INHERITED COMPLEMENT DEFICIENCY IN CHILDREN SURVIVING FULMINANT MENINGOCOCCAL SEPTIC SHOCK

We evaluated the complement system in 29 children (mean age: 4.5 years ) who survived fulminant meningococcal septic shock. No terminal compl ement deficiencies were found. One patient, who experienced the most d ramatic disease course, had a decreased haemolytic activity in the hae molysis-in-gel test for the alternative pathway. The properdin concent ration in serum of this patient was < 0.1 mu g/ml (n = 17.1-27.7 mu g/ ml). Coagulation studies revealed a heterozygeous type I protein C def iciency as well. He was the only patient with a Neisseria meningitidis group Y infection. Conclusion Fulminant meningo-coccal disease due to uncommon groups should lead to screening ;of the alternative pathway of complement activation.