Complement Deficiencies in Patients Over Ten Years Old With Meningococcal Disease Due to Uncommon Serogroups

Fijen, C. A. P.

doi:10.1097/00006454-199001000-00029

Cited by 37 publications

(53 citation statements)

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“…Another notable feature of patients with MAC deficiencies is that they are susceptible to infection with a range of unusual capsular types [23,24], suggesting that the different meningococcal serogroups have inherently distinct capacities to prevent complement-mediated lysis. Surprisingly, individuals lacking MAC components typically present with their first attack of meningococcal disease during their early teens, rather than during childhood (which is the usual peak of incidence).…”

Section: Genetics Of Host Susceptibility To Meningococcal Infectionmentioning

confidence: 99%

Interactions between Neisseria meningitidis and the complement system

Schneider

Exley

Ram

et al. 2007

Trends in Microbiology

126

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Section: Genetics Of Host Susceptibility To Meningococcal Infectionmentioning

confidence: 99%

Interactions between Neisseria meningitidis and the complement system

Schneider

Exley

Ram

et al. 2007

Trends in Microbiology

126

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“…Dutch samples came from Subject 8, who has been described previously (Fijen et al 1982) and from Family 9 who were described previously by Daha et al (1982).…”

mentioning

confidence: 99%

The molecular basis of C6 deficiency in the western Cape, South Africa

et al. 1998

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Deficiency of the sixth component of human complement (C6) has been reported in a number of families from the western Cape, South Africa. Meningococcal disease is endemic in the Cape and almost all pedigrees of total C6 deficiency (C6Q0) have been ascertained because of recurrent disease. We have sequenced the expressed exons of the C6 gene from selected cases and have found three molecular defects leading to total deficiency: 879delG, which is the common defect in the Cape and hitherto unreported, and 1195delC and 1936delG, which have been previously reported in African-Americans. We also show that the 879delG and 1195delC defects are associated with characteristic C6/C7 region DNA marker haplotypes, although small variations were observed. The 1936delG defect was observed only once in the Cape, but its associated haplotype could be deduced. The data from the haplotypes indicate that these three molecular defects account for the defects in all the 38 unrelated C6Q0 individuals we have studied from the Cape. We have also observed the 879delG defect in two Dutch C6-deficient kindreds, but the 879delG defect in the Cape probably did not come from The Netherlands.

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“…As serogroup Y is considered to be an organism of relatively low virulence, 7 the possibility of underlying complement deficiency was raised. 8 Both inherited and acquired disorders of the complement system are associated with increased susceptibility to infections with encapsulated bacteria, particularly meningococcus. 9 10 Complement deficiency is present in about 5-18% of patients who have experienced a meningococcal infection.…”

Section: Discussionmentioning

confidence: 99%