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Complement deficiencies represent approximately 1–6% of all primary immunodeficiencies and they can be primary or secondary. The inheritance is usually autosomal recessive, except for properdin deficiency, which is X-linked and factor B, C1-INH and MCP/CD46 deficiency, which are autosomal dominant. Severe or multiple infections, caused by capsulated bacteria as Streptococcus, Haemophylus and Neisseriae or autoimmune diseases are the main clinical manifestations presented in complement deficiency. Polymorphism in components of lectin pathway have been associated with the susceptibility to infections or autoimmunity. Hereditary Angioedema causes edema affecting subcutaneous and mucosa, responsible for recurrent attacks in skin, gastrointestinal tract and obstruction of upper airways. The approach to recurrent infections in complement deficient patients includes vaccination and antibiotic prophylaxis. Specific therapies were developed improving the prognosis of these defects.
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Teele et al. (2000) studied this question.
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