ArticlePediatric nephrology (Berlin, Germany)2025
Characteristics of patients with autosomal polycystic kidney disease reaching kidney failure by age 40.
Article in Pediatric nephrology (Berlin, Germany), 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- Cystic burden meets blood pressure: cardiac remodelling in paediatric autosomal dominant polycystic kidney disease.Pediatric nephrology (Berlin, Germany) · 2026Article
- Review
- KDIGO 2025 ADPKD guideline through pediatric eyes.Pediatric nephrology (Berlin, Germany) · 2026Article
- Hypertension Resistant to RAAS Inhibitors as a Prognostic Indicator for Rapid Progression to ESRD in ADPKD: A Ten-Year Follow-Up.Diagnostics (Basel, Switzerland) · 2025Article
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14 authors.
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Abstract
backgroundAutosomal dominant polycystic kidney disease (ADPKD) demonstrates broad genetic and phenotypic variability, with kidney failure (KF) occurring across a wide age spectrum. Despite several predictor tools, there remains a need to identify factors associated with rapid disease progression. This study describes the phenotypic characteristics of a multicentric cohort experiencing early-onset KF by age 40.
methodsThis retrospective, multicenter cohort study analyzed longitudinal data of rapidly progressive ADPKD patients (n = 199). The prevalence of established risk factors was compared to nine existing ADPKD cohorts (n
resultsThe median age at ADPKD diagnosis was 22.3 years (IQR, 16.5-28.6) and median age of KF was 35.6 years (31.7-38.0). Hypertension was observed in 68.1% of cases, with early-onset hypertension being more common among those with accelerated progression towards KF. Urological events were present in 60.1% of cases, with a high burden of gross hematuria (30.4%). Existing ADPKD cohorts had a mean age of 45.5 years, with weighted prevalences of hypertension (71.1%), kidney stones (22.4%), hematuria (22.9%), and urinary tract infections (22.8%). Extrarenal manifestations were less prevalent compared to other ADPKD cohorts.
conclusionThis study outlines a cohort of ADPKD patients with accelerated disease progression, reaching KF before age 40. Hypertension and urological events were highly prevalent at a young age, emphasizing the importance of early and regular blood pressure monitoring.
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