Complement factor H dysfunction in atypical hemolytic uremic syndrome
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HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation
1Department of Pediatrics, Clinical Sciences Lund, Lund University, Lund, Sweden; 2Department of Biological Infection, Hans Knoell Institute for Natural Products Research, Jena, Germany; 3Department of Internal Medicine, Regional Hospital, Halmstad, Sweden; 4Department of Pediatric Nephrology, University of Texas Health Science Center at San Antonio, TX; 5Division of Renal Medicine, Karolinska ...
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To the Editor: Atypical hemolytic–uremic syndrome is a rare microangiopathic hemolytic condition characterized by thrombocytopenia and acute renal failure.1 The prognosis for patients with atypical hemolytic–uremic syndrome with a factor H mutation is poor; 60% have end-stage renal disease or die within a year.2 The expected rate of graft failure due to recurrent atypical hemolytic–uremic syndr...
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BACKGROUND Non-Shiga toxin-associated hemolytic uremic syndrome is known to be caused by dysregulation of the alternative complement pathway. Infections, drugs, pregnancy, bone marrow transplantation, malignancy, and autoimmune disorders have all been reported to trigger episodes of atypical hemolytic uremic syndrome. To the best of our knowledge, there have been no previous reports of an assoc...
متن کاملFactor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation.
Atypical hemolytic uremic syndrome (aHUS) may be associated with mutations in the C-terminal of factor H (FH). FH binds to platelets via the C-terminal as previously shown using a construct consisting of short consensus repeats (SCRs) 15 to 20. A total of 4 FH mutations, in SCR15 (C870R) and SCR20 (V1168E, E1198K, and E1198Stop) in patients with aHUS, were studied regarding their ability to all...
متن کاملAtypical hemolytic uremic syndrome due to factor H autoantibody.
Atypical hemolytic uremic syndrome (aHUS) is a disease caused by pathologies in the alternative complement system. The prevalence of aHUS is 10% of all aHUS cases. The subgroup of aHUS designated as DEAP (DEficiency of CFHR Proteins and CFH Autoantibody Positive)-HUS because of autoantibody to complement factor H (CFH) and CFH-related protein deficiency is seen very rarely, and the prevalence i...
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تاریخ انتشار 2011