AIM To investigate whether the preoperative neutrophil-tolymphocyte ratio(NLR) could predict the prognosis of hepatocellular carcinoma(HCC) patients with portal/hepatic vein tumor thrombosis(PVTT/HVTT) after hepatecto...AIM To investigate whether the preoperative neutrophil-tolymphocyte ratio(NLR) could predict the prognosis of hepatocellular carcinoma(HCC) patients with portal/hepatic vein tumor thrombosis(PVTT/HVTT) after hepatectomy.METHODS The study population included 81 HCC patients who underwent hepatectomy and were diagnosed with PVTT/HVTT based on pathological examination. The demographics, laboratory analyses, and histopathology data were analyzed.RESULTS Overall survival(OS) and disease-free survival(DFS) were determined in the patients with a high(> 2.9) and low(≤ 2.9) NLR. The median OS and DFS duration in the high NLR group were significantly shorter than those in the low NLR group(OS: 6.2 mo vs 15.7 mo, respectively, P = 0.007; DFS: 2.2 mo vs 3.7 mo, respectively, P = 0.039). An NLR > 2.9 was identified as an independent predictor of a poor prognosis of OS(P = 0.034, HR = 1.866; 95%CI: 1.048-3.322) in uni-and multivariate analyses. Moreover, there was a significantly positive correlation between the NLR and the Child-Pugh score(r = 0.276, P = 0.015) and the maximum diameter of the tumor(r = 0.435, P < 0.001). Additionally, the NLR could enhance the prognostic predictive power of the CLIP score for DFS in these patients. CONCLUSION The preoperative NLR is a prognostic predictor after hepatectomy for HCC patients with PVTT/HVTT. NLR > 2.9 indicates poorer OS and DFS.展开更多
AIM: To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India. METHODS: Forty-nine consecutive cases of Budd- Chiari syndrome (BCS) were p...AIM: To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India. METHODS: Forty-nine consecutive cases of Budd- Chiari syndrome (BCS) were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava (IVC) and/or hepatic vein (HV) obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation. RESULTS: Mean duration of symptoms was 41.5 ± 11.2 (range = 1-240) too. HV thrombosis (HVT) was present in 29 (59.1%), IVC thrombosis in eight (16.3%), membranous obstruction of IVC in two (4%) and both IVC-HV thrombosis in 10 (20.4%) cases. Of 35 cases tested for hypercoagulability, 27 (77.1%) were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 (97.3%): IVC stenting in seven (18.9%), IVC balloon angioplasty in two (5.4%), combined IVC-HV stenting in two (5.4%), HV stenting in 11 (29.7%), transjugular intrahepatic portosystemic shunt (TIPS) in 13 (35.1%) and combined TIPS-IVC stenting in two (5.4%). Complications encountered in follow-up: death in five, re-stenosis of the stent in five (17.1%), hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT. CONCLUSION: IN our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability, etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases.展开更多
BACKGROUND: In order to preserve functional liver parenchyma, extended central hepatectomy (segments 4, 5, 7 and 8 resection) was proposed for the management of centrally located hepatocellular carcinoma invading t...BACKGROUND: In order to preserve functional liver parenchyma, extended central hepatectomy (segments 4, 5, 7 and 8 resection) was proposed for the management of centrally located hepatocellular carcinoma invading the right and middle hepatic veins, reconstructing segment 6 outflow in the absence of the thick inferior right hepatic vein. The present study was to describe our surgical techniques of extended central hepatectomy.METHODS: Between 2008 and 2012, 5 patients with centrally located hepatocellular carcinoma invading or in the vicinity of the right and middle hepatic veins underwent extended central hepatectomy. The thick inferior right hepatic vein was preserved during dissection. Gore-Tex graft was used for segment 6 outflow reconstruction in the absence of the thick inferior right hepatic vein.RESULTS: The mean future remnant liver volume for segments 2 and 3 was 28% versus 45% on segment 6 preservation. The mean tumor diameter was 7.4 cm. The thick inferior right hepatic vein was found in 1 patient. Outflow reconstruction from segment 6 was performed in 4 patients. Postoperative complications included bile leakage (1 patient), pleural effusion (2) and liver failure (1). The rate of graft patency was 75%. There was no perioperative mortality.CONCLUSION: Extended central hepatectomy is a safe alternative for extended hepatic resection in selected patients attempting to preserve the functional liver parenchyma.展开更多
BACKGROUND: Budd-Chiari syndrome (BCS) is a type of disease characterized by portal hypertension and/or hy- pertension of the inferior vena cava (IVC) due to the ob- struction of the hepatic veins (HV) and/or intrahep...BACKGROUND: Budd-Chiari syndrome (BCS) is a type of disease characterized by portal hypertension and/or hy- pertension of the inferior vena cava (IVC) due to the ob- struction of the hepatic veins (HV) and/or intrahepatic IVC outlet. Being etiologically complicated and obscure, BCS can be acquired or idiopathic and several gene muta- tions may be contributable. This study was to explore whether prothrombin gene mutation (F G20210A) takes part in the pathogenesis of BCS and to investigate their cor- relativity. METHODS: In 38 proven BCS patients and 70 controls, polymerase chain reaction-restriction fragment length poly- morphism (PCR-RFLP) was used to find F G20210A mutation. To detect whether there are any mutations, four steps were taken: purification of genome DNA from whole blood, amplification of special fragment by polymerase chain reaction, digestion of the fragment via restriction en- donuclease, and analysis of results by polyacrylamide gel electrophoresis. RESULTS: F G20210A mutation was not detected in all patients and controls. CONCLUSIONS: No F G20210A mutation exists in Chi- nese patients with BCS, nor correlativity between the oc- currence of BCS and F G20210A mutation. The etiology of BCS in the Chinese needs further investigation.展开更多
A 31-year-old female who had well-established polycythemia vera one year before, presented with the sudden onset. She had severe ascites and hepatic encephalopathy 12 d prior to admission. Real-time ultrasonography re...A 31-year-old female who had well-established polycythemia vera one year before, presented with the sudden onset. She had severe ascites and hepatic encephalopathy 12 d prior to admission. Real-time ultrasonography revealed a supra hepatic thrombosis extending toward the inferior vena cava (IVC). Thrombolytic therapy with systemic streptokinase (250000 IU loading + 100000 IU/h infusion) was started. At the end of 72 h infusion, the patient's general condition improved. A color Doppler ultrasonography then showed complete and partial resolution of the thrombosis in the supra hepatic vein and IVC, respectively. Despite this good response, 12 d later, the symptoms recurred. Venography detected complete obstruction of the IVC. Percutanous balloon angioplasty with stent insertion was performed successfully and the patient was discharged without any evidence of liver disease. A combination of systemic streptokinase and radiological intervention was effective in our patient.展开更多
AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies condu...AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.展开更多
AIM: To investigate challenges, risk factors, prognostic indicators, and treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary care center.
BACKGROUND: Budd-Chiari syndrome (BCS) presents a kind of disease resulted from the occlusion of hepatic vein and/or the intrahepatic inferior vena cava. Its different pathological types were proposed. According to ou...BACKGROUND: Budd-Chiari syndrome (BCS) presents a kind of disease resulted from the occlusion of hepatic vein and/or the intrahepatic inferior vena cava. Its different pathological types were proposed. According to our expe- rience , the membranous type takes a large part of it, and we tried to explore the best treatment of membranous BCS through the analysis of 480 cases retrospectively. METHOD: The operative results of 480 patients with mem- branous BCS were analysed retrospectively. RESULTS: Patients after Kimura's finger rupture, inter- ventional treatment and membrane resection were followed up with rates of 84.62%, 86.55%, and 87.37%, respective- ly. The effective rates of the three methods were 61.4%, 91.7%, and 90.4%, respectively, and the recurrence rates of the disease after the 3 procedures were 38.6%, 8.3% and 9.6%, respectively. The long-term effects of interventional treatment and resection were significantly better than those of Kimura' s finger rupture (P <0.05). CONCLUSION: Balloon dilatation is the choice for mem- branous BCS. Patients with extensive lesion, thick mem- brane or recurrence after percutaneous transhepatic angio- graphy should undergo membrane resection.展开更多
BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or ...BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and I had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.展开更多
Background:Portal-vein stent combined with one iodine-125 (^125I) seed strand has become a new treatment for portal vein tumor thrombosis.However,dosimetric aspects of this irradiation stent have not been reported....Background:Portal-vein stent combined with one iodine-125 (^125I) seed strand has become a new treatment for portal vein tumor thrombosis.However,dosimetric aspects of this irradiation stent have not been reported.Therefore,we aimed to undertake dosimetric analyses comparing portal-vein stents combined with different numbers of ^125I seed strands.Methods:A water cylinder was created by a treatment-planning system to simulate a portal-vein stent.The stent was combined with one,two,or three ^125I seed strands (Groups Ⅰ,Ⅱ,and Ⅲ,respectively).At different prescribed doses (PDs),^125I seeds of identical activities were loaded on Groups Ⅰ-Ⅲ.Conformation number (CN),external volume index,and homogeneity index were calculated.Linear regression analyses were used to evaluate the obtained data.Results:For identical ^125I seed activity,when the 125I seed strand increased from one chain to two,D90 (dose delivered to 90% of the target volume) increased by ≥184%;when it increased from two chains to three,D90 increased by ≥63%.When the PD was 105 Gy and 125I seed strands increased from one chain to two,V100 (percentage of the target volume receiving ≥90% of the PD) increased by 158-249%;when it increased from two chains to three,V100 increased by 7-175%.CN was correlated positively with 125I seed activity (B =0.479,P 〈 0.001) and number of ^125I seed strands (B =0.201,P 〈 0.001) and was independent of PD (B =-0.002,P =0.078).Conclusions:A portal-vein stent combined with a single 125I seed strand could not meet dosimetry requirements.For a stent combined with two 125I seed strands,when the PD was 1 05 Gy and seed activity was 0.7 mCi,the dose distribution could satisfy dosimetry requirements.For a stent combined with three 125I seed strands,if the PD was 105,125,or 145 Gy,the recommended seed activities were 0.5,0.5,and 0.6 mCi,respectively.展开更多
Since Professor WANG Zhong-gao's creative work on thesystemic treatment of the Budd-Chiari syndrome(BCS), this debilitating disease has been more and more widely recognized in China. Several large-scale studies of ...Since Professor WANG Zhong-gao's creative work on thesystemic treatment of the Budd-Chiari syndrome(BCS), this debilitating disease has been more and more widely recognized in China. Several large-scale studies of surgery or intervention strategies for treating BCS have been reported. However, much controversy still remains regarding many aspects of this disease, including its etiology, treatment, and classification. This review explores these controversies with emphasis on areas that merit further study.展开更多
Background Budd-Chiari syndrome (BCS) is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava (IVC). Angiography is the "golden standard" for diagnosis...Background Budd-Chiari syndrome (BCS) is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava (IVC). Angiography is the "golden standard" for diagnosis, but it is an invasive examination. To assess the diagnostic value of a fresh blood imaging (FBI) relative to BCS, we used a magnetic resonance angiography (MRA) with an FBI sequence for a preoperative evaluation of the BCS patients in this study. Methods Fifty patients who were suspected of having BCS after they had been checked by a B-ultrasound were studied. 2D and 3D FBI were performed on a 1.5T superconductive MR scanner. Original images were rebuilt using a maximal intensity projection (MIP) method on the console. Two doctors reviewed all images before they learned of the angiography results. We then compared the diagnoses obtained from the FBI and angiography results to evaluate the diagnostic value of the FBI.Results Forty-one patients were diagnosed as BCS and 9 as non-BCS based on an angiography. The FBI correctly diagnosed 38 patients, incorrectly diagnosed 1 patient, and missed diagnosis in 3 patients. Thus, the diagnostic sensitivity of the FBI is 93% (38/41), the specificity is 89% (8/9) and the accuracy is 92% (46/50). The FBI images of the 13 membranous stenoses of the IVC showed a sudden stenosis of the post-liver segment of the IVC. The Images of the 5 patients with a membranous obstruction of the IVC showed IVC thickening and an absence of blood signals in the post-hepatic segment of the IVC. The images of the 4 patients with the segmental thrombosis of the IVC showed abnormal and intermittent signals in the IVC. The images of the 6 patients with a simple hepatic vein obstruction showed obstructive hepatic veins. The images of the 6 patients with the stenosis of both the IVC and the hepatic veins showed the stenosis of the IVC, the thickening of the hepatic veins and the formation of a compensatory circulation within the liver. Lastly, the images of the 7 patients showed a combination of the IVC thrombosis with stenosis or with the obstruction of one or two hepatic veins. Conclusions An FBI can show a membranous stenosis, and an obstruction and thrombosis of the IVC. In addition, it can also demonstrate the thickening of the flexural hepatic vein and the development of intra-hepatic compensatory branches with slow blood flow. Thus, it can guide the puncturing and opening of the hepatic vein involved in an interventional therapy for BCS patients.展开更多
Background Interventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome but the use of radical correctional therapy should not be discarded. This study describes radical ...Background Interventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome but the use of radical correctional therapy should not be discarded. This study describes radical correction by controlling bleeding from distal end of pathological segment of the inferior vena cava (IVC) and discusses potential surgical errors and postoperative complications. Methods Of the 216 patients in the study, 78 were treated with simple membranectomy, 64 with dissection of the pathological segment of the IVC and vascular prosthesis or pericardial patch plasty, 60 with resection of the pathological segment of the IVC and orthotopic graft transplantation with vascular prosthesis, and 14 with resection of the occlusive main hepatic vein and its upper IVC, hepatic venous outflow plasty and vascular prosthesis orthotopic graft transplantation from the hepatic venous entrance to the IVC of right atrial ostium. Results Except 14 cases who were discharged after hepatic vein outflow plasty, four cases died postoperatively, and 198 patients were discharged without complications. The symptoms of 15 patients were relieved partially and 2 without any change. There were no deaths intraoperatively. Of the 112 cases who were followed up for 72 months, 13 suffered from a relapse. Conclusions Radical correction is a beneficial therapy in the treatment of Budd-Chiari svndrome.展开更多
We report a case of Budd-Chiari syndrome occurring in a patient with coeliac disease,who presented with symptoms of increased abdominal girth,right upper quadrant pain and shortness of breath for three weeks prior to ...We report a case of Budd-Chiari syndrome occurring in a patient with coeliac disease,who presented with symptoms of increased abdominal girth,right upper quadrant pain and shortness of breath for three weeks prior to admission.Initial assessment revealed the presence of moderate ascites,hepatosplenomegaly and right-sided pleural effusion.Further diagnostic work-up established a diagnosis of chronic Budd-Chiari syndrome.Interestingly,complete screening for pro-thrombotic factors was negative.A review of the literature on this association disclosed only 28 similar cases,with the majority of them describing individuals of North African origin.Interestingly,in the majority of cases no specific thrombotic factor could be identified,suggesting that coeliac disease may play a role in this thrombotic disorder.展开更多
文摘AIM To investigate whether the preoperative neutrophil-tolymphocyte ratio(NLR) could predict the prognosis of hepatocellular carcinoma(HCC) patients with portal/hepatic vein tumor thrombosis(PVTT/HVTT) after hepatectomy.METHODS The study population included 81 HCC patients who underwent hepatectomy and were diagnosed with PVTT/HVTT based on pathological examination. The demographics, laboratory analyses, and histopathology data were analyzed.RESULTS Overall survival(OS) and disease-free survival(DFS) were determined in the patients with a high(> 2.9) and low(≤ 2.9) NLR. The median OS and DFS duration in the high NLR group were significantly shorter than those in the low NLR group(OS: 6.2 mo vs 15.7 mo, respectively, P = 0.007; DFS: 2.2 mo vs 3.7 mo, respectively, P = 0.039). An NLR > 2.9 was identified as an independent predictor of a poor prognosis of OS(P = 0.034, HR = 1.866; 95%CI: 1.048-3.322) in uni-and multivariate analyses. Moreover, there was a significantly positive correlation between the NLR and the Child-Pugh score(r = 0.276, P = 0.015) and the maximum diameter of the tumor(r = 0.435, P < 0.001). Additionally, the NLR could enhance the prognostic predictive power of the CLIP score for DFS in these patients. CONCLUSION The preoperative NLR is a prognostic predictor after hepatectomy for HCC patients with PVTT/HVTT. NLR > 2.9 indicates poorer OS and DFS.
文摘AIM: To evaluate patterns of obstruction, etiological spectrum and non-surgical treatment in patients with Budd-Chiari syndrome in India. METHODS: Forty-nine consecutive cases of Budd- Chiari syndrome (BCS) were prospectively evaluated. All patients with refractory ascites or deteriorating liver function were, depending on morphology of inferior vena cava (IVC) and/or hepatic vein (HV) obstruction, triaged for radiological intervention, in addition to anticoagulation therapy. Asymptomatic patients, patients with diuretic-responsive ascites and stable liver function, and patients unwilling for surgical intervention were treated symptomatically with anticoagulation. RESULTS: Mean duration of symptoms was 41.5 ± 11.2 (range = 1-240) too. HV thrombosis (HVT) was present in 29 (59.1%), IVC thrombosis in eight (16.3%), membranous obstruction of IVC in two (4%) and both IVC-HV thrombosis in 10 (20.4%) cases. Of 35 cases tested for hypercoagulability, 27 (77.1%) were positive for one or more hypercoagulable states. Radiological intervention was technically successful in 37/38 (97.3%): IVC stenting in seven (18.9%), IVC balloon angioplasty in two (5.4%), combined IVC-HV stenting in two (5.4%), HV stenting in 11 (29.7%), transjugular intrahepatic portosystemic shunt (TIPS) in 13 (35.1%) and combined TIPS-IVC stenting in two (5.4%). Complications encountered in follow-up: death in five, re-stenosis of the stent in five (17.1%), hepatic encephalopathy in two and hepatocellular carcinoma in one patient. Of nine patients treated medically, two showed complete resolution of HVT. CONCLUSION: IN our series, HVT was the predominant cause of BCS. In the last five years with the availability of sophisticated tests for hypercoagulability, etiologies were defined in 85.7% of cases. Non-surgical management was successful in most cases.
文摘BACKGROUND: In order to preserve functional liver parenchyma, extended central hepatectomy (segments 4, 5, 7 and 8 resection) was proposed for the management of centrally located hepatocellular carcinoma invading the right and middle hepatic veins, reconstructing segment 6 outflow in the absence of the thick inferior right hepatic vein. The present study was to describe our surgical techniques of extended central hepatectomy.METHODS: Between 2008 and 2012, 5 patients with centrally located hepatocellular carcinoma invading or in the vicinity of the right and middle hepatic veins underwent extended central hepatectomy. The thick inferior right hepatic vein was preserved during dissection. Gore-Tex graft was used for segment 6 outflow reconstruction in the absence of the thick inferior right hepatic vein.RESULTS: The mean future remnant liver volume for segments 2 and 3 was 28% versus 45% on segment 6 preservation. The mean tumor diameter was 7.4 cm. The thick inferior right hepatic vein was found in 1 patient. Outflow reconstruction from segment 6 was performed in 4 patients. Postoperative complications included bile leakage (1 patient), pleural effusion (2) and liver failure (1). The rate of graft patency was 75%. There was no perioperative mortality.CONCLUSION: Extended central hepatectomy is a safe alternative for extended hepatic resection in selected patients attempting to preserve the functional liver parenchyma.
基金This study was partly supported by a grant from the Science and TechnologyKey Project of Henan Province, China ( No. 0224630176 ).
文摘BACKGROUND: Budd-Chiari syndrome (BCS) is a type of disease characterized by portal hypertension and/or hy- pertension of the inferior vena cava (IVC) due to the ob- struction of the hepatic veins (HV) and/or intrahepatic IVC outlet. Being etiologically complicated and obscure, BCS can be acquired or idiopathic and several gene muta- tions may be contributable. This study was to explore whether prothrombin gene mutation (F G20210A) takes part in the pathogenesis of BCS and to investigate their cor- relativity. METHODS: In 38 proven BCS patients and 70 controls, polymerase chain reaction-restriction fragment length poly- morphism (PCR-RFLP) was used to find F G20210A mutation. To detect whether there are any mutations, four steps were taken: purification of genome DNA from whole blood, amplification of special fragment by polymerase chain reaction, digestion of the fragment via restriction en- donuclease, and analysis of results by polyacrylamide gel electrophoresis. RESULTS: F G20210A mutation was not detected in all patients and controls. CONCLUSIONS: No F G20210A mutation exists in Chi- nese patients with BCS, nor correlativity between the oc- currence of BCS and F G20210A mutation. The etiology of BCS in the Chinese needs further investigation.
文摘A 31-year-old female who had well-established polycythemia vera one year before, presented with the sudden onset. She had severe ascites and hepatic encephalopathy 12 d prior to admission. Real-time ultrasonography revealed a supra hepatic thrombosis extending toward the inferior vena cava (IVC). Thrombolytic therapy with systemic streptokinase (250000 IU loading + 100000 IU/h infusion) was started. At the end of 72 h infusion, the patient's general condition improved. A color Doppler ultrasonography then showed complete and partial resolution of the thrombosis in the supra hepatic vein and IVC, respectively. Despite this good response, 12 d later, the symptoms recurred. Venography detected complete obstruction of the IVC. Percutanous balloon angioplasty with stent insertion was performed successfully and the patient was discharged without any evidence of liver disease. A combination of systemic streptokinase and radiological intervention was effective in our patient.
文摘AIM:To re-examine whether hepatic vein thrombosis(HVT)(classical Budd-Chiari syndrome)and hepatic vena cava-Budd Chiari syndrome(HVC-BCS)are the same disorder.METHODS:A systematic review of observational studies conducted in adult subjects with primary BCS,hepatic vein outflow tract obstruction,membranous obstruction of the inferior vena cava(IVC),obliterative hepatocavopathy,or HVT during the period of January2000 until February 2015 was conducted using the following databases:Cochrane Library,CINAHL,MEDLINE,Pub Med and Scopus.RESULTS:Of 1299 articles identified,26 were included in this study.Classical BCS is more common in women with a pure hepatic vein obstruction(49%-74%).HVCBCS is more common in men with the obstruction often located in both the inferior vena cava and hepatic veins(14%-84%).Classical BCS presents with acute abdominal pain,ascites,and hepatomegaly.HVC-BCS presents with chronic abdominal pain and abdominalwall varices.Myeloproliferative neoplasms(MPN)are the most common etiology of classical BCS(16%-62%)with the JAK2V617-F mutation found in 26%-52%.In HVCBCS,MPN are found in 4%-5%,and the JAK2V617-F mutation in 2%-5%.Classical BCS responds well to medical management alone and 1st line management of HVC-BCS involves percutaneous recanalization,with few managed with medical management alone.CONCLUSION:Systematic review of recent data suggests that classical BCS and HVC-BCS may be two clinically different disorders that involve the disruption of hepatic venous outflow.
文摘AIM: To investigate challenges, risk factors, prognostic indicators, and treatment outcomes associated with Budd-Chiari syndrome (BCS) at a tertiary care center.
文摘BACKGROUND: Budd-Chiari syndrome (BCS) presents a kind of disease resulted from the occlusion of hepatic vein and/or the intrahepatic inferior vena cava. Its different pathological types were proposed. According to our expe- rience , the membranous type takes a large part of it, and we tried to explore the best treatment of membranous BCS through the analysis of 480 cases retrospectively. METHOD: The operative results of 480 patients with mem- branous BCS were analysed retrospectively. RESULTS: Patients after Kimura's finger rupture, inter- ventional treatment and membrane resection were followed up with rates of 84.62%, 86.55%, and 87.37%, respective- ly. The effective rates of the three methods were 61.4%, 91.7%, and 90.4%, respectively, and the recurrence rates of the disease after the 3 procedures were 38.6%, 8.3% and 9.6%, respectively. The long-term effects of interventional treatment and resection were significantly better than those of Kimura' s finger rupture (P <0.05). CONCLUSION: Balloon dilatation is the choice for mem- branous BCS. Patients with extensive lesion, thick mem- brane or recurrence after percutaneous transhepatic angio- graphy should undergo membrane resection.
文摘BACKGROUND: Budd-Chiari syndrome (BCS) refers to posthepatic portal vein hypertension and/or inferior vena cava hypertension syndrome caused by obstruction of the blood flow at the portal cardinal hepatic vein and/or posterior hepatic inferior vena cava. The main surgical treatments of BCS include operations on pathological lesioned membrane, shunt, and combined operations. There are more than ten treatments available and reports on their therapeutic effects vary. As to operations on lesioned membrane, there are Kimura's finger rupture, balloon dilatation and membrane removal. With reference to our experience, the clinical value of membrane resection at normal temperature and under direct vision is discussed. METHODS: A total of 292 patients with BCS undergoing membrane resection at normal temperature and under direct vision from June 1996 to June 2005 were retrospectively analyzed. RESULTS: The short-term therapeutic effect in 256 patients was satisfactory and the effective rate was 87.7% (256/292). Within a week, ascitic fluid disappeared, the liver shrank and edema of the lower extremities was greatly relieved or even disappeared. Perioperative death occurred in 14 patients (4.8%). Of these, 3 had acute heart failure (one during the operation, one after 6 hours and one 7 days later). Six patients had thoracic cavity bleeding within 12 hours after the operation, 3 had acute respiratory distress syndrome (ARDS), 2 had disseminated intravascular coagulation (DIC), and I had pulmonary embolism. 158 patients were followed up for 6 months to 12 years, and 12 (7.6%) had recurrences. CONCLUSIONS: After membrane resection at normal temperature and under direct vision, hemodynamics was found to be close to normal, damage was slight, effectiveness was evident and the recurrence rate low. So this method is effective in treating BCS.
文摘Background:Portal-vein stent combined with one iodine-125 (^125I) seed strand has become a new treatment for portal vein tumor thrombosis.However,dosimetric aspects of this irradiation stent have not been reported.Therefore,we aimed to undertake dosimetric analyses comparing portal-vein stents combined with different numbers of ^125I seed strands.Methods:A water cylinder was created by a treatment-planning system to simulate a portal-vein stent.The stent was combined with one,two,or three ^125I seed strands (Groups Ⅰ,Ⅱ,and Ⅲ,respectively).At different prescribed doses (PDs),^125I seeds of identical activities were loaded on Groups Ⅰ-Ⅲ.Conformation number (CN),external volume index,and homogeneity index were calculated.Linear regression analyses were used to evaluate the obtained data.Results:For identical ^125I seed activity,when the 125I seed strand increased from one chain to two,D90 (dose delivered to 90% of the target volume) increased by ≥184%;when it increased from two chains to three,D90 increased by ≥63%.When the PD was 105 Gy and 125I seed strands increased from one chain to two,V100 (percentage of the target volume receiving ≥90% of the PD) increased by 158-249%;when it increased from two chains to three,V100 increased by 7-175%.CN was correlated positively with 125I seed activity (B =0.479,P 〈 0.001) and number of ^125I seed strands (B =0.201,P 〈 0.001) and was independent of PD (B =-0.002,P =0.078).Conclusions:A portal-vein stent combined with a single 125I seed strand could not meet dosimetry requirements.For a stent combined with two 125I seed strands,when the PD was 1 05 Gy and seed activity was 0.7 mCi,the dose distribution could satisfy dosimetry requirements.For a stent combined with three 125I seed strands,if the PD was 105,125,or 145 Gy,the recommended seed activities were 0.5,0.5,and 0.6 mCi,respectively.
文摘Since Professor WANG Zhong-gao's creative work on thesystemic treatment of the Budd-Chiari syndrome(BCS), this debilitating disease has been more and more widely recognized in China. Several large-scale studies of surgery or intervention strategies for treating BCS have been reported. However, much controversy still remains regarding many aspects of this disease, including its etiology, treatment, and classification. This review explores these controversies with emphasis on areas that merit further study.
文摘Background Budd-Chiari syndrome (BCS) is a rare disease with portal hypertension caused by the blockage of the hepatic vein and/or the inferior vena cava (IVC). Angiography is the "golden standard" for diagnosis, but it is an invasive examination. To assess the diagnostic value of a fresh blood imaging (FBI) relative to BCS, we used a magnetic resonance angiography (MRA) with an FBI sequence for a preoperative evaluation of the BCS patients in this study. Methods Fifty patients who were suspected of having BCS after they had been checked by a B-ultrasound were studied. 2D and 3D FBI were performed on a 1.5T superconductive MR scanner. Original images were rebuilt using a maximal intensity projection (MIP) method on the console. Two doctors reviewed all images before they learned of the angiography results. We then compared the diagnoses obtained from the FBI and angiography results to evaluate the diagnostic value of the FBI.Results Forty-one patients were diagnosed as BCS and 9 as non-BCS based on an angiography. The FBI correctly diagnosed 38 patients, incorrectly diagnosed 1 patient, and missed diagnosis in 3 patients. Thus, the diagnostic sensitivity of the FBI is 93% (38/41), the specificity is 89% (8/9) and the accuracy is 92% (46/50). The FBI images of the 13 membranous stenoses of the IVC showed a sudden stenosis of the post-liver segment of the IVC. The Images of the 5 patients with a membranous obstruction of the IVC showed IVC thickening and an absence of blood signals in the post-hepatic segment of the IVC. The images of the 4 patients with the segmental thrombosis of the IVC showed abnormal and intermittent signals in the IVC. The images of the 6 patients with a simple hepatic vein obstruction showed obstructive hepatic veins. The images of the 6 patients with the stenosis of both the IVC and the hepatic veins showed the stenosis of the IVC, the thickening of the hepatic veins and the formation of a compensatory circulation within the liver. Lastly, the images of the 7 patients showed a combination of the IVC thrombosis with stenosis or with the obstruction of one or two hepatic veins. Conclusions An FBI can show a membranous stenosis, and an obstruction and thrombosis of the IVC. In addition, it can also demonstrate the thickening of the flexural hepatic vein and the development of intra-hepatic compensatory branches with slow blood flow. Thus, it can guide the puncturing and opening of the hepatic vein involved in an interventional therapy for BCS patients.
文摘Background Interventional therapy is widely accepted as the first choice for the treatment of the Budd-Chiari syndrome but the use of radical correctional therapy should not be discarded. This study describes radical correction by controlling bleeding from distal end of pathological segment of the inferior vena cava (IVC) and discusses potential surgical errors and postoperative complications. Methods Of the 216 patients in the study, 78 were treated with simple membranectomy, 64 with dissection of the pathological segment of the IVC and vascular prosthesis or pericardial patch plasty, 60 with resection of the pathological segment of the IVC and orthotopic graft transplantation with vascular prosthesis, and 14 with resection of the occlusive main hepatic vein and its upper IVC, hepatic venous outflow plasty and vascular prosthesis orthotopic graft transplantation from the hepatic venous entrance to the IVC of right atrial ostium. Results Except 14 cases who were discharged after hepatic vein outflow plasty, four cases died postoperatively, and 198 patients were discharged without complications. The symptoms of 15 patients were relieved partially and 2 without any change. There were no deaths intraoperatively. Of the 112 cases who were followed up for 72 months, 13 suffered from a relapse. Conclusions Radical correction is a beneficial therapy in the treatment of Budd-Chiari svndrome.
文摘We report a case of Budd-Chiari syndrome occurring in a patient with coeliac disease,who presented with symptoms of increased abdominal girth,right upper quadrant pain and shortness of breath for three weeks prior to admission.Initial assessment revealed the presence of moderate ascites,hepatosplenomegaly and right-sided pleural effusion.Further diagnostic work-up established a diagnosis of chronic Budd-Chiari syndrome.Interestingly,complete screening for pro-thrombotic factors was negative.A review of the literature on this association disclosed only 28 similar cases,with the majority of them describing individuals of North African origin.Interestingly,in the majority of cases no specific thrombotic factor could be identified,suggesting that coeliac disease may play a role in this thrombotic disorder.