Autosomal-Dominant (Adult) Polycystic Kidney Disease
Autosomal-dominant polycystic kidney disease is the most common genetically transmitted renal cystic disease.
The incidence is estimated between 1:500 and 1:1000.
Most patients present in the third to fourth decade of life; however pediatric cases can occur.
Slight male predominance is reported (male-to-female ratio 3:2).
The kidneys are mostly affected bilaterally.
Renal dialysis is the standard of care followed by renal transplant, when necessary.
Renal failure occurs in roughly 59% of patients.
The most frequent clinical findings are positive family history, flank mass, hematuria, hypertension, and azotemia. Hepatic fibrosis and biliary dysgenesis may be present. Renal cell adenoma and carcinoma can be seen among the cysts.