title: Using birth defects registry data to evaluate infant and childhood mortality associated with birth defects: an alternative to traditional mortality assessment using underlying cause of death statistics.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Michigan Birth Defects Registry, Michigan Department of Community Health, Lansing, Michigan 48913, USA. Copelandg@Michigan.gov
authors.forename: Glenn E
authors.initials: GE
authors.lastname: Copeland
authors.forename: Russell S
authors.initials: RS
authors.lastname: Kirby
mesh_terms: D001719:Birth Certificates; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D000013:Congenital Abnormalities / Q000401:mortality*; D003644:Death Certificates; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008498:Medical Record Linkage; D009026:Mortality / Q000639:trends*; D012042:Registries; D015996:Survival Rate; D014798:Vital Statistics