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      Atypical Hemolytic Uremic Syndrome Associated With Clostridium Difficile Infection

      case-report
      1 , 2 , 3 , 4 , 5 , 6 , 7 , 2 ,
      ,
      Cureus
      Cureus
      atypical hemolytic uremic syndrome, eculizumab

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          Abstract

          Atypical hemolytic uremic syndrome (aHUS), defines as non-Shiga toxin HUS, is thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, consumptive thrombocytopenia, and renal impairment. aHUS is associated with high morbidity and mortality, necessitating the need for an early diagnosis to limit target organ damage. Mutations or autoantibodies against specific complement factors over‐activate the complement system forming microthrombi. aHUS has the potential to cause multi‐organ system dysfunction, but it predominantly affects the kidneys. aHUS is treated with eculizumab, a terminal blocker of the complement system. Clostridium difficile infection is a rare precipitant of aHUS. We present a case of aHUS associated with Clostridium difficile infection in a 60-year-old female patient that was successfully treated with eculizumab.

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          Atypical hemolytic-uremic syndrome.

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              Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura.

              The term thrombotic microangiopathy (TMA) defines a lesion of vessel wall thickening (mainly arterioles or capillaries), intraluminal platelet thrombosis, and partial or complete obstruction of the vessel lumina. Depending on whether renal or brain lesions prevail, two pathologically indistinguishable but somehow clinically different entities have been described: the hemolytic uremic syndrome (HUS) and the thrombotic thrombocytopenic purpura (TTP). Injury to the endothelial cell is the central and likely inciting factor in the sequence of events leading to TMA. Loss of physiological thromboresistance, leukocyte adhesion to damaged endothelium, complement consumption, abnormal von Willebrand factor release and fragmentation, and increased vascular shear stress may then sustain and amplify the microangiopathic process. Intrinsic abnormalities of the complement system and of the von Willebrand factor pathway may account for a genetic predisposition to the disease that may play a paramount role in particular in familial and recurrent forms. Outcome is usually good in childhood, Shiga toxin-associated HUS, whereas renal and neurological sequelae are more frequently reported in adult, atypical, and familial forms of HUS and in TTP. Plasma infusion or exchange is the only treatment of proven efficacy. Bilateral nephrectomy and splenectomy may serve as rescue therapies in very selected cases of plasma resistant HUS or recurrent TTP, respectively.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                5 July 2020
                July 2020
                : 12
                : 7
                : e9005
                Affiliations
                [1 ] Internal Medicine, Xinjiang Medical University, Urumqi, CHN
                [2 ] Internal Medicine, Icahn School of Medicine at Mount Sinai, Queens General Hospital, New York, USA
                [3 ] Internal Medicine, Chandka Medical College, Larkana, PAK
                [4 ] Internal Medicine, Memorial Hermann Medical Center, Houston, USA
                [5 ] Internal Medicine, California Institute of Behavioral Neurosciences & Psychology, Fairfield, USA
                [6 ] Internal Medicine, Sheikh Zayed Hospital, Lahore, PAK
                [7 ] Internal Medicine, Allama Iqbal Medical College, Lahore, PAK
                Author notes
                Article
                10.7759/cureus.9005
                7402547
                b6d452bc-51c8-42c5-809a-c32a08c65885
                Copyright © 2020, Khurshid et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 12 June 2020
                : 5 July 2020
                Categories
                Internal Medicine
                Nephrology

                atypical hemolytic uremic syndrome,eculizumab
                atypical hemolytic uremic syndrome, eculizumab

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