Scott H W, Shull H J, Law D H, Burko H, Page D L
Ann Surg. 1975 May;181(5):765-77. doi: 10.1097/00000658-197505000-00036.
Three patients with Menetrier's disease and protein-losing gastropathy who were studied during a 12 year period have been presented. The characteristic findings which differentiate them from patients with hypertrophic hypersecretory gastropathy, including the Zollinger-Ellison syndrome, are: 1) hypertrophy of gastric mucosa with giant rugal folds involving the fundus, cardia and body of the stomach but sparing the antrum; 2) muscosal hypertrophy consisting of gastric mjcus-secreting cells while parietal cells and chief cells are diminished in number and may be absent from many microscopic sections; 3) gastric secretion of large volume containing excess mucus, low to absent hydrochloric acid and protein concentration 5 or 6 times normal (1.7 mg/ml); 4) hypoalbuminemia and hypoglobulinemia due to loss of serum proteins fron gastric mucosa into the gastric lumen; 5) rare association with gastric ulcer. Unlike the Zollinger-Ellison syndrome none of our patients had duodenal ucler or multiple endocrine adenomatosis or a family history of these conditions. We have found no authenticated reports in the literature which document a relationship of Menetrier's disease ( as defined above) with multiple endocrine adenomatosis. Menetrier's disease with protein-losing gastropathy is a potentially lethal disorder of unknown cause with no specific treatment. Resection of the site of gastric protein losses as first done by Waugh is logical and effective. One of our three patients died in hospital before gastrectomy was done. Two others have done well for 11 months and 12 years, respectively, after total gastrectomy with Roux-en-Y esophagojejunostomy and Hunt-Lawrence jejunal pouch.
本文报告了在12年期间研究的3例梅内特里耶病（Menetrier's disease）合并蛋白丢失性胃病的患者。将其与肥厚性高分泌性胃病患者（包括佐林格-埃利森综合征）区分开来的特征性表现为：1）胃黏膜肥大，伴有巨大皱襞，累及胃底、贲门和胃体，但胃窦未受累；2）黏膜肥大由胃黏液分泌细胞组成，而壁细胞和主细胞数量减少，在许多显微镜切片中可能缺如；3）大量胃液分泌，含有过量黏液，盐酸含量低或无，蛋白质浓度为正常的5或6倍（1.7mg/ml）；4）由于血清蛋白从胃黏膜进入胃腔而导致低白蛋白血症和低球蛋白血症；5）与胃溃疡罕见相关。与佐林格-埃利森综合征不同，我们的患者均无十二指肠溃疡、多发性内分泌腺瘤病或这些疾病的家族史。我们在文献中未发现有经证实的报告记录梅内特里耶病（如上所定义）与多发性内分泌腺瘤病之间的关系。梅内特里耶病合并蛋白丢失性胃病是一种病因不明的潜在致命性疾病，没有特效治疗方法。如沃（Waugh）首先所做的那样，切除胃蛋白丢失部位是合理且有效的。我们的3例患者中有1例在胃切除术之前死于医院。另外2例患者在接受全胃切除加 Roux-en-Y 食管空肠吻合术和亨特-劳伦斯（Hunt-Lawrence）空肠袋手术后，分别在11个月和12年内情况良好。
