Background:Porto-sinusoidal vascular disease(PSVD)and portal vein thrombosis(PVT)are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal sys...Background:Porto-sinusoidal vascular disease(PSVD)and portal vein thrombosis(PVT)are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal system.As PVT may be a consequence of PSVD,in PVT patients at presentation,a pre-existing PSVD should be suspected.In these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management,but it could be challenging.In this setting ultrasonography may be valuable in differential diagnosis.The aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and“pure”PVT and then to suspect PVT secondary to a pre-existing PSVD.Methods:Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse(ARFI).Results:ARFI was higher and superior mesenteric vein(SMV)diameter was wider in PSVD patients than in PVT patients.Thus,a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT(the area under the curve=0.780;95%confidence interval:0.690-0.869).Conclusions:A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy.展开更多
Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterizati...Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterization and,clinical outcome.Because of the similarities in clinical presentation and imaging signs,frequently these patients,and particularly those with porto-sinusoidal vascular disease(PSVD),are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis.The most challenging differentiation to be considered is between PSVD and cirrhosis and,although not pathognomonic,liver biopsy is still the standard of diagnosis.Although they still require extended validation before being broadly used,new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease,like transient elastography,contrast-enhanced ultrasound or metabolomic profiling,have shown promising results.Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction,especially now when it is known that 40%of patients suffering from PSVD develop portal vein thrombosis.In this particular case,once the portal vein thrombosis occurred,the diagnosis of PSVD is impossible according to the current guidelines.Moreover,so far,the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances.In this review we highlighted the diagnostic challenges regarding the PSVD,as well as the current techniques used in the evaluation of these patients.展开更多
Porto sinusoidal vascular liver disease (PSVD) and portal vein thrombosis (PVT) are distinct vascular liver diseases characterized, respectively, by an intrahepatic and a prehepatic obstacle to the flow in the liver p...Porto sinusoidal vascular liver disease (PSVD) and portal vein thrombosis (PVT) are distinct vascular liver diseases characterized, respectively, by an intrahepatic and a prehepatic obstacle to the flow in the liver portal system. PVT may also occur as a complication of the natural history of PSVD, especially if a prothrombotic condition coexists. In other cases, it is associated to local and systemic pro-thrombotic conditions, even if its cause remains unknown in up to 25% despite an active search. In our opinion, the presence of PSVD should be suspected in patients with PVT especially in those with PVT “sine causa” and the active search of this condition should be included in their diagnostic work-out. However, sometimes the diagnosis of pre-existing PSVD is very hard. Biopsy cannot be fully discriminant as similar histological data have been described in both conditions. Liver stiffness may help as it has been shown to be higher in PSVD than in “pure” PVT, due to the presence of sclerosis in the portal venous radicles observable in PSVD patients. Nevertheless, comparing liver stiffness between PVT and PSVD has until now been restricted to very limited series of patients. In conclusion, even if it is still totally hypothetical, our point of view may have clinical consequences, especially when deciding to perform a liver biopsy in patients with a higher liver stiffness and suspending the anticoagulation in patients with PVT and no detectable prothrombotic factors.展开更多
The recently published guidelines on screening,monitoring and treatment update previous guidelines published 25 years ago(1,2).This update is timely as much has happened in the interim.Cystic fibrosis(CF)modulator the...The recently published guidelines on screening,monitoring and treatment update previous guidelines published 25 years ago(1,2).This update is timely as much has happened in the interim.Cystic fibrosis(CF)modulator therapy has dramatically improved prognosis for patients with CF,although it is not yet clear whether liver disease is improved.Our understanding of the pathophysiology of CF liver disease has also changed with the recognition of the importance of non-cirrhotic portal hypertension(3,4).Non-invasive tests for fibrosis and elastography have changed the practice of hepatology and reduced the need for liver biopsy.The guideline committee was made up of experts from North America and Europe and included adult and paediatric hepatologists and pulmonologists together with allied health practitioners and representatives of the CF community.A systematic literature search was performed and a vote of 80%was required to adopt a recommendation.The guidelines contain 7 recommendations for screening,13 for disease monitoring and 14 for treatment.展开更多
Cystic fibrosis-associated liver disease(CFLD)is a significant cause of morbidity and mortality affecting people with cystic fibrosis(PwCF)(1).Approximately 40%of PwCF have liver involvement,defined as the existence o...Cystic fibrosis-associated liver disease(CFLD)is a significant cause of morbidity and mortality affecting people with cystic fibrosis(PwCF)(1).Approximately 40%of PwCF have liver involvement,defined as the existence of any hepatic manifestation,including biochemical liver abnormalities(2).In a small percentage of these patients,liver involvement may ultimately result in the development of portal hypertension(PH)and its complications.The presence of at least two of the following variables-abnormal liver tests,abnormal liver ultrasound(US),abnormal physical examination with hepatosplenomegaly or histologic evidence of liver disease-were historically the basis for the European criteria to define CFLD(3).Nevertheless,the emergence of a new approach for liver assessment as liver elastography has led to the proposal of its inclusion in new diagnostic criteria(4).展开更多
OBJECTIVE: To evaluate the value of angiography and cholangiography on the diagnosis and interventional treatment of vascular and biliary complications after liver transplantation. METHODS: Sixteen of 46 patients (15 ...OBJECTIVE: To evaluate the value of angiography and cholangiography on the diagnosis and interventional treatment of vascular and biliary complications after liver transplantation. METHODS: Sixteen of 46 patients (15 men and 1 woman, 17 - 60 years old) after orthotopic liver transplantation received angiography due to abnormal ultrasonography or edema of lower limbs, or cholangiography due to progressing jaundice. Percutaneous transluminal angioplasty or drainage was performed in some patients. RESULTS: Fifteen patients experienced vascular complications and 4 patients had biliary complications. Three of them appeared to have both vascular and biliary complications. Hepatic artery complications were the most common complications (9/16), including hepatic artery thrombosis or stenosis (6/9), bleeding (2/9) and hepatic artery-dissecting aneurysm (1/9). One patient with hepatic artery thrombosis received transcatheter thrombolysis and two patients with bleeding received coil embolization. Inferior vena cava and portal vein stenosis were observed in 6 and 2 patients, respectively. After balloon angioplasty or stent placement, clinical symptoms were alleviated. Biliary complications, including biliary stricture and anastomotic bile leak, occurred in 4 patients. Jaundice decreased after percutaneous transhepatic cholangiography and drainage. CONCLUSIONS: Besides diagnosis, interventional methods include mini-invasive treatment for patients with vascular and biliary complications after liver transplantation. Balloon angiography and stent placement for venous stenosis are useful procedure for the treatment of these problems.展开更多
基金This study was approved by Ethical Committee of The Sapienza University of Rome(5068/2018).
文摘Background:Porto-sinusoidal vascular disease(PSVD)and portal vein thrombosis(PVT)are causes of portal hypertension characterized respectively by an intrahepatic and a pre-hepatic obstacle to the flow in the portal system.As PVT may be a consequence of PSVD,in PVT patients at presentation,a pre-existing PSVD should be suspected.In these patients the identification of an underlying PSVD would have relevant implication regarding follow-up and therapeutic management,but it could be challenging.In this setting ultrasonography may be valuable in differential diagnosis.The aim of the study was to use ultrasonography to identify parameters to discriminate between PSVD and“pure”PVT and then to suspect PVT secondary to a pre-existing PSVD.Methods:Fifty-three patients with histologically proven PSVD and forty-eight patients affected by chronic PVT were enrolled and submitted to abdominal ultrasonography with elastography by acoustic radiation force impulse(ARFI).Results:ARFI was higher and superior mesenteric vein(SMV)diameter was wider in PSVD patients than in PVT patients.Thus,a prognostic score was obtained as linear combinations of the two parameters with a good discrimination capacity between PSVD and PVT(the area under the curve=0.780;95%confidence interval:0.690-0.869).Conclusions:A score based on ARFI and SMV diameter may be useful to suspect an underlying PSVD in patients with PVT and to identify a subgroup of patients to be submitted to liver biopsy.
基金Supported by UEFISCDI,Ministry of Education of Romania,No.PNIII-P1-1.1-PD-2016-0689.
文摘Non-cirrhotic portal hypertension consists of a group of diseases characterized by signs and complications of portal hypertension,which differ from cirrhosis through histological alterations,hemodynamic characterization and,clinical outcome.Because of the similarities in clinical presentation and imaging signs,frequently these patients,and particularly those with porto-sinusoidal vascular disease(PSVD),are misdiagnosed as having liver cirrhosis and thus raising difficulties in their diagnosis.The most challenging differentiation to be considered is between PSVD and cirrhosis and,although not pathognomonic,liver biopsy is still the standard of diagnosis.Although they still require extended validation before being broadly used,new non-invasive methods for the diagnosis of porto-sinusoidal vascular disease,like transient elastography,contrast-enhanced ultrasound or metabolomic profiling,have shown promising results.Another issue is the differentiation between PSVD and chronic extrahepatic portal vein obstruction,especially now when it is known that 40%of patients suffering from PSVD develop portal vein thrombosis.In this particular case,once the portal vein thrombosis occurred,the diagnosis of PSVD is impossible according to the current guidelines.Moreover,so far,the differentiation between PSVD and sinusoidal obstruction syndrome has not been clear so far in particular circumstances.In this review we highlighted the diagnostic challenges regarding the PSVD,as well as the current techniques used in the evaluation of these patients.
文摘Porto sinusoidal vascular liver disease (PSVD) and portal vein thrombosis (PVT) are distinct vascular liver diseases characterized, respectively, by an intrahepatic and a prehepatic obstacle to the flow in the liver portal system. PVT may also occur as a complication of the natural history of PSVD, especially if a prothrombotic condition coexists. In other cases, it is associated to local and systemic pro-thrombotic conditions, even if its cause remains unknown in up to 25% despite an active search. In our opinion, the presence of PSVD should be suspected in patients with PVT especially in those with PVT “sine causa” and the active search of this condition should be included in their diagnostic work-out. However, sometimes the diagnosis of pre-existing PSVD is very hard. Biopsy cannot be fully discriminant as similar histological data have been described in both conditions. Liver stiffness may help as it has been shown to be higher in PSVD than in “pure” PVT, due to the presence of sclerosis in the portal venous radicles observable in PSVD patients. Nevertheless, comparing liver stiffness between PVT and PSVD has until now been restricted to very limited series of patients. In conclusion, even if it is still totally hypothetical, our point of view may have clinical consequences, especially when deciding to perform a liver biopsy in patients with a higher liver stiffness and suspending the anticoagulation in patients with PVT and no detectable prothrombotic factors.
文摘The recently published guidelines on screening,monitoring and treatment update previous guidelines published 25 years ago(1,2).This update is timely as much has happened in the interim.Cystic fibrosis(CF)modulator therapy has dramatically improved prognosis for patients with CF,although it is not yet clear whether liver disease is improved.Our understanding of the pathophysiology of CF liver disease has also changed with the recognition of the importance of non-cirrhotic portal hypertension(3,4).Non-invasive tests for fibrosis and elastography have changed the practice of hepatology and reduced the need for liver biopsy.The guideline committee was made up of experts from North America and Europe and included adult and paediatric hepatologists and pulmonologists together with allied health practitioners and representatives of the CF community.A systematic literature search was performed and a vote of 80%was required to adopt a recommendation.The guidelines contain 7 recommendations for screening,13 for disease monitoring and 14 for treatment.
文摘Cystic fibrosis-associated liver disease(CFLD)is a significant cause of morbidity and mortality affecting people with cystic fibrosis(PwCF)(1).Approximately 40%of PwCF have liver involvement,defined as the existence of any hepatic manifestation,including biochemical liver abnormalities(2).In a small percentage of these patients,liver involvement may ultimately result in the development of portal hypertension(PH)and its complications.The presence of at least two of the following variables-abnormal liver tests,abnormal liver ultrasound(US),abnormal physical examination with hepatosplenomegaly or histologic evidence of liver disease-were historically the basis for the European criteria to define CFLD(3).Nevertheless,the emergence of a new approach for liver assessment as liver elastography has led to the proposal of its inclusion in new diagnostic criteria(4).
文摘OBJECTIVE: To evaluate the value of angiography and cholangiography on the diagnosis and interventional treatment of vascular and biliary complications after liver transplantation. METHODS: Sixteen of 46 patients (15 men and 1 woman, 17 - 60 years old) after orthotopic liver transplantation received angiography due to abnormal ultrasonography or edema of lower limbs, or cholangiography due to progressing jaundice. Percutaneous transluminal angioplasty or drainage was performed in some patients. RESULTS: Fifteen patients experienced vascular complications and 4 patients had biliary complications. Three of them appeared to have both vascular and biliary complications. Hepatic artery complications were the most common complications (9/16), including hepatic artery thrombosis or stenosis (6/9), bleeding (2/9) and hepatic artery-dissecting aneurysm (1/9). One patient with hepatic artery thrombosis received transcatheter thrombolysis and two patients with bleeding received coil embolization. Inferior vena cava and portal vein stenosis were observed in 6 and 2 patients, respectively. After balloon angioplasty or stent placement, clinical symptoms were alleviated. Biliary complications, including biliary stricture and anastomotic bile leak, occurred in 4 patients. Jaundice decreased after percutaneous transhepatic cholangiography and drainage. CONCLUSIONS: Besides diagnosis, interventional methods include mini-invasive treatment for patients with vascular and biliary complications after liver transplantation. Balloon angiography and stent placement for venous stenosis are useful procedure for the treatment of these problems.
