RENAL INVOLVEMENT IN ADULT GAUCHERS-DISEASE AFTER SPLENECTOMY

  • 1 January 1979
    • journal article
    • research article
    • Vol. 103  (9) , 440-445
Abstract
Gaucher''s disease with severe clinical and pathologic renal involvement is exceptionally rare. Severe renal involvement and proteinuria was described in a young black woman with Gaucher''s disease. The accumulation of glucocerebroside (Gaucher bodies) in glomerular mesangial and endothelial cells and in interstitial cells of the kidney is indicative of the phagocytic potential and the deficiency of glucocerebroside-cleaving enzyme in these cells. Severe proteinuria in this patient was attributed to the extensive glomerular involvement by Gaucher''s disease.