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1.
Indian J Pathol Microbiol ; 67(1): 96-101, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38358196

RESUMO

Background: Budd-Chiari syndrome (BCS) requires a constellation of clinical, imaging, and histological findings for diagnosis. Liver biopsy serves as a tool for confirming the diagnosis, even though the histological characteristics are not pathognomonic. Aims: To determine which constellation of morphologic findings could aid in establishing a diagnosis of BCS in clinically suspected cases. Materials and Methods: A 5-year retrospective observational study was conducted. The clinical, laboratory, and histological findings of liver biopsies in patients with a clinical diagnosis of BCS were studied. Cases were segregated into two groups on the basis of the number of histological features present. A scoring system was then devised to assess the efficacy of the histological findings in diagnosing BCS. Statistical Analysis Used: The continuous variables were compared using the Mann-Whitney U-test, and categorical variables were compared using the Fisher-exact test. Results: The common histopathological findings were the presence of red blood cells in the space of disse (100%), peri-portal fibrosis (97.1%), sinusoidal dilation (97.1%), portal inflammation (67.6%), centrilobular necrosis (61.8%) and pericellular/sinusoidal fibrosis (61.8%). Comparison between the two groups showed that centrilobular necrosis, lobular inflammation, portal inflammation, central vein fibrosis, and pericellular/sinusoidal fibrosis were significant parameters. No correlation was found between the clinical and laboratory parameters and the two groups. Conclusions: The liver biopsy features in BCS are often nonspecific, and no single feature in isolation is characteristic. A constellation of features (centrilobular necrosis, lobular inflammation, portal inflammation, central vein fibrosis, and pericellular/sinusoidal fibrosis), when present together, indicate the possibility of BCS.


Assuntos
Síndrome de Budd-Chiari , Humanos , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/patologia , Fígado/patologia , Fibrose , Necrose/patologia , Inflamação/patologia , Biópsia
2.
Int J Mol Sci ; 25(3)2024 Jan 26.
Artigo em Inglês | MEDLINE | ID: mdl-38338802

RESUMO

Myeloproliferative neoplasms (MPNs) are the leading causes of unusual site thrombosis, affecting nearly 40% of individuals with conditions like Budd-Chiari syndrome or portal vein thrombosis. Diagnosing MPNs in these cases is challenging because common indicators, such as spleen enlargement and elevated blood cell counts, can be obscured by portal hypertension or bleeding issues. Recent advancements in diagnostic tools have enhanced the accuracy of MPN diagnosis and classification. While bone marrow biopsies remain significant diagnostic criteria, molecular markers now play a pivotal role in both diagnosis and prognosis assessment. Hence, it is essential to initiate the diagnostic process for splanchnic vein thrombosis with a JAK2 V617F mutation screening, but a comprehensive approach is necessary. A multidisciplinary strategy is vital to accurately determine the specific subtype of MPNs, recommend additional tests, and propose the most effective treatment plan. Establishing specialized care pathways for patients with splanchnic vein thrombosis and underlying MPNs is crucial to tailor management approaches that reduce the risk of hematological outcomes and hepatic complications.


Assuntos
Síndrome de Budd-Chiari , Transtornos Mieloproliferativos , Neoplasias , Trombose , Trombose Venosa , Humanos , Veia Porta , Neoplasias/patologia , Trombose Venosa/genética , Trombose Venosa/complicações , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/genética , Transtornos Mieloproliferativos/complicações , Transtornos Mieloproliferativos/diagnóstico , Transtornos Mieloproliferativos/genética , Trombose/patologia , Mutação , Janus Quinase 2/genética
3.
J Pediatr Gastroenterol Nutr ; 78(1): 17-26, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38291698

RESUMO

OBJECTIVES: Radiological intervention (RI) is the preferred treatment in children with Budd-Chiari syndrome (BCS). We studied the comparative long-term outcome of BCS children, with and without RI and utility of liver and splenic stiffness measurement (LSM, SSM) by 2-dimensional shear wave elastography (2D-SWE) in assessing response. METHODS: Sixty children (40 boys, median age 10.5 [6.5-15.25] years) with BCS (29 newly diagnosed, 31 follow-up) were evaluated. LSM and SSM by 2D-SWE and vascular patency were monitored pre- and postprocedure (≥ 6 months postprocedure) in those undergoing RI. Medical therapy without anticoagulation and monitoring was done in subjects without RI. The RI and no-RI groups were compared. RESULTS: Ascites (54,90%), hepatomegaly (56,93%) and prominent abdominal-veins (42,70%), were the commonest features. The majority (46,78%) had isolated hepatic vein block. 44 (73%) cases underwent RI, while 16 (27%) were managed conservatively. Both groups were similar at baseline. Post-RI subjects showed significant improvement in clinical findings, liver functions and portal hypertension. LSM [33 (32-34.5) to 19.2 (18-20.67) kPa] and SSM [54.5 (52.3-57.6) to 28.9 (27.6-30.25) kPa] showed a significant decline from baseline value over a follow-up of 12 (6-13) months. Gradual reduction occurred in the LSM and SSM over 1-5 years, with near-normal LSM [10.2 (9.2-11.5) kPa] and SSM [22.3 (20.5-24.3) kPa] values in patients (n-16) with > 5 years follow-up. Patients without RI showed worsening in LSM and SSM. Hepatopulmonary syndrome and hepatocellular carcinoma developed in 4 (8%) and 1 (1.7%) cases respectively. CONCLUSION: RI leads to clinical recovery and reduction with near normalization of LSM and SSM over long-term follow-up in children with BCS. 2D-SWE is a promising tool to monitor outcomes.


Assuntos
Síndrome de Budd-Chiari , Técnicas de Imagem por Elasticidade , Hipertensão Portal , Neoplasias Hepáticas , Masculino , Criança , Humanos , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/terapia , Técnicas de Imagem por Elasticidade/métodos , Fígado/patologia , Hipertensão Portal/patologia , Neoplasias Hepáticas/patologia , Cirrose Hepática/patologia
4.
Transplant Proc ; 56(1): 125-134, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38177046

RESUMO

BACKGROUND: Living-donor liver transplantation (LDLT) is established as a standard therapy for end-stage liver disease; however, vessel reconstruction is more demanding due to the short length and small size of the available structures compared with deceased-donor whole liver transplantation. Interventional radiology (IR) has become the first-line treatment for vascular complications after LDLT. Hepatic venous outflow obstruction (HVOO) is a life-threatening complication after LDLT. The aim of this study of 592 adult-to-adult LDLT cases was to investigate the safety and efficacy of stent implantation for HVOO after LDLT. METHODS: Records of patients who developed HVOO requiring any treatment were collected with special reference to the metallic stent implantation. There were 232 left-side grafts and 360 right-side grafts. Sixteen cases developed HVOO after LDLT with an incidence rate of 2.7%, 5 with a left liver graft (2%), and 11 with a right-side graft (3%). The IR was attempted for 14 cases; among those, 8 cases were treated by stent implantation. RESULTS: The technical success rate of the initial stent implantation was 100%. The pressure gradient at the stenotic site significantly improved from 12.2 (range, 10.9-20.4 cm H2O) to 3.9 cm H2O (range, 1.4-8.2 cm H2O; P = .03). The volume of the congested graft liver decreased significantly from 1448 (range, 788-2170 mL) to 1265 mL (range, 748-1665 mL; P = .01), and the serum albumin level improved significantly from 3.3 (range, 1.7-3.7 g/dL) to 3.7 g/dL (range, 2.9-4.1 g/dL; P = .02). No procedure-related complication was noted, and the long-term stent patency was 100%. CONCLUSION: Metallic stent implantation for stenotic venous anastomosis after LDLT is a safe and effective treatment.


Assuntos
Síndrome de Budd-Chiari , Transplante de Fígado , Adulto , Humanos , Síndrome de Budd-Chiari/diagnóstico por imagem , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/efeitos adversos , Doadores Vivos , Veias Hepáticas/diagnóstico por imagem , Veias Hepáticas/cirurgia , Resultado do Tratamento , Stents/efeitos adversos , Constrição Patológica/etiologia
6.
Pediatr Transplant ; 28(1): e14674, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38054589

RESUMO

INTRODUCTION: In pediatric patients with Budd-Chiari syndrome (BCS), living donor liver transplantation (LDLT) raises substantial challenges regarding IVC reconstruction. CASE PRESENTATION: We present a case of an 8-year-old girl with BCS caused by myeloproliferative syndrome with JAK2 V617F mutation. She had a complete thrombosis of the inferior vena cava (IVC) with multiple collaterals, developing a Budd-Chiari syndrome. She underwent LDLT with IVC reconstruction with a cryopreserved pulmonary vein graft obtained from a provincial biobank. The living donor underwent a laparoscopic-assisted left lateral hepatectomy. The reconstruction of the vena cava took place on the back table and the liver was implanted en bloc with the reconstructed IVC in the recipient. Anticoagulation was immediately restarted after the surgery because of her pro-thrombotic state. Her postoperative course was complicated by a biliary anastomotic leak and an infected biloma. The patient recovered progressively and remained well on outpatient clinic follow-up 32 weeks after the procedure. CONCLUSION: IVC reconstruction using a cryopreserved pulmonary vein graft is a valid option during LDLT for pediatric patients with BCS where reconstruction of the IVC entails considerable challenges. Early referral to a pediatric liver transplant facility with a multidisciplinary team is also important in the management of pediatric patients with BCS.


Assuntos
Síndrome de Budd-Chiari , Transplante de Fígado , Veias Pulmonares , Feminino , Humanos , Criança , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/métodos , Veias Hepáticas/cirurgia , Doadores Vivos , Veia Cava Inferior/cirurgia
9.
Clin J Gastroenterol ; 17(1): 84-92, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37773425

RESUMO

A 24-year-old man was admitted to our hospital with abdominal distension. He was found to have acute liver failure and diagnosed with Budd-Chiari syndrome based on angiography and liver biopsy. Liver transplantation was deemed necessary when angiography showed extensive thrombotic occlusion of the hepatic veins and liver biopsy revealed submassive hepatic necrosis. The patient was found to have the JAK2V617F mutation, indicating a myeloproliferative neoplasm as the background disease. He developed hepatic encephalopathy but remained conscious on on-line hemodiafiltration. Brain-dead donor liver transplantation was performed on hospital day 30. Since then, the patient has remained well.


Assuntos
Síndrome de Budd-Chiari , Falência Hepática Aguda , Transplante de Fígado , Masculino , Humanos , Adulto Jovem , Adulto , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/efeitos adversos , Doadores Vivos , Falência Hepática Aguda/cirurgia , Falência Hepática Aguda/complicações , Encéfalo
10.
Rev Med Interne ; 45(1): 17-25, 2024 Jan.
Artigo em Francês | MEDLINE | ID: mdl-37838484

RESUMO

Splanchnic vein thrombosis includes Budd-Chiari syndrome and portal vein thrombosis. These diseases share common features: (i) they are rare diseases and (ii) they can lead to portal hypertension and its complications. Budd-Chiari syndrome and portal vein thrombosis in the absence of underlying liver disease share many risk factors, the most common being myeloproliferative neoplasms. A rapid and comprehensive workup for thrombosis risk factors is necessary in these patients. Long-term anticoagulation is indicated in most patients. Portal vein thrombosis can also develop in patients with cirrhosis, and is associated with a worse course of cirrhosis. Indications for anticoagulation in patients with cirrhosis are increasing. Transjugular intrahepatic portosystemic shunt is a second-line procedure in this setting. Because of the rarity of these diseases, high-level evidence studies are rare. However, collaborative studies have provided a better understanding of their natural history and allowed to improve the management of these patients. This review focuses on the causes, diagnosis, and management of patients with Budd-Chiari syndrome, patients with portal vein thrombosis without underlying liver disease, and patients with cirrhosis and portal vein thrombosis.


Assuntos
Síndrome de Budd-Chiari , Derivação Portossistêmica Transjugular Intra-Hepática , Trombose , Trombose Venosa , Humanos , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/epidemiologia , Síndrome de Budd-Chiari/etiologia , Veia Porta , Trombose Venosa/complicações , Trombose Venosa/diagnóstico , Trombose Venosa/epidemiologia , Trombose/complicações , Cirrose Hepática/complicações , Cirrose Hepática/diagnóstico , Anticoagulantes/uso terapêutico
11.
Curr Med Res Opin ; 40(2): 303-313, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38006404

RESUMO

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


Assuntos
Síndrome de Budd-Chiari , Hepatopatia Veno-Oclusiva , Hipertensão Portal , Complicações na Gravidez , Feminino , Humanos , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/terapia , Hepatopatia Veno-Oclusiva/complicações , Veias Hepáticas , Hipertensão Portal/complicações
13.
BJU Int ; 133(4): 480-486, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38102752

RESUMO

OBJECTIVE: To present the early results of a new technique for the treatment of renal cell carcinoma with intra-cardiac tumour extension and Budd-Chiari syndrome. PATIENTS AND METHODS: The first stage involves transdiaphragmatic debulking of the right heart, inferior vena cava (IVC) and hepatic veins via median sternotomy, followed by a purse-string suture placed in the IVC below the hepatic veins. The second stage is performed separately and involves en bloc resection of the affected kidney, and IVC and vascular reconstruction via an abdominal incision. RESULTS: Three of five patients presented with clinical Budd-Chiari syndrome; two had radiological features only. The median time between surgical procedures was 12 days (IQR 13 days). Four of the five patients had a R0 resection. While all five patients successfully completed both operative stages, one patient died 22 days after the second stage. Of the remaining four, all survive with no disease recurrence. CONCLUSION: While we continue to compile longer-term data for a larger follow-up series, these preliminary findings show the feasibility of this technique and support the development of this programme of surgery.


Assuntos
Síndrome de Budd-Chiari , Carcinoma de Células Renais , Neoplasias Cardíacas , Neoplasias Renais , Humanos , Síndrome de Budd-Chiari/cirurgia , Síndrome de Budd-Chiari/patologia , Carcinoma de Células Renais/cirurgia , Recidiva Local de Neoplasia , Veia Cava Inferior/cirurgia , Veia Cava Inferior/patologia , Neoplasias Renais/cirurgia
14.
Zhonghua Er Ke Za Zhi ; 62(1): 71-75, 2024 Jan 02.
Artigo em Chinês | MEDLINE | ID: mdl-38154981

RESUMO

Objective: To summarize the clinical features and prognosis of Budd-Chiari syndrome with hepatopulmonary syndrome (HPS) in children. Methods: The clinical data of a child who had Budd-Chiari syndrome with HPS treated at the Department of Pediatrics of the First Affiliated Hospital of Zhengzhou University in December 2016 was analyzed retrospectively. Taking "Budd-Chiari syndrome" and "hepatopulmonary syndrome" in Chinese or English as the keywords, literature was searched at CNKI, Wanfang, China Biomedical Literature Database and PubMed up to July 2023. Combined with this case, the clinical characteristics, diagnosis, treatment and prognosis of Budd-Chiari syndrome with HPS in children under the age of 18 were summarized. Results: A 13-year-old boy, presented with cyanosis and chest tightness after activities for 6 months, and yellow staining of the skin for 1 week. Physical examination at admission not only found mild yellow staining of the skin and sclera, but also found cyanosis of the lips, periocular skin, and extremities. Laboratory examination showed abnormal liver function with total bilirubin 53 µmol/L, direct bilirubin 14 µmol/L, and indirect bilirubin 39 µmol/L, and abnormal blood gas analysis with the partial pressure of oxygen of 54 mmHg (1 mmHg=0.133 kPa), the partial pressure of carbon dioxide of 31 mmHg, and the alveolar-arterial oxygen gradient of 57 mmHg. Hepatic vein-type Budd-Chiari syndrome, cirrhosis, and portal hypertension were indicated by abdominal CT venography. Contrast-enhanced transthoracic echocardiography (CE-TTE) was positive. After symptomatic and supportive treatment, this patient was discharged and received oxygen therapy outside the hospital. At follow-up until March 2023, there was no significant improvement in hypoxemia, accompanied by limited daily activities. Based on the literature, there were 3 reports in English while none in Chinese, 3 cases were reported. Among a total of 4 children, the chief complaints were dyspnea, cyanosis, or hypoxemia in 3 cases, and unknown in 1 case. There were 2 cases diagnosed with Budd-Chiari syndrome with HPS at the same time due to respiratory symptoms, and 2 cases developed HPS 1.5 years and 8.0 years after the diagnosis of Budd-Chiari syndrome respectively. CE-TTE was positive in 2 cases and pulmonary perfusion imaging was positive in 2 cases. Liver transplantation was performed in 2 cases and their respiratory function recovered well; 1 case received oxygen therapy, with no improvement in hypoxemia; 1 case was waiting for liver transplantation. Conclusions: The onset of Budd-Chiari syndrome with HPS is insidious. The most common clinical manifestations are dyspnea and cyanosis. It can reduce misdiagnosis to confirm intrapulmonary vascular dilatations with CE-TTE at an early stage. Liver transplantation is helpful in improving the prognosis.


Assuntos
Síndrome de Budd-Chiari , Síndrome Hepatopulmonar , Masculino , Humanos , Criança , Adolescente , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/diagnóstico , Síndrome de Budd-Chiari/terapia , Síndrome Hepatopulmonar/complicações , Síndrome Hepatopulmonar/diagnóstico , Síndrome Hepatopulmonar/terapia , Estudos Retrospectivos , Hipóxia/complicações , Oxigênio , Dispneia/complicações , Cianose/complicações , Bilirrubina
15.
Indian J Pathol Microbiol ; 66(4): 683-693, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38084516

RESUMO

Liver involvement is commonly seen in various haematological disorders. They present clinically with elevation of liver enzymes and organomegaly, with or without mass lesions. However, liver involvement may be silent in many hematological disorders or there may be specific findings in liver biopsy that can lead to the diagnosis of clinically inapparent hematological disorders. Present review highlights features of hepatic manifestations in various hematological diseases with special emphasis on histopathological findings. Among RBC disorders, secondary hemochromatosis is the commonest among patients with hemolytic anemia; whereas Sickle Cell Hepatopathy is a well known complication in Sickle Cell Disease, characterised by sequestration of sickled RBCs in sinusoids. Vascular complications such as Budd Chiari syndrome and portal venopathy with portal vein thrombosis are seen in patients with myeloproliferative neoplasms. However, sometimes primary hematological disease may remain occult. Various lymphomas show characteristic pattern of hepatic involvement, most common being sinusoidal and portal infiltration. Pattern of infiltration may give clues to different types of lymphomas. Amongst all lymphomas, Diffuse large B cell lymphoma is the most common lymphoma involving liver. Disseminated intravascular coagulation is a fatal systemic condition and liver involvement by widespread fibrin thrombi, is not an exception. Assessing liver histopathology in context of hematological conditions makes better understanding of pathophysiology and progress of these diseases. It is important for hematologists and hepatologist to be aware of possible liver involvement in various hematological diseases presenting with elevated LFTs and have a logical approach to abnormal LFTs.


Assuntos
Síndrome de Budd-Chiari , Linfoma , Transtornos Mieloproliferativos , Trombose , Humanos , Fígado/patologia , Síndrome de Budd-Chiari/etiologia , Trombose/patologia , Linfoma/patologia
16.
Pediatrics ; 152(6)2023 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-38018238

RESUMO

We report the case of a male patient who had a history of early-onset protein-losing enteropathy, chronic diarrhea, and repeated thrombotic events since early childhood. He developed Budd-Chiari syndrome with consequent acute liver failure that required liver transplantation when he was 12 years old. The initial graft failed to function and he required retransplantation. Steroid-resistant rejection complicated the clinical course after the second transplant. Treatment with antithymocyte globulin stabilized graft function but abdominal symptoms and enteral protein loss persisted. The patient remained dependent on intravenous albumin and immunoglobulin. Extended work-up for thrombophilia was unremarkable. Flow cytometry analysis of the peripheral blood cells revealed an unexplained CD55 deficiency. By sequencing of CD55 and, later, exclusion of alternative rare diseases by whole-exome sequencing, we discovered a novel, likely pathogenic homozygous splice-site variant in CD55 c.578 + 5G>A, NM_000574.4, OMIM 125240. The staining of liver and colon biopsies revealed a lack of CD55 protein expression. After initiation of treatment with eculizumab, the patient achieved and has maintained a complete clinical remission throughout 56 months of follow-up. We recommend testing for CD55 deficiency in patients with protein-losing enteropathy. In addition, CD55 deficiency should be considered in the differential diagnosis of patients with Budd-Chiari syndrome in whom an underlying cause is uncertain.


Assuntos
Síndrome de Budd-Chiari , Transplante de Fígado , Enteropatias Perdedoras de Proteínas , Criança , Humanos , Masculino , Anticorpos Monoclonais Humanizados/uso terapêutico , Síndrome de Budd-Chiari/complicações , Síndrome de Budd-Chiari/tratamento farmacológico , Síndrome de Budd-Chiari/cirurgia , Enteropatias Perdedoras de Proteínas/complicações
17.
BMC Gastroenterol ; 23(1): 340, 2023 Oct 02.
Artigo em Inglês | MEDLINE | ID: mdl-37784064

RESUMO

BACKGROUND: Budd-Chiari syndrome (BCS) results when the outflow of the hepatic vein (HV) is obstructed. BCS patients exhibiting an accessory HV (AHV) that is dilated but obstructed can achieve significant alleviation of liver congestion after undergoing AHV recanalization. This meta-analysis was developed to explore the clinical efficacy of AHV recanalization in patients with BCS. MATERIALS AND METHODS: PubMed, Embase, and Wanfang databases were searched for relevant studies published as of November 2022, and RevMan 5.3 and Stata 12.0 were used for pooled endpoint analyses. RESULTS: Twelve total studies were identified for analysis. Pooled primary clinical success, re-stenosis, 1- and 5-year primary patency, 1- and 5-year secondary patency, 1-year overall survival (OS), and 5-year OS rates of patients in these studies following AHV recanalization were 96%, 17%, 91%, 75%, 98%, 91%, 97%, and 96%, respectively. Patients also exhibited a significant reduction in AHV pressure after recanalization relative to preoperative levels (P < 0.00001). Endpoints exhibiting significant heterogeneity among these studies included, AHV pressure (I2 = 95%), 1-year primary patency (I2 = 51.2%), and 5-year primary patency (I2 = 62.4%). Relative to HV recanalization, AHV recanalization was related to a lower rate of re-stenosis (P = 0.002) and longer primary patency (P < 0.00001), but was not associated with any improvements in clinical success (P = 0.88) or OS (P = 0.29) relative to HV recanalization. CONCLUSIONS: The present meta-analysis highlights AHV recanalization as an effective means of achieving positive long-term outcomes in patients affected by BCS, potentially achieving better long-term results than those associated with HV recanalization.


Assuntos
Síndrome de Budd-Chiari , Veias Hepáticas , Humanos , Veias Hepáticas/cirurgia , Síndrome de Budd-Chiari/cirurgia , Constrição Patológica , Estudos Retrospectivos , Resultado do Tratamento
19.
World J Gastroenterol ; 29(33): 4962-4974, 2023 Sep 07.
Artigo em Inglês | MEDLINE | ID: mdl-37731994

RESUMO

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.


Assuntos
Síndrome de Budd-Chiari , Tromboembolia Venosa , Humanos , Estudos Retrospectivos , Anticoagulantes/efeitos adversos , Heparina
20.
Indian J Gastroenterol ; 42(5): 629-641, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37610562

RESUMO

BACKGROUND: Both Budd-Chiari syndrome (BCS) and portal vein thrombosis (PVT) have been linked to various prothrombotic (PT) conditions. The PT profile in Asians is different from the west and there are no nationwide epidemiological surveys from India. Hence, the present meta-analysis was aimed at analyzing the prevalence of acquired and hereditary thrombophilia among Indian patients with non-cirrhotic PVT and BCS. METHODS: A comprehensive literature search of Embase, Medline and Scopus was conducted from January 2000 to February 2022 for studies evaluating the prevalence of various PT conditions in Indian patients with PVT and BCS. Pooled prevalence rates across studies were expressed with summative statistics. RESULTS: Thirty-five studies with 1005 PVT patients and 1391 BCS patients were included in the meta-analysis. At least one PT condition was seen in 46.2% (28.7-63.7) of the PVT patients and 44.9% (37.3-60.7) of the BCS patients. Multiple PT conditions were seen in 13.0% (4.2-21.8) of the PVT patients and 7.9% (3.5-12.4) of the BCS patients. Among PVT patients, hyperhomocysteinemia was the commonest prothrombotic condition (21.6%) followed by protein C (PC) deficiency (10.7%), Janus kinase 2 (JAK-2) mutation (8.5%) and antiphospholipid antibodies (APLA) (7.5%). Among patients with BCS, PC deficiency was the commonest prothrombotic condition (10.6%) followed by methylenetetrahydrofolate reductase (MTHFR) mutation (9.8%), APLA (9.7%) and JAK-2 mutation (9.1%). CONCLUSION: The PT profile in Indian patients with abdominal vein thrombosis is different from that of the western data with a lower prevalence of PT conditions in patients with BCS.


Assuntos
Síndrome de Budd-Chiari , Trombose , Trombose Venosa , Humanos , Síndrome de Budd-Chiari/etiologia , Síndrome de Budd-Chiari/genética , Veia Porta , Trombose Venosa/etiologia , Trombose Venosa/genética , Mutação
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