Myeloproliferative Disease in the Pathogenesis and Survival of Budd-Chiari Syndrome.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 1480-1480
Author(s):  
Jasper H. Smalberg ◽  
Sarwa D. Murad ◽  
Eric Braakman ◽  
Peter Valk ◽  
Mies C. Kappers-Klunne ◽  
...  
Keyword(s):  
Mutation Analysis ◽  
Survival Rates ◽  
Vena Cava ◽  
Aetiological Factor ◽  
Budd Chiari Syndrome ◽  
Jak2 Mutation ◽  
Who Criteria ◽  
Significant Difference ◽  
Chiari Syndrome

Abstract Budd-Chiari syndrome (BCS) is a rare disorder caused by obstruction of the hepatic veins or the suprahepatic inferior vena cava. Myeloproliferative diseases (MPD) are the most important aetiological factor in BCS. The aim of this study was to evaluate multifactorial aetiology in BCS patients with MPD, to assess potential added value of the recently discovered JAK2 mutation in the diagnostics of MPD in BCS, and to determine the survival of MPD patients in BCS. All patients referred to our university hospital with primary, non-malignant BCS between January 1980 and January 2006 were included in this study (n=40). Median age was 28.4 years (18.4–53.3), 26 patients (65%) were female and in nine patients (23%) additional portal vein thrombosis (PVT) was present. Overall mean follow-up was 7.1 ± 6.9 years. Only two patients were lost to follow-up, of which one MPD patient. In addition to standard MPD work-up according to WHO criteria, JAK2 mutation analysis was performed in 17 patients. MPD was present in 33% of the patients: Polycythemia Vera (n=6), Essential Thrombocythemia (ET) (n=6) and unclassifiable MPD (n=1). JAK2 mutation was identified in seven out of the 17 tested BCS patients (41%). In two patients suspect for ET, but who failed to meet WHO criteria, JAK2 mutation analysis led to the diagnosis of MPD. In 38% of patients with MPD additional pro-thrombotic factors were present. Survival rates in patients with MPD at 1, 5, and 10 years remained constant at 92% (95% CI, 78%–100%). Survival rates in patients without MPD at 1, 5, and 10 years were 89% (95% CI, 77%–100%), 64% (95% CI, 44%–85%) and 53% (95% CI, 28%–79%), respectively. There was no significant difference in survival between the two (P=0.18). Additional PVT was only present in patients without MPD in our population, which may account for the slightly lowered survival of these patients, since more extensive thrombosis in the splanchnic area is associated with poorer survival. In addition, three patients with and one patients without MPD underwent liver transplantation, which may have affected survival rate in favour of MPD patients. In conclusion, we report MPD in 33% of our BCS patients. In 38% of the patients with MPD, additional pro-thrombotic factors were present. These results enforce the necessity of extensive screening for additional pro-thrombotic conditions. Our study clearly confirms the importance of JAK2 mutation analysis in patients with BCS. Long term follow-up did not show a significant difference in survival between patients with and without MPD.

scholarly journals Budd-Chiari Syndrome

2017 ◽  
Vol 118 (2-3) ◽  
pp. 69-80 ◽  
Author(s):  
Tomáš Grus ◽  
Lukáš Lambert ◽  
Gabriela Grusová ◽  
Rohan Banerjee ◽  
Andrea Burgetová
Keyword(s):  
Inferior Vena Cava ◽  
Structural Changes ◽  
Inferior Vena ◽  
Survival Rates ◽  
Vena Cava ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Venous Outflow ◽  
Secondary Pathology ◽  
Chiari Syndrome

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. Without treatment, 90% of patients die within 3 years, mostly due to complications of liver cirrhosis. BCS can be classified according to etiology (primary, secondary), clinical course (acute, chronic, acute or chronic lesion), and morphology (truncal, radicular, and venooclusive type). The diagnosis is established by demonstrating obstruction of the venous outflow and structural changes of the liver, portal venous system, or a secondary pathology by ultrasound, computed tomography, or magnetic resonance. Laboratory and hematological tests are an integral part of the comprehensive workup and are invaluable in recognizing hematological and coagulation disorders that may be identified in up to 75% of patients with BCS. The recommended therapeutic approach to BCS is based on a stepwise algorithm beginning with medical treatment (a consensus of expert opinion recommends anticoagulation in all patients), endovascular treatment to restore vessel patency (angioplasty, stenting, and local thrombolysis), placement of transjugular portosystemic shunt (TIPS), and orthotopic liver transplantation as a last resort rescue treatment.

Budd-Chiari syndrome due to segmental obstruction of the inferior vena cava successfully managed with endovascular stenting

MedPharmRes ◽  
2018 ◽  
Vol 2 (3) ◽  
pp. 22-26
Author(s):  
Uyen Vo ◽  
Duc Quach ◽  
Luan Dang ◽  
Thao Luu ◽  
Luan Nguyen
Keyword(s):  
Inferior Vena Cava ◽  
Inferior Vena ◽  
Laboratory Data ◽  
Transaminase Level ◽  
Vena Cava ◽  
Budd Chiari Syndrome ◽  
Endovascular Stenting ◽  
Massive Ascites ◽  
Chiari Syndrome ◽  
History Of

Budd–Chiari syndrome (BCS), a rare and life-threatening disorder due to hepatic venous outflow obstruction, is occasionally associated with hypoproteinemia. We herein report the first case of BCS with segmental obstruction of the intrahepatic portion of inferior vena cava (IVC) and hepatic veins (HVs) successfully treated by endovascular stenting in Vietnam. A 32-year-old female patient presented with a 2-month history of massive ascites and leg swelling. She refused history of oral contraceptives use. Hepatosplenomegaly without tenderness was noted. Laboratory data showed polycythemia, mild hypoalbuminemia and hypoproteinemia, slightly high total bilirubin and normal transaminase level. The serum ascites albumin gradient was 1.9 g/dL and ascitic protein level was 1.1 g/dL. The other data were normal. BCS was suspected because of the discrepancy between mild liver failure and massive ascites; and the presence of hepatosplenomegaly and polycythemia. On abdominal magnetic resonance imaging, the segmental obstruction of three HVs and IVC was 2-3 cm long without thrombus. Cavogram revealed the severe segmental stenosis of intrahepatic portion of IVC with no visualized HV and extensive collateral veins. A Protégé stent was deployed to IVC. Leg swelling and ascites were completely resolved within 3 days after stenting. During 1-year follow-up, edema was not recurred and repeated laboratory results were all normal.

scholarly journals Simultaneous implant placement with autogenous onlay bone grafts: a systematic review and meta-analysis

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Guoqiang Ma ◽  
Chaoan Wu ◽  
Miaoting Shao
Keyword(s):  
Meta Analysis ◽  
Survival Rates ◽  
Bone Grafts ◽  
Implant Survival ◽  
Analysis Model ◽  
Implant Placement ◽  
Significant Difference ◽  
Delayed Implant Placement ◽  
Onlay Grafts

AbstractSeveral authors have suggested that implants can be placed simultaneously with onlay bone grafts without affecting outcomes. Therefore, the purpose of this study was to answer the following clinical questions: (1) What are the outcomes of implants placed simultaneously with autogenous onlay bone grafts? And (2) is there a difference in outcomes between simultaneous vs delayed placement of implants with autogenous onlay bone grafts? Databases of PubMed, Embase, and Google Scholar were searched up to 15 November 2020. Data on implant survival was extracted from all the included studies (single arm and comparative) to calculate point estimates with 95% confidence intervals (CI) and pooled using the DerSimonian–Laird meta-analysis model. We also compared implant survival rates between the simultaneous and delayed placement of implants with data from comparative studies. Nineteen studies were included. Five of them compared simultaneous and delayed placement of implants. Dividing the studies based on follow-up duration, the pooled survival of implant placed simultaneously with onlay grafts after <2.5 years of follow-up was 93.1% (95% CI 82.6 to 97.4%) and after 2.5–5 years was 86% (95% CI 78.6 to 91.1%). Implant survival was found to be 85.8% (95% CI 79.6 to 90.3%) with iliac crest grafts and 95.7% (95% CI 83.9 to 93.0%) with intra-oral grafts. Our results indicated no statistically significant difference in implant survival between simultaneous and delayed placement (OR 0.43, 95% 0.07, 2.49, I2=59.04%). Data on implant success and bone loss were limited. Data indicates that implants placed simultaneously with autogenous onlay grafts have a survival rate of 93.1% and 86% after a follow-up of <2.5 years and 2.5–5years respectively. A limited number of studies indicate no significant difference in implant survival between the simultaneous and delayed placement of implants with onlay bone grafts. There is a need for randomized controlled trials comparing simultaneous and delayed implant placement to provide robust evidence.

Recanalization and Reconstruction of a Chronically Occluded Inferior Vena Cava Through an Existing Transjugular Intrahepatic Portosystemic Shunt in the Setting of Budd-Chiari Syndrome

2021 ◽  
pp. 153857442110020
Author(s):  
Reza Talaie ◽  
Hamed Jalaeian ◽  
Nassir Rostambeigi ◽  
Anthony Spano ◽  
Jafar Golzarian
Keyword(s):  
Inferior Vena Cava ◽  
Transjugular Intrahepatic Portosystemic Shunt ◽  
Inferior Vena ◽  
Vena Cava ◽  
Portosystemic Shunt ◽  
Hepatic Veins ◽  
Budd Chiari Syndrome ◽  
Chiari Syndrome ◽  
Intrahepatic Portosystemic Shunt ◽  
The Right

Budd-Chiari syndrome (BCS) results from the occlusion or flow reduction in the hepatic veins or inferior vena cava and can be treated with transjugular intrahepatic portosystemic shunt when hepatic vein recanalization fails.1-3 Hypercoagulable patients with primary BCS are predisposed to development of new areas of thrombosis within the TIPS shunt or IVC. This case details a patient with BCS, pre-existing TIPS extending to the right atrium, and chronic retrohepatic IVC thrombosis who underwent sharp recanalization of the IVC with stenting into the TIPS stent bridging the patient until his subsequent hepatic transplantation.

scholarly journals Partial pulpotomy without age restriction: a retrospective assessment of permanent teeth with carious pulp exposure

2021 ◽  
Author(s):  
Florin Eggmann ◽  
Thomas J. W. Gasser ◽  
Hanjo Hecker ◽  
Mauro Amato ◽  
Roland Weiger ◽  
...  
Keyword(s):  
Survival Rates ◽  
Age Groups ◽  
Permanent Teeth ◽  
Eligibility Criteria ◽  
Presumptive Diagnosis ◽  
Tooth Discoloration ◽  
Significant Difference ◽  
Pulp Exposure ◽  
The Mean

Abstract Objectives This study aimed to retrospectively evaluate clinical and radiographic outcomes of partial pulpotomy performed in permanent teeth with carious pulp exposure. Materials and methods Records of patients undergoing treatment at an undergraduate dental clinic between 2010 and 2019 were screened for partial pulpotomies in teeth with a presumptive diagnosis of normal pulp or reversible pulpitis. The follow-up had to be ≥ 1 year. Patient data were retrieved and analyzed using Mantel-Cox chi square tests and Kaplan–Meier statistics. The level of significance was set at α = 0.05. Results Partial pulpotomy was performed in 111 cases, of which 64 (58%) fulfilled the eligibility criteria. At the time of partial pulpotomy, the mean age was 37.3 (± 13.5) years (age range 18–85). The mean observation period was 3.1 (± 2.0) years. Two early failures (3.1%) and five late failures (7.7%) were recorded. The overall success rate of maintaining pulp vitality was 89.1%, with 98.4% tooth survival. The cumulative pulp survival rates of partial pulpotomy in patients aged < 30 years, between 30 and 40 years, and > 40 years were 100%, 75.5%, and 90.5%, respectively, with no significant difference between the age groups (p = 0.225). At follow-up, narrowing of the pulp canal space and tooth discoloration were observed in 10.9% and 3.1% of cases, respectively. Conclusions Across age groups, partial pulpotomy achieved favorable short and medium-term outcomes in teeth with carious pulp exposure. Clinical relevance Adequate case selection provided, partial pulpotomy is a viable operative approach to treat permanent teeth with deep carious lesions irrespective of patients’ age.

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