[Comparison of clinical features of JAK2V617F gene mutation and non-mutation in patients with Budd-Chiari syndrome].

Objective: To investigate the clinical features of JAK2V617F gene mutation and non-mutation in patients with Budd-Chiari syndrome (BCS). Methods: 17 and 127 BCS cases with JAK2V617F gene mutation (mutation group) and non-gene mutation (non-mutation group) who were continuously treated with interventional therapy between January 2016 to December 2020 in the Affiliated Hospital of Xuzhou Medical University were selected as the research object for a comparative study. The hospitalization and follow-up data of the two groups were analyzed retrospectively, and the deadline for follow-up was June 2021. Quantitative data group differences were analyzed using the independent sample t-test and Wilcoxon rank sum test. Qualitative data group differences were analyzed with χ2 test or Fisher's exact test. Mann-Whitney U test was used to analyze the differences between groups in rank data. Kaplan-Meier method was used to calculate the patient survival and recurrence rate. Results: Age [(35.41±17.10) years vs. (50.09±14.16) years, t=3.915, P<0.001], time of onset (median duration: 3 months vs. 12 months), and the cumulative survival rate (65.5% vs 95.1%; χ2=5.21, P=0.022) were lower in mutation than non-mutation group. Aaspartate aminotransferase, alanine aminotransferase, prothrombin time, Child-Pugh score, Rotterdam score, Model for End-stage Liver Disease score, hepatic vein thrombosis incidence, and the cumulative recurrence rate after intervention were higher in mutation than non-mutation group. The above all indexes had statistically significant differences (P<0.05) between the groups. Conclusion: Younger age, acute onset, severe liver injury, high incidence of hepatic vein thrombosis, and poor prognosis are the features of patients with BCS with JAK2V617F gene mutation than non-mutation.

[Analysis of curative efficacy of transjugular intrahepatic portosystemic shunt using viabahn stent in the treatment of Budd-Chiari syndrome with extensive hepatic vein occlusion].

Objective: To investigate the efficacy of transjugular intrahepatic portosystemic shunt (TIPS) using viabahn stent in patients with Budd-Chiari syndrome (BCS) with extensive hepatic vein occlusion. Methods: Technical success rates, portal pressure gradient (PPG), complications and shunt patency rate of 25 cases with BCS with extensive hepatic vein occlusion treated by TIPS using viabahn stent were retrospectively analyzed. Color Doppler ultrasound was used for postoperative follow-up. Patency rates were evaluated by Kaplan-Meier curve. Results: The technical success rate of TIPS was 100%. Mean PPG decreased from (22.7 ± 6.2) mmHg preoperatively to (9.7 ± 2.6) mmHg postoperatively (t = 9.58, P < 0.05). Hepatic encephalopathy incidence was 8.0% (2/25), and there were no complications such as intra-abdominal hemorrhage. Twenty-five cases were followed up for 1 to 39 ( mean 19.0 ± 11.9) months, and one patient died of liver failure three months after interventional treatment. The primary patency rates at 1, 2, and 3 years after interventional treatment were 91.3%, 91.3%, and 78.2%, respectively, while the repatency rates were 100%, 92.9%, and 92.9%, respectively. Conclusion: TIPS using viabahn stent can bring better short-term and mid-term curative efficacy in treatment of BCS with extensive hepatic vein occlusion.

[Preliminary application of transjugular intrahepatic portosystemic shunt in individualized treatment of patients with cirrhotic portal hypertension complicated with refractory ascites and variceal hemorrhage].

Objective: To investigate the feasibility, efficacy and safety of transjugular intrahepatic portosystemic shunt (TIPS) in the individualized treatment of patients with refractory ascites and variceal hemorrhage caused by portal hypertension. Methods: Prospective study of clinical data of 47 patients with portal hypertension and refractory ascites and variceal bleeding admitted to the Affiliated Hospital of Xuzhou Medical University from August 2017 to December 2018, 26 males and 21 females, aged 23-75 (52±14) years old. The Viabahn stent was used to control the diameter of the shunt and the preoperative interval of PPG after individualized TIPS was determined according to the preoperative liver function Child-Pugh classification. The PPG of the Child A and B patients was <10 mm Hg (1 mmHg=0.133 kPa), Child-Pugh C grade patients with postoperative PPG interval values of 12 to 15 mmHg. The success rate, hemostasis rate, ascites remission rate, and complication rate were recorded. Results: Forty-seven patients were with a success rate of 100%, and there was no surgically related fatal complications. The portal pressure gradients of patients with Child-Pugh A, B and Child-Pugh C were reduced from preoperative (22.5±5.4), (24.4±2.6) mm Hg to postoperative (8.8±2.5), (13.2±1.1) mm Hg (all P<0.05). All the patients were followed up for 6 to 24 months, with a median follow-up of 13 months. The success rate of hemostasis in patients with upper gastrointestinal bleeding was 93.5% (29/31), the remission rate of patients with refractory ascites was 14/16, the postoperative rebleeding rate was 6.5% (2/31), the incidence of hepatic encephalopathy was 8.5% (4/47), and the shunt disorder was 2.1% (1/47). Conclusion: The use of Viabahn stent for individualized TIPS in the treatment of portal hypertension with refractory ascites and variceal hemorrhage is feasible, and the clinical efficacy is affirmative, which can reduce the incidence of postoperative hepatic encephalopathy and shunt dysfunction.

[Application study of ultrasound-guided percutaneous portal vein punctures combined with bi-directionalangiography in the treatment by TIPS].

Objective: To investigate the safety, feasibility, and preliminary clinical experience of ultrasonic guided percutaneous portal vein punctures combined bi-directional angiography in the treatment by transjugular intrahepatic portosystemic shunt(TIPS). Methods: From January 2016 to June 2018, 15 patients with TIPS from our hospital who were treated by ultrasonic guided percutaneous portal vein punctures combined with bi-directional angiography were enrolled,and were recruited as experimental group. During the same period, 30 patients who were treated by TIPS combined with traditional methods were enrolled, and were recruited as control group. There was no statistical difference in baseline characteristics between the two groups (P > 0.05). The portal pressure difference in preoperative and postoperative, the fluoroscopy time, the number of puncture needles and complications were recorded. After treatment, the patients were followed up through outpatient service or telephone method. Results: The technical success rate was 100% in experimental group, and 96.7% in control group. In the experimental group, number of percutaneous transhepatic portal vein puncture by needle was 1-3 (average, 2.13 ± 0.74), and the number of portal vein puncture needles in the control group were 1-11 (average, 4.16 ± 2.13). The number of puncture needles in the experimental group were significantly decreased than in the control group (P < 0.001). In the experimental group, the fluoroscopy time was 18 ~ 46 (average 29.64 ± 8.79) minutes. In the control group, the fluoroscopy time was 12 ~ 150 (average 44.57 ± 26.84) minutes.The fluoroscopy time was significantly reduced in the experimental group compared with the control group(P = 0.023). Conclusion: Ultrasound-guided portal vein combined with bidirectional angiog-raphy is safe, feasible, and reliable in the treatment by TIPS. Compare with traditional TIPS, it can reduce the fluoroscopy time, the number of puncture needles and the liver injury.

Magnetic resonance venography in the diagnosis of inferior vena cava obstruction in Budd-Chiari syndrome.

OBJECTIVE: To evaluate the informativeness of magnetic resonance venography (MRV) in the diagnosis of different types of inferior vena cava (IVC) obstruction. PATIENTS AND METHODS: 56 patients with IVC obstruction underwent MRV scans. These scans were evaluated for morphology of the obstruction and compared with digital subtraction angiography (DSA) images. RESULTS: Using DSA, we determined that 47 patients had complete obstruction and 9 had partial obstruction. MRV scans revealed 6 cases of partial obstruction. Using MRV, we determined the morphology of the proximal and distal ends of the complete obstructions in the IVC. We classified our observations into cone, plateau, and irregular subtypes. Both DSA and MRV scans were compared to assess the consistency between two methods. MRV demonstrated high sensitivity (100%) for diagnosing a complete obstruction of the IVC, and its specificity was 66.7%. The positive and negative predictive values of MRV were 94% and 100%, respectively. CONCLUSIONS: MRV imaging is a valuable alternative to DSA for detection of obstructions in the IVC in Budd-Chiari syndrome, particularly in the distal end, which could expedite the decision making for interventional treatment programmes. However, MRV imaging is less suitable for observing the morphology of the proximal ends of obstructions due to the limitations of the scanning plane and the influence of pseudo shadows.

Correlations of iodide ions with vascular endothelial growth factor and its receptors during the proliferation of vascular endothelial cells.

The aim of this study was to explore the correlations of iodide ions with vascular endothelial growth factor (VEGF) and its receptors during the proliferation of vascular endothelial cells (VECs). The proliferation rates of VECs in the presence of iodide ions and VEGF inhibitor were determined using the CCK-8 method. The effect of iodide ions on the phosphorylation of vascular endothelial growth factor receptor 2 (VEGFR-2) was observed using Western blot analysis. In the presence of 300 µg/L iodide ions, the application of VEGF inhibitor did not inhibit VEC proliferation (P < 0.05). At a certain concentration, iodide ions upregulated the phosphorylation level of VEGFR-2 at the Tyr1214 site (P < 0.05). Iodide ions did not influence the phosphorylation of VEGFR-2 at the Tyr1175 and Tyr951 sites. At an appropriate concentration, iodide ions serve as an independent VEC proliferation-promoting factor. They mediate VEC migration by stimulating the upregulation of the phosphorylation level of VEGFR-2 (Tyr1214) and do not influence VEGFR-2 phosphorylation at Tyr1175. Therefore, their VEC proliferation-promoting effect is independent of membrane receptors.

Strategy and long-term outcomes of endovascular treatment for Budd-Chiari syndrome complicated by inferior vena caval thrombosis.

OBJECTIVES: The aim of this study was to evaluate the strategy and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) complicated by inferior vena cava (IVC) thrombosis. METHODS: The treatment strategy and outcomes of BCS complicated by IVC thrombosis were retrospectively evaluated in a single-center study. The treatment was aimed at the IVC thrombus, not hepatic vein occlusion. All 133 patients with BCS complicated by IVC thrombosis from February 2003 to March 2013 underwent endovascular treatment. For the fresh thrombus group (n=75) recanalization was performed after transcatheter thrombolysis with urokinase. For the mixed thrombus group (n=19) a small balloon pre-dilation of the IVC was performed first, followed by transcatheter thrombolysis using urokinase and a large balloon dilation of the IVC. For the old thrombus group (n=39) a large balloon dilation or/and stent placement was performed directly. Pre- and post-treatment follow-ups were recorded. RESULTS: The endovascular treatment was successful in 131 out of 133 patients (98.5%). Thirty seven patients had synchronous hepatic vein occlusion. The incidence of serious complications was 4.5% (6/133). Symptomatic pulmonary embolism occurred in three cases, cerebral hemorrhage in two, and cardiac tamponade in one. The cumulative 1-, 5-, and 10-year primary patency rate was 96.3%, 84.0%, and 64.6%, respectively. The cumulative 1-, 5-, and 10-year secondary patency rate was 99.0%, 96.1% and 91.3%, respectively. Segmental occlusion of the IVC and duration of anticoagulant therapy less than 6 months were independent risk factors for reocclusion. CONCLUSIONS: For patients with BCS complicated by IVC thrombosis, an individualized treatment strategy based on the property of the thrombus can result in excellent long-term patency.