Seventeen cirrhotics with refractory ascites were treated with transjugular intrahepatic portosystemic shunt (TIPS) and followed for 15.5 +/- 3.4 months. Five patients died, four within 3 months after TIPS (hepatocellular failure) and one after 22 months (cholangiocarcinoma). Six patients received transplants 1 to 10 months after the procedure. Actuarial survival at 6, 12, and 24 months was 75%, 75%, and 63%, respectively. Portosystemic venous pressure gradient decreased by 46% at 1 month and by 38% at 7 to 12 months. Eight patients presented 18 stenoses 1 to 18 months after TIPS. Twelve stenoses required balloon dilatation. Tense ascites was present before TIPS in 100% of the patients, whereas it was mild or absent in 56% at 1 month, in 66% at 3 to 6 months, in 57% at 7 to 12 months, and in 100% at 24 months after TIPS. Requirements for diuretics and paracentesis decreased after TIPS (P < .001, both). One month after TIPS, urinary and fractional sodium excretion increased (P < .001, both), plasma renin activity, plasma aldosterone (P < .005, both), and plasma norepinephrine (P < .05) decreased and cardiac output (P < .01) increased, systemic vascular resistances (P < .005) decreased, and arterial pressure did not change. Acute hepatic encephalopathy was frequent early after TIPS but was responsive to treatment and caused no long-term disability. In conclusion, TIPS is useful in the treatment of refractory ascites through lowering portal pressure and improving renal sodium excretion. This effect could be attributable to an increase in effective blood volume causing deactivation of vasopressor systems.

The molecular basis of Wilson disease (WD), an autosomal recessive disorder, is the presence of mutations in the ATP7B gene, a copper transporting ATPase. Hospital records indicated a higher prevalence of WD (1 in 2,600) in some counties in the northeastern region of the island of Gran Canaria (Canary Islands, Spain) that was around 10-fold higher than that described for European populations (1 in 30,000). The ATP7B gene was analyzed for mutations in 24 affected subjects, revealing a high prevalence of the rare Leu708Pro mutation present in 12 homozygous and 7 heterozygous individuals. In these patients, who constitute one of the largest described cohorts of WD homozygotes, we found a variable clinical presentation of the disease, although the biochemical picture was homogenous and characteristic, thereby confirming that the Leu708Pro change is indeed a mutation associated with WD. Haplotype analysis of subjects homozygous for the Leu708Pro mutation showed a conserved shared region smaller than 1 centimorgan (cM), and the region of linkage disequilibrium between the Leu708Pro mutation and neighboring microsatellite markers extended approximately 4.6 cM. When comparing the amount of linkage disequilibrium versus genetic distance from the disease mutation, it was estimated that a common ancestral Leu708Pro chromosome may have been introduced in Gran Canaria over 56 generations ago, dating it back to pre-Hispanic times. The prevalence, and the tight geographical distribution of the Leu708Pro chromosome suggests that the Canary Islands can be considered a genetic isolate for linkage disequilibrium studies.

Variceal hemorrhage continues to be a major cause of morbidity and mortality in cirrhotic patients. Transjugular intrahepatic portosystemic shunt (TIPS) is gaining wide acceptance as a treatment for several complications of portal hypertension. The aim of the current randomized study was to compare the transjugular shunt and endoscopic sclerotherapy (ES) for the prevention of variceal rebleeding (VB) in cirrhotic patients. Forty-six consecutive cirrhotic patients with variceal bleeding were randomly allocated to receive either transjugular shunt (22 patients) or ES (24 patients) 24 hours after control of bleeding. VB (50% vs. 9%) and early (first 6 weeks) VB (33% vs. 5%) were significantly more frequent in sclerotherapy patients; the actuarial probability of being free of VB was higher in the shunt group (P F .002). Eight patients (33%) of the sclerotherapy group and 3 patients (15%) of the shunt group died; the actuarial probability of survival was higher for the shunted patients (P F .05); 6 patients in the sclerotherapy group and none in the shunt group died from VB (P F .05). No difference was found in the proportion of patients with clinically evident hepatic encephalopathy (HE). These results show that the transjugular shunt is more effective than sclerotherapy in the prevention of both early and long-term VB. Moreover, a significant improvement in survival was found in the shunt group. (HEPATOLOGY 1999;29:27-32.)Variceal bleeding in cirrhotic patients is the most important complication of portal hypertension for two main reasons: the high mortality of each episode of bleeding and the high proportion of patients that rebleed with the associated additional risk of death. 1,2 This second fact makes mandatory the application of therapeutic strategies to prevent recurrent hemorrhage. 3 The best results in terms of rebleeding prevention are obtained by surgical portal systemic shunts but are associated with high procedural mortality and high incidence of encephalopathy; this has limited its wide application and has encouraged investigations on other therapeutic options like endoscopic sclerotherapy (ES) and band ligation and drugs. 4 More recently, transjugular intrahepatic portosystemic shunt (TIPS) has been introduced in clinical use for several complications of portal hypertension 5 ; it enables the decompression of the portal venous system by means of a communication between the hepatic and the portal veins through the liver parenchyma with a percutaneous approach avoiding the risks and limitations of surgery. Several initial series have suggested that it could be of great value for variceal bleeding, 6,7 but data from comparative studies with accepted therapies are scarce. [8][9][10][11][12][13][14][15] Our aim was to compare TIPS with a widely accepted therapy with low procedural mortality and morbidity and low inclusion limitations, like ES, for the prevention of variceal rebleeding (VB) in cirrhotic patients. PATIENTS AND METHODSStudy Design. All patients presenting with a variceal hemorrhage were resusc...

Variceal hemorrhage continues to be a major cause of morbidity and mortality in cirrhotic patients. Transjugular intrahepatic portosystemic shunt (TIPS) is gaining wide acceptance as a treatment for several complications of portal hypertension. The aim of the current randomized study was to compare the transjugular shunt and endoscopic sclerotherapy (ES) for the prevention of variceal rebleeding (VB) in cirrhotic patients. Forty-six consecutive cirrhotic patients with variceal bleeding were randomly allocated to receive either transjugular shunt (22 patients) or ES (24 patients) 24 hours after control of bleeding. VB (50% vs. 9%) and early (first 6 weeks) VB (33% vs. 5%) were significantly more frequent in sclerotherapy patients; the actuarial probability of being free of VB was higher in the shunt group (P F .002). Eight patients (33%) of the sclerotherapy group and 3 patients (15%) of the shunt group died; the actuarial probability of survival was higher for the shunted patients (P F .05); 6 patients in the sclerotherapy group and none in the shunt group died from VB (P F .05). No difference was found in the proportion of patients with clinically evident hepatic encephalopathy (HE). These results show that the transjugular shunt is more effective than sclerotherapy in the prevention of both early and long-term VB. Moreover, a significant improvement in survival was found in the shunt group. (HEPATOLOGY 1999;29:27-32.)Variceal bleeding in cirrhotic patients is the most important complication of portal hypertension for two main reasons: the high mortality of each episode of bleeding and the high proportion of patients that rebleed with the associated additional risk of death. 1,2 This second fact makes mandatory the application of therapeutic strategies to prevent recurrent hemorrhage. 3 The best results in terms of rebleeding prevention are obtained by surgical portal systemic shunts but are associated with high procedural mortality and high incidence of encephalopathy; this has limited its wide application and has encouraged investigations on other therapeutic options like endoscopic sclerotherapy (ES) and band ligation and drugs. 4 More recently, transjugular intrahepatic portosystemic shunt (TIPS) has been introduced in clinical use for several complications of portal hypertension 5 ; it enables the decompression of the portal venous system by means of a communication between the hepatic and the portal veins through the liver parenchyma with a percutaneous approach avoiding the risks and limitations of surgery. Several initial series have suggested that it could be of great value for variceal bleeding, 6,7 but data from comparative studies with accepted therapies are scarce. [8][9][10][11][12][13][14][15] Our aim was to compare TIPS with a widely accepted therapy with low procedural mortality and morbidity and low inclusion limitations, like ES, for the prevention of variceal rebleeding (VB) in cirrhotic patients. PATIENTS AND METHODSStudy Design. All patients presenting with a variceal hemorrhage were resus...