Nephrology Dialysis Transplantation · 2008 · 89 citations · 6 references
Life SciencesImmunologyPathologyAutoimmunityClinical ImmunologyTransplant ImmunologyAutoantibodiesAutoantibody-mediated DiseaseChronic RejectionComplement DisordersComplement SystemTransplant RejectionC3 ConvertaseComplement AutoantibodiesComplement AutoimmunityComplement Factor H DeficiencyComplement In TransplantationPathogenic AutoantibodiesPre-transplant Patient AssessmentPre-transplant Recipient Assessment
Atypical haemolytic uraemic syndrome (aHUS) is known to be associated with mutations involving regulatory proteins of the complement alternative pathway, including factor H (CFH), factor I and MCP, in ∼50% of patients [1,2]. Eight patients have been reported with an acquired CFH deficiency because of the presence of anti-CFH autoantibodies [3,4]. These autoantibodies have been shown to influence the binding of CFH to the C3bBb convertase, thereby compromising CFH activity in vivo [3,4]. We now report the pre-transplant therapeutic management and post-transplant course of one of these patients.
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Silvia Ferrari, Friedrich Scheiflinger, Manfred Rieger et al. · Blood · 2006 · 294 citations · Full text
Rebecca E. Saunders, Cynthia Abarrategui-Garrido, Véronique Frémeaux‐Bacchi et al. · Human Mutation · 2006 · 168 citations · Full text
Life Sciences, Genetics, Hematology +16