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结肠穿孔与先天性脂肪营养不良并存。

Coexistence of Colon Perforation and Congenital Lipodystrophy.

机构信息

Department of General Surgery, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey.

Department of Pathology, Izmir Katip Celebi University Ataturk Training and Research Hospital, Izmir, Turkey.

出版信息

J Coll Physicians Surg Pak. 2022 Sep;32(9):1222-1224. doi: 10.29271/jcpsp.2022.09.1222.

DOI:10.29271/jcpsp.2022.09.1222
PMID:36089727
Abstract

Lipodystrophy (LD) is an acquired or congenital rare condition consisting of hyperlipidaemia, glucose intolerance/ insulin resistance, and almost complete absence and storage of adipose tissue. Colon perforations can be observed in type 4 congenital LD. Here, we aimed to present a case of sigmoid colon perforation which developed in a young woman with the diagnosis of LD. Extensive purulent peritonitis, significant wall thickening, and oedema in the sigmoid colon were detected during surgical exploration. Anterior resection with end colostomy procedure was then performed. Although bowel perforation has been theoretically reported to occur in LD, the presented case is the first adult patient in the literature. These individuals tend to develop colon perforation as a result of histological changes in their gastrointestinal tract. This situation should always be taken into consideration in order to avoid delay in diagnosis, especially in patients who present with abdominal pain and have a history of LD. Key Words: Intestinal perforation, Congenital lipodystrophy, Peritonitis, Sigmoid colon.

摘要

脂肪营养不良(LD)是一种获得性或先天性罕见疾病,表现为高脂血症、葡萄糖耐量/胰岛素抵抗,以及几乎完全缺乏和储存脂肪组织。4 型先天性 LD 可观察到结肠穿孔。在此,我们旨在报告一例在 LD 诊断的年轻女性中发生的乙状结肠穿孔。术中探查发现广泛脓性腹膜炎、乙状结肠明显壁增厚和水肿。然后进行了乙状结肠前切除术加末端结肠造口术。虽然理论上有报道称 LD 会发生肠穿孔,但本例是文献中首例成人患者。这些患者由于胃肠道的组织学变化,易发生结肠穿孔。对于出现腹痛且有 LD 病史的患者,为避免延误诊断,应始终考虑到这种情况。

关键词

肠穿孔、先天性脂肪营养不良、腹膜炎、乙状结肠。

相似文献

1
Coexistence of Colon Perforation and Congenital Lipodystrophy.结肠穿孔与先天性脂肪营养不良并存。
J Coll Physicians Surg Pak. 2022 Sep;32(9):1222-1224. doi: 10.29271/jcpsp.2022.09.1222.
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Surgical treatment of perforations of the sigmoid colon.乙状结肠穿孔的外科治疗
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Perforation of the sigmoid colon by swallowed chicken bone: case reports and review of literature.吞食鸡骨导致乙状结肠穿孔:病例报告及文献综述
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引用本文的文献

1
Assessment of aortomesenteric distance and mesenteric and retroperitoneal adipose tissue thickness in genetic forms of lipodystrophy.脂肪营养不良遗传形式中主动脉肠系膜距离及肠系膜和腹膜后脂肪组织厚度的评估
J Endocrinol Invest. 2025 Feb;48(2):445-454. doi: 10.1007/s40618-024-02429-9. Epub 2024 Oct 1.
2
Metabolic and other morbid complications in congenital generalized lipodystrophy type 4.先天性全身性脂肪营养不良 4 型的代谢及其他病态并发症。
Am J Med Genet A. 2024 Jun;194(6):e63533. doi: 10.1002/ajmg.a.63533. Epub 2024 Jan 17.