Polycystic kidney disease (PKD) is passed down through families (inherited), usually as an autosomal dominant trait. If one parent carries the gene, the children have a 50% chance of developing the disorder.
The disease gets worse slowly. Eventually it leads to end-stage kidney failure. It is also associated with liver disease, including infection of liver cysts.
Medical treatment may relieve symptoms for many years.
People with PKD who don’t have other diseases may be good candidates for a kidney transplant.
Prognosis of adult onset polycystic kidney disease re-evaluated. To appraise the prognosis of adult onset polycystic kidney disease (APKD), an inception cohort containing 140 subjects from 17 kindreds was assembled. Multiple renal cysts, demonstrable by ultrasonography, or clinical APKD, or both were present in 100 subjects. APKD was predicted in 32 subjects unavailable for ultrasonography and could not be excluded in eight deceased subjects. All had been at risk for endstage renal disease (ESRD) since birth. The probability of either developing ESRD, requiring dialysis or transplantation, or dying was estimated using a time-to-event analysis. The earliest age at which ESRD occurred was 36 years. For those with APKD, the probability of being alive and not having ESRD was 77% by age 50, 57% by age 58, and 52% by age 73 years. Excluding those predicted to have APKD changes these probabilities to 75, 53, and 47%, respectively. The serum creatinine values were less than 1.5 mg/dl for most subjects who had not developed ESRD. The prognosis for subjects with APKD is much better than most reports suggest and can be estimated from the time-to-event data presented.