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Chacko J, Haward R, Shah NA, Pathmanathan SV, Sundaramoorthy B, Tukaram JH. A Rare Case of Budd-Chiari Syndrome in a Young Female. Clin Case Rep 2025; 13:e70478. [PMID: 40321226 PMCID: PMC12050134 DOI: 10.1002/ccr3.70478] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/01/2024] [Revised: 02/08/2025] [Accepted: 02/24/2025] [Indexed: 05/08/2025] Open
Abstract
Budd-Chiari syndrome is a rare disorder characterized by hepatic vein obstruction, with an incidence of 1 in 100,000. It can be diagnosed through imaging studies and initially managed with anticoagulants. Acute Budd-Chiari syndrome can be treated with thrombolysis to dissolve blood clots obstructing hepatic veins. Venous obstructions in Budd-Chiari syndrome can be fixed through angioplasty or venous stenting to restore blood flow. Liver decompression can be achieved with TIPS, and severe cases may require liver transplantation.
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Affiliation(s)
- Joshua Chacko
- Father Muller Medical CollegeGeneral MedicineBangaloreIndia
| | - Raymond Haward
- Vydehi Institute of Medical Sciences and Research CentreBangaloreIndia
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Martins PL, Silva MJ, Bilhim T, Ribeiro R. Budd-Chiari syndrome in a patient with antiphospholipid syndrome: hiding in plain sight. BMJ Case Rep 2025; 18:e259746. [PMID: 40262925 DOI: 10.1136/bcr-2024-259746] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 04/24/2025] Open
Abstract
We describe the case of a woman in her 40s on lifelong anticoagulation with warfarin due to antiphospholipid syndrome presenting with acute right upper quadrant pain. Liver tests were only slightly elevated, and non-invasive imaging such as Doppler ultrasound and contrast-enhanced CT were non-diagnostic. As suspicion for a thrombotic complication persisted, repeat CT imaging ultimately confirmed the diagnosis of Budd-Chiari syndrome (BCS), and angiography was performed allowing for effective treatment with balloon angioplasty. This case highlights both the need for a high degree of clinical suspicion for BCS in patients with prothrombotic features (even those on anticoagulation) and also the diagnostic and therapeutic potential of interventional radiology in selected cases.
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Affiliation(s)
- Pedro Lages Martins
- Gastroenterology Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
| | - Mário Jorge Silva
- Gastroenterology Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
- NOVA Medical School, Lisboa, Portugal
| | - Tiago Bilhim
- Interventional Radiology Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
| | - Rita Ribeiro
- Internal Medicine Department, Centro Hospitalar Universitario de Lisboa Central EPE, Lisboa, Portugal
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Zhang JW, Zhang N, Lyu Y, Zhang XF. Influence of Sex in the Development of Liver Diseases. Semin Liver Dis 2025; 45:15-32. [PMID: 39809453 DOI: 10.1055/a-2516-0261] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/16/2025]
Abstract
The liver is a sexually dimorphic organ. Sex differences in prevalence, progression, prognosis, and treatment prevail in most liver diseases, and the mechanism of how liver diseases act differently among male versus female patients has not been fully elucidated. Biological sex differences in normal physiology and disease arise principally from sex hormones and/or sex chromosomes. Sex hormones contribute to the development and progression of most liver diseases, with estrogen- and androgen-mediated signaling pathways mechanistically involved. In addition, genetic factors in sex chromosomes have recently been found to contribute to the sex disparity of many liver diseases, which might explain, to some extent, the difference in gene expression pattern, immune response, and xenobiotic metabolism between men and women. Although increasing evidence suggests that sex is one of the most important modulators of disease prevalence and outcomes, at present, basic and clinical studies have long been sex unbalanced, with female subjects underestimated. As such, this review focuses on sex disparities of liver diseases and summarizes the current understanding of sex-specific mechanisms, including sex hormones, sex chromosomes, etc. We anticipate that understanding sex-specific pathogenesis will aid in promoting personalized therapies for liver disease among male versus female patients.
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Affiliation(s)
- Jie-Wen Zhang
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- Institute of Advanced Surgical Technology and Engineering, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- National-Local Joint Engineering Research Center for Precision Surgery and Regenerative Medicine, Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
| | - Nan Zhang
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- Institute of Advanced Surgical Technology and Engineering, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- National-Local Joint Engineering Research Center for Precision Surgery and Regenerative Medicine, Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
| | - Yi Lyu
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- Institute of Advanced Surgical Technology and Engineering, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- National-Local Joint Engineering Research Center for Precision Surgery and Regenerative Medicine, Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
| | - Xu-Feng Zhang
- Department of Hepatobiliary Surgery, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- Institute of Advanced Surgical Technology and Engineering, The First Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
- National-Local Joint Engineering Research Center for Precision Surgery and Regenerative Medicine, Xi'an Jiaotong University, Xi'an, Shaanxi Province, People's Republic of China
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Tran TT, Truong TYN, Nguyen HVK, Luu NAT, Than TT, Bui HT, Ho PD, Pirotte T, Reding R. Liver Transplantation for Children With Budd-Chiari Syndrome: A Case Report From Vietnam and Literature Review. Pediatr Transplant 2025; 29:e70038. [PMID: 39900470 DOI: 10.1111/petr.70038] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/04/2024] [Revised: 01/10/2025] [Accepted: 01/18/2025] [Indexed: 02/05/2025]
Abstract
Budd-Chiari syndrome (BCS) presents with various degrees of liver damage, and the choice of treatment depends on the type and extent of hepatic injury. Liver transplantation (LT) is considered as the final treatment option when other interventions are not feasible and when the liver injury is irreversible. We report a case of a pediatric patient with BCS who underwent liver transplantation from a living donor in the context of thrombophilic disorder. CASE PRESENTATION A 14-month-old girl was admitted to the hospital with ascites. She was malnourished, and an abdominal CT scan confirmed significant ascites with no visualization of the hepatic veins and retrohepatic inferior vena cava (IVC). A liver biopsy revealed fibrosis, necrosis, and parenchymal hemorrhage. Patient's portal hypertension was managed with prophylactic beta-blocker Propranolol and endoscopic esophageal variceal ligation. However, she was hospitalized four times due to gastrointestinal bleeding from ruptured esophageal varices. Protein C deficiency was found as probable etiology of BCS. The patient underwent liver transplantation at 3 years and 8 months old with a liver from a parental living donor. The surgery and postoperative course were uneventful, and the patient was discharged 25 days after the transplant. CLINICAL DISCUSSION Hypercoagulability is often the underlying cause of BCS. Maintaining anticoagulation/thrombophilic balance postoperatively contributed to the successful liver transplantation in this pediatric patient. CONCLUSION Liver transplantation is a safe and effective treatment for pediatric patients with BCS who meet the criteria for the procedure.
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Affiliation(s)
- Thanh Tri Tran
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Thi Yen Nhi Truong
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Hong Van Khanh Nguyen
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Nguyen An Thuan Luu
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | | | - Hai Trung Bui
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Phi Duy Ho
- Department of Hepato-Pancreato-Biliary Diseases and Liver Transplant, Children's Hospital 2, Ho Chi Minh City, Vietnam
| | - Thierry Pirotte
- Department of Anesthesiology, Cliniques Universitaires Saint-Luc, Brussels, Belgium
| | - Raymond Reding
- Department of Abdominal Surgery and Transplantation, Cliniques Universitaires Saint-Luc, Brussels, Belgium
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Joueidi F, Alhanaee A, Alsuhaibani H, Albenmousa A, Joueidi A, Elhassan A, Nasir AN, Marquez KAH, Alghamdi S, Al Hamoudi W, Abualganam S, Broering D, Bzeizi KI. Transjugular Intrahepatic Portosystemic Shunt for Budd-Chiari Syndrome: A Single-Centre Experience. J Clin Med 2024; 13:5858. [PMID: 39407918 PMCID: PMC11478255 DOI: 10.3390/jcm13195858] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/01/2024] [Revised: 08/08/2024] [Accepted: 09/14/2024] [Indexed: 10/20/2024] Open
Abstract
Background: Despite several challenges in clinical management, there has been significant progress in understanding the aetiology, natural history and outcomes of Budd-Chiari syndrome (BCS) treatments. This study aims to evaluate the outcomes of transjugular intrahepatic portosystemic shunt (TIPS) using covered stent in management of BCS. Methods: We conducted a retrospective analysis of 70 BCS patients who underwent TIPS using covered stents between January 2010 and December 2022 at a single tertiary liver transplant centre. Patients' clinical features, laboratory parameters, and imagine findings were collected before and after TIPS. The primary endpoint was overall survival. Results: TIPS was performed on 70 patients with BCS out of a total of 88 patients. The remaining patients (18) underwent liver transplantation. The mean age was 37.7 ± 11.2 years at time of diagnosis and the majority were female (64.35). The most common symptoms and signs at presentation were abdominal pain, jaundice, ascites, and variceal bleeding. Over a median followup of 76 months, the survival rates at 1, 3, and 5 years were 98.8%, 97.9%, and 97.7%, respectively. Patients who underwent TIPS alone had better survival that patients with BCS who required liver transplantation (LTx) (p = 0.003). Conclusions: In our study TIPS provided a highly effective treatment option for BCS patients. The long-term favourability of the outcome was not impacted by the need for repeated TIPS revision. Use of covered stents was instrumental in reducing shunt dysfunction rates. Prospective and larger studies are needed to further optimize therapeutic strategies in this challenging population.
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Affiliation(s)
- Faisal Joueidi
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Amnah Alhanaee
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Hamad Alsuhaibani
- Radiology Department, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (H.A.); (S.A.)
| | - Ali Albenmousa
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Ahmad Joueidi
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Ahmed Elhassan
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Abdallah Nabeel Nasir
- College of Medicine, Alfaisal University, Riyadh 11451, Saudi Arabia; (A.J.); (A.E.); (A.N.N.)
| | - Kris Ann Hervera Marquez
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Saad Alghamdi
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Waleed Al Hamoudi
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Saad Abualganam
- Radiology Department, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (H.A.); (S.A.)
| | - Dieter Broering
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
| | - Khalid Ibrahim Bzeizi
- Department of Liver & Small Bowel Health Centre, Organ Transplant Centre of Excellence, King Faisal Specialist Hospital & Research Centre, Riyadh 11451, Saudi Arabia; (A.A.); (A.A.); (K.A.H.M.); (S.A.); (W.A.H.); (D.B.); (K.I.B.)
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Rao K, Aswani Y, Bindner H, Patel A, Averill S, Davis T, Amarneh M. Intra-abdominal Venous Thromboses and Their Management. Acad Radiol 2024; 31:3212-3222. [PMID: 38184416 DOI: 10.1016/j.acra.2023.12.018] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/30/2023] [Revised: 12/09/2023] [Accepted: 12/12/2023] [Indexed: 01/08/2024]
Abstract
While a plethora of articles discuss management of deep venous thromboses in extremities, there is a relative scarcity of literature comprehensively describing intra-abdominal venous thromboses, and their management. Intra-abdominal venous thromboses include iliocaval venous obstruction (ICVO), hepatic venous thrombosis (HVT), portal venous thrombosis (PVT), renal vein thrombosis (RVT), splenic vein thrombosis (SVT), and gonadal vein thrombosis (GVT); each of which provides unique microenvironmental challenges to management. Doppler ultrasound is the first line imaging modality for diagnosis, and computed tomography and magnetic resonance imaging can help define the extent of thrombus burden and aid with interventional planning. Systemic anticoagulation remains the common medical treatment for intra-abdominal venous thrombosis, however, catheter directed thrombolysis and thrombectomy show positive outcomes in ICVO, HVT, PVT, and RVT, with transjugular intrahepatic portosystemic shunt (TIPS) creation especially beneficial in HVT and PVT. In this review article, we describe pathophysiology, clinical features, imaging findings, and current management options for intra-abdominal venous thromboses.
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Affiliation(s)
- Karan Rao
- Department of Vascular and Interventional Radiology, University of Iowa, lowa city, lowa, USA (K.R., Y.A., H.B., A.P.)
| | - Yashant Aswani
- Department of Vascular and Interventional Radiology, University of Iowa, lowa city, lowa, USA (K.R., Y.A., H.B., A.P.)
| | - Hans Bindner
- Department of Vascular and Interventional Radiology, University of Iowa, lowa city, lowa, USA (K.R., Y.A., H.B., A.P.)
| | - Aditi Patel
- Department of Vascular and Interventional Radiology, University of Iowa, lowa city, lowa, USA (K.R., Y.A., H.B., A.P.)
| | - Sarah Averill
- Roswell Park Comprehensive Cancer Institute, Buffalo, New York, USA (S.A.)
| | - Trent Davis
- Dignity Health St. Joseph's Radiology, Phoenix, Arizona, USA (T.D.)
| | - Mohammad Amarneh
- Department of Pediatric Interventional Radiology, Boston Children's Hospital, Harvard Medical School, 300 Longwood Ave, Boston, Massachusetts 02115, USA (M.A.).
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Liu SY, Li LH, Liu ZC, Li SX, Dang XW. Development of a prognostic scoring system for hepatic vena cava Budd-Chiari syndrome with hepatocellular carcinoma. Hepatobiliary Pancreat Dis Int 2024; 23:370-375. [PMID: 36973112 DOI: 10.1016/j.hbpd.2023.03.005] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2022] [Accepted: 03/14/2023] [Indexed: 03/29/2023]
Abstract
BACKGROUND Hepatocellular carcinoma (HCC) is a serious complication of hepatic vena cava Budd-Chiari syndrome (HVC-BCS) that significantly reduces the survival time of patients. Our study aimed to analyze the prognostic factors influencing the survival of HVC-BCS patients with HCC and to develop a prognostic scoring system. METHODS The clinical and follow-up data of 64 HVC-BCS patients with HCC who received invasive treatment at the First Affiliated Hospital of Zhengzhou University between January 2015 and December 2019 were retrospectively analyzed. Kaplan-Meier curves and log-rank tests were used to analyze the survival curve of patients and the difference in prognoses between the groups. Univariate and multivariate Cox regression analyses were performed to analyze the influence of biochemical, tumor, and etiological characteristics on the total survival time of patients, and a new prognostic scoring system was developed according to the regression coefficients of the independent predictors in the statistical model. The prediction efficiency was evaluated using the time-dependent receiver operating characteristics curve and concordance index. RESULTS Multivariate analysis showed that serum albumin level < 34 g/L [hazard ratio (HR) = 4.207, 95% confidence interval (CI): 1.816-8.932, P = 0.001], maximum tumor diameter > 7 cm (HR = 8.623, 95% CI: 3.771-19.715, P < 0.001), and inferior vena cava stenosis (HR = 3.612, 95% CI: 1.646-7.928, P = 0.001) were independent predictors of survival. A prognostic scoring system was developed according to the above-mentioned independent predictors, and patients were classified into grades A, B, C and D. Significant differences in survival were found among the four groups. CONCLUSIONS This study successfully developed a prognostic scoring system for HVC-BCS patients with HCC, which is helpful for clinical evaluation of patient prognosis.
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Affiliation(s)
- Sheng-Yan Liu
- Department of Hepatopancreatobiliary Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Budd-Chiari Syndrome Diagnosis and Treatment Center of Henan Province, Zhengzhou 450052, China
| | - Lu-Hao Li
- Department of Hepatopancreatobiliary Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Budd-Chiari Syndrome Diagnosis and Treatment Center of Henan Province, Zhengzhou 450052, China
| | - Zhao-Chen Liu
- Department of Hepatopancreatobiliary Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Budd-Chiari Syndrome Diagnosis and Treatment Center of Henan Province, Zhengzhou 450052, China
| | - Su-Xin Li
- Department of Hepatopancreatobiliary Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Budd-Chiari Syndrome Diagnosis and Treatment Center of Henan Province, Zhengzhou 450052, China
| | - Xiao-Wei Dang
- Department of Hepatopancreatobiliary Surgery, the First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China; Budd-Chiari Syndrome Diagnosis and Treatment Center of Henan Province, Zhengzhou 450052, China.
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Cohen O, Caiano LM, Levy-Mendelovich S. Cancer-associated splanchnic vein thrombosis: Clinical implications and management considerations. Thromb Res 2024; 234:75-85. [PMID: 38183815 DOI: 10.1016/j.thromres.2023.12.014] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/07/2023] [Revised: 12/13/2023] [Accepted: 12/19/2023] [Indexed: 01/08/2024]
Abstract
Splanchnic vein thrombosis (SVT), a thrombosis which involves the portal, mesenteric, and splenic veins, and the Budd-Chiari syndrome, represents an uncommon type of venous thromboembolism (VTE). Like with deep vein thrombosis of the lower extremities and pulmonary embolism, ample evidence suggests a significant association between SVT and cancer, particularly intra-abdominal solid malignancies (e.g. hepatobiliary and pancreatic cancers) and myeloproliferative neoplasms (MPN). Clinical symptoms of SVT in cancer patients can be ambiguous, and frequently attributed to the primary cancer itself. Alternatively, SVT may be asymptomatic and detected incidentally during cancer staging or follow-up evaluations. SVT can also precede the diagnosis of cancer and has been associated with poorer outcomes in patients with liver or pancreatic cancers. Therefore, an unprovoked SVT warrants a thorough evaluation for an underlying malignancy or MPN. Cancer-associated SVT carries a high risk of VTE extension, recurrence and bleeding. Extended anticoagulant treatment is often required in the absence of a high bleeding risk. Guidelines suggest treatment with either low molecular weight heparin (LMWH) or direct oral anticoagulants (DOACs), although available data on the safety and effectiveness of DOACs in these patients is limited. This comprehensive review outlines the epidemiology, pathogenesis, risk factors, and diagnosis of cancer-associated SVT and underscores the importance of comprehensive patient evaluation and evidence-based management.
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Affiliation(s)
- Omri Cohen
- National Hemophilia Center, Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel-Hashomer, Israel; School of Medicine, Tel Aviv University, Israel; Department of Medicine and Surgery, University of Insubria, Varese, Italy.
| | - Lucia Maria Caiano
- Department of Medicine and Surgery, University of Insubria, Varese, Italy
| | - Sarina Levy-Mendelovich
- National Hemophilia Center, Institute of Thrombosis and Hemostasis, Sheba Medical Center, Tel-Hashomer, Israel; School of Medicine, Tel Aviv University, Israel
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Samanta A, Sen Sarma M, Yadav R. Budd-Chiari syndrome in children: Challenges and outcome. World J Hepatol 2023; 15:1174-1187. [PMID: 38075006 PMCID: PMC10698347 DOI: 10.4254/wjh.v15.i11.1174] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/29/2023] [Revised: 10/08/2023] [Accepted: 10/30/2023] [Indexed: 11/24/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is an uncommon disease of the liver, characterised by obstruction of the hepatic venous outflow tract. The etiological spectrum of BCS as well as venous obstruction pattern show wide geographical and demographic variations across the globe. Compared to adults with BCS, children have primary BCS as the predominant etiology, earlier clinical presentation, and hence better treatment outcome. Underlying prothrombotic conditions play a key role in the etiopathogenesis of BCS, though work-up for the same is often unyielding in children. Use of next-generation sequencing in addition to conventional tests for thrombophilia leads to better diagnostic yield. In recent years, advances in radiological endovascular intervention techniques have revolutionized the treatment and outcome of BCS. Various non-invasive markers of fibrosis like liver and splenic stiffness measurement are being increasingly used to assess treatment response. Elastography techniques provide a novel non-invasive tool for measuring liver and splenic stiffness. This article reviews the diagnostic and therapeutic advances and challenges in children with BCS.
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Affiliation(s)
- Arghya Samanta
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
| | - Moinak Sen Sarma
- Department of Pediatric Gastroenterology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India.
| | - Rajanikant Yadav
- Department of Radiology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow 226014, Uttar Pradesh, India
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10
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Găman MA, Cozma MA, Manan MR, Srichawla BS, Dhali A, Ali S, Nahian A, Elton AC, Simhachalam Kutikuppala LV, Suteja RC, Diebel S, Găman AM, Diaconu CC. Budd-Chiari syndrome in myeloproliferative neoplasms: A review of literature. World J Clin Oncol 2023; 14:99-116. [PMID: 37009527 PMCID: PMC10052333 DOI: 10.5306/wjco.v14.i3.99] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/20/2022] [Revised: 02/10/2023] [Accepted: 03/01/2023] [Indexed: 03/19/2023] Open
Abstract
Myeloproliferative neoplasms (MPNs) are defined as clonal disorders of the hematopoietic stem cell in which an exaggerated production of terminally differentiated myeloid cells occurs. Classical, Philadelphia-negative MPNs, i.e., polycythemia vera, essential thrombocythemia and primary myelofibrosis, exhibit a propensity towards the development of thrombotic complications that can occur in unusual sites, e.g., portal, splanchnic or hepatic veins, the placenta or cerebral sinuses. The pathogenesis of thrombotic events in MPNs is complex and requires an intricate mechanism involving endothelial injury, stasis, elevated leukocyte adhesion, integrins, neutrophil extracellular traps, somatic mutations (e.g., the V617F point mutation in the JAK2 gene), microparticles, circulating endothelial cells, and other factors, to name a few. Herein, we review the available data on Budd-Chiari syndrome in Philadelphia-negative MPNs, with a particular focus on its epidemiology, pathogenesis, histopathology, risk factors, classification, clinical presentation, diagnosis, and management.
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Affiliation(s)
- Mihnea-Alexandru Găman
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
- Department of Hematology, Center of Hematology and Bone Marrow Transplantation, Fundeni Clinical Institute, Bucharest 022328, Romania
| | - Matei-Alexandru Cozma
- Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Bucharest 050474, Romania
| | | | - Bahadar S Srichawla
- Department of Neurology, University of Massachusetts Chan Medical School, Worcester, Massachusetts 01655, United States
| | - Arkadeep Dhali
- Department of Internal Medicine, Nottingham University Hospitals NHS Trust, Nottingham NG7 2UH, United Kingdom
| | - Sajjad Ali
- Department of Medicine, Ziauddin University, Karachi 75600, Pakistan
| | - Ahmed Nahian
- California Baptist University-Lake Erie College of Osteopathic Medicine, Riverside, CA 92504, United States
| | - Andrew C Elton
- University of Minnesota Medical School, Minneapolis, MN 55455, United States
| | - L V Simhachalam Kutikuppala
- Department of General Surgery, Dr NTR University of Health Sciences, Andhra Pradesh, Vijayawada 520008, India
| | - Richard Christian Suteja
- Department of Undergraduate Medicine, Faculty of Medicine, Udayana University Denpasar, Bali 80232, Indonesia
| | - Sebastian Diebel
- Department of Family Medicine, Northern Ontario School of Medicine Timmins, Ontario 91762, Canada
| | - Amelia Maria Găman
- Department of Pathophysiology, University of Medicine and Pharmacy of Craiova, Romania & Clinic of Hematology, Filantropia City Hospital, Craiova 200143, Romania
| | - Camelia Cristina Diaconu
- Department of Internal Medicine, Faculty of Medicine, “Carol Davila” University of Medicine and Pharmacy, Romania & Internal Medicine Clinic, Clinical Emergency Hospital of Bucharest, Bucharest 105402, Romania
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11
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Tanabayeva S, Almabayev Y, Kamyspaev M, Kulmanbetov R, Kopbayeva M, Akhmad N, Altynbekova G, Fakhradiyev I. Study of Morphological Changes in Rat Liver Caused by Occlusion of Inferior Vena Cava. J Clin Exp Hepatol 2022; 12:1451-1462. [PMID: 36340320 PMCID: PMC9630039 DOI: 10.1016/j.jceh.2022.06.001] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/11/2022] [Accepted: 06/01/2022] [Indexed: 12/12/2022] Open
Abstract
Background/objectives Up to date, there are no reports on animal survival rate and morphological changes in the liver caused by the impairment of blood outflow from the liver and its time dependence. Moreover, the impact of duration and degree of occlusion of inferior vena cava on pathological changes was not investigated yet. This study aimed at the assessment of the survival rate and morphological changes in the liver with varying degrees of occlusion of inferior vena cava. The exact timing of the reversibility of pathological processes was determined. Methods Rats (n = 160) were randomly divided into five groups: I - control group (CG) (n = 20); II - sham group (SG) (n = 20); III - intervention group (IG-1) (narrowing of the lumen of the inferior vena cava by 25%) (n = 40); IV intervention group (IG-2) (narrowing by 50%) (n = 40); and intervention group V (IG-3) (narrowing by 75%) (n = 40). The level of postoperative pain, the body and liver weight of the animals, histological examination, morphometry, and macroscopic evaluation of abdominal organs were carried out on the 1, 3, 7, 14, and 30 days following the surgical intervention. The survival rate of animals was assessed using the Kaplan-Meier method. Results On the 30th day, the rat grimace scale indices in the IG-1 (P ≤ 0.05), IG-2, and IG-3 (P ≤ 0.001) groups were higher. By body weight, this indicator on the 30th day was lower in the IG-1 (P ≤ 0.05), IG-2, and IG-3 (P ≤ 0.001) groups compared to the CG and SG groups. In the IG1 and IG2 groups, the survival rates were 72.5% and 65.0%, respectively. The lowest survival rate was observed in the IG3 group (22.5%). Conclusions Compression of the inferior vena cava by 75% led to an increase in animal mortality and the development of persistent morphological changes in the liver. At the same time, the survival rate of animals and the extent of changes in the liver with narrowing of the inferior vena cava by 25% and 50% had similar results. The results acquired possess scientific and practical importance.
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Affiliation(s)
- Shynar Tanabayeva
- S.D. Asfendiyarov Kazakh National Medical University, Almaty, Kazakhstan
| | | | - Marat Kamyspaev
- Khoja Akhmet Yassawi International Kazakh-Turkish University, Turkestan, Kazakhstan
| | - Ruslan Kulmanbetov
- S.D. Asfendiyarov Kazakh National Medical University, Almaty, Kazakhstan
| | - Maira Kopbayeva
- S.D. Asfendiyarov Kazakh National Medical University, Almaty, Kazakhstan
| | - Nurgulim Akhmad
- S.D. Asfendiyarov Kazakh National Medical University, Almaty, Kazakhstan
| | | | - Ildar Fakhradiyev
- S.D. Asfendiyarov Kazakh National Medical University, Almaty, Kazakhstan
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12
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Elkilany A, Alwarraky M, Denecke T, Geisel D. Percutaneous transluminal angioplasty for symptomatic hepatic vein-type Budd-Chiari syndrome: feasibility and long-term outcomes. Sci Rep 2022; 12:14095. [PMID: 35982064 PMCID: PMC9388522 DOI: 10.1038/s41598-022-16818-8] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2022] [Accepted: 07/15/2022] [Indexed: 11/09/2022] Open
Abstract
For management of Budd-Chiari syndrome (BCS), a step-wise therapeutic approach starting with medical treatment, followed by endovascular recanalization, transjugular intrahepatic portosystemic shunt, and finally liver transplantation has been adopted. We retrospectively analyzed 51 patients with symptomatic short segment (≤ 30 mm) hepatic vein (HV)-type BCS who underwent percutaneous transluminal balloon angioplasty (PTBA) with/without stenting to determine the feasibility, clinical effectiveness, and long-term outcomes. The intervention was technically successful in 94.1% of cases (48/51)—32 patients underwent PTBA and 16 patients underwent HV stenting. Procedure-related complications occurred in 14 patients (29.1%). The clinical success rate at 4 weeks was 91.7% (44/48). Nine patients underwent reintervention, six patients due to restenosis/occlusion and three patients with clinical failure. The mean primary patency duration was 64.6 ± 19.9 months (CI, 58.5–70.8; range, 1.2–81.7 months). The cumulative 1-, 2-, and 5-year primary patency rates were 85.4, 74.5, and 58.3%, respectively. The cumulative 1-, 2-, and 5-year secondary patency rates were 93.8, 87.2, and 75%, respectively. The cumulative 1-, 2-, and 5-year survival rates were 97.9, 91.5, and 50%, respectively. Percutaneous transluminal angioplasty with and without stenting is effective and achieves excellent long-term patency and survival rates in patients with symptomatic HV-type BCS. With its lower incidence of re-occlusion and higher clinical success rate, HV angioplasty combined with stenting should be the preferred option especially in patients with segmental HV-type BCS.
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Affiliation(s)
- Aboelyazid Elkilany
- Department of Diagnostic and Interventional Radiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, Berlin Institute of Health, Augustenburger Platz 1, 13353, Berlin, Germany. .,Department of Diagnostic Medical Imaging and Interventional Radiology, National Liver Institute, Menoufia University, Menoufia, Egypt.
| | - Mohamed Alwarraky
- Department of Diagnostic Medical Imaging and Interventional Radiology, National Liver Institute, Menoufia University, Menoufia, Egypt
| | - Timm Denecke
- Department of Diagnostic and Interventional Radiology, Leipzig University Hospital, Leipzig, Germany
| | - Dominik Geisel
- Department of Diagnostic and Interventional Radiology, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität Zu Berlin, Berlin Institute of Health, Augustenburger Platz 1, 13353, Berlin, Germany
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13
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Gavriilidis P, Marangoni G, Ahmad J, Azoulay D. State of the Art, Current Perspectives, and Controversies of Budd-Chiari Syndrome: A Review. J Clin Med Res 2022; 14:147-157. [PMID: 35573933 PMCID: PMC9076137 DOI: 10.14740/jocmr4724] [Citation(s) in RCA: 9] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/12/2022] [Accepted: 04/25/2022] [Indexed: 11/11/2022] Open
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) is an eponym that includes a group of conditions characterized by partial or complete hepatic venous tract outflow obstruction, and the site of obstruction may involve one or more hepatic veins, inferior vena cava, or the right atrium. The classification of BCS is based on etiology, site of obstruction, and duration. Its etiology is very heterogeneous; in particular, hepatic vein thrombosis is the most common type of obstruction and myeloproliferative disorder, the most common thrombophilic disorder, in the West. In Asian countries, the type of obstruction, thrombophilic disorders, clinical features, and treatment strategies vary widely from region to region. Although the cause can be identified in 90% of patients with the help of gene mutation testing, BCS remains under-recognized in many countries. A higher prevalence of acute cases has been reported in the West than in the East. This global and regional heterogeneity raises several challenges regarding the evaluation, management strategy, and individualized approach of BCS. This study aimed to conduct a systematic review of BCS to elucidate treatment strategy options. METHODS PubMed, Embase, Cochrane Library, and Google Scholar databases were searched systematically. RESULTS Sixty-nine pertinent articles were retrieved and included in the present study. CONCLUSIONS Further research on the following three topics would help define individualized treatment strategies. The first is a better understanding of the molecular pathways underlying the thrombophilic conditions implicated in the pathogenesis of BCS. The second is the role of the genotype and gene mutations in the determination of coagulation status of patients with BCS. The third is the definition of clear criteria and development of a common prognostic index to risk stratify the patients at presentation and consequently detect candidates for invasive therapies.
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Affiliation(s)
- Paschalis Gavriilidis
- Department of HBP Surgery, University Hospitals of Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry CV2 2DX, UK
| | - Gabriele Marangoni
- Department of HBP Surgery, University Hospitals of Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry CV2 2DX, UK
| | - Jawad Ahmad
- Department of HBP Surgery, University Hospitals of Coventry and Warwickshire NHS Trust, Clifford Bridge Road, Coventry CV2 2DX, UK
| | - Daniel Azoulay
- Department of Hepato-Biliary and Liver Transplantation Surgery, Paul Brousse University Hospital, Paris-Saclay University, 94800 Villejuif, France
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14
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Aktas H, Ozer A, Yilmaz TU, Keceoglu S, Can MG, Emiroglu R. Liver transplantation for Budd-Chiari syndrome: A challenging but handable procedure. Asian J Surg 2021; 45:1396-1402. [PMID: 34688524 DOI: 10.1016/j.asjsur.2021.09.007] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/07/2021] [Revised: 08/26/2021] [Accepted: 09/26/2021] [Indexed: 11/25/2022] Open
Abstract
BACKROUND Establishing venous outflow in liver transplantation for patients with Budd-Chiari syndrome is crucial and requires various surgical techniques. The outcomes of these patients exibits distinct problems including vasculary thrombosis and biliary complications. METHODS In this single center study, the outcomes and surgical features of 33 patients with Budd-Chiari syndrome who were carried out liver translantation (27 patients from living donor). Another group was formed among patients underwent liver transplantation due to other etiologies and the outcomes were compared. RESULTS The most-seen type was the classical type of Budd-Chiari syndrome (25, 75.8%). For twenty-six patients inherited or acquired prothrombotic disorders were identified (78.2%) in Budd-Chiari group. Average follow-up was 29.7 ± 15.5 months. We have observed no recurrence of disease in our BCS patients. When the two groups was compared in terms of thrombotic complications, there was a significantly increased risk in BCS group (p = 0.014). Our 1 and 3-year survival rates for the BCS group were 81.8% and 78.8%, respectively. In the control group, 1 and 3-year survival rates were 93.3% and 88.9%, respectively. Log-rank test analyses showed no statistically significant results. CONCLUSION Liver transplantation with individual surgical and postoperative treatment strategy for patients with Budd-Chiari syndrome provides comparable outcomes.
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Affiliation(s)
- Hikmet Aktas
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Ali Ozer
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey.
| | - Tonguc Utku Yilmaz
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Selim Keceoglu
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Meltem Guner Can
- Department of Anaesthesiology and Reanimation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
| | - Remzi Emiroglu
- Department of Organ Transplantation, Acibadem Mehmet Ali Aydinlar Atakent University Hospital, Istanbul, Turkey
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15
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Coe TM, Tanaka M, Bethea ED, D'Alessandro DA, Kimura S, Yeh H, Markmann JF. Liver transplantation with suprahepatic caval anastomosis including inferior vena cava stent. TRANSPLANTATION REPORTS 2021; 5. [PMID: 34485756 DOI: 10.1016/j.tpr.2020.100062] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/23/2022] Open
Abstract
Budd Chiari syndrome (BCS) results from hepatic outflow obstruction. Endovascular management to restore venous patency, including inferior vena cava (IVC) angioplasty with stenting, and transjugular intrahepatic shunt (TIPS) placement to decompress liver congestion, have become standard of care. Herein, we describe a patient with BCS requiring liver transplantation and the surgical technique of suprahepatic IVC anastomosis including thoracic extension of an IVC stent with a review of the relevant literature. A 29-year-old female with BCS due to polycythemia vera, who had been previously managed with TIPS and IVC stent placement, was taken for liver transplantation. Preoperative imaging confirmed stent extension into the thoracic IVC and the stent was unable to be removed intraoperatively. The thoracic IVC was clamped through the diaphragm at the level of the right atrium and the stent was left in place and incorporated within the suprahepatic anastomosis with good vascular outcome at one year follow up. Diligent preoperative preparation is essential with adequate imaging and cardiac surgical consultation in patients with malpositioned stents. Review of the literature shows four cases in which performing the suprahepatic anastomosis including an embedded stent is a viable alternative that allows for avoidance of a thoracotomy.
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Affiliation(s)
- Taylor M Coe
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
| | - Mari Tanaka
- Harvard Medical School, Boston, MA, United States.,Department of Radiology, Massachusetts General Hospital, Boston, MA, United States
| | - Emily D Bethea
- Harvard Medical School, Boston, MA, United States.,Division of Gastroenterology, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States
| | - David A D'Alessandro
- Harvard Medical School, Boston, MA, United States.,Division of Cardiac Surgery, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States
| | - Shoko Kimura
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
| | - Heidi Yeh
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
| | - James F Markmann
- Division of Transplantation, Department of Surgery, Massachusetts General Hospital, Boston, MA, United States.,Harvard Medical School, Boston, MA, United States
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16
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Shukla A, Shreshtha A, Mukund A, Bihari C, Eapen CE, Han G, Deshmukh H, Cua IHY, Lesmana CRA, Al Meshtab M, Kage M, Chaiteeraki R, Treeprasertsuk S, Giri S, Punamiya S, Paradis V, Qi X, Sugawara Y, Abbas Z, Sarin SK. Budd-Chiari syndrome: consensus guidance of the Asian Pacific Association for the study of the liver (APASL). Hepatol Int 2021; 15:531-567. [PMID: 34240318 DOI: 10.1007/s12072-021-10189-4] [Citation(s) in RCA: 46] [Impact Index Per Article: 11.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/28/2020] [Accepted: 04/11/2021] [Indexed: 02/07/2023]
Abstract
Budd Chiari syndrome (BCS) is a diverse disease with regard to the site of obstruction, the predisposing thrombophilic disorders and clinical presentation across the Asia-Pacific region. The hepatic vein ostial stenosis and short segment thrombosis are common in some parts of Asia-Pacific region, while membranous obstruction of the vena cava is common in some and complete thrombosis of hepatic veins in others. Prevalence of myeloproliferative neoplasms and other thrombophilic disorders in BCS varies from region to region and with different sites of obstruction. This heterogeneity also raises several issues and dilemmas in evaluation and approach to management of a patient with BCS. The opportunity to recanalize hepatic vein in patients with hepatic vein ostial stenosis or inferior vena cava stenting or pasty among those membranous obstruction of the vena cava is a unique opportunity in the Asia-Pacific region to restore hepatic outflow closely mimicking physiology. In order to address these issues arising out of the diversity as well as the unique features in the region, the Asia Pacific Association for Study of Liver has formulated these guidelines for clinicians.
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Affiliation(s)
- Akash Shukla
- Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India.
| | | | - Amar Mukund
- Institute of Liver and Biliary Sciences, New Delhi, India
| | - Chhagan Bihari
- Institute of Liver and Biliary Sciences, New Delhi, India
| | - C E Eapen
- Christian Medical College, Vellore, India
| | - Guohong Han
- Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xian, China
| | - Hemant Deshmukh
- Dean and Head of Radiology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
| | - Ian Homer Y Cua
- Institute of Digestive and Liver Diseases, St Lukes Medical Center, Global City, Philippines
| | - Cosmas Rinaldi Adithya Lesmana
- Dr. Cipto Mangunkusumo National General Hospital, Universitas Indonesia, Jakarta, Indonesia
- Digestive Disease & GI Oncology Center, Medistra Hospital, Jakarta, Indonesia
| | - Mamun Al Meshtab
- Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh
- Center for Innovative Cancer Therapy, Kurume University Research, 67 Asahi-machi, Kurume, 830-0011, Japan
| | - Masayoshi Kage
- Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
| | - Roongruedee Chaiteeraki
- Division of Gastroenterology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Sombat Treeprasertsuk
- Division of Gastroenterology, Department of Medicine, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand
| | - Suprabhat Giri
- Department of Gastroenterology, King Edward Memorial Hospital and Seth Gordhandas Sunderdas Medical College, Mumbai, India
| | - Sundeep Punamiya
- Vascular and Interventional Radiology, Tan Tock Seng Hospital, Singapore, Singapore
| | - Valerie Paradis
- Dpt dAnatomie Pathologique, Hôpital Beaujon, 100 bd du Gal Leclerc, Clichy, 92110, France
| | - Xingshun Qi
- General Hospital of Northern Theater Command (formerly General Hospital of Shenyang Military Area), No. 83 Wenhua Road, Shenyang, China
| | - Yasuhiko Sugawara
- Department of Transplantation and Pediatric Surgery, Kumamoto University, Kumamoto, Japan
| | - Zaigham Abbas
- Department of Hepatogastroenterology, Dr. Ziauddin University Hospital Clifton, Karachi, Pakistan
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17
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Sharma A, Keshava SN, Eapen A, Elias E, Eapen CE. An Update on the Management of Budd-Chiari Syndrome. Dig Dis Sci 2021; 66:1780-1790. [PMID: 32691382 DOI: 10.1007/s10620-020-06485-y] [Citation(s) in RCA: 25] [Impact Index Per Article: 6.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/05/2020] [Accepted: 07/04/2020] [Indexed: 12/17/2022]
Abstract
Budd-Chiari syndrome (BCS) is an uncommon condition, caused by obstruction to hepatic venous outflow. It is largely underdiagnosed, and a high index of suspicion is required for any patient with unexplained portal hypertension. The understanding of its etiology and pathology is improving with advances in diagnostic techniques. Recent studies reported an identifiable etiology in > 80% of cases. Myeloproliferative neoplasm (MPN) is the most common etiology, and genetic studies help in diagnosing latent MPN. Better cross-sectional imaging helps delineate the site of obstruction accurately. The majority of BCS patients are now treated by endovascular intervention and anticoagulation which have improved survival in this disease. Angioplasty of hepatic veins/inferior vena cava remains under-utilized at present. While surgical porto-systemic shunts are no longer done for BCS, liver transplantation is reserved for select indications. Some of the unresolved issues in the current management of BCS are also discussed in this review.
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Affiliation(s)
- A Sharma
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India
| | - S N Keshava
- Department of Interventional Radiology, Christian Medical College, Vellore, India
| | - A Eapen
- Department of Radiodiagnosis, Christian Medical College, Vellore, India
| | - E Elias
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.,Liver Unit, University Hospitals Birmingham, Birmingham, UK
| | - C E Eapen
- Hepatology Department, Christian Medical College, Vellore, Tamil Nadu, India.
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18
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Wilson NR, Abbas HA, Kala J. Paroxysmal Nocturnal Hemoglobinuria Presenting with Hemorrhagic Esophageal Varices. Am J Med 2021; 134:e147-e149. [PMID: 33121958 DOI: 10.1016/j.amjmed.2020.09.026] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 09/06/2020] [Revised: 09/10/2020] [Accepted: 09/11/2020] [Indexed: 11/27/2022]
Affiliation(s)
- Nathaniel R Wilson
- Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center, Houston.
| | - Hussein A Abbas
- Department of Hematology and Medical Oncology, University of Texas M.D. Anderson Cancer Center, Houston
| | - Jaya Kala
- Department of Internal Medicine, McGovern Medical School, University of Texas Health Science Center, Houston
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19
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Yin J, Qi Z, Chen Y, Chen Y. A case report of membranous occlusion of the subclavian vein: a rare cause of McCleery syndrome. ANNALS OF TRANSLATIONAL MEDICINE 2021; 9:78. [PMID: 33553371 PMCID: PMC7859811 DOI: 10.21037/atm-20-2862] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Indexed: 11/08/2022]
Abstract
Patients presenting with periodic swelling of the upper extremity without thrombosis are diagnosed with McCleery syndrome. There have been sporadic cases reported over the past decades. Due to the rarity of this disease, no standard consensus on diagnosis and treatment of McCleery syndrome was established. Subclavius tendon and anterior scalene muscle compression were proposed as the primary cause of McCleery syndrome. Partial resecting muscle, tendon or ligament was recommended as therapies. We report one rare case of membranous occlusion of the subclavian vein (SCV) that leads to periodic swelling of upper extremity and diagnosis of McCleery syndrome was made. This 21-year-old man complained of swelling and pain in the right upper extremity after strenuous exercise lasting for 3 months. Physical examination, spinal X-ray and magnetic resonance imaging showed no signs related to classic venous thoracic outlet syndrome (VTOS). Duplex ultrasonography demonstrated membranous occlusion without thrombosis at the proximal end of the right SCV. The lesion was confirmed by venography. Treated by balloon dilation alone, the patient recovered uneventfully during 18 months of follow-up. Repeated duplex ultrasonography revealed patency of the SCV. To our best knowledge, our case provides the first reported membranous occlusion of the SCV. Excluding the presence of thrombosis in SCV, he was diagnosed with McCleery syndrome and was cured by balloon dilation alone. We can learn from this rare case that membranous occlusion of veins can be a rare cause of McCleery syndrome and is worthy of careful consideration and differentiation of VOTS.
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Affiliation(s)
- Jingjing Yin
- Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Zhenhong Qi
- Department of Ultrasonography, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yu Chen
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yuexin Chen
- Department of Vascular Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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20
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Niknam R, Hajizadegan N, Mohammadkarimi V, Mahmoudi L. A study of the different parameters in acute and chronic Budd–Chiari syndrome. EGYPTIAN LIVER JOURNAL 2020; 10:48. [DOI: 10.1186/s43066-020-00058-4] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/03/2020] [Accepted: 09/21/2020] [Indexed: 11/10/2022] Open
Abstract
Abstract
Background
Budd–Chiari syndrome (BCS) is a rare and potentially life-threatening vascular disease of the liver. There are a few studies on the differences between acute and chronic BCS in clinical and laboratory characteristics, as well as the outcomes, so we designed this research to study the different parameters in acute and chronic BCS. Diagnosis of BCS was made using Doppler ultrasound, magnetic resonance imaging, and venography. Patients with BCS were then divided into chronic and acute groups based on both imaging characteristics and disease duration. Finally, the outcomes, clinical features, and laboratory parameters of acute and chronic BCS were compared.
Results
In total, 60 patients were included in this study, of whom 28.3% and 71.7% had acute and chronic BCS, respectively. According to clinical features, spontaneous bacterial peritonitis (PR 1.289; 95% CI 1.115–1.489; P = 0.001) and jaundice (PR 1.308; 95% CI 1.148–1.490; P < 0.001) were significantly associated with chronic than acute BCS. According to laboratory parameters, the levels of international normalized ratio (INR) (PR 0.953; 95% CI 0.918–0.989; P = 0.012), blood urea nitrogen (BUN) (PR 0.996; 95% CI 0.993–1.000; P = 0.039), and model for end-stage liver disease (MELD) score (PR 1.024; 95% CI 1.012–1.037; P < 0.001) in chronic group were significantly higher than in the acute BCS. The hospital length of stay (LOS) and mortality rate between the two groups did not differ significantly.
Conclusions
This study showed that the spontaneous bacterial peritonitis, jaundice, increased levels of MELD score, INR, and BUN were significantly associated with the chronic group compared with the acute group. The hospital LOS and mortality rate between the two groups did not differ significantly. Further research is recommended to clarify this issue.
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21
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Venous thrombosis of the liver: current and emerging concepts in management. Transl Res 2020; 225:54-69. [PMID: 32407789 DOI: 10.1016/j.trsl.2020.04.012] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Revised: 04/11/2020] [Accepted: 04/16/2020] [Indexed: 02/08/2023]
Abstract
Venous thrombosis within the hepatic vasculature is associated with a distinct array of risk factors, characteristics, and potential complication. As such, it entails unique management considerations and strategies relative to the more common categories of venous thromboembolic disease. Although broadly divided into thrombosis of the afferent vasculature (the portal venous system) and efferent vasculature (the hepatic venous system), presentations and management strategies within these groupings are heterogeneous. Management decisions are influenced by a variety of factors including the chronicity, extent, and etiology of thrombosis. In this review we examine both portal vein thrombosis and hepatic vein thrombosis (and the associated Budd-Chiari Syndrome). We consider those factors which most impact presentation and most influence treatment. In so doing, we see how the particulars of specific cases introduce nuance into clinical decisions. At the same time we attempt to organize our understanding of such cases to help facilitate a more systematic approach. Critically, we must recognize that although increasing evidence is emerging to help guide our management strategies, the available data remain limited and largely retrospective. Indeed, current paradigms are based largely on observational experiences and expert consensus. As new and more rigorous studies emerge, treatment strategies are likely to be continually refined, and paradigm shifts are sure to occur.
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22
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Inchingolo R, Posa A, Mariappan M, Tibana TK, Nunes TF, Spiliopoulos S, Brountzos E. Transjugular intrahepatic portosystemic shunt for Budd-Chiari syndrome: A comprehensive review. World J Gastroenterol 2020; 26:5060-5073. [PMID: 32982109 PMCID: PMC7495032 DOI: 10.3748/wjg.v26.i34.5060] [Citation(s) in RCA: 19] [Impact Index Per Article: 3.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/19/2020] [Revised: 06/06/2020] [Accepted: 08/25/2020] [Indexed: 02/06/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is a relatively rare clinical condition with a wide range of symptomatology, caused by the obstruction of the hepatic venous outflow. If left untreated, it has got an high mortality rate. Its management is based on a step-wise approach, depending on the clinical presentation, and includes different treatment from anticoagulation therapy up to Interventional Radiology techniques, such as transjugular intrahepatic portosystemic shunt (TIPS). TIPS is today considered a safe and highly effective treatment and should be recommended for BCS patients, including those awaiting orthotopic liver transplantation. In this review the pathophysiology, diagnosis and treatment options of BCS are presented, with a special focus on published data regarding the techniques and outcomes of TIPS for the treatment of BCS. Moreover, unresolved issues and future research will be discussed.
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Affiliation(s)
- Riccardo Inchingolo
- Interventional Radiology Unit, "F. Miulli" Regional Hospital, Acquaviva delle Fonti 70021, Italy
- Department of Radiology, King´s College Hospital, London SE5 9RS, United Kingdom
| | - Alessandro Posa
- Department of Radiology, Gemelli Hospital, Roma 00135, Italy
| | - Martin Mariappan
- Interventional Radiology Department, Aberdeen Royal Infirmary Hospital, Aberdeen AB25 2ZN, United Kingdom
| | - Tiago Kojun Tibana
- Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
| | - Thiago Franchi Nunes
- Interventional Radiology Department, Universidade Federal de Mato Grosso do Sul, Campo Grande 79070-900, Brazil
| | - Stavros Spiliopoulos
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
| | - Elias Brountzos
- 2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Chaidari Athens GR 12461, Greece
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Ding PX, Han XW, Liu C, Zhang Y, Cheng AL, Wu Y, Liang XX, Gao XM, Lee EW. Long-term outcomes of individualized treatment strategy in treatment of type I Budd-Chiari syndrome in 456 patients. Liver Int 2019; 39:1577-1586. [PMID: 30963702 DOI: 10.1111/liv.14114] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/29/2019] [Revised: 03/17/2019] [Accepted: 04/02/2019] [Indexed: 12/14/2022]
Abstract
AIM To evaluate individualized treatment strategy (ITS) and long-term outcomes of endovascular treatment of Budd-Chiari syndrome (BCS) with obstructed inferior vena cava (IVC) based on different degrees of hepatic vein (HV) involvement. METHODS From January 2006 to June 2017, 456 consecutive patients with BCS with obstructed IVC underwent endovascular treatment with ITS. All patients received IVC recanalization. Then, 426 patients with at least one patent HV received no additional treatment. Twenty-fivepatients with membranous or segmental occlusion of HVs underwent HV recanalization and for the remaining five patients with diffuse HVs occlusion, a transjugular intrahepatic portosystemic shunt (TIPS) was performed. RESULTS The endovascular treatment was technically successful in 455 of the 456 patients (99.8%). The complication rate was 5.0% (23/456), with major complications in 13 patients (2.8%) and minor complications in 10 patients (2.2%). Median follow-up time was 60.5 months (range, 4-120 months). The cumulative 1-, 2-, 5- and 10-year primary vessel patency rates were 93.6%, 89.9%, 80.5% and 74.3% respectively and the cumulative 1-, 2-, 5-, 10- year secondary patency rates were 99.8%, 99.8%, 98.2% and 97.2% respectively. The cumulative 1-, 2-, 5- and 10-year survival rates were 98.4%, 95.8%, 91.2% and 76.5% respectively. Illness duration and decreased serum albumin were independent predictors of survival. CONCLUSION The ITS for Asian BCS with obstructed IVC and varying degrees of HV involvement appears to be effective and with good long-term outcomes.
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Affiliation(s)
- Peng-Xu Ding
- Department of Intervention, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xin-Wei Han
- Department of Intervention, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Chao Liu
- Department of Intervention, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yan Zhang
- Department of Ultrasound, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - An-Ling Cheng
- Department of Ultrasound, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Yan Wu
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xiao-Xue Liang
- Department of Radiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Xue-Mei Gao
- Department of Magnetic Resonance, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, China
| | - Edward Wolfgang Lee
- Division of Interventional Radiology, Department of Radiology, UCLA Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA
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Van Wettere M, Purcell Y, Bruno O, Payancé A, Plessier A, Rautou PE, Cazals-Hatem D, Valla D, Vilgrain V, Ronot M. Low specificity of washout to diagnose hepatocellular carcinoma in nodules showing arterial hyperenhancement in patients with Budd-Chiari syndrome. J Hepatol 2019; 70:1123-1132. [PMID: 30654065 DOI: 10.1016/j.jhep.2019.01.009] [Citation(s) in RCA: 30] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/26/2018] [Revised: 12/11/2018] [Accepted: 01/04/2019] [Indexed: 12/15/2022]
Abstract
BACKGROUND & AIMS It remains unclear whether the classic imaging criteria for the non-invasive diagnosis of hepatocellular carcinoma (HCC) can be applied to chronic vascular liver diseases, such as Budd-Chiari syndrome (BCS). Herein, we aimed to evaluate the diagnostic value of washout for the discrimination between benign and malignant lesions in patients with BCS. METHODS This retrospective study included all patients admitted to our institution with a diagnosis of BCS and focal lesions on MRI from 2000 to 2016. MRI images were reviewed by 2 radiologists blinded to the nature of the lesions. Patient and lesion characteristics were recorded, with a focus on washout on portal venous and/or delayed phases. Lesions were compared using Chi-square, Fisher's, Student's t or Mann-Whitney U tests. RESULTS A total of 49 patients (mean age 35 ± 12 years; 34 women [69%] and 15 men [31%]) with 241 benign lesions and 12 HCC lesions were analyzed. Patients with HCC were significantly older (mean age 44 ± 16 vs. 33 ± 9 years, p = 0.005), with higher alpha-fetoprotein (AFP) levels (median 16 vs. 3 ng/ml, p = 0.007). Washout was depicted in 9/12 (75%) HCC, and 69/241 (29%) benign lesions (p <0.001). A total of 52/143 (36%) lesions ≥1 cm with arterial hyperenhancement showed washout (9 HCC and 43 benign lesions). In this subgroup, the specificity of washout for the diagnosis of HCC was 67%. Adding T1-w hypointensity raised the specificity to 100%. A serum AFP >15 ng/ml was associated with 95% specificity. CONCLUSION Washout was observed in close to one-third of benign lesions, leading to an unacceptably low specificity for the diagnosis of HCC. The non-invasive diagnostic criteria proposed for cirrhotic patients cannot be extrapolated to patients with BCS. LAY SUMMARY Washout on MRI is depicted in a significant proportion of benign nodules in patients with Budd-Chiari syndrome (BCS), limiting its value for the differentiation between benign and malignant lesions. Criteria proposed for the non-invasive diagnosis of hepatocellular carcinoma in patients with cirrhosis cannot be extrapolated to patients with BCS. Additional imaging findings and patient characteristics, including alpha-fetoprotein serum level, can help determine the probability of a nodule being HCC in patients with BCS.
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Affiliation(s)
- Morgane Van Wettere
- Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France
| | - Yvonne Purcell
- Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France
| | - Onorina Bruno
- Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France
| | - Audrey Payancé
- Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France; University Paris Diderot. Sorbonne Paris Cité, Paris, France
| | - Aurélie Plessier
- Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France; University Paris Diderot. Sorbonne Paris Cité, Paris, France
| | - Pierre-Emmanuel Rautou
- Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France; University Paris Diderot. Sorbonne Paris Cité, Paris, France; Inserm, U970, Paris Cardiovascular Research Center - PARCC, Université Paris Descartes, Sorbonne Paris Cité, Paris, France; DHU Unity, Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Hôpital Beaujon, AP-HP, Clichy, France
| | - Dominique Cazals-Hatem
- Department of Pathology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France
| | - Dominique Valla
- Department of Hepatology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France; University Paris Diderot. Sorbonne Paris Cité, Paris, France; DHU Unity, Pôle des Maladies de l'Appareil Digestif, Service d'Hépatologie, Centre de Référence des Maladies Vasculaires du Foie, Hôpital Beaujon, AP-HP, Clichy, France
| | - Valérie Vilgrain
- Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France; University Paris Diderot. Sorbonne Paris Cité, Paris, France; INSERM U1149, CRI, Paris, France
| | - Maxime Ronot
- Department of Radiology, APHP, University Hospitals Paris Nord Val de Seine, Beaujon, Clichy, France; University Paris Diderot. Sorbonne Paris Cité, Paris, France; INSERM U1149, CRI, Paris, France.
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Jeschke B, Gottlieb A, Sowa JP, Jeschke S, Treckmann JW, Gerken G, Canbay A. Single-Center Retrospective Study of Clinical and Laboratory Features That Predict Survival of Patients With Budd-Chiari Syndrome After Liver Transplant. EXP CLIN TRANSPLANT 2019; 17:665-672. [PMID: 31050620 DOI: 10.6002/ect.2018.0274] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.2] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/05/2022]
Abstract
OBJECTIVES Budd-Chiari syndrome is a rare but critical condition that can progress to liver failure and death. For severe cases, orthotopic liver transplant remains the only curative option. The present study aimed to identify predictive parameters to assess outcomes of liver transplant. MATERIALS AND METHODS Medical records of 33 individuals with Budd-Chiari syndrome who received orthotopic liver transplant were retrospectively assessed. Twenty-seven eligible patients were identified and grouped by outcome (survived/deceased) after transplant for Budd-Chiari syndrome. Demographic, clinical, and serum parameters taken at the time of Budd-Chiari syndrome diagnosis were evaluated for prognostic value. RESULTS Differences between patients who survived and those who died were found for nausea/vomiting (P < .01) and splenomegaly (P < .01), which were both more common in patients who died after transplant. In addition, patients in the deceased group exhibited significantly lower serum cholinesterase levels (P < .01) and higher alkaline phosphatase levels (P < .01). Scoring systems to assess liver status or Budd-Chiari syndrome severity (Model for End-Stage Liver Disease and Child-Pugh scores, Rotterdam score, and the transjugular intrahepatic portosystemic shunting prognostic index) did not differ between groups. CONCLUSIONS Nausea/vomiting, splenomegaly, low serum cholinesterase, and high alkaline phosphatase were associated with adverse outcomes after orthotopic liver transplant for Budd-Chiari syndrome. These factors may be surrogate markers for a severely impaired health status at time of diagnosis and should be evaluated prospectively in larger cohorts.
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Affiliation(s)
- Barbara Jeschke
- the Department of Gastroenterology and Hepatology, University Hospital, University Duisburg-Essen, Essen, Germany
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Khan F, Armstrong MJ, Mehrzad H, Chen F, Neil D, Brown R, Cain O, Tripathi D. Review article: a multidisciplinary approach to the diagnosis and management of Budd-Chiari syndrome. Aliment Pharmacol Ther 2019; 49:840-863. [PMID: 30828850 DOI: 10.1111/apt.15149] [Citation(s) in RCA: 34] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/17/2018] [Revised: 11/06/2019] [Accepted: 12/29/2018] [Indexed: 12/13/2022]
Abstract
BACKGROUND Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by obstruction in the hepatic venous outflow tract. AIM To provide an update of the pathophysiology, aetiology, diagnosis, management and follow-up of BCS. METHODS Analysis of recent literature by using Medline, PubMed and EMBASE databases. RESULTS Primary BCS is usually caused by thrombosis and is further classified into "classical BCS" type where obstruction occurs within the hepatic vein and "hepatic vena cava BCS" which involves thrombosis of the intra/suprahepatic portion of the inferior vena cava (IVC). BCS patients often have a combination of prothrombotic risk factors. Aetiology and presentation differ between Western and certain Asian countries. Myeloproliferative neoplasms are present in 35%-50% of European patients and are usually associated with the JAK2-V617F mutation. Clinical presentation is diverse and BCS should be excluded in any patient with acute or chronic liver disease. Non-invasive imaging (Doppler ultrasound, computed tomography, or magnetic resonance imaging) usually provides the diagnosis. Liver biopsy should be obtained if small vessel BCS is suspected. Stepwise management strategy includes anticoagulation, treatment of identified prothrombotic risk factors, percutaneous revascularisation and transjugular intrahepatic portosystemic stent shunt to re-establish hepatic venous drainage, and liver transplantation in unresponsive patients. This strategy provides a 5-year survival rate of nearly 90%. Long-term outcome is influenced by any underlying haematological condition and development of hepatocellular carcinoma. CONCLUSIONS With the advent of newer treatment strategies and improved understanding of BCS, outcomes in this rare disease have improved over the last three decades. An underlying haematological disorder can be the major determinant of outcome.
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Affiliation(s)
- Faisal Khan
- Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Matthew J Armstrong
- Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
- NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, UK
- Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
| | - Homoyon Mehrzad
- Imaging and Interventional Radiology Department, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Frederick Chen
- NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, UK
- Department of Clinical Haematology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Desley Neil
- Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Rachel Brown
- Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Owen Cain
- Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
| | - Dhiraj Tripathi
- Liver Unit, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
- NIHR Birmingham Biomedical Research Centre, University Hospitals Birmingham NHS Foundation Trust and University of Birmingham, Birmingham, UK
- Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
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Kurzbard-Roach N, Jha P, Poder L, Menias C. Abdominal and pelvic imaging findings associated with sex hormone abnormalities. Abdom Radiol (NY) 2019; 44:1103-1119. [PMID: 30483844 DOI: 10.1007/s00261-018-1844-1] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/21/2022]
Abstract
Hormones are substances that serve as chemical communication between cells. They are unique biological molecules that affect multiple organ systems and play a key role in maintaining homoeostasis. In this role, they are usually produced from a single organ and have defined target organs. However, hormones can affect non-target organs as well. As such, biochemical and hormonal abnormalities can be associated with anatomic changes in multiple target as well as non-target organs. Hormone-related changes may take the form of an organ parenchymal abnormality, benign neoplasm, or even malignancy. Given the multifocal action of hormones, the observed imaging findings may be remote from the site of production, and may actually be multi-organ in nature. Anatomic findings related to hormone level abnormalities and/or laboratory biomarker changes may be identified with imaging. The purpose of this image-rich review is to sensitize radiologists to imaging findings in the abdomen and pelvis that may occur in the context of hormone abnormalities, focusing primarily on sex hormones and their influence on these organs.
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Affiliation(s)
- Nicole Kurzbard-Roach
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, San Francisco, CA, USA
| | - Priyanka Jha
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, San Francisco, CA, USA.
| | - Liina Poder
- Department of Radiology and Biomedical Imaging, University of California, San Francisco, San Francisco, CA, USA
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Dhamija E, Deshmukh A, Meena P, Kumar M, Bhatnagar S, Thulkar S. Complementary role of intervention radiology in palliative care in oncology setting. Indian J Palliat Care 2019; 25:462-467. [PMID: 31413465 PMCID: PMC6659525 DOI: 10.4103/ijpc.ijpc_24_19] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/25/2022] Open
Abstract
Owing to advances in treatment of cancer, there has been increase in life expectancy. Palliative care aims at improving quality of life of patients suffering from malignancy and is now recognized as a separate subspecialty. Management of cancer patients needs a multidisciplinary approach, and radiology has a key role to play at every step of it. Interventional radiology has broadened its scope immensely over the last decade with development of newer and less invasive applications useful in oncology and palliative care. The role of interventional radiologists begins from obtaining tissue for histopathological examination and extends to controlling disease spread with ablation or chemoembolization, to managing the tumor-related complications and relieving stressful symptoms such as dyspnea and pain. This article aims to review the interventional radiologist's arsenal in managing patients with malignancies with a special emphasis on palliative care, providing a more holistic approach in improving the quality of life of cancer patients.
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Grandhe S, Lee JA, Chandra A, Marsh C, Frenette CT. Trapped vessel of abdominal pain with hepatomegaly: A case report. World J Hepatol 2018; 10:887-891. [PMID: 30533189 PMCID: PMC6280163 DOI: 10.4254/wjh.v10.i11.887] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/03/2018] [Revised: 08/17/2018] [Accepted: 10/10/2018] [Indexed: 02/06/2023] Open
Abstract
Abdominal pain with elevated transaminases from inferior vena cava (IVC) obstruction is a relatively common reason for referral and further workup by a hepatologist. The differential for the cause of IVC obstruction is extensive, and the most common etiologies include clotting disorders or recent trauma. In some situations the common etiologies have been ruled out, and the underlying process for the patient’s symptoms is still not explained. We present one unique case of abdominal pain and hepatomegaly secondary to IVC constriction from extrinsic compression of the diaphragm. Based on this patient’s presentation, we urge that physicians be cognizant of the IVC diameter and consider extrinsic compression as a contributor to the patient’s symptoms. If IVC compression from the diaphragm is confirmed, early referral to vascular surgery is strongly advised for further surgical intervention.
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Affiliation(s)
- Sirisha Grandhe
- Department of Gastroenterology and Hepatology, University of California Davis Medical Center, Sacramento, CA 95817, United States
| | - Joy A Lee
- Department of Internal Medicine, Scripps Green Hospital, La Jolla, CA 92037, United States
| | - Ankur Chandra
- Department of Vascular Surgery, Scripps Green Hospital, La Jolla, CA 92037, United States
| | - Christopher Marsh
- Scripps Center for Organ Transplant, Scripps Green Hospital, La Jolla, CA 92037, United States
| | - Catherine T Frenette
- Scripps Center for Organ Transplant, Scripps Green Hospital, La Jolla, CA 92037, United States
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Cheng DL, Zhu N, Xu H, Li CL, Lv WF, Fang WW, Li CT. Outcomes of endovascular interventional therapy for primary Budd-Chiari syndrome caused by hepatic venous obstruction. Exp Ther Med 2018; 16:4141-4149. [PMID: 30402156 PMCID: PMC6201046 DOI: 10.3892/etm.2018.6708] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/11/2018] [Accepted: 08/09/2018] [Indexed: 12/13/2022] Open
Abstract
To date, interventional therapy for patients with Budd-Chiari syndrome (BCS) due to hepatic vein obstruction (HVO) has not been standardized in China. In Western countries, BCS primarily occurs due to thrombosis and the majority of patients receive thrombolysis. In China, BCS is mostly caused by the membranous occlusion of the HV or IVC. The present retrospective study evaluated the efficacy of recanalization techniques in patients with primary BCS due to HVO. The data of 69 patients with BCS due to HVO, who underwent endovascular therapy at 2 centers in China between December 2010 and December 2012, were analyzed. All of the patients underwent balloon angioplasty. In addition, 14, 6 and 5 patients received thrombolysis, endovascular stent and thrombolysis + endovascular stent, respectively. The overall technical success rate was 95.7% (66/69), and was comparable among the treatments. The HV pressure after the treatments was significantly lower compared with that prior to the procedures (23.3±6.9 vs. 46.5±8.6 cmH2O; P<0.001). The mean follow-up duration was 75 months (range, 60–84 months). During the 5-year follow-up, 10 patients (15.2%) had developed a recurrence of BCS-associated symptoms, of which 7 were successfully treated. The cumulative survival rates at 12, 36 and 60 months after endovascular interventional therapy (balloon angioplasty or combined treatment) were 98.5, 98.5 and 93.9%, respectively. After treatment by endovascular therapy, the patients with BCS caused by HVO had high survival rates and low recurrence rates in the short- and mid-term.
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Affiliation(s)
- De-Lei Cheng
- Shandong Medical Imaging Research Institute, Shandong University, Jinan, Shandong 250021, P.R. China.,Department of Interventional Radiology, Anhui Provincial Hospital, Hefei, Anhui 230000, P.R. China
| | - Nan Zhu
- Department of Radiology, People's Hospital of Wuhe County, Bengbu, Anhui 233300, P.R. China
| | - Hao Xu
- Department of Interventional Radiology, Affiliated Hospital of Xuzhou Medical University, Xuzhou, Jiangsu 221100, P.R. China
| | - Cheng-Li Li
- Shandong Medical Imaging Research Institute, Shandong University, Jinan, Shandong 250021, P.R. China
| | - Wei-Fu Lv
- Department of Interventional Radiology, Anhui Provincial Hospital, Hefei, Anhui 230000, P.R. China
| | - Wei-Wei Fang
- Department of Interventional Radiology, Anhui Provincial Hospital, Hefei, Anhui 230000, P.R. China
| | - Chuan-Ting Li
- Shandong Medical Imaging Research Institute, Shandong University, Jinan, Shandong 250021, P.R. China
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Khan F, Mehrzad H, Tripathi D. Timing of Transjugular Intrahepatic Portosystemic Stent-shunt in Budd-Chiari Syndrome: A UK Hepatologist's Perspective. J Transl Int Med 2018; 6:97-104. [PMID: 30425945 PMCID: PMC6231303 DOI: 10.2478/jtim-2018-0022] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open
Abstract
Budd-Chiari syndrome (BCS) is a rare but fatal disease caused by the obstruction in hepatic venous outflow tract (usually by thrombosis) and is further classified into two subtypes depending on the level of obstruction. Patients with BCS often have a combination of prothrombotic risk factors. Clinical presentation is diverse. Stepwise management strategy has been suggested with excellent 5-year survival rate. It includes anticoagulation, treatment of identified prothrombotic risk factor, percutaneous recanalization, and transjugular intrahepatic portosystemic shunt (TIPS) to reestablish hepatic venous outflow and liver transplantation in unresponsive patients. Owing to the rarity of BCS, there are no randomized controlled trials (RCTs) precisely identifying the timing for TIPS. TIPS should be considered in patients with refractory ascites, variceal bleed, and fulminant liver failure. Liver replacement is indicated in patients with progressive liver failure and in those in whom TIPS is not technically possible. The long-term outcome is usually influenced by the underlying hematologic condition and the development of hepatocellular carcinoma. This review focuses on the timing and the long-term efficacy of TIPS in patients with BCS.
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Affiliation(s)
- Faisal Khan
- Liver Unit, Queen Elizabeth Hospital, Birmingham, UK
| | - Homoyon Mehrzad
- Imaging and Interventional Radiology Department, Queen Elizabeth Hospital, Birmingham, UK
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A Sakr M, Abdelhakam SM, Dabbous HM, Abdelmoaty AS, Ebada HE, Al-Banna WM, Ghazy MS, Aboelmaaty ME, Eldorry AK. Pattern of Vascular Involvement in Egyptian Patients with Budd-Chiari Syndrome: Relation to Etiology and Impact on Clinical Presentation. Ann Hepatol 2018; 17:638-644. [PMID: 29893705 DOI: 10.5604/01.3001.0012.0933] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 02/04/2023]
Abstract
INTRODUCTION AND AIM Budd-Chiari syndrome (BCS) is caused by hepatic venous outflow obstruction. This work aims to analyze the pattern of vascular involvement in Egyptian patients with BCS, demonstrates its relation to etiology and shows its impact on clinical presentation. MATERIAL AND METHODS The current retrospective study was conducted at The Tropical Medicine Department, Ain Shams University on one hundred Egyptian patients with confirmed diagnosis of primary BCS who were presented to the Budd-Chiari Study Group (BCSG) from April 2014 to May 2016 by collecting clinical, laboratory and radiological data from their medical records. RESULTS Isolated hepatic vein occlusion (HVO) was the most common pattern of vascular involvement (43%), followed by combined HVO and inferior vena cava (IVC) compression by enlarged caudate lobe (32%), then combined HVO and IVC stenosis/webs (21%), and lastly isolated IVC occlusion (4%). Ascites was more significantly encountered in BCS patients with HVO than in those with isolated inferior vena cava (IVC) occlusion and patent HVs (P = 0.005). Abdominal pain was significantly encountered in patients with occluded three major HVs (P = 0.044). Behcet's disease was significantly detected in isolated IVC occlusion. Protein C deficiency was significantly detected in patients with combined HVO and IVC compression. CONCLUSION Isolated HVs occlusion was the most common pattern of vascular involvement in Egyptian patients with primary BCS. Vascular pattern of involvement affected the clinical presentation and was related to the underlying thrombophilia in those patients.
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Affiliation(s)
- Mohammad A Sakr
- Department of Tropical Medicine, Ain Shams University, Cairo, Abbasia, Egypt
| | - Sara M Abdelhakam
- Department of Tropical Medicine, Ain Shams University, Cairo, Abbasia, Egypt
| | - Hany M Dabbous
- Department of Tropical Medicine, Ain Shams University, Cairo, Abbasia, Egypt
| | - Ahmed S Abdelmoaty
- Department of Tropical Medicine, Ain Shams University, Cairo, Abbasia, Egypt
| | - Hend E Ebada
- Department of Tropical Medicine, Ain Shams University, Cairo, Abbasia, Egypt
| | - Wael M Al-Banna
- Department of Tropical Medicine, Ain Shams University, Cairo, Abbasia, Egypt
| | - Mohamed S Ghazy
- Department of Radiodiagnosis and Interventional Radiology, Ain Shams University, Cairo, Abbasia, Egypt
| | - Mohamed E Aboelmaaty
- Department of Radiodiagnosis and Interventional Radiology, Ain Shams University, Cairo, Abbasia, Egypt
| | - Ahmed K Eldorry
- Department of Radiodiagnosis and Interventional Radiology, Ain Shams University, Cairo, Abbasia, Egypt
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Friedman T, Quencer KB, Kishore SA, Winokur RS, Madoff DC. Malignant Venous Obstruction: Superior Vena Cava Syndrome and Beyond. Semin Intervent Radiol 2017; 34:398-408. [PMID: 29249864 DOI: 10.1055/s-0037-1608863] [Citation(s) in RCA: 56] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/18/2022]
Abstract
Venous obstruction in the cancer population can result in substantial morbidity and, in extreme cases, mortality. While venous obstruction can be caused by both benign and malignant etiologies in this population, the management of malignant venous obstruction as a palliative measure can be somewhat nuanced with respect to nonprocedural and procedural management, both with respect to treatment of the underlying malignancy as well as treatment of venous hypertension, which may be associated with venous thrombosis. Symptom severity, primary malignancy, functional status, and prognosis are all fundamental to the patient workup and dictate both the timing and extent of endovascular intervention. The morbidity and mortality associated with malignant obstructions of central venous structures, specifically the superior vena cava and inferior vena cava, can be significantly improved with endovascular management in appropriately selected patients. Thus, the pertinent literature regarding the clinical presentation, workup, and endovascular management of malignant central venous obstruction syndromes, with directed attention to superior vena cava syndrome and inferior vena cava syndrome, will be reviewed in this article.
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Affiliation(s)
- Tamir Friedman
- Department of Radiology, Division of Interventional Radiology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, New York
| | - Keith B Quencer
- Department of Radiology, Division of Interventional Radiology, University of Utah School of Medicine, Salt Lake City, Utah
| | - Sirish A Kishore
- Department of Radiology, Division of Interventional Radiology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, New York
| | - Ronald S Winokur
- Department of Radiology, Division of Interventional Radiology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, New York
| | - David C Madoff
- Department of Radiology, Division of Interventional Radiology, New York Presbyterian Hospital/Weill Cornell Medical Center, New York, New York
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35
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Cardiovascular disease in patients with autoinflammatory syndromes. Rheumatol Int 2017; 38:37-50. [DOI: 10.1007/s00296-017-3854-7] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/09/2017] [Accepted: 10/13/2017] [Indexed: 12/16/2022]
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36
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Chloros T, Burt C, Dunning J. The Role of Intraoperative Transesophageal Echocardiography in Identifying a Fenestrated Occlusion of the Inferior Vena Cava During Pulmonary Thromboendarterectomy. J Cardiothorac Vasc Anesth 2017; 32:1329-1332. [PMID: 29217240 DOI: 10.1053/j.jvca.2017.09.004] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2017] [Indexed: 11/11/2022]
Affiliation(s)
- Thomas Chloros
- Department of Anaesthesia and Critical Care, Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, United Kingdom
| | - Christiana Burt
- Department of Anaesthesia and Critical Care, Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, United Kingdom.
| | - John Dunning
- Department of Cardiothoracic Surgery, Papworth Hospital NHS Foundation Trust, Papworth Everard, Cambridge, United Kingdom
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37
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Buzzetti E, Parikh PM, Gerussi A, Tsochatzis E. Gender differences in liver disease and the drug-dose gender gap. Pharmacol Res 2017; 120:97-108. [PMID: 28336373 DOI: 10.1016/j.phrs.2017.03.014] [Citation(s) in RCA: 57] [Impact Index Per Article: 7.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/12/2016] [Revised: 03/17/2017] [Accepted: 03/17/2017] [Indexed: 12/14/2022]
Abstract
Although gender-based medicine is a relatively recent concept, it is now emerging as an important field of research, supported by the finding that many diseases manifest differently in men and women and therefore, might require a different treatment. Sex-related differences regarding the epidemiology, progression and treatment strategies of certain liver diseases have long been known, but most of the epidemiological and clinical trials still report results only about one sex, with consequent different rate of response and adverse reactions to treatment between men and women in clinical practice. This review reports the data found in the literature concerning the gender-related differences for the most representative hepatic diseases.
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Affiliation(s)
- Elena Buzzetti
- UCL Institute for Liver and Digestive Health, Royal Free Hospital, London, UK.
| | - Pathik M Parikh
- UCL Institute for Liver and Digestive Health, Royal Free Hospital, London, UK
| | - Alessio Gerussi
- UCL Institute for Liver and Digestive Health, Royal Free Hospital, London, UK; Internal Medicine Unit, Department of Experimental and Clinical Medical Sciences, University of Udine, Udine, Italy
| | - Emmanuel Tsochatzis
- UCL Institute for Liver and Digestive Health, Royal Free Hospital, London, UK
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