Abstract
Background. A 38-year-old female presented with a 3-year history of postprandial abdominal pain, refractory nausea, vomiting and hematemesis. She appeared malnourished and her symptoms were refractory to previous treatment with acid-suppressive drugs, prokinetics and antiemetics. Her medical history was significant for a diagnosis of juvenile polyposis syndrome at the age of 14 resulting in a transverse colectomy, and a diagnosis of Crohn's disease in her residual colon at the age of 35 resulting in a total colectomy.
Investigations. Physical examination, blood analysis, esophagogastroduodenoscopy with biopsy, abdominal endoscopic ultrasound, abdominal CT scan, MRI, 24 h urine analysis, MIBG scintigraphy, ocreotide scintigraphy, fluorodeoxyglucose-PET scan and genetic testing for defined polyposis syndromes (SMAD4, BMPR1A).
Diagnosis. Juvenile polyposis syndrome with outlet obstruction of the stomach and excessive hypergastrinemia.
Management. Continuous acid-suppressive therapy, prokinetic therapy and total parenteral nutrition. Repetitive endoscopic polypectomy (also known as debulking) was performed twice and was followed by gastrectomy with duodenoesophageal anastomosis.
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Acknowledgements
Written consent for publication was obtained from the patient. The authors acknowledge the excellent support provided by Heidi Paulsen, Lise Strange and Grazyna Poulsen with regard to immunohistochemical stainings and artwork.
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All authors researched data for the article and contributed equally to reviewing and/or editing of the manuscript before submission. K. D. Papay and M. A. Storr wrote the article. K. D. Papay, S. S. Poulsen, R. Panaccione, J. F. Rehfeld and M. A. Storr provided a substantial contribution to the discussion of content.
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Papay, K., Falck, V., Poulsen, S. et al. Juvenile polyposis of the stomach—a novel cause of hypergastrinemia. Nat Rev Gastroenterol Hepatol 7, 583–588 (2010). https://doi.org/10.1038/nrgastro.2010.138
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DOI: https://doi.org/10.1038/nrgastro.2010.138
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