{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T01:40:23Z","timestamp":1789609223390,"version":"build-2803163510"},"reference-count":29,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,12,17]],"date-time":"2017-12-17T00:00:00Z","timestamp":1513468800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Ther Apher Dial"],"published-print":{"date-parts":[[2018,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Atypical hemolytic uremic syndrome (aHUS) is a rare life\u2010threatening thrombotic microangiopathy (TMA) affecting multiple organ systems. Recently, aHUS has been shown to be associated with uncontrolled complement activation due to mutations in the alternative pathway of complement components paving the way for targeted drug therapy. By meta\u2010analysis of case reports, we discuss the impact of new treatment strategies on the resolution time of aHUS symptoms and mortality, and the distribution of genetic mutations. A PubMed\/Medline search was conducted for \u201catypical hemolytic uremic syndrome\u201d case reports published between November 2005 and November 2015. R Version 3.2.2 was used to calculate descriptive statistics and perform univariate analyses. Wilcoxon rank\u2010sum test was used to compare time to symptoms resolution, creatinine and platelet count normalization across the treatment and mutation carrier groups. A total of 259 aHUS patients were reported in 176 articles between 2005 and 2015. In the last 5\u2010year period compared to the precedent, there was an increase in the number of aHUS cases reported (180 vs. 79 cases) and the use of eculizumab also increased (6.3% to 46.1%, <jats:italic>P<\/jats:italic>\u2009&lt;\u20090.000), although plasma exchange usage did not change (<jats:italic>P<\/jats:italic>\u2009=\u20090.281). CFH antibodies were present in a significantly higher number of patients treated with plasma exchange therapy (19.1%, <jats:italic>P<\/jats:italic>\u2009=\u20090.000) while none of the non\u2010plasma exchange therapy group had CFH antibodies. Most common mutation was CFH (50%, 69\/139) followed by CFHR1 (35%, 30\/85), MCP (22.8%, 23\/101) and CFI (16.6%, 17\/102). Time to symptoms resolution and serum creatinine or platelet count normalization were not significantly different between eculizumab and non\u2010eculizumab group (<jats:italic>P<\/jats:italic>\u2009=\u20090.166, <jats:italic>P<\/jats:italic>\u2009=\u20090.361, <jats:italic>P<\/jats:italic>\u2009=\u20090.834), and between plasma exchange and non\u2010plasma exchange group (<jats:italic>P<\/jats:italic>\u2009=\u20090.150, <jats:italic>P<\/jats:italic>\u2009=\u20090.135, <jats:italic>P<\/jats:italic>\u2009=\u20090.784). However, both eculizumab and plasma exchange groups had early platelet recovery (22 vs. 30\u2009days and 25.5 vs. 32.5\u2009days), faster creatinine normalization (27 vs. 30.5\u2009days and 27 vs. 37\u2009days) and interestingly, a longer period for symptoms resolution (45.5 vs. 21\u2009days and 30 vs. 18.5\u2009days) compared to non\u2010eculizumab and non\u2010plasma exchange groups. Mortality rate decreased with the use of eculizumab significantly (<jats:italic>P<\/jats:italic>\u2009=\u20090.045) compared to non\u2010eculizumab group and there was no change in mortality rate with the use of plasma exchange therapy (<jats:italic>P<\/jats:italic>\u2009=\u20090.760) compared to non\u2010plasma exchange group. Plasma exchange continues to be the initial treatment of choice for aHUS. Although significant reduction in the mortality rate was noted with the use of eculizumab, there were no differences in time to resolution of symptoms or serum creatinine or platelet normalization with the use of either eculizumab or plasma therapy. Atypical HUS is acute and life\u2010threatening, so plasma exchange may be initiated before the confirmed diagnosis and in patients positive for CFH antibodies. Eculizumab therapy should be considered once aHUS is confirmed by genetic testing.<\/jats:p>","DOI":"10.1111\/1744-9987.12641","type":"journal-article","created":{"date-parts":[[2017,12,18]],"date-time":"2017-12-18T04:46:43Z","timestamp":1513572403000},"page":"178-188","source":"Crossref","is-referenced-by-count":33,"title":["Atypical Hemolytic Uremic Syndrome: A Meta\u2010Analysis of Case Reports Confirms the Prevalence of Genetic Mutations and the Shift of Treatment Regimens"],"prefix":"10.1111","volume":"22","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1697-4526","authenticated-orcid":false,"given":"Vinod","family":"Krishnappa","sequence":"first","affiliation":[{"name":"Cleveland Clinic Akron General\/Akron Nephrology Associates  Akron Ohio USA"},{"name":"Department of Internal Medicine and Nephrology Cleveland Clinic Akron General  Akron Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohit","family":"Gupta","sequence":"additional","affiliation":[{"name":"Department of Nephrology Weill Cornell Medicine\/NewYork Presbyterian  New York USA"},{"name":"Department of Internal Medicine and Nephrology Cleveland Clinic Akron General  Akron Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mohamed","family":"Elrifai","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Akron General\/Akron Nephrology Associates  Akron Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bahar","family":"Moftakhar","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Summa Health System  Akron Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael J","family":"Ensley","sequence":"additional","affiliation":[{"name":"Department of Political Science Kent State University  Kent Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tushar J","family":"Vachharajani","sequence":"additional","affiliation":[{"name":"Division of Nephrology and Hypertension Salisbury VA Health Care System  Salisbury NC USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sidharth Kumar","family":"Sethi","sequence":"additional","affiliation":[{"name":"Kidney Institute Medanta, The Medicity Hospital  Gurgaon India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rupesh","family":"Raina","sequence":"additional","affiliation":[{"name":"Cleveland Clinic Akron General\/Akron Nephrology Associates  Akron Ohio USA"},{"name":"Department of Internal Medicine and Nephrology Cleveland Clinic Akron General  Akron Ohio USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,12,17]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2005-10-007252"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/ng.2590"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1208981"},{"key":"e_1_2_6_5_1","first-page":"39","article-title":"Critical appraisal of eculizumab for atypical hemolytic uremic syndrome","volume":"7","author":"Palma LMP","year":"2016","journal-title":"J Blood 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