A 48 year old man is found to have BP 170/95 and a follow-up blood test shows eGFR 55. The abdomen seems to contain hard masses. On following this up, this investigation is undertaken:
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So that’s the diagnosis. What is the prognosis likely to be? Is there anything you can do to alter it?
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This is advanced PKD with very large kidneys and not much normal kidney tissue in between the cysts. This patient is likely to be heading for dialysis and/or a renal transplant. It is amazing that liver failure is extremely rare in PKD, despite sometimes massive cyst formation – though there can be physical symptoms from all this extra volume in the abdomen.
PKD accounts for close to 10% of end stage renal disease in most countries.
Management of PKD has been mostly symptomatic until recently, but the first drug to slow down the progressive growth of cysts is now licensed: Tolvaptan, an ADH receptor antagonist. CKD issues in PKD are the same as for any other patient with renal impairment.
- PKD – advanced info for patients – including about Tolvaptan (Edren Info)
- Inherited kidney diseases – very concise (Edren textbook)
- PKD on Radiopaedia – excellent images, and links to browse.
The image shown is courtesy of Prof Frank Gaillard (Radiopaedia)