BACKGROUND:: Familial occurrence of Ménétrier disease is very rare and has been reported only in few instances. METHODS:: affected patients from a large pedigree were evaluated at the clinical, endoscopic, and pathological levels. RESULTS:: affected members presented with gastropathy of variable severity but without protein loss. Endoscopy and pathology findings were consistent with Ménétrier disease. However, gastric TGF-α immunohistochemistry and real time PCR showed no increase in TGF-α expression. CONCLUSIONS:: We describe a unique, four-generation pedigree with autosomal dominant gastropathy exhibiting the typical clinical, endoscopic, and pathological findings of Ménétrier-like disease, though in the absence of protein loss and with normal levels of gastric TGF-α. Members of this family may be affected by a novel and previously unrecognized hereditary form of gastric hyperplasia.

Autosomal dominant Ménétrier-like disease / C., Strisciuglio; Corleto, Vito Domenico; N., Brunetti Pierri; P., Piccolo; R., Sangermano; G., Rindi; M., Martini; F. P., D'Armiento; A., Staiano; E., Miele. - In: JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION. - ISSN 0277-2116. - STAMPA. - (2012). [10.1097/MPG.0b013e3182645c2f]

Autosomal dominant Ménétrier-like disease.

CORLETO, Vito Domenico;
2012

Abstract

BACKGROUND:: Familial occurrence of Ménétrier disease is very rare and has been reported only in few instances. METHODS:: affected patients from a large pedigree were evaluated at the clinical, endoscopic, and pathological levels. RESULTS:: affected members presented with gastropathy of variable severity but without protein loss. Endoscopy and pathology findings were consistent with Ménétrier disease. However, gastric TGF-α immunohistochemistry and real time PCR showed no increase in TGF-α expression. CONCLUSIONS:: We describe a unique, four-generation pedigree with autosomal dominant gastropathy exhibiting the typical clinical, endoscopic, and pathological findings of Ménétrier-like disease, though in the absence of protein loss and with normal levels of gastric TGF-α. Members of this family may be affected by a novel and previously unrecognized hereditary form of gastric hyperplasia.
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Autosomal dominant Ménétrier-like disease / C., Strisciuglio; Corleto, Vito Domenico; N., Brunetti Pierri; P., Piccolo; R., Sangermano; G., Rindi; M., Martini; F. P., D'Armiento; A., Staiano; E., Miele. - In: JOURNAL OF PEDIATRIC GASTROENTEROLOGY AND NUTRITION. - ISSN 0277-2116. - STAMPA. - (2012). [10.1097/MPG.0b013e3182645c2f]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/450745
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