Renal Lesions in Cockayne's Syndrome

Abstract

Three males, 4 years, 6 years and 5 years of age, with typical Cockayne's syndrome were described. Differential diagnostic criteria between Cockayne's syndrome and progeria were discussed and the authors were of the opinion that the two syndromes represented two different disease entities. Histologic findings of the kidney biopsied in 2 cases with this syndrome revealed thickening of glomerular basement membrane and mesangium, collapse or atrophy of capillary loops, hyalinization of glomeruli, atrophy of tubules, thickening of the basement membrane of atrophic tubules and interstitial fibrosis. These renal lesions have not been reported so far in Cockayne's syndrome. In this respect, possible relationships between Cockayne's syndrome and so-called hereditary nephropathy were discussed.