Acute liver failure in children: A regional experience.
OBJECTIVE
To review the outcome of acute liver failure (ALF) and the effect of liver transplantation in children in Australia.


METHODS
A retrospective review was conducted of all paediatric patients referred with acute liver failure between 1985 and 2000 to the Queensland Liver Transplant Service, a paediatric liver transplant centre based at the Royal Children's Hospital, Brisbane, that is one of three paediatric transplant centres in Australia.


RESULTS
Twenty-six patients were referred with ALF. Four patients did not require transplantation and recovered with medical therapy while two were excluded because of irreversible neurological changes and died. Of the 20 patients considered for transplant, three refused for social and/or religious reasons, with 17 patients listed for transplantation. One patient recovered spontaneously and one died before receiving a transplant. There were 15 transplants of which 40% (6/15) were < 2 years old. Sixty-seven per cent (10/15) survived > 1 month after transplantation. Forty per cent (6/15) survived more than 6 months after transplant. There were only four long-term survivors after transplant for ALF (27%). Overall, 27% (6/22) of patients referred with ALF survived. Of the 16 patients that died, 44% (7/16) were from neurological causes. Most of these were from cerebral oedema but two patients transplanted for valproate hepatotoxicity died from neurological disease despite good graft function.


CONCLUSIONS
Irreversible neurological disease remains a major cause of death in children with ALF. We recommend better patient selection and early referral and transfer to a transplant centre before onset of irreversible neurological disease to optimize outcome of children transplanted for ALF.
Ee
L C
LC
Queensland Liver Transplant Service, Royal Children's Hospital, Brisbane, Queensland. Looi_Ee@health.qld.gov.au
Shepherd
R W
RW
Cleghorn
G J
GJ
Lewindon
P J
PJ
Fawcett
J
J
Strong
R W
RW
Lynch
S V
SV
D017677:Age Distribution; D002648:Child; D002675:Child, Preschool; D005260:Female; D006084:Graft Rejection; D006085:Graft Survival; D006801:Humans; D015994:Incidence; D007223:Infant; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000453:epidemiology* / Q000601:surgery*; D016031:Liver Transplantation / Q000401:mortality / Q000706:statistics & numerical data*; D008297:Male; D011379:Prognosis; D011793:Queensland / Q000453:epidemiology; D012189:Retrospective Studies; D018570:Risk Assessment; D012720:Severity of Illness Index; D017678:Sex Distribution; D016019:Survival Analysis