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- W1980897780 abstract "Background The complement system is a vital component of innate immunity. Deficiencies in any part of the complement pathway characteristically present with recurrent infections. C2 factor deficiency is the most common complement deficiency. The presentation can vary from being asymptomatic to developing recurrent infections. Thus empiric vaccinations have been recommended, despite the lack of substantial evidence to support this practice. Recently, Jonnson and colleagues revisited this controversial topic and demonstrated the importance of vaccinations in this patient population. They further established that C2 deficient patients can mount an immune response to vaccination, undergo class-switching and develop a ‘more efficient’ phagocytosis. C2 deficiency is inherited in an autosomal recessive pattern. Homozygotes generally present with increased severity of disease as compared to heterozygotes. Alper and colleagues state that 25% of C2-deficient homozygotes have increased susceptibility to severe bacterial infections. Furthermore, they found that these C2-deficient patients had significantly lower mean levels of IgG4 and IgA than those patients that did not demonstrate an increased susceptibility for recurrent infections." @default.
- W1980897780 created "2016-06-24" @default.
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- W1980897780 date "2014-03-01" @default.
- W1980897780 modified "2023-10-16" @default.
- W1980897780 title "Unusual presentation of a patient with complement deficiency and immunoglobulin deficiency" @default.
- W1980897780 cites W1950734237 @default.
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- W1980897780 doi "https://doi.org/10.1186/1710-1492-10-s1-a39" @default.
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