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Polycystic Kidney Disease (PKD), Part 2

Neda Frayha, MD and Terry Watnick, MD
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Autosomal dominant polycystic kidney disease is broken down into two main subtypes: PKD-1 and PKD-2.  PKD-1 leads to renal impairment approximately 20 years before PKD-2.  While there is a 50% chance that the offspring of a parent with PKD will have the condition, routine screening before the age of 18 is not recommended due to to potential emotional or psychological impact and impact on insurability. Extra renal manifestations include polycystic liver disease and intracranial aneurysms. Hypertension management is key, with ACE or ARB being first line to keep BP below 130/80. Tolvaptan, a vasopressin 2 receptor antagonist, has recently been approved in the European Union, Canada, and Japan for the treatment of PKD, and it may be approved soon in the United States.

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Chris -

I'm a bit confused about the pearl that includes "currently there is no treatment" when tolvaptan is an option, at least in some areas outside of the USA. The pearl might benefit from being reworded to better acknowledge the results of TEMPO and REPRISE, even while Americans await tolvaptan approval.

Heidi J., MD -

That's true - good catch!

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A Feverish Frenzy! Full episode audio for MD edition 203:03 min - 95 MB - M4AHippo Primary Care RAP - January 2018 Summary 386 KB - PDF

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