Copeland Glenn E, Kirby Russell S
Michigan Birth Defects Registry, Michigan Department of Community Health, Lansing, Michigan 48913, USA.
Birth Defects Res A Clin Mol Teratol. 2007 Nov;79(11):792-7. doi: 10.1002/bdra.20391.
Although birth defects are a leading cause of death in infancy and early childhood, the proportion of all deaths to children with clinically diagnosed birth defects is not well documented. The study is intended to measure the proportion of all deaths to infants and children under age 10 occurring to children with birth defects and how and why this proportion differs from the proportion of deaths due to an underlying cause of congenital anomalies using standard mortality statistics.
A linked file of Michigan livebirths and deaths was combined with data from a comprehensive multisource birth defects registry of Michigan livebirths born during the years 1992 through 2000. The data were analyzed to determine the mortality rate for infants and children with birth defects and for children with no reported birth defect. Mortality risk ratios were calculated. The underlying causes of death for children with birth defects were also categorized and compared to cause- specific mortality rates for the general population.
Congenital anomalies were the underlying cause of death for 17.8% of all infant deaths while infants with birth defects were 33.7% of all infant deaths in the study. Almost half of all Michigan deaths to children aged 1 to 2 were within the birth defects registry, though only 15.0% had an underlying cause of death of a congenital anomaly based upon standard mortality statistics. The mortality experience among children with birth defects was significantly higher than other children throughout the first 9 years of life, ranging from 4.6 for 5 year olds to 12.8 for children 1 to 2. Mortality risk ratios examined by cause of death for infants with birth defects were highest for other endocrine (28.1), other CNS (28.1), and heart (21.9) conditions. For children 1 through 9, the highest differential risk was seen for other perinatal conditions (39.0), other endocrine (29.7), other CNS (24.5), and heart (21.4).
Childhood mortality analyses that incorporate birth defects registry data provide a more comprehensive picture of the full burden of birth defects on mortality in infant and children and can provide an effective mechanism for monitoring the survival and mortality risks of children with selected birth defects on a population basis.
尽管出生缺陷是婴儿期和幼儿期死亡的主要原因之一，但临床诊断为出生缺陷的儿童在所有死亡儿童中所占的比例尚无充分记录。本研究旨在利用标准死亡率统计数据，测算出生缺陷儿童在10岁以下婴儿和儿童的所有死亡中所占的比例，以及该比例与先天性异常潜在病因导致的死亡比例存在差异的方式和原因。
密歇根州出生与死亡的关联文件与1992年至2000年期间在密歇根州出生的综合多源出生缺陷登记处的数据相结合。对数据进行分析，以确定出生缺陷儿童和未报告出生缺陷儿童的死亡率。计算死亡风险比。还对出生缺陷儿童的潜在死因进行了分类，并与一般人群的特定病因死亡率进行了比较。
先天性异常是所有婴儿死亡的17.8%的潜在死因，而本研究中出生缺陷婴儿占所有婴儿死亡的33.7%。密歇根州1至2岁儿童死亡中，几乎一半在出生缺陷登记处，但根据标准死亡率统计，只有15.0%的潜在死因为先天性异常。在生命的前9年中，出生缺陷儿童的死亡经历显著高于其他儿童，5岁儿童为4.6，1至2岁儿童为12.8。出生缺陷婴儿按死因检查的死亡风险比，其他内分泌疾病（28.1）、其他中枢神经系统疾病（28.1）和心脏病（21.9）最高。对于1至9岁儿童，其他围产期疾病（39.0）、其他内分泌疾病（29.7）、其他中枢神经系统疾病（24.5）和心脏病（21.4）的差异风险最高。
纳入出生缺陷登记数据的儿童死亡率分析能更全面地反映出生缺陷对婴幼儿死亡率的全部负担，并能提供一种有效的机制，以在人群基础上监测特定出生缺陷儿童的生存和死亡风险。
