2 mutation: 0 points; nontruncating PKD1 mutation: 2 points; and truncating PKD1 mutation: 4 points. Three risk categories were subsequently defined as low risk (0–3 points), intermediate risk (4–6 points), and high risk (7–9 points) of progression to ESRD, with corresponding median ages for ESRD onset of 70.6, 56.9, and 49 years, respectively. Whereas a score ≤3 eliminates evolution to ESRD before 60 years of age with a negative predictive value of 81.4%, a score >6 forecasts ESRD onset before 60 years of age with a positive predictive value of 90.9%. This new prognostic score accurately predicts renal outcomes in patients with ADPKD and may enable the personalization of therapeutic management of ADPKD.
Keywords:end-stage renal disease   ADPKD   risk factors   genetic renal disease   progression of renal failure
设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号