Background/purpose: Acute liver failure in the pediatric population is a rare but highly lethal health problem. Sometimes it is difficult to predict who will benefit from liver transplantation. The authors report on their experience in the past 8 years at a pediatric transplant center.
Methods: A retrospective chart review was performed on all children referred to the liver transplant (TX) service with the diagnosis of acute hepatocellular dysfunction (AHD) from 1988 to 1996. Presentation, chemistries, and clinical course were evaluated. Statistical analysis was performed using analysis of variance.
Results: Twenty-six children underwent evaluation. Seventeen patients fulfilled the criteria for fulminant hepatic failure (FHF). Eleven patients recovered without TX, 14 received a TX, and one died awaiting TX. Of those that received a TX, four died in the early postoperative period and 10 survived (mean follow-up of 4.2 years). There was a wide range in most laboratory values. Serum bilirubin levels, ammonia levels, and coagulation parameters, however, reached statistical significance in patients requiring transplant. The most consistent discriminators of need for transplantation and outcome were neurological findings and multisystem organ failure. Children who recovered without TX had no seizures and minimal encephalopathy. Of the 15 children who were recommended for TX, six had seizures and all had encephalopathy, 12 having grade III or IV. All five nonsurvivors had respiratory failure early in their clinical course, and four of five nonsurvivors also had renal failure.
Conclusions: There is significant overlap in the presentation and laboratory findings of children who present with AHD or FHF. Neurological status was an important discriminator of need for transplantation. Patients who presented with multisystem organ failure, including renal failure and respiratory failure, had 100% mortality rate despite liver transplantation.