Objective To study the clinical features and prognosis of patients with primary BuddChiari syndrome (BCS) caused by hepatic vein thrombosis.Method 16 patients with primary BCS caused by hepatic vein thrombosis treated in our hospital between June 2010 to December 2012 were used as the study group while 132 patients with primary BCS caused by other causes were used as the control group.A retrospective study was then employed to analyze the clinical data of the two groups of patients during hospitalization and on follow-up.The study was censored in June 2013.The median follow-up was 24 months (range,6 months to 36 months).The difference in quantitative data between the 2 groups were analyzed using the independent-samples t test and the Wilcoxon W rank sum test,and the difference in qualitative data were analyzed using the Chi-square test and the Fisher's exact test.The survival rates and recurrence rates were calculated using the Kaplan-Meier method.Result The study group was significantly lower than the control group in age,duration of symptoms,albumin level,diameter of spleen and survival rate,but it was significantly higher in the proportion of patients with ascites,average hospitalization time,alanine transaminase,aspartate aminotransferase,total bilirubin,carbohydrate antigen-125 and recurrence rate after percutaneous transluminal angioplasty.The differences were significant (P ＜ 0.05).The Rotterdam BCS prognosis grades of the study group were:9 patients grade Ⅱ and 7 patients grade Ⅲ.In the control group,there were 65 patients with grade Ⅰ,51 patients with grade Ⅱ,and 16 patients with grade Ⅲ.The prognosis grade of the study group was significantly higher than the control group (P ＜ 0.05).Conclusion When compared to the patients with BCS due to other causes,patients with BCS caused by hepatic vein thrombosis were more common in the young,most of them were diagnosed in the acute period,they had worse clinical outcomes and had more severe clinical symptoms and liver damage.

Objective To explore the therapeutic efficacy of endovascular embolization for the treatment of tiny intracranial aneurysms (≤3.0 mm) and to discuss its technical skill. Methods During the period from Dec. 2010 to July 2013, a total of 12 patients with tiny intracranial aneurysms (≤3.0 mm) were admitted to authors’ hospital to receive endovascular embolization therapy. Of the 12 patients, narrow-necked aneurysm (neck-to-body ratio ≤0.5) was seen in 7 and embolization with coils only was carried out, while wide-necked aneurysm (neck-to-body ratio > 0.5) was seen in 5 and stent-assisted coils embolization was adopted. Based on Raymond grading standard, the immediate therapeutic results were evaluated, and the procedure-related complications were recorded. Thirty days after the treatment , GOS grade was used to assess the results. Follow-up evaluation with angiography or through call was conducted. Results Successful embolization of the aneurysm was obtained in all the 12 aneurysms , with a success rate of 100%. Angiography performed immediately after the treatment showed that complete embolization was achieved in 8 aneurysms (66.7%) and residual aneurysm cavity was seen in 4 aneurysms (33.3%). GOS grading indicated that 12 cases belonged to grade V. The 12 patients were followed up for 1 - 12 months through telephone , and no re-bleeding occurred. Conclusion For the treatment of tiny intracranial aneurysms (≤ 3.0 mm), endovascular embolization is feasible although its safety and efficacy need to be further observed.

Objective To investigated the serum level of carcinoma antigen 125 (CA-125) and its clinical significance in patients with Budd-Chiari syndrome.Methods We reviewed medical records and laboratory tests of patients with BCS first diagnosed in our hospital between August 2011 and April 2013.235 patients were included as experiment group,while 120 healthy adult volunteers were randomly selected as control group.The serum level of CA-125 were detected by electrochemilumescence immunization assay in this single-center retrospective control study.Results The average serum level of CA-125 in experiment group is higher than that of control group [(147.9 ±246.6) kU/L vs (16.0 ±7.2) kU/L,P ＜0.001].In experiment group,the relative coefficient for serum CA-125 with ascites,alanine aminotransferase,aspartate aminotransferase,albumin and Rotterdam BCS scores was 0.79,0.45,0.29,-0.393 and 0.71,respec tively,P ＜0.001.As of October 2013,we found that the 68 BCS patients with serum CA-125 level 5-fold higher than the upper limit of normal (＞ 175 kU/L) presented much lower survival rates and asymptomatic survival rates than the rest 167 BCS patients after intervention therapy:(95.6％ and 79.8％) vs (98.8％ and 92.0％),P ＜ 0.05.Conclusions The serum level of CA-125 in BCS patients have positive correlation with ascites volume,liver injury degree and Rotterdam BCS scores.Serum CA-125 evaluation appears to be a valuable examination option in BCS as CA-125 levels negatively correlate with worse prognosis,thus could be applied as an efficient tool for prognostication.

Objective To evaluate the safety and efficacy of interventional treatment of occlusion of the entire inferior vena cava (IVC).Methods The clinical data of 6 patients with entire IVC occlusion were analyzed retrospectively.All patients were diagnosed by color Doppler ultrasound and DSA.Venography was performed under local anesthesia via internal jugular vein and femoral vein approach.The occlusion of IVC and hepatic vein were treated with balloon dilatation and/or stent placement.Follow-up examination with color Doppler ultrasound was taken 1,3,6,12 months after treatment and annually thereafter to assess the patency of IVC and hepatic vein.The pressure gradient of hepatic vein-right atrium and IVC-right atrium before and after interventional treatment were compared with paired t test.Results In 5 cases,both IVC and 1 hepatic vein were recanalized successfully.In 1 case,recanalization of IVC failed,but the right hepatic vein was recanalized successfully.The mean pressure gradient of hepatic vein-right atrium decreased from (23.2 ± 2.0) cmH2O (1 cmH2O =0.098 kPa) before treatment to (8.7 ± 3.2) cmH2O after treatment in 6 cases (t =21.6,P ＜ 0.05).The mean pressure gradient of IVC-right atrium decreased from (26.6 ± 2.7) cmH2O before treatment to (9.4 ± 1.1) cmH2O after treatment (t =16.1,P ＜ 0.05).Abdominal pains occurred in 3 patients after stent implantation which disappeared in 24 hours.No other complications such as bleeding and death occurred.During a mean follow-up of(42 ± 27)months (16 to 90 months),hepatic vein patency was maintained in 6 cases and IVC patency was maintained in 5 cases.Conclusion Interventional treatment of occlusion of the entire IVC is a safe and effective method.

Objective To evaluate the changes in the number and activities of endothelial progenitor cells (EPCs) from peripheral blood in patients with primary Budd-Chiari syndrome (BCS),and to explore the possible mechanisms of BCS.Methods Eighty-two patients with BCS and 20 healthy subjects used as controls were recruited for this study.The EPCs from peripheral blood were counted by flow cytometry for CD34,CDl33 and KDR for positivity.The peripheral blood mononuclear cells were isolated by density gradient centrifugation and cultured for 7 days.Characterization of EPCs as adherent cells was done using double staining of FITC-UEA-1 and DiI-Ac-LDL binding.The proliferation,adhesion and migration activities were assayed by MTT chromatometry,adhesion activity assay and Transwell assay,respectively.Results EPCs (CD34+/CD133 +/KDR+) were depleted in the BCS patients as compared to the healthy controls [(0.020 ± 0.005) ％ vs (0.038 ± 0.007) ％].The proliferation activities (0.20 ± 0.04 vs 0.58 ± 0.07),adhesion activities (15.8 ± 1.6 vs 35.0 ± 2.5) and migration activities (16.1 ± 1.7 vs 23.9 ± 2.6) were significantly lower in the BCS group than the control group (P ＜ 0.05).Conclusion EPCs from the peripheral blood in patients with BCS exhibited reduced numbers and impaired proliferation,adhesion and migration activity,which may be the key factors for vasculopathy formation in primary BCS patients.

Objective To investigate the value of guide-wire loop in interventional therapy of patients with Budd-Chiari syndrome with hepatic vein obstruction.Methods A retrospective study was conducted on 25 patients with Budd-Chiari syndrome (BCS) with hepatic vein obstruction treated from May 2011 to August 2014.The technique of guide-wire loop was used in these patients.The pressure of the hepatic vein was measured before and after treatment.The difference in the pressure was analyzed by the t test.Results All the patients were treated successfully using guide-wire loop angioplasty.No complications of bleeding,pericardial tamponade and liver capsule hemorrhage were observed.After treatment,the pressure of the hepatic vein reduced from (48.3± 8.0) cmH2O to (20.9 ± 3.8) cmH2O (t =26.82,P ＜ 0.05);The symptoms and physical signs of the patients were relieved or disappeared.BCS-related symptoms reappeared on follow-up in 4 patients,2 were treated by balloon dilation successfully.1 patient was treated with transjugular intrahepatic portosystemic shunt (TIPS) because of decompensated liver cirrhosis.1 patient with ankylosing spondylitis had treatment failure and the symptoms relapsed for the third time.Conclusion The technique of guide-wire loop in interventional therapy of patients with Budd-Chiari syndrome with hepatic vein obstruction is safe and effective,and it can improve the overall success rate of treatment.

Objective To study the intra-and extra-hepatic collateral pathways in various types of Budd-Chiari syndrome (BCS) using liver acceleration volume acquisition (LAVA) enhanced MRI.Methods The clinical data and imaging findings of 240 patients with BCS were collected and analyzed.The types of BCS confirmed by DSA.Intra-and extra-hepatic collateral pathways were studied using LAVA enhanced MRI with a 3.0T scanner.Correlations of the intra-/extra-hepatic collateral pathways with the types of BCS were analyzed using the Chi-square test.Then,the degrees of correlation were calculated by the Cramet correction coefficient of contingency.Results Among the 240 patients,DSA confirmed 60 patients to have hepatic vein occlusion,39 patients to have inferior vena cava occlusion and 141 patients to have both hepatic vein and inferior vena cava occlusion.MRI demonstrated dilated accessory hepatic veins in 157 patients,intra-hepatic communicating branches in 69 patients,inferior phrenic veins in 43 patients,superficial epigastric veins in 135 patients,umbilical veins in 94 patients and hemiazygos/azygos veins in 195 patients.Accessory hepatic veins and hemiazygos/azygos veins as collateral pathways were associated with the types of BCS (x2 =30.239,P ＜ 0.05;x2 =51.295,P ＜ 0.05,respectively).The degrees of correction were 0.355 and 0.462,respectively.Accessory hepatic veins as collateral pathways were most common in the mix type,accounting for 79.4％.Hemiazygos/azygos veins were most common in the inferior vena cava occlusion type and the mix type,accounting for 92.3％ and 91.5 ％,respectively.Conclusions Accessory hepatic veins and hemiazygos/azygos veins as collateral pathways were associated with the types of BCS,while the intra-hepatic communicating branches,inferior phrenic veins,superficial epigastric veins and umbilical veins were not correlated with the types of BCS.LAVA may help to diagnose and determine the best choice of treatment for the various types of BCS.

Objective To evaluate the clinical effect of reduced glutathione in protecting the liver function for patients with hepatocellular carcinoma (HCC) after receiving international therapy.Methods The clinical data of 110 HCC patients,who were admitted to authors' hospital during the period from January 2010 to June 2013,were retrospectively analyzed.According to the random number table method the patients were divided into the control group (n=50) and the treatment group (n=60),and transcatheter arterial chemoembolzation (TACE) and transcatheter arterial infusion (TAI) were carried out in all patients.After the treatment,intravenous infusion of 2.0 g vitamin C,0.2 g vitamin B6,2.0 g inosine,and 250 ml branched chain amino acid was employed per day for one week for the patients of both groups,and additional intravenous dripping of 1.8 g reduced glutathione (added in 250 ml of 5％ glucose solution) was adopted for the patients of the treatment group.Various liver function indexes were tested before and one week after the treatment,the results were compared between the two groups.Results Before the treatment,no obvious differences in the hepatic functions and in the clinical condition existed between the two groups.One week after the treatment,in both groups the aminotransferase (ALT and AST) and total bilirubin (TBIL) were significantly increased when compared with the preoperative data (P＜0.05),while the serum pre-albumin (PA) was remarkably decreased when compared with the preoperative data (P＜0.05);these changes of various indexes in the treatment group were apparently better than those in the control group (P＜0.05).Conclusion The use of reduced glutathione can effectively relieve the liver function damage caused by interventional therapy in HCC patients,therefore,reduced glutathione has a very good clinical application value.

Objective To evaluate the efficacy of interventional therapy for hepatocellular carcinoma complicated by Budd-Chiari syndrome. Methods Clinical data and imaging studies of 17 patients with hepatocellular carcinoma complicated by Budd-Chiari syndrome were retrospectively analyzed. Budd-Chiari syndrome was diagnosed by color Doppler ultrasound and confirmed by cavography in 17 patients. Hepatocellular carcinoma was diagnosed by fine-needle aspiration cytology in 5 patients,and by color Doppler ultrasound, computed tomography and /or MRI, and elevated level of alpha-fetoprotein in 12 patients. Both percutaneous transluminal angioplasty for treatment of obstruction of the inferior vena cava and transcatheter arterial chemoembolization (TACE) for hepatocellular carcinoma were performed in all patients. During follow-up, the tumor size, liver function, serum alpha-fetoprotein level and the recanalized inferior vena cava were evaluated by liver ultrasound, CT and laboratory examination. t test was used to compare the pressure. Results Thirty-nine interventional procedures were performed in 17 patients and all operations were successful without complications. Over the follow-up period of 2 to 90 months after percutaneous transluminal angioplasty in 17 patients, re-occlusion of inferior vena cava occurred in only one patient, which was redilated successfully. Following TACE, all 17 patients survived at two months followup, 13 patients survived at 6 months follow-up, 10 patients survived at 1 year follow-up, 5 patients survived at 2 years follow-up. The pressure of vena cava was (20.5±2.1) cm H2O (1cm H2O=0.098 kPa) before the interventional theraphy, while it was (3.6±1.0) cm H2O after it (t=30.32, P＜0.05). Conclusion Interventional therapy can be effectively performed for treatment of hepatocellular carcinoma complicated by Budd-Chiari syndrome.

Objective To investigate the feasibility,safety and efficiency of the establishment of model for Budd-Chiari syndrome with hepatic vein obstruction through endovascular technology in canine.Methods Twenty four dogs were randomly divided into experimental group (n=18) and control group (n=6).Under the surveillance of digital subtraction angiography,the balloon catheter was sent to the target hepatic vein via right external jugular vein,and then the balloon was filled by contrast agent until the target hepatic vein was blocked completely.In the experimental group,3～5 ml the mixture of N butyl-cyanoacrylate and lipiodol was infused into the target hepatic vein through the end hole of the balloon catheter until the hepatic vein flow stasis was achieved.In the control group,equal volume of normal saline was injected.The changes of liver function,portal vein pressure were measured and pathological varieties of target hepatic vein as well as the liver parenchyma were observed in the different periods in the two groups.Results The successful rate of the technique was 100 percent.There were no serious complications such as pulmonary embolism and death in the two groups.In the experimental group,the serum levels of alanine transpeptidase were (52.5 ± 12.5)U/L,(61.3±5.7)U/L,(38.6±9.4)U/L,which were higher than those in control group(P＜0.05) and prealbuminwere (0.18±0.04)g/L,(0.22±0.02)g/L,(0.19±0.06)g/L,which were lower than those in control group(P＜0.05) in the fourth,sixth and eighth weeks after the procedure,respectively.A common trunk formed by the middle and left hepatic veins which was looked as the targetic hepatic vein were completely occluded.the color of the liver appeared light red,dark red and dull black in the fourth,sixth and eighth weeks after the procedure,respectively.However,the hepatic veins were patented in the control group.In experimental group,histopathological observation revealed hepatic cells congestion and edema while a lot of inflammatory cells were seen in the wall of hepatic vein in the fourth week,the hepatic cells changed with severe edema,adipose kind,inner and middle membranes became thicker in the sixth week,and part of the hepatic cells showed hydropic degeneration,besides,inner and middle membrane became more thicker,there was substantially proliferation in elastic fiber hyperplasia in the eighth week.Conclusion Endovascular technology was a safely and effectively way to establish the canine model of Budd-Chiari syndrome with hepatic vein obstruction.