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budd chiary syndrome

Mohammad A Sakr, Sara M Abdelhakam, Hany M Dabbous, Ahmed S Abdelmoaty, Hend E Ebada, Wael M Al-Banna, Mohamed S Ghazy, Mohamed E Aboelmaaty, Ahmed K Eldorry
INTRODUCTION AND AIM: Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction. This work aims to analyze the pattern of vascular involvement in Egyptian patients with BCS, demonstrates its relation to etiology and shows its impact on clinical presentation. MATERIAL AND METHODS: The current retrospective study was conducted at The Tropical Medicine Department, Ain Shams University on one hundred Egyptian patients with confirmed diagnosis of primary BCS who were presented to the Budd-Chiari Study Group (BCSG) from April 2014 to May 2016 by collecting clinical, laboratory and radiological data from their medical records...
July 2018: Annals of Hepatology
Brian W Wakefield, Crystal M C Masterson, Manuel T Borges, K Joseph Hurt
Stroke and hepatic vein thrombosis are highly associated with neoplasia but are extremely rare events in young, pregnant women. Rare and recurrent thrombotic events in pregnancy increase the suspicion for occult malignancy. We describe the case of a healthy 31-year-old G2P1 who presented with visual changes and dysarthria during pregnancy. Imaging showed cerebral infarcts. Her thrombophilia evaluation was negative. During delivery, she was diagnosed with fulminant Budd-Chiari Syndrome. Hepatic ultrasound suggested malignancy or metastasis, and postpartum CT scan and biopsy confirmed the diagnosis of Stage IV pancreatic cancer...
June 8, 2018: Gynecologic and Obstetric Investigation
Bhupinder Singh, Budnur C Srinivas
No abstract text is available yet for this article.
June 4, 2018: CMAJ: Canadian Medical Association Journal, Journal de L'Association Medicale Canadienne
Yonghua Bi, Hongmei Chen, Pengxu Ding, Pengli Zhou, Jianzhuang Ren, Xinwei Han
PURPOSE: To evaluate the safety and efficacy of percutaneous transhepatic route creation for hepatic vein (HV) angioplasty in Budd-Chiari syndrome (BCS). PATIENTS AND METHODS: Between April 2012 and August 2016, a total of 19 BCS patients underwent percutaneous transhepatic route creation for HV angioplasty after transvenous catheterization failure in this study. Color Doppler ultrasonography was required in all patients after admission and during follow-up. Data were retrospectively collected, and follow-up observations were performed...
May 31, 2018: Journal of Laparoendoscopic & Advanced Surgical Techniques. Part A
Yonghua Bi, Hongmei Chen, Penxu Ding, Jianzhuang Ren, Xinwei Han
BACKGROUND & AIMS: To compare long-term outcomes of retrievable stents and permanent stents for BCS due to long-segment obstructive IVC. METHODS: Between July 2000 and August 2016, 42 patients with BCS due to long-segment obstructive IVC were treated with retrievable stents (RS) and 41 patients were treated with permanent stents (PS). The retrievable stents was removed eventually after thrombus disappeared. Patients were subsequently followed-up by color Doppler sonography or CT scanning...
May 30, 2018: Journal of Gastroenterology and Hepatology
Hong-Wei Wang, Hua-Ning Shi, Jia Cheng, Fang Xie, Yu-Kun Luo, Jie Tang
PURPOSE: The first aim of this study was to analyze the relationships between liver stiffness measurement, hepatic venous pressure and liver fibrosis. The second aim was to demonstrate the utility of real-time shear wave elastography for evaluation of Budd-Chiari syndrome patients before and after balloon hepatic venous angioplasty. MATERIALS AND METHODS: A total of 32 patients with Budd-Chiari syndrome slated for successful balloon angioplasty met the inclusion and exclusion criteria...
2018: PloS One
Christine Sempoux, Charles Balabaud, Valérie Paradis, Paulette Bioulac-Sage
Hepatocellular nodules have been recognized in vascular liver diseases for a long time and mostly described and studied in the imaging literature. Some confusions in their identification and overlap in their definitions exist, especially in this specific clinical context. Pathology descriptions report the development of nodular regenerative hyperplasia, large regenerative nodule, and focal nodular hyperplasia, as adaptive responses of the liver parenchyma to the modified blood flow. True neoplastic hepatocellular nodules such as hepatocellular adenoma and hepatocellular carcinoma can also appear, mainly in Budd-Chiari syndrome, and have to be correctly diagnosed...
May 26, 2018: Virchows Archiv: An International Journal of Pathology
Isabelle Ollivier-Hourmand, Manon Allaire, Nathalie Goutte, Rémy Morello, Carine Chagneau-Derrode, Odile Goria, Jerôme Dumortier, Jean Paul Cervoni, Sébastien Dharancy, Nathalie Ganne-Carrié, Christophe Bureau, Nicolas Carbonell, Armand Abergel, Jean Baptiste Nousbaum, Rodolphe Anty, Hélène Barraud, Marie Pierre Ripault, Victor De Ledinghen, Anne Minello, Frédéric Oberti, Sylvie Radenne, Noelle Bendersky, Olivier Farges, Isabelle Archambeaud, Anne Guillygomarc'h, Marie Ecochard, Violaine Ozenne, Marie Noelle Hilleret, Eric Nguyen-Khac, Barbara Dauvois, Jean Marc Perarnau, Pascale Lefilliatre, Jean Jacques Raabe, Michel Doffoel, Jean Philippe Becquart, Eric Saillard, Dominique Valla, Thong Dao, Aurélie Plessier
INTRODUCTION: Epidemiological data is lacking on primary Budd-Chiari syndrome (BCS) in France. METHODS: Two approaches were used: (1) A nationwide survey in specialized liver units for French adults. (2) A query of the French database of discharge diagnoses screening to identify incident cases in adults. BCS associated with cancer, alcoholic/viral cirrhosis, or occurring after liver transplantation were classified as secondary. RESULTS: Approach (1) 178 primary BCS were identified (prevalence 4...
April 12, 2018: Digestive and Liver Disease
Miaomiao Li, Valerio De Stefano, Tingxue Song, Xinmiao Zhou, Zeqi Guo, Jia Zhu, Xingshun Qi
BACKGROUND: The prevalence of calreticulin (CALR) mutations in splanchnic vein thrombosis (SVT) varies among studies. The role of routine screening for CALR mutations in SVT patients remains a debate. AIM: To synthesize the prevalence of CALR mutations according to the different types (i.e., Budd-Chiari syndrome [BCS] and portal vein thrombosis [PVT]) and characteristics (i.e., with and without myeloproliferative neoplasms [MPNs] and JAK2V617F mutation) of SVT patients...
May 8, 2018: Thrombosis Research
Xixiang Gao, Zhu Tong, Lianrui Guo, Jianxin Li, Jian Zhang, Yongquan Gu
Budd-Chiari syndrome (BCS) is a rare but life-threatening disease. If not treated promptly, it is almost always lethal of progressive liver failure, severe nutritional depletion, and renal failure at the late stage. We report the successful treatment of a 5-year-old boy with BCS due to a stenosis of the IVC with a 26-year follow-up. We performed percutaneous transluminal angioplasty (PTA), which resulted in a great improvement of BCS, and the disappearance of ascites. Restenosis occurred at 25 years after the initial angioplasty, for which balloon angioplasty was repeatedly performed...
May 16, 2018: Annals of Vascular Surgery
A M Al-Sharydah, A H Al-Abdulwahhab, H A Abu AlOla
INTRODUCTION: In patients with a hypercoagulable state, such as patients with pulmonary embolism and/or Budd-Chiari syndrome, the complications from multiple gene mutations are more numerous than those from a single mutation. The authors present a woman with no major prior medical history who presented with pulmonary embolism and Budd-Chiari syndrome; this enigmatic co-occurrence has never been solely reported without underlying aetiology in a patient without prior medical conditions...
May 3, 2018: International Journal of Surgery Case Reports
Z-Y Guan, C-W Yu, T Song, Y Gao
OBJECTIVE: Budd-Chiari syndrome (BCS) is a life-threatening hepatic disease characterized by hepatic venous obstruction at the level of hepatic vein, hepatic venules, or inferior vena cava. No evidence reported the relationship between the endothelial progenitor cells and the deficiency of factor V Leiden and protein C in patients with primary Budd-Chiari syndrome. PATIENTS AND METHODS: We recruited participants between June 2014 and July 2015. For primary BCS group, 28 patients were collected...
May 2018: European Review for Medical and Pharmacological Sciences
Yonghua Bi, Hongmei Chen, Penxu Ding, Jianzhuang Ren, Xinwei Han
This study aimed to present long-term results of a 12-year patient follow-up of recoverable stents for BCS complicated by inferior vena cava (IVC) thrombosis. Forty consecutive patients with BCS complicated by IVC thrombosis were treated with recoverable stents. The median duration of symptoms was 24 months. Recoverable stents was placed after predilation of the obstructed IVC, and then agitation thrombolysis or catheter-directed thrombolysis of IVC was performed. The recoverable stents was removed eventually after thrombus disappeared...
May 9, 2018: Scientific Reports
T Yagi, K Takagi, R Yoshida, Y Umeda, D Nobuoka, T Kuise, T Fujiwara, A Takaki
When the Budd-Chiari syndrome (BCS) lesion extends to the inferior vena cava (IVC) or the orifices of the hepatic vein, the thickened IVC and/or hepatic vein wall must be removed and IVC reconstruction is required in living-donor liver transplantation (LDLT). In various reports about IVC resection in LDLT for BCS, there are none about left lobe liver transplantation with reconstruction of the retrohepatic IVC (rhIVC). To overcome removal and reconstruction of the rhIVC in LDLT for BCS, we introduced a composite IVC graft that is applicable to both right and left lobe partial liver grafts for LDLT for BCS...
May 2018: Transplantation Proceedings
Ru-Xin Song, Shi-Feng Cai, Shuang Ma, Zhi-Ling Liu, Yong-Hao Gai, Chun-Qing Zhang, Guang-Chuan Wang
Objective: This study aimed to illustrate the magnetic resonance venography (MRV) manifestations of obstructed hepatic veins (HVs), the inferior vena cava (IVC), and accessory hepatic veins (AHVs) in patients with Budd-Chiari syndrome (BCS) and to evaluate the visualization capacity of MRV in the diagnosis of BCS. Materials and Methods: Fifty-two patients with chronic BCS were included in this study. All patients were examined via MRV performed with a 3T system following injections of gadolinium-diethylene triamine pentaacetic acid (Gd-DTPA) or Gd-ethoxibenzyl-DTPA...
May 2018: Korean Journal of Radiology: Official Journal of the Korean Radiological Society
Amar Mukund, Kartik Mittal, Aniket Mondal, Shiv Kumar Sarin
PURPOSE: To assess overall outcome and midterm transplant-free survival of patients with Budd-Chiari syndrome (BCS) undergoing radiologic interventions including anatomic recanalization of the hepatic vein (HV) and inferior vena cava (IVC) and direct intrahepatic portosystemic shunt (DIPS) creation, both as combined and as independent groups. MATERIALS AND METHODS: From November 2010 to October 2014, 136 patients with BCS were treated with HV/IVC recanalization (group 1) or DIPS creation (group 2)...
June 2018: Journal of Vascular and Interventional Radiology: JVIR
Nadia Waheed, Huma Arshad Cheema, Hassan Suleman, Iqra Mushtaq, Zafar Fayyaz, Nadeem Anjum
OBJECTIVE: To differentiate between clinical and demographic spectrum, and outcome in hepatovenocaval syndrome (HVCS) and Budd-Chiari syndrome (BCS). STUDY DESIGN: Descriptive study. PLACE AND DURATION OF STUDY: Division of Pediatric Gastroenterology, Hepatology and Nutrition, The Children Hospital, Lahore, from January 2014 to January 2017. METHODOLOGY: All children less than 18 years of age, presenting with ascites and visible veins over abdomen, flanks and back were enrolled in the study...
May 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Russell Rosenblatt, Robert S Brown
Acute liver failure (ALF) is a rare but highly fatal condition. The most common causes include drug-induced and viral hepatitis, but other less common etiologies, especially autoimmune hepatitis, Budd-Chiari syndrome, and Wilson disease, need to be considered. Because diagnosis is frequently tied to potential for reversibility of ALF and prognosis, early identification in a timely manner is crucial. Other causes of ALF are more easily recognizable based on specific circumstances, such as ALF in pregnancy or ischemic hepatitis...
May 2018: Clinics in Liver Disease
Wei Zhang, Qiao-Zheng Wang, Xiao-Wei Chen, Hong-Shan Zhong, Xi-Tong Zhang, Xu-Dong Chen, Ke Xu
AIM: To investigate 30-year treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary hospital in China. METHODS: A total of 256 patients diagnosed with primary BCS at our tertiary hospital between November 1983 and September 2013 were followed and retrospectively studied. Cumulative survival rates and cumulative mortality rates of major causes were calculated by Kaplan-Meier analysis, and the independent predictors of survival were identified using a Cox regression model...
March 14, 2018: World Journal of Gastroenterology: WJG
C J Das, M Soneja, S Tayal, A Chahal, S Srivastava, A Kumar, U Baruah
Budd-Chiari syndrome (BCS) is a clinical condition resulting from impaired hepatic venous drainage, in which there is obstruction to the hepatic venous outflow at any level from the small hepatic veins to the junction of the inferior vena cava and the right atrium leading to hepatic congestion. The diagnosis of BCS is based on imaging, which can be gathered from non-invasive investigations such as ultrasonography coupled with venous Doppler, triphasic computed tomography (CT) and magnetic resonance imaging (MRI)...
March 14, 2018: Clinical Radiology
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