nephronophthisis

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nephronophthisis

 [nef″ron-of´thĭ-sis]
wasting disease of the kidney substance.
familial juvenile nephronophthisis a progressive hereditary kidney disease marked by anemia, polyuria, renal loss of sodium, progressing to chronic renal failure, tubular atrophy, interstitial fibrosis, glomerular sclerosis, and medullary cysts.

nephronophthisis

A progressive tubulo-interstitial kidney disorder histologically characterised by modified tubules, thickened basement membrane, interstitial fibrosis and, in advanced cases, medullary cysts.

Clinical findings
Chronic renal failure, anaemia, polyuria, polydipsia, isosthenuria and growth retardation.