nephronophthisis
Last reviewed 01/2018
Nephronophthisis is a congenital disease of the kidney with unknown incidence, but known to be a significant cause of inherited chronic renal failure.
There is progressive cortical atrophy as a result of the development of cysts from the tubules.
Patients usually present in their first decade with:
- polyuria
- proteinuria
- polydipsia
- anaemia
- growth failure