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Budd–Chiari syndrome

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https://www.readbyqxmd.com/read/28923635/living-donor-liver-transplantation-for-budd-chiari-syndrome-case%C3%A2-series
#1
C Karaca, C Yilmaz, R Ferecov, Z Iakobadze, K Kilic, L Caglayan, S Aydogdu, M Kilic
BACKGROUND: Venous reconstruction in living-donor liver transplantation for Budd-Chiari syndrome (BCS) has challenges because the grafts from living donors lack vena cava, and hepatic venous anastomosis must be performed on an already-thrombosed and/or stenosed inferior vena cava. Several techniques are described to overcome this problem, and we represent our experience with 22 patients. METHODS: Medical recordings of 22 patients were retrospectively collected, and disease-specific data as well as recordings about surgical technique were analyzed...
October 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28922972/perioperative-anesthetic-management-of-patients-having-liver-transplantation-for-uncommon-conditions
#2
Anthony Bonavia, Justin Pachuski, Dmitri Bezinover
This review focuses on the perioperative anesthetic management of patients having liver transplantation (LT) performed for several uncommon indications or in combination with rare pathology. Conditions discussed in the article include Alagille syndrome, hypertrophic cardiomyopathy, Gilbert's syndrome, porphyria, Wilson's disease, and Budd-Chiari syndrome. In comparison to other indications, LT in these settings is infrequent because of the low incidence of these pathologies. Most of these conditions (with the exception of Gilbert syndrome) are associated with a high probability of significant perioperative complications and increased mortality and morbidity...
September 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28922103/budd-chiari-syndrome
#3
Tomáš Grus, Lukáš Lambert, Gabriela Grusová, Rohan Banerjee, Andrea Burgetová
Budd-Chiari syndrome (BCS) is a rare disease with an incidence of 0.1 to 10 per million inhabitants a year caused by impaired venous outflow from the liver mostly at the level of hepatic veins and inferior vena cava. Etiological factors include hypercoagulable conditions, myeloprolipherative diseases, anatomical variability of the inferior vena cava, and environmental conditions. Survival rates in treated patients range from 42 to 100% depending on the etiology and the presence of risk factors including parameters of Child-Pugh score, sodium and creatinine plasma levels, and the choice of treatment...
2017: Prague Medical Report
https://www.readbyqxmd.com/read/28870052/-clinical-efficacy-of-enhanced-recovery-after-surgery-in-atrial-caval-shunting-for-type-%C3%A2-budd-chiari-syndrome
#4
G J Tian, D Y Li, H B Yu, Y D Dong, Y N Peng, P Liu, Y K Wei, H Z Xue
Objective: To investigate the clinical efficacy of enhanced recovery after surgery(ERAS) in atrial caval shunting (ACS) for type Ⅱ Budd-Chiari syndrome(BCS). Methods: The clinical data of patients underwent ACS for type Ⅱ BCS in the Henan Province People's Hospital from January 2014 to June 2016 were prospectively analyzed.Randomized and single-blind, controlled study was performed among the patients, and all of them underwent ACS and were divided into control group (patients underwent traditional perioperative management) and ERAS group (patients underwent ERAS perioperative management) based on a random number table...
September 1, 2017: Zhonghua Wai Ke za Zhi [Chinese Journal of Surgery]
https://www.readbyqxmd.com/read/28838449/successful-blood-transfusion-management-of-a-living-donor-liver-transplant-recipient-in-the-presence-of-anti-jr-a-a-case-report
#5
N Kurata, Y Onishi, H Kamei, T Hori, M Komagome, C Kato, T Matsushita, Y Ogura
A 48-year-old Japanese woman was diagnosed with Budd-Chiari syndrome and transferred for possible living donor liver transplantation (LDLT). Examinations before LDLT revealed that the recipient had anti-Jr(a) and preformed donor-specific anti-human leukocyte antigen (HLA) antibodies (DSA). Rituximab was administrated at 16 days prior to the patient's scheduled LDLT for the prophylaxis of antibody-mediated rejection by DSA. The clinical significance of anti-Jr(a) has not been clearly established because of the rarity of this antibody, so we discussed blood transfusion strategy with the Department of Blood Transfusion Service and prepared for Jr(a)-negative packed red blood cells (RBCs)...
September 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28834866/liver-cirrhosis-caused-by-chronic-budd-chiari-syndrome
#6
Mengjie Lin, Feng Zhang, Yi Wang, Bin Zhang, Wei Zhang, Xiaoping Zou, Ming Zhang, Yuzheng Zhuge
Chronic Budd-Chiari syndrome (BCS) is a rare cause of liver cirrhosis (LC) and tends to be misdiagnosed in clinical practice. In order to characterize LC caused by chronic BCS, we conducted this retrospective observational study. Medical records of all patients who were initially diagnosed as chronic BCS with LC when discharged from our department from January, 2011 to October, 2016 were reviewed. Cirrhotic patients with known causes and cases lacked key data were excluded. Data of remaining patients was collected and analyzed...
August 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28834123/pregnancy-outcomes-in-women-with-budd-chiari-syndrome-before-onset-of-symptoms-and-after-treatment
#7
Akash Shukla, Abhishek Sadalage, Deepak Gupta, Amit Gupte, Anupam Mahapatra, Dipendu Mazumder, Chirag Shah, Tejas Modi, Megha Meshram, Prashant Dhore, Shobna Bhatia
BACKGROUND AND AIMS: Pregnancy is known to have poor outcomes in women with Budd Chiari syndrome. There are no data on fertility and pregnancy outcomes prior to onset of symptoms or the effect of therapy on these parameters. We therefore evaluated reproductive profile of women with Budd Chiari Syndrome before the onset of symptoms and after therapy. PATIENTS AND METHODS: Eighty women with Budd Chiari Syndrome (29 years [20-45]) were enrolled over 8 years. Baseline demographic characteristics, disease severity, thrombophilic disorders, and treatment were reviewed...
August 21, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28828590/direct-reopening-of-the-occluded-hepatic-veins-of-budd-chiari-syndrome-verification-of-our-operative-method-by-the-perioperative-course-of-esophageal-varices
#8
Yukio Kuniyoshi, Hitoshi Inafuku, Satoshi Yamashiro, Yuya Kise, Takaaki Nagano, Ryoko Arakaki, Tatsuya Maeda, Mizuki Ando, Shotaro Higa
OBJECTIVE: A total of 69 patients with Budd-Chiari syndrome (BCS) were operated by direct approach under cardiopulmonary bypass (CPB). To assess the operative procedure, the perioperative course of esophageal varices (EVs) was evaluated. PATIENTS AND METHODS: Of the 69 patients, 59 (22 females) were enrolled in this study because they had complete follow-up data for endoscopic evaluation of EVs. Their mean age was 46.3 ± 13.0 years (range 21-73.3 years). EVs were found in 52 patients...
August 21, 2017: General Thoracic and Cardiovascular Surgery
https://www.readbyqxmd.com/read/28828121/percutaneous-sharp-recanalization-of-a-membranous-ivc-occlusion-with-an-occlusion-balloon-as-a-needle-target
#9
Michael D Rivers-Bowerman, Christopher B Lightfoot, Ruairi P Meagher, Michael D Carter, Robert F Berry
A 50-year-old male with right upper quadrant symptoms and hepatic dysfunction was found to have multiple dilated hepatic veins (HVs) with intrahepatic collateralization and membranous occlusion of the intrahepatic inferior vena cava (IVC) consistent with primary Budd-Chiari syndrome. Venacavograms depicted drainage of the intrahepatic collaterals through a left-sided HV entering the IVC above the level of the occlusion. Sharp recanalization of the membranous IVC occlusion was performed with an occlusion balloon as a needle target under echocardiographic monitoring followed by balloon angioplasty with restoration of IVC patency...
September 2017: Radiology case reports
https://www.readbyqxmd.com/read/28763339/long-term-outcomes-of-transjugular-intrahepatic-portosystemic-shunt-in-indian-patients-with-budd-chiari-syndrome
#10
Shalimar, Shivanand R Gamanagatti, Arpan H Patel, Saurabh Kedia, Baibaswata Nayak, Deepak Gunjan, Gyan Ranjan, Shashi B Paul, Subrat K Acharya
BACKGROUND/AIM: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in Budd-Chiari syndrome (BCS) based on current data. Our objective was to evaluate outcomes and assess prognostic factors in BCS patients undergoing TIPS. PATIENTS AND METHODS: In this retrospective analysis of a propectively maintained database, all consecutive BCS patients undergoing TIPS from September 2010 to February 2017 were included. Complete response after TIPS was defined as resolution of symptoms (ascites/pedal edema) with no requirement of diuretics at the end of 4 weeks...
October 2017: European Journal of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28761473/multiple-complications-by-hydatid-cyst-induced-budd-chiary-syndrome-a-case-report
#11
Feidoun Sabzi, Reza Faraji
A case of the Budd Chiary Syndrome (BCS) in a 43-yr-old man with hydatid cyst (HC) in the liver is reported from Imam Ali Hospital, Kermanshah, western Iran in 2016. This case was complicated by inferior vena cava (IVC) thrombosis, right atrial clot and pulmonary emboli. Compression of IVC was the likely mechanism. Abdominal ultrasonography revealed a huge HC located in segments near IVC and caused compression of IVC. Transthoracic echocardiography (TTE) revealed IVC and right atrium thrombosis, however pulmonary emboli was not documented in TTE but intra operative exploration showed multiple clot in main and left pulmonary artery branch...
January 2017: Iranian Journal of Parasitology
https://www.readbyqxmd.com/read/28745424/orthotopic-liver-transplantation-in-a-pediatric-patient-with-iatrogenic-budd-chiari-syndrome-complicated-by-bronchobiliary-fistula
#12
Lei Yang, Zhiyong Guo, Lu Yang, Weiqiang Ju, Dongping Wang, Xiaoshun He
We report a clinical case of OLT in a 10-year-old boy with iatrogenic BCS and BBF following three operations because of traumatic liver rupture and pulmonary contusion caused by a traffic accident 4 years ago. On follow-up, the child was asymptomatic.
July 26, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/28740853/prothrombotic-fibrin-clot-phenotype-in-patients-with-deep-vein-thrombosis-and-pulmonary-embolism-a-new-risk-factor-for-recurrence
#13
REVIEW
Anetta Undas
Prothrombotic fibrin clot phenotype, involving faster formation of dense meshwork composed of thinner and highly branched fibers that are relatively resistant to plasmin-induced lysis, has been reported in patients with not only myocardial infarction or stroke, but also venous thromboembolism (VTE), encompassing deep vein thrombosis (DVT), and/or pulmonary embolism (PE). Prothrombotic fibrin clot phenotype, in particular prolonged clot lysis time, is considered a novel risk factor for VTE as well as venous thrombosis at unusual location, for example, cerebral sinus venous thrombosis, retinal vein obstruction, and Budd-Chiari syndrome...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28739463/mesoatrial-shunt-for-budd-chiari-syndrome-a-30-year-experience
#14
Guang-Chang Zhu, Zhong-Gao Wang, Ce Bian, Jian-Wei Zhang, Zhi-Wei Hu, Guo-Feng Hou, Wei Guo, Chao Ma
BACKGROUND: The long-term efficacy of mesoatrial shunt (MAS) for Budd-Chiari syndrome (BCS) is not well studied. The purpose of our study was to investigate the long-term outcome and efficacy of MAS for BCS. METHODS: We retrospectively evaluated eleven patients who underwent MAS for BCS from April 1986 to November 1995. Records of patients' clinical presentations, laboratorial inves- tigation, Doppler duplex ultrasonography, radiologic image and treatment outcomes were all retrieved and analyzed...
July 21, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28738751/a-single-center-retrospective-study-clinical-features-of-different-types-of-budd-chiari-syndrome-in-chinese-patients-in-the-hubei-area
#15
Gangping Li, Ying Huang, Shunyu Tang, Yuhu Song, Huimin Liang, Dehan Liu, Ling Yang, Xiaohua Hou
Background The characteristics and prevalence of Budd-Chiari syndrome in China remain unclear. This study aimed to analyze the clinical features of Budd-Chiari syndrome in Chinese patients in the Hubei area. Methods One-hundred and thirty patients with Budd-Chiari syndrome, admitted to Union Hospital from January 2002 to January 2011, were included in this retrospective study. Clinical features, laboratory data, imaging characteristics, and cumulative patency rates were analyzed. Results Of the 130 patients with Budd-Chiari syndrome, 77 were men (59...
January 1, 2017: Vascular
https://www.readbyqxmd.com/read/28710306/ruxolitinib-treatment-in-an-infant-with-jak2-polycythaemia-vera-associated-budd-chiari-syndrome
#16
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
#17
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
#18
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
#19
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
#20
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
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