Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disorder, with cyst formation and structural kidney changes present during childhood or before birth. The 2025 KDIGO guidelines build on earlier frameworks to improve screening, monitoring, and treatment methodology for pediatric ADPKD patients. Methods: This executive summary synthesizes the updated 2025 KDIGO recommendations and supporting evidence to outline advancements in diagnosis, blood pressure and proteinuria management, follow-up, and transition planning. Results: The guidelines introduce more precise definitions of disease subtypes, tighter blood pressure targets, and expanded recommendations for genetic testing, ultrasound screening, and symptom-driven follow-up. KDIGO emphasizes ambulatory blood pressure monitoring from age five, renin-angiotensin system inhibitors as first-line therapy, and structured transition planning during adolescence. Conclusion: The 2025 KDIGO pediatric ADPKD guidelines provide a practical, family-centered framework for individualized care and improved transition to adult services, promoting early detection, proactive management, and shared decision making to support long-term outcomes.
Subhash et al. (2026) studied this question.