{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,30]],"date-time":"2026-04-30T09:31:28Z","timestamp":1777541488696,"version":"3.51.4"},"reference-count":0,"publisher":"Index Copernicus","issue":"1","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pol Przegl Chir"],"published-print":{"date-parts":[[2018,2,28]]},"abstract":"<jats:p>Introduction: Bleeding from esophageal varices is a serious clinical condition in hemophilia patients due to congenital deficiency\nor lack of clotting factors VIII (in hemophilia A) and IX (in hemophilia B), decreased clotting factor II, VII, IX, X synthesis\nin the course of chronic liver disease and hipersplenic thrombocytopenia. The aim of this study was to assess the efficacy and\nsafety of endoscopic sclerotherapy in acute esophageal variceal bleeding and in secondary prophylaxis of hemorrhage. The\naim was also to investigate the optimal activity of deficiency factors VIII or IX and duration of replacement therapy required\nto ensure proper hemostasis after sclerotherapy procedures.\nMaterial and methods: 22 hemophilia patients (A-19, B-4) with coexistent liver cirrhosis and active esophageal variceal bleeding\ntreated with endoscopic sclerotherapy were subjected to prospective analysis. The patients who survived were qualified\nto repeated sclerotherapy procedures every 3 weeks within secondary prophylaxis of bleeding (investigated group).\nA 3-day substitution therapy enhanced the infusion of the deficient or lacking factor in doses allowing to reach 80-100% of\nnormal value activity of factor VIII on the 1st day and 60-80% in the next two days. The desired activity of factor IX was 60-\n80% and 40-60% respectively. The control group consisted of 20 non-hemophiliac patients with liver cirrhosis comparable in\nterms of age, sex, stage of advancement of liver cirrhosis, who underwent the same medical proceedings as the investigated\ngroup.\nResults: Active esophageal bleeding was stopped in 21 of 22 (95%) hemophilia patients. Complications were observed in 3 patients;\n2 patients died. The rate of hemostasis, complications and deaths in the control group were comparable and no statistical\ndifferences were found. In hemophilia patients subjected to secondary prophylaxis of hemorrhage, in 18 of 20 (80%),\ncomplete eradication of esophageal varices was achieved after 4 to 7 sclerotherapy procedures in 1 patient (average 5.4). Recurrent\nbleeding was observed in 15% of patients, complication in 20%; 1 patient died. Time lapse from bleeding to eradication\nwas 12-21 weeks (average 15.2).\nIn the control group the rate of variceal eradication, complication and deaths was comparable and no statistical differences\nwere found. The usage of factor VIII concentrates was as follows: in hemophilia A, in a severe form - 80.9 U\/kg b.w.\/day, in hemophilia\nA in a severe form with an inhibitor &lt;5 BU \u2013 95.2 U\/kg b.w.\/day, in mild form \u2013 64.2 U\/kg b.w.\/day and in severe hemophilia\nB \u2013 91.6 U\/kg b.w.\/day. Conclusions: Sclerotherapy is an effective method in the management of esophageal variceal\nbleeding in hemophilia patients. It is also effective for total eradication of varices when applied as a secondary prophylaxis\nof hemorrhage. In our opinion, a 3-day replacement therapy at the applied doses is sufficient to ensure hemostasis and avoid\nbleeding complications.\n\n<\/jats:p>","DOI":"10.5604\/01.3001.0011.5957","type":"journal-article","created":{"date-parts":[[2018,3,2]],"date-time":"2018-03-02T04:54:23Z","timestamp":1519966463000},"page":"29-34","source":"Crossref","is-referenced-by-count":3,"title":["Sclerotherapy of esophageal varices in hemophilia\npatients with liver cirrhosis \u2013 a prospective, controlled\nclinical study"],"prefix":"10.5604","volume":"90","author":[{"given":"Andrzej B.","family":"Szczepanik","sequence":"first","affiliation":[{"name":"Department of General, Oncological and Metabolic Surgery, Institute of Hematology and Blood Transfusion in Warsaw. Head: prof. dr hab. A.B. Szczepanik"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Konrad","family":"Pielaci\u0144ski","sequence":"additional","affiliation":[{"name":"Department of General, Oncological and Metabolic Surgery, Institute of Hematology and Blood Transfusion in Warsaw. Head: prof. dr hab. A.B. Szczepanik"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna M.","family":"Oses-Szczepanik","sequence":"additional","affiliation":[{"name":"Department of Internal Diseases and Geriatrics, Jagiellonian University Collegium Medicum in Cracow. Head: prof. dr hab. T. Grodzicki"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S\u0142awomir","family":"Huszcza","sequence":"additional","affiliation":[{"name":"Department of General, Oncological and Metabolic Surgery, Institute of Hematology and Blood Transfusion in Warsaw. Head: prof. dr hab. A.B. Szczepanik"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrzej","family":"Misiak","sequence":"additional","affiliation":[{"name":"Department of General, Oncological and Metabolic Surgery, Institute of Hematology and Blood Transfusion in Warsaw. Head: prof. dr hab. A.B. Szczepanik"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wojciech P.","family":"D\u0105browski","sequence":"additional","affiliation":[{"name":"Department of General, Oncological and Metabolic Surgery, Institute of Hematology and Blood Transfusion in Warsaw. Head: prof. dr hab. A.B. Szczepanik"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S\u0142awomir","family":"Gajda","sequence":"additional","affiliation":[{"name":"Department of General, Oncological and Metabolic Surgery, Institute of Hematology and Blood Transfusion in Warsaw. Head: prof. dr hab. A.B. Szczepanik"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"3689","container-title":["Polish Journal of Surgery"],"original-title":[],"deposited":{"date-parts":[[2018,3,2]],"date-time":"2018-03-02T04:54:27Z","timestamp":1519966467000},"score":1,"resource":{"primary":{"URL":"https:\/\/ppch.pl\/gicid\/01.3001.0011.5957"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,2,28]]},"references-count":0,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2018,2,28]]}},"URL":"https:\/\/doi.org\/10.5604\/01.3001.0011.5957","relation":{},"ISSN":["0032-373X","2299-2847"],"issn-type":[{"value":"0032-373X","type":"print"},{"value":"2299-2847","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,2,28]]}}}