The PROPKD Score

Gall, Emilie Cornec-Le; Audrézet, Marie-Pierre; Rousseau, Annick; Hourmant, Maryvonne; Renaudineau, Éric; Charasse, Christophe; Morin, Marie‐Pascale; Moal, Marie-Christine; Dantal, Jacques; Wehbe, Bassem; Perrichot, Régine; Frouget, T.; Vigneau, Cécile; Potier, Jérôme; Jousset, Philippe; Guillodo, Marie-Paule; Siohan, P.; Terki, Nazim; Sawadogo, Théophile; Legrand, Didier; Menoyo-Calonge, Victorio; Benarbia, Seddik; Besnier, Dominique; Longuet, Hélène; Férec, Claude; Meur, Yann Le

doi:10.1681/asn.2015010016

Cited by 274 publications

(159 citation statements)

References 28 publications

Supporting

Mentioning

149

Contrasting

Unclassified

Order By: Relevance

“…It is likely that the combination of texture feature analysis with other non-image based biomarkers will improve personalized clinical decision making of ADPKD. We believe that these texture metrics can function as a complementary tool to existing classification methods, such as the HtTKV-based prognostic model developed by Irazabal et al 39 and the proPKD score developed by Cornec-Le Gall et al 41 . In particular, based on these results (Table 2), there is a 23% narrowing of the prediction intervals when including the MR-derived texture features.…”

Section: Discussionmentioning

confidence: 99%

Image texture features predict renal function decline in patients with autosomal dominant polycystic kidney disease

Kline

Korfiatis

Edwards

et al. 2017

Kidney International

View full text Add to dashboard Cite

Magnetic resonance imaging (MRI) examinations provide high-resolution information about the anatomic structure of the kidneys and are used to measure total kidney volume (TKV) in patients with Autosomal Dominant Polycystic Kidney Disease (ADPKD). Height adjusted TKV (HtTKV) has become the gold-standard imaging biomarker for ADPKD progression at early stages of the disease when estimated glomerular filtration rate (eGFR) is still normal. However, HtTKV does not take advantage of the wealth of information provided by MRI. Here we tested whether image texture features provide additional insights into the ADPKD kidney that may be used as complementary information to existing biomarkers. A retrospective cohort of 122 patients from The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP study) was identified who had T2-weighted MRIs, and eGFR values over 70 mL/min/1.73m2 at the time of their baseline scan. We computed nine distinct image texture features for each patient. The ability of each feature to predict subsequent progression to CKD stage 3A, 3B, and 30% reduction in eGFR at eight-year follow up was assessed. A multiple linear regression model was developed incorporating age, baseline eGFR, HtTKV, and three image texture features identified by stability feature selection (Entropy, Correlation, and Energy). Including texture in a multiple linear regression model (predicting percent change in eGFR) improved Pearson correlation coefficient from -0.51 (using age, eGFR, and HtTKV) to -0.70 (adding texture). Thus, texture analysis offers an approach to refine ADPKD prognosis and should be further explored for its utility in individualized clinical decision making and outcome prediction.

show abstract

Section: Discussionmentioning

confidence: 99%

Image texture features predict renal function decline in patients with autosomal dominant polycystic kidney disease

Kline

Korfiatis

Edwards

et al. 2017

Kidney International

View full text Add to dashboard Cite

show abstract

“…11,12 Through this population-based study, we aimed to describe the clinical presentation of PKD2- related ADPKD and investigate factors affecting progression to chronic kidney disease (CKD). In addition, we explored the prevalence of PKD2 mutations using publicly available whole-exome sequencing data and have provided, we believe for the first time, an estimate of the genetic prevalence of ADPKD.…”

mentioning

confidence: 99%

PKD2 -Related Autosomal Dominant Polycystic Kidney Disease: Prevalence, Clinical Presentation, Mutation Spectrum, and Prognosis

Gall

Audrézet

Renaudineau

et al. 2017

American Journal of Kidney Diseases

Self Cite

View full text Add to dashboard Cite

Background PKD2-related autosomal dominant polycystic kidney disease (ADPKD) is widely acknowledged to be of milder severity than PKD1-related disease, but population-based studies depicting the exact burden of the disease are lacking. We aimed to revisit PKD2 prevalence, clinical presentation, mutation spectrum, and prognosis through the Genkyst cohort. Study Design Case series, January 2010 to March 2016. Settings & Participants Genkyst study participants are individuals older than 18 years from 22 nephrology centers from western France with a diagnosis of ADPKD based on Pei criteria or at least 10 bilateral kidney cysts in the absence of a familial history. Publicly available whole-exome sequencing data from the ExAC database were used to provide an estimate of the genetic prevalence of the disease. Outcomes Molecular analysis of PKD1 and PKD2 genes. Renal survival, age- and sex-adjusted estimated glomerular filtration rate. Results The Genkyst cohort included 293 patients with PKD2 mutations (203 pedigrees). PKD2 patients with a nephrology follow-up corresponded to 0.63 (95% CI, 0.54–0.72)/10,000 in Brittany, while PKD2 genetic prevalence was calculated at 1.64 (95% CI, 1.10–3.51)/10,000 inhabitants in the European population. Median age at diagnosis was 42 years. Flank pain was reported in 38.9%; macroscopic hematuria, in 31.1%; and cyst infections, in 15.3% of patients. At age 60 years, the cumulative probability of end-stage renal disease (ESRD) was 9.8% (95% CI, 5.2%–14.4%), whereas the probability of hypertension was 75.2% (95% CI, 68.5%–81.9%). Although there was no sex influence on renal survival, men had lower kidney function than women. Nontruncating mutations (n = 36) were associated with higher age-adjusted estimated glomerular filtration rates. Among the 18 patients with more severe outcomes (ESRD before age 60), 44% had associated conditions or nephropathies likely to account for the early progression to ESRD. Limitations Younger patients and patients presenting with milder forms of PKD2-related disease may not be diagnosed or referred to nephrology centers. Conclusions Patients with PKD2-related ADPKD typically present with mild disease. In case of accelerated degradation of kidney function, a concomitant nephropathy should be ruled out.

show abstract

“…50 The scoring system assigns points as shown in Table 5. Thus, an individual’s PROPKD score can range from 0 to 9 points.…”

Section: Predicting Disease Progressionmentioning

confidence: 99%

“…50 In these cases, genetic scoring may be carried out, comprising only genetic data and gender. In this scoring system, patients fall into 1 of 4 prognostic groups:…”

Section: Predicting Disease Progressionmentioning

confidence: 99%

Assessing Risk of Disease Progression and Pharmacological Management of Autosomal Dominant Polycystic Kidney Disease

Soroka

Alam

Bevilacqua

et al. 2017

Can J Kidney Health Dis

View full text Add to dashboard Cite

Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder worldwide. The disease is characterized by renal cysts and progressive renal failure due to progressive enlargement of cysts and renal fibrosis. An estimated 45% to 70% of patients with ADPKD progress to end-stage renal disease by age 65 years. Although both targeted and nontargeted therapies have been tested in patients with ADPKD, tolvaptan is currently the only pharmacological therapy approved in Canada for the treatment of ADPKD. The purpose of this consensus recommendation is to develop an evidence-informed recommendation for the optimal management of adult patients with ADPKD. This document focuses on the role of genetic testing, the role of renal imaging, predicting the risk of disease progression, and pharmacological treatment options for ADPKD. These areas of focus were derived from 2 national surveys that were disseminated to nephrologists and patients with ADPKD with the aim of identifying unmet needs in the management of ADPKD in Canada. Specific recommendations are provided for the treatment of ADPKD with tolvaptan.

show abstract

The PROPKD Score

Cited by 274 publications

References 28 publications

Image texture features predict renal function decline in patients with autosomal dominant polycystic kidney disease

Image texture features predict renal function decline in patients with autosomal dominant polycystic kidney disease

PKD2 -Related Autosomal Dominant Polycystic Kidney Disease: Prevalence, Clinical Presentation, Mutation Spectrum, and Prognosis

Assessing Risk of Disease Progression and Pharmacological Management of Autosomal Dominant Polycystic Kidney Disease

Contact Info

Product

Resources

About