Autosomal Dominant Ménétrier‐like Disease

Strisciuglio, Caterina; Corleto, Vito D.; Brunetti‐Pierri, Nicola; Piccolo, Pasquale; Sangermano, Riccardo; Rindi, Guido; Martini, Maurizio; D’Armiento, Francesco Paolo; Staiano, Annamaria; Miele, Erasmo

doi:10.1097/mpg.0b013e3182645c2f

Cited by 9 publications

(6 citation statements)

References 30 publications

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“…Gastroscopy, biopsies, and cultures must be performed to confirm the diagnosis of MD. MD is also characterized by endoscopic findings of thickened gastric mucosal folds that are predominantly present in the body and the fundus of the stomach, relatively sparing the antrum (Table 1) [1][2][3][4][5][6][7][8][9]. The most striking feature of MD, a histological sine qua non, is foveolar hyperplasia (expansion of the mucosal cell surface), which leads to thickening of the gastric mucosa.…”

Section: Diagnostics and Histological Findingsmentioning

confidence: 99%

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Diagnosis and Management of Ménétrier Disease in Children: A Case Series Review

Krikilion

Levy

Vandenplas

2021

Pediatr Gastroenterol Hepatol Nutr

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Purpose: Ménétrier disease (MD) was first described in 1888, and 50 cases have been reported until now. We aimed to discuss the etiology, diagnostics, and management of MD in children. Methods: We searched for case reports published from 2014 till 2019 in English using PubMed. Articles were selected using subject headings and key words of interest to the topic. Interesting references of the included articles were also included. Results: The pathophysiology of MD is still uncertain. However, overexpression of transforming growth factor alpha with transformation of the gastric mucosa has been observed, which may be mediated by genetics and provoked by an infectious trigger. Clinically, MD is diagnosed by abdominal pain, vomiting, anorexia, and edema secondary to hypoalbuminemia. A gastroscopy with biopsy is the gold standard for the diagnosis of MD. In children, the disease is self-limiting and only requires supportive treatment. In general, children have a good prognosis and recover spontaneously within a few weeks. Conclusion: Few pediatric cases of MD have been described in recent years, and with all different etiology. Endoscopy with biopsy remains the golden standard for the diagnosis of MD, and in children, the disease is self-limiting.

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Section: Diagnostics and Histological Findingsmentioning

confidence: 99%

“…Until now, there have only been approximately 50 pediatric cases of MD (Table 1) [1][2][3][4][5][6][7][8][9] reported in the literature, the majority of which are case series. In this review, we discuss the etiology and propose guidance for the diagnosis and management of MD.…”

Section: Introductionmentioning

confidence: 99%

Diagnosis and Management of Ménétrier Disease in Children: A Case Series Review

Krikilion

Levy

Vandenplas

2021

Pediatr Gastroenterol Hepatol Nutr

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“…Ménétrier disease 2 , we speculate that NOTCH pathway might also be involved in the pathogenesis of Ménétrier syndrome.…”

Section: Textmentioning

confidence: 99%

“…Ménétrier syndrome, the gastric lesions in the family with Ménétrier-like syndrome 2 showed EGFR overexpression (Fig. 1B).…”

Section: Textmentioning

confidence: 99%

MIB2variants altering NOTCH signalling result in left ventricle hypertrabeculation/non-compaction and are associated with Ménétrier-like gastropathy

et al. 2016

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“…Menetrier's disease is a rare gastropathy characterized by the presence of hypertrophic gastric folds, lack of acid production, excess mucus secretion, anemia, and low serum albumin (1). The onset of the disease shows clinical and prognostic differences in pediatric and adult patients.…”

Section: Introductionmentioning

confidence: 99%

Cytomegalovirus-Associated Protein-Losing Gastropathy (Menetrier’s Disease) in Childhood

Urgancı¹,

Gulec²,

Kılıçaslan³

et al. 2016

JAREM

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Pediatric Menetrier disease is a rare clinical event with unknown etiology, which has a different course from that in adults. It is characterized by gastric hypertrophy and hypoalbuminemia secondary to protein loss throughout the gastric mucosa. Menetrier disease is usually self-limiting in children. In particular, in patients with immune deficiency, one of the most important cause for Menetrier disease is cytomegalovirus (CMV) infection. This infection can also be observed in immunocompetent children. A girl aged two and a half years with a one-week history of generalized edema, weakness, and decreased urine output was admitted at the Pediatric Department of Şişli Hamidiye Etfal Training and Research Hospital. Periorbital and pretibial edema, abdominal distension, and ascites were detected and laboratory studies revealed hypoalbuminemia and elevated fecal alpha-1 antitrypsin excretion. According to the serological investigations, anti-CMV IgM antibodies were detected positive and CMV DNA values were found to be 1170 copies/mL. The patient was treated with intravenous albumin infusion, PPI therapy, and a high-protein and low-salt diet. On the 10 th day of treatment, her condition gradually improved without any antiviral drug. We prepared this presentation because we determined CMV as an etiological agent of protein-losing gastropathy in a child with a healthy immune system. (JAREM 2016; 6: 56-8)

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Autosomal Dominant Ménétrier‐like Disease

Cited by 9 publications

References 30 publications

Diagnosis and Management of Ménétrier Disease in Children: A Case Series Review

Diagnosis and Management of Ménétrier Disease in Children: A Case Series Review

MIB2variants altering NOTCH signalling result in left ventricle hypertrabeculation/non-compaction and are associated with Ménétrier-like gastropathy

Cytomegalovirus-Associated Protein-Losing Gastropathy (Menetrier’s Disease) in Childhood

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