Case Report

Clinical and Complement Long-Term Follow-Up of a Pediatric Patient with C3 Mutation-Related Atypical Hemolytic Uremic Syndrome

Figure 1

Patient samples and control sera assayed for eculizumab-C5 (E-C5) complexes and total complement activity. Three samples from the aHUS patient were examined: (1) sample obtained 1 hr before eculizumab infusion (“1 hr before ecu”), (2) sample obtained 1 hr after eculizumab infusion (“1 hr after ecu”), and (3) sample obtained 1 week after and 2 weeks before eculizumab infusion (“between ecu”). Control sera were obtained from 20 healthy controls. (a) Samples were incubated with increasing concentrations of eculizumab (0-100 μg/mL) and E-C5 complexes were measured (concentration is given in eculizumab-equivalent amounts). (b) Samples were incubated with increasing concentrations of eculizumab (0-100 μg/mL) and classical pathway complement activation was measured by the Wieslab® assay, detected as % of a normal human standard sera by the kit, defined to contain 100% activity (% of NHS).
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