Related Experiment Videos
Inherited complement deficiency in children surviving fulminant meningococcal septic shock
H H Derkx1, E J Kuijper, C A Fijen
1University of Amsterdam, Department of Paediatrics, The Netherlands.
European Journal of Pediatrics
|September 1, 1995
Summary
Fulminant meningococcal disease survivors rarely have complement deficiencies. However, severe cases may indicate alternative pathway complement activation issues, warranting further screening.
Area of Science:
- Immunology
- Infectious Diseases
- Pediatrics
Background:
- Meningococcal septic shock is a severe infection.
- The complement system plays a crucial role in innate immunity against Neisseria meningitidis.
Purpose of the Study:
- To evaluate the complement system in children who survived meningococcal septic shock.
- To identify potential complement deficiencies associated with severe disease courses.
Main Methods:
- Assessed complement system activity in 29 pediatric survivors.
- Utilized haemolysis-in-gel tests for alternative pathway activity.
- Measured properdin concentration and performed coagulation studies.
Main Results:
- No terminal complement deficiencies were detected in the study group.
- One patient with a severe disease course exhibited decreased alternative pathway haemolytic activity.
- This patient also had low properdin concentration and heterozygous type I protein C deficiency.
- This patient was uniquely infected with Neisseria meningitidis group Y.
Conclusions:
- Fulminant meningococcal disease, particularly from rare serogroups, suggests the need for alternative complement pathway screening.
- Complement deficiencies may predispose individuals to severe meningococcal infections.