Figure 312

Diffuse mesangial sclerosis. This disorder is exhibited within the first few months of life with massive proteinuria, often with

1. Abrahamson D, Van der Heurel GB, Clapp WL, et al.: Nephritogenic antigens in the glomerular basement membrane. In Immunologic Renal Diseases. Edited by Nielson EG, Couser, WG. Philadelphia: Lippincott-Raven, 1997.

hematuria and progressive renal insufficiency. Currently, no evidence exists that this disorder is an inherited process with genetic linkage. The glomeruli characteristically are small compact masses of extracellular matrix with numerous or all capillary lumina being obliterated. As here, the visceral epithelial cells typically are arranged as a corona or crown overlying the contracted capillary tufts. Earlier stages of glomerular involvement are characterized by variable increase in mesangial cellularity. Immunofluorescence is typically negative for immunoglobulin deposits because this disorder is not immune mediated. In some patients, diffuse mesangial sclerosis may be part of the triad of the Drash syndrome characterized by ambiguous genitalia, Wilms' tumor, and diffuse mesangial sclerosis. In some patients, only two of the three components may be present; however, some investigators consider all patients with diffuse mesangial sclerosis to be at risk for the development of Wilms' tumor even in the absence of genital abnormalities. Thus, close observation or bilateral nephrecto-my as prophylaxis against the development of Wilms' tumor is employed occasionally.

2. Gregory M, Atkin C: Alport's syndrome, Fabry disease and nail-patella syndrome. In Diseases of the Kidney, Vol. I. edn 6. Edited by Schrier RW, Gottschalk CW. Boston: Little Brown, 1995.

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