[The kidney failure of Bourneville's tuberous sclerosis: a new form of glomerular damage as a result of hyperfiltration?].
Chronic renal failure, rarely seen in tuberous sclerosis (TS), is usually related to renal hamartomas and/or cysts, but has only been very rarely linked to glomerular changes. In a patient with TS, we have performed repeated renal biopsies at early and advanced stages of renal failure. We have correlated the renal function deterioration with the evolution of focal and segmental glomerulosclerosis. Renal insufficiency would be in relation not only with renal involvement by multiple tumors, but also with the progression of focal and segmental glomerulosclerosis. This would be caused by hemodynamic changes in remnant glomeruli, after reduction in renal mass induced by renal tumors, and would be a new example of renal injury due to hyperfiltration.
D000328:Adult; D005919:Glomerular Filtration Rate; D005921:Glomerulonephritis / Q000209:etiology*; D005923:Glomerulosclerosis, Focal Segmental / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology*; D007678:Kidney Glomerulus / Q000473:pathology*; D008297:Male; D014402:Tuberous Sclerosis / Q000150:complications* / Q000473:pathology / Q000503:physiopathology
L'insuffisance rénale de la sclérose tubéreuse de Bourneville: une nouvelle forme d'atteinte glomérulaire par hyperfiltration?