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先天性巨结肠相关疾病的临床病理特征及现状更新

Clinicopathologic features of allied disorders of Hirschsprung disease and status update.

作者信息

Lu Miao

机构信息

Department of Pathology, Pingshan District Central Hospital of Shenzhen, Shenzhen 518100, Guangdong Province, China.

出版信息

World J Gastrointest Surg. 2025 Aug 27;17(8):104954. doi: 10.4240/wjgs.v17.i8.104954.

Abstract

Allied disorders of Hirschsprung diseases (ADHDs) have abnormal enteric ganglia and symptoms that are similar to congenital megacolon. The classification of ADHDs includes neuronal dysplasia, enteric gangliocytomatosis, isolated ganglion dysfunction, immature ganglion, and internal anal sphincter achalasia. The diagnosis, treatment and prognosis of ADHDs are distinct from congenital megacolon. This review summarizes the classification, epidemiology, pathogenesis, gene basis, pathology, diagnosis, treatment, and prognosis of ADHDs based on existing diagnostic criteria.

摘要

先天性巨结肠相关疾病(ADHDs)具有异常的肠神经节且症状与先天性巨结肠相似。ADHDs的分类包括神经元发育异常、肠神经节细胞瘤病、孤立性神经节功能障碍、未成熟神经节以及内括约肌失弛缓症。ADHDs的诊断、治疗和预后与先天性巨结肠不同。本综述基于现有诊断标准总结了ADHDs的分类、流行病学、发病机制、基因基础、病理学、诊断、治疗及预后。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fd89/12427069/e684faac543a/wjgs-17-8-104954-g001.jpg

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