Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome

Thérèsa Kwon, Marie‐Agnès Dragon‐Durey, Marie‐Alice Macher, Véronique Baudouin, Anne Maisin, Michel Peuchmaur, Véronique Frémeaux‐Bacchi, Chantal Loirat

Nephrology Dialysis Transplantation · 2008 · 89 citations · 6 references

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Abstract

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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