Table 1.1 (Genetic diseases causing polycystic kidney disease) (Harris, 2001) 1
Disorder Pattern of inheritance Approximate frequency
Autosomal dominant polycystic kidney disease (ADPKD) Dominant 1 in 1000
Autosomal recessive polycystic kidney disease (ARPKD) Recessive 1 in 40 000
Familial juvenile nephronophthisis (NPH) Recessive Rare
Medullary cystic disease (MCD) Dominant Very rare
Tuberous sclerosis (TSC) Dominant 1 in 10 000
Von Hippel–Lindau (VHL) disease Dominant 1 in 40