[Complement factor I deficiency revealed by repeated systemic Streptococcus pneumoniae infection].
Recurrent and serious otitis media, and 2 Streptococcus pneumoniae bacteraemia episodes evoked an immune system deficiency in a 6-year-old girl. Upon investigation of the complement system, CH50 activity was moderately reduced and C4 antigen level was normal contrasting with low C3 antigen level. Factor 1 was undetectable. Factor I deficiency is rare, and its diagnosis has important practical consequences. Thanks to preventive antibiotic therapy with penicillin V and vaccinations against Neisseria meningitidis and S. pneumoniae, life expectancy and quality of this child can be significantly improved.
Monfort-Gouraud
M
M
Service de pédiatrie, centre hospitalier intercommunal de Montfermeil-Le-Raincy, 10, avenue du Général-Leclerc, 93370 Montfermeil, France. marie.monfortgouraud@laposte.net
Vignon
C
C
Vincens
A
A
Blouin
J
J
Frémeaux-Bacchi
V
V
Talon
P
P
D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D025861:Blood Coagulation Disorders, Inherited / Q000175:diagnosis*; D002648:Child; D005260:Female; D005340:Fibrinogen / Q000235:genetics*; D006801:Humans; D010033:Otitis Media / Q000175:diagnosis / Q000188:drug therapy / Q000382:microbiology; D011008:Pneumococcal Infections / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology; D016896:Treatment Outcome
D000900:Anti-Bacterial Agents; D005340:Fibrinogen