title: Diagnosis, etiology and management of the Budd-Chiari Syndrome: a bloodcoagulation and hepatological study on the course of the disease treated with TIPS.
abstract: BACKGROUND
  Budd-Chiari Syndrome (BCS) is characterized by obstruction of blood flow in hepatic veins. The aim of the study was to analyze diagnosis, etiology and management of BCS.
  
  
  METHODS
  We analyzed 44 patients (32 females, 12 males, the mean age <35y of age) treated with TIPS. Ascites was found in 35 patients as the most frequent symptom. The median of total follow-up was 52 months. Non-covered (bare) or covered stent was inserted to all patients. Diagnosis of myeloproliferative neoplasm (MPN) was based on WHO criteria. Other inherited or acquired thrombophilia were assessed as well. Therapy of BCS was with regard to the etiology.
  
  
  RESULTS
  The etiology of BCS was identified in 38 cases. Ph- MPN was found as the most common risk factor (50%, N.=22), especially polycythemia vera. JAK2V617F mutation was detected in the most of 22 MPN cases (82.5%). The second most common etiologic factor was inherited thrombophilia (18%, N.=8). In the non-covered (bare) stent group, a primary patency rates 52.9% in 1 year and 20% in 5 years after TIPS (Portasystemic Shunt, Transjugular Intrahepatic) creation. In the covered stent group the 1-year and 5-year primary patency rates were was 80% and 33.3% respectively. The average 5-year re-intervention rate per patient was 1.65 procedures in the bare stent group and 0.67 in the covered stent group. Re-interventions were more frequent in MPN patients. All patients were anticoagulated with heparin at the beginning, switched to vitamin K antagonist. On top of TIPS, anticoagulant and a vigorous therapy of underlying disorder are necessary.
  
  
  CONCLUSIONS
  BCS is a serious and life-threatening disorder in MPD is a major cause of morbidity and mortality. Therapy requires a multidisciplinary approach. Insertion of TIPS dedicated covered stent is a very effective treatment in cases resistant to conservative approach with lower dysfunction rate and the number of re-interventions.
authors.affiliation: Fourth Department of Internal Medicine, Hematology, Faculty of Medicine in Hradec Kralove, University Hospital in Hradec Kralove, Hradec Kralove, Czech Republic - PetrDulicek@seznam.cz.
authors.forename: Petr
authors.initials: P
authors.lastname: Dulíček
authors.forename: Petr
authors.initials: P
authors.lastname: Hůlek
authors.forename: Antonin
authors.initials: A
authors.lastname: Krajina
authors.forename: Ondrej
authors.initials: O
authors.lastname: Renc
authors.forename: Václav
authors.initials: V
authors.lastname: Šafka
authors.forename: Tomas
authors.initials: T
authors.lastname: Fejfar
authors.forename: Petr
authors.initials: P
authors.lastname: Sadílek
authors.forename: Martin
authors.initials: M
authors.lastname: Beránek
authors.forename: Jan J
authors.initials: JJ
authors.lastname: Michiels
authors.forename: Pavel
authors.initials: P
authors.lastname: Žák
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001777:Blood Coagulation; D006502:Budd-Chiari Syndrome / Q000097:blood / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000150:complications; D008297:Male; D008875:Middle Aged; D019168:Portasystemic Shunt, Transjugular Intrahepatic; D055815:Young Adult