Emergency Liver Transplantation for Fulminant Liver Failure in Infants and Children

Abstract

We report our results with orthotopic liver transplantation in children with fulminant liver failure. Thirty-five children with fulminant liver failure were evaluated for liver transplantation. The main causes of liver failure were viral hepatitis (54.2%), drug-induced liver injury (14.2%) and Wilson's disease (11.4%). Children were considered as candidates for liver transplantation only if hepatic encephalopathy was associated with a decrease in the level of factor V to below 25%. Seven children (20%) did not meet this criterion and recovered spontaneously. Six children (17.1%) had contraindications for liver transplantation and died. In three of these six children, contraindications included irreversible brain damage at the time of admission. Twenty-two children (62.8%) met the criteria for liver transplantation and were placed on the emergency transplant list. Three of them died awaiting grafts. Nineteen children underwent liver transplantation; 13 of them (68.4%) are alive without sequelae, after 6 mo to 4 yr of follow-up, at this writing. Four of the children who died after surgery had severe encephalopathy on admission that did not improve after liver transplantation. In conclusion, emergency liver transplantation appears to be an effective treatment for children with fulminant liver failure. Nevertheless, irreversible brain damage developed in 10 patients, and they died before or after surgery. We postulate that many of these deaths could have been avoided if children had been transferred to a liver transplantation facility and had undergone transplantation earlier. We emphasize that children with acute liver failure should be transferred to a center that performs liver transplantation before the development of hepatic encephalopathy.

Safety and efficacy of N-acetylcysteine in children with non-acetaminophen-induced acute liver failure

“…1,3,21,27 This includes immediate transfer to a unit experienced in the management of this condition and with facilities of LT because it is the only absolute treatment for many causes of this devastating condition. 28,29 As shown in early studies in adults' with acetaminophen-induced ALF 18,[30][31][32][33][34][35] NAC is a safe drug with minor side effects, most of them self-limited or resolved with either the use of antihistamine drugs or by lowering the infusion rate.…”

“…In this retrospective study NAC was associated with a shorter length of hospital stay, higher incidence of native liver recovery without transplantation, and better survival after transplantation. Liver Transpl 14: [25][26][27][28][29][30] 2008 …”

Early Evidence of Bone Marrow Dysfunction in Children with Indeterminate Fulminant Hepatic Failure Who Ultimately Develop Aplastic Anemia

“…Liver transplantation (LTx) remains the only definitive mode of treatment for individuals with FHF who fail to recover spontaneously. Although LTx has drastically improved survival in patients with FHF with 1-year survival rates that approach 90%, various complications influence long-term morbidity and mortality (7)(8)(9).…”

Acute pancreatitis after orthotopic liver transplantation in children

“…On admission, the child had a clinical grade 2 hepatic encephalopathy. 5 Liver function test results were as follows: serum total bilirubin, 46 mmol/ dL; serum direct bilirubin, 28 mmol/dL; serum transaminase activities: aspartate aminotransferase (AST), 48 IU/L (normal, Ͻ50 IU/L; alanine aminotransferase (ALT), 30 IU/L (normal, Ͻ40 IU/L). Blood coagulation studies showed prothrombin time of 72 seconds (normal, 14 seconds); fibrinogen, 0.1 g/dL; factor II, 23%; factor V, 14%; factor VII ϩ X, Ͻ10% of normal.…”

“…A left-lobe reducedsize liver graft was implanted with standard techniques that have been previously reported. 5 Postoperative management and initial immunosuppression, which included cyclosporine, methylprednisolone, and azathioprine, have been described previously. 5,6 The immediate postoperative course was satisfactory except for an episode of acute transient renal failure.…”