| A Case of Hereditary C7 Deficiency Associated with Meningococcal Meningitis |
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Hyun Woo We1, Won Duck Kim1, Sun Ju Lee1, Dong Seok Lee1, Doo Kwun Kim1, Sung Min Choi1, Gyoung Yim Ha2 |
1Department of Pediatrics, College of Medicine, Dongguk University, Gyeongju, Korea
2Department of Clinical Pathology, College of Medicine, Dongguk University, Gyeongju, Korea |
| 수막구균성 뇌수막염을 동반한 유전성 C7 보체 결핍증 1례 |
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위현우1, 김원덕1, 이선주1, 이동석1, 김두권1, 최성민1, 하경임2 |
1동국대학교 의과대학 소아과학교실
2동국대학교 의과대학 진단검사의학교실 |
Correspondence:
Won Duck Kim, Email: Neogubugi@yahoo.co.kr
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| Abstract |
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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. |
| Key Words:
Complement 7, Deficiency, Complement membrane attack complex(MAC), Neisseria meningitidis |
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