Polycystic Kidney Disease
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Contents |
More Specific Terms
- acquired polycystic kidney disease
- adult polycystic kidney disease
- childhood polycystic kidney disease
- infantile polycystic kidney disease
- medullary cystic kidney disease
- medullary sponge kidney disease
- nephronophthisis
Etiology
-
- see adult & childhood polycystic kidney disease
- von Hippel-Lindau disease ( autosomal dominant)
- acquired renal cystic disease
- associated with long-term renal insufficiency
- can affect up to 50% of patients on long-term dialysis
Pathology
- polycystic kidney disease affecting the kidneys & the hepatic biliary
Genetics
- autosomal dominant forms
- defects in PKHD1 are the cause of all typical forms of autosomal recessive polycystic kidney disease
Clinical-manifestations
- clinical spectrum is widely variable
- most cases present during infancy
- hypertension at a young age
- flank pain
- gross hematuria
- palplable irregular kidneys or liver
Laboratory
Radiology
- renal ultrasound will identify renal cysts
- routine screening for intracranial aneurysm not indicated
Complications
- subarachnoid hemorrhage from intracranial aneurysm
- renal cysts are prone to hemorrhage
- increased risk of renal cell carcinoma with acquired form
Differential-diagnosis
- rupture of renal cyst can mimic nephrolithiasis
Management
- ACE inhibitor of ARB for hypertension if renal function permits - target blood pressure is < 130/80 mm Hg
- empiric antibiotic therapy for suspected infects cysts
- fluoroquinolone
- trimethoprim/sufamethazole ( Bactrim, Septra)
More General Terms
References
- Mayo Internal Medicine Board Review, 1998-99, Prakash UBS (ed) Lippincott-Raven, Philadelphia, 1998, pg 613-14
- Medical Knowledge Self Assessment Program (MKSAP) 11, 14, American College of Physicians, Philadelphia 1998, 2006
- Polycystic Kidney Disease: NIH Institute and Center Resources [1]
