肠神经元性异常疾病的临床与病理  

Clinical and Pathologic Characteristics of Neuronal Intestinal Disorder

作　　者：施诚仁[1] 陈其民[1] 潘伟华[1] 李明磊[1] 杜勇 张忠德[2] 

机构地区：[1]上海第二医科大学附属新华医院小儿外科,200092 [2]上海第二医科大学附属新华医院病理科,200092

出　　处：《外科理论与实践》2000年第3期181-184,共4页Journal of Surgery Concepts & Practice

摘　　要：本文旨在从临床与病理学特点上对肠神经元异常疾病加以区分及认识。方法：回顾分析51例肠神经元异常性疾病的临床与病理，并对肛门直肠测压检查在诊断中的作用进行讨论。结果：所有患儿的临床表现均以便秘为主；其肛管压力明显高于正常对照组；根据病理学差异，肠神经元异常性疾病可分为4型：①肠神经元性发育异常；②神经节细胞减少症；③神经节细胞未成熟症；④神经元性发育不良。结论：肠神经元异常性疾病的诊断难以在临床上加以区别，肛门直肠测压的诊断价值亦较低，必需辅以病理检查方可明确。Neuronal intestinal disorder is clinically similar to Hirschsprung's disease, it is thus called the HD-allied disease. This study was designed to prove that pathologic examination was indispensible in posing the precise diagnosis of the disease entity. Methods: Fifty-one patients with neuronal intestinal disorders were admitted to our division during the past 20 years, analysis regarding their clinical and pathologic characteristics were carried out retrospectively. The use of anorectal manometry in diagnosing this disorder was also discussed. Results: All the 51 patients had a history of constipation. The pressure in their anal canal was significantly higher than that of the control group. Basing on the pathologic characteristics, the neuronal intestinal disorder could be divided into 4 types: ①neuronal intestinal dysplasia, NID; ②hypoganglionosis; ③immaturity of ganglion cells; and ④neuronal hypogenesis. Conclusions: Diagnosing neuronal intestinal disorder on clinical and manometric basis is inadequate, pathologic examination is indispensable.

关 键 词：肠神经元性异常疾病 病理学 肛门直肠测压 

分 类 号：R656.9[医药卫生—外科学]