Fact-sheet: Polycystic kidney disease
Updated on 09/05/2019 at 9:23 AM
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Definition
Autosomal dominant polycystic kidney disease (ADPKD) is a common hereditary disease (1/1000). Accounts for
10% of end-stage renal disease.
Type 1 or 2.
Clinical features
Hypertension
Workup for renal insufficiency
Incidental finding
Family screening
Laboratory findings
Progressive renal insufficiency
Ultrasound
In addition to the family history, ultrasound diagnosis:
- 15-39 years: diagnosis if ≥ 3 renal cysts (unilateral or bilateral)
- ≥ 40-59 years: diagnosis if ≥ 2 renal cysts in each kidney
- > 60 years: diagnosis if > 4 cysts in each kidney
Between 30 and 39 years: absence of cysts excludes the disease with 99% certainty.
After 40 years, finding 0 or 1 cyst excludes the disease.
The cysts are most often benign (Bosniak I).
There is an increased risk of renal neoplasia (x2) in native kidneys.
The kidneys are enlarged due to these cysts (renal insufficiency with enlarged kidneys).
Associated with hepatic polycystic disease in 30 to 70% of cases.
CT
Same as ultrasound (higher sensitivity for small cysts)
Complications: intracystic hemorrhage and cyst superinfection
Associated finding: cerebral artery aneurysm (circle of Willis)
MRI
Same as ultrasound
Management
In the presence of cysts/compatible ultrasound diagnostic criteria, ask about family history and refer for nephrology consultation.