Research advances in the diagnosis and treatment of hepatic vein occlusion-type Budd-Chiari syndrome / 临床肝胆病杂志

Budd-Chiari syndrome is a rare liver vascular disease and can be classified into hepatic vein occlusion type， inferior vena cava occlusion type， and mixed type according to the location of hepatic outflow tract obstruction. This article reviews the hepatic vein occlusion type from the aspects of epidemiology， subtypes， clinical diagnosis， and treatment strategies.

Eficácia do TIPS em 10 anos na síndrome de Budd-Chiari: revisão sistemática e metanálise / Ten-year outcomes of tips for budd-chiari syndrome: systematic review and meta-analysis

ABSTRACT Background: Budd-Chiari syndrome (BCS) results from the obstruction of the hepatic venous flow, usually at the level of the hepatic vein or inferior vena cava. When left untreated, it can progress with several complications, including liver cirrhosis. Transjugular intrahepatic portosystemic shunt (TIPS) appears to be effective in a subgroup of BCS patients. Objective: To perform a systematic review and meta-analysis of TIPS effectiveness in BCS treatment, considering the survival rate, reduction in portosystemic pressure, need for liver transplantation, technical failure, and shunt dysfunction for up to 10 years of follow-up. Methods: We evaluated 17 studies published in PubMed, Science Direct, Web of Science, and SCOPUS databases, which used TIPS as a treatment for BCS, comprising 618 subjects between 18 and 78 years old. We assessed the bias risk by the NOS, NHI, and JBI scales for cohort stu­dies, before-after studies, and case series, respectively. We conducted the meta-analyses by extracting the number of events and the total patients evaluated to perform the proportion meta-analyses using the R software ("meta" package - version 4.9-6). Results: The pooled results (95%CI) showed a 19% (25.9-12.5%) rate of portosystemic pressure reduction, 6% (1-12%) rate for the need for liver transplants despite the use of TIPS, 2% (1-6%) technical failure rate, 30% (18-46%) shunt dysfunction rate, and 88% (81-93%) for the mean frequency of patients alive between 1 and 10 years after the procedure. We stratified survival rate and found an 86% (74-93%) prevalence of living subjects during less than five years, 92% (83-97%) at five years, and a 77% frequency (68-83%) of patients alive ten years after the TIPS placement. Conclusion: TIPS is an effective treatment for BCS, providing a high 10-year frequency of living patients and a significant decrease in portosystemic pressure. The need for liver transplants after TIPS and the technical failure rate is low.

RESUMO Contexto: A síndrome de Budd-Chiari (SBC) é resultante da obstrução do fluxo venoso hepático, usualmente no nível da veia hepática ou da veia cava inferior. Quando não tratada, pode evoluir com complicações, incluindo a cirrose hepática. O tratamento com TIPS parece ser eficaz em um subgrupo de pacientes. Objetivo: Realizar uma revisão sistemática e meta-análise da eficácia do TIPS no tratamento da síndrome de Budd-Chiari, considerando sobrevida, redução de pressão portossistêmica, necessidade de transplante hepático, falha técnica e disfunção no shunt em 10 anos de seguimento. Métodos: Foram avaliados 17 estudos publicados nas bases de dados PubMed, Science direct, Web of Science e SCOPUS, que utilizaram TIPS como tratamento da SBC, correspondendo a um total de 618 pacientes, entre 18 e 78 anos. O risco de viés foi avaliado pelas escalas de NOS, NHI e JBI, para estudos de coorte, antes e depois e séries de casos, respectivamente. As meta-análises foram conduzidas, extraindo o número de eventos e o total de pacientes avaliados para realizar as meta-análises de proporções usando o software R, com o pacote "meta" (versão 4.9-6). Resultados: Os resultados agrupados (IC de 95%) foram de 19% (25,9-12,5%) para a taxa de redução da pressão portossistêmica, 6% (1-12%) para a taxa de necessidade de transplantes hepáticos a despeito do uso do TIPS, 2% (1-6%) para a taxa de falha técnica, 30% (18-46%) para a taxa de disfunção do shunt e 88% (81-93%) para a sobrevida entre 1 e 10 anos depois do procedimento. Estratificando essa sobrevida, foi detectada 86% (74-93%) no período menor do que 5 anos, 92% (83-97%) com 5 anos, e 77% (68-83%) de frequência de pacientes vivos 10 anos após a realização do TIPS. Conclusão: O TIPS é um tratamento eficaz para a SBC, proporcionando elevada sobrevida em 10 anos e uma diminuição significativa da pressão portossistêmica. A necessidade de transplantes de fígado após TIPS e a taxa de falha técnica são baixas.

Rare Association of Budd-Chiari Syndrome with COVID-19: A Case Report

Introduction: Severe acute respiratory syndrome coronavirus type 2 (SARS-CoV-2) is a virus that can cause respiratory infections and pose a risk to patients' lives. While it primarily affects the airways, it can also lead to extrapulmonary clinical manifestations, such as hypercoagulable states, resulting in conditions like cerebrovascular disease, acute myocardial infarction, and, in rare cases, Budd-Chiari syndrome. Clinical case: This case involves a patient who was admitted to the emergency room with dyspnea on moderate exertion, progressive functional class deterioration, lower extremity edema, cough, fever, and weakness. The patient had a history of coronavirus disease 2019 (COVID-19) infection one month prior. Abdominal CT scan revealed subacute Budd-Chiari syndrome involving the middle sub-hepatic vein, along with right ventricle thrombosis, and a positive test for SARS-CoV-2. Conclusions: Budd-Chiari syndrome has multiple causes, primarily associated with hypercoagulable states or the presence of neoplasms that disrupt liver function or obstruct hepatic venous drainage. However, the understanding of the relationship between COVID-19 hypercoagulability and Budd-Chiari syndrome is still limited. Further research is needed to explore the heterogeneity of its pathogenesis in the context of the SARS-CoV-2 pandemic.

Introducción: el coronavirus del síndrome respiratorio agudo grave de tipo 2 (SARS-CoV-2) es un virus que puede causar infecciones respiratorias y poner en riesgo la vida de los pacientes. Aunque afecta principalmente las vías respiratorias, se pueden presentar manifestaciones clínicas extrapulmonares como estados de hipercoagulabilidad, lo que causa patologías como enfermedad cerebrovascular e infarto agudo de miocardio, y en casos menos frecuentes, síndrome de Budd-Chiari. Caso clínico: se presenta el caso de una paciente que ingresó al servicio de urgencias por disnea de medianos esfuerzos, deterioro progresivo de la clase funcional y edema en extremidades inferiores asociado con tos, fiebre y adinamia. Refirió el antecedente de infección por enfermedad por coronavirus de 2019 (COVID-19) un mes antes y la tomografía de abdomen contrastada reveló síndrome de Budd-Chiari subagudo de la vena subhepática media asociada a trombosis del ventrículo derecho con prueba positiva para SARS-CoV-2. Conclusiones: El síndrome de Budd-Chiari es de origen multicausal, principalmente por estados de hipercoagulabilidad o presencia de neoplasias que ocasionan disrupción de la función del hígado o causan la ocupación de espacio sobre el drenaje venoso hepático; sin embargo, en el contexto de la pandemia de SARS CoV-2, aún se conoce muy poco la heterogeneidad de su patogénesis, como es la hipercoagulabilidad de la COVID-19 y el síndrome de Budd-Chiari, por lo que inferimos que están altamente relacionados.

Transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation for Budd-Chiari syndrome complicated with liver cancer: a case report with surgical video / 器官移植

Objective To summarize clinical experience of transabdominal pericardial anastomosis of suprahepatic vena cava of the donor and right atrium of the recipient in liver transplantation for Budd-Chiari syndrome (BCS) complicated with liver cancer. Methods Clinical data of a BCS patient complicated with liver cancer undergoing transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation were retrospectively analyzed. Results The hepatic vein and suprahepatic vena cava were partially occluded in the patient. Liver transplantation was completed by transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium with beating-heart. In addition, due to pathological changes of the recipient's hepatic artery, splenic artery of the recipient was cut off, distal ligation was performed, and the proximal end was reversed and anastomosed with the common hepatic artery of the donor liver, and the reconstruction of hepatic artery was completed. The surgery was successfully performed. At approximately postoperative 1 week, the function of the liver allograft was gradually restored to normal, and no major complications occurred. The patient was discharged at postoperative 25 d. No signs of BCS recurrence was reported after 8-month follow-up. Conclusions It is safe and feasible to treat BCS by liver transplantation with transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium. BCS patients complicated with liver cancer obtain favorable prognosis.

Discussion of issues related to Budd-Chiari syndrome / 国际外科学杂志

Budd-Chiari syndrome (B-CS) is a rare disease caused by hepatic vein outflow obstruction, and its etiology is complex and inconclusive. Current studies suggest that vascular dysplasia, gut microbiota and trace element imbalance may be related to the pathogenesis of B-CS, and the development of high-throughput sequencing technology may help to clarify the exact pathogenesis of B-CS. The symptoms of B-CS are not specific and rely mainly on imaging methods to establish the diagnosis, so there is an urgent need to find new noninvasive biological diagnostic markers. In addition, there are many pathological types and different criteria of B-CS, which mostly can′t fully reflect the pathophysiological changes of B-CS patients and guide clinical treatment. Therefore, we recommend pathophysiological classification according to the hemodynamic changes and collateral circulation compensation of B-CS, and then develop personalized treatment strategies for stratified management different from the traditional early diagnosis and treatment protocols. This article summarizes and discusses the above contents.

The application of liver shear wave velocity and its correlation with portal vein pressure in evaluating the efficacy of interventional treatment for Budd-Chiari syndrome / 介入放射学杂志

Objective To discuss the application value of liver shear wave velocity(SWV)and its correlation with portal vein pressure in evaluating the efficacy of interventional therapy for Budd-Chiari syndrome(BCS).Methods The clinical data of 40 BCS patients,who were admitted to the Affiliated Hospital of Xuzhou Medical University of China to receive treatment between April 2020 and April 2022,were collected.During interventional procedure,the hepatic venous pressure gradient(HVPG)was determined separately before and after recanalization of the treated vessels,the liver SWV was determined at one day before,2 days,1 month and 3 months after the treatment,and the above indexes were statistically analyzed.Results Successful initial interventional therapy was accomplished in all patients.The preoperative one-day,postoperative 2-day,one-month and 3-month mean liver SWV values were(2.34±0.36)m/s,(1.74±0.36)m/s,(1.62±0.30)m/s,and(1.56±0.28)m/s respectively.The differences in the mean liver SWV between its preoperative value and its postoperative 2-day,one-month and 3-month value were statistically significant(all P＜0.05),and statistically significant difference in the mean liver SWV also existed between postoperative 2-day value and postoperative 3-month value(P＜0.05).The mean HVPG decreased from preoperative(15.19±2.35)mmHg(1 mmHg=0.133 kPa)to postoperative(6.44±1.34)mmHg(P＜0.05).The preoperative one-day liver SWV was positively correlated with preoperative HVPG(r=0.803,P＜0.01).The postoperative 2-day liver SWV also carried a positive correlation with the postoperative HVPG(r=0.844,P＜0.01).The difference value(D-value)between preoperative liver SWV value and postoperative 2-day liver SWV value was(0.59±0.27)m/s,and the D-value between preoperative HVPG value and postoperative HVPG value was(8.75±1.92)mmHg,and a positive correlation existed between the above two D-values(r=0.676,P＜0.01).Conclusion There is a good correlation between liver SWV and HVPG,which can be used to evaluate the postoperative efficacy of BCS patients after receiving interventional therapy.(J Intervent Radiol,2023,32:1178-1183)

Research progress in hepaticalveolar echinococcosis leading to cirrhosis / 中国人兽共患病学报

Alveolar echinococcosis is a zoonotic parasitic disease caused by an infection with Echinococcosis multilocularis.The liver is the primary organ of alveolar echinococcosis.Alveolar echinococcosis is usually characterized by invasive growth and consequently iscalled"parasitic cancer."Resection of radical lesions is a preferred and effective treatment for hepatic alveolar echinococcosis.End-stage hepatic alveolar echinococcosis often occurs with parasiticcirrhosis,such as secondary biliary cirrhosis,congestive liver cirrhosis or Budd-Chiari syndrome.Few studies have examined hepatic multilocular echinococcosis leading to cirrhosis.This article reviews the aspects of hepatic alveolar echinococcosis involving the invasion of important blood vessels and bile ducts,thereby leading to secondary biliary cirrhosis and congestive liver cirrhosis caused by hepatic alveolar echinococcosis.

Diagnosis and treatment strategy of Budd-Chiari syndrome complicated with liver cirrhosis and hepatocellular carcinoma:an analysis of 42 cases / 肿瘤

Objective:To investigate the diagnosis and treatment strategies as well as prognostic factors of Budd-Chiari syndrome(B-CS)patients complicated with liver cirrhosis and hepatocellular carcinoma(HCC). Methods:Clinical data of 42 B-CS patients complicated with HCC admitted to Department of Hepatopancreatobiliary Surgery,the First Affiliated Hospital of Zhengzhou University from January 2014 to December 2020 were retrospectively analyzed,and the association between the clinical characteristics of patients and whether they had undergone B-CS treatment or not before HCC diagnosis was analyzed.Kaplan-Meier method was used to plot the survival curve of the patients.COX regression model was used to analyze the risk factors affecting the prognosis of B-CS patients complicated with HCC. Results:All 42 B-CS patients complicated with HCC had liver cirrhosis,their median survival time was 28 months,and the 1-,3-and 5-year survival rates were 76.2％,50.0％and 42.9％,respectively.The maximum tumor diameter,multiple tumor ratio and total bilirubin level in patients who had not received B-CS treatment before HCC diagnosis were higher than those in patients who had.Serum albumin level(hazard ratio:0.866,95％confidence interval:0.771-0.972,P=0.015)and not receiving B-CS treatment before HCC diagnosis(hazard ratio:2.796,95％confidence interval:1.020-7.666,P=0.046)were independent risk factors for the prognosis of B-CS patients complicated with HCC. Conclusion:The prognosis of B-CS patients complicated with HCC is relatively good.Serum albumin level and not receiving B-CS treatment before HCC diagnosis are independent risk factors for the prognosis of B-CS patients complicated with HCC.

Alternativas de manejo intervencionista en el síndrome de Budd-Chiari. Serie de casos y revisión de la literatura / Interventional management alternatives in Budd-Chiari syndrome. Series of cases and review of the literature

La obstrucción en el tracto de salida del flujo venoso hepático, también conocida como síndrome de Budd-Chiari, es una condición infrecuente que causa congestión hepática, hipertensión portal, ne-crosis de los hepatocitos y, eventualmente, falla hepática aguda o crónica. Actualmente, el manejo de esta condición representa un reto para el médico, quien debe estar preparado para determinar la mejor alternativa entre las diferentes opciones terapéuticas disponibles. Este artículo pretende ilus-trar las alternativas del manejo intervencionista de esta enfermedad, a través de una serie de casos de pacientes tratados en el servicio de Radiología Intervencionista de un hospital de referencia de la ciudad de Medellín, entre 2011 y 2017.

Hepatic venous outflow tract obstruction, also known as Budd-Chiari syndrome, is a rare condition that causes hepatic congestion, portal hypertension, hepatocyte necrosis and eventually acute or chronic liver failure. Currently, the management of this condition represents a challenge for the physi-cian, who must be prepared to determine the best alternative among the different therapeutic options available. This article aims to illustrate the alternatives of interventional management of this disease, through a series of cases of patients treated in the Interventional Radiology service of a referral hos-pital in the city of Medellin, between 2011 and 2017.

Síndrome de Budd-Chiari secundario asociado a hepatitis alcohólica grave: reporte de caso / Secondary Budd-Chiari syndrome associated with severe alcoholic hepatitis: A case report

Resumen La hepatitis alcohólica es la inflamación aguda del hígado secundaria al consumo de alcohol en cantidades hepatotóxicas, su fisiopatología está influida por diversos factores asociados. El diagnóstico tiene tres escenarios: la hepatitis alcohólica probable, posible y definitiva. La hepatitis alcohólica probable se basa en el cumplimiento de criterios diagnósticos clínicos y laboratoriales; la hepatitis alcohólica posible corresponde a casos de cumplimiento de estos criterios, pero con presencia de factores potencialmente confusores del diagnóstico, y la definitiva se sustenta sobre una base histopatológica. El síndrome de Budd-Chiari es una entidad poco frecuente, heterogénea y potencialmente letal, caracterizada por la presencia de trombos a nivel de las venas suprahepáticas, así como de la vena cava inferior. El diagnóstico definitivo está basado en pruebas de imagen. Por su parte, el síndrome de Budd-Chiari secundario es una entidad aún más infrecuente, poco estudiada, cuyo diagnóstico es difícil debido a su gran similitud al síndrome de Budd-Chiari primario, tanto clínica como en imágenes diagnósticas, por lo que se tiene que acudir a métodos diagnósticos de mayor complejidad e incluso invasivos. A continuación, se presenta un caso inusual de un paciente con consumo crónico de alcohol que presenta síndrome de Budd-Chiari secundario asociado a hepatitis alcohólica grave confirmada mediante biopsia hepática y sometido a múltiples estudios de imagen que descartaron la presencia de trombosis a nivel de las venas suprahepáticas.

Abstract Alcoholic hepatitis is an acute inflammation of the liver secondary to the consumption of alcohol in hepatotoxic amounts; various associated factors influence its pathophysiology. The diagnosis has three scenarios: probable, possible, and definite alcoholic hepatitis. Probable alcoholic hepatitis is based on compliance with clinical and laboratory diagnostic criteria; possible alcoholic hepatitis corresponds to cases that meet these criteria but with potentially confounding factors in the diagnosis, and the definite one is based on histopathology. Budd-Chiari syndrome is a rare, heterogeneous, potentially lethal entity characterized by thrombi in the suprahepatic veins and the inferior vena cava. The final diagnosis relies on imaging tests. Moreover, secondary Budd-Chiari syndrome is an even rarer entity, little studied, whose diagnosis is difficult due to its remarkable similarity to primary Budd-Chiari syndrome, both clinically and in diagnostic images, for which more complex and even invasive diagnostic methods must be used. Then, we describe an unusual case of a male patient with chronic alcohol consumption presenting with secondary Budd-Chiari syndrome associated with severe alcoholic hepatitis confirmed by liver biopsy and subjected to multiple imaging studies that ruled out thrombosis in the suprahepatic veins.

A case report of Budd-Chiari syndrome with cardiac complications and low SPO2 successfully managed by an integrated-approach

Budd-Chiari syndrome [BCS] is a rare, potentially life-threatening condition characterized by the triad of abdominal pain, ascites, and hepatomegaly (with or without jaundice). There will be an underlying disorder in most cases. The diagnosis, as well as management of the case, requires a multidisciplinary approach. The treatment should aim at reducing the presenting symptoms as well as removing the underlying pathology. Here we explain a case report of a 21-year-old male patient in the subacute stage of BCS with its complications and considered as a candidate for liver transplantation. His liver enzymes, PT/INR, D- dimer, and homocysteine values were above normal levels. The arterial oxygen saturation level was subnormal, and he was on supportive oxygen supplement. Inferior venacava [IVC] Doppler revealed a non-obstructive intrahepatic thrombus. The patient was treated with Homoeopathic medicine Arsenicum album and Arnica montana, given as an adjuvant to conventional treatment.

Síndrome de Budd Chiari secundario al síndrome antifosfolipídico y lupus eritematoso sistémico en una adolescente: reporte de un caso

El síndrome de Budd-Chiari es causado por una obstrucción del flujo sanguíneo del hígado. Los casos publicados del síndrome antifosfolípido asociado a SBC son limitados en la población pediátrica. Reportamos el caso de una adolescente de 15 años que presentó fiebre, ascitis y hepatoesplenomegalia. En la ecografía Doppler hepática se observó ausencia de flujo en la vena hepática derecha y media, y en la vena cava inferior. En la tomografía abdominal se observó una extensa trombosis de la vena cava inferior. Durante la hospitalización se le diagnosticó SAF y lupus eritematoso sistémico. Se le administró tratamiento con heparina no fraccionada, heparina de bajo peso molecular y anticoagulantes. El síndrome de Budd-Chiari secundario al síndrome antifosfolípido es una enfermedad potencialmente mortal. El diagnóstico y el tratamiento oportunos permiten mejorar la calidad de vida del paciente.

Budd-Chiari syndrome is caused by an obstruction of blood flow to the liver. Published cases of the antiphospholipid syndrome associated with BCS are limited in the pediatric population. We report a 15-year-old adolescent who presented with fever, ascites, and hepatosplenomegaly. Hepatic Doppler ultrasound revealed no flow in the right and middle hepatic veins and in the inferior vena cava. Abdominal tomography revealed extensive thrombosis of the inferior vena cava. During hospitalization, she was diagnosed with antiphospholipid syndrome and systemic lupus erythematosus. She was given treatment with unfractionated heparin, low molecular weight heparin, and anticoagulants. Budd-Chiari syndrome secondary to the antiphospholipid syndrome is a life-threatening disease. Timely diagnosis and treatment improve the quality of life of the patient.

Post-COVID Pediatric Budd-Chiari Syndrome

Introduction: Budd-Chiari syndrome (BCS) is rare in children. Underlying etiologies, natural history and management differ in children and adults. Treatment options like liver transplantation and transjugular intrahepatic portosystemic shunt have also been less well-studied in children. Presented here is the case of a male child with BCS after coronavirus disease 2019 (COVID-19) infection. Case presentation: A 3-year-old male child presented with complaints of gradually increasing abdominal distension, constipation, decreased urine output, visible veins over abdomen and swelling in lower limbs and periorbital edema since last 15 days. He was diagnosed as BCS after multiple imaging investigations, including triple phase contrast-enhanced computed tomography (CECT) abdomen. His COVID antibody tested positive. Conclusion: Practice guidelines for children with BCS should be formulated, expert group recommendations should be reviewed and a consensus statement should be issued. Underlying etiology remains obscure despite extensive work-up in most of the children. Association of COVID-19 with BCS may be incidental but should be studied further as COVID is known to cause thrombotic complications

Impact of left hepatic vein classification on hepatic vein reconstruction and prognosis after pediatric living-donor liver transplantation using left lateral liver segments / 中华肝胆外科杂志

Objective:To study the impact of donor left hepatic vein classification and the reconstruction methods on hepatic venous outflow obstruction (HVOO) after pediatric living-donor liver transplantation using left lateral liver segments.Methods:A retrospective study was performed on the clinical data of 653 children recipients who underwent living-donor liver transplantation with left lateral liver segments from January 2014 to December 2020 at Tianjin First Central Hospital. There were 309 males and 344 females, aged 7.0 (6.0, 10.0) months, with an age range of 3-121 months. Based on the left hepatic vein on preoperative donor enhancement CT as well as the intraoperative reconstruction methods, the recipients were divided into 3 groups: type Ⅰ group ( n=514), anastomosis using a single opening was performed directly between the donor and the recipient; type Ⅱ group ( n=118), angioplasty was performed on two adjacent recipient venous orifices before anastomosis, and type Ⅲ group ( n=21), an interposition vessel was anastomosed to two widely spaced openings or the two veins were anastomosed separately. The preoperative general status of the patient, postoperative HVOO incidences, and graft and recipient survival rates were compared among the three groups. The patients were followed up by outpatient reexamination or telephone. Results:Graft to recipient weight ratio in the type Ⅲ group was smaller than that in the type Ⅰ group and the type Ⅱ group ( P<0.05). For all the 653 patients, the incidence of postoperative HVOO was 4.59% (30/653), with the incidences of HVOO in the 3 groups of patients were 4.1% for the type Ⅰ group (21/514), 5.1% for the type Ⅱ group (6/118), and 14.3% for the type Ⅲ group (3/21), respectively. There was no significant difference among the groups ( P>0.05). The recipient cumulative survival rates at 1 and 3 years after surgery in the type I group were 97.8% and 97.0%, and the corresponding rates in the type Ⅱ group were 96.5% and 94.2%, and in the type Ⅲ group were 94.1% and 86.9%, respectively. There was a significant difference between the type Ⅰ and type Ⅲ groups ( P=0.048). The graft cumulative survival rates at 1 and 3 years in the type Ⅰ group were 97.4% and 96.9%, and the corresponding rates in the type Ⅱ group were 94.9% and 92.5%, and in the type Ⅲ group were 94.1% and 86.9%, respectively. The difference in the postoperative graft cumulative survival rates between the type Ⅰ group and type Ⅱ group was significant ( P=0.044). Conclusions:The anatomy of the left hepatic vein supplying the left lateral liver segment was highly variable, and the majority of the variations could be reconstructed. A reasonable reconstructive method could reduce the incidence of postoperative HVOO and improved the outcomes of the graft.

Analysis of risk factors of Budd-Chiari syndrome associated with hepatocellular carcinoma in patients who underwent endovascular recanalization / 中华肝胆外科杂志

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.

Study on the mechanism of mTOR/HIF-1α signaling pathway in Budd-Chiari syndrome liver fibrosis / 国际外科学杂志

Objective:To explore the mechanism of mTOR/HIF-1α signaling pathway in Budd-Chiari syndrome (B-CS) liver fibrosis.Methods:Twenty male C57 mice were randomly divided into Sham operation group (Sham), sham operation+ rapamycin (Sham+ Ra) group, B-CS group, B-CS+ rapamycin (B-CS+ Ra) Group, 5 in each group. The B-CS mouse model was constructed by partial ligation of the inferior vena cava(IVC) at the posterior segment of the liver; IVC was not ligated in the Sham group. Mice in Sham+ Ra and B-CS+ Ra groups were intraperitoneally injected with rapamycin (2 mg/kg, 5% DMSO solution preparation) every other day, Sham group and B-CS group were injected with the same dose of 5% DMSO solution.After 6 weeks, samples were taken, and part of the liver tissue was used to make paraffin sections for hematoxylin-eosin (HE) and Sirus Red staining to observe the pathological changes, and immunohistochemical staining to detect the expression of α-SMA and Fibrinogen in liver tissues; Protein and RNA were extracted from fresh liver tissue, and Western-blot was used to detect α-SMA, Fibrinogen, p-mTOR, mTOR, HIF-1α, Collagen I, and VEGF protein levels. Real-time fluorescent quantitative PCR was used to detect mTOR, HIF-1α, CollagenⅠ, VEGF mRNA levels.Measurement data were expressed as mean±standard deviation ( ± s), and the comparison between groups was performed by one-way ANOVA test. Results:The results of pathological staining showed that in the B-CS group, there was severe congestion around the central vein of the liver and sinusoids, widening of the sinus space, and increased collagen deposition, indicating that this study successfully established a mouse B-CS liver fibrosis model. The expression levels of fibrosis indicators α-SMA and Collagen I protein, mTOR pathway related indicators p-mTOR and HIF-1α protein, and microthrombus indicator Fibrinogen protein in the Sham group were 0.027±0.012, 0.337±0.008, 0.138±0.024, 0.296±0.113, 0.733±0.192; B-CS group were 0.986±0.001, 0.927±0.055, 0.936±0.044, 1.693±0.443, 1.612±0.068, and the differences were statistically significant ( P<0.05). The expression levels of B-CS+ Ra group were 0.707±0.078, 0.311±0.024, 0.332±0.094, 0.254±0.117, 0.569±0.075, which were statistically significant compared with B-CS group ( P<0.05). Conclusions:The mTOR/HIF-1α signaling pathway is significantly activated in mouse B-CS liver fibrosis. This pathway may participate in the development of liver fibrosis by regulating microthrombosis.

Effect of metformin on liver fibrosis in a mouse model of Budd-Chiari syndrome / 临床肝胆病杂志

Objective To investigate the effect of metformin on liver fibrosis in a mouse model of Budd-Chiari syndrome and its mechanism. Methods A total of 30 male C57 mice were randomly divided into sham-operation group (SHAM group) with 6 mice, sham operation+ metformin group (SHAM+M group) with 5 mice, Budd-Chiari model group (BCS group) with 10 mice, and Budd-Chiari model+metformin group (BCS+M group) with 9 mice. The mice in the model group were treated with partial ligation of the inferior vena cava, those in the SHAM group were not treated with ligation, and those in the metformin group were given 0.1% metformin in drinking water besides modeling. The mice were sacrificed after 6 weeks. HE staining and picrosirius red staining were used to observe liver histopathology and collagen deposition; immunohistochemistry was used to measure the expressions of α-smooth muscle actin (α-SMA) and fibrinogen; quantitative real-time PCR was used to measure the mRNA expression of hypoxia-inducible factor 1α (HIF-1α) and type Ⅰ collagen (collagen 1), and Western blot was used to measure the relative protein expression levels of HIF-1α, vascular endothelial growth factor (VEGF), fibrinogen, α-SMA, and collagen 1. A one-way analysis of variance was used for comparison of continuous data between multiple groups, and the least significant difference t -test was used for further comparison between two groups. Results Pathological staining showed that compared with the SHAM group, the BCS group had significant liver fibrosis, disordered arrangement of hepatocytes near the central vein, sinusoidal expansion with red blood cell deposition and a small amount of inflammatory cell infiltration, and collagen deposition. The BCS group had significant increases in the mRNA expression levels of HIF-1α and collagen 1 and the protein expression levels of α-SMA, collagen 1, HIF-1α, VEGF, and fibrinogen (all P < 0.05); compared with the BCS group, the BCS+M group had significant alleviation of liver fibrosis, red blood cell deposition, and collagen deposition and significant reductions in the mRNA expression levels of HIF-1α and collagen 1 and the protein expression levels of α-SMA, collagen 1, HIF-1α, VEGF, and fibrinogen (all P < 0.05). Conclusion Metformin can improve congestive liver fibrosis caused by Budd-Chiari syndrome, possibly by reducing microthrombus in hepatic sinusoid and inhibiting the HIF-1α/VEGF pathway.

Platelet-albumin-bilirubin score in assessing short-term prognosis of patients with Budd-Chiari syndrome presenting with upper gastrointestinal bleeding / 中华肝胆外科杂志

Objective:To study the factors influencing short-term prognosis of patients with Budd-Chiari syndrome (B-CS) presenting with upper gastrointestinal bleeding and to assess the predictive value of platelet-albumin-bilirubin score (PALBI) on death within 30 d in these patients.Methods:A retrospective study was conducted on 74 patients with B-CS who presented with upper gastrointestinal bleeding and were treated at the First Affiliated Hospital of Zhengzhou University from January 2014 to February 2020. There were 51 males and 23 females, with age of (46.5±11.1) years old. These patients were divided into the survival group ( n=58) and the death group ( n=16) according to the disease outcomes up to 30 d of follow-up. Factors influencing short-term deaths of these patients were analyzed, and the predictive values of PALBI, ALBI, CTP and MELD scores on short-term prognosis of the patients were assessed. The receiver operating characteristic (ROC) curves were plotted, and the areas under the curve (AUC) were calculated and compared. Results:The differences between patients in the survival and death groups for white blood cell, platelet, PALBI score, PALBI classification, ALBI score, CTP score, MELD score, and presence or absence of hepatic encephalopathy were significantly different (all P<0.05). Multivariate logistic regression analysis showed that CTP score≥10 or CTP grade C ( OR=1.669, 95% CI: 1.048-2.661), and PALBI score >-2.09 or PALBI grade 3 ( OR=5.245, 95% CI: 2.128-12.924) were independent risk factors for predicting death within 30 days. The areas under the ROC curves for PALBI, ALBI, CTP and MELD score were 0.89, 0.72, 0.77 and 0.76, with the cut-off values of -1.92, -1.60, 8.50 and 13.60, respectively. The differences between the PALBI score and ALBI, CTP scores were significantly different ( P<0.05). Conclusion:The PALBI score showed a positive predictive value on short-term prognostic assessment of patients with B-CS presenting with upper gastrointestinal bleeding. It was comparable to the effect of the MELD score but was significantly better than the ALBI and CTP scores.

Síndrome de Budd-Chiari por obstrução da via de saída da veia hepática por membrana: tratamento percutâneo por angioplastia com balão / Budd-Chiari syndrome due to hepatic venous web outflow obstruction: percutaneous treatment with balloon angioplasty

Resumo A síndrome de Budd-Chiari é uma doença venosa hepática rara, mais incidente em adultos jovens, podendo se apresentar na forma aguda, subaguda ou crônica, o que resulta em hipertensão portal. O tratamento tradicional consiste em técnicas de trombólise e de shunts portossistêmicos intra-hepáticos, como pontes para o transplante hepático. Recentemente, técnicas de angioplastia com balão ou stents têm sido relatadas para o tratamento dessa afecção. Neste artigo, é relatado e discutido um caso de síndrome de Budd-Chiari por obstrução membranosa da via de saída da veia supra-hepática com trombose da veia hepática média em uma paciente de 24 anos. O tratamento estabelecido foi a angioplastia transjugular com balão, que obteve resultados satisfatórios e boa evolução clínica.

Abstract The Budd-Chiari syndrome is a rare hepatic venous disease. It is more prevalent in young adults and may present in acute, subacute, or chronic forms, causing portal hypertension. Traditional treatment consists of thrombolysis techniques and transjugular intrahepatic portosystemic shunt, as a bridge to liver transplantation. Recently, use of balloon or stent angioplasty techniques has been reported for treatment of this condition. In this article, we report and discuss a case of BCS by membranous obstruction in the hepatic vein outflow tract, with middle hepatic vein thrombosis, in a 24-year-old patient. The treatment chosen and employed was transjugular balloon angioplasty, which achieved satisfactory results and good clinical evolution.

Effect of hepatic volume change on clinical practice in patients with extensive hepatic vein occluded Budd-Chiari Syndrome treated with TIPS / 中华肝胆外科杂志

Objective:To investigate the changes of liver volume and liver function in patients with extensive hepatic vein occluded Budd-Chiari syndrome (BCS) treated with transjugular intrahepatic portosystem shunt (TIPS).Methods:The clinical data of 29 BCS patients from Affiliated Hospital of Xuzhou Medical University during March 2016 to June 2019 were retrospectively collected and analyzed. The BCS was caused by extensive hepatic vein occlusion and patients were treated with TIPS. Pre- and postoperative abdominal CT/MRI images were collected and analyzed, and hepatic volume was measured with 3D-reconstruction. The liver volume and liver function during before and post the surgery were also collected and analyzed with preoperative value.Results:Patients including 8 males and 21 females, aged (33.3±6.3) years, were enrolled in this study. TIPS was successfully performed in all patients, with a technical success rate 100%. No serious complications related to TIPS occurred. Patients were followed up for 12-33 months (median, 16 months). Compared with preoperative [(2 124.6±420.9) cm 3] , the hepatic volume of time points after operation [1 week: (1 926.3±372.3) cm 3; 3 months: (1 480.6±183.1) cm 3; 6 months: (1 461.9±153.0) cm 3; 12 months: (1 469.3±148.5) cm 3] were all significantly reduced, and the differences were statistically significant ( P<0.05). Compared with preoperative values, the hepatic function indexes at each time point after operation were significantly improved ( P<0.05). The complete remission rate of ascites was 96.4% (27/28), 100.0% (28/28) and 100.0% (28/28) at 3, 6 and 12 months, respectively. Conclusion:The extensive hepatic vein occlusive BCS patients were benefit from TIPS therapy. Six months after operation, the hepatic volume and the hepatic function returned to normal level.