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Systemic Veins of the Thorax.
Published in Fred W Wright, Radiology of the Chest and Related Conditions, 2022
The Budd-Chiari syndrome is a rare disorder of the hepatic veins, or impaired hepatic venous drainage due to anomalies of the IVC. There are two types: Primary due to congenital obstruction of the hepatic veins or hepatic portion of the IVC by webs or diaphragms, andSecondary due to tumour (especially a hepatoma), thrombosis or trauma.
Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Acute Budd-Chiari syndrome typically presents with abdominal pain, hepatomegaly and ascites. It is often secondary to thrombus in the suprahepatic vena cava or hepatic veins. The finding of hyperenhancement in the central liver on early phase post-contrast imaging with peripheral hyperenhancement on delayed images is called the ‘flip-flop’ pattern. Splenomegaly, absent hepatic veins and increased portal vein diameter are also features.
A Histopathologic Classification of Chemical-Induced Injury of the Liver
Published in Robert G. Meeks, Steadman D. Harrison, Richard J. Bull, Hepatotoxicology, 2020
John M. Cullen, Boris H. Ruebner
The Budd-Chiari syndrome is seen characteristically after obstruction of the blood flow through the hepatic veins or the inferior vena cava, usually by thrombosis. It develops when there is an increased tendency for thrombosis, as for example, in polycythemia. It has also been reported in women taking contraceptive steroids (Lalonde et al., 1982).
The digestive system involvement of antiphospholipid syndrome: pathophysiology, clinical characteristics, and treatment strategies
Published in Annals of Medicine, 2021
Jin Zhang, Cheng Li, Xiaorong Han, Zhongbo Chen, Binay Kumar Adhikari, Yinghui Wang, Yonggang Wang, Jian Sun
Budd-Chiari syndrome (BCS) may be the first clinical manifestation of APS [7]. After Pomeroy C et al [44] published the first case report describing the correlation between BCS and APL, several cases were reported afterwards [45–54]. BCS is a major vascular disorder of the liver, characterised by structural and functional abnormalities of the liver resulting from the obstruction of the hepatic venous outflow from hepatic veins(the small hepatic veins, main hepatic veins and suprahepatic inferior vena cava) [55,56]. Some myeloproliferative diseases (MPDs) and predisposition to thrombotic conditions are considered as possible causes of BCS. In contrast, primary BCS is usually associated with risk factors for thrombosis (e.g. protein C and protein S deficiency, primary thrombocytopenia, etc.) [55,56]. APS may be the second most common cause of non-neoplastic BCS after primary MPDs [49].
Portal vein thrombosis in a child with essential thrombocythemia seven years after diagnosis
Published in Pediatric Hematology and Oncology, 2019
Goutomi Chatterjee, Satya Prakash Yadav
There have been case reports of children with ET presenting as Budd-Chiari syndrome (BCS). Hermeziu et al. reported a 7-year old girl diagnosed with BCS, with history of polycythemia vera in 2 maternal aunts, who was found to have thrombocytosis. ET was confirmed with positive heterozygous JAK2V617F mutation testing. Hydroxyurea (20 mg/kg/day) decreased the platelet count and BCS was treated with transjugular intrahepatic portosystemic shunt (TIPS) along with heparin.7 Tokgoz et al. had reported the first case of pediatric ET with MPL W515K somatic mutation presenting with BCS.8 Randi et al. had analyzed a series of 20 pediatric patients with ET for polyclonal mutations, where one 8-year-old child with BCS was treated with orthotopic liver transplantation and warfarin.9 Wigton et al. reported a 12-year-old female with BCS underwent liver transplant and subsequent splenectomy. Her platelet count began to rise postoperatively after previous normal values. JAK2V617F-positive ET was diagnosed.10 Most of the described cases of children with Splanchnic vein thrombosis (SVT) prior to diagnosis of ET occured in children with JAK2 mutation, suggesting a possible future development of ET.7,9,10 The prevalence of JAK2V617F mutation in those with SVT ranges between 28% in nonmalignant, non-cirrhotic patients with PVT to 41% in patients with BCS. JAK2V617F screening in patients with SVT without overt myeloproliferative neoplasm (MPN) features identified MPN in about 15% of cases.11,12
Liver transplantation for hepatic alveolar echinococcosis: literature review and three new cases
Published in Infectious Diseases, 2018
Mohsen Aliakbarian, Fariba Tohidinezhad, Saeid Eslami, Kambiz Akhavan-Rezayat
Including our own case report, 27 studies describing 150 cases were identified from 1994 to 2017 (Table 1). The average age at LT for all recipients was 38 years, with a range of 12–71 years. From the total group, 77 LT recipients were male. Patients developed hydatidosis caused by echinococcosis vogelii (N = 1) [22], echinococcosis granulosus (N = 6) [5] and echinococcosis multilocularis (N = 143). A total of 18 patients presented Budd–Chiari syndrome prior to transplantation. Deceased donor and living donor transplantations were performed for 114 and 36 patients, respectively. Retrospective case series reported a disease recurrence rate of 0–60%. The overall 1-, 5- and 10-year survival rates were estimated at 60–100%, 67–85% and 49–75%, respectively. As we expected, most of reports were from countries located in the northern hemisphere (mostly in the central Europe) and no publication was found from Australia and Africa continents (Figure 2).