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瑞典先天性腹裂的全国流行率、相关畸形和死亡率。

National birth prevalence, associated anomalies and mortality for gastroschisis in Sweden.

机构信息

Division for Pediatric Surgery, Astrid Lindgren Children's Hospital, Karolinska University Hospital, Stockholm, Sweden.

Department of Women's and Children's Health, Karolinska Institute, Stockholm, Sweden.

出版信息

Acta Paediatr. 2021 Sep;110(9):2635-2640. doi: 10.1111/apa.15954. Epub 2021 Jun 4.

Abstract

AIM

An increased incidence has been reported for the congenital abdominal wall defect gastroschisis. The reason for this increasing trend is not known, nor the aetiology. The aim of this study was to examine the national birth prevalence in Sweden, the termination rate, associated anomalies and the mortality of gastroschisis within the cohort.

METHODS

A nationwide, population-based descriptive study of children born with gastroschisis in Sweden between 1/1 1997 and 31/12 2016 was conducted. The cohort was collected from the Swedish Medical Birth Register and the Swedish National Patient Register. Several other national registers were then interlinked to identify outcome data.

RESULTS

The study included 361 cases of gastroschisis, 54% female. The birth prevalence was 1.52 in 10,000 live births. The termination rate was 21%. The mortality within the cohort was 4.4% with a 1-year mortality of 3.9%. Most frequent associated anomalies were gastrointestinal (11.4%), musculoskeletal (9.8%) and cardiovascular anomalies (7.9%).

CONCLUSION

During the 20-year study period, a stable birth prevalence of 1.52 per 10 000 live births was seen in Sweden. The mortality was low, 4.4%, but the termination of pregnancies was high, 21%. Almost one-third had associated congenital anomalies where gastrointestinal anomalies were the most common.

摘要

目的

先天性腹壁缺陷腹裂的发病率有所增加。这种上升趋势的原因尚不清楚,病因也不清楚。本研究的目的是检查瑞典全国的发病率、终止率、相关畸形和腹裂的死亡率。

方法

对 1997 年 1 月 1 日至 2016 年 12 月 31 日期间在瑞典出生的患有腹裂的儿童进行了一项全国性、基于人群的描述性研究。该队列是从瑞典医学出生登记处和瑞典国家患者登记处收集的。然后将其他几个国家的登记处进行了链接,以确定结果数据。

结果

本研究包括 361 例腹裂病例,其中 54%为女性。发病率为每 10000 例活产中有 1.52 例。终止率为 21%。该队列的死亡率为 4.4%,1 年死亡率为 3.9%。最常见的相关畸形是胃肠道(11.4%)、肌肉骨骼(9.8%)和心血管畸形(7.9%)。

结论

在 20 年的研究期间,瑞典的发病率稳定在每 10000 例活产中有 1.52 例。死亡率较低,为 4.4%,但终止妊娠率较高,为 21%。近三分之一的患儿存在先天性畸形,其中胃肠道畸形最为常见。

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