​Clinical Pearls

• ADPKD diagnosis is established by imaging studies of the kidneys; genetic testing alone is inadequate for ADPKD diagnosis in most cases (16).

• Molecular genetic testing can be used when patients lack family history, when imaging results are equivocal, or to establish a diagnosis in a patient under 30 years of age (17).

• eGFR remains stable as kidney volume increases due to glomerular compensation; TKV is the best way to assess risk category and prognosis, evaluated together with eGFR (11).

• ADPKD risk category can be determined with widely available models, such as the Mayo Imaging Classification (8) and the PROPKD (10) models.

• Compared to patients with PKD1-related ADPKD, kidney growth is slower in patients with PKD2-related ADPKD, but the latter experience many of the same renal and nonrenal complications of ADPKD (2).

• Intensive blood pressure control (95/60 to 110/75 mm Hg) is associated with a significantly slower annual increase in TKV  (1).

• Obesity and overweight are significantly associated with greater rates of TKV growth (5).

• Recent data have shown that, in patients aged 55–65 years with an eGFR rate of decline ≥3 mL/min/1.73 m  per year, disease-modifying treatment with tolvaptan had efficacy similar to that observed in the overall indication (18).

• Patients like Brenda who are in Mayo category 1B but who have a rapid decline in eGFR may be appropriate for treatment with tolvaptan.