http://www.ncbi.nlm.nih.gov/pubmed/27496798

Narkewicz MR, Horslen S, Belle SH, Rudnick DA, Ng VL, Rosenthal P, Romero R, Loomes KM, Zhang S, Hardison RM, Squires RH; Pediatric Acute Liver Failure Study Group. J Pediatr Gastroenterol Nutr. 2016 Aug 4.

Abstract

OBJECTIVES:
The purpose of this study is to estimate autoantibody (autoAB) frequency, clinical characteristics and 21 day outcome of participants in the Pediatric Acute Liver Failure Study Group (PALFSG) by ANA, SMA and LKM antibody status.

METHODS:
AutoAB were determined at local and/or central laboratories. Subjects were assigned to autoimmune hepatitis (AIH), indeterminate and other diagnoses groups.

RESULTS:
Between 1999 and 2010, 986 subjects were enrolled in the PALFSG. At least one autoAB result was available for 722 (73.2%). At least one autoAB was positive for 202 (28.0%). Diagnoses for autoAB+ subjects were: AIH (63), indeterminate (75) and other (64). AutoAB were more common in Wilson disease (12/32, 37.5%) vs other known diagnoses (52/253, 20.6%, p = 0.03). LKM+ subjects were younger (median 2.4 vs 9.1 yrs, p < 0.001) and more likely to undergo liver transplantation (53.3% vs 31.4% p = 0.02) than other autoAB+/LKM- subjects. Steroid treatment of subjects who were autoAB+ was not significantly associated with survival and the sub group with known diagnoses other than AIH had a higher risk of death.

CONCLUSIONS:
AutoABs are common in children with ALF, occurring in 28%. AutoAB+ subjects have similar outcomes to autoAB negative subjects. LKM+ children are younger and more likely to undergo liver transplantation compared to other autoAB+ subjects. Although AutoAB may indicate a treatable condition, positivity does not eliminate the need for a complete diagnostic evaluation as autoABs are present in other conditions. The significance of autoAB in PALF remains uncertain, but LKM+ appears to identify a unique population of children who merit further study.

Published on: 
Aug-2016

CLF Intro movie

Financial Aid Offered by Trusts

Follow us on: