{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,27]],"date-time":"2025-10-27T04:48:56Z","timestamp":1761540536596},"reference-count":44,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,7,19]],"date-time":"2004-07-19T00:00:00Z","timestamp":1090195200000},"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":["Br J Haematol"],"published-print":{"date-parts":[[2004,8]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Beh\u00e7et's disease is a multi\u2010systemic inflammatory disorder of unknown cause. Most abnormalities have been associated with endothelial injury caused by vasculitis. Thrombosis occurs in about 25% of patients, although the mechanism is unknown. The objective of this study was to evaluate the protein C activation system in Beh\u00e7et's disease and its correlation with venous thromboembolism (VTE). Thirty\u2010nine patients (12 with VTE) and 78 age\u2010 and sex\u2010matched controls were included in the study, and levels of protein C, protein S, activated protein C (APC), protein C inhibitor (PCI), soluble thrombomodulin (TM), antithrombin (AT), <jats:italic>\u03b1<\/jats:italic><jats:sub>1<\/jats:sub>\u2010antitrypsin, fibrinogen, factor VIII, von Willebrand factor (VWF) and C\u2010reactive protein (CRP) were measured. APC and TM levels were significantly lower in patients than in controls, whereas protein S, AT, <jats:italic>\u03b1<\/jats:italic><jats:sub>1<\/jats:sub>\u2010antitrypsin, fibrinogen, factor VIII, VWF and CRP levels were significantly higher in patients than in controls. APC, PCI and TM levels were lower in patients with VTE (0\u00b765\u2003\u00b1\u20030\u00b719\u2003ng\/ml, 86%\u2003\u00b1\u200322% and 15\u00b75\u2003\u00b1\u20037\u00b71\u2003ng\/ml respectively) than in those without VTE (0\u00b778\u2003\u00b1\u20030\u00b717\u2003ng\/ml, 100%\u2003\u00b1\u200315% and 22\u00b71\u2003\u00b1\u200315\u00b73\u2003ng\/ml) (<jats:italic>P<\/jats:italic>\u2003&lt;\u20030\u00b705). In patients, APC levels below 0\u00b775\u2003ng\/ml (10th percentile of the control group) increased the risk of VTE about fivefold (odds ratio\u2003=\u20035\u00b71; 95% confidence interval\u2003=\u20031\u00b71\u201323\u00b74). These results show that reduced APC levels are associated with the high incidence of VTE in Beh\u00e7et's disease.<\/jats:p>","DOI":"10.1111\/j.1365-2141.2004.05072.x","type":"journal-article","created":{"date-parts":[[2004,7,30]],"date-time":"2004-07-30T13:37:00Z","timestamp":1091194620000},"page":"550-556","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["Activated protein C levels in Beh\u00e7et's disease and risk of venous thrombosis"],"prefix":"10.1111","volume":"126","author":[{"given":"Silvia","family":"Navarro","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jos\u00e9 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