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Ultra-short Celiac Disease Is a Distinct and Milder Phenotype of the Disease in Children

  • Reut Doyev
  • Shlomi Cohen
  • Amir Ben-Tov
  • Yael Weintraub
  • Achiya Amir
  • Tut Galai
  • Hadar Moran-Lev
  • Anat Yerushalmy-Feler
Original Article
  • 26 Downloads

Abstract

Background and Aims

Approximately 10% of children with celiac disease (CD) have ultra-short celiac disease (USCD), where histological abnormalities are limited to the duodenal bulb. The aim of our retrospective study was to identify clinical and serological characteristics at baseline and at follow-up of children with USCD.

Methods

All children that were diagnosed with CD in our unit during 7/2010–12/2017, in whom biopsies were taken from duodenal bulb and second part, were included. We compared disease characteristics and course between children with USCD and children with involvement in the second part of the duodenum.

Results

Out of 3740 children who underwent upper gastrointestinal endoscopies, 648 were diagnosed with CD. Seventy-one (11%) of those children had limited involvement in the duodenal bulb. The USCD group included more females (P = 0.021), were older (P = 0.005), had a lower prevalence of diarrhea (P = 0.003), anemia (P = 0.007), anti-tissue transglutaminase (TTG) antibodies count (P < 0.001) at presentation, lower frequency of endoscopic abnormality, lower Marsh score, and a trend toward shorter time to the normalization of anti-TTG antibodies under a gluten-free diet compared to the extensive CD. There were no differences in body mass index or duration of symptoms before diagnosis.

Conclusion

Children with USCD presented with a distinct phenotype of milder symptoms, lower celiac serology, and milder endoscopic and histological findings, with a trend toward faster normalization under a gluten-free diet compared to those with extensive CD. Further studies are needed to determine the long-term course and prognosis of USCD.

Keywords

Celiac disease Children Bulb Duodenum 

Notes

Compliance with ethical standards

Conflict of interest

All authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Reut Doyev
    • 1
    • 2
  • Shlomi Cohen
    • 1
    • 2
  • Amir Ben-Tov
    • 1
    • 2
  • Yael Weintraub
    • 1
    • 2
  • Achiya Amir
    • 1
    • 2
  • Tut Galai
    • 1
    • 2
  • Hadar Moran-Lev
    • 1
    • 2
  • Anat Yerushalmy-Feler
    • 1
    • 2
  1. 1.Pediatric Gastroenterology Unit, “Dana-Dwek” Children’s HospitalTel Aviv Sourasky Medical CenterTel AvivIsrael
  2. 2.Sackler Faculty of MedicineTel Aviv UniversityTel AvivIsrael

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