Phys Vivas · renal
Polycystic Kidney Disease (ADPKD) — Viva Defence
Structured DCE viva: long-case defence of a 40-year-old woman with Mayo 1D ADPKD, eGFR 52 and a sister with a cerebral aneurysm — the tolvaptan decision, selective aneurysm screening and family implications, with probing first-person answers.
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Opening statement (SASPOP, delivered aloud)
"Ms Kowalski is a 40-year-old woman with ADPKD whose disease is now demonstrably fast: her eGFR has fallen 16 mL/min over 4 years, and her height-adjusted total kidney volume places her in Mayo class 1D — a rapid-progression class — on typical bilateral imaging. Her main problems are: rapid progression with enough preserved function to protect, which makes her a genuine tolvaptan candidate under the TEMPO and REPRISE evidence; a family history of intracranial aneurysm in an affected sister, which is a selective-screening indication she has not yet acted on; hypertension that is currently well controlled on an ACE inhibitor; and a young family in whom the inheritance question is unresolved. My plan is to confirm her progression stage, run the tolvaptan shared-decision conversation properly — benefit quantified against aquaresis, monitoring and cost — arrange her MR angiography, and open the family counselling with her children's testing deferred until they can decide for themselves." [1] [2] [5]
References8ShowHide
- [1]Irazabal MV, Rangel LJ, Bergstralh EJ, et al. Imaging classification of autosomal dominant polycystic kidney disease: a simple model for selecting patients for clinical trials J Am Soc Nephrol, 2015.PMID 24904092
- [2]Torres VE, Chapman AB, Devuyst O, et al. Tolvaptan in patients with autosomal dominant polycystic kidney disease N Engl J Med, 2012.PMID 23121377
- [3]Torres VE, Chapman AB, Devuyst O, et al. Tolvaptan in Later-Stage Autosomal Dominant Polycystic Kidney Disease N Engl J Med, 2017.PMID 29105594
- [4]Schrier RW, Abebe KZ, Perrone RD, et al. Blood pressure in early autosomal dominant polycystic kidney disease N Engl J Med, 2014.PMID 25399733
- [5]Irazabal MV, Huston J 3rd, Kubly V, et al. Extended follow-up of unruptured intracranial aneurysms detected by presymptomatic screening in patients with autosomal dominant polycystic kidney disease Clin J Am Soc Nephrol, 2011.PMID 21551026
- [6]Kidney Disease: Improving Global Outcomes (KDIGO) ADPKD Work Group KDIGO 2025 Clinical Practice Guideline for the Evaluation, Management, and Treatment of Autosomal Dominant Polycystic Kidney Disease (ADPKD) Kidney Int, 2025.PMID 39848759
- [7]Wang CJ, Creed C, Winklhofer FT, et al. Water prescription in autosomal dominant polycystic kidney disease: a pilot study Clin J Am Soc Nephrol, 2011.PMID 20876670
- [8]Spithoven EM, Kramer A, Meijer E, et al. Renal replacement therapy for autosomal dominant polycystic kidney disease (ADPKD) in Europe: prevalence and survival--an analysis of data from the ERA-EDTA Registry Nephrol Dial Transplant, 2014.PMID 25165182