PMID- 24222955 OWN - NLM STAT- MEDLINE DCOM- 20140411 LR - 20211203 IS - 2219-2840 (Electronic) IS - 1007-9327 (Print) IS - 1007-9327 (Linking) VI - 19 IP - 41 DP - 2013 Nov 7 TI - Children with celiac disease and high tTGA are genetically and phenotypically different. PG - 7114-20 LID - 10.3748/wjg.v19.i41.7114 [doi] AB - AIM: To investigate whether celiac disease (CD) patients with tissue-transglutaminase antibody (tTGA) >/= 100 U/mL are different from patients with lower tTGA levels. METHODS: Biopsy-proven (Marsh III) pediatric CD patients (n = 116) were prospectively included between March 2009 and October 2012. The biopsies were evaluated by a single pathologist who was blinded to all of the patients' clinical data. The patients were distributed into 2 groups according to their tTGA level, which was measured using enzyme-linked immunoassay: tTGA >/= 100 U/mL and tTGA < 100 U/mL. The patients'characteristics, symptoms, human leukocyte antigen (HLA) genotype and degree of histological involvement were compared between the 2 groups. RESULTS: A total of 34 (29.3%) children had tTGA values < 100 U/mL and 82 (70.7%) tTGA levels of >/= 100 U/mL. Patients with high tTGA levels had lower average body weight-for-height standard deviation scores (SDS) than did patients with tTGA < 100 U/mL (-0.20 +/- 1.19 SDS vs 0.23 +/- 1.03 SDS, P = 0.025). In the low tTGA group, gastrointestinal symptoms were more common (97.1% vs 75.6%, P = 0.006). More specifically, abdominal pain (76.5% vs 51.2%; P = 0.012) and nausea (17.6% vs 3.7%, P = 0.018) were more frequent among patients with low tTGA. In contrast, patients with solely extraintestinal manifestations were only present in the high tTGA group (18.3%, P = 0.005). These patients more commonly presented with aphthous stomatitis (15.9% vs 0.0%, P = 0.010) and anemia (32.9% vs 11.8%, P = 0.019). In addition, when evaluating the number of CD-associated HLA-DQ heterodimers (HLA-DQ2.5, HLA-DQ2.2 and HLA-DQ8), patients with low tTGA levels more commonly had only 1 disease-associated heterodimer (61.8% vs 31.7%, P = 0.005), while patients with high tTGA more commonly had multiple heterodimers. Finally, patients with tTGA >/= 100 U/mL more often had a Marsh IIIc lesion (73.2% vs 20.6%, P < 0.001) while in patients with low tTGA patchy lesions were more common (42.4% vs 6.8%, P < 0.001). CONCLUSION: Patients with tTGA >/= 100 U/mL show several signs of more advanced disease. They also carry a larger number of CD associated HLA-DQ heterodimers. FAU - Mubarak, Amani AU - Mubarak A AD - Amani Mubarak, Victorien M Wolters, Roderick HJ Houwen, Department of Pediatric Gastroenterology, University Medical Center Utrecht, Wilhelmina Children's Hospital, 3508 AB Utrecht, The Netherlands. FAU - Spierings, Eric AU - Spierings E FAU - Wolters, Victorien M AU - Wolters VM FAU - Otten, Henny G AU - Otten HG FAU - ten Kate, Fiebo J W AU - ten Kate FJ FAU - Houwen, Roderick H J AU - Houwen RH LA - eng PT - Comparative Study PT - Journal Article PL - United States TA - World J Gastroenterol JT - World journal of gastroenterology JID - 100883448 RN - 0 (Autoantibodies) RN - 0 (HLA Antigens) RN - 0 (HLA-DQ Antigens) RN - 0 (Immunoglobulin A) RN - EC 2.3.2.13 (Protein Glutamine gamma Glutamyltransferase 2) RN - EC 2.3.2.13 (Transglutaminases) RN - EC 3.6.1.- (GTP-Binding Proteins) SB - IM MH - Adolescent MH - Age Factors MH - Asymptomatic Diseases MH - Autoantibodies/*blood MH - Biopsy MH - Celiac Disease/blood/complications/diagnosis/*genetics/*immunology MH - Chi-Square Distribution MH - Child MH - Child, Preschool MH - Duodenum/pathology MH - Enzyme-Linked Immunosorbent Assay MH - Female MH - GTP-Binding Proteins MH - Genetic Predisposition to Disease MH - HLA Antigens/*genetics MH - HLA-DQ Antigens/genetics MH - Humans MH - Immunoglobulin A/*blood MH - Infant MH - Infant, Newborn MH - Male MH - Phenotype MH - Prospective Studies MH - Protein Glutamine gamma Glutamyltransferase 2 MH - Severity of Illness Index MH - Transglutaminases/*immunology PMC - PMC3819547 OTO - NOTNLM OT - Anti-tissue transglutaminase antibodies OT - Celiac disease OT - Human leukocyte antigen OT - Phenotype OT - Serology EDAT- 2013/11/14 06:00 MHDA- 2014/04/12 06:00 PMCR- 2013/11/07 CRDT- 2013/11/14 06:00 PHST- 2013/01/21 00:00 [received] PHST- 2013/06/18 00:00 [revised] PHST- 2013/07/04 00:00 [accepted] PHST- 2013/11/14 06:00 [entrez] PHST- 2013/11/14 06:00 [pubmed] PHST- 2014/04/12 06:00 [medline] PHST- 2013/11/07 00:00 [pmc-release] AID - 10.3748/wjg.v19.i41.7114 [doi] PST - ppublish SO - World J Gastroenterol. 2013 Nov 7;19(41):7114-20. doi: 10.3748/wjg.v19.i41.7114.