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食管闭锁:西班牙巴伦西亚地区的患病率及相关畸形。

Oesophageal Atresia: Prevalence in the Valencian Region (Spain) and Associated Anomalies.

机构信息

Service of Preventive Medicine, Valencia General University Hospital Consortium, 46014 Valencia, Spain.

Rare Diseases Joint Research Unit, Foundation for the Promotion of Health and Biomedical Research in the Valencian Region-Valencia University (FISABIO-UVEG), 46020 Valencia, Spain.

出版信息

Int J Environ Res Public Health. 2023 Feb 24;20(5):4042. doi: 10.3390/ijerph20054042.

Abstract

The objective was to determine the prevalence of oesophageal atresia (OA) and describe the characteristics of OA cases diagnosed before the first year of life, born between 2007 and 2019, and residents in the Valencian Region (VR), Spain. Live births (LB), stillbirths (SB), and termination of pregnancy for fetal anomaly (TOPFA) diagnosed with OA were selected from the Congenital Anomalies population-based Registry of VR (RPAC-CV). The prevalence of OA per 10,000 births with 95% confidence interval was calculated, and socio-demographic and clinical variables were analyzed. A total of 146 OA cases were identified. The overall prevalence was 2.4/10,000 births, and prevalence by type of pregnancy ending was 2.3 in LB and 0.03 in both SB and TOPFA. A mortality rate of 0.03/1000 LB was observed. A relationship was found between case mortality and birth weight (-value < 0.05). OA was primarily diagnosed at birth (58.2%) and 71.2% of the cases were associated with another congenital anomaly, mainly congenital heart defects. Significant variations in the prevalence of OA in the VR were detected throughout the study period. In conclusion, a lower prevalence in SB and TOPFA was identified compared to EUROCAT data. As several studies have identified, an association between OA cases and birth weight was found.

摘要

目的是确定食管闭锁(OA)的患病率,并描述 2007 年至 2019 年间在西班牙瓦伦西亚地区(VR)出生、诊断于一岁以下的 OA 病例的特征。从 VR 的先天性畸形基于人群的登记处(RPAC-CV)中选择诊断为 OA 的活产(LB)、死产(SB)和因胎儿异常而终止妊娠(TOPFA)。计算每 10000 例出生中 OA 的患病率及其 95%置信区间,并分析社会人口统计学和临床变量。共确定了 146 例 OA 病例。总体患病率为 2.4/10000 例,终止妊娠类型的患病率分别为 LB 中的 2.3 和 SB 和 TOPFA 中的 0.03。观察到 LB 中死亡率为 0.03/1000。发现病例死亡率与出生体重之间存在关系(-值<0.05)。OA 主要在出生时诊断(58.2%),71.2%的病例与另一种先天性异常有关,主要是先天性心脏病。在整个研究期间,VR 中 OA 的患病率存在显著差异。总之,与 EUROCAT 数据相比,SB 和 TOPFA 中的患病率较低。正如几项研究所指出的,OA 病例与出生体重之间存在关联。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f0d9/10001480/0da99ffd347a/ijerph-20-04042-g001.jpg

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