Autosomal Dominant Polycystic Kidney Disease

Autosomal Dominance Polycystic Kidney Disease:

  • Bilateral firm flank masses
  • HTN
  • Hematuria, proteinuria, renal insufficiency
  • Other extra-renal complications:
    • Intracranial aneurysm (Circle of Willis)
      • Intracerebral or Subarahnoid hemorrhage
        • Higher risk in patients with FHx of ruptured aneurysm.
        • “Thunderclap” sudden onset severe headache, nausea, vomiting, neck stiffness, low-grade fever, uncontrolled HTN, lethargic
        • CT head to diagnose (may miss small bleeds)
          • Lumbar puncture: Xanthochromia
            • Positive CT head or LP: Angiography to localize source
      • Screening is controversial.
        • Best: Time-of-flight MRA (magnetic resonance angiography) – does not require gadolinium contrast and can be done at any GFR.
        • Alternate: CT Angiography
    • Cardiac valve disease and aortic root dilatation
      • Apical 2/6 holosystolic murmur
    • Hepatic and pancreatic cysts
    • Diverticulosis

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