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长发公主综合征(胃内毛粪石),一种伴有全身性水肿的罕见表现:病例报告及文献复习

Rapunzel syndrome (gastric trichobezoar), a rare presentation with generalised oedema: case report and review of the literature.

作者信息

Nour Islam, Abd Alatef Mona, Megahed Ahmed, Yahia Sohier, Wahba Yahya, Shabaan Abd Elazeez

机构信息

a Department of Paediatrics, Faculty of Medicine , Mansoura University , Mansoura , Egypt.

出版信息

Paediatr Int Child Health. 2019 Feb;39(1):76-78. doi: 10.1080/20469047.2017.1389809. Epub 2017 Oct 23.

Abstract

Rapunzel syndrome is a rare form of gastric trichobezoar. A 4-year-old girl presented with generalised oedema and an epigastric mass. Her family was of a relatively low socio-economic background. There was microcytic hypochromic anaemia, hypoalbuminaemia and an elevated α1-antitrypsin clearance. Abdominal ultrasound and non-contrast computed tomography demonstrated a heterogeneous mass related to the stomach. Upper gastro-intestinal tract endoscopy failed to remove it. Surgical laparotomy was undertaken through a single anterior gastrotomy incision and a large mass was extracted which was a cast of the duodenum and stomach and had a tail of approximately 60 cm in length which extended to the jejunum. Low socio-economic status, child neglect and pica are risk factors for trichobezoars. Surgical laparotomy is the optimal treatment of large bezoars.

摘要

长发公主综合征是胃毛石症的一种罕见形式。一名4岁女孩出现全身水肿和上腹部肿块。她的家庭社会经济背景相对较低。存在小细胞低色素性贫血、低白蛋白血症以及α1-抗胰蛋白酶清除率升高。腹部超声和非增强计算机断层扫描显示有一个与胃相关的不均匀肿块。上消化道内镜检查未能将其取出。通过单一的前胃切开术切口进行手术剖腹探查,取出了一个大肿块,该肿块是十二指肠和胃的铸型,有一条约60厘米长的尾巴延伸至空肠。社会经济地位低、儿童忽视和异食癖是毛石症的危险因素。手术剖腹探查是大型毛石症的最佳治疗方法。

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