|
|||||
|
|
Inherited complement deficiency in children surviving fulminant meningococcal septic shock |
|
| Authors: | H. H. F. Derkx E. J. Kuijper C. A. P. Fijen M. Jak J. Dankert S. J. H. van Deventer |
| Affiliation: | (1) Academic Medical Centre, University of Amsterdam, The Netherlands;(2) Department of Paediatrics, The Netherlands;(3) Department of Medical Microbiology and Reference Laboratory for Bacterial Meningitis (WHO), University of Amsterdam/ National Institute of Public Health and Environmental Protection, The Netherlands;(4) Academic Medical Centre, University of Amsterdam, Center of Haemostasis, Thrombosis, Atherosclerosis and Inflammation Research, The Netherlands |
| 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  g/ml (n = 17.1–27.7 g/ml). Coagulation studies revealed a heterozygeous type I protein C deficiency as well. He was the only patient with aNeisseria meningitidis group Y infection. |
| Keywords: | Neisseria meningitidis Complement system Properdin deficiency Protein C deficiency |
| 本文献已被 SpringerLink 等数据库收录！ | |