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本文报道日本1978～1982年间135个单位1628例的全国性调查结果.发病率估计为每年出生儿的1/4697,男女之比为3.0∶1,女性长段型较多见.少见于未成熟儿,出生体重<2500克者仅5.5%.家族发生率较低为3.0%,但长段型,特别是全结肠型为11.3%.伴发畸形为11.1%,最常见是Downs综合征和先天性心脏病.病变段在乙状结肠以下79.4%,全结肠8.5%.诊断方法是钡剂灌肠95.8%,直肠肛管测压法64.7%,组织化学染色法28.7%.最常用的是钡剂灌肠结合直肠测压法.确诊的年龄很重要,因为延迟诊断使小肠结肠炎发病率升高.美国儿科学会外科组调查结果表明,三个月内确诊率为40%,新生儿确诊率为8%,而本调查新生儿确诊率为48.7%,三个
This article reports on the nationwide survey of 1,628 cases of 135 units in Japan between 1978 and 1982. The incidence is estimated at 1/4697 of births per year, with a male-female ratio of 3.0: 1, and female long segments are more common. The incidence of familial morbidity was 3.0%, but the long segment, especially the colon, was 11.3% with associated malformations of 11.1%, most commonly Downs syndrome and congenital Heart disease.The lesion in the sigmoid colon below 79.4%, 8.5% of the whole colon.The diagnostic method is 95.8% barium enema, rectal anal canal manometry 64.7%, histochemical staining 28.7% .The most commonly used barium enema combination Rectal manometry method. The diagnosis of the age is very important, because delayed diagnosis of the incidence of enterocolitis increased the American Academy of Pediatrics Surgical Group survey showed that within three months the diagnosis rate was 40%, neonatal diagnosis rate was 8% The survey confirmed 48.7% of newborns, three