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食管闭锁:23 个欧洲地区的患病率、产前诊断和相关畸形。

Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions.

机构信息

Department of Pediatrics, Odense University Hospital, Odense, Denmark.

出版信息

Arch Dis Child. 2012 Mar;97(3):227-32. doi: 10.1136/archdischild-2011-300597. Epub 2012 Jan 13.

DOI:10.1136/archdischild-2011-300597
PMID:22247246
Abstract

OBJECTIVE

To describe prevalence, prenatal diagnosis and epidemiological data on oesophageal atresia from 23 well-defined European regions and compare the prevalence between these regions.

DESIGN

Population-based study using data from a large European database for surveillance of congenital anomalies (EUROCAT) for two decades (1987-2006).

SETTINGS

Twenty-three participating registries based on multiple sources of information including information about live births, fetal deaths with gestational age ≥20 weeks and terminations of pregnancy.

PATIENTS

1222 cases of oesophageal atresia in a population of 5 019 804 births.

RESULTS

The overall prevalence was 2.43 cases per 10 000 births (95% CI 2.30 to 2.57). There were regional differences in prevalence ranging from 1.27 to 4.55. Prenatal detection rates varied by registry from >50% of cases to <10% of cases. A total of 546 cases (44.7%) had an isolated oesophageal anomaly, 386 (31.6%) were multiple malformed and 290 (23.7%) had an association or a syndrome. There were 1084 live born cases (88.7%), 43 cases were fetal deaths and 95 cases were terminations of pregnancy. One-week survival for live births was 86.9% and 99.2% if the gestational age was ≥38 weeks and isolated oesophageal atresia was present. Males accounted for 57.3% of all cases and 38.5% of live born cases were born with gestational age <37 weeks.

CONCLUSION

There were regional differences in prevalence of oesophageal atresia in Europe. Half of all cases had associated anomalies. Prenatal detection rate increased from 26% to 36.5% over the two decades. Survival in infants with isolated oesophageal atresia born at term is high.

摘要

目的

描述 23 个明确界定的欧洲地区食管闭锁的流行率、产前诊断和流行病学数据,并比较这些地区之间的流行率。

设计

使用欧洲先天性异常监测大型数据库(EUROCAT)二十年(1987-2006 年)的数据进行基于人群的研究。

设置

23 个参与的登记处基于多种信息来源,包括活产、孕龄≥20 周的胎儿死亡和终止妊娠的信息。

患者

1222 例食管闭锁患者,来自 5019804 例出生人口。

结果

总体患病率为每 10000 例出生 2.43 例(95%CI 2.30 至 2.57)。患病率存在地区差异,范围为 1.27 至 4.55。各登记处的产前检出率从>50%的病例到<10%的病例不等。共有 546 例(44.7%)存在单纯食管异常,386 例(31.6%)为多发性畸形,290 例(23.7%)存在关联或综合征。有 1084 例活产(88.7%),43 例胎儿死亡,95 例终止妊娠。活产的 1 周生存率为 86.9%,如果孕龄≥38 周且存在单纯食管闭锁,生存率为 99.2%。所有病例中男性占 57.3%,活产中 38.5%的婴儿出生时孕龄<37 周。

结论

欧洲食管闭锁的流行率存在地区差异。一半的病例存在相关畸形。在这二十年中,产前检出率从 26%增加到 36.5%。在足月出生的单纯食管闭锁婴儿中,存活率较高。

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