Mengdie Xu, Lin Cao, Xiaokang Zhang, Yu Zhuang, Ying Zhang, Qinzhi Wang, Yansu Chen, Lichun Xu, Guixiang Sun
Membranous obstruction of the inferior vena cava (MOVC) has the highest incidence rate among the different types of Budd‑Chiari syndrome (BCS) in China. The inferior vena cava septum of patients with MOVC contains capillaries and the two surfaces of the membrane are composed of vascular endothelial tissue. Membrane formation occurs due to endothelial damage. MicroRNAs (miRNAs/miRs) have been verified to be involved in the pathogenesis and progression of various human diseases. A previous study by our group suggested that miR‑3133 was downregulated in the serum of patients with MOVC...
August 4, 2020: Oncology Reports
Kumble Seetharama Madhusudhan, Sanjay Sharma
Budd-Chiari syndrome is a vascular pathology of the liver, commonly seen in adults and uncommon in children. Most children with Budd-Chiari syndrome present with ascites, and an etiology is found in only about 50%. Ultrasonography (US) with color Doppler is the main modality used in the diagnosis. US imaging additionally guides radiologic interventions and follow-up after recanalization or shunt procedure. In this pictorial review, we illustrate the findings in pediatric Budd-Chiari syndrome as seen on B-mode and color Doppler US and describe the role of US in guiding percutaneous radiologic interventions, with a brief description of the role of US contrast agent and sonoelastography in this setting...
September 1, 2020: Pediatric Radiology
Tao Wang, Yong-Jun Mei, Zhi-Jun Li
No abstract text is available yet for this article.
August 28, 2020: Chinese Medical Journal
Wolfgang Füreder, W R Sperr, S Heibl, A Zebisch, M Pfeilstöcker, G Stefanzl, E Jäger, G Greiner, I Schwarzinger, M Kundi, F Keil, G Hoermann, P Bettelheim, P Valent
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematologic disease characterized by a deregulated complement system, chronic Coombs-negative, intravascular hemolysis, and a variable clinical course with substantial risk to develop thromboembolic events. We analyzed diagnostic and prognostic parameters as well as clinical endpoints in 59 adult patients suffering from PNH in 5 hematology centers in Austria (observation period: 1978-2015). Median follow-up time was 5.6 years. The median clone size at diagnosis amounted to 55% and was higher in patients with classical PNH (81%) compared to patients with PNH associated with aplastic anemia (AA) or myelodysplastic syndromes (MDS) (50%)...
August 27, 2020: Annals of Hematology
Seetha Lakshmanan, Dhanya Baskaran, Yashvin Onkarappa Mangala, Nabil Toubia
Budd-Chiari syndrome (BCS) occurs when there is hepatic venous outflow obstruction. Chronic BCS may result in liver cirrhosis due to long-standing obstruction and tend to present late. We present the first case of BCS secondary to Janus tyrosine kinase 2 (JAK2) mutation resulting in "pseudocirrhosis" rather than cirrhosis of the liver. Pseudocirrhosis clinically and radiologically mimics cirrhosis without the classical histopathological changes, and it is usually associated with metastatic cancers...
July 23, 2020: Curēus
Priyavrat Bhatia, Alok Sen, Gaurav Mohan Kohli, Pratik Shenoy
Behcet's disease (BD) is a multisystem disorder with a classical triad of recurrent oral ulcers, genital ulcers, and uveitis. It involves both arterial and venous system which can lead to life-threatening complications. Both superficial and deep venous systems can be involved leading to peripheral skin lesions and devastating complications like cerebral venous thrombosis and Budd-Chiari syndrome (BCS). This report describes a case of an HLA B-52 positive BD in a 22-year-old woman who presented with retinal vasculitis and venous ulcer on the foot and later on developed ascites due to obstruction of supra-hepatic inferior vena cava (BCS)...
September 2020: Indian Journal of Ophthalmology
Taryn J Rohringer, Christian Zaarour, Suzan Williams, Dimitri A Parra
This case report describes a 4-year-old male with a history of hepatoblastoma, treated with chemotherapy followed by a right extended hepatectomy. Secondary to this, the patient experienced recurrent hepatic vein stenosis. He was treated initially with conventional angioplasty, followed by paclitaxel-coated balloon dilatations in an attempt to prevent episodes of re-stenosis. During the catheterization of the hepatic vein in one of the treatments, hemodynamic instability due to an acute Budd-Chiari syndrome occurred...
October 2020: Radiology Case Reports
Pankaj Gupta, Suzanne Koshi, Saroj K Sinha, Vishal Sharma, Harshal Mandavdhare, Jayanta Samanta, Usha Dutta, Rakesh Kochhar
BACKGROUND: Doppler is the screening modality of choice for assessment of patients suspected of Budd Chiari syndrome (BCS). The aim of this study was to compare the diagnostic value of contrast enhanced ultrasound (CEUS) with Doppler in the initial evaluation of patients with BCS. METHODS: This was a retrospective study of patients with suspicion of BCS who underwent CEUS of the hepatic veins and inferior vena cava between July 2017 and April 2019. CEUS was performed using Sonovue...
July 25, 2020: Current Problems in Diagnostic Radiology
Wei Zhang, Yu-Long Tian, Qiao-Zheng Wang, Xiao-Wei Chen, Qi-Yang Li, Jin-Hang Han, Xu-Dong Chen, Ke Xu
BACKGROUND: Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction. For Asian Budd-Chiari syndrome patients, the major treatment modality is recanalization (percutaneous transluminal angioplasty with or without stent implantation). The cumulative 1-, 5-, and 10-year primary patency rates and survival rates are reported to be excellent or satisfactory, but the long-term outcome of patients with restenosis (the most common complication after recanalization) is unknown...
July 26, 2020: World Journal of Clinical Cases
Stefania Gioia, Emanuela De Santis, Bruna Cerbelli, Silvia Nardelli, Lorenzo Ridola, Adriano De Santis, Giulia d'Amati, Oliviero Riggio
Small hepatic veins Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this case only a liver biopsy can demonstrate the presence of a small vessels outflow block. Paroxysmal nocturnal haemoglobinuria (PNH) is one of the most severe acquired thrombophilic state and represents one of the main aetiological factors of Budd-Chiari syndrome. In patient affected by PNH with liver impairment and/or ascites, Budd-Chiari syndrome must be always taken into consideration and, if necessary, a liver biopsy performed to exclude the small hepatic veins involvement...
June 2020: Pathologica
Yonghua Bi, Xiaoyan Zhu, Mengfei Yi, Xinwei Han, Jianzhuang Ren
OBJECTIVE: We aimed to analyze the pathological changes of the occlusive area of the inferior vena cava (IVC) in patients with Budd-Chiari syndrome (BCS) and to explore its possible etiology and pathogenesis. METHODS: Color Doppler ultrasound and IVC angiography were used to evaluate causes of occlusion. Clamp biopsies were performed in the occlusive area of the IVC in 31 patients with BCS, and the specimens were examined pathologically. Clinical data were collected retrospectively...
July 2020: Journal of International Medical Research
A Sharma, S N Keshava, A Eapen, E Elias, C E Eapen
Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN...
July 20, 2020: Digestive Diseases and Sciences
Giovanna Ferraioli, Richard G Barr
Several guidelines have indicated that liver stiffness (LS) assessed by means of shear wave elastography (SWE) can safely replace liver biopsy in several clinical scenarios, particularly in patients with chronic viral hepatitis. However, an increase of LS may be due to some other clinical conditions not related to fibrosis, such as liver inflammation, acute hepatitis, obstructive cholestasis, liver congestion, infiltrative liver diseases. This review analyzes the role that SWE can play in cases of liver congestion due to right-sided heart failure, congenital heart diseases or valvular diseases...
June 28, 2020: World Journal of Gastroenterology: WJG
Amit Tanwar, Gaurav K Gupta, Virender Chauhan, Deepak Sharma, Mukesh K Jain, Hemendra Bhardwaj, Ashok Jhajharia, Sandeep Nijhawan
Introduction: Celiac disease (CD) has been linked to portal hypertension (PHT) of varied etiology, but the causality association has never been proved. We aim to study the prevalence of CD in patients of PHT of different etiology. Methods: A prospective observational study was conducted from June 2017 to December 2018 involving all the cases of PHT of varied etiology. Consecutive patients of PHT with chronic liver disease (CLD) of defined etiology like ethanol, viral hepatitis (B or C), Budd-Chiari syndrome (BCS), autoimmune-related cirrhosis, and cryptogenic CLD (cCLD) (group A) and those with noncirrhotic PHT (NCPHT), which included noncirrhotic portal fibrosis (NCPF) and extrahepatic portal vein obstruction (EHPVO) (group B), were screened for CD by IgA anti-tTG antibody followed by duodenal biopsy in serology-positive patients...
July 2020: Journal of Clinical and Experimental Hepatology
Lamia Y K Haque, Joseph K Lim
Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS...
August 2020: Clinics in Liver Disease
Wei Zhang, Chunyan Peng, Song Zhang, Shuling Huang, Shanshan Shen, Guifang Xu, Feng Zhang, Jiangqiang Xiao, Ming Zhang, Yuzheng Zhuge, Lei Wang, Xiaoping Zou, Ying Lv
BACKGROUND AND AIMS: EUS-guided portal pressure gradient (PPG) measurement is a novel method to evaluate portal hypertension severity. In this study, we determined the consistency between EUS-PPG and hepatic venous pressure gradient (HVPG) measurements in patients with acute or sub-acute portal hypertension. METHODS: Twelve patients were prospectively enrolled. EUS-guided PPG measurements were performed using a 22-gauge fine-needle aspiration (FNA) needle and a central venous pressure measurement monitor...
June 29, 2020: Gastrointestinal Endoscopy
Rajeev Redkar, Vinod Raj, Shruti Tewari, Rahul Sharma, Shirin Joshi
INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting children. It is characterized by occlusion of venous outflow from liver at the level of hepatic veins (HV) or inferior vena cava (IVC). The management of BCS in children revolves around forming new collaterals for venous outflow or by elimination of blockage in the venous outflow tracts. These can be achieved by balloon venoplasty (BV), transjugular intrahepatic portosystemic shunting (TIPSS) or open shunt surgeries...
May 23, 2020: Journal of Pediatric Surgery
K M Wcislo, C E Hall, N Abbassi-Ghadi
Acute Budd-Chiari syndrome is a rare condition characterised by obstruction of hepatic venous outflow. We describe the case of a 52-year-old man, with a congenital Morgagni diaphragmatic hernia, who presented with acute onset abdominal pain, shortness of breath, lactic acidosis, hyperbilirubinaemia and transaminasaemia. Computed tomography revealed strangulation of the diaphragmatic hernia and extrinsic compression of the inferior vena cava from the herniated viscera. Emergency surgery was carried out to repair the hernia with a biosynthetic mesh, with complete resolution of the Budd-Chiari syndrome...
June 15, 2020: Annals of the Royal College of Surgeons of England
Bai-Guo Xu, Jing Liang, Ke-Feng Jia, Tao Han
BACKGROUND: Hereditary hemorrhagic telangiectasia (HHT) often involves the liver, and belongs to abnormal blood vessel disease. The etiology of Budd-Chiari syndrome (BCS) is not clear, but congenital vascular dysplasia is considered to be one of the causes. Liver cirrhosis due to hepatic hereditary hemorrhagic telangiectasia concomitant with BCS has not been reported. Here, we report a case of cirrhosis with hepatic hereditary hemorrhagic telangiectasia (HHHT) and BCS. CASE PRESENTATION: A 58-year-old woman with hepatic hereditary hemorrhagic telangiectasia showed decompensated liver cirrhosis, and abdominal imaging revealed Budd-Chiari syndrome...
June 3, 2020: BMC Gastroenterology
Huawei Li, Xi Zhang, Hao Xu, Maoheng Zu, Ning Wei, Hongtao Liu, Jinchang Xiao, Bin Shen, Qianxin Huang, Qingqiao Zhang
OBJECTIVE: Interventional treatment is the main therapy for Budd-Chiari syndrome (BCS) with hepatic vein obstruction. The aim of this study was to investigate the long-term outcomes of endovascular management for BCS with chronic accessory hepatic vein (AHV) obstruction. METHODS: In total, 68 patients with primary BCS who underwent AHV dilation with or without stenting from January 2008 to December 2018 were included in this retrospective study. The technical success rate and complications were recorded...
May 29, 2020: European Journal of Gastroenterology & Hepatology
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