J Pediatr Surg 1998 Jul;33(7):998-1002; discussion 1002-3

Transplantation for acute hepatic failure in children

Nicolette L, Billmire D, Faulkenstein K, Pierson A, Vinocur C, Weintraub W, Dunn
S

Abstract
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