genitourinary disorders
Autosomal Dominant Polycystic Kidney Disease (ADPKD)
Prognosis for ADPKD
Renal failure develops in 35 to 45% of patients with autosomal dominant polycystic kidney disease (ADPKD) by age 60. By age 75, 50 to 75% of patients require Overview of Renal Replacement Therapy ( Earlier age at diagnosis Male sex Sickle Cell Disease trait PKD1genotype Larger or rapidly increasing kidney size Gross hematuria Hypertension None Black race Increasing proteinuria Cyst and kidney volume measurements predict risk of progression to Chronic Kidney Disease and end-stage kidney disease, often before changes in routine laboratory studies. For example, cyst size and kidney size predict 8-year risk of chronic kidney disease more accurately than age, degree of proteinuria, or serum blood Phosphaturic hormone fibroblast growth factor (FGF) 23 elevation was associated with increased kidney size and annualized rate of estimated glomerular filtration rate (eGFR) decline but interestingly did not enhance risk prediction for disease progression ( Prognosis references ). ADPKD does not increase risk of renal cancer, but if patients with ADPKD develop renal cancer, it is more likely to be bilateral. Renal cancer rarely causes death. Patients usually die of heart disease (sometimes valvular), disseminated infection, or ruptured cerebral aneurysm. 1.Grantham JJ, Torres VE, Chapman AB, et al: Volume progression in polycystic kidney disease.N Engl J Med18;354(20):2122-2130, 2006. doi: 10.1056/NEJMoa054341 2.Chonchol M, Gitomer B, Isakova T, et al: Fibroblast growth factor 23 and kidney disease progression in autosomal dominant polycystic kidney disease.Clin J Am Soc Nephrol12(9):1461-1469, 2017. doi: 10.2215/CJN.12821216 