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Budd chiari syndrome

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https://www.readbyqxmd.com/read/28710306/ruxolitinib-treatment-in-an-infant-with-jak2-polycythaemia-vera-associated-budd-chiari-syndrome
#1
Mehmet Enes Coskun, Sue Height, Anil Dhawan, Nedim Hadzic
Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction commonly seen with myeloproliferative neoplasms (MPNs). Polycythaemia vera (PV) is a very rare MPN in childhood. This is the youngest reported patient diagnosed with PV and BCS secondary to JAK V617F mutation.A 26-month-old girl was admitted with a 5-month history of abdominal distension, hepatosplenomegaly and ascites. Imaging studies revealed occlusion of the right hepatic vein and marked attenuation of the middle and left hepatic veins...
July 14, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28698856/the-first-case-of-paroxysmal-nocturnal-hemoglobinuria-and-budd-chiari-syndrome-treated-with-complement-inhibitor-eculizumab-in-korea
#2
Hyerim Kim, In-Suk Kim, Su-Hee Cho, Hyun Ji Lee, Chulhun L Chang, Ki Tae Yoon
No abstract text is available yet for this article.
June 2017: Blood Research
https://www.readbyqxmd.com/read/28685257/budd-chiari-syndrome-hepatic-venous-outflow-tract-obstruction
#3
REVIEW
Dominique-Charles Valla
BACKGROUND: Budd-Chiari syndrome (BCS) is a rare disease characterized by hepatic venous outflow tract obstruction (HVOTO). METHODS: Recent literature has been analyzed for this narrative review. RESULTS: Primary BCS/HVOTO is a result of thrombosis. The same patient often has multiple risk factors for venous thrombosis and most have at least one. Presentation and etiology may differ between Western and certain Eastern countries. Myeloproliferative neoplasms are present in 40% of patients and are usually associated with the V617F-JAK2 mutation in myeloid cells, in particular peripheral blood granulocytes...
July 6, 2017: Hepatology International
https://www.readbyqxmd.com/read/28675622/early-radiological-intervention-and-haematology-screening-is-associated-with-excellent-outcomes-in-budd-chiari-syndrome
#4
Allison Mo, Adam Testro, Janine French, Marcus Robertson, Peter Angus, Andrew Grigg
BACKGROUND: Budd Chiari Syndrome (BCS) is a rare and life-threatening disorder, resulting from thrombosis of the hepatic veins. Various treatments including pharmacological, radiological and surgical interventions have been used. AIM: We aimed to retrospectively describe our institution's experience with management of patients with BCS. RESULTS: Between 2000 and 2012, 27 patients with primary BCS presented with a median Rotterdam score of 1...
July 4, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/28673473/clinical-manifestations-and-imaging-tools-in-the-diagnosis-of-splanchnic-and-cerebral-vein-thromboses
#5
Nicoletta Riva, Walter Ageno
Splanchnic vein thrombosis (SVT) and cerebral vein thrombosis (CVT) are uncommon manifestation of venous thromboembolism (VTE), occurring less frequently than deep vein thrombosis of the lower extremities and pulmonary embolism. SVT encompasses portal vein thrombosis, mesenteric vein thrombosis, splenic vein thrombosis and the Budd-Chiari syndrome. It is therefore a heterogeneous disease, with differences in clinical manifestations according to the site of thrombosis. CVT includes thrombosis of the cortical or deep cerebral veins and thrombosis of the major dural venous sinuses...
June 27, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/28643816/-budd-chiari-syndrome-and-ulcerative-colitis-in-an-adolescent
#6
Federico Marchetti, Martina Mainetti, Michela Giovannini, Cristina Morelli
In patients with inflammatory bowel disease (IBD) there is an increased incidence of thromboembolic events. We report a case of a female, age 14, with a 6 months history of diarrhea with occasional presence of red blood and with a very distended abdomen with evident ascites. The diagnosis was Budd-Chiari syndrome (BCS) in ulcerative colitis (UC). Therapy with subcutaneous low molecular weight heparin, methylprednisolone, mesalazine was started. Clinical and radiological features quickly improved. No thrombophilia abnormality nor other risk factor for thrombosis were detected...
May 2017: Recenti Progressi in Medicina
https://www.readbyqxmd.com/read/28631648/leiomyosarcoma-of-inferior-vena-cava-with-intracardiac-extension-presenting-as-budd-chiari-syndrome-report-of-a-rare-case
#7
Kaniyappan Nambiyar, Arvind Ahuja, Minakshi Bhardwaj
Primary leiomyosarcoma of vascular origin is a rare malignant smooth muscle tumor. This report describes a case of primary leiomyosarcoma of the inferior vena cava (IVC) in a 52-year-old male who presented with complaints of abdominal pain, chest pain, and bilateral lower limb swelling for 4 months. Imaging of the chest and abdomen revealed a mass in the IVC extending into right atrium, heterogeneous enhancement of liver, hepatosplenomegaly, and ascites. Histopathological examination showed a malignant spindle cell tumor with cells arranged in fascicular and hemangiopericytomatous pattern...
April 2017: Indian Journal of Pathology & Microbiology
https://www.readbyqxmd.com/read/28631134/budd-chiari-syndrome-outcomes-of-endovascular-intervention-a-single-center-experience
#8
Nitin Jagtap, Mithun Sharma, Jagdeesh Singh, Manu Tandan, P N Rao, Rajesh Gupta, Sundeep Lakhtakia, Mohan Ramchandani, Harshal Shah, T Mahesh Kumar, Santosh Darishetty, G V Rao, D N Reddy
AIM: Outcomes of endovascular intervention in Budd-Chiari syndrome (BCS) have been reported with varied results. Clinical outcomes of endovascular interventions in BCS and role of various prognostic scores were critically evaluated in this study. METHODS: This study retrospectively analyzed consecutive patients of BCS who underwent endovascular intervention between January 2007 and May 2016 at our center. Technical, clinical successes and complications were documented...
June 20, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28629571/detection-and-characterization-of-budd-chiari-syndrome-with-inferior-vena-cava-obstruction-comparison-of-fixed-and-flexible-delayed-scan-time-of-computed-tomography-venography
#9
Peng-Li Zhou, Gang Wu, Xin-Wei Han, Yong-Hua Bi, Wen-Guang Zhang, Zheng-Yang Wu
PURPOSE: To compare the results of computed tomography venography (CTV) with a fixed and a flexible delayed scan time for Budd-Chiari syndrome (BCS) with inferior vena cava (IVC) obstruction. MATERIAL AND METHODS: A total of 209 consecutive BCS patients with IVC obstruction underwent either a CTV with a fixed delayed scan time of 180s (n=87) or a flexible delayed scan time for good image quality according to IVC blood flow in color Doppler ultrasonography (n=122)...
June 2017: European Journal of Radiology
https://www.readbyqxmd.com/read/28611533/functional-budd-chiari-syndrome-associated-with-severe-polycystic-liver-disease
#10
Precil Diego Miranda de Menezes Neves, Bruno Eduardo Pedroso Balbo, Elieser Hitoshi Watanabe, Vinicius Rocha-Santos, Wellington Andraus, Luiz Augusto Carneiro D'Albuquerque, Luiz Fernando Onuchic
A 50-year-old woman with end-stage renal disease secondary to autosomal dominant polycystic kidney disease was referred to a quaternary care center due to significantly increased abdominal girth. Her physical examination revealed tense ascites and abdominal collateral veins. A 10-L paracentesis improved abdominal discomfort and disclosed a transudate, suggestive of portal hypertension. A computed tomographic scan revealed massive hepatomegaly caused by multiple cysts of variable sizes, distributed throughout all hepatic segments...
2017: Clinical Medicine Insights. Gastroenterology
https://www.readbyqxmd.com/read/28610817/vascular-diseases-of-the-liver-clinical-guidelines-from-the-catalan-society-of-digestology-and-the-spanish-association-for-the-study-of-the-liver
#11
Marta Martín-Llahí, Agustín Albillos, Rafael Bañares, Annalisa Berzigotti, M Ángeles García-Criado, Joan Genescà, Virginia Hernández-Gea, Elba Llop-Herrera, Helena Masnou-Ridaura, José Mateo, Carmen A Navascués, Ángela Puente, Marta Romero-Gutiérrez, Macarena Simón-Talero, Luis Téllez, Fanny Turon, Cándido Villanueva, Roberto Zarrabeitia, Juan Carlos García-Pagán
Despite their relatively low prevalence, vascular diseases of the liver represent a significant health problem in the field of liver disease. A common characteristic shared by many such diseases is their propensity to cause portal hypertension together with increased morbidity and mortality. These diseases are often diagnosed in young patients and their delayed diagnosis and/or inappropriate treatment can greatly reduce life expectancy. This article reviews the current body of evidence concerning Budd-Chiari syndrome, non-cirrhotic portal vein thrombosis, idiopathic portal hypertension, sinusoidal obstruction syndrome, hepatic vascular malformations in hereditary haemorrhagic telangiectasia, cirrhotic portal vein thrombosis and other rarer vascular diseases including arterioportal fistulas...
June 11, 2017: Gastroenterología y Hepatología
https://www.readbyqxmd.com/read/28610476/budd-chiari-syndrome-with-upper-gastrointestinal-hemorrhage-characteristic-and-long-term-outcomes-of-endovascular-treatment
#12
Peng-Li Zhou, Gang Wu, Xin-Wei Han, Lei Yan, Wen-Guang Zhang
Purpose To identify the characteristics and evaluate the long-term outcomes of endovascular treatment of Budd-Chiari syndrome with upper gastrointestinal hemorrhage. Methods Forty-seven consecutive Budd-Chiari syndrome patients with upper gastrointestinal hemorrhage were referred for the treatment with percutaneous transluminal balloon angioplasty, and subsequently underwent follow-up. Data were retrospectively collected and follow-up observations were performed at 1, 2, 2-5, and 5-8 years postoperatively. Results Cirrhosis was presented in 16 patients, and splenoportography reviewed obvious varices in 18 patients...
January 1, 2017: Vascular
https://www.readbyqxmd.com/read/28604464/liver-transplantation-in-a-patient-with-antiphospholipid-syndrome-a-case-report
#13
Rachel C Steckelberg, Zarah D Antongiorgi, Randolph H Steadman
Antiphospholipid syndrome (APS) is an acquired thrombophilic disorder characterized by autoantibodies to cell membrane phospholipids. While altered coagulation can complicate end-stage liver disease, there are few reports describing the perioperative management for liver transplantation in recipients with a preexisting hypercoagulable disorder, such as APS. We present a patient with a history of APS, Budd-Chiari syndrome with cirrhosis, hepatopulmonary syndrome, and heparin-induced thrombocytopenia who underwent liver transplantation complicated by hepatic artery thrombosis...
June 8, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/28599693/clinical-characteristics-and-outcome-of-budd-chiari-syndrome-at-a-tertiary-care-hospital-in-pakistan
#14
Om Parkash, Aysha Khan, Saeed Hamid
OBJECTIVE: To determine the clinical characteristics of Budd-Chiari syndrome (BCS), its causes and outcome at a tertiary care hospital. STUDY DESIGN: An observational study. PLACE AND DURATION OF STUDY: The Aga Khan University Hospital,Karachi, from 2004 to 2014. METHODOLOGY: Aretrospective analysis of data was conducted. Apredesigned questionnaire was filled from medical records of patients with BCS. Clinical features, etiology, management and outcome was noted from 2004 to 2014...
May 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28592752/successful-vascular-intervention-without-embolic-complications-in-budd-chiari-syndrome
#15
Eiji Taguchi, Junjiroh Koyama, Masataka Kajiwara, Masayuki Inoue, Yoko Horibata, Kazuhiro Nishigami, Koichi Nakao, Tomohiro Sakamoto
No abstract text is available yet for this article.
June 8, 2017: Circulation Journal: Official Journal of the Japanese Circulation Society
https://www.readbyqxmd.com/read/28577156/recanalization-of-tipss-via-transsplenic-puncture
#16
Abhinav Jain, Vinit Kahalekar, Shaji Marar, Deepak Gupta, Sangeet Sawrav, S Kiran, Akash Shukla
Transjugular intrahepatic portosystemic shunt (TIPSS) has important role in the management of Budd-Chiari syndrome (BCS). Stent dysfunction rate up to 80% at 2 years limits the use of TIPSS. Reintervention is required if shunt develops occlusion or stenosis. Different techniques have been reported for the catheterization of TIPSS in cases of an occluded stent. In those failing a standard transvenous approach, the Colapinto needle technique, a combined transvenous transhepatic approach or transplenic approaches have been described...
June 2, 2017: Indian Journal of Gastroenterology: Official Journal of the Indian Society of Gastroenterology
https://www.readbyqxmd.com/read/28574877/budd-chiari-syndrome-demonstrated-on-pet-ct
#17
Brianna Abadie, Manu Singh, Samson Lu, Kenneth Daughters
Budd-Chiari syndrome is a rare clinical condition that involves diminution or interruption of hepatic venous blood flow from the liver. We describe a case of Budd-Chiari syndrome visualized via F-FDG PET/CT in a 60-year-old woman with medical history of B-cell non-Hodgkin lymphoma who presented with abdominal pain. FDG PET/CT fusion images demonstrate increased FDG uptake with SUVmax of 2.2 along the course of the middle hepatic vein with decreased attenuation within both the middle and right hepatic veins, consistent with thrombus...
August 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28561456/investigation-of-plasminogen-activator-inhibitor-1-pai-1-4g-5g-promoter-polymorphism-in-indian-venous-thrombosis-patients-a-case-control-study
#18
Aniket Prabhudesai, Shrimati Shetty, Kanjaksha Ghosh, Bipin Kulkarni
BACKGROUND: The role of PAI-1 4G/5G polymorphism in venous thrombosis has been contradictory. PAI-1 4G/4G genotype is associated with elevated levels of PAI-1 resulting in a hypofibrinolytic state and a higher thrombotic risk. OBJECTIVE: In this study, the distribution of genotypes and frequency of alleles of the 4G/5G polymorphism of PAI-1 gene in Indian patients with different types of venous thrombosis was investigated for its role in development of thrombosis...
May 31, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28508963/hemodiafiltration-for-hepatic-encephalopathy-induced-by-budd-chiari-syndrome-in-a-patient-with-end-stage-kidney-disease
#19
Takuya Wakamatsu, Suguru Yamamoto, Kenya Kamimura, Takeshi Nakatsue, Noriaki Iino, Seitaro Iguchi, Yoshikatsu Kaneko, Shin Goto, Junichiro James Kazama, Ichiei Narita
A 36-year-old woman who was undergoing dialysis for end-stage kidney disease (ESKD) was admitted to our hospital with consciousness disorder. She was diagnosed with Budd-Chiari syndrome due to antiphospholipid syndrome at the age of 28 years. Her kidney function and leg edema gradually deteriorated. After initiation of hemodialysis (HD), transient loss of consciousness due to hepatic encephalopathy during HD treatment occurred frequently. Her kidney replacement therapy was changed to online hemodiafiltration (HDF), which dramatically improved her hepatic coma...
November 2016: CEN Case Reports
https://www.readbyqxmd.com/read/28483616/endovascular-treatment-of-an-unusual-membranous-obstruction-of-the-inferior-vena-cava-in-budd-chiari-syndrome-complicated-by-mural-portal-vein-thrombosis
#20
Wen-Guang Zhang, Dong-Mei Liu, Xin-Wei Han, Jian-Zhuang Ren, Peng-Li Zhou, Zhong-Gao Wang
No abstract text is available yet for this article.
May 5, 2017: Annals of Vascular Surgery
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