Renal histopathology in the hemolytic-uremic syndrome following shigellosis.
The hemolytic-uremic syndrome (HUS) following dysentery caused by S. dysenteriae Type 1, characterized by microangiopathic hemolytic anemia and acute renal insufficiency, is clinically similar but not identical to the idiopathic HUS. We studied renal necropsy specimens of nine children who died of HUS following shigellosis by light and immunofluorescent microscopy and compared them to 12 controls: six cases with severe shigellosis without HUS, and six with pneumonia or sepsis. Eight of nine HUS cases showed cortical necrosis, extensive glomerular thrombosis or arterial thrombosis. Cases without HUS showed only scattered glomerular fibrin thrombin and widening of the mesangium. Among seven HUS cases studied by immunofluorescent microscopy, three demonstrated deposition of glomerular IgM and complement (C3) and one of the three had IgG and IgA as well; four cases had neither immunoglobulin or complement deposits. Among nine controls, two demonstrated IgM and three IgG, but none had C3. Both HUS and non-HUS cases had fibrin deposition. In the three HUS cases studied by electron microscopy intracapillary material (fibrin and platelets) was seen in all three, and sparse electron-dense deposits in mesangial matrix in one. The data indicate that the renal histopathology in the HUS following shigellosis consistently presents as a severe thrombotic microangiopathy, but lacks the characteristic endothelial and mesangial lesions of idiopathic HUS. The infrequent demonstration of glomerular immunoglobulin deposition fails to support an immunoglobulin-mediated pathogenesis.
D000743:Anemia, Hemolytic / Q000473:pathology; D001424:Bacterial Infections / Q000150:complications; D002648:Child; D002675:Child, Preschool; D003176:Complement C3 / Q000032:analysis; D004405:Dysentery, Bacillary / Q000150:complications*; D005260:Female; D005455:Fluorescent Antibody Technique; D006463:Hemolytic-Uremic Syndrome / Q000209:etiology / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007223:Infant; D007668:Kidney / Q000473:pathology*; D007678:Kidney Glomerulus / Q000276:immunology; D008297:Male
D003176:Complement C3; D007070:Immunoglobulin A; D007075:Immunoglobulin M