Inherited complement deficiency in children surviving fulminant meningococcal septic shock

European Journal of Pediatrics
H H DerkxS J van Deventer

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 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. Fulminant meningococcal disease due to uncommon serogroups should lead to screening of the alternative pathway of complement activation.

References

Jul 1, 1991·Clinical Microbiology Reviews·J E Figueroa, P Densen
Oct 1, 1990·APMIS : Acta Pathologica, Microbiologica, Et Immunologica Scandinavica·A G Sjöholm
Sep 1, 1990·Clinical and Experimental Immunology·M SchlesingerZ Fishelson
Jan 1, 1989·Scandinavian Journal of Infectious Diseases·H E NielsenI Lind
Jan 1, 1989·The Pediatric Infectious Disease Journal·P L HavensT J Troshynski
Jun 1, 1986·Pediatric Annals·B L Congeni
Apr 1, 1987·Thrombosis Research·H Vinazzer, U Pangraz
Jun 15, 1987·Clinica Chimica Acta; International Journal of Clinical Chemistry·A SturkJ W ten Cate
Mar 23, 1987·The American Journal of Medicine·E W GelfandS Ruddy
Aug 1, 1974·The Journal of Experimental Medicine·D T FearonS Ruddy
Aug 3, 1984·Journal of Immunological Methods·U R Nilsson, B Nilsson
Apr 21, 1983·The New England Journal of Medicine·R T EllisonL B Reller
Aug 1, 1983·The Journal of Infectious Diseases·J MerinoB Casanueva
May 1, 1994·Clinical Infectious Diseases : an Official Publication of the Infectious Diseases Society of America·C A FijenJ Dankert

❮ Previous
Next ❯

Citations

Jul 1, 1997·Critical Care Clinics·J A Carcillo, R E Cunnion
May 12, 1998·The Pediatric Infectious Disease Journal·M RottemM Schlezinger
Oct 12, 2010·Clinical Microbiology Reviews·Sanjay RamPeter A Rice
Jan 11, 2000·Clinical Microbiology Reviews·M van DeurenJ W van der Meer
Feb 28, 2002·Archives of Disease in Childhood·S HoareA J Cant
Oct 11, 2012·Clinical & Developmental Immunology·Jean CharchafliehMing Zhang
Mar 31, 2009·Clinical Immunology : the Official Journal of the Clinical Immunology Society·Lone SchejbelPeter Garred
Oct 1, 2015·Disease Markers·Jean CharchafliehMing Zhang
Oct 10, 2013·Virulence·Lisa A Lewis, Sanjay Ram
Jun 19, 2012·The Pediatric Infectious Disease Journal·Andrew TerranellaThomas A Clark
Aug 17, 2021·Intensive Care Medicine Experimental·O SommerfeldF Bloos

❮ Previous
Next ❯

Related Concepts

Related Feeds

Alternative Complement Pathway

The Alternative Complement Pathway is part of the innate immune system, and activation generates membrane attack complexes that kill pathogenic cells. Discover the latest research on the Alternative Complement Pathway.