Abstract

Background and Aim: Budd-Chiari syndrome is a relatively rare condition with multiple etiologies and high risk of complications and death. The aim of our study were to investigate the etiology of BCS, especially auto-inflammatory diseases and rare associations, analyze different clinical presentations, treatment modalities and evolutionary profile.

Methods: Retrospective cohort study of patients hospitalized from 2000 to 2018 with confirmed diagnosis of BCS on Doppler ultrasound, with which we have conducted a large etiological survey.

Results: 11 patients were included; mean age 29 years (16 to 45 years); sex ratio (M/F) 0,83.
All patients were symptomatic at diagnosis with classic subacute to chronic clinic presentation, 6 patients had extravascular manifestations.
The etiological survey conclusion was: Behçet’s syndrome in 6 cases (most severe forms), liver chronic disease in 2 cases (hepatitis C and cryptogenic cirrhosis), protein S deficiency associated with coeliac disease in 1 case, paroxysmal nocturnal hemoglobinuria associated with antiphospholipid syndrome in 1 case and factor five Leiden mutation in 1 case. The treatment included anticoagulants in 7 cases, specific treatment combining corticosteroids and immunosuppressive drugs in patients with BS, Gluten-free diet in celiac disease.

Conclusion: Behçet’s syndrome was the first etiology in our study, it must be systematically searched in countries with high prevalence, especially in young patients with extravascular manifestations. The association of 2 or plus prothrombotic states is not rare and often insidious. The systematic search of this kind of association must be discussed.

Keywords: Budd-Chiari Syndrome; Behçet’s Syndrome; Portal Hypertension; Vasculitis; Thrombosis

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Citation

Citation: Salim Larbani., et al. “Etiological Profile of 11 Cases of Bud-Chiari Syndrome". Acta Scientific Medical Sciences 4.5 (2020): 03-08.

Copyright

Copyright: © 2020 Salim Larbani., et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.