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Pediatr Nephrol. 1988 Apr;2(2):239-43.

Renal lesions in Cockayne syndrome.

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Department of Pediatrics, Iwate Prefectural Central Hospital, Morioka, Japan.


Two siblings with typical features of the Cockayne syndrome were studied at autopsy. Many glomeruli revealed a paucity of capillary loops and had thickened capillary walls. Some glomeruli with advanced lesions showed collapse of the glomerular tufts or complete hyalinization. Atrophy of tubules and interstitial fibrosis were also observed. There were no significant arteriosclerotic changes in the vessels. Ultrastructural studies demonstrated thickened glomerular basement membranes with bends and folds. These histopathological findings are different to those previously reported with the exception of the 1966 report by Ohno and Hirooka.

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