title: Evaluation of the ESPGHAN Celiac Guidelines in a North American Pediatric Population.
abstract: OBJECTIVE
  We retrospectively examined the performance of the tissue transglutaminase (TTG), endomysial antibody (EMA) tests, and the ESPGHAN (European Society of Paediatric Gastroenterology, Hepatology and Nutrition) nonbiopsy criteria in a pediatric population.
  
  
  METHODS
  Consecutive celiac serologies and corresponding intestinal biopsy results were obtained on children <18 years old over 3.5 years. Patients were classified into three categories: positive TTG, negative TTG, and IgA deficiency.
  
  
  RESULTS
  Of the 17,505 patients with celiac serology performed, 775 had a positive TTG, 574 with a negative TTG were biopsied, and 25 were IgA deficient. Of the patients with a TTG ≥10 × upper limit of normal (ULN), positive EMA, and symptoms, 98.2% had biopsies consistent with celiac disease (CD). Four human leukocyte antigen (HLA) DQ2/DQ8-positive patients who met the ESPGHAN nonbiopsy criteria did not have CD. In the group with a TTG 3-10 × ULN, 75.7% EMA-positive patients and only 40% EMA-negative patients had CD (P<0.001). Of those with a TTG 1-3 × ULN, 52.2% EMA-positive patients vs. only 13.3% EMA-negative patients had CD (P<0.01). Of the patients with bulbar and duodenal biopsies, 9.8% had CD confined only in the bulb, especially those with a low titer TTG (P<0.01). CD prevalence in our cohort was 34.6%. Sensitivity, specificity, and positive predictive value of the TTG were 98.7%, 86.4%, and 79.4%, respectively.
  
  
  CONCLUSIONS
  The TTG is a very sensitive screen for CD, but positive predictive value improves with a positive EMA titer. To apply the new ESPGHAN guidelines, clinicians must understand the performance of their celiac serology tests.
authors.affiliation: Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Dominica
authors.initials: D
authors.lastname: Gidrewicz
authors.affiliation: Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Kathryn
authors.initials: K
authors.lastname: Potter
authors.affiliation: Department of Pathology and Laboratory Medicine, University of Calgary, Calgary, Alberta, Canada.
authors.forename: Cynthia L
authors.initials: CL
authors.lastname: Trevenen
authors.affiliation: Department of Pathology and Laboratory Medicine, Saskatoon Health Region, Saskatoon, Saskatchewan, Canada.
authors.forename: Martha
authors.initials: M
authors.lastname: Lyon
authors.affiliation: Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada.
authors.forename: J Decker
authors.initials: JD
authors.lastname: Butzner
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000097:blood*; D001706:Biopsy; D002446:Celiac Disease / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D055050:Diet, Gluten-Free; D004386:Duodenum / Q000473:pathology*; D005260:Female; D019204:GTP-Binding Proteins; D006683:HLA-DQ Antigens / Q000097:blood; D006801:Humans; D007070:Immunoglobulin A / Q000097:blood; D007223:Infant; D008297:Male; D017410:Practice Guidelines as Topic; D011237:Predictive Value of Tests; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D012189:Retrospective Studies; D011503:Transglutaminases / Q000097:blood*
chemical_list: D001323:Autoantibodies; D006683:HLA-DQ Antigens; C053186:HLA-DQ2 antigen; C109756:HLA-DQ8 antigen; D007070:Immunoglobulin A; D000091345:Protein Glutamine gamma Glutamyltransferase 2; D011503:Transglutaminases; D019204:GTP-Binding Proteins
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