Autosomal dominant Ménétrier-like disease.

Guido Rindi, Maurizio Martini, C Stisciuglio, Vd Corleto, N Brunetti Pierri, P Piccolo, R Sangermano, Fp D'Armiento, A Staiano, E. Miele

Research output: Contribution to journalArticlepeer-review

5 Citations (Scopus)

Abstract

BACKGROUND: Familial occurrence of Ménétrier disease is 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 transforming growth factor α (TGF-α) immunohistochemistry and real-time polymerase chain reaction showed no increase in TGF-α expression. CONCLUSIONS: We describe a unique, 4-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 no increase in the levels of gastric TGF-α. Members of this family may be affected by a novel and previously unrecognised hereditary form of gastric hyperplasia.
Original languageEnglish
Pages (from-to)717-720
Number of pages4
JournalJournal of Pediatric Gastroenterology and Nutrition
DOIs
Publication statusPublished - 2012

Keywords

  • Metrier

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