Abstract

BackgroundClassic form of celiac disease (CCD) presents with diarrhea and is traditionally taught as malabsorption syndrome. This form of CD is diagnosed with ease but non-classical form presenting without diarrhea is often missed and heavily underdiagnosed.ObjectiveTo determine the clinical spectrum of patients with CD.MethodsThis study was conducted in the Department of Gastroenterology & Hepatology at the Children’s Hospital, Lahore. Confirmed CD children according to NASPGHAN (North American Society of Pediatric Gastroenterology, Hepatology & Nutrition) criteria were enrolled in this study from June to September, 2020. Initial positivity followed by small bowel biopsy with Modified Marsh 2 and above is considered consistent with the diagnosis of CD.ResultsA total of 90 patients were selected according to NASPGHAN criteria, 77 (85.6%) patients had CCD whereas 13 (14.4%) patients had non-classical CD (NCCD). The mean ± SD age at diagnosis of CCD was 2.6 ± 2.3 years whereas mean ± SD in NCCD was 9 ± 1.8 years. Females clearly predominate in our cohort in general. Chronic diarrhea followed by failure to thrive (85%) were the most common symptoms in CCD whereas short stature (69%) was the most frequent feature in NCCD. Among CD patients, tissue transglutaminase-​immunoglobulin A (TTG-IgA) titre was significantly high (>10 times) in 80% of CD patients and the rest had positivity but not up to 10 times. There was no significant difference in titre of anti-TTG between CCD and NCCD.ConclusionClassical CD is still the most common in developing countries like Pakistan. High index of suspicion of CD should be maintained for patients who present with short stature, recurrent abdominal pain and refractory anemia.

Highlights

  • Celiac disease (CD) is an immune-mediated systemic disorder elicited by gluten and related prolamines in genetically susceptible individuals resulting in injury to small intestinal mucosa [1]

  • A total of 90 patients were selected according to NASPGHAN criteria, 77 (85.6%) patients had celiac disease (CCD) whereas 13 (14.4%) patients had non-classical CD (NCCD)

  • Chronic diarrhea followed by failure to thrive (85%) were the most common symptoms in CCD whereas short stature (69%) was the most frequent feature in NCCD

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Summary

Introduction

Celiac disease (CD) is an immune-mediated systemic disorder elicited by gluten and related prolamines in genetically susceptible individuals resulting in injury to small intestinal mucosa [1]. The gliadin peptide gains entry into the small bowel submucosa where it binds with HLA peptides on antigen-presenting cells leading to intraepithelial cell proliferation through activation of helper T cells [4]. This causes villous atrophy, crypt hyperplasia and the formation of antibodies related to CD. Classic form of celiac disease (CCD) presents with diarrhea and is traditionally taught as malabsorption syndrome. This form of CD is diagnosed with ease but non-classical form presenting without diarrhea is often missed and heavily underdiagnosed

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