Harris J, Källén B, Robert E
California Birth Defects Monitoring Program, Emeryville 94608, USA.
Teratology. 1995 Jul;52(1):15-29. doi: 10.1002/tera.1420520104.
A study has been made on certain epidemiological characteristics of infants with alimentary tract atresia: esophageal atresia, small and large gut atresia, and anal atresia. Data were collected from three malformation registries and represent a material of more than 4.5 million births. A total of 3,550 infants with alimentary atresia were identified corresponding to a total rate of about 8 per 10,000 births. In 167 infants (4.7%) more than one of the major atresia types were present simultaneously. Racial differences were found (based on data from California) for esophageal atresia where whites had a higher rate than other races. For gastrointestinal atresia, a high rate in blacks was found, while no differences between races were seen for anal atresia. Also, differences in registered rates between the three programs were found, at least partly explainable by different ascertainment. The different forms of atresia were compared from the point of view of sex ratio, twinning rate, maternal age and parity distribution, presence of chromosome anomalies, and types of associated malformations. The pathogenesis and etiology of the various types of atresia are discussed based on these observations. The conclusion is that although undoubtedly other pathogenetic mechanisms may exist for gastrointestinal atresia, a substantial proportion of all infants with alimentary atresia had their malformations as a result of early disturbances of intestinal morphogenesis. Within each subgroup, apparently different etiologies may exist, resulting in differences in epidemiological characteristics.
对患有消化道闭锁(食管闭锁、小肠和大肠闭锁以及肛门闭锁)的婴儿的某些流行病学特征进行了一项研究。数据收集自三个畸形登记处,涵盖了超过450万例出生记录。共识别出3550例患有消化道闭锁的婴儿,总发生率约为每10000例出生中有8例。167例婴儿(4.7%)同时存在一种以上主要闭锁类型。(基于加利福尼亚的数据)发现食管闭锁存在种族差异,白人的发生率高于其他种族。对于胃肠道闭锁,发现黑人的发生率较高,而肛门闭锁在不同种族之间未发现差异。此外,还发现三个项目的登记率存在差异,至少部分原因可以用不同的确诊方式来解释。从性别比例、双胎率、母亲年龄和产次分布、染色体异常的存在以及相关畸形的类型等角度对不同形式的闭锁进行了比较。基于这些观察结果讨论了各种类型闭锁的发病机制和病因。结论是,虽然毫无疑问胃肠道闭锁可能存在其他发病机制,但相当一部分患有消化道闭锁的婴儿其畸形是由于肠道形态发生的早期紊乱所致。在每个亚组中,显然可能存在不同的病因,导致流行病学特征的差异。