Portal de Periódicos da CAPES

Progressive morphologic renal changes in the oculo-cerebro-renal syndrome of Lowe

1968; Elsevier BV; Volume: 44; Issue: 2 Linguagem: Inglês

10.1016/0002-9343(68)90163-0

1555-7162

C.L. Witzleben, Edgar J. Schoen, Wu-Hao Tu, Larry W. McDonald,

Ocular Disorders and Treatments

Abstract Fraternal twins with the oculo-cerebral-renal syndrome of Lowe have been followed clinically for nine and a half years. Renal biopsies with electron microscopic study were performed at ages twenty-two months and nine years. There has been no evidence of significant deterioration in renal function, but morphologic renal disease has progressed. The initial biopsy specimens showed minimal or equivocal pathologic changes; the recent study revealed advancing glomerular disease with basement membrane thickening, podocyte fusion, increased glomerular cellularity and glomerular fibrosis, as well as scattered thickening of renal tubular basement membranes, multifocal tubular atrophy, the formation of casts, with extension of these casts into the interstitium, and interstitial fibrosis.