Pattern of diagnostic evaluation for the causes of pediatric acute liver failure: an opportunity for quality improvement.
OBJECTIVE
To describe the frequency of diagnostic testing for the 4 most common causes of pediatric acute liver failure (PALF) (drugs, metabolic disease, autoimmune process, and infections) in indeterminate PALF within the PALF Study Group Database.


METHODS
PALF was defined by severe hepatic dysfunction within 8 weeks of onset of illness, with no known underlying chronic liver disease in patients from birth through 17 years of age.


RESULTS
Of the 703 patients in the database, 329 (47%) had indeterminate PALF. In this group, a drug history was obtained in 325 (99%) urine toxicology screenings performed in 118 (36%) and acetaminophen level measured in 124 (38%) patients. No testing for common metabolic diseases was done in 179 (54%) patients. Anti-nuclear antibody, anti-smooth muscle antibody, and anti-liver kidney microsomal autoantibodies associated with autoimmunity were determined in 239 (73%), 233 (71%), and 208 (63%) patients, and no tests were obtained in 70 (21%). Testing was performed for hepatitis A virus, hepatitis B virus, and Epstein Barr virus in 80%, 86%, and 68%, respectively.


CONCLUSIONS
Current practice indicates that investigation for metabolic and autoimmune causes of PALF are infrequent in patients ultimately given a diagnosis of indeterminate acute liver failure. This offers an opportunity to improve diagnosis and potential treatment options in children with acute liver failure.
Narkewicz
Michael R
MR
Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado Denver School of Medicine and the Pediatric Liver Center, The Children's Hospital, Aurora, CO, USA.
Dell Olio
Dominic
D
Karpen
Saul J
SJ
Murray
Karen F
KF
Schwarz
Kathy
K
Yazigi
Nada
N
Zhang
Song
S
Belle
Steven H
SH
Squires
Robert H
RH
D000293:Adolescent; D000367:Age Factors; D002170:Canada; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D016208:Databases, Factual; D005260:Female; D006801:Humans; D007223:Infant; D017114:Liver Failure, Acute / Q000175:diagnosis* / Q000209:etiology* / Q000628:therapy; D008111:Liver Function Tests / Q000706:statistics & numerical data*; D016031:Liver Transplantation; D008297:Male; D011787:Quality of Health Care; D012189:Retrospective Studies; D012307:Risk Factors; D006113:United Kingdom; D014481:United States