Abstract
Ménétrier disease (MD) was described for the first time in 1888, and 60 cases have been reported until now. MD is a hypertrophic gastropathy characterized by giant rugal folds, hypochlorhydria, protein loss, and symptoms such as nausea, vomiting, anorexia, abdominal pain, and edema secondary to hypo-albuminemia.
This chapter discusses its etiology, diagnosis, and management.
The pathophysiology of MD is still uncertain. Overexpression of TGF-α with transformation of the gastric mucosa has been observed, which is possibly mediated by genetics and provoked by an infectious trigger. A gastroscopy with biopsy is the gold standard for diagnosis of MD. In children, the disease is self-limiting and only needs supportive treatment. In general, children have a good prognosis and recover spontaneously within a few weeks.
Few cases of MD in children, all with a different etiology, were described in the past years.
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Abbreviations
- HP:
-
Helicobacter pylori
- MD:
-
Ménétrier disease
- TGF-α:
-
Transforming growth factor alpha
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Kikilion, J., Levy, E.I., Vandenplas, Y. (2022). Ménétrier Disease in Children. In: Guandalini, S., Dhawan, A. (eds) Textbook of Pediatric Gastroenterology, Hepatology and Nutrition. Springer, Cham. https://doi.org/10.1007/978-3-030-80068-0_13
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DOI: https://doi.org/10.1007/978-3-030-80068-0_13
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