Autosomal dominant polycystic kidney disease (ADPKD)
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A 35-year-old presents with hypertension and flank pain. Abdominal ultrasound reveals enlarged kidneys bilaterally with numerous cysts. His father had kidney failure at age 50.
AD; PKD1 (85%) or PKD2 (15%)
- PKD1: more severe, earlier ESRD (average age 54)
- PKD2: milder, later ESRD (average age 74)
- Renal: Progressive cyst development, enlarged kidneys, ESRD
- Hepatic: Liver cysts (common, usually asymptomatic)
- Vascular: Intracranial aneurysms (5-10%), MVP
- Other: Hypertension, hematuria, flank pain, nephrolithiasis
- Ultrasound criteria (age-based cyst number)
- Genetic testing (especially if family history uncertain)
- Blood pressure control
- Tolvaptan (vasopressin receptor antagonist) - slows progression
- MRA screening for intracranial aneurysms (if family history or symptoms)