Oligomeganephronic renal hypoplasia with tapetoretinal degeneration. Report of one case with ultrastructural study of the renal biopsy.
Bilateral renal hypoplasia with oligomeganephronia, associated with bilateral tapetoretinal degeneration was observed in a child; this association has been reported only once before. Light, ultrastructural and immunofluorescent microscopic studies of the renal tissue were performed. The glomeruli were few and hypertrophic, with numerous mesangial cells, mesangial deposits, focal glomerular sclerosis and prominent thickened basement membrane. Two types of tubular changes were observed: focal necrosis of proximal tubules and focal atrophy of tubules surrounded by a thickened basement membrane. Mild fibrosis with few lymphocytes could be observed in the interstitium. A congenital reduction in the number of nephrons, related to a yet unknown pathological process may explain these morphological changes in part.
D001485:Basement Membrane / Q000648:ultrastructure; D002648:Child; D006801:Humans; D007668:Kidney / Q000648:ultrastructure*; D007674:Kidney Diseases / Q000150:complications*; D007678:Kidney Glomerulus / Q000648:ultrastructure; D007684:Kidney Tubules / Q000648:ultrastructure; D008297:Male; D012162:Retinal Degeneration / Q000150:complications*