Menetrier Disease: A Nephrotic Syndrome of the Stomach
International Research Journal of Gastroenterology and Hepatology, Volume 5, Issue 1,
Page 137-146
Abstract
Menetrier Disease (MD) is a rare acquired disease that affects middle-aged men in their mid 40's and, to a lesser extent, women and children. Pierre Eugene Menetrier first described it. It is a premalignant disorder, sometimes associated with Helicobacter Pylori in adults, and Giardia lamblia and Cytomegalovirus in children. The enlargement of the rugae folds is due to increase production of transforming Growth Factor-α (TGF-α) ligand, which subsequently activates epidermal growth factor receptor (EGFR) that stimulate the proliferation (hyperplasia) of the mucosal epithelium. Glandular dysfunction and protein leaks into the stomach result in anemia, hypoproteinemia, and edema. These are distinguishing characteristics of the pathology. Diagnosis is made by barium esophagogram, esophagogastroduodenoscopy (EGD), and serology. A monoclonal antibody called cetuximab (anti-EGFR) is the first treatment for Menetrier disease. Other supportive therapies are regimens for peptic ulcer disease, proton pump inhibitors, anticholinergic drugs, steroids, a high-protein diet, and gastrectomy. Prognosis is better for children than adults because of higher risk of developing gastric cancer. The review article aims to discuss this rare gastric mucosa (foveolar) disorder called Menetrier and support integration, research and learning among medical students and health professionals.
- Menetrier syndrome
- hypertrophy of the stomach
- H. pylori
- EGFR
- TGF-α
How to Cite
References
Menetrier, Pierre. In: Archives de physiologie normale et pathologique. 1888(1):32-55.
Feldman, Mark, and William O. Tschumy Jr. “Menetrier Disease.” NORD (National Organization for Rare Disorders). (Latest version, 2017).
Available: https://rarediseases.org/rare-diseases/menetrier-disease/
Pesce F, Barabino A, Dufour C, Caffarena PE, Callea F, Gatti R. J Pediatr Gastroenterol Nutr. 1992;14:323-6.
Xiong, LI-Shou, and Ying-Ying Gong. Natural History of Adult-Onset Ménétrier’s Disease: Report of a Case with 9-Year Follow-Up.Experimental and Therapeutic Medicine. 2016;11(6):2462–2466.
DOI: 10.3892/etm.2016.3181.
Gore, Meghana, et al. “Menetrier Disease.” PubMed, StatPearls Publishing; 2022.
Coffey RJ, Tanksley J. Pierre Ménétrier and his disease. Trans Am Clin Climatol Assoc. 2012;123:126-33.
Suter WR, Neuweiler J, Borovicka J, Binek J, Fantin AC, Meyenberger C. Cytomegalovirus-induced transient protein-losing hypertrophic gastropathy in an immunocompetent adult. Digestion. 2000; 62:276–9.
Bluth RF, Carpenter HA, Pittelkow MR, Page DL, Coffey RJ. Immunolocalization of transforming growth factor-alpha in normal and diseased human gastric mucosa. Hum Pathol. 1995; 26(12):1333-1340.
DOI: 10.1016/0046-8177(95)90298-8
Xiong, LI-Shou, and Ying-Ying Gong. “Natural History of Adult-Onset Ménétrier’s Disease: Report of a Case with 9-Year Follow-Up.” Experimental and Therapeutic Medicine. 2016;11(6):2462–2466.
DOI: 3892/etm.2016.3181.
Dempsey PJ, Goldenring JR, Soroka CJ, et al. Possible role of transforming growth factor alpha in the pathogenesis of Ménétrier's disease: Supportive evidence from humans and transgenic mice. Gastroenterology 1992;103:1950–63.
Tan DT, Stempien SJ, Dagradi AE. "The clinical spectrum of hypertrophic hypersecretory gastropathy. Report of 50 patients". Gastrointest. Endosc. 1971; 18(2):69–73. .
Krikilion, Jasmina, et al. “Diagnosis and Management of Ménétrier Disease in Children: A Case Series Review.” Pediatric Gastroenterology, Hepatology & Nutrition. 2021;24(1):109,
DOI: 10.5223/pghn.2021.24.1.109.
Rodriguez, Nicole M, and Karen Shackelford. “Pernicious Anemia.” Nih.gov, StatPearls Publishing, 10 Apr. 2019,
“Ménètrier’s Disease Pathophysiology.” Www.youtube.com, Medicosis Perfectionalis, 13 Aug. 2017, www.youtube.com/watch? v=qblOVt-4WsM. Accessed 2 Aug. 2022.
Adapted from “Ménètrier’s Disease Pathophysiology.” Www.youtube.com, Medicosis Perfectionalis, 13 Aug. 2017, www.youtube.com/watch?v=qblOVt-4WsM. Accessed 2 Aug. 2022.
Setakhr V, Muller G, Hoang P, Lambert AS, Geubel A. Cytomegalovirus associated protein losing gastropathy in an immunocompetent adult: a case report. Acta Gastroenterol Belg. 2007; 70:296–9.
Smith, Robert. “Menetrier Disease - Gastrointestinal - Medbullets Step 1.” Step1.Medbullets.com, step1.medbullets.com/gastrointestinal/110041/menetrier-disease.
Eisenstat DD, Griffiths AM, Cutz E, Petric M, Drumm B. Acute cytomegalovirus infection in a child with Ménétrier’s disease. Gastroenterology. 1995; 109:592–5.
Wolfsen HC, Carpenter HA, Talley NJ. Ménétrier’s disease: A form of hypertrophic gastropathy or gastritis? Gastroenterology. 1993; 104:1310–9.
Chebli JMF, Chebli LA, Ribeiro TCDR, Gaburri PD. Severe hypoproteinemia as a harbinger of Ménétrier’s disease in autoimmune pancreatitis; 2017.
Friedman, Joseph, et al. “Ménétrier Disease.” Radio Graphics. 2009;29(1): 297–301.
DOI: 10.1148/rg.291075216.
Rich A, Toro TZ, Tanksley J, et al. Distinguishing Ménétrier’s disease from its mimics. Gut. 2010;59:1617–24].
Sisson G, Harris AW. Ménétrier's disease. Gut. 2007;56(1):156.
Distinguishing Ménétrier’s Disease from Its Mimics. Gut. 2010;59(12):1617–1624.
DOI: 10.1136/gut.2010.220061.- Table used with permission
Xiao SY, Hart J. Marked gastric foveolar hyperplasia associated with active cytomegalovirus infection. Am J Gastroenterol. 2001;96:223–6.
Almazar AE, Penfield JD, Saito YA, Talley NJ. Survival Times of Patients with Menetrier's Disease and Risk of Gastric Cancer. Clin Gastroenterol Hepatol. 2021; 19(4):707-712.
Takagi H, Fukusato T, Kawaharada U, Kuboyama S, Merlino G, Tsutsumi Y. Histochemical analysis of hyperplastic stomach of TGF-alpha transgenic mice. Dig Dis Sci. 1997;42:91–8.
Frederiksen HJ, Olsen N, Krag E. Cimetidine treatment of Menetrier’s syndrome. Ugeskr Laeger. 1978;140:298–9.
Krol TC, Isenberg JI. Remission in a patient with Menetrier’s disease: spontaneous or induced by treatment? J. Clin Gastroenterol. 1985;7:161–8.
Fiske, William H., et al. “Efficacy of Cetuximab in the Treatment of Ménétrier’s Disease.” Science Translational Medicine. 2009;1(8). www.ncbi.nlm.nih.gov/pmc/articles/PMC3638759/, 10.1126/scitranslmed.3000320. Accessed 29 July 2022.
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