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全胃切除术治疗内科治疗无效的梅内特里耶病:一例报告

Total gastrectomy for the treatment of Menetrier's disease persistent to medical therapy: A case report.

作者信息

Parianos Christos, Aggeli Chrysanthi, Sourla Antigoni, Zografos Georgios Nikolaos

机构信息

3rd Department of Surgery, General Hospital Of Athens 'G.Gennimatas", Greece.

Pathology Department Medical School, University of Athens, Athens, Greece.

出版信息

Int J Surg Case Rep. 2020;73:95-99. doi: 10.1016/j.ijscr.2020.06.033. Epub 2020 Jun 30.

Abstract

BACKGROUND

Menetrier's disease is a rare hyperproliferative protein-losing gastropathy of the gastric foveolar epithelium. It is characterized by giant hypertrophic folds, excess mucus secretion, decreased acid secretion and hypoproteinemia due to selective loss of serum proteins across the gastric mucosa. The discovery of transforming growth factor-α overexpression opened the way of epidermal growth factor receptor blockade with cetuximab as first-line treatment modality for Menetrier's disease.

CASE REPORT- ETHODS: We present the case of a 46-year-old female patient with Menetrier's disease. The diagnosis was based on clinical, endoscopic and histological criteria. Two years before the diagnosis of the disease the patient had an episode of deep vein thrombosis and pulmonary embolism. Real time PCR revealed mutation of the gene responsible for coagulation factor II. The patient received anticoagulation therapy and after a period of 6 months a severe anemia due to a gastrointestinal bleeding was presented. The appropriate investigation revealed the presence of Menetrier's disease. The patient was referred to the surgical clinic with persistence of symptoms such as excessive weight loss, anemia and weakness, even after multiple medical treatment, including the monoclonal antibody against the EGFR receptor. A total gastrectomy was performed and the postoperative course was uneventful. One year follow up showed remarkable improvement of her health status.

CONCLUSION

A combination of clinical, laboratory, endoscopic and histopathologic findings is necessary for the diagnosis of this rare disease. Gastrectomy is the treatment of choice for those patients with intractable symptoms and signs refractory to medical therapy.

摘要

背景

梅内特里耶病是一种罕见的胃小凹上皮增生性失蛋白性胃病。其特征为巨大肥厚性皱襞、黏液分泌过多、胃酸分泌减少以及由于血清蛋白经胃黏膜选择性丢失导致的低蛋白血症。转化生长因子-α过表达的发现开启了以西妥昔单抗作为梅内特里耶病一线治疗方式进行表皮生长因子受体阻断的道路。

病例报告——方法:我们报告一例46岁患梅内特里耶病的女性患者。诊断基于临床、内镜及组织学标准。在疾病诊断前两年,该患者曾发生深静脉血栓形成和肺栓塞。实时聚合酶链反应显示负责凝血因子II的基因突变。患者接受了抗凝治疗,6个月后出现因胃肠道出血导致的严重贫血。适当检查发现存在梅内特里耶病。即便经过包括抗表皮生长因子受体单克隆抗体在内的多种药物治疗后,患者仍持续存在体重过度减轻、贫血和虚弱等症状,遂被转诊至外科诊所。实施了全胃切除术,术后病程顺利。一年随访显示其健康状况有显著改善。

结论

对于这种罕见疾病的诊断,临床、实验室、内镜及组织病理学检查结果相结合是必要的。对于那些有难治性症状和体征且药物治疗无效的患者,胃切除术是首选治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4fcb/7355377/60ea66aa3493/gr1.jpg

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