Abstract
The hemolytic uremic syndrome (HUS) usually occurs as a single, acute episode. Recurrences with intervening periods of complete recovery have rarely been reported. Serum complement levels are stated to be normal. A girl who developed HUS at the age of 2 years had four recurrences during the next 2½ years. Each time she had microangiopathic anemia, thrombocytopenia and oliguric renal failure. During recovery from the third episode, renal biopsy showed focal and segmental thickening of the glomerular capillary walls due to endothelial edema, mesangial proliferation and thickening of the lamina interna rara. Between the acute episodes there were no detectable renal or hematological abnormalities. A transient fall in serum B1C (C3) complement was observed during each of the last three episodes; C1q was depressed during the third, while during the last one, C1q and C4 as well as components of the alternate pathway (C3PA and properdin) were normal. Enquiries to three other clinics revealed that the serum level of C3 was low in 8 of the 17 patients with HUS in whom it was measured. No correlation with course or outcome could be established. We postulate that hypocomplementemia results from non-specific consumption of complement during the acute process of intravascular coagulation. However, the possibility that the complement system is involved in the pathogenesis of certain forms of HUS cannot be excluded.
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Drukker, A., Winterborn, M., Bennett, B. et al. RECURRENT HEMOLYTIC UREMIC SYNDROME WITH HYPOCOMPLEMENTEMIA. Pediatr Res 8, 454 (1974). https://doi.org/10.1203/00006450-197404000-00687
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DOI: https://doi.org/10.1203/00006450-197404000-00687