摘要
The etiology of acute liver failure in children is unknown in a large number o f cases. Defects in fatty acid oxidation have been shown to lead to severe liver injury. This retrospective analysis examined the bile acylcarnitine profiles of 27 children with acute liver failure who underwent liver transplantation or die d.Results were compared with 758 postmortem samples from individuals without acu te liver failure. Cumulative amounts of free carnitine, medium-or long-chain s pecies in excess of the 95th percentile of the control group were considered abn ormal.Fourteen samples had normal profiles. Three had markedly elevated concentr ations of free carnitine, whereas ten showed elevations in medium-or long-chai n species. The relative risk of death was 2.86 (95%confidence interval, 1.08-7 .54, P = .01)in the 10 children with elevated concentrations of medium-or long -chain species compared with those with normal analyses.Overall, medium-and lo ng-chain acylcarnitines were increased in those patients who died compared with survivors, (dead vs. alive; medium-chain, 187±74 vs. 32±12 μmol/L, P=.008;l ong-chain, 146±74 vs.15±8 μmol/L, mean±standard error of the mean, P=.018). These studies describe biliary free and esterified carnitine profiles in childr en with acute liver failure. In conclusion, the findings raise the hypothesis th at abnormalities in fatty acid oxidation may predispose to a worse outcome in ac ute liver failure.
The etiology of acute liver failure in children is unknown in a large number o f cases. Defects in fatty acid oxidation have been shown to lead to severe liver injury. This retrospective analysis examined the bile acylcarnitine profiles of 27 children with acute liver failure who underwent liver transplantation or die d.Results were compared with 758 postmortem samples from individuals without acu te liver failure. Cumulative amounts of free carnitine, medium-or long-chain s pecies in excess of the 95th percentile of the control group were considered abn ormal.Fourteen samples had normal profiles. Three had markedly elevated concentr ations of free carnitine, whereas ten showed elevations in medium-or long-chai n species. The relative risk of death was 2.86 (95%confidence interval, 1.08-7 .54, P = .01)in the 10 children with elevated concentrations of medium-or long -chain species compared with those with normal analyses.Overall, medium-and lo ng-chain acylcarnitines were increased in those patients who died compared with survivors, (dead vs. alive; medium-chain, 187±74 vs. 32±12 μmol/L, P=.008;l ong-chain, 146±74 vs.15±8 μmol/L, mean±standard error of the mean, P=.018). These studies describe biliary free and esterified carnitine profiles in childr en with acute liver failure. In conclusion, the findings raise the hypothesis th at abnormalities in fatty acid oxidation may predispose to a worse outcome in ac ute liver failure.
