[Budd-Chiari syndrome--from diagnosis to treatment--case reports].
Budd-Chiari syndrome is a rare disease, caused by obstruction of the hepatic venous outflow at the level of either the large hepatic veins or (and) the subdiaphragm segment of the inferior vena cava. The hematological disorders (myeloproliferative disorders, factor V Leiden deficiency), tumor and chronic inflammatory diseases are the most frequent causes of BCS in Europe and North America. Two cases of BCS, recognized in 24 and 43 years old females with subacute and chronic forms of the disease are presented in this article. The underlying cause was polycythemia rubra vera and osteomyolofibrosis. In first case, except causal and anticoagulation therapy, a transjugular intrahepatic portosystemic stent has been performed. In the second one low - sodium diet and diuretic for the control of ascites and oedemas were used. Hydroxycarbamid was the first choice line medication in treatment hematological disorders and acenocumarol in the prevention of the trombotic complications.
Kozielewicz
Dorota
D
Uniwersytet Mikołaja Kopernika w Toruniu, Collegium Medicum w Bydgoszczy, Katedra i Klinika Chorób Zakaznych i Hepatologii. d.kozielewicz@wsoz.pl
Smukalska
Ewa
E
Dybowska
Dorota
D
D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D006502:Budd-Chiari Syndrome / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000627:therapeutic use; D011086:Polycythemia / Q000150:complications; D055728:Primary Myelofibrosis / Q000150:complications; D015607:Stents; D016896:Treatment Outcome
D000925:Anticoagulants; D006918:Hydroxyurea