作者收集了1978～1982年日本全国135个医疗机构的先天性巨结肠(Hirschsprung’s disease)1628例,对其诊断和治疗进行分析。总发生率:先天性巨结肠(下称 HD)占每年新生儿的1/4697。男:女为1220:408(3.0:1)。而小肠神经节细胞缺如的病例,男:女为0.8:1。出生时平均体重3218.3g,低于2500 g 者仅占5.5%;高于3500 g者占27.5%。合并 Down 氏综合征者占2.9%,心脏有畸形者占2.5%,泌尿道有变异如巨大输尿管者只有2例(0.1%)。根据肠管神经节细胞缺如的范围可 The author collected 1628 cases of Hirschsprung’s disease from 135 medical institutions in Japan from 1978 to 1982 and analyzed its diagnosis and treatment. The total incidence of: Hirschsprung ’s disease (hereinafter referred to as HD) accounted for 1/4697 newborns each year. Male: Female 1220: 408 (3.0: 1). The absence of small bowel ganglion cells, male: female 0.8: 1. Average birth weight 3218.3g, less than 2500g accounted for only 5.5%; higher than 3500g accounted for 27.5%. 2.9% had Down’s Syndrome, 2.5% had malformation of the heart, and only 2 patients (0.1%) had major urinary ureters with variability. Depending on the extent of gut ganglion cells may be absent