54例先天性巨结肠同源病临床分析  

The clinical analysis of 54 cases with allied Hirschsprung′s disease

作　　者：刘洪江[1] 朱进[2] 向丽[1] 李洪[1] 童科融 王海涛[1] 金先庆[1] 

机构地区：[1]重庆医科大学附属儿童医院胃肠新生儿外科,重庆市400014 [2]重庆医科大学附属儿童医院病理科,重庆市400014

出　　处：《临床小儿外科杂志》2015年第2期88-91,共4页Journal of Clinical Pediatric Surgery

基　　金：国家自然科学基金资助项目（项目号81370474）,国家临床重点专科建设资助项目（国卫办医函[2013]544）

摘　　要：目的：探讨先天性巨结肠同源病（HAD）的诊断、治疗及预后。方法分析2008年8月至2014年4月于本院确诊的426例先天性巨结肠（54例 HAD）患儿临床资料，所有患儿均由 BE、ARE、直肠黏膜吸引活检术等检查确诊，通过分析辅助检查结果，了解相关检查在 HAD 的特点。并对患儿近期效果进行随访。结果54例 HAD 病例约占本院先天性巨结肠症的11．3％，其中肠神经元性发育异常症25例（46．3％）；神经节减少症16例（29．6％）；神经节未成熟症13例（24．1％）。49例 HAD 患儿行钡剂灌肠检查，结果提示结肠、直肠扩张为主要表现，约28．3％的患儿结果类似先天性巨结肠症；40例 HAD 患儿行肛直肠测压，引出典型直肠肛门抑制反射 4例（10．0％），引出变异波形18例（45．0％），主要表现为有“M”、“U”、“W”波及波形延迟等异型波。钙视网膜蛋白（Calretinin CR）免疫组化染色检查阳性率在 HAD 为88．9％，较正常对照组显色浅，呈网状结构。19例予巨结肠根治术治疗，随访大部分患儿预后良好。结论先天性巨结肠同源病病理学特点介于先天性巨结肠症与正常之间，通过钡剂灌肠、肛直肠测压、CR 染色等相关检查综合分析可提高诊断准确率，当该病影响到患儿的生长发育及出现严重并发症时应及时手术治疗。Objective To determine the diagnostic criteria and treatment and prognosis of allied disea-ses of Hirschsprung＇s disease（HAD）in children.Methods During the period from Aug.2008 to Apr.2014, 426 consecutive patients were confirm the diagnosis of Hirschsprung＇s disease,54 cases were HAD.Diagnosis based on the clinical symptoms,anorectal manometry（ARM）,rectal suction biopsy,barium enema（BE）,Cal-retinin Immun-ohistochemistry.The characteristics were made through the analysis of auxiliary examination re-sults.All pathiets were received short-term follow-up.Results Among 54 patiets with HAD,there was 25 In-testinal neuronal dysplasia B （46.3%）,16 Hypoganglionosis （29.6%）and 13 Immature ganglia（24.1%）. All the patiets received BE examination,and the major phenomenon was expansion of the colon.18 cases showed a unnormal reflex wave with a shape of “W”,“U”or“M”（45.0% ）among the 40 patiets received ARMexamination.80.0% of HAD patients were positive in CR Immunohistochemistry,the phenomenon was net structure,and staining was thinner than normal group.19 patients received operation treatment,and the follow-up of most of them was good. Conclusions The pathological features of HAD is between HD and normal. Comprehensive analysis of BE,ARMand CR examination can improve the diagnostic accuracy of HAD.The op-eration should be consider once a serious complication occur.

关 键 词：HIRSCHSPRUNG 病 肠神经系统/免疫学 

分 类 号：R726.2[医药卫生—儿科]