Successful living-related renal transplantation in a patient with factor H antibody associated atypical hemolytic uremic syndrome (vol 19, pg E121, 2015)

PEDIATRIC TRANSPLANTATION(2018)

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摘要
CFH-Ab-associated aHUS requires different diagnostic and therapeutic approaches and then the genetically defined aHUS forms. The risk of post-transplant recurrence with graft dysfunction in CFH-Ab aHUS is not well documented. It is suggested that recurrence can be expected if a significant CFH-Ab load persists at the time of transplantation. A pretransplant procedure to reduce CFH-Ab titer seems reasonable, but accurate recommendations are lacking. Whether further prophylactic interventions after transplantation are necessary has to be decided on an individual basis. We report the case of a late diagnosed CFH-Ab HUS with initial ESRD and a successful living-related renal transplantation over a post-transplant period of four and a half years on the basis of a prophylactic pretransplant IVIG admission.
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关键词
complement factor H antibodies,atypical HUS,renal transplantation,intravenous immunoglobulins,plasma therapy
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