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• W2079181732 abstract "Survival After First Esophageal Variceal Hemorrhage in Patients With Biliary Atresia. Miga D, Sokol RJ, MacKenzie T, et al. J Pediatr 2001;139:291–6. Summary: Little is known about the clinical impact of esophageal variceal hemorrhage (EVH) on children with chronic liver diseases. In this report, Miga et al. advance our understanding of this important issue by assessing transplant-free survival after an initial episode of EVH in a group of children with biliary atresia. Moreover, the investigators evaluate the impact of demographic, clinical and laboratory variables on transplant-free survival in this cohort of patients. Importantly, the authors were able to conduct the study because EVH is not by itself considered an indication for liver replacement therapy in their center; therefore, a population of children was created who had been followed up with for extended intervals after EVH but who had not undergone liver transplantation. To accomplish their stated goals, the authors retrospectively analyzed the charts of children with biliary atresia who had undergone hepatoportoenterostomy (Kasai procedure) at their center between 1973 and 1992. Esophageal variceal hemorrhage was defined by evidence of gastrointestinal bleeding with a reduction in hematocrit concentration of greater than 5% in the setting of endoscopically confirmed varices. Beginning in 1979, EVH was treated with injection sclerotherapy and, after 1986, with band ligation within 24 hours of the bleeding episode. The primary end points were either death or liver transplantation after the first episode of EVH. New-onset EVH was noted in 40 (29%) of the 134 patients analyzed. Age at the time of the Kasai procedure was similar in children with and without EVH. The mean age at the end of follow-up, death, or liver transplantation was 2.3 years for the group as a whole, and 8.1 years and 1.3 years, respectively, for those with and without EVH. The median transplant-free survival was 6.7 years and 1.7 years for children with and without EVH, respectively. The seemingly paradoxical finding that patients with EVH survived transplant-free longer than those without EVH is explained by the inclusion of infants whose Kasai procedure failed “early” in the analysis. These children presumably either died or underwent liver transplantation before sufficient time had elapsed to allow the development of EVH. Indeed, if these early failures are excluded and only patients who survived the first 2 years of life are analyzed, transplant-free survival is similar between the groups with and without EVH. The overall estimated relative risk of death or transplant after the first episode of EVH in patients with biliary atresia was 3.4 and gradually decreased over the time of follow-up, from 4.2 within 1 year after EVH to 1.0 five or more years after the initial EVH. Interestingly, the risk of dying or requiring liver transplantation was strongly correlated with total bilirubin concentration at the time of the first EVH episode: the estimated relative risk of transplant-free survival was 0.6, 7.2, and 12.0 for children with a total bilirubin concentration less than 4.0 mg/dL, 4 to 10 mg/dL, or more than 10 mg/dL, respectively. Transplant-free survival was 80% at 4 years after EVH for children with total bilirubin concentrations less than 4.0 mg/dL, 50% at 1 year for those with a serum bilirubin concentration between 4 and 10 mg/dL and 50% at 4 months for those with serum bilirubin concentrations greater than 10 mg/dL. Aspartate aminotransferase concentrations and the presence of ascites weakly correlated with transplant-free survival, but these variables did not significantly add further prognostic value to that given by the total serum bilirubin concentration alone. None of the other variables analyzed, including age at the first episode of EVH and linear growth velocity, correlated with outcome in children with EVH. In conclusion, EVH was noted in 29% of children with biliary atresia and, contrary to general belief, was not associated with poor outcome, as measured by the time of survival without transplantation. Transplant-free survival was strongly linked to serum bilirubin concentration at the time of the first episode of EVH in this group of patients, so that the estimated relative risk of death or transplant was not increased in patients with bilirubin concentrations less than 4.0 mg/dL, was moderately increased in those with bilirubin concentrations between 4 and 10 mg/dL, and was highest for those with bilirubin concentration greater than 10 mg/dL. Based on these results, an initial episode EVH alone in children with biliary atresia should not be viewed as an absolute indication for liver transplantation. Comment: Esophageal variceal hemorrhage is a well-known complication of portal hypertension in patients with biliary atresia, occurring in up to 55% of children surviving more than 10 years after a Kasai procedure (Arch Surg 1996;131:493–6). However, little is known about the clinical impact of EVH on children with chronic liver disease, which is the focus of the recent study by Miga et al. In adults with liver disease, new-onset EVH is a consistent poor prognostic indicator, with reported mortality rates between 30% and 50% in affected individuals (Hepatology 1995;22:332–54 and references therein). Based on this well-documented adult experience, the development of EVH is generally regarded as an ominous incident in children with chronic liver disease and portal hypertension. In fact, the first episode of EVH alone frequently prompts expeditious evaluation and listing for transplantation in many pediatric liver centers. However, adults and children are sufficiently different with respect to many important factors, such as primary cause of liver disease, the common presence of comorbid disorders in adults, and the theoretical improved potential for the development of portosystemic collaterals in young patients. This brings into question the reliability of simply extrapolating adult data to children with EVH. Indeed, the current retrospective analysis by Miga et al. supports the tenet that EVH alone is not as ominous in children as in adults and need not be considered an absolute indication for childhood liver transplantation. More importantly, these results uncover the even more critical issue of managing EVH in children with chronic liver disease, particularly those with serum bilirubin concentrations less than 4.0 mg/dL. Again, much more is known in adults than in children in this regard. For example, clinical and endoscopic variables, such as Child-Pugh score and the presence of red wale markings on esophageal varices, clearly increase the risk of bleeding from varices in adults (N Engl J Med 2001;345:669–81 and references therein), but their impact on children are uncertain and not validated. As the report by Miga et al. points out, extrapolation of data from adults to children may be an unreliable exercise, especially in the setting of EVH. Nevertheless, useful information can be gleaned from the available published literature. Controlled clinical trials show that nonselective β-blockers, such as propranolol and nadolol, can effectively decrease the risk of first EVH (primary prophylaxis) and death in adults with portal hypertension and moderate to large varices (N Engl J Med 1991;324:1532–8). In addition, these agents can prevent rebleeding after an initial episode of EVH (secondary prophylaxis;Hepatology 1984;4:355–8). Experience with β-blockers in children is limited, but propranolol is well-tolerated and reduces splenic pulp pressure when the pulse rate is decreased by 25% (J Pediatr 1985;106:317–21). In another retrospective analysis, 14 of 21 children treated with propranolol given for both primary and secondary prophylaxis were free of EVH. Unfortunately, the severity of esophageal varices and duration of follow-up was variable and this study was uncontrolled (J Pediatr Gastroenterol Nutr 1999;29:12–7). The combination of a β-blocker in addition to vasodilators (isosorbide mononitrate) is therapeutically more efficacious than β-blockers alone in adults (Ann Intern Med 1991;114:869–73). In fact, this therapeutic combination was better than endoscopic ligation for primary prophylaxis of EVH (N Engl J Med 2001;245:647–55), but it remains untested in children. Endoscopic surveillance with variceal injection sclerotherapy or band ligation is routinely used in the management of esophageal varices in adults and children. In an early report, esophageal varices were obliterated in 15 of 16 children with biliary atresia and portal hypertension after repeated sclerotherapy. Rebleeding did not develop in these treated children after a mean of 2.8 years after injection sclerotherapy (J Pediatr Surg 1989;24:438–42). In another analysis, esophageal varices were effectively obliterated with injection endosclerosis in 31 children, 26 of whom (84%) were EVH-free after a mean follow-up of 2 years (J Pediatr Gastroenterol Nutr 1988;7:662–6). Results of a recent randomized trial demonstrate that children who underwent injection sclerotherapy had reduced risk of new-onset EVH compared with untreated controls. However, the mortality rate was similar among endoscopically treated and control groups, in this study (J Pediatr Surg 2000;35:401–5). In adults, band ligation of esophageal varices decreases the risk of new-onset EVH, and recurrent bleeding after an initial episode of EVH is associated with fewer complications than injection sclerotherapy and is the preferred procedure in adults (N Engl J Med 2001;345:669–81). Although repeated band ligation results in effective obliteration of esophageal varices and prevention of rebleeding in uncontrolled studies (J Pediatr Gastroenterol Nutr 1995;20:202–8, J Pediatr Surg 1996;31:1056–9), its role in preventing the first EVH or rebleeding after EVH is less defined than for injection sclerotherapy in children. The creation of a conduit to divert blood way from the portal system and into the systemic circulation (portosystemic shunt), thereby reducing portal pressure, is another method used to treat esophageal varices. In fact, placement of a transjugular intrahepatic portosystemic shunt may be more effective than band ligation to prevent rebleeding from esophageal varices in adults (Hepatol 1997;26:1115–22). Results of small and uncontrolled trials suggest that transjugular intrahepatic portosystemic shunt is effective in children who experience recurrent EVH despite aggressive endoscopic treatment (J Pediatr Gastroenterol Nutr 1999;29:240–9 and references therein). By decreasing the risks of rebleeding while waiting for organ replacement, transjugular intrahepatic portosystemic shunt can be successfully used as a bridge to liver transplantation in children with recalcitrant EVH (Transplantation 1996;62:1178–81). Surgical portosystemic conduits, such as mesocaval and splenorenal shunts, are generally reserved for children in whom other complications of portal hypertension develop, such as nonesophageal variceal bleeding and severe hypersplenism, or for those in whom endoscopic techniques fail to control EVH (J Pediatr Surg 1997;32:489–93). Transjugular intrahepatic portosystemic shunt and surgical shunts in children with portal hypertension are limited by an increased risk of occlusion in young patients with smaller vessels than adults. In addition, children can “outgrow” shunts in the process of normal growth. Individual institutional experience and skill are also clearly important in choosing these more technically demanding methods to treat EVH. Although the current report by Miga et al. is limited by its retrospective nature, it provides the first clear evidence that the development of EVH may not be as ominous in children as in adults. In addition, EVH alone should not be considered an absolute indication for liver transplantation in young patients without significant cholestasis. Moreover, by highlighting the importance of management issues, this study sets the stage for the design of large prospective, controlled trials for the treatment of EVH in children with portal hypertension and chronic liver disease." @default.
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• W2079181732 title "Bleeding “Ain't” (Necessarily) Bad in Biliary Atresia" @default.
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