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Abstract
The laboratory examination showed the following results: hemoglobin (HGB) 65 g/L, platelet (PTL) 79 × 109/L, serum creatinine (Scr) 1089 μmol/L, blood urea nitrogen 50.4 μmol/L, complement C3 0.44 g/L, and complement C4 0.24 g/L. After treatment with respiratory support, hormone pulse, hemodialysis, PTL transfusion, and antibiotics for 2 weeks, the respiratory symptoms of the patient disappeared, and his body temperature returned to normal. [...]we stopped the plasmapheresis treatment. Because the application of eculizumab might induce a serious Neisseria meningitidis infection, a meningitis vaccination was provided to the patient before the start of the antibody therapy. Discussion The typical symptoms, laboratory test results, clear factor H gene mutation, and renal biopsy evidence prompted us to confirm the diagnosis of aHUS. If a patient has a genetic defect in factor H, factor I, or MCP, or if a patient has autoantibodies against factor H, factor I, and MCP, C3b cannot be inactivated, and the excessively activated complement system in the alternative pathway will attack PTLs and endothelial cells to cause the typical clinical manifestation of aHUS. [...]starting at week 4, the inhibitory effect gradually decreases. [...]the factor H-deficient patient in this case requires long-term maintenance treatment at least for 6 months. C3b can still label red blood cells, which are cleared by the reticuloendothelial system, thus causing the persistent destruction of HGB. [7] Financial support and sponsorship This work is supported by grants from Project Yangfan-Special Funds for the Development of Clinical Medicine of Beijing (No. Tailored eculizumab therapy in the...