Figure 4.

Flow diagram to confirm and treat ADPKD

Bilateral renal cysts with enlarged kidneys and limited renal function in the context of extrarenal manifestations such as hepatic cysts and arterial hypertension lead to a diagnosis of ADPKD if family history is positive. If family history is negative (approx. 30% of cases), and if screening of relatives is negative (approx. 10%), diagnosis is largely certain if manifestation is typical, and it should be assumed that this is an initial manifestation. If manifestation is atypical, further genetic diagnostic testing is recommended. The best method of estimating the risk of progression is currently kidney volume in combination with age, sex, and renal function (eGFR).

ACE: Angiotensin-converting enzyme; ADPKD: Autosomal dominant polycystic kidney disease; GFR: Glomerular filtration rate; eGFR: Estimated glomerular filtration rate