Prognosis of Polycystic Kidney Disease?
Polycystic kidney disease is an inherited disease that involves bilateral renal cysts. The condition is broadly divided into 2 forms: autosomal dominent polycystic kidney disease (ADPKD) and autosomal recessive polycystic kidney disease (ARPKD). This article focuses on ADPKD; for full discussion of ARPKD, see Pediatric Polycystic Kidney Disease. However, note that although ADPKD was previously known as adult polycystic kidney disease and ARPKD was previously known as infantile polycystic kidney disease, those descriptions are not accurate, and that nomenclature is no longer used.
@koli The prognosis in patients with ADPKD covers a wide spectrum. Kidney failure has been reported in children; conversely, individuals with ADPKD may live a normal lifespan without knowing that they have the disease. More typically, however, ADPKD causes progressive kidney dysfunction, resulting in grossly enlarged kidneys and kidney failure by the fourth to sixth decade of life. There is an inverse association between the size of polycystic kidneys and the level of glomerular filtration.
An early study estimated that approximately 70% of patients with ADPKD would develop kidney insufficiency if they survived to age 65 years. Currently, half of all patients with ADPKD require renal replacement therapy by age 60 years. Risk factors for progression include the following:
Several episodes of gross hematuria
Severe and frequent kidney infections
Black racial background