Budd‐Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18‐year experience

  title={Budd‐Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18‐year experience},
  author={Rupesh Rajani and T Melin and Einar S. Bj{\"o}rnsson and Ulrika Broomé and Per Sangfelt and {\AA}ke Danielsson and Anders Gustavsson and Olof Grip and Hans Svensson and Lars L{\"o}{\"o}f and Sven Wallerstedt and Sven Alm{\'e}r},
  journal={Liver International},
Background: The exact incidence and prevalence of Budd‐Chiari syndrome (BCS) is unknown in the general population. Published reports differ in terms of the clinical characteristics, effects of therapy and survival. 
Incidence, prevalence and complications of Budd–Chiari syndrome in South Korea: a nationwide, population‐based study
This study aimed to elucidate the nationwide population‐based incidence, prevalence, complications, case fatalities and direct medical cost of Budd–Chiari syndrome in South Korea from 2009 to 2013.
Clinical features and etiology of Budd–Chiari syndrome in Chinese patients: A single‐center study
The clinical features and etiology of patients with incident BCS in China prospectively vary from region to region, and there is lack of large sample studies about BCS.
Rotterdam score predicts early mortality in Budd‐Chiari syndrome, and surgical shunting prolongs transplant‐free survival
This data indicates that among children born with Budd–Chiari syndrome, the likelihood of survival is low, but the chances of survival among those born with a second syndrome are high.
Review article: a multidisciplinary approach to the diagnosis and management of Budd‐Chiari syndrome
Budd‐Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract.
Early radiological intervention and haematology screening is associated with excellent outcomes in Budd–Chiari syndrome
Budd–Chiari syndrome (BCS) is a rare and life‐threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments, including pharmacological, radiological and surgical
Pregnancy outcomes in women with Budd–Chiari syndrome or portal vein thrombosis – a multicentre retrospective cohort study
To evaluate current practice and outcomes of pregnancy in women previously diagnosed with Budd–Chiari syndrome and/or portal vein thrombosis, with and without concomitant portal hypertension.
Review article: the aetiology of primary Budd–Chiari syndrome – differences between the West and China
Considering the discrepancy in the clinical profiles and preferred treatment selection of primary BCS between the West and China, understanding its aetiology in these two different regions is very important.
Budd-Chiari Syndrome: The Western Perspective
Primary Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow obstruction from small hepatic veins to the inferior vena cava (IVC), due to thrombosis or fibrous stenosis of these veins. In
Budd–Chiari Syndrome: East versus West
Budd–Chiari syndrome is an uncommon cause of liver disease, which is being diagnosed more frequently with better diagnostic techniques especially in the East, and excellent responses to radiological interventional techniques like angioplasty and transjugular intrahepatic portosystemic shunt have resulted in excellent long-term survivals in both the East and the West.


Myeloproliferative disease in the pathogenesis and survival of Budd-Chiari syndrome.
The etiology, diagnosis and natural course of myeloproliferative disease (MPD) in 40 consecutive patients with Budd-Chiari syndrome (BCS) and JAK2 mutation was present in 41% of the tested BCS patients.
Budd–Chiari syndrome: illustrated review of current management
The current literature with respect to presentation, management and prognosis of BCS is reviewed, and particular attention is paid to interventional and surgical aspects of management.
Outcome of Budd‐Chiari syndrome: A multivariate analysis of factors related to survival including surgical portosystemic shunting
Age, severity of liver failure, and presence of refractory ascites are the main prognostic factors in Budd‐Chiari syndrome, and surgical shunting should be restricted to management of refracted ascites or variceal bleeding in patients with otherwise good prognostic Factors.
Determinants of survival and the effect of portosystemic shunting in patients with Budd‐Chiari syndrome
Patients with BCS can be classified into good (I), intermediate (II), and poor (III) prognostic classes, according to simple baseline clinical and laboratory parameters, and an improved survival after surgical portosystemic shunting is suggested.
Aiming at minimal invasiveness as a therapeutic strategy for Budd‐Chiari syndrome
The 1‐year spontaneous mortality rate in patients with Budd‐Chiari syndrome (BCS) approaches 70%. No prospective assessment of indications and impact on survival of current therapeutic procedures has
Liver transplantation for Budd-Chiari syndrome: A European study on 248 patients from 51 centres.
Epidemiological and clinical features of Budd-Chiari syndrome in Japan.
Poor prognosis and limited therapeutic options in patients with Budd‐Chiari syndrome and portal venous system thrombosis
Patients with BCS and portal venous system thrombosis constitute a unique group with limited therapeutic options and poor prognosis, and the importance of early recognition and anticoagulation of patients with B CS is emphasized.
Incidence of parenchymal liver diseases in Denmark, 1981 to 1985: Analysis of hospitalization registry data
The sex‐specific and age‐specific incidence rates of the major parenchymal liver diseases in a North European population were estimated using a computerized registry of all admissions to somatic