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关于包裹性腹膜硬化的病例系列及文献简要综述:揭开“茧”的面纱

A Case Series and Brief Review of Literature on Encapsulating Peritoneal Sclerosis: Unveiling the Cocoon.

作者信息

Bright Bribin, Salam Roshna, Moorthy Srikanth

机构信息

Radiodiagnosis, Amrita Institute of Medical Sciences, Kochi, IND.

出版信息

Cureus. 2024 Nov 16;16(11):e73802. doi: 10.7759/cureus.73802. eCollection 2024 Nov.

DOI:10.7759/cureus.73802
PMID:39687804
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11648041/
Abstract

This case series explores four distinct instances of encapsulating peritoneal sclerosis (EPS), a rare but serious condition characterized by the encapsulation of abdominal viscera, commonly referred to as abdominal cocoon. EPS is associated with severe complications, including bowel obstruction and sepsis, which can significantly impact patient outcomes. The first case involves a 41-year-old male patient who had undergone a liver transplant and ultimately succumbed to extensively drug-resistant (XDR) sepsis. The second case features a 31-year-old male patient diagnosed with abdominal tuberculosis, who successfully recovered following comprehensive antitubercular therapy (ATT). The third case presents a 26-day-old neonate with CHARGE syndrome (coloboma of the eye, heart defects, atresia of the choanae, retardation of growth and development, genital abnormalities, and ear anomalies, including deafness), who tragically succumbed to septic shock. The last case is of a 41-year-old male patient with disseminated tuberculosis who showed marked improvement with appropriate treatment. These cases illustrate the diverse clinical backgrounds and grave outcomes associated with EPS, highlighting the urgent need for early diagnosis and intervention. Despite EPS being a leading cause of small bowel obstruction in many inpatient settings, its diagnosis is frequently overlooked due to insufficient awareness among healthcare professionals. This series aims to enhance understanding of the causes, imaging characteristics, and management strategies for EPS. By disseminating this knowledge, we hope to facilitate earlier identification of the condition, particularly through primary imaging techniques such as ultrasound (USG). Ultimately, increasing awareness and understanding of EPS is crucial to improving patient outcomes and reducing the associated morbidity and mortality.

摘要

本病例系列探讨了4例包裹性腹膜硬化症(EPS)的不同病例,这是一种罕见但严重的疾病,其特征是腹腔脏器被包裹,通常称为腹腔茧状包裹症。EPS与严重并发症相关,包括肠梗阻和败血症,这会对患者的预后产生重大影响。第一例涉及一名41岁男性患者,他接受了肝移植,最终死于广泛耐药(XDR)败血症。第二例是一名31岁男性患者,被诊断为腹部结核,经过全面抗结核治疗(ATT)后成功康复。第三例是一名26日龄患有CHARGE综合征(眼裂、心脏缺陷、后鼻孔闭锁、生长发育迟缓、生殖器异常和耳部异常,包括耳聋)的新生儿,不幸死于感染性休克。最后一例是一名41岁患有播散性结核的男性患者,经过适当治疗后病情明显改善。这些病例说明了与EPS相关的不同临床背景和严重后果,凸显了早期诊断和干预的迫切需要。尽管EPS在许多住院患者中是小肠梗阻的主要原因,但由于医护人员认识不足,其诊断常常被忽视。本系列旨在加深对EPS的病因、影像学特征和管理策略的理解。通过传播这些知识,我们希望促进对该疾病的早期识别,特别是通过超声(USG)等主要影像学技术。最终,提高对EPS的认识和理解对于改善患者预后以及降低相关的发病率和死亡率至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/328399592d29/cureus-0016-00000073802-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/a5c07308b91e/cureus-0016-00000073802-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/708558af47e4/cureus-0016-00000073802-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/b622f501c00c/cureus-0016-00000073802-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/347a00d01328/cureus-0016-00000073802-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/61d09eb42898/cureus-0016-00000073802-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/328399592d29/cureus-0016-00000073802-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/a5c07308b91e/cureus-0016-00000073802-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/708558af47e4/cureus-0016-00000073802-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/b622f501c00c/cureus-0016-00000073802-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/347a00d01328/cureus-0016-00000073802-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/61d09eb42898/cureus-0016-00000073802-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1b7d/11648041/328399592d29/cureus-0016-00000073802-i06.jpg

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Intern Med. 2023 Jun 1;62(11):1683-1689. doi: 10.2169/internalmedicine.9793-22. Epub 2022 Oct 26.
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Encapsulating Peritoneal Sclerosis: The Abdominal Cocoon.
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Sclerosing encapsulating peritonitis after living-donor liver transplantation: A case series, Kyoto experience.活体肝移植术后硬化性包裹性腹膜炎:病例系列,京都经验
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