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Atypical HUS predominantly affects the kidneys but has the potential to cause multi\u2010organ system dysfunction. This uncommon disorder is caused by a genetic abnormality in the complement alternative pathway resulting in over\u2010activation of the complement system and formation of microvascular thrombi. Abnormalities of the complement pathway may be in the form of mutations in key complement genes or autoantibodies against specific complement factors. We discuss the pathophysiology, clinical manifestations, diagnosis, complications, and management of aHUS. We also review the efficacy and safety of the novel therapeutic agent, eculizumab, in aHUS, pregnancy\u2010associated aHUS, and aHUS in renal transplant patients.<\/jats:p>","DOI":"10.1111\/1744-9987.12763","type":"journal-article","created":{"date-parts":[[2018,10,8]],"date-time":"2018-10-08T08:58:28Z","timestamp":1538989108000},"page":"4-21","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":159,"title":["Atypical Hemolytic\u2010Uremic Syndrome: An Update on Pathophysiology, Diagnosis, and Treatment"],"prefix":"10.1111","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3892-8376","authenticated-orcid":false,"given":"Rupesh","family":"Raina","sequence":"first","affiliation":[{"name":"Department of Nephrology Cleveland Clinic Akron General and Akron Children's Hospital  Akron OH USA"},{"name":"Cleveland Clinic Akron General\/Akron Nephrology Associates  Akron OH 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