Reevaluation of acetylcholinesterase staining for the diagnosis of hirschsprung disease and allied disorders.
Reevaluation of acetylcholinesterase staining for the diagnosis of hirschsprung disease and allied disorders.
复制标题
重新评估乙酰胆碱酯酶染色对先天性巨结肠及相关疾病诊断的作用。
DOI：
10.1097/mpg.0000000000000664
复制
发表时间：
2015
期刊：
影响因子：
--
通讯作者：
Taguchi T
中科院分区：
文献类型：
--
作者：
Budianto IR;Obata S;Kinoshita Y;Yoshimaru K;Yanagi Y;Miyata J;Nagata K;Ieiri S;Taguchi T
目的：乙酰胆碱酯酶（AChE）染色已成为明确诊断巨结肠病（HD）的金标准，尽管有一些缺陷被报道。为了验证乙酰胆碱酯酶染色检测HD的准确性，我们重新评估了我们研究所的一个大系列。方法：对13年来所有疑似HD患儿直肠黏膜标本进行回顾性研究。采用改良Karnovsky-Roots AChE染色法对标本进行染色。组织病理学诊断后的最终诊断、预后和处理通过发给患者原医院的问卷进行分析。结果：共采集标本358份。122例（34%）被诊断为HD， 198例（55%）被诊断为非HD， 25例（7%）被诊断为“不确定”，13例（4%）被诊断为“不合适”。非hd组190例（96%）外观正常，8例（4%）疑似肠神经元发育不良（IND）。358份问卷中有306份被退回。最终诊断结果显示，首次诊断为HD的标本没有被鉴定为非HD，反之亦然，敏感性和特异性均为100%。非hd组最终诊断为慢性特发性肠假性梗阻（CIIP） 4例。所有HD患者都接受了根治性手术。大多数非hd患者接受了保守治疗，尽管一些患者仍然存在便秘。结论：乙酰胆碱酯酶染色是鉴别HD与非HD的准确工具，具有较高的敏感性和特异性。非hd病例可包括CIIP；因此，仔细的跟踪是必须的。
Objectives:Acetylcholinesterase (AChE) staining has become the gold standard for definitively diagnosing Hirschsprung disease (HD), although some pitfalls have been reported. We reevaluated a large series at our institute in order to validate the accuracy of AChE staining for detecting HD.Methods:A retrospective study of the rectal mucosal specimens of all of the children with suspected HD during a 13-year period was performed. The specimens were stained according to the modified Karnovsky-Roots method for AChE staining. The final diagnosis, prognosis, and management after the histopathological diagnosis were analyzed with a questionnaire sent to the patient's original hospital.Results:Three hundred and fifty-eight specimens were collected. One hundred twenty-two (34%) specimens were diagnosed as HD, 198 (55%) as nonHD, 25 (7%) as “undetermined,” and 13 (4%) as “inappropriate.” The non-HD group contained 190 (96%) specimens with a normal appearance and 8 (4%) specimens with suspected intestinal neuronal dysplasia (IND). Three hundred and six of 358 questionnaires were returned. The final diagnosis showed that no specimens first diagnosed as HD were identified as non-HD and vice versa, for a sensitivity and specificity of 100%. Four cases were finally diagnosed as chronic idiopathic intestinal pseudo-obstruction (CIIP) in the non-HD group. All of the patients with HD underwent radical surgery. Most non-HD patients were managed conservatively, although some continued to have constipation.Conclusions:AChE staining is an accurate tool for differentiating between HD and non-HD with high sensitivity and specificity. CIIP can be included in cases of non-HD; therefore, careful follow-up is mandatory.