title: Reduced lysosomal acid lipase activity - A potential role in the pathogenesis of non alcoholic fatty liver disease in pediatric patients.
abstract: 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.
  
  
  OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Praveen Kumar Conjeevaram
authors.initials: PK
authors.lastname: Selvakumar
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Mohammad Nasser
authors.initials: MN
authors.lastname: Kabbany
authors.affiliation: Digestive Disease Institute, Cleveland Clinic, Cleveland, OH, USA.
authors.forename: Rocio
authors.initials: R
authors.lastname: Lopez
authors.affiliation: Neurologic and Muscle Disease Unit of IRCCS Bambino Gesù Children's Hospital - IRCCS, Rome, Italy.
authors.forename: Giulia
authors.initials: G
authors.lastname: Tozzi
authors.affiliation: Liver Unit of IRCCS Bambino Gesù Children's Hospital - IRCCS, Rome, Italy.
authors.forename: Anna
authors.initials: A
authors.lastname: Alisi
authors.affiliation: Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic, Cleveland, OH, USA; Digestive Disease Institute, Cleveland Clinic, Cleveland, OH, USA. Electronic address: alkhoun@ccf.org.
authors.forename: Naim
authors.initials: N
authors.lastname: Alkhouri
authors.affiliation: Liver Unit of IRCCS Bambino Gesù Children's Hospital - IRCCS, Rome, Italy. Electronic address: nobili66@yahoo.it.
authors.forename: Valerio
authors.initials: V
authors.lastname: Nobili
mesh_terms: D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007558:Italy; D008099:Liver / Q000000981:diagnostic imaging / Q000473:pathology; D008103:Liver Cirrhosis / Q000097:blood* / Q000473:pathology; D008297:Male; D065626:Non-alcoholic Fatty Liver Disease / Q000097:blood* / Q000473:pathology; D012720:Severity of Illness Index; D002787:Sterol Esterase / Q000097:blood*; D014463:Ultrasonography; D055815:Young Adult
chemical_list: D002787:Sterol Esterase
keywords: Liver fibrosis; Lysosomal acid lipase activity; Nonalcoholic fatty liver disease; Nonalcoholic steatohepatitis