Inherited complement deficiency in children surviving fulminant meningococcal septic shock

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Abstract
We evaluated the complement system in 29 children (mean age: 4.5 years) who survived fulminant meningococcal septic shock. No terminal complement deficiencies were found. One patient, who experienced the most dramatic disease course, had a decreased haemolytic activity in the haemolytis-in-gel test for the alternative pathway. The properdin concentration in serum of this patient was < 0.1 μg/ml (n = 17.1−27.7 μg/ml). Coagulation studies revealed a heterozygeous type I protein C deficiency as well. He was the only patient with aNeisseria meningitidis group Y infection.