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Budd-Chiari Syndrome (budd-chiari + syndrome)
Selected AbstractsAiming at minimal invasiveness as a therapeutic strategy for Budd-Chiari syndrome,HEPATOLOGY, Issue 5 2006Aurélie Plessier The 1-year spontaneous mortality rate in patients with Budd-Chiari syndrome (BCS) approaches 70%. No prospective assessment of indications and impact on survival of current therapeutic procedures has been performed. We evaluated a therapeutic strategy uniformly applied during the last 8 years in a single referral center. Fifty-one consecutive patients first received anticoagulation and were treated for associated diseases. Symptomatic patients were considered for hepatic vein recanalization; then for transjugular intrahepatic portosystemic shunt (TIPS), and finally for liver transplantation. The absence of a complete response led to the next procedure. Assessment was according to the strategy, whether procedures were technically applicable and successful. At entry, median (range) Child-Pugh score and Clichy prognostic index were 8 (5,12), and 5.4 (3.1,7.7), respectively. A complete response was achieved on medical therapy alone in 9 patients; after recanalization in 6, TIPS in 20, liver transplantation in 9, and retransplantation in 1. Of the 41 patients considered for recanalization, the procedure was not feasible in 27 and technically unsuccessful in 3. Of the 34 patients considered for TIPS, the procedure was considered not feasible in 9 and technically unsuccessful in 4. At 1 year of follow-up, a complete response to TIPS was achieved in 84%. One- and 5-year survival from starting anticoagulation were 96% (95% CI, 90,100) and 89% (95% CI, 79,100), respectively. In conclusion, excellent survival can be achieved in BCS patients when therapeutic procedures are introduced by order of increasing invasiveness, based on the response to previous therapy rather than on the severity of the patient's condition. (HEPATOLOGY 2006;44:1308,1316.) [source] TIPS is a useful long-term derivative therapy for patients with Budd-Chiari syndrome uncontrolled by medical therapyHEPATOLOGY, Issue 1 2002Antonia Perelló Patients with Budd-Chiari syndrome (BCS) may require treatment with portal decompressive surgery or liver transplantation. Transjugular intrahepatic portosystemic shunt (TIPS) represents a new treatment alternative, but its long-term effect on BCS outcome has not been evaluated. Twenty-one patients with BCS consecutively admitted to our unit were evaluated. The mean follow-up was 4 ± 3 years. Seven patients had nonprogressive forms and were successfully controlled with medical therapy; 1 case, with a short-length hepatic vein stenosis was successfully treated by angioplasty. All 8 patients are alive and asymptomatic. The remaining 13 patients, had a TIPS because of clinical deterioration (in one of them, because early TIPS thrombosis a successful side-to-side portacaval shunt [SSPCS] was performed) followed by an improvement in clinical condition. However, a patient with fulminant liver failure before TIPS insertion, died 4 months later and another patient with cirrhosis at diagnosis had liver transplantation 2 years later. The remaining 11 patients are alive and free of ascites. In 3 of these patients TIPS is patent after 3, 6, and 12 months. The remaining 8 patients developed late TIPS dysfunction. In two of these cases, after angioplasty and restenting, TIPS is patent after a follow-up of 9 and 80 months. In 5 other patients, recurring TIPS occlusion was not further corrected because no signs of portal hypertension were present. In conclusion, in patients with BCS uncontrolled with medical therapy, TIPS is a highly effective technique that is associated with long-term survival. [source] Severe aphthous stomatitis associated with oral calcineurin and mTOR inhibitorsINTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 1 2010Nancy Habib BS Background, Aphthous stomatitis, a common mucocutaneous disorder, is a well accepted complication of sirolimus therapy. This association has been reported less frequently with tacrolimus. Case, We present an 11-year old male with Budd-Chiari syndrome who experienced profound worsening of chronic aphthous ulcers after immunosuppressive therapy was changed from tacrolimus to sirolimus. Conclusion, Since these drugs are used widely in the pediatric transplantation population, this report serves to heighten awareness of this debilitating phenomenon, and to stress the importance of exercising caution when sirolimus and tacrolimus are administered in combination to pediatric patients. [source] Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survivalALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 10 2009A. J. MONTANO-LOZA Summary Background, Budd,Chiari syndrome carries significant mortality, but factors predicting this outcome are uncertain. Aim, To determine factors associated with 3-month mortality and compare outcomes after surgical shunting or liver transplantation. Methods, From 1985 to 2008, 51 patients with Budd,Chiari syndrome were identified. Results, By logistic regression analysis, features associated with higher risk of 3-month mortality were Rotterdam class III, Clichy >6.6, model for end-stage liver disease (MELD) >20 and Child,Pugh C. Rotterdam class III had the best performance to discriminate 3-month mortality with sensitivity of 0.89 and specificity of 0.63, whereas Clichy >6.60 had sensitivity of 0.78 and specificity of 0.69; MELD >20 had sensitivity of 0.78 and specificity of 0.75 and Child,Pugh C had sensitivity of 0.67 and specificity of 0.72. Eighteen patients underwent surgical shunts and 14 received liver transplantation with no significant differences in survival (median survival 10 ± 3 vs. 8 ± 2 years; log-rank, P = 0.9). Conclusions, Rotterdam score is the best discrimination index for 3-month mortality in Budd,Chiari syndrome and should be used preferentially to determine treatment urgency. Surgical shunts constitute an important therapeutic modality that may help save liver grafts and prolong transplantation-free survival in a selected group of patients with Budd,Chiari syndrome. [source] Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival , an 18-year experienceLIVER INTERNATIONAL, Issue 2 2009Rupesh Rajani Abstract Background: The exact incidence and prevalence of Budd-Chiari syndrome (BCS) is unknown in the general population. Published reports differ in terms of the clinical characteristics, effects of therapy and survival. Aims: To investigate the epidemiology, clinical presentation and survival in patients with BCS. Methods: Retrospective multicentre study in Sweden reviewing the medical records of all patients with BCS 1986,2003, identified from the computerised diagnosis database of 11 hospitals, including all university hospitals and liver transplantation centres. Results: Forty-three patients with BCS were identified, of whom nine (21%) had concomitant portal vein thrombosis. The mean age-standardised incidence and prevalence rates in 1990,2001 were calculated to be 0.8 per million per year and 1.4 per million inhabitants respectively. Myeloproliferative disorders (38%), thrombophilic factors (31%) and oral contraceptives (30%) were common aetiological factors. Two or more risk factors were present in 44%. In 23%, no risk factor was evident. The median follow-up time was 2.7 years. Seventy-two percent were on anticoagulant therapy during follow-up. Transjugular intrahepatic portosystemic shunting, surgical shunting procedures and liver transplantation were performed in 4, 6 and 18 patients respectively. Nineteen patients died. The overall transplantation-free survival at 1, 5 and 10 years was 47, 28 and 17% respectively. Conclusions: Budd-Chiari syndrome is a rare disorder; the mean age-standardised incidence and prevalence rates in Sweden in 1990,2001 were calculated to be 0.8 per million per year and 1.4 per million inhabitants respectively. The presence of a myeloproliferative disorder was a common aetiological factor in our cohort and about half of the patients had a multifactorial aetiology. The transplantation-free survival was poor. [source] Low recurrence of preexisting extrahepatic malignancies after liver transplantationLIVER TRANSPLANTATION, Issue 6 2008Daniel Benten The incidence of de novo malignancies is increased in organ transplant recipients, and patients with hepatic carcinomas are at high risk for tumor recurrence after liver transplantation. Data about recurrent cancer after orthotopic liver transplantation (OLT) in patients with a history of nonhepatic malignancy are very limited. We retrospectively analyzed data from 606 adult OLT recipients and identified 37 patients (6.1%) with a preexisting extrahepatic malignancy. In the same group, 43 patients (7.0%) developed de novo cancer. Preexisting malignancies included 26 solid tumors and 11 hematological malignancies, including 7 patients with Budd-Chiari syndrome due to myeloproliferative disorders (MPDs). Patients had been selected for OLT because of the expected good prognosis of their preexisting malignancy. Except for 3 patients, recipients were tumor-free at OLT. The median interval from tumor diagnosis to OLT was 44 months (range, <1-321). After a median follow-up of 66 months post transplantation (range, 4-131), all but 1 recipient with incidental colon carcinoma were free of recurrence. No patient with MPD showed leukemic transformation, whereas a patient with neurofibromatosis experienced growth of skin fibromas. Our data and an included review of published OLT recipients with preexisting malignancies have enabled us to show that recurrence rates are comparable for nontransplanted patients and renal-transplant recipients. In conclusion, cancer recurrence is low if OLT recipients are carefully selected. Therefore, previous extrahepatic malignancy should not be considered a contraindication for OLT per se, but the oncologic/hematologic prognosis should be considered, particularly with respect to the current 5-year survival rate of OLT. Liver Transpl, 2008. © 2008 AASLD [source] Use of splenic artery embolization to relieve tense ascites following liver transplantation in a patient with paroxysmal nocturnal hemoglobinuriaLIVER TRANSPLANTATION, Issue 11 2007Charissa Y. Chang Recurrent venous thrombosis following liver transplantation for Budd-Chiari syndrome is common, particularly in the setting of an underlying myeloproliferative disorder. We describe a patient who developed refractory ascites due to portal vein thrombosis following liver transplantation for Budd-Chiari syndrome in the setting of paroxysmal nocturnal hemoglobinuria. Extensive portal vein thrombosis, dense abdominal adhesions, and portosystemic collaterals precluded the use of a transjugular intrahepatic portosystemic shunt or surgical portosystemic shunt to manage the patient's ascites. Splenic artery embolization to decrease portal hypertension was performed, and this resulted in complete resolution of ascites. This case demonstrates the successful use of splenic artery embolization to manage ascites due to portal vein thrombosis following liver transplantation. Splenic artery embolization may be considered as an alternative option for the management of refractory ascites due to portal hypertension in patients who are unable to undergo safe transjugular intrahepatic portosystemic shunt or surgical shunt placement. Liver Transpl 13:1532,1537, 2007. © 2007 AASLD. [source] Isolated right hepatic vein obstruction after piggyback liver transplantationLIVER TRANSPLANTATION, Issue 5 2006Federico Aucejo The "piggyback" technique for liver transplantation has gained worldwide acceptance. Still, complications such as outflow obstruction have been observed, usually attributable to technical errors such as small-caliber anastomosis of the suprahepatic vena cava, twisting, or kinking. Iatrogenic Budd-Chiari syndrome after piggyback liver transplantation has been reported as a consequence of obstruction involving the entire anastomosis (usually the 3 hepatic veins). Here we describe technical issues, clinical presentation, diagnosis, and treatment of 3 cases in which outflow obstruction affected only the right hepatic vein. In conclusion, all 3 patients developed recurrent ascites requiring angioplasty and/or stent placement across the right hepatic vein to alleviate the symptoms. Liver Transpl 12:808,812, 2006. © 2006 AASLD. [source] Surgical treatment of Budd-Chiari syndromeLIVER TRANSPLANTATION, Issue 9 2003Andrew S. Klein Shunting and transplantation are satisfactory methods of treating Budd-Chiari syndrome (BCS). Selection of treatment is based on the degree of hepatic injury (clinical settings), liver biopsy results, potential for parenchymal recovery, and pressure measurements. Shunting is recommended in cases of preserved hepatic function and architecture. In the presence of fulminant forms of BCS, in cases of established cirrhosis or frank fibrosis, or for patients with defined hepatic metabolic defects (e.g., protein C or protein S deficiency), liver transplantation is the treatment of choice. Nonsurgical alternatives, although encouraging, have limited long-term outcome results at the present time. In most cases of BCS, a thrombophilic disorder can be identified. However, it is important to note that postoperative vascular thrombosis has been identified in patients with BCS who do not have a definable hypercoagulable predisposition. It therefore is our practice to recommend early (<24 hours postoperatively) initiation of intravenous heparin therapy in all patients with BCS, who then undergo life-long anticoagulation with coumadin. (Liver Transpl 2003;9:891-896.) [source] Mortality in Behçet's diseaseARTHRITIS & RHEUMATISM, Issue 9 2010D. Saadoun Objective To report the long-term mortality in patients with Behçet's disease (BD). Methods A cohort of 817 patients fulfilling the international criteria for BD from a single center in France were analyzed for causes of death, the standardized mortality ratio (SMR), and the factors associated with mortality. Results Among the 817 patients with BD, 41 (5%) died after a median followup of 7.7 years, of whom 95.1% were male. The mean ± SD age at death was 34.8 ± 11.9 years. Main causes of death included major vessel disease (mainly, arterial aneurysm and Budd-Chiari syndrome) (43.9%), cancer and malignant hemopathy (14.6%), central nervous system involvement (12.2%), and sepsis (12.2%). The mortality rate at 1 year and 5 years was 1.2% and 3.3%, respectively. There was an increased mortality among patients ages 15,24 years (SMR 2.99, 95% confidence interval [95% CI] 1.54,5.39) and those ages 25,34 years (SMR 2.90, 95% CI 1.80,4.49) as compared with age-and sex-matched healthy controls. The mortality decreased in patients older than age 35 years (SMR 1.23, 95% CI 0.75,1.92). In multivariate analyses, male sex (hazard ratio [HR] 4.94, 95% CI 1.53,16.43), arterial involvement (HR 2.51, 95% CI 1.07,5.90), and a high number of BD flares (HR 2.37, 95% CI 1.09,5.14) were independently associated with the risk of mortality. Conclusion The overall mortality in our BD cohort was 5% after a median followup of 7.7 years. Male sex, arterial involvement, and the number of flares were associated with mortality in BD. [source] | ||||||||||