Abstract
The current United Network for Organ Sharing (UNOS) policy is to allocate liver grafts to pediatric patients with chronic liver disease based on the pediatric end-stage liver disease (PELD) scoring system, while children with fulminant hepatic failure may be urgently listed as Status 1a. The objective of this study was to identify pre-transplant variables that influence patient and graft survival in those children undergoing LTx (liver transplantion) for FHF (fulminant hepatic failure) compared to those patients transplanted for extrahepatic biliary atresia (EHBA), a chronic form of liver disease. The UNOS Liver Transplant Registry was examined for pediatric liver transplants performed for FHF and EHBA from 1987 to 2002. Variables that influenced patient and graft survival were assessed using univariate and multivariate analysis. Kaplan-Meier analysis of FHF and EHBA groups revealed that 5 year patient and graft survival were both significantly worse (P < 0.0001) in those patients who underwent transplantation for FHF. Multivariate analysis of 29 variables subsequently revealed distinct sets of factors that influenced patient and graft survival for both FHF and EHBA. These results confirm that separate prioritizing systems for LTx are needed for children with chronic liver disease and FHF; additionally, our findings illustrate that there are unique sets of variables which predict survival following LTx for these two groups.
Highlights
Title Predictors of clinical outcome in children undergoing orthotopic liver transplantation for acute and chronic liver disease
In the absence of liver transplantation (LTx), Fulminant hepatic failure (FHF) of indeterminate etiology is associated with a poor clinical prognosis with a fatality rate as high as 90%
The initial database contained 53,833 transplantations that occurred between October 1987 and May 2002 in the United States. 7,894 of these transplants were performed in recipients 18 years of age or less; 3,013 carried the diagnosis of either FHF or extrahepatic biliary atresia (EHBA)
Summary
Title Predictors of clinical outcome in children undergoing orthotopic liver transplantation for acute and chronic liver disease. Predictors of Clinical Outcome in Children Undergoing Orthotopic Liver Transplantation for Acute and Chronic Liver Disease. Since most cases of FHF require LTx within a period of days to weeks after the onset of liver disease, clinicians must decide in a timely manner when to list a child for transplantation before his or her clinical condition deteriorates. In contrast to the acute nature of FHF, extrahepatic biliary atresia (EHBA) often leads to chronic end-stage liver disease, and is the most common cause of pediatric LTx. Children with EHBA generally present in early infancy with cholestasis and, if diagnosed in a timely manner, often undergo Kasai portoenterostomy during the first several months of life. More than half of the infants with EHBA
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