Ultrasonography shows bilateral small kidneys with loss of corticomedullary junction and multiple cysts only in the medulla. Cysts may only be seen if they are large enough, they are rarely visible early in disease. Differential diagnosis Patients with medullary cystic disease present with similar features as<unk>  but they can be differentiated by: # Absence of growth retardation. # Age of presentation is third or fourth decade. # Hypertension may occur (in JN, hypertension is not seen). In polycystic kidney disease, there is bilateral enlargement of kidneys (small kidneys in JN).
Juvenile nephronophthisis
Medullary cystic kidney disease
Glomerulocystic kidney disease
Cystic kidney disease
Medullary sponge kidney
Autosomal dominant polycystic kidney disease
Doege–Potter syndrome
Autosomal recessive polycystic kidney disease