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11岁营养不良女性患长发公主综合征并发脓毒症、肠穿孔和胰腺炎

Rapunzel Syndrome Complicated by Sepsis, Bowel Perforation, and Pancreatitis in an 11-year-old Malnourished Female.

作者信息

Daniel Rhea, Arbuthnot Mary, Ramireddy Srinivas, Nair Supriya

机构信息

From the Department of Pediatric Gastroenterology, Hepatology, and Nutrition, McGovern Medical School, University of Texas Health Science Center and Children's Memorial Hermann Hospital, Houston, TX.

Department of Pediatric Surgery, McGovern Medical School, University of Texas Health Science Center and Children's Memorial Hermann Hospital, Houston, TX.

出版信息

JPGN Rep. 2022 Jan 24;3(1):e171. doi: 10.1097/PG9.0000000000000171. eCollection 2022 Feb.

Abstract

The most common presenting symptoms of Rapunzel syndrome include abdominal pain (37%), nausea and vomiting (33.3%), obstruction (25.9%), and peritonitis (18.3%). Less commonly, patients may present with weight loss (7.4%) or intussusception (7.4%). Exceedingly rare complications of Rapunzel syndrome include gastric ulceration, obstructive jaundice, and acute pancreatitis as well as other malabsorptive-related complications including protein-losing enteropathy, iron deficiency, and megaloblastic anemia. This report details the case of an 11-year-old female with Rapunzel syndrome complicated by sepsis, a rare complication reported in only 2% of patients.

摘要

长发公主综合征最常见的症状包括腹痛(37%)、恶心和呕吐(33.3%)、肠梗阻(25.9%)和腹膜炎(18.3%)。较少见的情况下,患者可能出现体重减轻(7.4%)或肠套叠(7.4%)。长发公主综合征极为罕见的并发症包括胃溃疡、梗阻性黄疸和急性胰腺炎,以及其他与吸收不良相关的并发症,如蛋白丢失性肠病、缺铁和巨幼细胞贫血。本报告详细介绍了一名11岁女性患长发公主综合征并伴有败血症的病例,败血症是一种仅在2%的患者中报道过的罕见并发症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/02cf/10158331/f50056a97656/pg9-3-e171-g001.jpg

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