Barros Ana R, Monteiro Sara, Silva Jorge
Department of Gastroenterology, Unidade Local de Saúde Tâmega e Sousa, Penafiel 4560-136, Porto, Portugal.
World J Gastroenterol. 2025 Aug 28;31(32):108401. doi: 10.3748/wjg.v31.i32.108401.
Ménétrier disease (MD) is a rare gastric disorder characterized by hypertrophy of the gastric mucosa, resulting in giant gastric folds, excessive mucus secretion, and significant protein loss. It is most common in middle-aged males, although cases have also been reported in children. This condition, also known as hyperplastic hypersecretory gastropathy, primarily affects the fundus and body of the stomach, leading to protein-losing gastropathy due to increased mucosal permeability. The exact pathogenesis of MD remains unclear; however, it has been linked to excessive transforming growth factor-alpha signaling the epidermal growth factor receptor (EGFR), which promotes mucosal hyperplasia. Clinical manifestations include epigastric pain, nausea, vomiting, anorexia, weight loss, and peripheral edema due to protein-losing enteropathy. Diagnosis is based on clinical presentation, endoscopic findings, and histopathology revealing foveolar hyperplasia and glandular atrophy. Treatment options vary from symptomatic management with proton pump inhibitors and nutritional support to monoclonal antibodies targeting EGFR (, cetuximab) in severe cases. In refractory situations, gastrectomy may be required. This review highlights the current understanding, diagnostic approaches, and therapeutic strategies for MD.
门脉性胃病（MD）是一种罕见的胃部疾病，其特征为胃黏膜肥大，导致巨大胃皱襞、黏液分泌过多以及大量蛋白质丢失。该病在中年男性中最为常见，不过儿童病例也有报道。这种病症，也被称为增生性高分泌性胃病，主要影响胃底和胃体，由于黏膜通透性增加导致蛋白丢失性胃病。MD的确切发病机制尚不清楚；然而，它与转化生长因子-α信号过度激活表皮生长因子受体（EGFR）有关，EGFR会促进黏膜增生。临床表现包括上腹部疼痛、恶心、呕吐、厌食、体重减轻以及因蛋白丢失性肠病导致的外周水肿。诊断基于临床表现、内镜检查结果以及组织病理学显示的小凹增生和腺体萎缩。治疗方案从使用质子泵抑制剂进行对症治疗和营养支持到在严重病例中使用靶向EGFR的单克隆抗体（如西妥昔单抗）不等。在难治性情况下，可能需要进行胃切除术。本综述重点介绍了目前对MD的认识、诊断方法和治疗策略。
