Treatment Options for Polycystic Kidney Disease

Prognosis for Polycystic Kidney Disease

The rate of progression of polycystic kidney disease (PKD) to end stage renal failure varies widely among individuals with PKD. About 40% of children with ARPKD do not survive beyond the age of two. In adults, untreated ADPKD can progress slowly or rapidly to kidney failure.

Recently, some factors that have been identified that may, in part, determine the rate of progression include:

• Nature of the inherited mutation - disease progression is more rapid for ADPKD1 than for ADPKD2.
• Presence of mutations of both genes together is a marker of severe disease
• Presence of interstitial fibrosis (progressive formation of excessive connective tissue in the kidney) eventually interferes with kidney function and accelerates the pace of progression to ESRD.