J Pediatr Gastroenterol Nutr 1997 Feb;24(2):128-34

Delayed encephalopathy in fulminant hepatic failure in the pediatric population
and the role of liver transplantation

Rivera-Penera T, Moreno J, Skaff C, McDiarmid S, Vargas J, Ament ME
UCLA Medical Center, Department of Pediatrics, USA.

Abstract
BACKGROUND: Liver transplantation is the therapeutic choice for fulminant
hepatic failure in children. METHODS: All 66 cases of fulminant hepatic failure
in the pediatric population seen at UCLA from May, 1985 to November, 1993 were
reviewed to determine changes in survival rates since the advent of liver
transplantation. We evaluated the clinical course and events leading to the
exclusion of surgical management of nonsurvivors, who otherwise would have
benefited from a liver transplant. We also compared the latter's clinical course
with the nontransplant survivors to determine parameters for screening patients
for liver transplantation. RESULTS: Fifty-one patient (77%) were put on the
transplant list initially but eventually, only 38 (58%) patients underwent
orthotopic liver transplantation (OLT) and of these 30 (79%) patients survived.
Of the remaining 29 (42%) patients who did not undergo liver transplantation,
only 10 (36%) patients survived. Nine patients died while waiting for a donor
liver secondary to complications of hepatic failure. The majority of
nonsurvivors in the OLT and no-OLT groups succumbed because of irreversible
neurologic deterioration. In the no-OLT group, comparisons between survivors and
non-survivors were made. There were no significant demographic differences. It
took a mean of 8 days (+/-8) versus 22 days (+/-15), (p = 0.009), from onset of
illness to first hospital admission for survivors and nonsurvivors,
respectively. Time to reach stage II encephalopathy was a mean of 5 days (+/-5)
for survivors versus 18 days (+/-16), (p = 0.05) for nonsurvivors. Nonsurvivors
were transferred to the transplant center at a mean of 12.2 days (+/-12) after
being first admitted elsewhere as compared to a mean of 1.9 days (+/-18) for
survivors, (p = 0.02). Mean prothrombin time decreased by a mean of 13.4 s/day
(+/-16) for survivors as against 2.25 s/day (+/-6) for nonsurvivors, (p = 0.06).
Mean peak total bilirubin for nonsurvivors was 460 mumol/L (27 mg/dl) versus 220
mumol/L (13 mg/dl) for survivors, (p = 0.06). Nonsurvivors died at a mean of 30
days (+/-19) from onset and survivors' liver tests started to improve at a mean
of 11 days (+/-9) from onset. CONCLUSIONS: From these studies, we conclude that
liver transplantation remains the therapeutic choice for fulminant hepatic
failure in children. Early referral and closer follow-up is necessary for timely
admission to liver transplant centers to enable screening and proper preparation
of these patients for liver transplantation