Purpose Due to the patchy nature of mucosal involvement in Celiac disease (CD), histopathological changes may be confined to the duodenal bulb in some patients, leading to diagnostic challenges. This study aimed to investigate the frequency, as well as the clinical and serological characteristics, of ultra-short celiac disease (USCD) in children. Methods Between February 2024 and March 2026, pediatric patients diagnosed with CD based on separate biopsy specimens obtained from the duodenal bulb and distal duodenum, each placed in separate containers, were enrolled in this study. The patients were classified into two groups: the USCD group and the classic CD group. Results Of the 78 patients included, 47 (60.25%) were female, and the overall mean age at diagnosis was 9.58 ± 4.21 years. The median tissue transglutaminase IgA (tTG-IgA) level was 125.50 (IQR: 21.30–200.00) IU/L. Sixteen patients (20.51%) had histopathologically confirmed USCD. The median tTG-IgA level was significantly lower in the USCD group compared to the classic CD group (22.50 vs. 162.00 IU/L; p < 0.05). Additionally, 87.50% of the patients in the USCD group lacked the classic gastrointestinal symptoms typically associated with malabsorption, presenting instead with isolated growth retardation. Conclusion The rate of isolated duodenal bulb involvement in children was remarkably high (20.51%). Ultra-short celiac disease is more prevalent in pediatric patients presenting with fewer classic gastrointestinal findings and tTG-IgA levels below 10 × ULN. Therefore, it is critically important to obtain at least one biopsy specimen from the duodenal bulb and place it in a separate container. Missing bulb biopsies may result in a significant proportion of patients with isolated duodenal bulb involvement going undiagnosed.<Table Float="No" ID="Taba"> <tgroup cols="1"> <colspec align="left" colname="c1" colnum="1" /> <tbody> <row> <entry align="left" colname="c1"> What is known </entry> </row> <row> <entry align="left" colname="c1"> • The frequency of ultra-short celiac disease (USCD) varies between 2.4% and 22.0% for more accurate differentiation </entry> </row> <row> <entry align="left" colname="c1"> • Intestinal biopsy remains mandatory to diagnose CD in patients with tTG-IgA levels below 10 times the upper limit of normal (10 × ULN) </entry> </row> <row> <entry align="left" colname="c1"> • Duodenal bulb biopsies are not routinely performed even in specialized centers during endoscopy </entry> </row> <row> <entry align="left" colname="c1"> What is new </entry> </row> <row> <entry align="left" colname="c1"> • The frequency of USCD in children with CD was found to be relatively high (20.51%) </entry> </row> <row> <entry align="left" colname="c1"> • Among patients with USCD, 75.00% exhibited tTG-IgA levels below 10 × ULN </entry> </row> <row> <entry align="left" colname="c1"> • Systematic duodenal bulb biopsy substantially enhances USCD detection in patients with low tTG-IgA levels; without it, approximately one in five patients may remain undiagnosed </entry> </row> </tbody> </tgroup> </Table>

The frequency and characteristics of ultra-short celiac disease in children: A single-center experience

期刊论文
发表日期：

2026年6月30日

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Yasin Sahin,
Ahmet Uzger,
Ahmet Rauf Goktepe

摘要

Purpose

Due to the patchy nature of mucosal involvement in Celiac disease (CD), histopathological changes may be confined to the duodenal bulb in some patients, leading to diagnostic challenges. This study aimed to investigate the frequency, as well as the clinical and serological characteristics, of ultra-short celiac disease (USCD) in children.

Methods

Between February 2024 and March 2026, pediatric patients diagnosed with CD based on separate biopsy specimens obtained from the duodenal bulb and distal duodenum, each placed in separate containers, were enrolled in this study. The patients were classified into two groups: the USCD group and the classic CD group.

Results

Of the 78 patients included, 47 (60.25%) were female, and the overall mean age at diagnosis was 9.58 ± 4.21 years. The median tissue transglutaminase IgA (tTG-IgA) level was 125.50 (IQR: 21.30–200.00) IU/L. Sixteen patients (20.51%) had histopathologically confirmed USCD. The median tTG-IgA level was significantly lower in the USCD group compared to the classic CD group (22.50 vs. 162.00 IU/L; p < 0.05). Additionally, 87.50% of the patients in the USCD group lacked the classic gastrointestinal symptoms typically associated with malabsorption, presenting instead with isolated growth retardation.

Conclusion

The rate of isolated duodenal bulb involvement in children was remarkably high (20.51%). Ultra-short celiac disease is more prevalent in pediatric patients presenting with fewer classic gastrointestinal findings and tTG-IgA levels below 10 × ULN. Therefore, it is critically important to obtain at least one biopsy specimen from the duodenal bulb and place it in a separate container. Missing bulb biopsies may result in a significant proportion of patients with isolated duodenal bulb involvement going undiagnosed.

What is known

• The frequency of ultra-short celiac disease (USCD) varies between 2.4% and 22.0% for more accurate differentiation

• Intestinal biopsy remains mandatory to diagnose CD in patients with tTG-IgA levels below 10 times the upper limit of normal (10 × ULN)

• Duodenal bulb biopsies are not routinely performed even in specialized centers during endoscopy

What is new

• The frequency of USCD in children with CD was found to be relatively high (20.51%)

• Among patients with USCD, 75.00% exhibited tTG-IgA levels below 10 × ULN

• Systematic duodenal bulb biopsy substantially enhances USCD detection in patients with low tTG-IgA levels; without it, approximately one in five patients may remain undiagnosed