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长发公主综合征——儿童消化症状极其罕见的病因:一例病例报告及文献综述

Rapunzel Syndrome-An Extremely Rare Cause of Digestive Symptoms in Children: A Case Report and a Review of the Literature.

作者信息

Marginean Cristina Oana, Melit Lorena Elena, Sasaran Maria Oana, Marginean Razvan, Derzsi Zoltan

机构信息

Department of Pediatrics I, George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Târgu Mureş, Târgu Mureş, Romania.

Department of Pediatrics III, George Emil Palade University of Medicine, Pharmacy, Science, and Technology of Târgu Mureş, Târgu Mureş, Romania.

出版信息

Front Pediatr. 2021 Jun 9;9:684379. doi: 10.3389/fped.2021.684379. eCollection 2021.

DOI:10.3389/fped.2021.684379
PMID:34178901
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8219913/
Abstract

Rapunzel syndrome is an extremely rare condition seen in adolescents or young females with psychiatric disorders consisting of a gastric trichobezoar with an extension within the small bowel. The delays in diagnosis are common since in its early stages, it is usually asymptomatic. We report the case of a 13-year-old girl admitted in our clinic for abdominal pain, anorexia, and weight loss. The clinical exam pointed out diffuse alopecia, a palpable mass in the epigastric area, and abdominal tenderness at palpation, the patient weighing 32 kg. The laboratory tests showed anemia. The abdominal ultrasound showed a gastric intraluminal mass with a superior hyperechoic arc. The upper digestive endoscopy revealed a mass formed by hair, mucus, and food occupying the gastric cavity with the extension into the duodenum confirming the diagnosis of Rapunzel syndrome. The giant trichobezoar of 511 g, measuring 17 × 7 × 6.5 cm with a tail of approximately 3 cm, was successfully removed through laparotomy. Although rare, Rapunzel syndrome must never be forgotten as a differential diagnosis for digestive symptoms since its early detection hinders the occurrence of further complications.

摘要

长发公主综合征是一种极为罕见的病症,见于患有精神疾病的青少年或年轻女性,其特征为胃内毛石并向小肠延伸。诊断延迟很常见,因为在早期阶段通常没有症状。我们报告一例13岁女孩因腹痛、厌食和体重减轻入住我们诊所的病例。临床检查发现弥漫性脱发、上腹部可触及肿块以及触诊时有腹部压痛,患者体重32公斤。实验室检查显示贫血。腹部超声显示胃腔内有一个肿块,上方有高回声弧。上消化道内镜检查发现一个由毛发、黏液和食物组成的肿块占据胃腔并延伸至十二指肠,确诊为长发公主综合征。通过剖腹手术成功切除了重达511克、尺寸为17×7×6.5厘米且尾部约3厘米的巨大毛石。尽管罕见,但长发公主综合征作为消化症状的鉴别诊断绝不能被遗忘,因为早期发现可防止进一步并发症的发生。

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J Clin Med. 2024 Dec 8;13(23):7464. doi: 10.3390/jcm13237464.
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Endoscopic Removal of a Recurrent Trichobezoar in an Adolescent: A Case Report.青少年复发性毛粪石的内镜下取出:一例报告
JPGN Rep. 2023 Oct 30;4(4):e371. doi: 10.1097/PG9.0000000000000371. eCollection 2023 Nov.
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When the Gut Tells a Story: Bezoars in a Neglected Autistic Child.当肠道讲述一个故事:一名被忽视的自闭症儿童体内的胃石

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Pica: A Common Condition that is Commonly Missed - An Update Review.异食癖:一种常被漏诊的常见病症——最新综述
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Rapunzel syndrome: how to orient the diagnosis.长发公主综合征:如何进行诊断定位
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