A Case of Hereditary C7 Deficiency Associated with Meningococcal Meningitis.
- Author:
Hyun Woo WE
1
;
Won Duck KIM
;
Sun Ju LEE
;
Dong Seok LEE
;
Doo Kwun KIM
;
Sung Min CHOI
;
Gyoung Yim HA
Author Information
1. Department of Pediatrics, College of Medicine, Dongguk University, Gyeongju, Korea. Neogubugi@yahoo.co.kr
- Publication Type:Case Report
- Keywords:
Complement 7;
Deficiency;
Complement membrane attack complex(MAC);
Neisseria meningitidis
- MeSH:
Bacterial Infections;
Complement C7;
Complement System Proteins;
Homicide;
Membranes;
Meningitis;
Meningitis, Meningococcal*;
Neisseria meningitidis
- From:Korean Journal of Pediatrics
2004;47(7):799-802
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The complement system is composed of more than 25 different proteins and is usually divided into classical and alternative pathways. Complement component 7(C7) is one of the five terminal complement proteins that, upon activation of either the classical or the alternative pathway, interacts sequentially to form a large protein-protein complex, called membrane attack complex(MAC). Assembly of the MAC on target cells results in the formation of transmembrane pores that can lead to the killing of the cells. C7 deficiency is an autosomal recessive disorder that is mostly reported in Caucasians. The gene for C7 has been assigned to chromosome 5p13. To date, 15 different molecular defects leading to total or subtotal C7 deficient defects have been reported. C7 deficiency is associated frequently with recurrently bacterial infections, especially meningitis caused by Neisseria meningitidis. We report a case of a hereditary C7 deficiency associated with meningococcal meningitis.
