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

@article{Rajani2009BuddChiariSI,
  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},
  year={2009},
  volume={29}
}
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. 
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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.
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TLDR
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.
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TLDR
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.
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TLDR
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.
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TLDR
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.
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TLDR
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
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