{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T12:37:06Z","timestamp":1788871026590,"version":"build-2803163510"},"reference-count":43,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2014,9,3]],"date-time":"2014-09-03T00:00:00Z","timestamp":1409702400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002347","name":"Federal Ministry of Education and Research","doi-asserted-by":"crossref","award":["FKZ: 01 E0 1002"],"award-info":[{"award-number":["FKZ: 01 E0 1002"]}],"id":[{"id":"10.13039\/501100002347","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100001659","name":"German Research Foundation","doi-asserted-by":"crossref","award":["BR 4182\/1-1"],"award-info":[{"award-number":["BR 4182\/1-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Jena Center for interdisciplinary research"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2015,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background &amp; Aims<\/jats:title>\n                    <jats:p>\n                      Systemic inflammation in advanced cirrhosis represents a spectrum ranging from subclinical pathological bacterial translocation and immune activation to overt bacterial infection and sepsis. We hypothesized that systemic inflammation in cirrhosis is accompanied by a failure of\n                      <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                      13 to control the prothrombotic function of von Willebrand factor (\n                      <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                      ), which is increased in portal hypertension and hepatic fibrosis.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Patients with Child A cirrhosis (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a025), Child B\/C cirrhosis without clinical features of systemic inflammation (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a031), and Child B\/C cirrhosis with overt bacterial infections or systemic inflammatory response syndrome (\n                      <jats:italic>n<\/jats:italic>\n                      \u00a0=\u00a024) were analysed for\n                      <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                      13 and associated parameters and were followed to determine transplant\u2010free survival.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Plasma concentration and activity of\n                      <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                      13 were decreased in patients with systemic inflammation. Furthermore,\n                      <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                      13 inversely correlated with the extent of bacterial translocation and the severity of acute\u2010phase reaction. As a function of reduced\n                      <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                      13 activity and increased\n                      <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                      antigen, plasma from patients with superimposed inflammation strongly aggregated the platelet receptor glycoprotein Ib in presence of ristocetin.\n                      <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                      :\n                      <jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>\n                      o correlated with higher concentrations of leucocytes and lipopolysaccharide\u2010binding protein, organ dysfunction, augmented turnover of cross\u2010linked intravascular fibrin, and the occurrence of acute kidney injury during follow\u2010up.\n                      <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                      :\n                      <jats:styled-content style=\"fixed-case\">RC<\/jats:styled-content>\n                      o of 390% or more predicted transplant\u2010free survival in univariate analysis [\n                      <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\n                      \u00a0=\u00a08.24 (3.30\u201320.54)] and after adjustment for\n                      <jats:styled-content style=\"fixed-case\">MELD<\/jats:styled-content>\n                      [\n                      <jats:styled-content style=\"fixed-case\">HR<\/jats:styled-content>\n                      \u00a0=\u00a03.58 (1.30\u20139.88)]. However, adverse outcome was not associated with the accumulation of high\u2010molecular weight\n                      <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                      multimers.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Systemic inflammation complicating advanced cirrhosis is accompanied by reduced activity of\n                      <jats:styled-content style=\"fixed-case\">ADAMTS<\/jats:styled-content>\n                      13 promoting a prothrombotic function of\n                      <jats:styled-content style=\"fixed-case\">VWF<\/jats:styled-content>\n                      , which can be employed to predict clinical outcome.\n                    <\/jats:p>\n                  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