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Budd–Chiari syndrome

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https://www.readbyqxmd.com/read/29316565/transjugular-intrahepatic-portosystemic-shunt-in-patients-with-portal-hypertension-patency-depends-on-coverage-and-interventionalist-s-experience
#1
Matthias Buechter, Paul Manka, Guido Gerken, Ali Canbay, Sandra Blomeyer, Axel Wetter, Jens Altenbernd, Alisan Kahraman, Jens M Theysohn
BACKGROUND AND AIMS: Transjugular intrahepatic portosystemic shunt (TIPS) is the treatment of choice in decompensated portal hypertension. TIPS revision due to thrombosis or stenosis increases morbidity and mortality. Our aim was to investigate patient- and procedure-associated risk factors for TIPS-revision. PATIENTS AND METHODS: We retrospectively evaluated 189 patients who underwent the TIPS procedure. Only patients who required TIPS revision within 1 year (Group I, 34 patients) and patients who did not require re-intervention within the first year (Group II [control group], 54 patients) were included...
January 9, 2018: Digestive Diseases
https://www.readbyqxmd.com/read/29316476/numerical-simulation-of-hemodynamics-in-membranous-obstruction-of-the-suprahepatic-inferior-vena-cava-based-on-a-subject-specific-budd-chiari-syndrome-model
#2
Deqiang Cheng, Yinping Zhuang, Qiqi Kou, Min Zhang, Yinghong Zhao, Cuiping Han, Jingjing Li, Yong Wang, Kai Xu, Fei Mo, Jiawei Zhang
BACKGROUND: This study was performed to determine the hemodynamic changes of Budd-Chiari syndrome when the inferior vena vein membrane is developing. METHODS: A patient-specific Budd-Chiari syndrome vascular model was reconstructed based on magnetic resonance images using Mimics software and different degrees (16%, 37%, and 54%) of idealized membrane were built based on the Budd-Chiari syndrome vascular model using Geomagic software. Three membrane obstruction Budd-Chiari syndrome vascular models were established successfully and fluent software was used to simulate hemodynamic parameters, including blood velocity and wall shear stress...
January 2, 2018: Clinical Biomechanics
https://www.readbyqxmd.com/read/29297972/prognostic-scoring-systems-and-outcome-of-endovascular-radiological-intervention-of-chronic-budd-chiari-syndrome-in-children
#3
Sumit Kumar Singh, Moinak Sen Sarma, Rajanikant Yadav, Sheo Kumar, Raghunandan Prasad, Surender Kumar Yachha, Anshu Srivastava, Ujjal Poddar
BACKGROUND AND AIM: Prognostic scoring systems (PSS) have not been validated in children with chronic Budd-Chiari syndrome (BCS). We aimed to analyse the long-term outcome of radiological intervention (RI) and validate the PSS in children. METHODS: Chronic BCS children were analysed in four subgroups: SI: successful intervention (primary or secondary stent patency) b) PO: poor outcome (refractory stent block or requirement of liver transplantation), c) NU: naïve unintervened (awaiting RI) and d) DBI: died before intervention...
January 3, 2018: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/29296024/beh%C3%A3-et-syndrome-a-contemporary-view
#4
REVIEW
Hasan Yazici, Emire Seyahi, Gulen Hatemi, Yusuf Yazici
The presence of symptom clusters, regional differences in disease expression and similarities with, for example, Crohn's disease suggest multiple pathological pathways are involved in Behçet syndrome. These features also make formulating disease criteria difficult. Genetic studies have identified HLA-B*51 to be the important genetic risk factor. However, the low prevalence of HLA-B*51 in many patients with bone fide disease, especially in non-endemic regions, suggests other factors must also be operative in Behçet syndrome...
January 3, 2018: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/29285598/diagnosis-of-budd-chiari-syndrome
#5
Morgane Van Wettere, Onorina Bruno, Pierre-Emmanuel Rautou, Valérie Vilgrain, Maxime Ronot
Budd-Chiari syndrome (BCS) is defined by clinical and laboratory signs associated with partial or complete impairment of hepatic venous drainage in the absence of right heart failure or constrictive pericarditis. Primary BCS is the most frequent type and is a complication of hypercoagulable states, in particular myeloproliferative neoplasms. Secondary BCS involves tumor invasion or extrinsic compression. Most patients present with chronic BCS including a non-cirrhotic, dysmorphic, chronic liver disease with various degrees of fibrosis deposition...
December 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/29219632/budd-chiari-syndrome-associated-with-al-amyloidosis-a-coagulation-paradox
#6
Guilherme Grossi Lopes Cançado, Luciana Costa Faria, Fernanda Maria Farage Osório, Paula Vieira Teixeira Vidigal, Cláudia Alves Couto, Teresa Cristina de Abreu Ferrari
No abstract text is available yet for this article.
December 8, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29216531/living-donor-liver-transplantation-for-adult-budd-chiari-syndrome-resection-without-replacement-of-retrohepatic-ivc-a-case-report
#7
Tarek Abdelazeem Sabra, Hideaki Okajima, Tetsuya Tajima, Ken Fukumitsu, Koichiro Hata, Kentaro Yasuchika, Toshihiko Masui, Kojiro Taura, Toshimi Kaido, Shinji Uemoto
INTRODUCTION: Suprahepatic caval resection and replacement of inferior vena cava (IVC) is standard procedure in deceased donor liver transplantation for patients with Budd-Chiari syndrome (BCS). However, replacement of IVC in living donor liver transplantation (LDLT) is difficult. We report a case of BCS successfully treated by LDLT without replacement of IVC. PRESENTATION OF CASE: A 52-years-old female with a primary BCS due to IVC thrombosis. A vena cava (VC) stent placed after angioplasty without improvement of the hepatic, portal venous flow and liver functions, Transjugular intrahepatic portosystemic shunt was considered and the patient had a rapid deterioration and increased ascites...
December 1, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/29211374/-splanchnic-vein-thrombosis
#8
Bobby Gouin, Helia Robert-Ebadi, Alessandro Casini, Yan Beauverd, Pierre Fontana, Marc Righini, Laure Elkrief
Splanchnic vein thromboses include thrombosis of the portal venous system (including the portal, mesenteric and splenic vein) and hepatic vein thrombosis (also called Budd-Chiari syndrome). They are rare manifestations of venous thromboembolism. These thromboses are frequently associated with local or systemic factors. The therapeutic approach is often complex due to heterogeneity of patients and limited available data in the literature. The cornerstone of treatment is anticoagulation. However, the bleeding risk, related to portal hypertension, should be accurately assessed to individualize the treatment...
December 6, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/29202678/approach-to-thrombosis-at-unusual-sites-splanchnic-and-cerebral-vein-thrombosis
#9
REVIEW
Nicoletta Riva, Walter Ageno
Splanchnic vein thrombosis (SVT) and cerebral vein thrombosis (CVT) are two manifestations of unusual site venous thromboembolism (VTE). SVT includes thrombosis in the portal, mesenteric or splenic veins, and the Budd-Chiari syndrome. CVT encompasses thrombosis of the dural venous sinuses and thrombosis of the cerebral veins. Unusual site VTE often represents a diagnostic and therapeutic challenge because of the heterogeneity in clinical presentation, the limited evidence available in the literature on the acute and long-term prognosis of these diseases, and the lack of large randomized controlled trials evaluating different treatment options...
December 2017: Vascular Medicine
https://www.readbyqxmd.com/read/29198688/hanging-hepato-atrial-anastomosis-in-deceased-donor-liver-transplantation-for-budd-chiari-syndrome-with-extensive-vena-cava-obliteration-a-case-report
#10
S Y Hong, B-W Kim, H-J Wang, I-G Kim, X-G Hu
INTRODUCTION: Although outcomes of liver transplantation (LT) have improved as the result of progress in surgical procedures, a failure to restore sufficient graft outflow may yield fatal consequences including graft dysfunction and even graft loss to date. In particular, patients with pre-existing obliterated venous drainage, such as those with Budd-Chiari syndrome (BCS), are at high risk of having venous complications followed by conventional LT. In selected cases, the transplant surgeons are compelled to modify the surgical procedures of LT from the conventional procedure...
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29187341/single-access-liver-floss-technique-with-antegrade-hepatic-vein-access-and-recanalization-in-budd-chiari-syndrome
#11
John J Weaver, Ethan M Dobrow, Evelyn K Hsu, Eric J Monroe
A 14-year-old boy presented with several months of increasing abdominal girth and fatigue. Imaging confirmed massive ascites and hepatic congestion secondary to central hepatic venous obstruction. Several large intrahepatic collateral veins were seen draining via caudate and emissary veins. After an unsuccessful attempt at retrograde recanalization utilizing intravascular ultrasound, the right hepatic vein was recanalized in an antegrade fashion by way of a prominent caudate collateral vein, and subsequently stented...
November 30, 2017: Diagnostic and Interventional Radiology: Official Journal of the Turkish Society of Radiology
https://www.readbyqxmd.com/read/29054942/primary-hepatic-hemangioendothelioma-in-a-patient-with-budd-chiari-syndrome
#12
Preet Mukesh Shah, Vijay Waman Dhakre, Aabha Nagral
A 36-year-old woman was diagnosed with compensated cirrhosis of liver secondary to Budd-Chiari syndrome (BCS) and had undergone stenting of a thrombosed left hepatic vein. Eight months later, she presented with jaundice and right upper quadrant pain. CT revealed multiple focal lesions in the liver, which on biopsy proved to be hepatic hemangioendothelioma (HHE). Her liver disease and ascites progressively increased. Four months later, magnetic resonance cholangiopancreatography showed an advanced stage of HHE with infiltration of the common bile duct and vascular invasion with a blocked stent, with metastasis to the spleen and dorsolumbar vertebrae...
October 19, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/29043403/hepatic-morphology-abnormalities-beyond-cirrhosis
#13
REVIEW
Giuseppe Mamone, Kelvin Cortis, Aquilina Sarah, Settimo Caruso, Roberto Miraglia
The diagnosis of cirrhosis can be reached on the basis of established hepatic morphological changes. However, some other conditions can mimic cirrhosis. The aim of this pictorial essay is to review the CT and MRI appearances of hepatic morphology abnormalities in the cirrhotic liver and other diseases, describing pathologic conditions that can mimic cirrhosis, with useful tips for the differential diagnosis. Mimickers of cirrhosis include congenital hepatic fibrosis, Caroli disease, Budd-Chiari Syndrome, hepatoportal sclerosis, cavernous transformation of the portal vein, pseudocirrhosis from metastatic disease, acute liver failure, post-therapeutic morphologic changes in the liver, and infective conditions including schistosomiasis and oriental cholangiohepatitis...
October 17, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28988356/image-guided-treatment-of-budd-chiari-syndrome-a-giant-leap-from-the-past-a-small-step-towards-the-future
#14
Qiuhe Wang, Guohong Han
Budd-Chiari syndrome (BCS) is a relatively rare vascular disease characterized by hepatic outflow tract obstruction, and image-guided endovascular treatment, namely percutaneous angioplasty, stenting, and transjugular intrahepatic portosystemic shunt (TIPS), has proven to be effective treatment modalities to alleviate symptoms and markedly improve the prognosis of the disease. Specifically, a step-wise approach is recommended, i.e., angioplasty and stenting are the prioritized choice for patients with membranous obstruction and short-length stenosis, whereas TIPS is the option for patients who fail this treatment...
October 7, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28983615/identification-of-differentially-expressed-genes-in-budd%C3%A2-chiari-syndrome-by-rna%C3%A2-sequencing
#15
Bin Yang, Dong Qu, An-Li Zhao, Yu Li, Ran-Ran Meng, Ji-Xiang Yu, Peng Gao, Hua Peng Lin
Budd‑Chiari syndrome (BCS) is an uncommon disease characterized by the occlusion or obstruction of hepatic venous outflow. The mechanism of BCS is still unclear and there are no accurate and effective diagnostic or therapeutic tools. In the present study, blood samples from BCS patients and healthy controls were used for RNA‑sequencing. The differentially expressed genes (DEGs) in BCS patients compared with healthy controls were identified. Gene Ontology (GO) enrichment analysis, Kyoto Encyclopedia of Genes and Genomes pathway analysis and Protein‑Protein Interaction (PPI) networks construction were performed for DEGs...
December 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28932502/ultrasound-guided-direct-intrahepatic-portosystemic-shunt-in-patients-with-budd-chiari-syndrome-short-and-long-term-results
#16
Adam Hatzidakis, Nikolaos Galanakis, Elias Kehagias, Dimitrios Samonakis, Mairi Koulentaki, Erminia Matrella, Dimitrios Tsetis
BACKGROUND AND AIMS: Budd-Chiari syndrome (BCS) is treated with anticoagulation therapy, transjugular intrahepatic portosystemic shunt (TIPS), angioplasty, and liver transplantation. TIPS is not always technically feasible, due to the complete hepatic venous thrombosis. Direct intrahepatic portosystemic shunt (DIPS) is an alternative method for decompression of portal hypertension. This is a retrospective, single-center study aiming to evaluate the outcome of ultrasound (US)-guided DIPS in patients with BCS...
June 2017: Interventional Medicine & Applied Science
https://www.readbyqxmd.com/read/28923635/living-donor-liver-transplantation-for-budd-chiari-syndrome-case%C3%A2-series
#17
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
#18
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
#19
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
#20
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]
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