Ménétrier disease in Chinese children: a case report and systematic review

Objective: Childhood Ménétrier disease (MD) is an extremely rare protein-losing gastropathy. This case report and systematic literature review aimed to characterize the clinical presentation, diagnosis, treatment, and outcomes of MD in Chinese children, using a well-documented institutional case and all eligible published Chinese pediatric cases. Methods: We retrospectively analyzed one confirmed pediatric MD case admitted to our institution in January 2020. A systematic literature search of PubMed, CNKI, and Wanfang Data was conducted from database inception to January 2026. Eligible publications were single-patient case reports, brief case/image reports, or case-based articles describing Chinese patients younger than 18 years with endoscopic and histological evidence consistent with MD. The quality of included reports was evaluated using the JBI Critical Appraisal Checklist, and descriptive statistics were applied to the aggregated cohort of 16 patients. Results: The index case presented with abdominal pain, edema, polyserositis, severe hypoalbuminemia, giant gastric folds, and histological foveolar hyperplasia. Symptoms resolved after conservative management. The systematic review identified 15 additional eligible published cases, yielding 16 Chinese pediatric patients in total (68.8% male). The median age at presentation/diagnosis was 10.0 years, and most patients were school-aged children or adolescents. Core manifestations included abdominal pain (75.0%), edema (75.0%), and vomiting (62.5%). Hypoalbuminemia was documented in 87.5% of patients, with a median serum albumin level of 20 g/L. Endoscopy consistently showed hypertrophic gastric mucosa, with gastric body involvement in all cases. Histological findings were dominated by foveolar epithelial hyperplasia, tortuous or cystically dilated glands, and variable oxyntic gland atrophy. Among the 12 patients with documented conservative treatment, all achieved improvement or remission. The median time to symptomatic relief was 22 days, whereas endoscopic normalization required a median of 4.5 months. Conclusion: This case report with a systematic review indicates that pediatric Ménétrier disease in China has recognizable clinical, endoscopic, and histological features. It mainly affects school-aged boys and adolescents. Abdominal pain, edema, vomiting, and hypoalbuminemia are key clues. Diagnosis requires endoscopy and histopathology, while conservative treatment generally achieves favorable outcomes despite delayed mucosal recovery.