UNLABELLED: 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 haemolysis-in-gel test for the alternative pathway. The properdin concentration in serum of this patient was < 0.1 microgram/ml (n = 17.1-27.7 micrograms/ml). Coagulation studies revealed a heterozygeous type I protein C deficiency as well. He was the only patient with a Neisseria meningitidis group Y infection. CONCLUSION: Fulminant meningococcal disease due to uncommon serogroups should lead to screening of the alternative pathway of complement activation.
UNLABELLED: 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 haemolysis-in-gel test for the alternative pathway. The properdin concentration in serum of this patient was < 0.1 microgram/ml (n = 17.1-27.7 micrograms/ml). Coagulation studies revealed a heterozygeous type I protein C deficiency as well. He was the only patient with a Neisseria meningitidis group Y infection. CONCLUSION: Fulminant meningococcal disease due to uncommon serogroups should lead to screening of the alternative pathway of complement activation.
Authors: J M Rasmussen; I Brandslund; B Teisner; H Isager; S E Svehag; L Maarup; L Willumsen; J O Rønne-Rasmussen; H Permin; P L Andersen Journal: Clin Exp Immunol Date: 1987-05 Impact factor: 4.330
Authors: K Fijnvandraat; B Derkx; M Peters; R Bijlmer; A Sturk; M H Prins; S J van Deventer; J W ten Cate Journal: Thromb Haemost Date: 1995-01 Impact factor: 5.249
Authors: Jean Charchaflieh; Jiandong Wei; Georges Labaze; Yunfang Joan Hou; Benjamin Babarsh; Helen Stutz; Haekyung Lee; Samrat Worah; Ming Zhang Journal: Clin Dev Immunol Date: 2012-09-23