Hemolytic uremic syndrome with hypocomplementemia, serum C3NeF, and glomerular deposits of C3.
A 4-year-old boy had hemolytic uremic syndrome (HUS) associated with depression of serum C3 level, a B-hemolytic streptococcal throat infection, and an elevated level of antistreptolysin O titer. In addition to the characteristic histologic changes associated with this syndrome, substantial infiltration of polymorphonuclear leukocytes and nodular deposits of C3 globulin were seen in the glomeruli of the first biopsy specimen. Two months after clinical remission, he had a recurrence of hemolytic anemia, thrombocytopenia, and acute renal failure. The serum C3 concentration had decreased again, and serum C3NeF was detected in the serum. The typical changes associated with HUS were still present on electron microscopy. Bilateral nephrectomy and renal transplantation were done because of the development of uncontrollable severe hypertension and increasing azotemia. This patient had three manifestations of HUS, but because of several differences, such as hypocomplementemia, serum C3NeF, a recurrence, and persistent glomerular deposits of C3 globulin, he appears to have had a different form of the syndrome.
D002675:Child, Preschool; D003176:Complement C3 / Q000037:antagonists & inhibitors / Q000378:metabolism*; D003165:Complement System Proteins / Q000378:metabolism*; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000276:immunology* / Q000473:pathology / Q000648:ultrastructure; D008297:Male; D008854:Microscopy, Electron; D008856:Microscopy, Fluorescence
D003176:Complement C3; D003165:Complement System Proteins