ArticleScientific reports2023
Atypical Polycystic Kidney Disease as defined by Imaging.
Article in Scientific reports, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 15 citations in OpenAlex.
- Canadian Society of Nephrology Commentary on the 2025 Kidney Disease Improving Global Outcomes Clinical Practice Guidelines for Autosomal Dominant Polycystic Kidney Disease.Canadian journal of kidney health and disease · 2026Article
- Review
- The need for clinical, genetic and radiological characterization of atypical polycystic kidney disease.Journal of nephrology · 2025Article
- Management of high-grade kidney trauma on bilateral polycystic kidney disease: A case report.International journal of surgery case reports · 2024Article
- Improved predictions of total kidney volume growth rate in ADPKD using two-parameter least squares fitting.Scientific reports · 2024Article
- How Does ADPKD Severity Differ Between Family Members?Kidney international reports · 2024Review
- Familial Variability of Disease Severity in Adult Patients With ADPKD.Kidney international reports · 2024Article
- Article
- Real-life use of tolvaptan in ADPKD: a retrospective analysis of a large Canadian cohort.Scientific reports · 2023Article
- Baseline characteristics of the Korean genetic cohort of inherited cystic kidney disease.Kidney research and clinical practice · 2023Article
- Evidence for Kidney Volume as a Measure of ADPKD Severity "Marches On" in the OVERTURE Study.Kidney international reports · 2023Article
- Metformin Versus Standard of Care in Patients with Autosomal Dominant Polycystic Kidney Disease - A Randomized Control Trial.Indian journal of nephrologyArticle
Corrections and comments
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Authors and funding
14 authors at 3 institutions in 1 country.
Funding
Abstract
Using age- and height-adjusted total kidney volume, the Mayo Clinic Imaging Classification provides a validated approach to assess the risk of chronic kidney disease (CKD) progression in autosomal dominant polycystic kidney disease (ADPKD), but requires excluding patients with atypical imaging patterns, whose clinical characteristics have been poorly defined. We report an analysis of the prevalence, clinical and genetic characteristics of patients with atypical polycystic kidney disease by imaging. Patients from the extended Toronto Genetic Epidemiology Study of Polycystic Kidney Disease recruited between 2016 and 2018 completed a standardized clinical questionnaire, kidney function assessment, genetic testing, and kidney imaging by magnetic resonance or computed tomography. We compared the prevalence, clinical features, genetics, and renal prognosis of atypical versus typical polycystic kidney disease by imaging. Forty-six of the 523 (8.8%) patients displayed atypical polycystic kidney disease by imaging; they were older (55 vs. 43 years; P < 0.001), and less likely to have a family history of ADPKD (26.1% vs. 74.6%; P < 0.001), a detectable PKD1 or PKD2 mutation (9.2% vs. 80.4%; P < 0.001), or progression to CKD stage 3 or stage 5 (P < 0.001). Patients with atypical polycystic kidney disease by imaging represent a distinct prognostic group with a low likelihood of progression to CKD.
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