{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T11:37:39Z","timestamp":1775734659858,"version":"3.50.1"},"reference-count":38,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2013,2,26]],"date-time":"2013-02-26T00:00:00Z","timestamp":1361836800000},"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":["J of Gastro and Hepatol"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:title>Abstact<\/jats:title><jats:sec><jats:title>Background and Aim<\/jats:title><jats:p>The prevalence of inherited antithrombin (<jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>), protein <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>), and protein <jats:styled-content style=\"fixed-case\">S<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content>) deficiencies in portal vein system thrombosis (<jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content>) and <jats:styled-content style=\"fixed-case\">B<\/jats:styled-content>udd\u2010<jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>hiari syndrome (<jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>) are substantially varied in different studies. No quantitative syntheses of these studies have been performed. A systematic review and meta\u2010analysis were conducted to examine the prevalence of inherited <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> deficiencies in these patients and to compare the prevalence with healthy subjects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">PubMed<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">EMBASE<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">C<\/jats:styled-content>ochrane <jats:styled-content style=\"fixed-case\">L<\/jats:styled-content>ibrary databases were employed to identify all studies in which inherited <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> deficiencies in <jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content> and\/or <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> were evaluated by family study or gene analysis. Prevalence and odds ratios of these inherited deficiencies were pooled; heterogeneity among studies was evaluated.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Nine studies were included in our meta\u2010analysis. The pooled prevalence of inherited <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> deficiencies were 3.9%, 5.6%, and 2.6% in <jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content>, and 2.3%, 3.8%, and 3.0% in <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>, respectively. Heterogeneity among studies was not significant except for the analysis of inherited <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content> deficiency in <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>. Three studies compared the prevalence of these inherited deficiencies between <jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content> patients and healthy subjects. The pooled odds ratios of inherited <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> deficiencies for <jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content> patients were 8.89 (95% confidence interval [<jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content>] 2.34\u201333.72, <jats:italic><jats:styled-content style=\"fixed-case\">P<\/jats:styled-content><\/jats:italic>\u2009=\u20090.0011), 17.63 (95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.97\u2013158.21, <jats:italic><jats:styled-content style=\"fixed-case\">P<\/jats:styled-content><\/jats:italic>\u2009=\u20090.0032), and 8.00 (95% <jats:styled-content style=\"fixed-case\">CI<\/jats:styled-content> 1.61\u201339.86, <jats:italic><jats:styled-content style=\"fixed-case\">P<\/jats:styled-content><\/jats:italic>\u2009=\u20090.011), respectively. Only one study demonstrated that no inherited deficiency was found in both <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content> patients and healthy subjects.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Inherited <jats:styled-content style=\"fixed-case\">AT<\/jats:styled-content>, <jats:styled-content style=\"fixed-case\">PC<\/jats:styled-content>, and <jats:styled-content style=\"fixed-case\">PS<\/jats:styled-content> deficiencies are rare in <jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">BCS<\/jats:styled-content>. These inherited deficiencies increase the risk of <jats:styled-content style=\"fixed-case\">PVST<\/jats:styled-content>.<\/jats:p><\/jats:sec>","DOI":"10.1111\/jgh.12085","type":"journal-article","created":{"date-parts":[[2012,12,7]],"date-time":"2012-12-07T12:58:22Z","timestamp":1354885102000},"page":"432-442","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":73,"title":["Prevalence of inherited antithrombin, protein <scp>C<\/scp>, and protein <scp>S<\/scp> deficiencies in portal vein system thrombosis and <scp>B<\/scp>udd\u2010<scp>C<\/scp>hiari syndrome: A systematic review and meta\u2010analysis of observational studies"],"prefix":"10.1111","volume":"28","author":[{"given":"Xingshun","family":"Qi","sequence":"first","affiliation":[{"name":"Xijing Hospital of Digestive Diseases Fourth Military Medical University  Xi'an China"},{"name":"Department of Gastroenterology No. 463 Hospital of Chinese PLA  Shenyang China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Valerio","family":"De Stefano","sequence":"additional","affiliation":[{"name":"Institute of Hematology Catholic University  Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology No. 463 Hospital of Chinese PLA  Shenyang China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ming","family":"Bai","sequence":"additional","affiliation":[{"name":"Xijing Hospital of Digestive Diseases Fourth Military Medical University  Xi'an China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Zhiping","family":"Yang","sequence":"additional","affiliation":[{"name":"Xijing Hospital of Digestive Diseases Fourth Military Medical University  Xi'an China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Guohong","family":"Han","sequence":"additional","affiliation":[{"name":"Xijing Hospital of Digestive Diseases Fourth Military Medical University  Xi'an China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daiming","family":"Fan","sequence":"additional","affiliation":[{"name":"Xijing Hospital of Digestive Diseases Fourth Military Medical University  Xi'an China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2013,2,26]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-119-8-199310150-00008"},{"key":"e_1_2_7_3_1","first-page":"516","article-title":"Inherited antithrombin deficiency causing thrombophilia","volume":"13","author":"Egeberg O","year":"1965","journal-title":"Thromb. 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