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Cystic Diseases of the Kidney

Practice Essentials

One third of people older than 50 years develop cysts in their kidneys. Although most are simple cysts, renal cystic disease has multiple etiologies. Broad categories of cystic disease include the following
:

Developmental – Multicystic dysplastic kidney (MCDK)

Genetic – Autosomal recessive polycystic kidney disease (ARPKD), autosomal dominant polycystic kidney disease (ADPKD), juvenile nephronophthisis (JNPHP), medullary cystic kidney disease (MCKD), glomerulocystic kidney disease (GCKD)

Cysts associated with systemic disease – Von Hippel-Lindau syndrome (VHLS), tuberous sclerosis (TS)

Acquired – Simple cysts, acquired cystic renal disease, medullary sponge kidney (MSK)

Malignancy – Cystic renal cell carcinoma (RCC); accounts for < 5% of all RCCs

The most common larger cysts include acquired cysts, simple cysts, and cysts associated with ADPKD. Smaller cysts characterize ARPKD, JNPHP, MCKD, and MSK. In adults, renal angiomyolipomas and RCC may also have cystic components.

The presentation and workup in patients with renal cysts varies with the underlying disease. Treatment is aimed at symptom control. In general, therapy is reserved for pain, hypertension, infection, renal salt wasting, and nephrolithiasis.

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