Table 2.

Comparison of clinical, pathological, and genetic findings of adolescent NPH and polycystic kidney disease in mice (pcy)a

Adolescent NPHPolycystic Kidney Disease (pcy)
aNPH, nephronophthisis.
InheritanceAutosomal recessiveAutosomal recessive
Manifestation of renal failureLate (median age, 19 yr)Late (adult mice)
Range of age at renal failureWide range (12 to 47 yr)Wide range depending on genetic background
Renal cyst locationPredominantly at the corticomedullary junctionIn the beginning at the corticomedullary junction, later throughout the entire kidney
Kidney sizeNormal or reducedEnlarged
Extrarenal disease manifestationNot knownCerebral vascular aneurysms in 12%
HistologyTubular basement membrane changes, tubular dilation and atrophy, sclerosing tubulointerstitial nephropathyTubular basement membrane changes, tubular dilation and atrophy, sclerosing tubulointerstitial nephropathy