PMID41101718: Associations of Skeletal Muscle Mass and Body Mass Index With Clinical Outcomes in Autosomal-Dominant Polycystic Kidney Disease
2026, Retrospective multicenter cohort (n=1,443), Tier 2
Key claim: Higher and better-quality skeletal muscle mass (NAMA) was associated with lower all-cause mortality and lower ESKD risk in ADPKD
Limitations: Observational design with potential residual confounding; muscle strength/performance not captured; single-country tertiary-center cohort
Open source PMID41705096: Ketogenic dietary interventions for ADPKD: systematic review (SWiM)
2026, Systematic review without meta-analysis, Tier 2
Key claim: Ketogenic-style interventions show metabolic feasibility but renal structural disease-modifying evidence remains uncertain
Limitations: Small heterogeneous short-duration studies; no robust pooled effect on hard renal outcomes
Open source PMID41774609: Long-term tolvaptan administration in Chinese ADPKD patients (real-world retrospective)
2026, Retrospective multicentre cohort, Tier 2
Key claim: Tolvaptan group showed slower htTKV growth and better 2-year eGFR trajectory vs untreated controls in this cohort
Limitations: Small non-randomized sample and potential selection bias; country/cohort-specific
Open source PMID41077128: Pravastatin RCT in adults with early-stage ADPKD (double-blind, placebo-controlled)
2026, Randomized controlled trial, Tier 1
Key claim: Pravastatin did not reduce annual htTKV growth or slow kidney blood-flow/kidney-function decline versus placebo over 2 years in adults with preserved kidney function
Limitations: Single pharmacologic class/intervention; findings apply to pravastatin regimen tested and do not exclude other pathways
Open source PMID41816084: Quantitative dynamic contrast-enhanced magnetic resonance imaging for renal perfusion measurement in autosomal dominant polycystic kidney disease
2026, Human imaging biomarker study (repeatability/reproducibility, n=25), Tier 2
Key claim: DCE-MRI intrarenal Ktrans was reproducible with phantom correction, distinguished mild vs severe ADPKD, and correlated with htTKV and eGFR, supporting perfusion biomarker feasibility
Limitations: Small single-center cohort and short repeat-interval imaging design; biomarker validation does not establish treatment efficacy or hard-outcome benefit
Open source PMID41811969: Renal blood flow and progression to kidney failure in ADPKD
2026, Longitudinal cohort analysis (HALT-PKD linkage), Tier 2
Key claim: Lower baseline renal blood flow independently associated with higher long-term risk of kidney failure and faster progression metrics in early-stage ADPKD
Limitations: Observational/prognostic design and MRI-based RBF measurement may limit generalizability and interventional inference
Open source PMID41758739: Tolvaptan for ADPKD: real-world propensity score weighted retrospective cohort
2026, Retrospective cohort (propensity-weighted), Tier 2
Key claim: Association with modest chronic eGFR slope benefit estimate but not statistically significant; high discontinuation rates
Limitations: Observational design with residual confounding and wide confidence intervals; tertiary-centre cohort
Open source PMID40810467: KDIGO 2025 ADPKD guideline commentary on hepatopancreatic manifestations
2025, Guideline commentary, Tier 2
Key claim: Supports structured management of extrarenal manifestations and highlights implementation/research gaps
Limitations: Commentary not primary efficacy trial; focus on implementation and hepatopancreatic scope
Open source PMID39848746: KDIGO 2025 ADPKD guideline executive summary
2025, Guideline summary, Tier 1
Key claim: Risk-stratified management and evidence-based treatment framework
Limitations: Needs full-text extraction for recommendation-level detail
Open source PMID39848759: KDIGO 2025 Clinical Practice Guideline for ADPKD (full guideline)
2025, Practice guideline, Tier 1
Key claim: Provides full recommendation set for evaluation/risk stratification and treatment of ADPKD
Limitations: No abstract-level efficacy quantification in PubMed listing; full-text extraction needed for recommendation granularity
Open source PMID40303212: Open-Label, Randomized, Controlled, Crossover Trial on the Effect of Dapagliflozin in Patients With ADPKD Receiving Tolvaptan
2025, Open-label randomized crossover trial (n=27), Tier 2
Key claim: In tolvaptan-treated ADPKD, 6-month dapagliflozin add-on was associated with attenuated eGFR slope decline and lower TKV growth versus control period
Limitations: Small single-country open-label crossover and short follow-up; surrogate endpoints; needs larger blinded confirmation
Open source PMID36191725: Clinical Pattern of Tolvaptan-Associated Liver Injury in Pivotal Trials
2022, Pooled trial safety analysis, Tier 2
Key claim: Tolvaptan has clinically relevant liver-injury risk requiring monitoring
Limitations: Generalizability outside trial settings
Open source NATCOMM2022: PKD1 and PKD2 mRNA cis-inhibition drives polycystic kidney disease progression
2022, Preclinical mechanistic, Tier 4
Key claim: CRISPR-based mechanistic editing reduced disease severity in models
Limitations: Preclinical only; no human efficacy
Open source PMID38319283: Prescribed Water Intake in Autosomal Dominant Polycystic Kidney Disease
2022, Randomized controlled trial (3-year), Tier 1
Key claim: Prescribed high water intake reduced urine osmolality but did not slow htTKV growth versus ad libitum intake over 3 years
Limitations: Only about half achieved urine-osmolality adherence target and no long-term copeptin reduction was seen; not a no-hydration trial
Open source PMID29105594: Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease (REPRISE)
2017, Phase 3 RCT, Tier 1
Key claim: Slower eGFR decline than placebo over 1 year
Limitations: Monitoring and tolerability constraints remain
Open source PMID23121377: Tolvaptan in patients with autosomal dominant polycystic kidney disease (TEMPO 3:4)
2012, Phase 3 RCT, Tier 1
Key claim: Slowed TKV growth and kidney function decline vs placebo over 3 years
Limitations: Adverse effects/discontinuation burden; selected population
Open source