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Patients have an inability to form membrane attack complex (MAC) and bactericidal activity is depressed. Patients are susceptible to recurrent pyogenic infections. Typically, they present with meningococcal meningitis and disseminated extragenital gonococcal infection. Two thirds of patients have at least 1 episode of meningococcal disease. Many patients experience recurrent infections.
Diagnosis
Diagnostic recommendations
Additional Information
Therapeutic options
- Fresh frozen plasma is used for emergent replacement of complements components. Supportive therapy is used for complement deficiencies. Prophylactic antibiotics for the infections. Specific treatment of autoimmune disease is needed.
- Complement deficiency, eMedicine
- Complement deficiency, eMedicine