Suvi Alikärri, Ilkka Helenius, Susanna Heiskanen, Johanna Syvänen, Teemu Kemppainen, Eliisa Löyttyniemi, Mika Gissler, Arimatias Raitio
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引用次数: 0
Abstract
Introduction: The aim of the study was to investigate the prevalence of esophageal atresia (EA), frequency of associated anomalies, and mortality from 2004 to 2017 in the Finnish population. We hypothesized the Spitz classification and the presence of other congenital malformations would predict mortality in patients with EA as well as assumed the survival to be high among patients with EA.
Materials and methods: This retrospective, population-based study was based on the registries maintained by THL Finnish Institute for Health and Welfare and Statistics Finland. The cases were identified and classified according to the World Health Organization's International Classification of Diseases and Health Related Problems (ICD) revisions 9 and 10 (ICD-9 and ICD-10) codes and accompanying written diagnoses. Associated anomalies were classified based on the EUROCAT criteria, and minor anomalies were excluded. All statistical tests were performed as a two-sided significance level set at p < 0.05. The chi-square or Fisher's exact test was utilized for categorical variables. The change in prevalence rates during the study period was evaluated with linear regression.
Results: In total, 337 cases with EA were identified including 295 (87.5%) live births, 17 (5.0%) stillbirths, and 25 (7.4%) terminations of pregnancy. The total prevalence for EA in Finland was 4.17/10,000 births with no significant change during the study period, p = 0.35. Neonatal mortality was 5% (n = 15) and 1-year survival was 91.5%. Mortality was associated with syndromic cases (p = 0.002). The Spitz classification predicted neonatal mortality better than cardiac anomalies alone (p < 0.001 and p = 0.6, respectively). Type C was the most common atresia type (65.9%) followed by type A (14.8%) and B (6.8%). The most common group of associated malformations were heart defects (35.0%) followed by other gastrointestinal tract malformations (15.3%) and limb anomalies (12.2%). Syndromic cases (12.2%) were associated with type A and B atresias (p = 0.001). VACTERL association was observed in 16.6% of the cases.
Conclusion: The overall prevalence of EA remains stable and relatively high in Finland. Despite the high prevalence of co-occurring malformations, the overall survival rate is high. Spitz classification predicted neonatal survival well.
导言 本研究旨在调查 2004-2017 年期间芬兰人口中食管闭锁(EA)的发病率、相关畸形的频率和死亡率。我们假设斯皮茨分类和其他先天性畸形的存在将预测食管闭锁患者的死亡率,并假设食管闭锁患者的存活率较高。材料与方法 这项以人群为基础的回顾性研究基于芬兰卫生与福利研究所(THL Finnish Institute for Health and Welfare)和芬兰统计局(Statistics Finland)的登记资料。病例根据 ICD-9 和 ICD-10 编码及随附的书面诊断进行识别和分类。伴发异常根据 EUROCAT 标准进行分类,轻微异常则不包括在内。所有统计检验均以双侧显著性水平 p
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