Acute liver failure in children: the first 348 patients in the pediatric acute liver failure study group.
OBJECTIVE
To determine short-term outcome for children with acute liver failure (ALF) as it relates to cause, clinical status, and patient demographics and to determine prognostic factors.


METHODS
A prospective, multicenter case study collecting demographic, clinical, laboratory, and short-term outcome data on children from birth to 18 years with ALF. Patients without encephalopathy were included if the prothrombin time and international normalized ratio remained > or = 20 seconds and/or >2, respectively, despite vitamin K. Primary outcome measures 3 weeks after study entry were death, death after transplantation, alive with native liver, and alive with transplanted organ.


RESULTS
The cause of ALF in 348 children included acute acetaminophen toxicity (14%), metabolic disease (10%), autoimmune liver disease (6%), non-acetaminophen drug-related hepatotoxicity (5%), infections (6%), other diagnosed conditions (10%); 49% were indeterminate. Outcome varied between patient sub-groups; 20% with non-acetaminophen ALF died or underwent liver transplantation and never had clinical encephalopathy.


CONCLUSIONS
Causes of ALF in children differ from in adults. Clinical encephalopathy may not be present in children. The high percentage of indeterminate cases provides an opportunity for investigation.
Squires
Robert H
RH
University of Pittsburgh, Children's Hospital of Pittsburgh, PA 15213, USA. Robert.squires@chp.edu
Shneider
Benjamin L
BL
Bucuvalas
John
J
Alonso
Estella
E
Sokol
Ronald J
RJ
Narkewicz
Michael R
MR
Dhawan
Anil
A
Rosenthal
Philip
P
Rodriguez-Baez
Norberto
N
Murray
Karen F
KF
Horslen
Simon
S
Martin
Martin G
MG
Lopez
M James
MJ
Soriano
Humberto
H
McGuire
Brendan M
BM
Jonas
Maureen M
MM
Yazigi
Nada
N
Shepherd
Ross W
RW
Schwarz
Kathleen
K
Lobritto
Steven
S
Thomas
Daniel W
DW
Lavine
Joel E
JE
Karpen
Saul
S
Ng
Vicky
V
Kelly
Deirdre
D
Simonds
Nancy
N
Hynan
Linda S
LS
D000293:Adolescent; D002170:Canada / Q000453:epidemiology; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006304:Health Status; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D016031:Liver Transplantation; D008297:Male; D020380:Needs Assessment; D011237:Predictive Value of Tests; D011379:Prognosis; D006113:United Kingdom; D014481:United States / Q000453:epidemiology