Insufficient protection by Neisseria meningitidis vaccination alone during eculizumab therapy
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KeywordsComplement System Hemolytic Uremic Syndrome Paroxysmal Nocturnal Hemoglobinuria Eculizumab Meningococcal Disease
Spurred by the reported spectacular results of eculizumab treatment in atypical hemolytic uremic syndrome (aHUS) due to aberrations in the complement system, an increasing number of children will receive this treatment in future. The main adverse effect of this therapy is an increased susceptibility to meningococcal infection due to inhibition of the complement system’s membrane-attack complex. In patients with paroxysmal nocturnal hemoglobinuria treated with eculizumab, the reported occurrence of meningococcal infection is between 0% and 1.5% [1, 2, 3]. These data, concerning adult patients, cannot be extrapolated to children because the age-specific incidence of meningococcal disease is much higher in children.
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