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Monaco G, Bucherini L, Stefanini B, Piscaglia F, Foschi FG, Ielasi L. Direct oral anticoagulants for the treatment of splanchnic vein thrombosis: A state of art. World J Gastroenterol 2023; 29:4962-4974. [PMID: 37731994 PMCID: PMC10507502 DOI: 10.3748/wjg.v29.i33.4962] [Citation(s) in RCA: 6] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/27/2023] [Revised: 08/07/2023] [Accepted: 08/17/2023] [Indexed: 09/01/2023] Open
Abstract
Splanchnic vein thrombosis (SVT) is a manifestation of venous thromboembolism in an unusual site. Portal, mesenteric, and splenic veins are the most common vessels involved in SVT which occurs mainly in patients with liver cirrhosis, although non-cirrhotic patients could be affected as well. Thrombosis of hepatic veins, also known as Budd-Chiari syndrome, is another manifestation of SVT. Prompt diagnosis and intervention are mandatory in order to increase the recalization rate and reduce the risk of thrombus progression and hypertensive complications. Traditional anticoagulation with heparin and vitamin-K antagonists is the treatment of choice in these cases. However, recent studies have shown promising results on the efficacy and safety of direct oral anticoagulants (DOACs) in this setting. Available results are mainly based on retrospective studies with small sample size, but first clinical trials have been published in the last years. This manuscript aims to provide an updated overview of the current evidence regarding the role of DOACs for SVT in both cirrhotic and non-cirrhotic patients.
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Affiliation(s)
- Giovanni Monaco
- Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
- Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
| | - Luca Bucherini
- Department of Internal Medicine, Ospedale degli Infermi di Faenza, Faenza 48018, Italy
| | - Bernardo Stefanini
- Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
- Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
| | - Fabio Piscaglia
- Department of Medical and Surgical Sciences, University of Bologna, Bologna 40138, Italy
- Division of Internal Medicine, Hepatobiliary and Immunoallergic Diseases, IRCCS Azienda Ospedaliero-Universitaria di Bologna, Bologna 40138, Italy
| | | | - Luca Ielasi
- Department of Internal Medicine, Ospedale degli Infermi di Faenza, Faenza 48018, Italy
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Affiliation(s)
- Juan Carlos Garcia-Pagán
- From the Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic (a provider of the European Reference Network on Rare Liver Disorders [ERN-Liver]), Institut de Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, and Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid - both in Spain (J.C.G.-P.); and Université Paris Cité, Unite Mixte de Recherche 1149, INSERM, Paris, and Centre de Référence des Maladies Vasculaires du Foie, Service d'Hépatologie, Assistance Publique-Hôpitaux de Paris, Hôpital Beaujon (a provider of the ERN-Liver), Clichy - both in France (D.-C.V.)
| | - Dominique-Charles Valla
- From the Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic (a provider of the European Reference Network on Rare Liver Disorders [ERN-Liver]), Institut de Investigacions Biomèdiques August Pi i Sunyer, University of Barcelona, Barcelona, and Centro de Investigación Biomédica en Red Enfermedades Hepáticas y Digestivas, Madrid - both in Spain (J.C.G.-P.); and Université Paris Cité, Unite Mixte de Recherche 1149, INSERM, Paris, and Centre de Référence des Maladies Vasculaires du Foie, Service d'Hépatologie, Assistance Publique-Hôpitaux de Paris, Hôpital Beaujon (a provider of the ERN-Liver), Clichy - both in France (D.-C.V.)
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Elkrief L, Payancé A, Plessier A, d’Alteroche L, Ronot M, Paradis V, Valla D, Rautou PE. Management of splanchnic vein thrombosis. JHEP Rep 2023; 5:100667. [PMID: 36941824 PMCID: PMC10023986 DOI: 10.1016/j.jhepr.2022.100667] [Citation(s) in RCA: 21] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/15/2022] [Revised: 12/11/2022] [Accepted: 12/23/2022] [Indexed: 01/04/2023] Open
Abstract
The expression splanchnic vein thrombosis encompasses Budd-Chiari syndrome and portal vein thrombosis. These disorders have common characteristics: they are both rare diseases which can cause portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, among which myeloproliferative neoplasms represent the most common; a rapid comprehensive work-up for risk factors of thrombosis is needed in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis and in those with porto-sinusoidal vascular liver disease. The presence and nature of underlying liver disease impacts the management of portal vein thrombosis. Indications for anticoagulation in patients with cirrhosis are growing, while transjugular intrahepatic portosystemic shunt is now a second-line option. Due to the rarity of these diseases, studies yielding high-grade evidence are scarce. However, collaborative studies have provided new insight into the management of these patients. This article focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, portal vein thrombosis without underlying liver disease, or cirrhosis with non-malignant portal vein thrombosis.
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Key Words
- BCS, Budd-Chiari syndrome
- CALR, calreticulin
- Cavernoma
- DOACs, direct-acting oral anticoagulants
- Direct oral anticoagulants
- EHPVO, extrahepatic portal vein obstruction
- GFR, glomerular filtration rate
- JAK2, Janus kinase 2
- LMWH, low-molecular-weight heparin
- MPN, myeloproliferative neoplasm
- MTHFR, methylene-tetrahydrofolate reductase
- PNH, paroxysmal nocturnal hemoglobinuria
- PVT, portal vein thrombosis
- Portal biliopathy
- Portal vein recanalisation
- SVT, splanchnic vein thrombosis
- TIPS, transjugular intrahepatic portosystemic shunt
- VKAs, vitamin K antagonists
- Vascular liver diseases
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Affiliation(s)
- Laure Elkrief
- Service d’Hépato-Gastroentérologie CHU de Tours, France
| | - Audrey Payancé
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Aurélie Plessier
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | | | - Maxime Ronot
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service de radiologie, Hôpital Beaujon APHP.Nord, Clichy, France
| | - Valérie Paradis
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d’anatomie et cytologie pathologique, Hôpital Beaujon APHP.Nord, Clichy, France
| | - Dominique Valla
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
| | - Pierre-Emmanuel Rautou
- Université Paris-Cité, Inserm, Centre de recherche sur l'inflammation, UMR 1149, Paris, France
- Service d'Hépatologie, AP-HP, Hôpital Beaujon, DMU DIGEST, Centre de Référence des Maladies Vasculaires du Foie, FILFOIE, ERN RARE-LIVER, Clichy, France
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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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Pérez-González A, Argibay A, Lorenzo-Castro R, Martín-Granizo I, Rivera-Gallego A. Budd-Chiari syndrome: epidemiological and clinical characteristics of a case series in Northwest Spain. EGYPTIAN LIVER JOURNAL 2022. [DOI: 10.1186/s43066-022-00192-1] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/10/2022] Open
Abstract
Abstract
Introduction
Budd-Chiari syndrome (BCS) is a rare vascular disease of the liver, characterised by occlusion of the venous outflow tract. Cancer, pyogenic liver infection, and prothrombotic haematological conditions are the most frequent causes of BCS. The treatment and prognosis of the disease are closely related to the underlying cause.
Methods
This is a retrospective case-series study performed in Spain, in a health area of around 523,000 inhabitants. Cases were identified in the discharge database of the hospital between 2000 and 2020. Epidemiological, clinical, therapeutic, and prognosis data were obtained from the patient medical records.
Results
A total of 15 cases were identified. Most of them were male patients (n = 8, 53.3%) with a median age of 52 years. The most common cause of BCS was cancer (n = 6, 40.0%) followed by liver abscesses (n = 4, 26.7%). The most frequent clinical course was subacute hepatitis (n = 8, 53.3%); 12 of the 15 patients (80%) received anticoagulant treatment, and interventional treatment was carried out in 4 patients (26.7%). Seven patients died within 6 months (46.7%), 6 of them due to progression of the underlying disease, most often cancer; 2 patients (13.3%) developed liver cirrhosis after BCS.
Discussion
The incidence of BCS was low but higher than in other European studies. In addition, this current research showed a different aetiology than previously described. The mortality rate was extremely high and closely related to the underlying disease. The involvement of classic prothrombotic haematological factors was less common than previously described.
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Le Joncour A, Saadoun D. Systemic Diseases Affecting Liver Vessels. VASCULAR DISORDERS OF THE LIVER 2022:329-343. [DOI: 10.1007/978-3-030-82988-9_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Northup PG, Garcia-Pagan JC, Garcia-Tsao G, Intagliata NM, Superina RA, Roberts LN, Lisman T, Valla DC. Vascular Liver Disorders, Portal Vein Thrombosis, and Procedural Bleeding in Patients With Liver Disease: 2020 Practice Guidance by the American Association for the Study of Liver Diseases. Hepatology 2021; 73:366-413. [PMID: 33219529 DOI: 10.1002/hep.31646] [Citation(s) in RCA: 358] [Impact Index Per Article: 89.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/16/2020] [Accepted: 11/16/2020] [Indexed: 12/12/2022]
Affiliation(s)
- Patrick G Northup
- Division of Gastroenterology and Hepatology, Center for the Study of Hemostasis in Liver Disease, University of Virginia, Charlottesville, VA
| | - Juan Carlos Garcia-Pagan
- Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clinic, Institut d'Investigacions Biomèdiques August Pi I i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain.,Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Barcelona, Spain.,Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver), Barcelona, Spain
| | - Guadalupe Garcia-Tsao
- Department of Internal Medicine, Section of Digestive Diseases, Yale University, New Haven, CT.,Veterans Administration Healthcare System, West Haven, CT
| | - Nicolas M Intagliata
- Division of Gastroenterology and Hepatology, Center for the Study of Hemostasis in Liver Disease, University of Virginia, Charlottesville, VA
| | - Riccardo A Superina
- Department of Transplant Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, IL
| | - Lara N Roberts
- Department of Haematological Medicine, King's Thrombosis Centre, King's College Hospital National Health Service (NHS) Foundation Trust, London, United Kingdom
| | - Ton Lisman
- Section of Hepatobiliary Surgery and Liver Transplantation, Surgical Research Laboratory, Department of Surgery, University Medical Centre Groningen, University of Groningen, Groningen, the Netherlands
| | - Dominique C Valla
- Hepatology Service, Hospital Beaujon, Clichy, France.,Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver), Barcelona, Spain
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Abdel Hameed MR, Elbeih EAMS, Abd El-Aziz HM, Afifi OAH, Khalaf LMR, Ali Abu Rahma MZ, Sabry A. Epidemiological Characteristics and Etiology of Budd-Chiari Syndrome in Upper Egypt. J Blood Med 2020; 11:515-524. [PMID: 33408545 PMCID: PMC7779296 DOI: 10.2147/jbm.s278678] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/13/2020] [Accepted: 12/14/2020] [Indexed: 12/17/2022] Open
Abstract
Background and Objectives Budd-Chiari syndrome (BCS) is a rare disorder caused by obstruction to hepatic venous outflow. It affects all races, usually during the third or fourth decade of life. Higher prevalence had being evident in developing countries. The aim of the present study was to clarify sociodemographic features, clinical, radiological presentations, and etiology of BCS among Upper Egyptian patients. Patients and Methods This retrospective cohort study enrolled 50 Upper Egyptian Patients with confirmed primary BCS. Liver, coagulation, and thrombophilia workup profiles were performed as anticardiolipin antibodies, lupus anticoagulant, protein C, protein S, and antithrombin III assays. Factor V Leiden and JAK2 mutations were assessed. Full radiological assessment was done. Results Fifty patients were included. There were 28 males (56%) and 22 females (44%) with mean age (32.5 ± 11.1 years). The etiological factor was not identified in 22% of cases (n=11). Isolated factor C deficiency was found in 26% (n=13) with male predominance 39.3% and protein S deficiency in 10% (n=5). Factor V Leiden mutation was the etiology in 5 patients (10%). Membranous web and antiphospholipid syndrome each were the etiology in 8% (n=4). Behςet’s disease was diagnosed in 4% (n=2). Cases of liver cirrhosis(LC) were 41/50(82%)they were :33/50(66%) LC child class C, 8 /50(16%) LC child class B, and 0/50 (0%) LC child class A. Abdominal pain was the most common symptom (96%), and ascites was the most common sign (82%). Obstruction of hepatic veins was present in 80%. Conclusion BCS in Upper Egyptian patients was mainly occurred in males in the third and fourth decade of life, mostly with liver cirrhosis. The most common etiology is isolated protein C deficiency followed by Factor V Leiden mutation and isolated protein S deficiency. Hepatic veins obstruction was the most common pattern of vascular involvement.
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Affiliation(s)
- Muhamad R Abdel Hameed
- Department of Internal Medicine & Hematology Unit, Assiut University Hospitals and Bone Marrow Transplantation Unit, South Egypt Cancer Institute, Assiut University, Assiut, Egypt
| | - Esam Abdel-Moneim Sadek Elbeih
- Department of Internal Medicine & Hematology Unit, Assiut University Hospitals and Bone Marrow Transplantation Unit, South Egypt Cancer Institute, Assiut University, Assiut, Egypt
| | | | - Ola Abdel-Haleem Afifi
- Department of Clinical Pathology, Assiut University Hospitals, Assiut University, Assiut, Egypt
| | | | | | - Abeer Sabry
- Department of Internal Medicine, Helwan University, Helwan, Egypt
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Soret J, Debray D, Fontbrune FSD, Kiladjian JJ, Saadoun D, Latour RPD, Valla D, Hernandez-Gea V, Hillaire S, Dutheil D, Plessier A, Bureau C, De Raucourt E. Risk factors for vascular liver diseases: Vascular liver diseases: position papers from the francophone network for vascular liver diseases, the French Association for the Study of the Liver (AFEF), and ERN-rare liver. Clin Res Hepatol Gastroenterol 2020; 44:410-419. [PMID: 32651075 DOI: 10.1016/j.clinre.2020.03.010] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/26/2020] [Accepted: 03/03/2020] [Indexed: 02/04/2023]
Affiliation(s)
- Juliette Soret
- Center of Clinical Investigation, Saint-Louis Hospital APHP, 1, avenue Claude Vellefaux, 75010 Paris, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France.
| | - Dominique Debray
- Pediatric hepatology Unit, Necker Hospital APHP, 149, rue de Sèvres, 75015 Paris, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
| | - Flore Sicre de Fontbrune
- Service d'hématologie, French referral centre for Aplastic anemia and PNH and filière de santé maladies rares immunohématologiques, Saint-Louis Hospital APHP, ERN eurobloodnet, 75010 Paris, France
| | - Jean-Jacques Kiladjian
- Center of Clinical Investigation, Saint-Louis Hospital APHP, 1, avenue Claude Vellefaux, 75010 Paris, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France
| | - David Saadoun
- Departement of Internal Medecine, Médecine interne, La Pitié Salpêtrière Hospital APHP, CMR maladies auto_immunes systémiques rares ; CMR maladies auto inflammatoires et amylose, ERN RITA, 47-83, boulevard de l'Hôpital, 75651 Paris, France
| | - Régis Peffault de Latour
- Service d'hématologie, French referral centre for Aplastic anemia and PNH and filière de santé maladies rares immunohématologiques, Saint-Louis Hospital APHP, ERN eurobloodnet, 75010 Paris, France
| | - Dominique Valla
- French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Hepatology, Beaujon Hospital AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver
| | - Virginia Hernandez-Gea
- Barcelona Hepatic Hemodynamic Unit, Liver Unit. IMDIM. CIBERehd, Hospital Clinic, Barcelona, Spain
| | - Sophie Hillaire
- Department of Internal Medicine, Foch Hospital, 40, rue Worth, 92150 Suresnes, France
| | - Danielle Dutheil
- French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Association of patients with vascular liver diseases (AMVF), Beaujon Hospital, Department of Hepatology, 100, boulevard du Général-Leclerc, 92118 Clichy, France
| | - Aurélie Plessier
- French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Hepatology, Beaujon Hospital AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver
| | - Christophe Bureau
- French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Department of Gastroenterology and Hepatology, Rangueil Hospital, University Hospital of Toulouse, 1, avenue du Professeur Jean-Poulhès, 31400 Toulouse, France
| | - Emmanuelle De Raucourt
- Department of Laboratory Hematology, Beaujon Hospital AP-HP, 100, boulevard du Général Leclerc, 92118 Clichy, France; French Network for Rare Liver Diseases FILFOIE, Saint-Antoine Hospital AP-HP, 184, rue du Faubourg Saint-Antoine, 75012 Paris, France; Reference center of vascular liver diseases, European Reference Network (ERN) Rare-Liver
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Abstract
Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare cause of liver disease that should not be missed. Variable clinical presentation among patients with BCS necessitates a high index of suspicion to avoid missing this life-threatening diagnosis. BCS is characterized as primary or secondary, depending on etiology of venous obstruction. Most patients with primary BCS have several contributing risk factors leading to a prothrombotic state. A multidisciplinary stepwise approach is integral in treating BCS. Lifelong anticoagulation is recommended. Long-term monitoring of patients for development of cirrhosis, complications of portal hypertension, hepatocellular carcinoma, and progression of underlying diseases is important.
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Fan J, Wang Q, Luo B, Chen H, Wang Z, Niu J, Yuan J, Yuan X, Bai W, He C, Guo W, Li K, Yin Z, Fan D, Han G. Prevalence of prothrombotic factors in patients with Budd-Chiari syndrome or non-cirrhotic nonmalignant portal vein thrombosis: A hospital-based observational study. J Gastroenterol Hepatol 2020; 35:1215-1222. [PMID: 31711259 DOI: 10.1111/jgh.14925] [Citation(s) in RCA: 13] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/28/2019] [Revised: 10/21/2019] [Accepted: 11/04/2019] [Indexed: 01/18/2023]
Abstract
BACKGROUND AND AIM Comprehensive investigations on the prothrombotic factors of splanchnic vein thrombosis (SVT), including Budd-Chiari syndrome (BCS) and non-cirrhotic nonmalignant portal vein thrombosis (PVT), in Eastern patients are scarce. METHODS Between March 2012 and July 2017, 812 consecutive patients, including 418 BCS and 394 non-cirrhotic nonmalignant PVT patients, were admitted to Xijing Hospital (a Chinese tertiary academic hospital) and screened for prothrombotic factors. Odds ratios (ORs), 95% confidence intervals (CIs), and P-trends were calculated by using conditional logistic regression. RESULTS The prevalence of myeloproliferative neoplasms (MPNs) was only 6.3% among BCS patients but 28.3% among PVT patients. Notably, the presence of MPNs was associated with a higher risk of hepatic vein-type BCS (OR 9.9, 95% CI 3.6-26.7, P-trend < 0.001) and extensive thrombosis in PVT (OR 4.1, 95% CI 1.9-8.9, P-trend < 0.001). Calreticulin mutations existed in 2.7% of SVT patients. Furthermore, the prevalence of antiphospholipid antibody syndrome and protein C, protein S, or antithrombin deficiency in BCS patients was 7.3% and 22.5%, respectively, similar to that in patients with PVT (7.4% and 25.7%). In addition, factor V Leiden mutation, prothrombin G20210A mutation, and paroxysmal nocturnal hemoglobinuria were identified in < 1% of both BCS and PVT patients. CONCLUSION There is a significant positive association between MPNs and hepatic vein-type BCS or non-cirrhotic nonmalignant PVT with extensive thrombosis. Additionally, calreticulin mutations should be tested in JAK2V617F -negative SVT patients in China. However, screening for factor V Leiden mutation, prothrombin G20210A mutation, and paroxysmal nocturnal hemoglobinuria may be unnecessary.
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Affiliation(s)
- Jiahao Fan
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Qiuhe Wang
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Bohan Luo
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Hui Chen
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Zhengyu Wang
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Jing Niu
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Jie Yuan
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Xulong Yuan
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Wei Bai
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Chuangye He
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Wengang Guo
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Kai Li
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Zhanxin Yin
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Daiming Fan
- State Key laboratory of Cancer Biology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
| | - Guohong Han
- Department of Liver Disease and Digestive Interventional Radiology, National Clinical Research Center for Digestive Disease and Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi'an, China
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Abstract
Celiac disease is a common form of enteropathy with frequent extraintestinal manifestations (EIM). Misrecognition of these presentations may lead to significant delays in diagnosis. Any organ may be involved, either through an immune/inflammatory phenomenon, or nutritional deficiencies. Some EIM, such as gluten ataxia, may be irreversible if left untreated, but most will improve with a gluten-free diet. Knowledge of the various EIM, as well as the associated conditions which do not improve on a gluten-free diet, will avoid delays in the diagnosis and management of celiac disease and associated manifestations.
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Affiliation(s)
- Amelie Therrien
- Department of Medicine, Celiac Center, Division of Gastroenterology, Beth Israel Deaconess Medical Center
- Celiac Research Program, Harvard Medical School
| | - Ciaran P Kelly
- Department of Medicine, Celiac Center, Division of Gastroenterology, Beth Israel Deaconess Medical Center
- Celiac Research Program, Harvard Medical School
| | - Jocelyn A Silvester
- Celiac Research Program, Harvard Medical School
- Division of Gastroenterology, Hepatology and Nutrition, Boston Children Hospital, Boston, MA
- Rady College of Medicine, Faculty of Health Sciences, University of Manitoba, Winnipeg, MB, Canada
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13
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Elkrief L, Valla D. Hepatic Venous Outflow Syndromes and Splanchnic Venous Thrombosis. EVIDENCE‐BASED GASTROENTEROLOGY AND HEPATOLOGY 4E 2019:645-661. [DOI: 10.1002/9781119211419.ch42] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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14
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Abdominal pain, nausea, vomiting, and ascites in a 14-year-old girl with systemic lupus erythematosus: Answers. Pediatr Nephrol 2019; 34:431-433. [PMID: 30187126 DOI: 10.1007/s00467-018-4059-3] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/18/2018] [Revised: 08/01/2018] [Accepted: 08/13/2018] [Indexed: 10/28/2022]
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15
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Jadallah KA, Sarsak EW, Khazaleh YM, Barakat RMK. Budd-Chiari syndrome associated with coeliac disease: case report and literature review. Gastroenterol Rep (Oxf) 2018; 6:308-312. [PMID: 27604577 PMCID: PMC6225809 DOI: 10.1093/gastro/gow030] [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] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/27/2016] [Revised: 07/15/2016] [Accepted: 08/01/2016] [Indexed: 01/02/2023] Open
Abstract
We report a case of Budd-Chiari syndrome occurring in a patient with coeliac disease, who presented with symptoms of increased abdominal girth, right upper quadrant pain and shortness of breath for three weeks prior to admission. Initial assessment revealed the presence of moderate ascites, hepatosplenomegaly and right-sided pleural effusion. Further diagnostic work-up established a diagnosis of chronic Budd-Chiari syndrome. Interestingly, complete screening for pro-thrombotic factors was negative. A review of the literature on this association disclosed only 28 similar cases, with the majority of them describing individuals of North African origin. Interestingly, in the majority of cases no specific thrombotic factor could be identified, suggesting that coeliac disease may play a role in this thrombotic disorder.
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Affiliation(s)
- Khaled Ali Jadallah
- Department of Internal Medicine, King Abdullah University Hospital, Irbid, Jordan
| | - Enas Walid Sarsak
- Department of Internal Medicine, King Abdullah University Hospital, Irbid, Jordan
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16
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Diagnosis and treatment guidelines for aberrant portal hemodynamics: The Aberrant Portal Hemodynamics Study Group supported by the Ministry of Health, Labor and Welfare of Japan. Hepatol Res 2017; 47:373-386. [PMID: 28058764 DOI: 10.1111/hepr.12862] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/06/2016] [Revised: 12/29/2016] [Accepted: 01/04/2017] [Indexed: 12/18/2022]
Abstract
Idiopathic portal hypertension (IPH), causing aberrant portal hemodynamics, is a disease with an as yet unidentified cause and no established treatment protocol. The Japanese research group on IPH in Japan was set up in 1975 by the Ministry of Health, Labor and Welfare. Extrahepatic portal obstruction and Budd-Chiari syndrome (BCS) have since been added to the group's research subjects. The aims of the research group are to accurately evaluate the current status of the three diseases in Japan, elucidate their etiology and pathogenesis, and develop new treatments. Due to the long-term efforts of the Japanese research group, aberrant portal hemodynamics has been investigated in a variety of aspects, from epidemiological and pathological studies to molecular biology analyses. As a result, it has been shown that there are abnormal genes in the liver, specific for IPH. In addition, pathological findings of BCS were internationally compared and the difference in findings between Japan and Europe (or North America) has been clarified. Furthermore, it was found that complication rates of hepatocellular carcinoma in BCS were higher in Japan. Based on the research, "Diagnosis and treatment of aberrant portal hemodynamics (2001)", including diagnostic criteria for aberrant portal hemodynamics, was published in 2001. In 2013, it was revised to "Diagnosis and treatment guidelines for aberrant portal hemodynamics (2013)" after the incorporation of diagnosis and treatment in accordance with its current status.
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17
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Pandiaraja J, Sathyaseelan A. Budd- Chiari Syndrome as an Initial Manifestation of Systemic Lupus Erythematosus. J Clin Diagn Res 2016; 10:OD01-2. [PMID: 27190864 DOI: 10.7860/jcdr/2016/16623.7532] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/03/2015] [Accepted: 12/07/2015] [Indexed: 12/26/2022]
Abstract
Budd- Chiari syndrome is caused by obstruction of hepatic venous outflow. There are numerous causes for Budd-Chiari syndrome. One of the causes is systemic lupus erythematosus due to antiphospholipid antibodies. Only few cases have reported Budd-Chiari syndrome as an initial manifestation of systemic lupus erythematosus (SLE). This is a case report of Budd-Chiari syndrome due to SLE.
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Affiliation(s)
- Jayabal Pandiaraja
- Assistant Professor, Department of General Surgery, SRM Medical College , Potheri, Kattankulathur, Kancheepuram, Tamil Nadu, India
| | - Arumugam Sathyaseelan
- Resident, Department of General Surgery, SRM Medical College , Potheri, Kattankulathur, Kancheepuram, Tamil Nadu, India
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18
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Faraoun SA, Boudjella MEA, Debzi N, Benidir N, Afredj N, Guerrache Y, Bentabak K, Soyer P, Bendib SE. Budd-Chiari syndrome: an update on imaging features. Clin Imaging 2016; 40:637-46. [PMID: 27317208 DOI: 10.1016/j.clinimag.2016.01.006] [Citation(s) in RCA: 22] [Impact Index Per Article: 2.4] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/06/2015] [Revised: 01/05/2016] [Accepted: 01/15/2016] [Indexed: 12/15/2022]
Abstract
Budd-Chiari syndrome (BCS) is a rare cause of portal hypertension and liver failure. This condition is characterized by an impaired hepatic venous drainage. The diagnosis of BCS is based on imaging, which helps initiate treatment. Imaging findings can be categorized into direct and indirect signs. Direct signs are the hallmarks of BCS and consist of visualization of obstructive lesions of the hepatic veins or the upper portion of the inferior vena cava. Indirect signs, which are secondary to venous obstruction, correspond to intra- and extrahepatic collateral circulation, perfusion abnormalities, dysmorphy and signs of portal hypertension.
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Affiliation(s)
- Sid Ahmed Faraoun
- Department of Radiology, Pierre and Marie Curie Center, Place du 1er Mai, 16016, Algiers, Algeria.
| | | | - Nabil Debzi
- Department of Hepatology, CHU Mustapha, Place du 1er Mai, 16016, Algiers, Algeria.
| | | | - Nawel Afredj
- Department of Hepatology, CHU Mustapha, Place du 1er Mai, 16016, Algiers, Algeria.
| | - Youcef Guerrache
- Department of Radiology, Pierre and Marie Curie Center, Place du 1er Mai, 16016, Algiers, Algeria.
| | - Kamel Bentabak
- Department of Durgery, Centre Pierre et Marie Curie, Place du 1er Mai, 16016, Algiers, Algeria.
| | - Philippe Soyer
- Université Sorbonne Paris Cité, Diderot Paris 7, 10 Avenue de Verdun, 75010, Paris, France.
| | - Salah Eddine Bendib
- Department of Radiology & Université Benyoucef Benkhedda d'Alger, Pierre and Marie Curie Center, Place du 1er Mai, 16016, Algiers, Algeria.
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