title: The plasma activities of lysosomal enzymes in infants with necrotizing enterocolitis: new promising class of biomarkers?
abstract: BACKGROUND
  Intestinal ischemia plays a major role in the pathogenesis of necrotizing enterocolitis (NEC). The diagnosis of intestinal ischemia would be highly desirable, as it is impossible to achieve with the current diagnostic regimes. Preliminary data from an animal NEC model indicate a possible correlation between the plasma activity of the lysosomal enzyme beta-glucosidase and intestinal ischemia.
  
  
  METHODS
  In this case-control study the plasma activities of six different lysosomal enzymes were detected by high-performance liquid-chromatography tandem mass-spectrometry in 15 infants with NEC and compared to 18 controls.
  
  
  RESULTS
  The plasma activities of β-glucosidase (ABG), α-glucosidase (GAA), and galactocerebrosidase (GALC) were significantly higher in the NEC group compared with controls (ABG, p=0.009; GAA, p<0.001; GALC, p<0.001). GAA and GALC showed the highest diagnostic value with areas under the curve of 0.91 and 0.87.
  
  
  CONCLUSIONS
  We identified GAA and GALC as new promising biomarkers for gut wall integrity in infants with NEC, and report first results on the plasma activity of ABG. The present study supports the hypothesis that the plasma activity of ABG might serve as a marker of intestinal ischemia in NEC. The identification of intestinal ischemia could facilitate early discrimination of infants at risk for NEC from infants with benign gastrointestinal disorders.
authors.affiliation: Department of Pediatric Surgery, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria. Electronic address: thomas.benkoe@meduniwien.ac.at.
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Benkoe
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Research Core Unit for Pediatric Biochemistry and Analytics, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
authors.forename: Thomas P
authors.initials: TP
authors.lastname: Mechtler
authors.affiliation: Department of Pediatric Surgery, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
authors.forename: Mario
authors.initials: M
authors.lastname: Pones
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
authors.forename: Andrea-Romana
authors.initials: AR
authors.lastname: Prusa
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Neonatology, Pediatric Intensive Care and Neuropediatrics, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
authors.forename: Katrin
authors.initials: K
authors.lastname: Klebermass-Schrehof
authors.affiliation: Department of Pediatric Surgery, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
authors.forename: Winfried
authors.initials: W
authors.lastname: Rebhandl
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Research Core Unit for Pediatric Biochemistry and Analytics, Medical University of Vienna, Waehringerguertel 18-20, 1090 Vienna, Austria.
authors.forename: David C
authors.initials: DC
authors.lastname: Kasper
mesh_terms: D019540:Area Under Curve; D015415:Biomarkers / Q000097:blood; D016022:Case-Control Studies; D002853:Chromatography, Liquid; D020345:Enterocolitis, Necrotizing / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D005698:Galactosylceramidase / Q000097:blood*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008247:Lysosomes / Q000201:enzymology; D065666:Mesenteric Ischemia / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D053719:Tandem Mass Spectrometry; D000520:alpha-Glucosidases / Q000097:blood*; D001617:beta-Glucosidase / Q000097:blood*
chemical_list: D015415:Biomarkers; D000520:alpha-Glucosidases; D001617:beta-Glucosidase; D005698:Galactosylceramidase
keywords: Biomarker; Enzyme; Intestinal ischemia; Lysosomal; NEC; Necrotizing enterocolitis