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      A rare encounter with two cases of Budd‑Chiari syndrome in the Emergency Department: A case report

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          Abstract

          Budd-Chiari syndrome (BCS) is a rare disorder clinically characterized by abdominal pain, hepatomegaly and ascites. The condition is often related to thrombosis of the hepatic veins or the terminal portion of the inferior vena cava. A myeloproliferative disorder is the most identified underlying prothrombotic risk factor, although almost one-half of affected patients are now recognized as having multiple underlying prothrombotic risk factors. Doppler ultrasound may be enough to confirm the diagnosis of BCS; however, computed tomography or magnetic resonance imaging is often employed. Anticoagulant therapy is the cornerstone of BCS treatment, but most patients also need additional treatment strategies. Most patients with BCS are now treated by endovascular intervention, which has improved survival rate in those afflicted by this disease. The long-term course of the disease can be complicated by progression or recurrence of the underlying myeloproliferative disorder. The present study reports the cases of two patients with BCS with the aim of alerting healthcare workers in Emergency Departments of this less common diagnosis in patients presenting with frequent complaints of abdominal pain.

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          Etiology, management, and outcome of the Budd-Chiari syndrome.

          The Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases. To characterize the causes and treatment of incident BCS. Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006. Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland. Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded. Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival. 163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years. Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients. Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure. Fifth Framework Programme of the European Commission.
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            Cause of portal or hepatic venous thrombosis in adults: the role of multiple concurrent factors.

            According to a recent hypothesis, venous thrombosis results from the concurrence of several factors. This hypothesis was assessed in patients with portal or hepatic venous thrombosis by simultaneously investigating most of the currently identified prothrombotic disorders, local precipitating factors, and other risk factors such as oral contraceptive use. Patients with a tumorous obstruction and patients with cirrhosis with portal vein thrombosis were excluded. The prothrombotic disorders that were investigated included classical and occult myeloproliferative disorders; antiphospholipid syndrome; protein C; protein S and antithrombin deficiency; factor V Leiden; factor II; and methylene-tetrahydrofolate-reductase gene mutations. We found 1 or several prothrombotic disorders and a local precipitating factor in 26 and 10 of the 36 patients with portal vein thrombosis, respectively; and in 28 and none of the 32 patients with hepatic vein thrombosis, respectively. We found a combination of prothrombotic disorders in 5 and 9 patients with portal and hepatic vein thrombosis, respectively, whereas such a combination is expected in less than 1% of asymptomatic subjects. Of the 10 patients with a local precipitating factor, 8 had a prothrombotic disorder. Of the 13 patients who use oral contraceptives, 10 had a prothrombotic disorder. We conclude that portal or hepatic venous thrombosis should be regarded as an index for 1 or several prothrombotic disorders, whether or not local precipitating factors or oral contraceptive use are found. Concurrence of prothrombotic disorders is more common than expected. Extensive investigation of prothrombotic disorders and anticoagulation should be considered in patients with portal or hepatic venous thrombosis.
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              Good long-term outcome of Budd-Chiari syndrome with a step-wise management.

              Budd-Chiari syndrome (BCS) is a rare, life-threatening disease caused by obstruction of hepatic venous outflow. The aim of the study was to assess long-term outcome and identify prognostic factors in BCS patients managed by a step-wise approach using anticoagulation, angioplasty/thrombolysis, transjugular intrahepatic portosystemic shunting (TIPS), and orthotopic liver transplantation (OLT). We reviewed long-term data on 157 patients previously included by the European Network for Vascular Disorders of the Liver, a multicenter prospective study of newly diagnosed BCS patients in nine European countries. Patients were followed for a median of 50 months (range, 0.1-74.0). During the study, 88 patients (56%) received at least one invasive intervention (22 patients angioplasty/thrombolysis, 62 TIPS, and 20 OLT) and 36 (22.9%) died. Most interventions and/or deaths occurred in the first 2 years after diagnosis. The Rotterdam score was excellent in predicting intervention-free survival, and no other variable could significantly improve its prognostic ability. Moreover, BCS-TIPS prognostic index (PI) score (based on international normalized ratio, bilirubin, and age) was strongly associated with survival and had a discriminative capacity, which was superior to the Rotterdam score.
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                Author and article information

                Journal
                Exp Ther Med
                Exp Ther Med
                ETM
                Experimental and Therapeutic Medicine
                D.A. Spandidos
                1792-0981
                1792-1015
                December 2022
                24 October 2022
                24 October 2022
                : 24
                : 6
                : 730
                Affiliations
                [1 ]Department of Surgery, Faculty of Medicine, ‘Victor Babes’ University of Medicine and Pharmacy, 300041 Timisoara, Romania
                [2 ]Department of Emergency, Emergency Clinical Municipal Hospital, 300079 Timisoara, Romania
                [3 ]Department of Emergency, ‘Pius Brînzeu’ Emergency Clinical County Hospital, 300736 Timisoara, Romania
                [4 ]Department of Cardiology, Faculty of Medicine, Multidisciplinary Center for Research, Evaluation, Diagnosis and Therapies in Oral Medicine, ‘Victor Babes’ University of Medicine and Pharmacy, 300041 Timisoara, Romania
                [5 ]Department of Ophthalmology, ‘Victor Babes’ University of Medicine and Pharmacy, 300041 Timisoara, Romania
                [6 ]Department of Ear, Nose and Throat, ‘Victor Babes’ University of Medicine and Pharmacy, 300041 Timisoara, Romania
                [7 ]Department of Surgery, Faculty of Medicine, Multidisciplinary Center for Research, Evaluation, Diagnosis and Therapies in Oral Medicine, ‘Victor Babes’ University of Medicine and Pharmacy, 300041 Timisoara, Romania
                Author notes
                Correspondence to: Dr Sorin Dan Chiriac, Department of Surgery, Faculty of Medicine, ‘Victor Babes’ University of Medicine and Pharmacy, Piata Eftimie Murgu 2, 300041 Timisoara, Romania iurciuc.mirceaumft.ro s_chiriac@ 123456yahoo.com

                Dr Mircea Iurciuc, Department of Cardiology, Faculty of Medicine, Multidisciplinary Center for Research, Evaluation, Diagnosis and Therapies in Oral Medicine, ‘Victor Babes’ University of Medicine and Pharmacy, Piata Eftimie Murgu 2, 300041 Timisoara, Romania iurciuc.mircea@ 123456umft.ro

                *Contributed equally

                Article
                ETM-24-6-11666
                10.3892/etm.2022.11666
                9634885
                36349062
                c2294633-1ab0-4590-ab00-56dda67af454
                Copyright: © Stanciugelu et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

                History
                : 10 February 2022
                : 10 March 2022
                Funding
                Funding: No funding was received.
                Categories
                Case Report

                Medicine
                budd-chiari syndrome,neoplastic disease,hepatomegaly,hepatic failure,thrombosis,endovascular intervention

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