{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,2,12]],"date-time":"2024-02-12T05:41:02Z","timestamp":1707716462194},"reference-count":19,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2017,6,15]],"date-time":"2017-06-15T00:00:00Z","timestamp":1497484800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Nephrology"],"published-print":{"date-parts":[[2017,7]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Complement\u2010mediated atypical haemolytic uraemic syndrome (aHUS) is a rare disease with high mortality and morbidity if left untreated. The diagnostic work\u2010up is complicated and the manifestations overlap with other conditions. Therefore, we hypothesize that complement\u2010mediated aHUS is an under diagnosed disease.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A cohort of 768 referrals referred to the Coagulation Unit in Malmo, Sweden, for analysis of a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13), 2007\u20132012, were retrospectively reviewed. Subjects were included on the basis of presence of haemolytic anaemia, thrombocytopaenia, renal failure and ADAMTS13\u2009&gt;\u20090.05. They were excluded if tested positive for <jats:italic>Escherichia coli<\/jats:italic>. Included subjects were categorized as \u201csuspected HUS\u201d with and without potential causes and triggers. Levels of C3 and C4, presence of complement factor H (CFH)\u2010specific antibodies and associated deficiency in complement factor H related protein 1 (CFHR1) were analyzed on frozen samples.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 134\/316 (42%) unique subjects fulfilled inclusion criteria; 103 were categorized as \u201csuspected HUS associated with potential causes\/triggers\u201d and 31 subjects categorized as \u201csuspected HUS\u201d without such association. One case of complement\u2010mediated aHUS had been confirmed during the treatment period. Laboratory analyses performed showed that in total 78 cases had findings consistent with complement\u2010mediated aHUS: 24 cases indicated presence of CFH\u2010specific antibodies whereof five cases had isolated low C3 titres and six cases had deficiency of CFHR1. Additionally 54 cases indicated isolated alternative pathway consumption.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The results suggest that the presence of complement\u2010mediated aHUS was under diagnosed in this cohort calling for improvement of diagnostic availability.<\/jats:p><\/jats:sec>","DOI":"10.1111\/nep.12818","type":"journal-article","created":{"date-parts":[[2016,5,13]],"date-time":"2016-05-13T16:33:36Z","timestamp":1463157216000},"page":"555-561","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Indications of underdiagnosis of atypical haemolytic uraemic syndrome in a cohort referred to the Coagulation Unit in Malmo, Sweden, for analysis of ADAMTS13 2007\u20132012"],"prefix":"10.1111","volume":"22","author":[{"given":"Alexander","family":"\u00c5kesson","sequence":"first","affiliation":[{"name":"Department of Translational Medicine Lund University  Malm\u00f6 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna M","family":"Blom","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine Lund University  Malm\u00f6 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jenny","family":"Klintman","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine Lund University  Malm\u00f6 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva","family":"Zetterberg","sequence":"additional","affiliation":[{"name":"Department of Translational Medicine Lund University  Malm\u00f6 Sweden"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,6,15]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-6-60"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejim.2013.05.008"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.lpm.2011.11.013"},{"key":"e_1_2_8_5_1","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1182\/asheducation.V2012.1.617.3798924","article-title":"Atypical hemolytic uremic syndrome: what is it, how is it diagnosed, and how is it treated?","volume":"2012","author":"Nester CM","year":"2012","journal-title":"Hematol. 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