Related Experiment Video
Updated: May 21, 2026
Calcification of Vascular Smooth Muscle Cells and Imaging of Aortic Calcification and Inflammation
Published on: May 31, 2016
Autosomal dominant Ménétrier-like disease
Caterina Strisciuglio1, Vito D Corleto, Nicola Brunetti-Pierri
1Department of Pediatrics, Federico II University of Naples, Naples, Italy.
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.
Related Concept Videos
01:43Sex-linked Disorders
01:35Pedigree Analysis
01:21Huntington Disease l: Introduction
02:41Lethal Alleles
Lucien Cuénot discovered lethal alleles in 1905 while studying the inheritance of coat color in mice. The agouti gene is responsible for the color of the coat in mice. This gene codes for an agouti-signaling protein, which is responsible for melanin distribution in mammals. The wild-type allele gives rise to gray-brown coat color in mice, while the mutant allele gives rise to yellow coat color. In addition to coat color, the agouti gene is associated with the yellow...
01:43Incomplete Dominance
01:29Cardiomyopathy III: Hypertrophic Cardiomyopathy