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一名马凡综合征患者在自发性复发性气胸后出现乙状结肠自发性破裂。

Spontaneous rupture of the colon sigmoid following spontaneous recurrent pneumothorax in a patient with Marfan syndrome.

作者信息

Kouerinis Ilias A, Zografos George C, Apostolakis Eustratios I, Menenakos Evangelos I, Anthopoulos Prodromos, Fotiadis Constantine I, Gorgoulis Vassilios G, Argiriou Michalis E, Bellenis Ion P

机构信息

Department of Cardiothoracic Surgery, Evangelismos Hospital, Athens, Greece.

出版信息

Med Sci Monit. 2006 Mar;12(3):CS21-3. Epub 2006 Feb 23.

Abstract

BACKGROUND

Spontaneous ruptures of the colon and rectum are very rare clinical entities and always require laparotomy. In such cases, despite meticulous exploration an underlying pathology cannot be revealed and most reports in the world literature relate spontaneous colon ruptures with unusual connective tissue disorders such as Marfan or Ehlers-Danlos syndrome. Connective tissue diseases affect the structure and the synthesis of elastin and type III collagen, resulting in various systemic disorders. Among the several pulmonary manifestations, spontaneous pneumothorax is observed in about 5% of patients expressing one of the above syndromes.

CASE REPORT

In the following report we present a unique case of spontaneous rupture of the sigmoid colon secondary to spontaneous recurrent contralateral pneumothorax.

CONCLUSIONS

The aim of this presentation is to raise the level of suspicion in every surgeon and gastroenterologist to include connective tissue disorders in their differential diagnosis in any case of spontaneous colon rupture and, if found, to follow further diagnostic procedures in anticipation of any other visceral complications.

摘要

背景

结肠和直肠的自发性破裂是非常罕见的临床病症,通常需要进行剖腹手术。在这类病例中,尽管进行了细致的探查,潜在病因仍无法查明,世界文献中的大多数报道将自发性结肠破裂与诸如马凡氏综合征或埃勒斯-当洛综合征等罕见的结缔组织疾病相关联。结缔组织疾病会影响弹性蛋白和III型胶原蛋白的结构与合成,从而导致各种全身性疾病。在多种肺部表现中,约5%患有上述综合征之一的患者会出现自发性气胸。

病例报告

在以下报告中,我们呈现了一例独特的病例,即乙状结肠自发性破裂继发于对侧自发性复发性气胸。

结论

本报告的目的是提高每位外科医生和胃肠病学家的怀疑意识,在任何自发性结肠破裂病例的鉴别诊断中纳入结缔组织疾病,若发现此类疾病,则应进一步进行诊断程序,以预防任何其他内脏并发症。

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