title: Budd-Chiari syndrome complicating essential thrombocythemia in an adolescent: favorable outcome of TIPS procedure.
abstract: Budd-Chiari syndrome (BCS) is a liver disorder characterized by hepatic venous outflow obstruction, mainly resulting from thrombosis of the terminal part of the hepatic veins or the inferior vena cava. It causes hepatic congestion, ascites, portal hypertension, and collateral circulation between the obstructed and contiguous patent venous territories. BCS is reported complicating myeloproliferative disorders, as well other prothrombotic events. Essential thrombocythemia is one of the most frequent myeloproliferative disorders that cause BCS, and in some cases, it may be the initial presentation. Many treatment options have been proposed for BCS, routine anticoagulation therapy being recommended as the first therapeutic approach.
authors.affiliation: Division of Pediatric Hematology and Oncology, Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
authors.forename: Azza Abdel-Gawad
authors.initials: AA
authors.lastname: Tantawy
authors.forename: Amira Abdel-Monem
authors.initials: AA
authors.lastname: Adly
authors.forename: Yasmine Ibrahim
authors.initials: YI
authors.lastname: Elhenawy
mesh_terms: D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000209:etiology*; D002648:Child; D005260:Female; D006801:Humans; D013920:Thrombocythemia, Essential; D016896:Treatment Outcome