74 research outputs found

    Etiology, management, and outcome of the Budd-Chiari syndrome

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    Background: The Budd-Chiari syndrome (BCS) is hepatic venous outflow obstruction. What is known about the syndrome is based on small studies of prevalent cases. Objective: To characterize the causes and treatment of incident BCS. Design: Consecutive case series of patients with incident BCS, enrolled from October 2003 to October 2005 and followed until May 2006. Setting: Academic and nonacademic hospitals in France, Spain, Italy, Great Britain, Germany, Belgium, the Netherlands, Portugal, and Switzerland. Patients: Persons older than 16 years with definite hepatic outflow obstruction diagnosed by imaging. Persons with hepatic outflow obstruction due to heart failure, sinusoidal obstruction syndrome, cancer, or liver transplantation were excluded. Measurements: Signs and symptoms; laboratory and imaging findings; diagnosis; treatment; and overall, transplantation-free, and intervention-free survival. Results: 163 incident cases of BCS were identified. Median follow-up was 17 months (range, 0.1 to 31 months). Most patients (84%) had at least 1 thrombotic risk factor, and many (46%) had more than 1; the most common was myeloproliferative disorders (49% of 103 tested patients). Patients were mainly treated with anticoagulation (140 patients [86%]), transjugular intrahepatic portosystemic shunting (56 patients [34%]), or liver transplantation (20 patients [12%]), and 80 patients (49%) were managed noninvasively. Only 3 patients underwent surgical shunting. The survival rate was 87% (95% CI, 82% to 93%) at 1 year and 82% (CI, 75% to 88%) at 2 years. Limitation: Treatment was not standardized across all centers, and data on important clinical variables were missing for some patients. Conclusion: Most patients with BCS have at least 1 thrombotic risk factor, and many have more than 1; myeloproliferative disorders are most common. One- and 2-year survival rates are good with contemporary management, which includes noninvasive therapies (anticoagulation and diuretics) and invasive techniques. Transjugular intrahepatic portosystemic shunting seems to have replaced surgical shunting as the most common invasive therapeutic procedure. Primary Funding Source: Fifth Framework Programme of the European Commission

    Budd-Chiari Syndrome: Long term success via hepatic decompression using transjugular intrahepatic porto-systemic shunt

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    <p>Abstract</p> <p>Background</p> <p>Budd-Chiari syndrome (BCS) generally implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. Treatment depends on the underlying cause, the anatomic location, the extent of the thrombotic process and the functional capacity of the liver. It can be divided into medical treatment including anticoagulation and thrombolysis, radiological procedures such as angioplasty and transjugular intrahepatic porto-systemic shunt (TIPS) and surgical interventions including orthotopic liver transplantation (OLT). Controlled trials or reports on larger cohorts are limited due to rare disease frequency. The aim of this study was to report our single centre long term results of patients with BCS receiving one of three treatment options i.e. medication only, TIPS or OLT on an individually based decision of our local expert group.</p> <p>Methods</p> <p>20 patients with acute, subacute or chronic BCS were treated between 1988 and 2008. Clinical records were analysed with respect to underlying disease, therapeutic interventions, complications and overall outcome.</p> <p>Results</p> <p>16 women and 4 men with a mean age of 34 ± 12 years (range: 14-60 years) at time of diagnosis were included. Myeloproliferative disorders or a plasmatic coagulopathy were identified as underlying disease in 13 patients, in the other patients the cause of BCS remained unclear. 12 patients presented with an acute BCS, 8 with a subacute or chronic disease. 13 patients underwent TIPS, 4 patients OLT as initial therapy, 2 patients required only symptomatic therapy, and one patient died from liver failure before any specific treatment could be initiated. Eleven of 13 TIPS patients required 2.5 ± 2.4 revisions (range: 0-8). One patient died from his underlying hematologic disease. The residual 12 patients still have stable liver function not requiring OLT. All 4 patients who underwent OLT as initial treatment, required re-OLT due to thrombembolic complications of the graft. Survival in the TIPS group was 92.3% and in the OLT group 75% during a median follow-up of 4 and 11.5 years, respectively.</p> <p>Conclusion</p> <p>Our results confirm the role of TIPS in the management of patients with acute, subacute and chronic BCS. The limited number of patients with OLT does not allow to draw a meaningful conclusion. However, the underlying disease may generate major complications, a reason why OLT should be limited to patients who cannot be managed by TIPS.</p

    Combining facial and postural expressions of emotions in a virtual character

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    Percutaneous radiofrequency ablation for hepatocellular carcinoma before liver transplantation: a prospective study with histopathologic comparison.

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    OBJECTIVE. The aims of this study were to determine the feasibility and efficacy of percutaneous radiofrequency ablation in patients with hepatocellular carcinoma waiting for liver transplantation and to compare the radiologic and pathologic findings. SUBJECTS AND METHODS. Twenty-six patients with 35 hepatocellular carcinomas were addressed for transplantation. Complications of the procedures were recorded. Primary and secondary technique effectiveness and causes of exclusion from the waiting list were assessed. After transplantation, tumor recurrence was evaluated for at least 1 year in all patients. Radiologic-pathologic comparison of the explant was performed. RESULTS. Percutaneous radiofrequency ablation was performed in 21 (81%) patients for 28 tumors. Both minor and major complications occurred in three patients (10% each per session). The rates of primary technique effectiveness, secondary technique effectiveness for percutaneous radiofrequency ablation alone (seven tumors), and combined percutaneous radiofrequency ablation and transcatheter arterial chemoembolization (three tumors) were 56%, 76%, and 86%, respectively. After a mean follow-up of 11.9 months, 16 patients (76%) received transplants, whereas five patients were excluded from the waiting list because of distant tumor progression (n = 3, 14%) or other causes (n = 2, 10%). After transplantation, tumor recurred in one (6%) of 16 patients. Histopathologic examinations were performed for 13 (81%) of 16 patients and showed complete necrosis and satellite nodules in, respectively, 12 (75%) and seven (44%) of 16 tumors. CONCLUSION. Percutaneous radiofrequency ablation can be performed on hepatocellular carcinoma patients waiting for transplantation, allows most patients to undergo transplantation, and does not impair posttransplantation outcomes. The procedure produces complete necrosis of the treated tumor in most cases but is associated with a high rate of satellite nodules. © American Roentgen Ray Society
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