Abstract
Complement studies have been performed in three siblings with hereditary kidney disease. In all three patients oliguric renal failure and malignant hypertension developed after a period of 6 to 10 years with slight proteinuria. High levels of the C3 activation product C3b could be detected in serum before as well as after bilateral nephrectomy, whereas the C3 concentrations were slightly decreased and the C4 levels normal or moderately increased. Sera from the patients were able to break down C3 in normal serum in vitro. The demonstration of in vivo activation of complement before and after nephrectomy may suggest that the underlying mechanism of the renal involvement in these patients is an inherited, abnormal immunological response.
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