two related cases of primary complement deficiency

primary complement deficiencies are rare and two related patients are reported here. the first patient is a 41- year- old man with eighteen episodes of pneumo¬coccal meningitis and other purulent infections. the serum c3 level was checked at three separate times, showing that this was a primary c3 deficient case; other immunological tests were however normal. this patient now takes prophylactic antibiotics and the meningitis has not recurred, but he does have glomerulone¬phritis. the second case is a 40 - year-old woman with repeated episodes of orofacial and laryngeal edema and dyspnea. the serum c1inh levels were 4.3 to 7 mg/dl which were very low compared with normal healthy subjects (c,inh was 40-50 mg/dl in ten normal controls) and c4 was lower than normal but other immunological tests were normal. other causes of angioedema such as lymphoproliferative disorders were excluded. she had hereditary angioedema with¬out a family background. the condition may be due to genetic mutation. the angioedema was controlled with danazol and stanasol. as our patients are re¬lated, this may suggest a genetic relationship between these two disorders.