​Clinical Pearls

• ADPKD diagnosis is established by imaging studies of the kidneys; genetic testing alone is inadequate to diagnose ADPKD in most cases, but it can help to establish a diagnosis in patients without a family history (21,22).

• Patients without a family history may have de novo mutations (2).

• In patients diagnosed with ADPKD, TKV is the best way to assess risk category and prognosis because GFR remains stable as kidney volume increases due to glomerular compensation (23).

• ADPKD disease progression risk can be assessed with available models such as the Mayo risk classification and PROPKD (24,25).

• Use eGFR estimating equations that do not include race (18).

• Early treatment of ADPKD with tolvaptan is estimated to delay advancement of kidney function loss by up to 5 years (5).

• Cardiovascular morbidity and total mortality are higher in younger patients (aged 20 to 49 years compared to aged 65 years or older) (26).

• Polycystic liver disease is the most common extrarenal manifestation of ADPKD; treatments include somatostatin analog therapy and surgery (27).

• Patients with ADPKD and cystic liver disease receiving tolvaptan who were older and had rapid liver growth progression experienced less annual liver growth (10).