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Patient have meningococcemia and a decreased classical pathway activity and no detectable alternative pathway activity. No functional factor B can be detected, and factor B protein has abnormal molecular weight. The
Factor B deficiency is associated with severe fulminant pyogenic neisserial infections with a high mortality rate.
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.
- Complement deficiency, eMedicine
- Complement deficiency, eMedicine