Terminal Complement Component Deficiencies in Japan

Fukumori, Yasuo; Horiuchi, Takahiko

doi:10.1159/000019078

Cited by 15 publications

(6 citation statements)

References 8 publications

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“…137 The incidence of these deficiencies varies worldwide but C9 deficiency is frequent among Japanese population (up to 1/10 0 0). 138 The bacteria are also adept at evading complement-mediated killing through molecular mimicry, as well as phase and antigenic variation. 138…”

Section: Occupational Risksmentioning

confidence: 99%

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Meningococcal disease surveillance in the Asia–Pacific region (2020): The global meningococcal initiative

Aye

Bai

Borrow

et al. 2020

Journal of Infection

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The incidence of IMD is low and similar across the represented countries (< 0.2 cases per 10 0,0 0 0 persons per year), with the predominant serogroups of Neisseria meningitidis being B, W and Y, although serogroups A and X are present in some areas. Resistance to ciprofloxacin is also of concern, with the close monitoring of antibiotic-resistant clonal complexes (e.g., cc4821) being a priority. Meningococcal vaccination is only included in a few National Immunization Programs, but is recommended for high-risk groups, including travellers (such as pilgrims) and people with complement deficiencies or human immunodeficiency virus (HIV). Both polysaccharide and conjugate vaccines form part of recommendations. However, cost and misconceptions remain limiting factors in vaccine uptake, despite conjugate vaccines preventing the acquisition of carriage.

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Section: Occupational Risksmentioning

confidence: 99%

“…138 The bacteria are also adept at evading complement-mediated killing through molecular mimicry, as well as phase and antigenic variation. 138…”

Section: Occupational Risksmentioning

confidence: 99%

Meningococcal disease surveillance in the Asia–Pacific region (2020): The global meningococcal initiative

Aye

Bai

Borrow

et al. 2020

Journal of Infection

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“…In contrast to the situation in Japan, where C9 deficiency has a very high incidence (0.045-0.104%) [86][87][88][89], it is a rare finding in Europe [90,91]. This may be, in part, due to the different methods used in Japan and Europe [91]: the CH50 at low strength, as routinely done in Japan, favours the detection of C9D, whereas the sucrose-GVB buffer routinely used in Europe may only yield values just outside or even still within the normal range.…”

Section: Molecular Bases Of C9 Deficiencies and Dna Typingmentioning

confidence: 71%

Reference Typing Report for Complement Components C6, C7 and C9 Including Mutations Leading to Deficiencies

Würzner

Witzel-Schlömp

Tokunaga

et al. 1998

Exp Clin Immunogenet

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The results of the present (VIIth Complement Genetics Workshop and Conference, Mainz, May 1998) and past reference typing workshops for the terminal complement components C6, C7 and C9 are compiled and discussed both on the protein level and on the DNA level. This report also focusses on the molecular bases of expressed and silent polymorphisms and reviews the molecular bases of subtotal and complete deficiencies of these proteins and their associations with protein and DNA markers. The results of the protein typing for C6 are published in the following paper of this issue.

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“…8,9 Although C9D subjects are usually healthy, they carry a significantly higher risk of developing meningococcal meningitis than normal controls. 8,9 Although C9D subjects are usually healthy, they carry a significantly higher risk of developing meningococcal meningitis than normal controls.…”

Section: Discussionmentioning

confidence: 99%

Behçet's disease associated with complement component 9 (C9) deficiency

Horiuchi

Tsukamoto

Sawabe

et al. 2000

Modern Rheumatology

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Behçet's disease is a multisystem inflammatory disorder with unknown etiology. It has been shown that the titer of plasma complement component 9 (C9) is a good indicator of the disease activity. Therefore, the involvement of C9 in the pathogenesis of Behçet's disease has been suggested. We report a case of Behçet's disease associated with complete C9 deficiency (C9D) carrying the homozygous nonsense mutation at Arg-95 of C9 (R95X). The patient presented the typical characteristics of Behçet's disease, such as uveitis, recurrent oral aphthae and genital ulcers, and arthritis, suggesting that C9 does not play an essential role in the pathogenesis of Behçet's disease.

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Terminal Complement Component Deficiencies in Japan

Cited by 15 publications

References 8 publications

Meningococcal disease surveillance in the Asia–Pacific region (2020): The global meningococcal initiative

Meningococcal disease surveillance in the Asia–Pacific region (2020): The global meningococcal initiative

Reference Typing Report for Complement Components C6, C7 and C9 Including Mutations Leading to Deficiencies

Behçet's disease associated with complement component 9 (C9) deficiency

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