Ascites is one of the major complications of liver cirrhosis and is associated with a poor prognosis. It is important to distinguish noncirrhotic from cirrhotic causes of ascites to guide therapy in patients with nonc...Ascites is one of the major complications of liver cirrhosis and is associated with a poor prognosis. It is important to distinguish noncirrhotic from cirrhotic causes of ascites to guide therapy in patients with noncirrhotic ascites. Mild to moderate ascites is treated by salt restriction and diuretic therapy. The diuretic of choice is spironolactone. A combination treatment with furosemide might be necessary in patients who do not respond to spironolactone alone. Tense ascites is treated by paracentesis, followed by albumin infusion and diuretic therapy. Treatment options for refractory ascites include repeated paracentesis and transjugular intrahepatic portosystemic shunt placement in patients with a preserved liver function. Potential complications of ascites are spontaneous bacterial peritonitis (SBP) and hepatorenal syndrome (HRS). SBP is diagnosed by an ascitic neutrophil count > 250 cells/mm3 and is treated with antibiotics. Patients who survive a first episode of SBP or with a low protein concentration in the ascitic fluid require an antibiotic prophylaxis. The prognosis of untreated HRS type 1 is grave. Treatment consists of a combination of terlipressin and albumin. Hemodialysis might serve in selected patients as a bridging therapy to liver transplantation. Liver transplantation should be considered in all patients with ascites and liver cirrhosis.展开更多
The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morpho...The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.展开更多
More than five decades after it was originally conceptualized as rescue therapy for patients with intractable variceal bleeding,the transjugular intrahepatic portosystemic shunt(TIPS)procedure continues to remain a fo...More than five decades after it was originally conceptualized as rescue therapy for patients with intractable variceal bleeding,the transjugular intrahepatic portosystemic shunt(TIPS)procedure continues to remain a focus of intense clinical and biomedical research.By the impressive reduction in portal pressure achieved by this intervention,coupled with its minimally invasive nature,TIPS has gained increasing acceptance in the treatment of complications of portal hypertension.The early years of TIPS were plagued by poor long-term patency of the stents and increased incidence of hepatic encephalopathy.Moreover,the diversion of portal flow after placement of TIPS often resulted in derangement of hepatic functions,which was occasionally severe.While the incidence of shunt dysfunction has markedly reduced with the advent of covered stents,hepatic encephalopathy and instances of early liver failure continue to remain a significant issue after TIPS.It has emerged over the years that careful selection of patients and diligent post-procedural care is of paramount importance to optimize the outcome after TIPS.The past twenty years have seen multiple studies redefining the role of TIPS in the management of variceal bleeding and refractory ascites while exploring its application in other complications of cirrhosis like hepatic hydrothorax,portal hypertensive gastropathy,ectopic varices,hepatorenal and hepatopulmonary syndromes,non-tumoral portal vein thrombosis and chylous ascites.It has also been utilized to good effect before extrahepatic abdominal surgery to reduce perioperative morbidity and mortality.The current article aims to review the updated literature on the status of TIPS in the management of patients with liver cirrhosis.展开更多
AIM: To compare the liver transplantation-free (LTF) survival rates between patients who underwent transjugular intrahepatic portosystemic shunts (TIPS) and those who underwent paracentesis by an updated meta-analysis...AIM: To compare the liver transplantation-free (LTF) survival rates between patients who underwent transjugular intrahepatic portosystemic shunts (TIPS) and those who underwent paracentesis by an updated meta-analysis that pools the effects of both number of deaths and time to death.展开更多
AIM: To evaluate the effect of the shunting branch of the portal vein (PV) (left or right) and the initial stent position (optimal or suboptimal) of a transjugular intrahepatic portosystemic shunt (TIPS).
Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cau...Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cause is haematopoietic stem cell transplantation （STC） and is also seen after solid organ transplantation. The incidence of veno occlusive disease （VOD） after STC ranges from 0 to 70%, but is decreasing. Survival is good when VOD is a mild form, but when it is severe and associated with an increase of hepatic venous pressure gradient 〉 20 mmHg, and mortality is about 90%. Prevention remains the best therapeutic strategy, by using non-myeloablative conditioning regimens before STC. Prophylactic administration of ursodeoxycholic add, being an antioxidant and antiapoptotic agent, can have some benefit in reducing overall mortality. Defibrotide, which has pro-fibrinolytic and antithrombotic properties, is the most effective therapy; decompression of the sinusoids by a b-ansjugular intrahepatic portosystemic shunt （TIPS） can be tried, especially to treat VOD after liver transplantation and when multiorgan failure （HOF） is not present. Liver transplantation can be the last option, but can not be considered a standard rescue therapy, because usually the concomitant presence of multiorgan failure contraindicates this procedure.展开更多
Background:The purpose of this article was to clarify the optimal management concerning transjugular intrahepatic portosystemic shunts (TIPSs) and surgical shunting in treating portal hypertension.Methods:All database...Background:The purpose of this article was to clarify the optimal management concerning transjugular intrahepatic portosystemic shunts (TIPSs) and surgical shunting in treating portal hypertension.Methods:All databases,including CBM,CNKI,WFPD,Medline,EMBASE,PubMed and Cochrane up to February 2014,were searched for randomized controlled trials (RCTs) comparing TIPS with surgical shunting.Four RCTs,which were extracted by two independent investigators and were evaluated in postoperative complications,mortality,2-and 5-year survival,hospital stay,operating time and hospitalization charges.Results:The morbidity in variceal rehemorrhage was significantly higher in TIPS than in surgical shunts (odds ratio [OR] =7.45,95％confidence interval [CI]:(3.93-14.15),P ＜ 0.00001),the same outcomes were seen in shunt stenosis (OR =20.01,95％ CI:(6.67-59.99),P ＜ 0.000001) and in hepatic encephalopathy (OR =2.50,95％ CI:(1.63-3.84),P ＜ 0.0001).Significantly better 2-year survival (OR =0.66;95％ CI:(0.44-0.98),P =0.04) and 5-year survival (OR =0.44; 95％ CI:(0.30-0.66),P ＜ 0.00001) were seen in patients undergoing surgical shunting compared with TIPS.Conclusions:Compared with TIPS,postoperative complications and survival after surgical shunting were superior for patients with portal hypertension.Application of surgical shunting was recommended for patients rather than TIPS.展开更多
AIM:To compare early use of transjugular intrahepatic portosystemic shunt(TIPS) with endoscopic treatment(ET) for the prophylaxis of recurrent variceal bleeding.METHODS:In-patient data were collected from 190 patients...AIM:To compare early use of transjugular intrahepatic portosystemic shunt(TIPS) with endoscopic treatment(ET) for the prophylaxis of recurrent variceal bleeding.METHODS:In-patient data were collected from 190 patients between January 2007 and June 2010 who suffured from variceal bleeding.Patients who were older than 75 years;previously received surgical treatment or endoscopic therapy for variceal bleeding;and complicated with hepatic encephalopathy or hepatic cancer,were excluded from this research.Thirty-five cases lost to follow-up were also excluded.Retrospective analysis was done in 126 eligible cases.Among them,64 patients received TIPS(TIPS group) while 62 patients received endoscopic therapy(ET group).The relevant data were collected by patient review or telephone calls.The occurrence of rebleeding,hepatic encephalopathy or other complications,survival rateand cost of treatment were compared between the two groups.RESULTS:During the follow-up period(median,20.7 and 18.7 mo in TIPS and ET groups,respectively),rebleeding from any source occurred in 11 patients in the TIPS group as compared with 31 patients in the ET group(Kaplan-Meier analysis and log-rank test,P = 0.000).Rebleeding rates at any time point(6 wk,1 year and 2 year) in the TIPS group were lower than in the ET group(Bonferroni correction α' = α/3).Eight patients in the TIPS group and 16 in the ET group died with the cumulative survival rates of 80.6% and 64.9%(Kaplan-Meier analysis and log-rank test c2 = 4.864,P = 0.02),respectively.There was no significant difference between the two groups with respect to 6-wk survival rates(Bonferroni correction α' = α/3).However,significant differences were observed between the two groups in the 1-year survival rates(92% and 79%) and the 2-year survival rates(89% and 64.9%)(Bonferroni correction α' = α/3).No significant differences were observed between the two treatment groups in the occurrence of hepatic encephalopathy(12 patients in TIPS group and 5 in ET group,KaplanMeier analysis and log-rank test展开更多
文摘Ascites is one of the major complications of liver cirrhosis and is associated with a poor prognosis. It is important to distinguish noncirrhotic from cirrhotic causes of ascites to guide therapy in patients with noncirrhotic ascites. Mild to moderate ascites is treated by salt restriction and diuretic therapy. The diuretic of choice is spironolactone. A combination treatment with furosemide might be necessary in patients who do not respond to spironolactone alone. Tense ascites is treated by paracentesis, followed by albumin infusion and diuretic therapy. Treatment options for refractory ascites include repeated paracentesis and transjugular intrahepatic portosystemic shunt placement in patients with a preserved liver function. Potential complications of ascites are spontaneous bacterial peritonitis (SBP) and hepatorenal syndrome (HRS). SBP is diagnosed by an ascitic neutrophil count > 250 cells/mm3 and is treated with antibiotics. Patients who survive a first episode of SBP or with a low protein concentration in the ascitic fluid require an antibiotic prophylaxis. The prognosis of untreated HRS type 1 is grave. Treatment consists of a combination of terlipressin and albumin. Hemodialysis might serve in selected patients as a bridging therapy to liver transplantation. Liver transplantation should be considered in all patients with ascites and liver cirrhosis.
文摘The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.
文摘More than five decades after it was originally conceptualized as rescue therapy for patients with intractable variceal bleeding,the transjugular intrahepatic portosystemic shunt(TIPS)procedure continues to remain a focus of intense clinical and biomedical research.By the impressive reduction in portal pressure achieved by this intervention,coupled with its minimally invasive nature,TIPS has gained increasing acceptance in the treatment of complications of portal hypertension.The early years of TIPS were plagued by poor long-term patency of the stents and increased incidence of hepatic encephalopathy.Moreover,the diversion of portal flow after placement of TIPS often resulted in derangement of hepatic functions,which was occasionally severe.While the incidence of shunt dysfunction has markedly reduced with the advent of covered stents,hepatic encephalopathy and instances of early liver failure continue to remain a significant issue after TIPS.It has emerged over the years that careful selection of patients and diligent post-procedural care is of paramount importance to optimize the outcome after TIPS.The past twenty years have seen multiple studies redefining the role of TIPS in the management of variceal bleeding and refractory ascites while exploring its application in other complications of cirrhosis like hepatic hydrothorax,portal hypertensive gastropathy,ectopic varices,hepatorenal and hepatopulmonary syndromes,non-tumoral portal vein thrombosis and chylous ascites.It has also been utilized to good effect before extrahepatic abdominal surgery to reduce perioperative morbidity and mortality.The current article aims to review the updated literature on the status of TIPS in the management of patients with liver cirrhosis.
文摘AIM: To compare the liver transplantation-free (LTF) survival rates between patients who underwent transjugular intrahepatic portosystemic shunts (TIPS) and those who underwent paracentesis by an updated meta-analysis that pools the effects of both number of deaths and time to death.
文摘AIM: To evaluate the effect of the shunting branch of the portal vein (PV) (left or right) and the initial stent position (optimal or suboptimal) of a transjugular intrahepatic portosystemic shunt (TIPS).
文摘Hepatic veno-occlusive disease is a clinical syndrome characterized by hepatomegaly, ascites, weight gain and jaundice, due to sinusoidal congestion which can be caused by alkaloid ingestion, but the most frequent cause is haematopoietic stem cell transplantation （STC） and is also seen after solid organ transplantation. The incidence of veno occlusive disease （VOD） after STC ranges from 0 to 70%, but is decreasing. Survival is good when VOD is a mild form, but when it is severe and associated with an increase of hepatic venous pressure gradient 〉 20 mmHg, and mortality is about 90%. Prevention remains the best therapeutic strategy, by using non-myeloablative conditioning regimens before STC. Prophylactic administration of ursodeoxycholic add, being an antioxidant and antiapoptotic agent, can have some benefit in reducing overall mortality. Defibrotide, which has pro-fibrinolytic and antithrombotic properties, is the most effective therapy; decompression of the sinusoids by a b-ansjugular intrahepatic portosystemic shunt （TIPS） can be tried, especially to treat VOD after liver transplantation and when multiorgan failure （HOF） is not present. Liver transplantation can be the last option, but can not be considered a standard rescue therapy, because usually the concomitant presence of multiorgan failure contraindicates this procedure.
文摘Background:The purpose of this article was to clarify the optimal management concerning transjugular intrahepatic portosystemic shunts (TIPSs) and surgical shunting in treating portal hypertension.Methods:All databases,including CBM,CNKI,WFPD,Medline,EMBASE,PubMed and Cochrane up to February 2014,were searched for randomized controlled trials (RCTs) comparing TIPS with surgical shunting.Four RCTs,which were extracted by two independent investigators and were evaluated in postoperative complications,mortality,2-and 5-year survival,hospital stay,operating time and hospitalization charges.Results:The morbidity in variceal rehemorrhage was significantly higher in TIPS than in surgical shunts (odds ratio [OR] =7.45,95％confidence interval [CI]:(3.93-14.15),P ＜ 0.00001),the same outcomes were seen in shunt stenosis (OR =20.01,95％ CI:(6.67-59.99),P ＜ 0.000001) and in hepatic encephalopathy (OR =2.50,95％ CI:(1.63-3.84),P ＜ 0.0001).Significantly better 2-year survival (OR =0.66;95％ CI:(0.44-0.98),P =0.04) and 5-year survival (OR =0.44; 95％ CI:(0.30-0.66),P ＜ 0.00001) were seen in patients undergoing surgical shunting compared with TIPS.Conclusions:Compared with TIPS,postoperative complications and survival after surgical shunting were superior for patients with portal hypertension.Application of surgical shunting was recommended for patients rather than TIPS.
文摘AIM:To compare early use of transjugular intrahepatic portosystemic shunt(TIPS) with endoscopic treatment(ET) for the prophylaxis of recurrent variceal bleeding.METHODS:In-patient data were collected from 190 patients between January 2007 and June 2010 who suffured from variceal bleeding.Patients who were older than 75 years;previously received surgical treatment or endoscopic therapy for variceal bleeding;and complicated with hepatic encephalopathy or hepatic cancer,were excluded from this research.Thirty-five cases lost to follow-up were also excluded.Retrospective analysis was done in 126 eligible cases.Among them,64 patients received TIPS(TIPS group) while 62 patients received endoscopic therapy(ET group).The relevant data were collected by patient review or telephone calls.The occurrence of rebleeding,hepatic encephalopathy or other complications,survival rateand cost of treatment were compared between the two groups.RESULTS:During the follow-up period(median,20.7 and 18.7 mo in TIPS and ET groups,respectively),rebleeding from any source occurred in 11 patients in the TIPS group as compared with 31 patients in the ET group(Kaplan-Meier analysis and log-rank test,P = 0.000).Rebleeding rates at any time point(6 wk,1 year and 2 year) in the TIPS group were lower than in the ET group(Bonferroni correction α' = α/3).Eight patients in the TIPS group and 16 in the ET group died with the cumulative survival rates of 80.6% and 64.9%(Kaplan-Meier analysis and log-rank test c2 = 4.864,P = 0.02),respectively.There was no significant difference between the two groups with respect to 6-wk survival rates(Bonferroni correction α' = α/3).However,significant differences were observed between the two groups in the 1-year survival rates(92% and 79%) and the 2-year survival rates(89% and 64.9%)(Bonferroni correction α' = α/3).No significant differences were observed between the two treatment groups in the occurrence of hepatic encephalopathy(12 patients in TIPS group and 5 in ET group,KaplanMeier analysis and log-rank test
