Scant information exists on the role of thrombophilia in extrahepatic portal vein obstruction (EHPVO). We studied 65 patients with EHPVO, 500 with deep vein thrombosis (DVT) of the lower limbs, and 700 healthy control...Scant information exists on the role of thrombophilia in extrahepatic portal vein obstruction (EHPVO). We studied 65 patients with EHPVO, 500 with deep vein thrombosis (DVT) of the lower limbs, and 700 healthy controls referred for throm bophilia screening, including the search for gain- of- function mutations in g enes encoding coagulation factor V(factor V Leiden) and prothrombin (prothrombin G20210A); antithrombin, protein C, and protein S deficiency; and hyperhomocyste inemia. At least one abnormality in the thrombophilia screening was found in 40 % of patients with either EHPVO or lower limb DVT and in 13% of controls, fo r odds ratios of 4.0 (95% CI, 2.3- 7.0) and 4.4 (95% CI, 3.3- 5.9), respec tively. Statistically significant associations with EHPVO were observed for the prothrombin G20210A mutation (odds ratio, 8.1; 95% CI, 3.8- 17.5) and the def iciencies of antithrombin, protein C, or protein S taken together (odds ratio, 4 .5; 95% CI, 1.1- 18.0). The odds ratio for the prothrombin G20210A was approximately twice that for lower limb DVT. Patients with factor V Leiden had an odds ratio for EHPVO of 0.8 (95% CI, 0.1- 6.4) and for lower limb DVT of 7.5 (95% CI, 4.4- 13.0). The odds ratio for EHPVO in patients wit h hyperhomocysteinemia was 2.0 (95% CI, 0.9- 4.9). At variance with lower lim b DVT, oral contraceptive use was not associated with an increased risk of EHPVO . Myeloproliferative disorders were diagnosed in 35% of patients with EHPVO. I n conclusion, the risk for EHPVO is increased in the presence of thrombophilia r esulting from the prothrombin G20210A mutation and from the deficiencies of the naturally occurring anticoagulant proteins, but not from factor V Leiden.展开更多
Background and study aims: Figures for the prevalence of anorectal varices and portal hypertensive colopathy have varied considerably in the available studies of patients with cirrhosis, and few studies have observed ...Background and study aims: Figures for the prevalence of anorectal varices and portal hypertensive colopathy have varied considerably in the available studies of patients with cirrhosis, and few studies have observed these changes in patients with extrahepatic portal vein obstruction (EHPVO). Our aim was to investigate the colonic changes, if any, in patients with EHPVO and to compare them with those seen in patients with cirrhosis of the liver. Patients and methods: A total of 50 patients with cirrhosis and 35 patients with EHPVO, all of whom had a history of at least one episode of bleeding from esophageal varices, underwent both upper gastrointestinal endoscopy and colonoscopy. Results: Anorectal varices were seen more commonly in patients with EHPVO than in patients with cirrhosis (63%vs. 38%, P < 0.03). Of the patients with anorectal varices, large anorectal varices were also more common in patients with EHPVO than in patients with cirrhosis (73%vs. 32%, P < 0.01). Colopathy was noted in 40%of patients with EHPVO and in 62%of patients with cirrhosis (P< 0.05). Of the patients with EHPVO, colopathy was noted in 27%of patients who also had anorectal varices and in 61.5%of patients without anorectal varices (P < 0.05). Similarly, patients with both cirrhosis and anorectal varices were found to have a lower prevalence of colopathy than cirrhotic patients without anorectal varices (42%vs. 74%, P < 0.03). Conclusions: Anorectal varices are more common in patients with EHPVO, while portal hypertensive colopathy is more common in patients with cirrhosis. Large anorectal varices are also more common in patients with EHPVO than in patients with cirrhosis and there is an inverse relationship between anorectal varices and colopathy in both cirrhotic patients and patients with EHPVO.展开更多
Endoscopic sclerotherapy is a well-established treatment for bleeding esophageal varices, although it has a substantial complication rate. A prospective randomized trial was conducted to determine whether endoscopic v...Endoscopic sclerotherapy is a well-established treatment for bleeding esophageal varices, although it has a substantial complication rate. A prospective randomized trial was conducted to determine whether endoscopic variceal ligation is safer and more effective than sclerotherapy in adults with bleeding esophageal varices because of extrahepatic portal venous obstruction. Thirty-six patients underwent sclerotherapy and 37 had band ligation. Ligation and sclerotherapy were equally effective for achieving variceal eradication (94.6%vs. 91.7%, respectively; p = 0.67). However, ligation achieved eradication with fewer endoscopic sessions (3.7 [1.2] vs. 7.7 [3.3]; p < 0.0001) and within a shorter time interval (50.1 [17.7] days vs. 99 [54.8]-days; p< 0.0001). In the ligation group, recurrent bleeding was less frequent (2.7%vs. 19.4%; p = 0.028; however, Bonferroni correction for multiple testing removes this significance) and the rate of major complications was lower (2.7%vs. 22.2%; p = 0.014). Total cost per patient was significantly higher in the sclerotherapy vs. the ligation group ($216.6 [71.8] vs. $182.6 [63.4]; p = 0.035). During the follow-up period after variceal eradication, no significant differences were found between the sclerotherapy and the ligation groups with respect to recurrent bleeding (3%vs. 2.9%; p = 1.0), esophageal variceal recurrence (9.1%vs. 11.4%; p = 1.0), and formation of new gastric varices (9.1%vs. 14.3%; p = 0.51). Variceal band ligation is superior to sclerotherapy, because it is less costly and achieves variceal eradication more quickly, with lower relative frequencies of recurrent variceal bleeding and complications.展开更多
Although endoscopic band ligation (EVL) plus sclerotherapy (EST) has shown to be superior to any individualmethod, there is no study in children. We therefore analyzed our experience of EST and EVL+ EST in childrenwit...Although endoscopic band ligation (EVL) plus sclerotherapy (EST) has shown to be superior to any individualmethod, there is no study in children. We therefore analyzed our experience of EST and EVL+ EST in childrenwith extrahepatic portal venous obstruction (EHPVO). Over a period of 8 years, 136 children who presented with a history of recent variceal bleeding due to EHPVO were studied; 30 of them received EVL + EST and 106 received EST alone. In the EVL + EST group, after the first session of EVL, EST was done three times weekly until variceal eradication was achieved. The mean age of these children was 7 ± 3.6 years with a male- to- female ratio of 2.6∶ 1. The rate of eradication was comparable in both groups (100% in EVL + EST and 96% in EST). However, EVL + EST group required a significantly fewer sessions and lower volume of sclerosant (2 ± 1 vs. 4.4 ± 2 sessions, P< 0.001 and 3.1 ± 2 mL versus 7.5 ± 4 mL, P< 0.001 respectively). There were significantly less complications in EVL + EST group (10% vs. 36% , P< 0.01). Over a mean follow- up of 27 months, varices recurred in 6.6% and 10% cases, respectively. EVL + EST is a better method in the treatment of esophageal varices in children with EHPVO than EST alone, as it required fewer session and had fewer complication.展开更多
文摘Scant information exists on the role of thrombophilia in extrahepatic portal vein obstruction (EHPVO). We studied 65 patients with EHPVO, 500 with deep vein thrombosis (DVT) of the lower limbs, and 700 healthy controls referred for throm bophilia screening, including the search for gain- of- function mutations in g enes encoding coagulation factor V(factor V Leiden) and prothrombin (prothrombin G20210A); antithrombin, protein C, and protein S deficiency; and hyperhomocyste inemia. At least one abnormality in the thrombophilia screening was found in 40 % of patients with either EHPVO or lower limb DVT and in 13% of controls, fo r odds ratios of 4.0 (95% CI, 2.3- 7.0) and 4.4 (95% CI, 3.3- 5.9), respec tively. Statistically significant associations with EHPVO were observed for the prothrombin G20210A mutation (odds ratio, 8.1; 95% CI, 3.8- 17.5) and the def iciencies of antithrombin, protein C, or protein S taken together (odds ratio, 4 .5; 95% CI, 1.1- 18.0). The odds ratio for the prothrombin G20210A was approximately twice that for lower limb DVT. Patients with factor V Leiden had an odds ratio for EHPVO of 0.8 (95% CI, 0.1- 6.4) and for lower limb DVT of 7.5 (95% CI, 4.4- 13.0). The odds ratio for EHPVO in patients wit h hyperhomocysteinemia was 2.0 (95% CI, 0.9- 4.9). At variance with lower lim b DVT, oral contraceptive use was not associated with an increased risk of EHPVO . Myeloproliferative disorders were diagnosed in 35% of patients with EHPVO. I n conclusion, the risk for EHPVO is increased in the presence of thrombophilia r esulting from the prothrombin G20210A mutation and from the deficiencies of the naturally occurring anticoagulant proteins, but not from factor V Leiden.
文摘Background and study aims: Figures for the prevalence of anorectal varices and portal hypertensive colopathy have varied considerably in the available studies of patients with cirrhosis, and few studies have observed these changes in patients with extrahepatic portal vein obstruction (EHPVO). Our aim was to investigate the colonic changes, if any, in patients with EHPVO and to compare them with those seen in patients with cirrhosis of the liver. Patients and methods: A total of 50 patients with cirrhosis and 35 patients with EHPVO, all of whom had a history of at least one episode of bleeding from esophageal varices, underwent both upper gastrointestinal endoscopy and colonoscopy. Results: Anorectal varices were seen more commonly in patients with EHPVO than in patients with cirrhosis (63%vs. 38%, P < 0.03). Of the patients with anorectal varices, large anorectal varices were also more common in patients with EHPVO than in patients with cirrhosis (73%vs. 32%, P < 0.01). Colopathy was noted in 40%of patients with EHPVO and in 62%of patients with cirrhosis (P< 0.05). Of the patients with EHPVO, colopathy was noted in 27%of patients who also had anorectal varices and in 61.5%of patients without anorectal varices (P < 0.05). Similarly, patients with both cirrhosis and anorectal varices were found to have a lower prevalence of colopathy than cirrhotic patients without anorectal varices (42%vs. 74%, P < 0.03). Conclusions: Anorectal varices are more common in patients with EHPVO, while portal hypertensive colopathy is more common in patients with cirrhosis. Large anorectal varices are also more common in patients with EHPVO than in patients with cirrhosis and there is an inverse relationship between anorectal varices and colopathy in both cirrhotic patients and patients with EHPVO.
文摘Endoscopic sclerotherapy is a well-established treatment for bleeding esophageal varices, although it has a substantial complication rate. A prospective randomized trial was conducted to determine whether endoscopic variceal ligation is safer and more effective than sclerotherapy in adults with bleeding esophageal varices because of extrahepatic portal venous obstruction. Thirty-six patients underwent sclerotherapy and 37 had band ligation. Ligation and sclerotherapy were equally effective for achieving variceal eradication (94.6%vs. 91.7%, respectively; p = 0.67). However, ligation achieved eradication with fewer endoscopic sessions (3.7 [1.2] vs. 7.7 [3.3]; p < 0.0001) and within a shorter time interval (50.1 [17.7] days vs. 99 [54.8]-days; p< 0.0001). In the ligation group, recurrent bleeding was less frequent (2.7%vs. 19.4%; p = 0.028; however, Bonferroni correction for multiple testing removes this significance) and the rate of major complications was lower (2.7%vs. 22.2%; p = 0.014). Total cost per patient was significantly higher in the sclerotherapy vs. the ligation group ($216.6 [71.8] vs. $182.6 [63.4]; p = 0.035). During the follow-up period after variceal eradication, no significant differences were found between the sclerotherapy and the ligation groups with respect to recurrent bleeding (3%vs. 2.9%; p = 1.0), esophageal variceal recurrence (9.1%vs. 11.4%; p = 1.0), and formation of new gastric varices (9.1%vs. 14.3%; p = 0.51). Variceal band ligation is superior to sclerotherapy, because it is less costly and achieves variceal eradication more quickly, with lower relative frequencies of recurrent variceal bleeding and complications.
文摘Although endoscopic band ligation (EVL) plus sclerotherapy (EST) has shown to be superior to any individualmethod, there is no study in children. We therefore analyzed our experience of EST and EVL+ EST in childrenwith extrahepatic portal venous obstruction (EHPVO). Over a period of 8 years, 136 children who presented with a history of recent variceal bleeding due to EHPVO were studied; 30 of them received EVL + EST and 106 received EST alone. In the EVL + EST group, after the first session of EVL, EST was done three times weekly until variceal eradication was achieved. The mean age of these children was 7 ± 3.6 years with a male- to- female ratio of 2.6∶ 1. The rate of eradication was comparable in both groups (100% in EVL + EST and 96% in EST). However, EVL + EST group required a significantly fewer sessions and lower volume of sclerosant (2 ± 1 vs. 4.4 ± 2 sessions, P< 0.001 and 3.1 ± 2 mL versus 7.5 ± 4 mL, P< 0.001 respectively). There were significantly less complications in EVL + EST group (10% vs. 36% , P< 0.01). Over a mean follow- up of 27 months, varices recurred in 6.6% and 10% cases, respectively. EVL + EST is a better method in the treatment of esophageal varices in children with EHPVO than EST alone, as it required fewer session and had fewer complication.