Figure 1. The natural history of autosomal dominant polycystic kidney disease as depicted by renal function decline as well as the onset of physical and psychological symptoms.

Reductions in total nephron mass are masked by compensatory changes in glomerular filtration rate (GFR) such that total GFR remains apparently normal for many years until compensation fails. Subclinical physiological changes are detectable from the earliest stages of disease (for example, in children), whereas clinical symptoms usually occur later. The psychological burden of having the diagnosis made is generally underestimated.