Pattern of Diagnostic Evaluation for the Causes of Pediatric Acute Liver Failure: An Opportunity for Quality Improvement

• Published in: The Journal of pediatrics Vol. 155; no. 6; pp. 801 - 806.e1
• Main Authors: Narkewicz, Michael R., MD, Dell Olio, Dominic, MD, Karpen, Saul J., MD, PhD, Murray, Karen F., MD, Schwarz, Kathy, MD, Yazigi, Nada, MD, Zhang, Song, MS, Belle, Steven H., PhD, MScHyg, Squires, Robert H., MD
• Format: Journal Article
• Language: English
• Published: Maryland Heights, MO Mosby, Inc 01.12.2009; Elsevier
• Subjects: Adolescent; Age Factors; Biological and medical sciences; Canada; Child; Child, Preschool; Cohort Studies; Databases, Factual; Female; Gastroenterology. Liver. Pancreas. Abdomen; General aspects; Humans; Infant; Liver Failure, Acute - diagnosis; Liver Failure, Acute - etiology; Liver Failure, Acute - therapy; Liver Function Tests - utilization; Liver Transplantation; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical sciences; Other diseases. Semiology; Pediatrics; Quality of Health Care; Retrospective Studies; Risk Factors; United Kingdom; United States; AIH; Fatty acid oxidation; Acute liver failure; Pediatric acute liver failure; Anti-nuclear antibody; Epstein Barr virus; HSV; Herpes simplex virus; PALF; LKM; Liver kidney microsomal antibody; Autoimmune hepatitis; ANA; FAO; Galactose-1-phosphate uridyltransferase; ASMA; EBV; Gal-1-PUT; ALF; Hepatitis B virus; Anti-smooth muscle antibody; HBV; Human; Evaluation; Pediatrics; Improvement; Liver failure; Acute; Quality; Cause; Digestive diseases; Hepatic disease; Diagnosis; Child
• DOI: 10.1016/j.jpeds.2009.06.005
• ISSN: 0022-3476

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. Study design 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.