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  • OC81 Eosinophilic esophagitis successfully treated with elimination diet and proton pump inhibitors in a patient with glycogen storage disease type 9c

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OC81 Eosinophilic esophagitis successfully treated with elimination diet and proton pump inhibitors in a patient with glycogen storage disease type 9c
  1. Francesco Pellegrino1,2,
  2. Aimee Wiseman1,
  3. Lucy Jackman1,
  4. Leanne Goh3,
  5. Edward Gaynor1
  1. 1Department of Paediatric Gastroenterology, Great Ormond Street Hospital, London UK
  2. 2Department of Pediatric and Public Health Sciences, Postgraduate School of Pediatrics, Regina Margherita Children Hospital, University of Turin, Turin, Italy
  3. 3Department of Paediatric Allergy. St. Mary, Imperial College Healthcare, London, UK

Abstract

We present the case of a pediatric patient with glycogen storage disease type 9c (GSD9c) who introduced cornstarch into dietary regimen to keep blood glucose levels within safe limits and avoid hypoglycemia.

After the introduction of cornstarch, the patient developed dysphagia and growth impairment (weight 22 kg, z-score -1.12 and height 123 cm, z-score -1.21) and struggled to control his glucose levels properly.

Following multiple admissions, an esophagogastroduodenoscopy revealed eosinophilic esophagitis (EoE). His symptoms slightly improved after a three-months proton pump inhibitor treatment, but the next endoscopy revealed persistent macroscopic changes of the esophagus and absence of histological remission.

After replacement of cornstarch with tapioca starch, his symptoms and his growth improved (weight 33.7 kg, z-score -0.45 and height 140.6 cm, z-score -0.77).

In patients with GSD, slow digestion and absorption of cornstarch is essential to maintain adequate blood glucose levels,1 but in our case its introduction in patient’s diet was associated to EoE development, poor control of metabolic symptoms and poor growth. This is the second case reported in literature of GSD developing EoE after cornstarch introduction to diet,2 with clinical and histological remission after identification of corn as a main allergen trigger and its subsequent elimination.3

References

  1. Ross KM, Ferrecchia IA, Dahlberg KR, et al. Dietary Management of the glycogen storage diseases: evolution of treatment and ongoing controversies. Adv Nutr. 2020 Mar 1;11(2):439–446.

  2. Thornhill C, Saavendra H, Tatevian N, et al. A case of eosinophilic gastroenteritis in a patient with glycogen storage disease type 1a. Open J Clin Med Case Rep. 2017;1272.

  3. Dhar A, Haboubi HN, Attwood SE. British Society of Gastroenterology (BSG) and British Society of Paediatric Gastroenterology, Hepatology and Nutrition (BSPGHAN) joint consensus guidelines on the diagnosis and management of eosinophilic oesophagitis in children and adults. Gut. 2022 Aug;71(8):1459–1487.

https://doi.org/10.1136/flgastro-2024-bspghan.77

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