Background:Budd-Chiari syndrome (BCS) presents diagnostic and treatment challenges owing to its insidious onset. Genetic variants associated with BCS vary geographically; in Asian populations, the condition is primarily caused by membranous obstruction composed of endothelial cells (ECs). A better understanding of the genetic pathogenesis of membranous BCS may offer new insights into disease mechanisms. Methods:This study employed whole-exome sequencing to identify candidate genes responsible for EC abnormalities in 485 patients with membranous BCS and 329 patients with vascular malformations (VaMs). Functional investigations were conducted to validate the selected genes in vitro and in vivo. Results:Whole-exome data revealed that the frequency of variants in the vascular function-related KLHDC2 exceeded that of JAK2 in BCS. Knockdown of KLHDC2 promoted adhesion and suppressed proliferation of ECs. In addition, 92 genes enriched for rare variants overlapped between BCS and VaMs. Systems biology analysis revealed two gene clusters, including COMMD9, enriched in proteins intolerant to loss-of-function mutations. Furthermore, suppression of COMMD9 impaired EC migration and tube formation, inhibited subintestinal angiogenic sprouting in zebrafish, and elevated EC adhesion. Transcriptomic analysis linked COMMD9 to EC abnormalities via the PI3K-Akt pathway. Commd9 knockdown promoted venous hypercoagulability in vivo following drug or ligation-induced stenosis. Conclusions:These findings indicate that multiple rare genetic variants, particularly in COMMD9, are involved in the development of membranous BCS by regulating hypercoagulability induced by EC abnormalities. These findings may help guide future clinical research towards improved understanding and treatment of BCS.

Patients with Budd–Chiari syndrome (BCS) often experience a strong sense of illness uncertainty due to complex and diverse symptoms, unclear etiology, and insufficient popularization of the disease knowledge. Therefore, this study examined illness uncertainty and its influencing factors in BCS patients to help develop targeted interventions. A cross-sectional study was conducted among 183 BCS patients recruited from a tertiary Grade-A hospital in China between January 2024 and July 2025. Data were collected using the General Information Questionnaire, the Chinese version of the Mishel Illness Uncertainty Scale, and the Chinese version of the Multidimensional Scale of Perceived Social Support. Independent samples T-tests, one-way ANOVA, and multiple linear regression analysis were used for data analysis. BCS patients demonstrated a moderate level of illness uncertainty, with a mean total score of 96.82 ± 10.78. Among all dimensions, unpredictability had the highest mean score of items (3.49 ± 0.59). Multivariate regression analysis identified place of residence, education level, monthly per capita family income, hospitalization frequency, and social support as factors associated with illness uncertainty. Healthcare professionals should focus more on these aspects of patients and deliver individualized psychological interventions.

Background: Budd-Chiari syndrome (BCS) is a rare thrombotic liver disorder with a substantial risk of recurrence, even after treatment. Inflammation-based markers such as the neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), and lymphocyte-to-monocyte ratio (LMR) have been associated with thrombotic diseases, but their predictive value for BCS recurrence remains unclear. Methods: This retrospective cohort study included 708 (median 51 y, male: 57.8%) patients diagnosed with primary BCS between January 2015 and July 2022. Nine systemic inflammatory indices were calculated from baseline and biochemical parameters. The primary outcome was recurrence, defined by clinical symptoms and imaging-confirmed re-obstruction or thrombosis. Associations were evaluated using logistic regression models, quartile-based logistic regression, and weighted quantile sum (WQS) regression. Subgroup and interaction analyses were also performed. Results: During follow-up, 226 patients (31.9%) experienced post-treatment recurrence. Elevated NLR and PLR were associated with increased recurrence risk, while higher LMR was protective. WQS regression identified novel indices such as the neutrophil-toalbumin ratio (NAR) and monocyte-to-albumin ratio (MAR) as top contributors. Subgroup analyses showed consistent associations, especially in IVC-type BCS and cirrhotic patients. Conclusion: Several inflammation-based indices were independently associated with recurrence risk in primary BCS. These findings highlight the potential utility of inflammatory markers in recurrence surveillance.

BACKGROUND AND AIMS:Endovascular treatment has improved Budd-Chiari syndrome (BCS) patient outcomes, but patients remain at risk for developing hepatocellular carcinoma (HCC). We aimed to analyse the characteristics and risk factors for HCC development in BCS patients after endovascular treatment. METHODS:Clinical data of BCS patients who had received endovascular treatment were retrospectively reviewed. Characteristics of BCS patients who developed HCC post-treatment were compared with those without HCC development. Univariable and multivariable Cox regression analyses were used to determine the risk factors. RESULTS:We enrolled 302 BCS patients. HCC was confirmed in 31 patients after treatment. Early-stage tumours were the most common (11/31, 35.5 %) according to the Barcelona Clinic Liver Cancer staging system. A serum alpha fetoprotein (AFP) cut-off level of > 15.7 ng/mL showed a sensitivity of 69.3 % and specificity of 97.4 % for detecting HCC in these patients. The presence of preoperative liver cirrhosis (hazard ratio (HR)=4.677; P = 0.043) and postoperative restenosis (HR=6.867; P < 0.001) were independent risk factors associated with HCC development in BCS patients after endovascular treatment. CONCLUSION:HCCs that develop after endovascular treatment in BCS patients are often detected at an early stage. Preoperative liver cirrhosis and postoperative restenosis were independent risk factors for HCC development in these individuals.

>Budd-Chiari syndrome(BCS) is a series of clinical syndromes caused by obstruction of the hepatic vein and/or hepatic segment inferior vena cava, which can seriously damage the health of the body and lead to patient death [1].Over recent years, with the improvement of diagnostic technology, especially the introduction of multidisciplinary intervention methods, more and more potential cases have been discovered and treated [2]. However, conventional clinical diagnosis is usually based on traditional indicators [3],

Background and aims To investigate the feasibility and long-term outcomes of hepatic vein (HV) recanalization using intrahepatic collateral pathways in patients with Budd–Chiari syndrome (BCS) with HV obstruction. Methods Clinical data of 29 BCS patients with HV obstruction and intrahepatic collateral pathways were reviewed. All patients underwent HV recanalization through the intrahepatic collaterals. Follow-up was performed at 1, 3, 6, and 12 months after treatment and annually thereafter. Cumulative patency and survival rates were assessed using Kaplan–Meier curves. The independent predictors of patency were determined using a Cox regression model. Results HV recanalization was successful in 28 of the 29 patients (96.6%), with no complications. Of the 28 cases, simultaneous recanalization of the accessory HV and right HV was achieved in 11 patients, accessory HV and middle HV in six, accessory HV and left HV in three, right HV and middle HV in five, and left HV and middle HV in three. Twenty-eight patients were followed from 4 to 87 (mean, 53.6 ± 26.7) months after treatment, and six patients developed reocclusion. The overall cumulative 1-, 3-, 5-, and 7-year primary HV patency rates were 96.3, 82.9, 74.6, and 59.7%, respectively. The cumulative 1-, 3-, 5-, and 7-year survival rates were 100, 95.8, 95.8, and 86.3%, respectively. Conclusion Interventional treatment of HV obstruction in BCS patients through intrahepatic collateral approaches is well tolerated and feasible and can result in excellent long-term patency and survival rates.

目的 观察以小球囊预扩张联合导管溶栓治疗髂股动脉硬化闭塞症伴急性血栓形成的价值.方法 纳入33例接受小球囊预扩张联合经导管溶栓治疗及血管成形术的单侧髂股动脉硬化闭塞症伴急性血栓形成患者,其中14例病变仅累及髂动脉、8例仅累及股动脉、11例同时累及髂股动脉;记录治疗情况及随访资料,包括临床症状、下肢动脉彩超及CT血管造影.结果 33例均治疗成功,经导管溶栓时间为3(2,4)天,25例血栓完全溶解、8例血栓部分溶解;溶栓后22例接受单纯球囊扩张、11例接受球囊扩张+支架植入术;治疗后复查数字减影血管造影示33例靶血管及膝下流出道血流均通畅.共随访(19.5±8.2)个月,期间7例出现下肢动脉再闭塞,经二次球囊扩张及植入支架后恢复通畅;无截肢及死亡病例.结论 小球囊预扩张联合经导管溶栓治疗髂股动脉硬化闭塞症伴急性血栓形成具有较高价值.

To evaluate the changes in the liver volume and function after direct intrahepatic portocaval shunt (DIPS) in patients with Budd-Chiari syndrome (BCS) with diffuse hepatic vein (HV) occlusion. The clinical data of 29 patients with BCS who underwent DIPS for intractable ascites caused by diffuse hepatic vein occlusion in the Affiliated Hospital of Xuzhou Medical University were analysed retrospectively; the patients included 8 males and 21 females, with an average age of 33.3 ± 6.3 years. The patients underwent abdominal CT scanning and liver function examinations before DIPS, and 1 week, 3 months, 6 months and 12 months after DIPS. The changes of the liver volume and liver function before and after DIPS were compared. All 29 patients underwent DIPS successfully. 28 patients survived during the follow-up of 12–33 months, with a median follow-up of 16 months. The patients’ liver function were significantly improved at 3, 6 and 12 months after the operation compared to before the operation (P < 0.05). The liver volumes measured before the operation and 1 week, 3 months, 6 months and 12 months after the operation were 2124.586 ± 420.889 cm3, 1926.263 ± 372.268 cm3, 1480.592 ± 183.061 cm3, 1461.904 ± 153.027 cm3 and 1469.286 ± 148.549 cm3, respectively. Compared with the preoperative liver volume, the liver volume had decreased significantly at 1 week, 3 months, 6 months and 12 months after the operation (P < 0.05). However, there was no significant difference in the liver volumes at 6 and 12 months after the operation (P = 0.35). Direct intrahepatic portocaval shunt has achieved satisfactory clinical results in the treatment of BCS with diffuse hepatic vein occlusion. The congestive hepatomegaly was gradually reduced after the operation. The liver volume (which was defined as the clinical efficacy in this study) remained stable after 6 months.

To investigate the independent risk factors for the first recurrence after endovascular management in patients with Budd–Chiari syndrome (BCS), and to establish a prediction model for predicting recurrence in target patients. BCS patients who underwent endovascular treatment in the Affiliated Hospital of Xuzhou Medical University from January 2010 to December 2015 were retrospectively examined, with their clinical, laboratory test, and imaging data collected and analyzed. Independent risk factors for recurrence were identified, and a prediction model was established and validated. A total of 450 patients met the filtering criteria, and 102 recurred during the follow-up. The median follow-up time was 87 months, ranging from 1 to 137 months. The 1-, 3-, 5- and 10-year cumulative recurrence rate was 9.11 https://mrbet.shinyapps.io/dynnomapp . Liver cirrhosis, ascites, thrombosis, and obstructed HV + AHV are independent risk factors for the first recurrence; age is an independent protective factor. The prediction model can effectively and conveniently predict the risk of recurrence and screen out patients at a high recurrence risk.

To investigate the efficacy, feasibility, and safety of transjugular intrahepatic portosystemic shunt (TIPS) as a treatment for patients with recurrent portal hypertension with variceal bleeding (RPHVB) who have previously undergone open splenectomy and esophagogastric devascularization (OSED). The data were retrospectively retrieved from 39 cirrhotic RPHVB patients who had undergone OSED from August 2015 to December 2020. All patients were treated with TIPS using the Viabahn stent. Out of the 39 patients included in the study, TIPS was successfully performed in 38 patients with a success rate of 97.44

目的 探讨肝尾状叶交通静脉(HCCV)在Budd-Chiari综合征(BCS)患者临床诊断和介入治疗中的价值.方法 收集2015年1月至2016年12月在徐州医科大学附属医院行上腹部MRA检查并于1周内行DSA检查的247例BCS患者临床资料.观查患者HCCV发生率,测量HCCV开口直径和开口至右心房入口距离,分析HCCV与肝静脉的交通关系.结果 247例BCS患者中检出110例有HCCV,发生率为44.5％,其中下腔静脉阻塞型患者中发生率为58.1％.HCCV开口直径为(11.3±4.3)mm,开口至右心房入口距离为(5.3±1.5)cm.HCCV与肝中静脉和/或肝左静脉存在交通关系.结论 HCCV在肝左静脉和肝中静脉阻塞时具有代偿性引流肝脏静脉血液的作用,有助于直接或间接诊断BCS,为介入治疗提供了一种选择.

PurposeThis study was designed to assess the clinical efficiency and long-term outcomes of hepatic vein (HV) and accessory hepatic vein (AHV) recanalization in patients with HV-type Budd-Chiari syndrome (BCS).Material and methodsA total of 27 patients with HV-type BCS underwent AHV recanalization and 94 patients had HV recanalization at our center from January 2012 to December 2019. The treatment effectiveness and long-term outcomes were compared.ResultsTechnical success was accomplished in all patients, without any procedure-related complications. The clinical success rates were 96.3% (26/27) and 95.7% (90/94) (p = 1.000). In the AHV and HV groups, re-obstruction was observed in 5 and 36 patients, respectively (p = 0.056). The median primary durations of AHV and HV patency were 64 and 49 months, respectively (p = 0.036), while the median secondary durations of AHV and HV patency were 70 and 64 months, respectively (p = 0.134). The median overall survival after AHV and HV recanalization was 73 and 78 months, respectively (p = 0.263).ConclusionsOur findings suggest that AHV could be employed as a replacement for HV, as a hepatic drainage vein, in HV-type BCS patients.

Background Anticoagulation therapy (AT) is often used as the initial treatment for pyrrolizidine alkaloid (PA)-induced hepatic sinusoidal obstruction syndrome (HSOS). However, transjugular intrahepatic portosystemic shunt (TIPS) is an alternative treatment. This study aimed to determine the mid- to long-term outcomes of TIPS versus AT as the initial treatment for PA-induced HSOS. Methods We retrospectively analyzed the clinical data of 61 patients with PA-induced HSOS that were collected between November 2015 and July 2021. The patients were allocated to the TIPS group ( n = 20) or the AT group ( n = 41). These two groups were divided into subgroups according to the severity grading. The clinical data of the patients in both groups were analyzed. Cumulative survival rates were calculated and compared between the two groups and among the subgroups. Results The clinical symptoms and signs improved or stabilized in 100% of the patients following TIPS and in 85% of the patients following AT at discharge ( P = 0.166). The mortality rate was 0.0% in the TIPS group and 34.1% in the AT group ( P = 0.005). The patients were followed up for 2–69 months (mean, 26.3 ± 20.5 months). In the mild- and moderate-grade subgroups, there was no difference in the cumulative survival rate between the TIPS and AT groups ( P = 0.589 and P = 0.364, respectively). In the severe and very severe-grade subgroups, the cumulative survival rate was higher in the TIPS group than in the AT group ( P = 0.018 and P = 0.025, respectively). Conclusion AT is a suitable initial treatment for mild or moderate PA-induced HSOS, whereas TIPS should be considered the appropriate initial treatment for severe or very severe PA-induced HSOS.

目的 探讨递增式扩张分流道在肝硬化门静脉高压经颈静脉肝内门体分流术(TIPS)中的疗效.方法 选取肝硬化门静脉高压症施行TIPS治疗的68例患者资料,术中先采用直径6 mm球囊扩张,根据PPG下降情况决定是否采用直径8 mm球囊扩张者为递增扩张组(n=37),直接采用直径8 mm球囊扩张者为对照组(n=31),全部患者球囊扩张后均置入直径8 mm覆膜支架.术后1、3、6、12个月及以后每6个月进行随访,对比分析两组患者术后肝性脑病、分流道通畅率及生存率.结果 两组患者术前基线水平比较,差异无统计学意义(P＞0.05).68例患者技术成功率100％.两组患者术后中位随访时间26.6(3.3～38.8)个月,递增扩张组术后1、2、3年肝性脑病累积发生率分别为8.3％、12.6％和17.2％,对照组为22.7％、45.4％和59％,差异有统计学意义(P＜0.05).递增扩张组术后1、2、3年分流道首次累积通畅率分别为94.5％、89.8％和89.8％,对照组为96.8％、93.2％和93.2％(P=0.75).递增扩张组术后1、2、3年累积生存率分别为91.8％、88.1％和83％,对照组为90.3％、90.3％和57.5％(P=0.34).结论 递增式扩张与非递增式扩张分流道施行TIPS的疗效相似,但可以减少TIPS术后肝性脑病的发生率.

目的 评价肝癌伴门静脉高压食管胃静脉曲张破裂出血(EGVB)患者行经颈静脉肝内门体分流术(TIPS)联合经动脉化疗栓塞术(TACE)的安全性及疗效.方法 16例肝癌伴门静脉高压EGVB的患者施行TIPS联合TACE治疗,男11例,女5例,年龄49～74岁,平均(59.5±8.4)岁.术后1、3、6、12个月及以后每3个月采用彩色多普勒超声和上腹部增强CT进行随访.TACE术后1个月采用改良实体肿瘤疗效评价标准(mRECIST)评价肝癌疗效.采用Kaplan-Meier曲线评估存活率.结果 16例患者TIPS技术成功率100％,门静脉压力梯度(PPG)由术前平均(36.5±4.9)mmHg降至术后平均(8.2±2.5)mmHg(t=18.595,P＜0.01).16例患者共施行36次TACE,术后出现肝脓肿1例,经穿刺引流脓腔消失.16例患者随访3～122个月,平均(34.9±34.3)个月.TIPS联合TACE治疗术后1个月完全缓解(CR)、部分缓解(PR)、疾病稳定(SD)、疾病进展(PD)分别为6、5、2、3例.TIPS术后24个月支架内再狭窄1例,经再次球囊扩张后通畅.随访期间全部患者均无再次出血;出现轻度肝性脑病3例、肝性脊髓病1例;死亡3例.术后3、6、12、24、36个月累积生存率分别为100％、91.7％、83.3％、83.3％、83.3％.结论 TIPS联合TACE治疗肝癌伴门静脉高压EGVB是安全、有效的方法.

目的 探讨胆道双金属支架联合125I粒子条植入治疗高位恶性梗阻性黄疸的疗效与安全性.方法 选取2015年6月～2020年1月我院诊治的67例高位恶性胆道梗阻患者,38例行胆道双支架联合125I粒子条植入为联合组,29例行胆道双支架植入为对照组.分析两组患者术前与术后实验室指标变化情况.比较两组间术后引流有效率、并发症发生率、支架通畅时间及生存时间.结果 两组患者术后引流有效率、并发症发生率比较差异无统计学意义(P＞0.05).联合组中位生存时间高于对照组,差异有统计学意义(P＜0.05).联合组患者中位支架通畅时间明显高于对照组,差异有统计学意义(P＜0.05).结论 胆道双金属支架联合125I粒子条植入治疗高位恶性梗阻性黄疸安全、有效,可改善临床症状,提高生活质量,延长生存时间及支架通畅时间.

目的 探讨经皮左锁骨下动脉途径植入导管药盒系统(port-catheter system,PCS)技术的优势和特点.方法 选取我院不可切除的原发性肝脏恶性肿瘤41例患者的临床资料,通过经皮植入导管药盒系统行肝动脉灌注化疗(hepatic arterial infusion chemotherapy,HAIC)治疗,记录并发症情况(出血、感染、导管头移位、脱管、闭塞)和患者生活状态.结果 技术成功率100％,4例(9.7％)出现与置管相关(出血、感染)的并发症,其中2例为穿刺点周围血肿,1例为囊袋内血肿,1例为切口愈合不良;3例(7.3％)患者发生导管移位或脱管,2例患者取出药盒系统.所有患者生活基本不受影响.本组随访期间客观缓解率ORR为48.4％.结论 经左侧锁骨下动脉途径植入导管药盒系统行HAIC治疗安全可行,对患者生活影响较小,接受度高.

目的 探讨超声引导经皮肝穿刺放射性125 I粒子植入治疗I、Ⅱ型门静脉癌栓的临床疗效及安全性.方法 选取69例原发性肝癌合并I、II型门静脉癌栓患者,按照引导方式不同分为超声组35例及数字剪影血管造影(digital subtraction angiography,DSA)机组34例,比较2组患者术后缓解率、有效率及局部疼痛、出血等不良反应发生率.结果 超声组缓解率(51.4％)高于DSA机组(23.5％)(P<0.05);2组有效率比较差异无统计学意义(P>0.05).术后3 d患者肝功能及血白细胞计数组间比较差异无统计学意义(P>0.05),各组患者术后3 d与术前比较差异亦无统计学意义(P>0.05).出血、局部疼痛、恶心呕吐、发热等其他不良反应发生率均较低,组间比较差异无统计学意义(P>0.05).2组患者术后均未发生腹腔大出血、气胸、胆管损伤、肝脓肿、肝衰竭等严重并发症.结论 超声引导经皮肝穿刺放射性125 I粒子植入治疗I、Ⅱ型门静脉癌栓临床缓解率高于DSA机引导粒子植入治疗,不良反应发生率低,临床应用相对安全.

Objective:To study the risk factors of Budd-Chiari syndrome (BCS) associated with hepatocellular carcinoma in patients who underwent endovascular recanalization.Methods:The data of 340 patients with BCS who underwent endovascular recanalization at the Affiliated Hospital of Xuzhou Medical University between January 2015 and June 2021 were retrospectively collected. Using propensity score matching, a total of 57 patients (40 males and 17 females) were enrolled into this study, with the age of (50.4±8.7) years. Patients were divided into the hepatocellular carcinoma group ( n=19) and the control group ( n=38) according to whether occurrence of hepatocellular carcinoma after cardovascular recanalization. Preoperative indicators including gender, age, BCS type, and model for end-stage liver disease (MELD) score, and postoperative indicators including alpha fetoprotein, intrahepatic nodule formation, vascular restenosis, aspartate aminotransferase (AST) and alanine aminotransferase (ALT) were compared between the two groups after propensity score matching. Multivariate logistic regression analysis was used to analyze the risk factors of BCS associated with after endovascular recanalization in these patients. Results:There were no significant differences in gender, age, BCS type, MELD score and other preoperative data between the two groups (all P>0.05). The proportions of patients with postoperative alpha fetoprotein>9.0 μg/L, AST>40 U/L, ALT>50 U/L, intrahepatic nodules and vascular restenosis after endovascular treatment in the hepatocellular carcinoma group were significantly higher than those in the control group (all P<0.05). Multivariate analysis showed postoperative alpha fetoprotein >9.0 μg/L ( OR=46.778, 95% CI: 3.310-661.140), AST>40 U/L ( OR=36.307, 95% CI: 1.317-1 001.009), intrahepatic nodule formation ( OR=66.254, 95% CI: 4.225-1 038.974) and vascular restenosis ( OR=16.276, 95% CI: 1.712-154.773) to have an increased risk of being associated with hepatocellular carcinoma in these BCS patients (all P<0.05). Conclusion:Postoperative alpha fetoprotein>9.0 μg/L, AST>40 U/L, intrahepatic nodule formation and vascular restenosis were independent risk factors of BCS associated with hepatocellular carcinoma in patients who underwent endovascular recanalization.

目的 通过对比股动脉(CFA)入路探讨经桡动脉(RA)入路行TACE的安全性、可行性及优缺点.方法 376例行TACE手术患者,采取CFA入路、右侧RA入路、左侧RA入路的例数依次为238例、88例、50例,就手术时间、手术与穿刺成功率、X线透视时间、发生并发症状况、病例选择倾向,对3组展开对比分析.结果 在手术与穿刺成功率方面,两侧RA组皆未表现出明显不同(P＞0.05),但在这2项指标上,相较CFA组,两侧RA组皆表现为显著偏低(P＜0.05).在手术时间与X线透视时间上相较左侧RA组与CFA组,右侧RA皆表现为显著偏高(P＜0.05),同时在这2项指标中,相较CFA组,左侧RA组皆表现为显著偏高(P＜0.05).在并发症发生状况相较CFA组,两侧RA组皆表现出优势,多数患者更倾向于再次手术时选择两侧RA入路进行手术.结论 经桡动脉行TACE是安全可行的,在患者中接受度更高,虽存在操作难度较高等不足,但仍值得进一步发展及应用.