[Fatal pneumococcal meningitis in a 1-year-old child with homozygous C2 deficiency].
The 1-year old girl died of recurrent bacterial meningitis. Streptococcus pneumoniae was isolated from the cerebrospinal fluid. The analysis of the immune system revealed only a defect of the complement system. The following results were obtained: 1. No function of the classical complement pathway. 2. Reduced function of the alternative complement pathway. 3. No functional C2 activity. 4. No C2 protein. The parents had half normal C2 titers. HLA typing was only possible for the parents with the following results: A1, A32(w19), B18, DR2, DRw11(5) (father) and A3, A10, B18, B7, DR2 (mother). These data are compatible with a B18, DR2 haplotype of the child which is found in most cases of homozygous C2 deficiency. Our patient list another example for the high risk of recurrent severe infectious diseases in persons with a total complement defect.
Klinik für Kinder und Jugendliche, Universität Erlangen-Nürnberg.
U
U
Glöckel
P M
PM
Schneider
J D
JD
Beck
V
V
Brade
D003175:Complement C2 / Q000172:deficiency* / Q000235:genetics; D003170:Complement Pathway, Alternative / Q000235:genetics; D003171:Complement Pathway, Classical / Q000235:genetics; D005260:Female; D006680:HLA Antigens / Q000235:genetics; D006720:Homozygote; D006801:Humans; D007223:Infant; D008586:Meningitis, Pneumococcal / Q000235:genetics* / Q000276:immunology; D012008:Recurrence
D003175:Complement C2; D006680:HLA Antigens
Letale Pneumokokkenmeningitis bei einem 1 jährigen Kind mit homozygotem C2-Defekt.