Abstract
Hemolytic uremic syndrome (HUS) associated with anti-Factor H (anti-FH) autoantibodies is a recently described pathophysiological entity. Monitoring of anti-FH IgG titer may be a sensitive marker of disease activity and guide treatment to eliminate circulating anti-FH antibodies. We report here a case of atypical HUS (aHUS) in which anti-FH autoantibodies were detected during the course of a fifth kidney transplant, 30 years after the first flare of aHUS. This exceptional case suggests that early, specific management based on immunosuppressive therapy and plasma exchanges monitored by anti-FH IgG titer may result in long-term graft survival.
Publication types
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Case Reports
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Research Support, Non-U.S. Gov't
MeSH terms
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Autoantibodies / blood*
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Blood Proteins / genetics
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Child
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Complement C3b Inactivator Proteins / genetics
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Complement Factor B / immunology
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Complement Factor H / immunology*
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Female
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Gene Deletion
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Hemolytic-Uremic Syndrome / classification
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Hemolytic-Uremic Syndrome / immunology
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Hemolytic-Uremic Syndrome / surgery*
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Humans
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Kidney Transplantation / immunology*
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Recurrence
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Reoperation / statistics & numerical data
Substances
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Autoantibodies
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Blood Proteins
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CFHR1 protein, human
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CFHR3 protein, human
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Complement C3b Inactivator Proteins
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Complement Factor H
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Complement Factor B