Reference Center for Congenital Oesophageal Anomalies, University Hospital Lille, France.
J Pediatr Surg. 2013 Aug;48(8):1664-9. doi: 10.1016/j.jpedsurg.2013.03.075.
A prospective national register was established in 2008 to record all new cases of live-birth newborns with esophageal atresia (EA). This epidemiological survey was recommended as part of a national rare diseases plan.
All 38 national centers treating EA participated by completing for each patient at first discharge a questionnaire validated by a national committee of experts. Data were centralized by the national reference center for esophageal anomalies. Quantitative and qualitative analyses were performed, with P-values of less than 0.05 considered statistically significant. Results of the 2008-2009 data collection are presented in this report.
Three hundred seven new living cases of EA were recorded between January 1, 2008, and December 31, 2009. The male/female sex ratio was 1.3, and the live-birth prevalence of EA was 1.8 per 10,000 births. Major characteristics were comparable to those reported in the literature. Survival was 95%, and no correlation with caseload was noted.
Epidemiologic surveys of congenital anomalies such as EA, which is a rare disease, provide valuable data for public health authorities and fulfill one important mission of reference centers. When compared with previous epidemiological data, this national population-based registry suggests that the incidence of EA remains stable.
2008 年建立了一个前瞻性国家登记处，以记录所有患有食管闭锁（EA）的活产新生儿的新病例。这一流行病学调查是作为国家罕见病计划的一部分而推荐的。
所有 38 个治疗 EA 的国家中心均通过为首次出院的每位患者填写一份由国家专家委员会验证的问卷参与其中。数据由国家食管异常参考中心集中。进行了定量和定性分析，P 值小于 0.05 被认为具有统计学意义。本报告介绍了 2008-2009 年数据收集的结果。
2008 年 1 月 1 日至 2009 年 12 月 31 日期间，共记录了 307 例新的 EA 活产病例。男/女比例为 1.3，EA 的活产患病率为每 10,000 例活产 1.8 例。主要特征与文献报道的相似。存活率为 95%，且与病例量无关。
对 EA 等罕见疾病的先天性畸形进行流行病学调查为公共卫生当局提供了有价值的数据，并履行了参考中心的一项重要使命。与以前的流行病学数据相比，这个基于全国人口的登记处表明 EA 的发病率保持稳定。
