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肠道神经元发育异常

Intestinal neuronal dysplasia.

作者信息

Puri P, Wester T

机构信息

Children's Research Centre, Our Lady's Hospital for Sick Children, Dublin, Ireland.

出版信息

Semin Pediatr Surg. 1998 Aug;7(3):181-6. doi: 10.1016/s1055-8586(98)70015-0.

Abstract

Intestinal neuronal dysplasia (IND) is a disorder of the enteric nervous system that was first described by Meier-Ruge in 1971. IND may be associated with Hirschsprung's disease or may occur as an isolated disorder. The incidence of isolated IND varies from 0.3% to 40% of suction rectal biopsies. The uncertainty regarding the incidence of IND has resulted from considerable confusion concerning essential diagnostic criteria. The diagnostic difficulties reflect the wide variability in the literature in terms of diagnostic criteria, biopsy procedures, staining techniques, and patient age. The recent application of histochemical and immunohistochemical techniques indicates that IND is a distinct histopathologic entity. The majority of patients with IND can be treated conservatively with laxatives and enemas. If bowel symptoms persist after at least 6 months of conservative treatment, internal sphincter myectomy should be considered.

摘要

肠道神经元发育异常(IND)是一种肠道神经系统疾病,由迈尔 - 鲁格于1971年首次描述。IND可能与先天性巨结肠相关,也可能作为一种孤立性疾病出现。孤立性IND在吸引直肠活检中的发生率从0.3%到40%不等。IND发生率的不确定性源于基本诊断标准方面的大量混淆。诊断困难反映出文献中在诊断标准、活检程序、染色技术和患者年龄方面存在广泛差异。组织化学和免疫组织化学技术的最新应用表明,IND是一种独特的组织病理学实体。大多数IND患者可以通过使用泻药和灌肠进行保守治疗。如果在至少6个月的保守治疗后肠道症状仍然持续,应考虑进行内括约肌肌切除术。

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