title: Survival of children with trisomy 13 and trisomy 18: A multi-state population-based study.
abstract: Trisomy 13 (T13) and trisomy 18 (T18) are among the most prevalent autosomal trisomies. Both are associated with a very high risk of mortality. Numerous instances, however, of long-term survival of children with T13 or T18 have prompted some clinicians to pursue aggressive treatment instead of the traditional approach of palliative care. The purpose of this study is to assess current mortality data for these conditions. This multi-state, population-based study examined data obtained from birth defect surveillance programs in nine states on live-born infants delivered during 1999-2007 with T13 or T18. Information on children's vital status and selected maternal and infant risk factors were obtained using matched birth and death certificates and other data sources. The Kaplan-Meier method and Cox proportional hazards models were used to estimate age-specific survival probabilities and predictors of survival up to age five. There were 693 children with T13 and 1,113 children with T18 identified from the participating states. Among children with T13, 5-year survival was 9.7%; among children with T18, it was 12.3%. For both trisomies, gestational age was the strongest predictor of mortality. Females and children of non-Hispanic black mothers had the lowest mortality. Omphalocele and congenital heart defects were associated with an increased risk of death for children with T18 but not T13. This study found survival among children with T13 and T18 to be somewhat higher than those previously reported in the literature, consistent with recent studies reporting improved survival following more aggressive medical intervention for these children. © 2015 Wiley Periodicals, Inc.
authors.affiliation: N.C. Division of Public Health, Birth Defects Monitoring Program, State Center for Health Statistics, Raleigh, North Carolina.
authors.forename: Robert E
authors.initials: RE
authors.lastname: Meyer
authors.affiliation: Department of Epidemiology and Biostatistics, University of Albany, State University of New York, Albany, New York.
authors.forename: Gang
authors.initials: G
authors.lastname: Liu
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia.
authors.forename: Suzanne M
authors.initials: SM
authors.lastname: Gilboa
authors.affiliation: Texas Department of State Health Services, Birth Defects Epidemiology and Surveillance Branch, Austin, Texas.
authors.forename: Mary K
authors.initials: MK
authors.lastname: Ethen
authors.affiliation: Departments of Pediatrics and Genetics, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
authors.forename: Arthur S
authors.initials: AS
authors.lastname: Aylsworth
authors.affiliation: Departments of Pediatrics and Genetics, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
authors.forename: Cynthia M
authors.initials: CM
authors.lastname: Powell
authors.affiliation: Arizona Department of Health Services, Birth Defects Monitoring Program, Phoenix, Arizona.
authors.forename: Timothy J
authors.initials: TJ
authors.lastname: Flood
authors.affiliation: National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia.
authors.forename: Cara T
authors.initials: CT
authors.lastname: Mai
authors.affiliation: New York State Department of Health, Office of Primary Care and Health System Management, Albany, New York.
authors.forename: Ying
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Texas Department of State Health Services, Birth Defects Epidemiology and Surveillance Branch, Austin, Texas.
authors.forename: Mark A
authors.initials: MA
authors.lastname: Canfield
mesh_terms: D002675:Child, Preschool; D025063:Chromosome Disorders / Q000453:epidemiology / Q000401:mortality*; D002882:Chromosomes, Human, Pair 13; D002887:Chromosomes, Human, Pair 18; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D053208:Kaplan-Meier Estimate; D008297:Male; D011159:Population Surveillance; D016016:Proportional Hazards Models; D014314:Trisomy; D000073839:Trisomy 13 Syndrome; D000073842:Trisomy 18 Syndrome; D014481:United States / Q000453:epidemiology
keywords: epidemiology; mortality; survival; trisomy 13; trisomy 18