先天性巨结肠症和先天性巨结肠相关性小肠结肠炎患者的克罗恩病。文献综述

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近十年来，儿童炎症性肠病的发病率持续上升。根据数据显示，在对先天性巨结肠症和先天性巨结肠相关性小肠结肠炎进行手术治疗后，出现了克罗恩病。人们对早期肠道切除后儿童出现克罗恩病样变化的可能原因提出了建议。本研究的目的是总结信息，并分析有关先天性巨结肠儿童克罗恩病发展和克罗恩样改变的文献。在科学数据库 eLibrary、PubMed、SinceDirect、GooglScolar 中以 “克罗恩病”、“先天性巨结肠病”、“先天性巨结肠相关小肠结肠炎”、“先天性巨结肠相关炎症性肠病”、“克罗恩病”, “先天性巨结肠症”为关键词进行了检索。检索时间跨度为 2000 年至 2023 年。共发现 14 篇关于先天性巨结肠相关肠道疾病的文章。这种病理现象在国内文献中尚未见描述。在炎症性肠病和先天性巨结肠相关性肠炎中，可以发现共同的发病因素，如微生物群紊乱和慢性炎症。在对菌群失调和小肠结肠炎发作做出病理免疫反应的先决条件中，可以确定：肠道神经系统和神经免疫调节机制功能失调、免疫状态普遍失调、肠道粘膜屏障功能下降或“不成熟”。 患有先天性巨结肠病并反复发作肠结肠炎的患者有可能并没有发展成 “典型形式”的克罗恩病，而是出现了类似克罗恩病的炎症变化。在对先天性巨结肠病进行手术治疗后，持续存在的功能性或器质性梗阻会加剧易感因素的负面影响，导致肠粘膜慢性炎症。如果先天性巨结肠相关性肠炎反复发作或对标准疗法反应不佳，建议进行深入检查以排除克罗恩病或吻合口区域的慢性炎症病变，从而选择治疗策略。

作者简介

Victoria A. Glushkova

Saint Petersburg State Pediatric Medical University

编辑信件的主要联系方式.
Email: pedsurgspb@yandex.ru
ORCID iD: 0009-0002-4768-1539
SPIN 代码: 8703-3966
俄罗斯联邦, Saint Petersburg

Aleksey V. Podkamenev

Saint Petersburg State Pediatric Medical University

Email: av.podkamenev@hotmail.com
ORCID iD: 0000-0003-0885-0563
SPIN 代码: 7052-0205

MD, Dr. Sci. (Medicine), Professor

俄罗斯联邦, Saint Petersburg

Tatyana V. Gabrusskaya

Saint Petersburg State Pediatric Medical University

Email: tatyanagabrusskaya@yandex.ru
ORCID iD: 0000-0002-7931-2263
SPIN 代码: 2853-5956

MD, Cand. Sci. (Medicine)

俄罗斯联邦, Saint Petersburg

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