{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,23]],"date-time":"2026-08-23T16:42:30Z","timestamp":1787503350003,"version":"build-2736575974"},"reference-count":41,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2019,6,18]],"date-time":"2019-06-18T00:00:00Z","timestamp":1560816000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Alexion Pharma Germany"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,4,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Data are lacking on the relative incidence of thrombotic thrombocytopenic purpura (TTP), haemolytic uraemic syndrome (HUS) caused by Shiga toxin\u2013producing Escherichia coli (STEC) and atypical HUS (aHUS) in patients presenting with thrombotic microangiopathies (TMAs).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This was a prospective, cross-sectional, multicentre and non-interventional epidemiological study. Patients fulfilling criteria for TMAs (platelet consumption, microangiopathic haemolytic anaemia and organ dysfunction) were included in the study. The primary objective was to assess the relative incidence of TTP, STEC-HUS, aHUS and \u2018other\u2019 physician-defined diagnoses. The secondary objective was to develop an algorithm to predict a severe deficiency in ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) activity (\u226410%) using routine laboratory parameters. A post hoc classification using the recent Kidney Disease: Improving Global Outcomes diagnostic criteria was then undertaken to further classify patient groups.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>aHUS was diagnosed with a relative incidence of 61%, whereas TTP, STEC-HUS and \u2018other\u2019 were diagnosed in 13, 6 and 20% of patients, respectively. In the post hoc analysis, 27% of patients with a TMA were classified as \u2018primary aHUS\u2019 and 53% as \u2018secondary aHUS\u2019. Multivariate analysis revealed that severe deficiency in ADAMTS13 activity (\u226410%) was unlikely to underlie TMA if platelet and serum creatinine were above threshold values of 30 \u00d7 109\/L and 1.8\u2009mg\/dL, respectively (negative predictive value of 92.3 and 98.1, respectively, if one or both values were above the threshold).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>In this study, aHUS was the most common single diagnosis among patients presenting with a TMA. In the absence of an ADAMTS13 activity result, platelet count and serum creatinine may aid in the differential diagnosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/ckj\/sfz066","type":"journal-article","created":{"date-parts":[[2019,5,9]],"date-time":"2019-05-09T23:20:25Z","timestamp":1557444025000},"page":"208-216","source":"Crossref","is-referenced-by-count":23,"title":["Relative incidence of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome in clinically suspected cases of thrombotic microangiopathy"],"prefix":"10.1093","volume":"13","author":[{"given":"Ulf","family":"Sch\u00f6nermarck","sequence":"first","affiliation":[{"name":"Medizinische Klinik IV, Klinikum der Universit\u00e4t, LMU, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wolfgang","family":"Ries","sequence":"first","affiliation":[{"name":"Internal Medicine, Diakonissenkrankenhaus, Flensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bernd","family":"Schr\u00f6ppel","sequence":"first","affiliation":[{"name":"Medical Clinic I, Section of Nephrology, University of Ulm, Ulm, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Pape","sequence":"first","affiliation":[{"name":"Pediatric Nephrology, Medizinische Hochschule Hannover, Hannover, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Malgorzata","family":"Dunaj-Kazmierowska","sequence":"first","affiliation":[{"name":"Internal Medicine, Klinikum Koblenz, Koblenz, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Volker","family":"Burst","sequence":"first","affiliation":[{"name":"Department II of Internal Medicine, University of Cologne, Cologne, Germany"},{"name":"Center for Molecular Medicine Cologne, University of Cologne, Cologne, Germany"},{"name":"Faculty of Medicine, University Hospital Cologne, Cologne, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Steffen","family":"Mitzner","sequence":"first","affiliation":[{"name":"Division of Nephrology, Rostock University Medical Centre, Rostock, Germany"},{"name":"Fraunhofer IZI Project Group \u2018Extracorporeal Immunomodulation\u2019, Rostock, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nadezda","family":"Basara","sequence":"first","affiliation":[{"name":"Medizinische Klinik I, St. Franziskus-Hospital, Flensburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Starck","sequence":"first","affiliation":[{"name":"Clinic for Hematology, Clinic Munich-Schwabing, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Schmidbauer","sequence":"first","affiliation":[{"name":"Clinical Research Institute, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexander","family":"Mellmann","sequence":"first","affiliation":[{"name":"Institute of Hygiene, University Hospital M\u00fcnster, M\u00fcnster, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rita","family":"Dittmer","sequence":"first","affiliation":[{"name":"Medilys Laborgesellschaft, Hamburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Jeglitsch","sequence":"first","affiliation":[{"name":"Alexion Pharma Germany, Munich, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian S","family":"Haas","sequence":"first","affiliation":[{"name":"Internal Medicine, Nephrology and Intensive Care Medicine, Phillips University of Marburg, Marburg, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,6,18]]},"reference":[{"key":"2020041009152988200_sfz066-B1","doi-asserted-by":"crossref","first-page":"421","DOI":"10.1016\/j.nefro.2015.07.005","article-title":"An update for atypical haemolytic uraemic syndrome: diagnosis and treatment. 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