A multi-country study of prevalence and early childhood mortality among children with omphalocele.
A multi-country study of prevalence and early childhood mortality among children with omphalocele.
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DOI：
10.1002/bdr2.1822
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发表时间：
2020-12
影响因子：
2.1
通讯作者：
Mastroiacovo P
中科院分区：
文献类型：
--
作者：
Nembhard WN;Bergman JEH;Politis MD;Arteaga-Vázquez J;Bermejo-Sánchez E;Canfield MA;Cragan JD;Dastgiri S;de Walle HEK;Feldkamp ML;Nance A;Gatt M;Groisman B;Hurtado-Villa P;Kallén K;Landau D;Lelong N;Lopez-Camelo J;Martinez L;Morgan M;Pierini A;Rissmann A;Šípek A;Szabova E;Tagliabue G;Wertelecki W;Zarante I;Bakker MK;Kancherla V;Mastroiacovo P
脐膨出是第二常见的腹部出生缺陷，通常与其他结构和遗传缺陷一起发生。人们对不同人群一岁以后的死亡率、模式和趋势所知甚少。本研究的目的是根据地理区域和相关异常的存在，确定脐膨出婴儿在幼儿期的患病率、时间趋势和死亡率。我们对18个国家的23个出生缺陷监测系统进行了回顾性研究，这些系统都是出生缺陷监测和研究国际信息中心的成员，该中心提交了2000年至2012年间确定病例的数据。调查了大约1600万例因活产、死产或胎儿异常（ETOPFA）而终止妊娠的病例，包括脐膨出病例。计算了特定时间段（24小时、新生儿、婴儿和幼儿）的总体患病率和死亡率。我们使用95%置信区间（CI）的Kaplan-Meier估计来计算累积死亡率，并使用接合点回归进行时间趋势分析。2000年至2012年间，脐膨出的患病率为2.6 / 10000例新生儿（95%置信区间：2.5,2.7）。2000-2012年期间患病率下降幅度最小（平均年变化百分比= - 0.19%，P=0.52）。这一时期的总死亡率为32.1% （95% CI: 30.2, 34.0）。大多数死亡发生在新生儿期和患有多发性或综合征性脐膨出的儿童中。患病率和死亡率因登记类型（例如，以医院为基础与以人群为基础）和纳入或排除ETOPFA而异。在研究期间，脐膨出的患病率没有随时间变化。大约三分之一患有脐膨出的儿童无法在幼儿期存活，大多数死亡发生在新生儿期。在我们的多国研究中，我们发现脐膨出的患病率在研究期间没有改变，大约30%的这些儿童将无法在幼儿期存活，其中大多数死亡发生在新生儿期。
Omphalocele is the second most common abdominal birth defect and often occurs with other structural and genetic defects. Little is known about rates, patterns and trends of mortality after the first year of life in diverse populations. The objective of this study was to determine the prevalence, time trends and mortality during early childhood for infants with omphalocele overall, by geographical region, and by presence of associated anomalies. We conducted a retrospective study with 23 birth defect surveillance systems in 18 countries who are members of the International Clearinghouse for Birth Defects Surveillance and Research, which submitted data on cases ascertained between 2000 and 2012. Approximately 16 million pregnancies that ended in livebirths, stillbirths, or elective terminations for fetal anomalies (ETOPFA) were surveyed and cases with omphalocele were included. Overall prevalence and mortality rates for specific time periods were calculated (24 hours, neonatal, infant and early childhood). We used Kaplan-Meier estimates with 95% confidence intervals (CI) to calculate cumulative mortality and joinpoint regression for time trend analyses. Between 2000 and 2012, the prevalence of omphalocele was 2.6 per 10,000 births (95% CI: 2.5, 2.7). Prevalence decreased minimally during 2000-2012 (average annual percent change = −0.19%, P=0.52). The overall mortality rate for this period was 32.1% (95% CI: 30.2, 34.0). Most deaths occurred during the neonatal period and among children with multiple or syndromic omphalocele. Prevalence and mortality varied by registry type (e.g., hospital- vs. population-based) and inclusion or exclusion of ETOPFA. The prevalence of omphalocele did not change over time during the study period. Approximately one-third of children with omphalocele did not survive early childhood with most deaths occurring in the neonatal period. In our multi-country study, we found that the prevalence of omphalocele did not change over the study period and about thirty percent of these children will not survive early childhood, with most deaths occurring in the neonatal period.
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影响因子：
6.3
作者：
DOYLE, PE;BERAL, V;WALE, CJ
通讯作者：
WALE, CJ
影响因子：
7.1
作者：
Brantberg, A;Blaas, HGK;Eik-Nes, SH
通讯作者：
Eik-Nes, SH
影响因子：
6.3
作者：
HEMMINKI, K;SALONIEMI, I;KEKOMAKI, M
通讯作者：
KEKOMAKI, M
DOI：
10.1002/ajmg.1320580218
发表时间：
1995-08-28
期刊：
AMERICAN JOURNAL OF MEDICAL GENETICS
影响因子：
--
作者：
CALZOLARI, E;BIANCHI, F;STONE, DH
通讯作者：
STONE, DH
影响因子：
2.9
作者：
Allman, R.;Sousa, J.;Clark, R. H.
通讯作者：
Clark, R. H.