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Ulcerative jejunitis in a child with celiac disease.

Sigman T, Nguyen VH, Costea F, Sant'Anna A, Seidman EG - BMC Gastroenterol (2014)

Bottom Line: Initial upper endoscopy with biopsies was not conclusive for celiac disease.A repeat capsule study and upper endoscopy, 1 year and 4 years following a strict gluten free diet showed endoscopic and histological normalization of the small bowel.The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Pediatric Gastroenterology, Montreal Children's Hospital, Faculty of Medicine, McGill University, 2300 Tupper St, Montreal, Quebec H3H 1P3, Canada. terry.sigman@muhc.mcgill.ca.

ABSTRACT

Background: Celiac disease can present in children and adults with a variety of manifestations including a rare complication known as ulcerative jejunitis. The latter has been associated with refractory celiac disease in adult onset patients. The objective of this case report is to describe the first pediatric case of ulcerative jejunitis in celiac disease, diagnosed by capsule endoscopy, which was not associated with refractory celiac disease.

Case presentation: The 9 year old girl presented with a history of abdominal pain and vomiting. Laboratory investigations revealed a slightly elevated IgA tissue transglutaminase antibody level in the setting of serum IgA deficiency. Initial upper endoscopy with biopsies was not conclusive for celiac disease. Further investigations included positive IgA anti-endomysium antibody, and positive HLA DQ2 typing. Video capsule endoscopy showed delayed appearance of villi until the proximal to mid jejunum and jejunal mucosal ulcerations. Push enteroscopy with biopsies subsequently confirmed the diagnosis of celiac disease and ulcerative jejunitis. Immunohistochemical studies of the intraepithelial lymphocytes and PCR amplification revealed surface expression of CD3 and CD8 and oligoclonal T cell populations. A repeat capsule study and upper endoscopy, 1 year and 4 years following a strict gluten free diet showed endoscopic and histological normalization of the small bowel.

Conclusion: Ulcerative jejunitis in association with celiac disease has never previously been described in children. Capsule endoscopy was essential to both the diagnosis of celiac disease and its associated ulcerative jejunitis. The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.

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Histological findings on a normal diet. Histological confirmation of celiac disease by push enteroscopy.
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Figure 2: Histological findings on a normal diet. Histological confirmation of celiac disease by push enteroscopy.

Mentions: In view of persistent symptoms, further investigations were pursued, which revealed positive EMA and HLA DQ2 typing. CE of the small bowel showed patchy villous atrophy with delayed appearance of villi until the proximal to mid jejunum, as well as areas with slight mucosal scalloping. Moreover, fibrin covered actively bleeding mucosal ulcerations were observed in the jejunum (Figure 1). Her tTG remained slightly elevated (7 U/ml; normal < 5). Push enteroscopy performed 7 months following her initial upper endoscopy, confirmed ulcerative jejunitis. Biopsies revealed subtotal villous atrophy, crypt hyperplasia and an IEL count of 58 lymphocytes/100 enterocytes in the second stage of the duodenum (Marsh classification III B). Changes in the jejunum varied from preserved villous architecture with 82 IEL/100 enterocytes (Marsh I at 65 cm) to partial villous atrophy and crypt hyperplasia with an IEL count of 89/100 enterocytes (Marsh III B at 95 cm), consistent with a diagnosis of celiac disease (Figure 2).


Ulcerative jejunitis in a child with celiac disease.

Sigman T, Nguyen VH, Costea F, Sant'Anna A, Seidman EG - BMC Gastroenterol (2014)

Histological findings on a normal diet. Histological confirmation of celiac disease by push enteroscopy.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4016221&req=5

Figure 2: Histological findings on a normal diet. Histological confirmation of celiac disease by push enteroscopy.
Mentions: In view of persistent symptoms, further investigations were pursued, which revealed positive EMA and HLA DQ2 typing. CE of the small bowel showed patchy villous atrophy with delayed appearance of villi until the proximal to mid jejunum, as well as areas with slight mucosal scalloping. Moreover, fibrin covered actively bleeding mucosal ulcerations were observed in the jejunum (Figure 1). Her tTG remained slightly elevated (7 U/ml; normal < 5). Push enteroscopy performed 7 months following her initial upper endoscopy, confirmed ulcerative jejunitis. Biopsies revealed subtotal villous atrophy, crypt hyperplasia and an IEL count of 58 lymphocytes/100 enterocytes in the second stage of the duodenum (Marsh classification III B). Changes in the jejunum varied from preserved villous architecture with 82 IEL/100 enterocytes (Marsh I at 65 cm) to partial villous atrophy and crypt hyperplasia with an IEL count of 89/100 enterocytes (Marsh III B at 95 cm), consistent with a diagnosis of celiac disease (Figure 2).

Bottom Line: Initial upper endoscopy with biopsies was not conclusive for celiac disease.A repeat capsule study and upper endoscopy, 1 year and 4 years following a strict gluten free diet showed endoscopic and histological normalization of the small bowel.The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Pediatric Gastroenterology, Montreal Children's Hospital, Faculty of Medicine, McGill University, 2300 Tupper St, Montreal, Quebec H3H 1P3, Canada. terry.sigman@muhc.mcgill.ca.

ABSTRACT

Background: Celiac disease can present in children and adults with a variety of manifestations including a rare complication known as ulcerative jejunitis. The latter has been associated with refractory celiac disease in adult onset patients. The objective of this case report is to describe the first pediatric case of ulcerative jejunitis in celiac disease, diagnosed by capsule endoscopy, which was not associated with refractory celiac disease.

Case presentation: The 9 year old girl presented with a history of abdominal pain and vomiting. Laboratory investigations revealed a slightly elevated IgA tissue transglutaminase antibody level in the setting of serum IgA deficiency. Initial upper endoscopy with biopsies was not conclusive for celiac disease. Further investigations included positive IgA anti-endomysium antibody, and positive HLA DQ2 typing. Video capsule endoscopy showed delayed appearance of villi until the proximal to mid jejunum and jejunal mucosal ulcerations. Push enteroscopy with biopsies subsequently confirmed the diagnosis of celiac disease and ulcerative jejunitis. Immunohistochemical studies of the intraepithelial lymphocytes and PCR amplification revealed surface expression of CD3 and CD8 and oligoclonal T cell populations. A repeat capsule study and upper endoscopy, 1 year and 4 years following a strict gluten free diet showed endoscopic and histological normalization of the small bowel.

Conclusion: Ulcerative jejunitis in association with celiac disease has never previously been described in children. Capsule endoscopy was essential to both the diagnosis of celiac disease and its associated ulcerative jejunitis. The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.

Show MeSH
Related in: MedlinePlus