Reduced lysosomal acid lipase activity - A potential role in the pathogenesis of non alcoholic fatty liver disease in pediatric patients

Background: Within the spectrum of nonalcoholic fatty liver disease (NAFLD), recent evidence suggests that adult patients with nonalcoholic steatohepatitis (NASH) have significantly lower blood lysosomal acid lipase (LAL) activity than those with steatosis. This has not been studied in pediatric patients with NAFLD. Aim: Investigate blood LAL activity in pediatric patients with NAFLD and assess its correlation with histological severity. Methods: We collected data on consecutive children with biopsy-proven NAFLD including demographics, anthropometrics, and routine laboratory tests. The histological features were graded according to the NAFLD activity scoring proposed by Kleiner et al. Blood LAL activity was measured prospectively using Lalistat 2. Results: A total of 168 children were included for analysis. Mean age was 12.6 +/- 8.5 years, 60.1% were males and 52.4% had NASH. Children with significant fibrosis (stage 2-3, n = 64) had a significantly lower LAL activity compared to those with mild fibrosis (stage 0-1, n = 104). There was no significant difference in LAL activity between children with NASH compared to those without NASH. Conclusion: Reduced blood LAL activity correlates with severity of liver fibrosis in children with NAFLD indicating a potential role of reduced LAL activity in the pathogenesis of NAFLD-induced fibrosis. (C) 2016 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.