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长发公主综合征:低蛋白血症的罕见病因及文献综述

Rapunzel syndrome: a rare cause of hypoproteinaemia and review of literature.

作者信息

Ullah Waqas, Saleem Kaiser, Ahmad Ejaz, Anwer Faiz

机构信息

University of Arizona, Tucson, Arizona, USA.

Griffin Hospital, Derby, Connecticut, USA.

出版信息

BMJ Case Rep. 2016 Sep 26;2016:bcr2016216600. doi: 10.1136/bcr-2016-216600.

Abstract

Rapunzel syndrome is an extremely rare condition associated with trichophagia (hair eating disorder) secondary to a psychiatric illness called trichotillomania (hair-pulling behaviour). It is most commonly seen in children and adolescents. Untreated cases can lead to a number of complications. We present a case of a middle-aged woman with sudden intractable vomiting and constipation associated with bilateral pedal oedema and significant weight loss. Laboratory investigations revealed low serum protein levels. Laparotomy was performed, and a hairball was removed from her stomach and ileum. The patient was managed with the help of a psychiatrist and was given nutritional support. We performed a comprehensive search and summarised data for a total of 88 cases. No time or language limit was placed. The purpose of this discussion is to highlight the clinical spectrum of Rapunzel syndrome and also to report its rare association with hypoproteinaemia.

摘要

长发公主综合征是一种极其罕见的病症,与因一种名为拔毛癖(拔毛发行为)的精神疾病继发的食毛症(吃毛发障碍)相关。它最常见于儿童和青少年。未经治疗的病例会导致多种并发症。我们报告一例中年女性病例,该患者突然出现顽固性呕吐和便秘,并伴有双侧足部水肿和显著体重减轻。实验室检查显示血清蛋白水平较低。进行了剖腹手术,从她的胃和回肠中取出了一个毛球。患者在精神科医生的帮助下接受治疗,并给予营养支持。我们进行了全面检索并总结了总共88例病例的数据。未设置时间或语言限制。本次讨论的目的是突出长发公主综合征的临床谱,同时报告其与低蛋白血症的罕见关联。

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Rev Gastroenterol Mex. 2016 Jul-Sep;81(3):178-9. doi: 10.1016/j.rgmx.2015.08.005. Epub 2016 Mar 2.
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Tale of a hairy tail: Rapunzel Syndrome.多毛尾巴的故事:长发公主综合征。
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Rapunzel Syndrome in a Postpartum Patient after Caesarian Delivery.剖宫产术后一名产后患者的长发公主综合征。
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