title: Primary Budd-Chiari syndrome - a single center experience.
abstract: OBJECTIVE
  Budd-Chiari syndrome (BCS) is recognized as a clinical manifestation of various prothrombotic conditions which may be lethal within 3 years of the onset of symptoms if untreated. This study is a retrospective analysis of patients with BCS managed between 2004 and 2011.
  
  
  METHODS
  The diagnosis was confirmed with contrast CT-angiography and/or Doppler ultrasound.
  
  
  RESULTS
  BCS was diagnosed in 20 patients (11 females and 9 males), median age 38 years (ranging from 18 to 56). Twelve patients were referred as acute BCS for the liver transplant (LTx) assessment. Thrombosis of the hepatic veins was caused by myeloproliferative disorders (n=8), end-stage liver disease (n=4), protein C deficiency (n=3), paroxysmal nocturnal hemoglobinuria (PNH) (n=1), antiphospholipid syndrome (n=1) and secondary poliglobulia (n=1). In two patients the origin of BCS could not be established despite appropriate screening. Median follow-up was 29 months. Low molecular heparin with subsequent conversion to vitamin K antagonists was routinely applied in all patients. Two patients underwent TIPS procedure with good long term outcome and 10 subjects received LTx; 1 patient was lost to follow-up and 1 died of chest infection 9 years since the diagnosis of BCS was made; 14 patients, including those who received LTx, were alive and well at least one year after BCS diagnosis. All survivors remain stable and are followed-up on a regular basis.
  
  
  CONCLUSIONS
  Strict adherence to the diagnostic and therapeutic guidelines plays a crucial role in the management of BCS patients. Our results confirm the efficacy of anticoagulation as well as TIPS and/or OLT in treatment of this rare condition.
authors: Liver Unit, Pomeranian Medical University, Szczecin, Poland. jorasz@sci.pum.edu.pl
authors: Joanna
authors: J
authors: Raszeja-Wyszomirska
authors: Joanna
authors: J
authors: Mieżyńska-Kurtycz
authors: Wojciech
authors: W
authors: Marlicz
authors: Małgorzata
authors: M
authors: Lawniczak
authors: Maciej
authors: M
authors: Wójcicki
mesh_terms: D000293:Adolescent; D000328:Adult; D000925:Anticoagulants / Q000008:administration & dosage*; D006502:Budd-Chiari Syndrome / Q000175:diagnosis / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D057915:Drug Substitution; D005260:Female; D019983:Guideline Adherence; D006495:Heparin, Low-Molecular-Weight / Q000008:administration & dosage; D006801:Humans; D016031:Liver Transplantation / Q000009:adverse effects / Q000401:mortality; D008297:Male; D008875:Middle Aged; D011044:Poland; D019168:Portasystemic Shunt, Transjugular Intrahepatic / Q000009:adverse effects / Q000401:mortality; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D018608:Ultrasonography, Doppler; D014812:Vitamin K / Q000037:antagonists & inhibitors; D055815:Young Adult
chemical_list: D000925:Anticoagulants; D006495:Heparin, Low-Molecular-Weight; D014812:Vitamin K