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 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.


CONCLUSIONS
Fulminant meningococcal disease due to uncommon serogroups should lead to screening of the alternative pathway of complement activation.
Derkx
H H
HH
University of Amsterdam, Department of Paediatrics, The Netherlands.
Kuijper
E J
EJ
Fijen
C A
CA
Jak
M
M
Dankert
J
J
van Deventer
S J
SJ
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003170:Complement Pathway, Alternative; D003165:Complement System Proteins / Q000172:deficiency*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008589:Meningococcal Infections / Q000097:blood*; D011414:Properdin / Q000172:deficiency; D012772:Shock, Septic / Q000097:blood* / Q000382:microbiology*; D017741:Survivors
D011414:Properdin; D003165:Complement System Proteins