Evidence of Altered Enteric Nervous System Structure Associated With Severe Dysmotility in a Patient With Ehlers-Danlos Syndrome.

IF 0.5 Q4 GASTROENTEROLOGY & HEPATOLOGY

ACG Case Reports Journal Pub Date : 2025-09-24 eCollection Date: 2025-09-01 DOI:10.14309/crj.0000000000001823

Karina Oelerich, Kanwal Bains, Scott B Minchenberg, Vikram Deshpande, Johanna Iturrino-Moreda, Subhash Kulkarni

引用次数: 0

Abstract

Patients with Ehlers-Danlos syndrome (EDS) suffer from gastroparesis, constipation, and gut dysmotility. We present a case of histopathological assessment of the enteric nervous system from an EDS patient with severe gut dysmotility. Full-thickness small bowel biopsies with immunostains for c-kit and S-100 within the myenteric plexus revealed no significant abnormality. Using the method by Boschetti et al of Neuronal Specific Enolase-based immunostaining, we found that interganglionic distance in the myenteric plexus (∼800 um) exceeded the normal range (380-570 µm). This clinical experience provides rationale for pursuing histopathology in patients with EDS.

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ehers - danlos综合征患者肠神经系统结构改变与严重运动障碍相关的证据。

ehers - danlos综合征（EDS）患者患有胃轻瘫、便秘和肠道运动障碍。我们提出一个病例的组织病理学评估肠神经系统从一个EDS患者严重的肠道运动障碍。全层小肠活检与免疫染色c-kit和S-100肌丛未见明显异常。使用Boschetti等人基于神经元特异性烯醇化酶的免疫染色方法，我们发现肌肠丛的节间距离（~ 800 um）超过了正常范围（380-570µm）。这一临床经验为EDS患者进行组织病理学检查提供了依据。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

ACG Case Reports Journal GASTROENTEROLOGY & HEPATOLOGY-

自引率

14.30%

发文量

170

审稿时长

12 weeks

期刊介绍： ACG Case Reports Journal is a peer-reviewed, open-access publication that provides GI and hepatology fellows, private practice clinicians, and other healthcare providers an opportunity to share interesting case reports with their peers and with leaders in the field. ACG Case Reports Journal publishes case reports, images, videos and letters to the editor in all topics of gastroenterology and hepatology, including: Biliary Colon Endoscopy Esophagus Functional Bowel Disorders Inflammatory Bowel Disease Liver Nutrition and Obesity Pancreas Pathology Pediatric Small Bowel Stomach.