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Budd-Chiari Syndrome (BCS) refers to a posterior hepatic hilum caused by occlusion of the hepatic vein and its opening above the superior segment of the inferior vena cava, often accompanied by inferior vena cava hypertension.
arterial hypertension
.
Primary BCS refers to the presence of venous thrombosis, and secondary BCS refers to mechanical obstruction due to malignant tumors, abscesses, cysts,
etc.
This review will focus on the management of primary BCS – a step-by-step approach
.
Compiled and organized by Yimaitong, please do not reprint without authorization
.
Overview Primary BCS is often the result of a combination of risk factors, the most common being hereditary and acquired thrombophilia
.
Because of the rarity of the disease, high clinical suspicion must be maintained, especially in young adult patients with new-onset ascites, hepatomegaly, or an enlarged caudate lobe
.
Despite the lack of high-quality evidence guided by randomized controlled trials, progress has been made in the management of BCS with individualized, stepwise, multidisciplinary strategies including anticoagulation and other medical therapy, endovascular interventions, transjugular intrahepatic portal Body shunts (TIPSs), surgical shunts and liver transplantation
.
Step-by-step multidisciplinary management in an individualized manner is the recommended approach for BCS treatment
.
The main goals of treatment include improving symptoms and signs of portal hypertension and protecting liver function
.
Anticoagulation and Drug Therapy Review In the past, despite the lack of relevant studies to clarify the optimal intensity of anticoagulation therapy, anticoagulation therapy with heparin and vitamin K antagonists is still recommended for BCS patients
.
Low molecular weight heparin (LMWH) anticoagulation therapy is recommended to be initiated immediately after diagnosis and titrated to anti-factor Xa activity of 0.
5 IU/mL-0.
8 IU/mL
.
In 43 consecutive patients receiving warfarin, the target INR ranged from 2 to 3, and the follow-up results at 23 months were ascites resolution and liver normalization in over 60% of patients
.
Although not yet approved, direct oral anticoagulants (DOACs) have been used to treat well-indicated patients with BCS, which may be a possibility in patients who have experienced recurrent thrombosis at therapeutic levels of warfarin and/or low molecular weight heparin means another option
.
A recent study compared the outcomes of DOACs in patients with acute venous embolism in atypical sites (such as splanchnic veins) and patients with typical sites (deep veins of extremities/pulmonary embolism), and the study showed that the rates of thrombus recurrence and major bleeding were similar between the two, suggesting that DOACs treatment May be as effective on visceral thrombosis as other thrombosis
.
However, more research is needed before the standard can be used
.
Systemic thrombolysis with recombinant tissue plasminogen activator has been used in rare cases, but there is little evidence of its use
.
Systemic thrombolysis as the mainstay of treatment for BCS may be limited to situations where the thrombus burden is too high to allow for other endovascular or surgical treatment
.
Furthermore, systemic thrombolysis is unlikely to benefit patients with chronic BCS
.
Locally delivered thrombolytics may increase the likelihood of recanalization if acute thrombosis is caused or exacerbated by endovascular devices
.
Patients with BCS may experience massive bleeding due to the use of anticoagulants
.
Bleeding is associated with the invasive procedures of BCS treatment, with the most common bleeding being portal hypertensive, non-portal hypertensive gastrointestinal bleeding, and genital bleeding
.
The severity of BCS correlates with prognosis after a bleeding event
.
For patients with myeloproliferative disease, cytoreductive therapy may reduce the risk of thrombotic recurrence by addressing the underlying cause
.
Hydroxyurea is the first-line treatment for potential patients with polycythemia vera and essential thrombocythemia
.
Ruxolitinib may be considered in patients with polycythemia vera or in patients with myelofibrosis who are unresponsive or intolerant to hydroxyurea
.
In guiding the treatment of underlying diseases in patients with myeloproliferative diseases, the participation of hematologists is particularly important
.
An important aspect of initial management of patients with BCS is managing complications of portal hypertension, such as ascites, encephalopathy, renal insufficiency, and gastrointestinal bleeding
.
Patients should be screened for gastroesophageal varices and, if found, prophylaxis with nonselective beta-blockers
.
For noncirrhotic portal hypertension patients with variceal bleeding, secondary prevention measures include beta-blockers and endoscopic variceal ligation
.
Percutaneous Angioplasty and Stenting Endovascular approaches, such as angioplasty and stenting, have been widely used over the past 20 years
.
The use of high-quality diagnostic imaging to help guide interventional approaches is encouraged
.
Angioplasty is usually performed when a short stenosis of the hepatic vein or inferior vena cava is suspected
.
Angioplasty can be combined with placement of permanent or retrievable stents, especially in the presence of inferior vena cava occlusion
.
Results of a recently published randomized trial showed that compared with angioplasty alone, patients with angioplasty combined with a stent had higher patency (98% vs.
60%) and better restenosis-free survival at 3 years (90% vs.
60.
4%)
.
Although the combination of angioplasty and local thrombolysis with streptokinase or urokinase has an unstable effect and is associated with a high incidence of major bleeding, it can be used in rare cases and the benefit of local thrombolysis may be limited to endovascular procedures After acute recurrence of thrombosis
.
Transjugular intrahepatic portosystemic shunts involve the establishment of a portosystemic shunt.
Surgical options include side-to-side portocaval shunts and combined side-to-side portocaval and vena cava shunts
.
The type of surgery performed depends on the type and location of the blocked vein
.
With the widespread use of transjugular intrahepatic portosystemic shunts and endovascular therapy, this surgical approach is less common
.
Additional tests include angiography and portal pressure gradient measurements to determine whether portal shunt is feasible
.
Surgical recanalization is more common in patients with inferior vena cava occlusion and/or rupture of the occlusive membrane or reconstruction of the inferior vena cava
.
Surgery has greater procedural risks than staged percutaneous treatment
.
Surgical shunting may become less common as experience with percutaneous and minimally invasive endovascular treatment develops
.
Liver transplantation should be considered when hepatic decompensation is severe, progressive, or fails percutaneous or surgical treatment
.
For patients with underlying myeloproliferative disease, post-transplant treatment with hydroxyurea and aspirin is recommended to reduce the likelihood of thrombotic recurrence while avoiding the risk of anticoagulation-related bleeding
.
Liver transplantation When fulminant hepatic failure or decompensated cirrhosis with BCS causes progressive hepatic decompensation, liver transplantation is the last resort
.
Although BCS is a rare indication for liver transplantation, treatment outcomes are favorable and have greatly improved over time
.
A recent study showed no significant difference in long-term survival after transplantation in BCS patients with or without underlying myeloproliferative disease
.
A recent prognostic analysis of BCS patients after liver transplantation showed that the 10-year survival rate of patients was 68%-84%
.
Guidelines for the treatment of Budd-Chiari syndrome are provided in the table below
.
(Click to view larger image) Summary Treatment of BCS usually requires multidisciplinary, individualized, step-by-step management
.
Despite attempts at endovascular recanalization or surgical shunting, liver transplantation is the last option for patients with BCS unresponsive to medical therapy or with persistent/progressive hepatic decompensation
.
Clinicians need to maintain a high degree of suspicion for BCS and should consider typical features, including ascites, hepatomegaly, or enlarged caudate lobe, and timely recognition is the key to preventing and managing critical illness
.
Compiled by: Lamia YK Haque, Joseph K.
Lim.
Budd-Chiari Syndrome An Uncommon Cause of Chronic Liver Disease that Cannot Be Missed .
Clinics in Liver Disease.
VOLUME 24, ISSUE 3, P453-481, AUGUST 01, 2020 .
DOI: https://doi.
org/10.
1016/j.
cld.
2020.
04.
012
arterial hypertension
.
Primary BCS refers to the presence of venous thrombosis, and secondary BCS refers to mechanical obstruction due to malignant tumors, abscesses, cysts,
etc.
This review will focus on the management of primary BCS – a step-by-step approach
.
Compiled and organized by Yimaitong, please do not reprint without authorization
.
Overview Primary BCS is often the result of a combination of risk factors, the most common being hereditary and acquired thrombophilia
.
Because of the rarity of the disease, high clinical suspicion must be maintained, especially in young adult patients with new-onset ascites, hepatomegaly, or an enlarged caudate lobe
.
Despite the lack of high-quality evidence guided by randomized controlled trials, progress has been made in the management of BCS with individualized, stepwise, multidisciplinary strategies including anticoagulation and other medical therapy, endovascular interventions, transjugular intrahepatic portal Body shunts (TIPSs), surgical shunts and liver transplantation
.
Step-by-step multidisciplinary management in an individualized manner is the recommended approach for BCS treatment
.
The main goals of treatment include improving symptoms and signs of portal hypertension and protecting liver function
.
Anticoagulation and Drug Therapy Review In the past, despite the lack of relevant studies to clarify the optimal intensity of anticoagulation therapy, anticoagulation therapy with heparin and vitamin K antagonists is still recommended for BCS patients
.
Low molecular weight heparin (LMWH) anticoagulation therapy is recommended to be initiated immediately after diagnosis and titrated to anti-factor Xa activity of 0.
5 IU/mL-0.
8 IU/mL
.
In 43 consecutive patients receiving warfarin, the target INR ranged from 2 to 3, and the follow-up results at 23 months were ascites resolution and liver normalization in over 60% of patients
.
Although not yet approved, direct oral anticoagulants (DOACs) have been used to treat well-indicated patients with BCS, which may be a possibility in patients who have experienced recurrent thrombosis at therapeutic levels of warfarin and/or low molecular weight heparin means another option
.
A recent study compared the outcomes of DOACs in patients with acute venous embolism in atypical sites (such as splanchnic veins) and patients with typical sites (deep veins of extremities/pulmonary embolism), and the study showed that the rates of thrombus recurrence and major bleeding were similar between the two, suggesting that DOACs treatment May be as effective on visceral thrombosis as other thrombosis
.
However, more research is needed before the standard can be used
.
Systemic thrombolysis with recombinant tissue plasminogen activator has been used in rare cases, but there is little evidence of its use
.
Systemic thrombolysis as the mainstay of treatment for BCS may be limited to situations where the thrombus burden is too high to allow for other endovascular or surgical treatment
.
Furthermore, systemic thrombolysis is unlikely to benefit patients with chronic BCS
.
Locally delivered thrombolytics may increase the likelihood of recanalization if acute thrombosis is caused or exacerbated by endovascular devices
.
Patients with BCS may experience massive bleeding due to the use of anticoagulants
.
Bleeding is associated with the invasive procedures of BCS treatment, with the most common bleeding being portal hypertensive, non-portal hypertensive gastrointestinal bleeding, and genital bleeding
.
The severity of BCS correlates with prognosis after a bleeding event
.
For patients with myeloproliferative disease, cytoreductive therapy may reduce the risk of thrombotic recurrence by addressing the underlying cause
.
Hydroxyurea is the first-line treatment for potential patients with polycythemia vera and essential thrombocythemia
.
Ruxolitinib may be considered in patients with polycythemia vera or in patients with myelofibrosis who are unresponsive or intolerant to hydroxyurea
.
In guiding the treatment of underlying diseases in patients with myeloproliferative diseases, the participation of hematologists is particularly important
.
An important aspect of initial management of patients with BCS is managing complications of portal hypertension, such as ascites, encephalopathy, renal insufficiency, and gastrointestinal bleeding
.
Patients should be screened for gastroesophageal varices and, if found, prophylaxis with nonselective beta-blockers
.
For noncirrhotic portal hypertension patients with variceal bleeding, secondary prevention measures include beta-blockers and endoscopic variceal ligation
.
Percutaneous Angioplasty and Stenting Endovascular approaches, such as angioplasty and stenting, have been widely used over the past 20 years
.
The use of high-quality diagnostic imaging to help guide interventional approaches is encouraged
.
Angioplasty is usually performed when a short stenosis of the hepatic vein or inferior vena cava is suspected
.
Angioplasty can be combined with placement of permanent or retrievable stents, especially in the presence of inferior vena cava occlusion
.
Results of a recently published randomized trial showed that compared with angioplasty alone, patients with angioplasty combined with a stent had higher patency (98% vs.
60%) and better restenosis-free survival at 3 years (90% vs.
60.
4%)
.
Although the combination of angioplasty and local thrombolysis with streptokinase or urokinase has an unstable effect and is associated with a high incidence of major bleeding, it can be used in rare cases and the benefit of local thrombolysis may be limited to endovascular procedures After acute recurrence of thrombosis
.
Transjugular intrahepatic portosystemic shunts involve the establishment of a portosystemic shunt.
Surgical options include side-to-side portocaval shunts and combined side-to-side portocaval and vena cava shunts
.
The type of surgery performed depends on the type and location of the blocked vein
.
With the widespread use of transjugular intrahepatic portosystemic shunts and endovascular therapy, this surgical approach is less common
.
Additional tests include angiography and portal pressure gradient measurements to determine whether portal shunt is feasible
.
Surgical recanalization is more common in patients with inferior vena cava occlusion and/or rupture of the occlusive membrane or reconstruction of the inferior vena cava
.
Surgery has greater procedural risks than staged percutaneous treatment
.
Surgical shunting may become less common as experience with percutaneous and minimally invasive endovascular treatment develops
.
Liver transplantation should be considered when hepatic decompensation is severe, progressive, or fails percutaneous or surgical treatment
.
For patients with underlying myeloproliferative disease, post-transplant treatment with hydroxyurea and aspirin is recommended to reduce the likelihood of thrombotic recurrence while avoiding the risk of anticoagulation-related bleeding
.
Liver transplantation When fulminant hepatic failure or decompensated cirrhosis with BCS causes progressive hepatic decompensation, liver transplantation is the last resort
.
Although BCS is a rare indication for liver transplantation, treatment outcomes are favorable and have greatly improved over time
.
A recent study showed no significant difference in long-term survival after transplantation in BCS patients with or without underlying myeloproliferative disease
.
A recent prognostic analysis of BCS patients after liver transplantation showed that the 10-year survival rate of patients was 68%-84%
.
Guidelines for the treatment of Budd-Chiari syndrome are provided in the table below
.
(Click to view larger image) Summary Treatment of BCS usually requires multidisciplinary, individualized, step-by-step management
.
Despite attempts at endovascular recanalization or surgical shunting, liver transplantation is the last option for patients with BCS unresponsive to medical therapy or with persistent/progressive hepatic decompensation
.
Clinicians need to maintain a high degree of suspicion for BCS and should consider typical features, including ascites, hepatomegaly, or enlarged caudate lobe, and timely recognition is the key to preventing and managing critical illness
.
Compiled by: Lamia YK Haque, Joseph K.
Lim.
Budd-Chiari Syndrome An Uncommon Cause of Chronic Liver Disease that Cannot Be Missed .
Clinics in Liver Disease.
VOLUME 24, ISSUE 3, P453-481, AUGUST 01, 2020 .
DOI: https://doi.
org/10.
1016/j.
cld.
2020.
04.
012
