在Hirschsprung病中,靠近的性肠是纤维化和硬的
Prisca C Obidike1, Britney A Hsu2, Chioma Moneme1
1Division of Pediatric Surgery, Department of Surgery, University of Virginia, Charlottesville, United States of America.
JCI insight
|February 10, 2026
概括
赫施普朗格病 (HSCR) 不仅仅涉及结肠. 这项研究发现,在HSCR患者和小鼠模型中,近道质肠中纤维化和性增加,导致持续的肠道功能障碍.
科学领域:
- 胃肠病学 胃肠病学
- 发展生物学 发展生物学
- 病理学 病理学 病理学
背景情况:
- 赫施普朗格病 (HSCR) 是一种先天性肠道疾病,其定义是肠道远端缺少神经.
- 在HSCR患者中,手术后的肠功能障碍仍然存在,这表明除了切除的角质节之外还有异常.
- 在HSCR病理生理学中,近端质肠的作用仍然不完全理解.
研究的目的:
- 为了调查赫施普朗格病 (HSCR) 患者近端状肠中的潜在异常.
- 为了确定HSCR患者手术后持续性肠功能障碍的原因.
- 为了比较HSCR模型和人类患者与对照中的近端状肠.
主要方法:
- 在HSCR中使用Ednrb-null鼠标模型.
- 分析了HSCR患者的组织样本和年龄/位置匹配的对照.
- 评估了肠道硬,纤维化和细胞外基因表达 (ECM) 基因表达.
主要成果:
- 与野生型 littermates 相比,HSCR小鼠的近端结肠表现出增加的刚性和纤维化.
- 人类HSCR患者在临近状肠中表现出明显更大的下纤维化,而不是对照.
- 在HSCR小鼠的近端质肠中观察到ECM相关基因的失调表达.
结论:
- 靠近性结节肠中的纤维化和度增加可能导致HSCR中的持续性肠功能障碍.
- 希尔施普朗格病的病理生理延伸到近端质肠,不仅仅是质肠段.
- 研究结果表明,HSCR是一种更复杂的疾病,涉及广泛的肠道异常.
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