Intravenous leiomyomatosis (IVL) is a rare gynecological-related tumor. It can invade and extend in the blood vessel and eventually involve the cardiac cavity or even the pulmonary artery. IVL generally does not adhere to the vein wall and infrequently leads to the manifestation of Budd-Chiari syndrome (BCS). In this case report, the presence of a sizable tumor obstructed the second hepatic portal, impeding the return flow of the hepatic veins, thereby precipitating the development of BCS. The presence of collateral veins and dilation of the accessory hepatic vein were identified through computed tomography venography and ultrasonography, thus supporting the diagnosis of BCS. The patient underwent a comprehensive surgical resection and was found to have a favorable prognosis.

Budd-Chiari syndrome (B-CS) is a rare and lethal condition characterized by hepatic venous outflow tract blockage. Gut microbiota has been linked to numerous hepatic disorders, but its significance in B-CS pathogenesis is uncertain. First, we performed a case-control study (Ncase = 140, Ncontrol = 63) to compare the fecal microbiota of B-CS and healthy individuals by metagenomics sequencing. B-CS patients\' gut microbial composition and activity changed significantly, with a different metagenomic makeup, increased potentially pathogenic bacteria, including Prevotella, and disease-linked microbial function. Imbalanced cytokines in patients were demonstrated to be associated with gut dysbiosis, which led us to suspect that B-CS is associated with gut microbiota and immune dysregulation. Next, 16S ribosomal DNA sequencing on fecal microbiota transplantation (FMT) mice models examined the link between gut dysbiosis and B-CS. FMT models showed damaged liver tissues, posterior inferior vena cava, and increased Prevotella in the disturbed gut microbiota of FMT mice. Notably, B-CS-FMT impaired the morphological structure of colonic tissues and increased intestinal permeability. Furthermore, a significant increase of the same cytokines (IL-5, IL-6, IL-9, IL-10, IL-17A, IL-17F, and IL-13) and endotoxin levels in B-CS-FMT mice were observed. Our study suggested that gut microbial dysbiosis may cause B-CS through immunological dysregulation.
OBJECTIVE: This study revealed that gut microbial dysbiosis may cause Budd-Chiari syndrome (B-CS). Gut dysbiosis enhanced intestinal permeability, and toxic metabolites and imbalanced cytokines activated the immune system. Consequently, the escalation of causative factors led to their concentration in the portal vein, thereby compromising both the liver parenchyma and outflow tract. Therefore, we proposed that gut microbial dysbiosis induced immune imbalance by chronic systemic inflammation, which contributed to the B-CS development. Furthermore, Prevotella may mediate inflammation development and immune imbalance, showing potential in B-CS pathogenesis.

Hepatic sinus obstruction syndrome (HSOS) is easy to be misdiagnosed or missed, and there is no unified and effective treatment for it. A patient was considered to have Budd-Chiari syndrome. He underwent a transjugular liver biopsy, and pathological examination revealed HSOS without liver cirrhosis. After the failure of anticoagulation therapy, he successfully received a transjugular intrahepatic portosystemic shunt (TIPS). After discharge, he was followed-up for four years with a good prognosis. G. segetum-induced HSOS can be easily overlooked, especially in patients with underlying liver diseases. When medical therapy fails, TIPS can control ascites and portal hypertension, and the long-term prognosis is optimistic.

OBJECTIVE: 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.
CONCLUSIONS: 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.

Acute liver failure is an uncommon presentation in the clinic. Common causes for acute liver failure include viral hepatitis and drug-related hepatotoxicity. However, acute liver failure due to Budd-Chiari syndrome is rare. This case highlights the importance of necessary constrast-enhanced imaging studies to rule out vascular etiologies of acute liver failure, in addition to common causes like viral or drug-induced hepatic failure. We present a case of a male Chinese patient who presented with nausea, vomiting, fatigue, and fever after eating a large amount of fatty food. Six days after hospitalization, the patient developed acute liver failure and hepatic encephalopathy. Contrast-enhanced computerized tomography and ultrasound examinations revealed thromboses in the hepatic veins and inferior vena cava. Further testing also showed decreased protein C activity. Therefore, a diagnosis of Budd-Chiari syndrome secondary to protein C deficiency was made. He received supportive care and a transjugular intrahepatic portal shunt. Hepatic function, coagulation panel results, and clinical presentations gradually returned to normal. Budd-Chiari syndrome from protein C deficiency could be a rare but valid cause of acute liver failure in Chinese patients.

Objective: To explore the risk factors of short-term prognosis of severe Budd-Chiari syndrome (BCS) patients,established and verified the nomogram prediction model for these BCS patients and evaluated its clinical application value. Methods: This study is a retrospective cohort study. The clinical data of 171 patients with severe BCS diagnosed were retrospectively analyzed in the Department of Hepatopancreatobiliary Surgery First Affiliated Hospital of Zhengzhou University from January 2018 to December 2023. There were 105 males and 66 females, aged (52.1±12.8) years (range: 18 to 79 years). The patients were divided into two groups based on whether they died within 28 days: the death group (n=38) and the survival group (n=133). The risk factors for short-term death of patients were analyzed,and independent risk factors were screened by univariate and multivariate analysis. Furthermore,these factors were used to establish the nomogram prediction model. The area under the curve(AUC),the Bootstrap Resampling,the Hosmer-Lemeshow test and the Decision Curve Analysis(DCA) were used to verify the model\'s differentiation,internal verification,calibration degree and clinical effectiveness,respectively. Results: Univariate and multivariate Logistics regression analysis showed that the history of hepatic encephalopathy,white blood cell,glomerular filtration rate and prothrombin time were independent risk factors (P<0.05). The above factors were used to successfully establish the prediction model with 0.908 of AUC and 0.895 of the internal verification of AUC,indicating that the predictive model was valuable. The 0.663 P-values in the Hosmer-Lemeshow test indicated the high calibration degree of the model. The clinical effectiveness of the model was proved by the 18% clinical benefit population using the DCA curve with the 17% probability threshold. Conclusions: The independent risk factors are the history of hepatic encephalopathy,white blood cell,glomerular filtration rate and prothrombin time. An adequate basis was acquired by establishing a nomogram prediction model of the short-term prognosis of severe BCS,which was helpful for early clinical screening and identification of high-risk patients with severe BCS who could die in the short term and timely providing timely intervention measures for improving the prognosis.
目的： 探讨重症巴德-吉亚利综合征患者的短期预后因素，建立重症巴德-吉亚利综合征患者短期预后的列线图预测模型并评价其临床应用价值。 方法： 本研究为回顾性队列研究。回顾性分析2018年1月至2023年12月郑州大学第一附属医院肝胆胰外科收治的171例重症巴德-吉亚利综合征患者的临床资料。其中男性105例，女性66例，年龄（52.1±12.8）岁（范围：18~79岁）。根据患者确诊后28 d内是否死亡，分为死亡组（38例）和生存组（133例）。分析患者短期死亡的危险因素，通过单因素及多因素Logistic回归分析筛选出重症巴德-吉亚利综合征患者短期死亡的独立危险因素，将这些因素纳入并建立列线图预测模型。通过受试者工作特征曲线的曲线下面积（AUC）验证模型区分度，并采用自举重采样法对其进行内部验证；通过Hosmer-Lemeshow检验验证模型的校准度；通过临床决策曲线（DCA）验证模型临床有效性。 结果： 单因素及多因素Logistic回归分析结果显示，肝性脑病史、白细胞计数、肾小球滤过率和凝血酶原时间是患者死亡的独立相关因素（P值均<0.05）。建立患者短期死亡的预测模型AUC为 0.908（内部验证AUC为 0.895），具有良好的区分度和校准度（P=0.663）。DCA中阈概率值设定为17%，人群临床净获益为18%，表明预测模型具有临床有效性。 结论： 肝性脑病史、白细胞计数、肾小球滤过率和凝血酶原时间是重症巴德-吉亚利综合征患者死亡的独立相关因素。重症巴德-吉亚利综合征短期预后列线图预测模型可以在临床早期筛选并识别重症巴德-吉亚利综合征短期死亡的高危患者。.

暂无摘要。

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking \"Budd-Chiari syndrome\" and \"hepatopulmonary syndrome\" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 μmol/L, direct bilirubin 14 μmol/L, and indirect bilirubin 39 μmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.
目的： 总结Budd-Chiari综合征合并肝肺综合征（HPS）患儿的临床特征及预后。 方法： 回顾性分析2016年12月郑州大学第一附属医院儿科收治的1例Budd-Chiari综合征合并HPS患儿的临床资料。以“Budd-Chiari综合征”“肝肺综合征”“Budd-Chiari syndrome”“hepatopulmonary syndrome”为关键词分别在中国知网、万方数据库、中国生物医学文献数据库、PubMed数据库进行检索（建库至2023年7月），结合本例资料，总结Budd-Chiari综合征合并HPS患儿（<18岁）的临床特征、诊疗经过及预后。 结果： 患儿，男，13岁，因“发绀、活动后胸闷6个月，皮肤黄染1周”入院。入院体格检查可见全身皮肤黏膜、巩膜轻度黄染，口唇、眼周、四肢末端发绀。辅助检查可见肝功能及动脉血气分析异常，总胆红素53 μmol/L，直接胆红素14 μmol/L，间接胆红素39 μmol/L；氧分压54 mmHg（1 mmHg=0.133 kPa），二氧化碳分压31 mmHg，肺泡动脉血氧梯度57 mmHg；腹部CT静脉造影示Budd-Chiari综合征（肝静脉型）、肝硬化、门静脉高压；增强经胸超声心动图造影（CE-TTE）阳性。患儿经对症支持治疗后院外氧疗，随访至2023年3月，低氧血症无明显改善，日常活动受限。文献复习符合检索条件中文文献0篇，英文文献3篇，结合本例患儿共4例，3例首诊原因为呼吸困难、发绀或低氧血症，1例不详。2例因呼吸系统症状就诊诊断Budd-Chiari综合征合并HPS，2例HPS分别发生于Budd-Chiari综合征确诊1.5和8.0年。CE-TTE阳性2例，肺灌注显像阳性2例。2例患儿行肝移植，呼吸功能恢复良好；吸氧治疗1例，低氧血症无改善；等待肝移植1例。 结论： Budd-Chiari综合征合并HPS早期起病隐匿，临床表现以呼吸困难、发绀多见，早期行CE-TTE明确肺内血管扩张可减少误诊。肝移植有助于改善预后。.

暂无摘要。

Budd-Chiari syndrome (BCS) and sinusoidal obstruction syndrome (SOS) are two major vascular disorders of the liver, of which both can cause portal hypertension related complications, but their locations of obstruction are different. BCS refers to the obstruction from the hepatic vein to the junction between the inferior vena cava and right atrium, which is the major etiology of post-sinusoidal portal hypertension; by comparison, SOS is characterized as the obstruction at the level of hepatic sinusoids and terminal venulae, which is a cause of sinusoidal portal hypertension. Both of them can cause hepatic congestion with life-threatening complications, especially acute liver failure and chronic portal hypertension, and share some similar features in terms of imaging and clinical presentations, but they have heterogeneous risk factors, management strategy, and prognosis. Herein, this paper reviews the current evidence and then summarizes the difference between primary BCS and SOS in terms of risk factors, clinical features, diagnosis, and treatment.