A 6-year-old white girl with a longstanding history of severe constipation who was referred for suspected Irritable Bowel Syndrome. Instead, serologies showed all three of these tests to be markedly positive for tissue transglutaminase. IgA endomysial antibody was also positive, though testing of total immunoglobulin A and HLA did not show increased risk on repeat levels. After several routine consultations, a duodenal biopsy was performed and her Marsh score was 2 (intraepithelial lymphocytosis only). Given the discordance between her symptoms and her biopsy as well as persistently high titers of antibodies since beginning a gluten-free diet, a repeat biopsy was performed over 4 years later. Patient re-attended clinic at ten years of age. On repeat endoscopy, she had changes consistent with increased intraepithelial lymphocytosis and maleylation (Villa score of 1b), a score lower than at her initial duodenal biopsy: Marsh 1 (inflammation) instead of Marsh 0 (normal). While it is atypical to have duodenal lymphocytosis alone represent symptomatic celiac, in this case the evidence warranted a diagnosis of celiac disease (with hyperplastic lymphocytosis representing early endoscopic changes). Her anti-tTG was always significantly elevated as well as the EMA. It should be noted that initial biopsies are not available for CD3+ staining to look for lymphocytosis alone at her original presentation. However, this article discusses her presentation and diagnosis in the context of the risk stratification theme of this Special Issue. Bloating or swelling (Q1 for Gastrointestinal-Irritable Bowel Syndrome), Seek medical care for abdominal swelling (Q2 for Gastrointestinal-Irritable Bowel Syndrome), Abdominal tenderness (Q3 for Gastrointestinal-Irritable Bowel Syndrome), Take medication for abdominal pain (Q5 for Gastrointestinal-Irritable Bowel Syndrome) were all rated in the negative, consistent with the diagnosis of celiac disease on the CHOP Modified-Fickie.
Keywords: Celiac disease; Gluten; Immune system
Copyright © 2019 by The American Society for BioMedicine and BM-Publisher, Inc.
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How to cite this article
Wakeman MC, Slavin PA, Gismondi RT. Risk stratification in pediatric celiac disease. American Journal of BioMedicine 2019;7(2):50-63.
Article outline
1. Abstract
2. Keywords
3. Introduction
4. Discussion
7. Acknowledgements
8. References