title: Prevalence and Significance of Autoantibodies in Children With Acute Liver Failure.
abstract: The purpose of the present study is to estimate autoantibody (auto-AB) frequency, clinical characteristics, and 21-day outcome of participants in the Pediatric Acute Liver Failure Study Group (PALFSG) by antinuclear antibody, smooth muscle antibody, and liver-kidney microsomal (LKM) antibody status.
  
  
  
  Auto-ABs were determined at local and/or central laboratories. Subjects were assigned to autoimmune hepatitis (AIH), indeterminate, and other diagnoses groups.
  
  
  
  Between 1999 and 2010, 986 subjects were enrolled in the PALFSG. At least 1 auto-AB result was available for 722 (73.2%). At least 1 auto-AB was positive for 202 (28.0%). Diagnoses for auto-AB+ subjects were AIH (63), indeterminate (75), and other (64). Auto-ABs were more common in Wilson disease (12/32, 37.5%) compared with other known diagnoses (52/253, 20.6%, P = 0.03). LKM+ subjects were younger (median 2.4 vs 9.1 years, P < 0.001) and more likely to undergo liver transplantation (53.3% vs 31.4% P = 0.02) than other auto-AB+/LKM- subjects. Steroid treatment of subjects who were auto-AB+ was not significantly associated with survival and the subgroup with known diagnoses other than AIH had a higher risk of death.
  
  
  
  Auto-ABs are common in children with acute liver failure, occurring in 28%. Auto-AB+ subjects have similar outcomes to auto-AB negative subjects. LKM+ children are younger and more likely to undergo liver transplantation compared with other auto-AB+ subjects. Although auto-AB may indicate a treatable condition, positivity does not eliminate the need for a complete diagnostic evaluation because auto-ABs are present in other conditions. The significance of auto-AB in pediatric acute liver failure remains uncertain, but LKM+ appears to identify a unique population of children who merit further study.
authors: *Department of Pediatrics, Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine and Children's Hospital Colorado, Aurora, CO †Division of Gastroenterology and Hepatology, Seattle Children's Hospital ‡Department of Pediatrics, University of Washington School of Medicine, Seattle, WA §Department of Epidemiology ||Department of Biostatistics, University of Pittsburgh Graduate School of Public Health, Pittsburgh, PA ¶Department of Pediatrics #Department of Developmental Biology, Washington University School of Medicine, St. Louis, MO **Division of Pediatric Gastroenterology, Hepatology/Nutrition and Transplant and Regenerative Medicine Centre, The Hospital for Sick Children ††Department of Pediatrics, University of Toronto, Toronto, Canada ‡‡Division of Pediatric Gastroenterology, Hepatology and Nutrition, UCSF Benioff Children's Hospital §§Department of Pediatrics and Surgery, University of California, San Francisco ||||Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, Emory University School of Medicine and Children's Healthcare of Atlanta, Atlanta, GA ¶¶Division of Gastroenterology, Hepatology and Nutrition, The Children's Hospital of Philadelphia ##Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, Philadelphia ***Department of Pediatrics, University of Pittsburgh School of Medicine †††Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA.
authors: Michael R
authors: MR
authors: Narkewicz
authors: Simon
authors: S
authors: Horslen
authors: Steven H
authors: SH
authors: Belle
authors: David A
authors: DA
authors: Rudnick
authors: Vicky L
authors: VL
authors: Ng
authors: Philip
authors: P
authors: Rosenthal
authors: Rene
authors: R
authors: Romero
authors: Kathleen M
authors: KM
authors: Loomes
authors: Song
authors: S
authors: Zhang
authors: Regina M
authors: RM
authors: Hardison
authors: Robert H
authors: RH
authors: Squires
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D015415:Biomarkers / Q000097:blood; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D019693:Hepatitis, Autoimmune / Q000150:complications / Q000175:diagnosis* / Q000453:epidemiology / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D017114:Liver Failure, Acute / Q000175:diagnosis / Q000276:immunology* / Q000401:mortality / Q000601:surgery; D016031:Liver Transplantation; D016015:Logistic Models; D008297:Male; D015995:Prevalence; D011379:Prognosis
chemical_list: D001323:Autoantibodies; D015415:Biomarkers