title: Mortality of New York children with sickle cell disease identified through newborn screening.
abstract: OBJECTIVE
  Long-term follow-up of newborn screening for conditions such as sickle cell disease can be conducted using linkages to population-based data. We sought to estimate childhood sickle cell disease mortality and risk factors among a statewide birth cohort with sickle cell disease identified through newborn screening.
  
  
  METHODS
  Children with sickle cell disease identified by newborn screening and born to New York residents in 2000-2008 were matched to birth and death certificates. Mortality rates were calculated (using numbers of deaths and observed person-years at risk) and compared with mortality rates for all New York children by maternal race/ethnicity. Stratified analyses were conducted to examine associations between selected factors and mortality.
  
  
  RESULTS
  Among 1,911 infants with sickle cell disease matched to birth certificates, 21 deaths were identified. All-cause mortality following diagnosis was 3.8 per 1,000 person-years in the first 2 years of life and 1.0 per 1,000 person-years at ages 2-9 years. The mortality rate was significantly lower among children of foreign-born mothers and was significantly higher among preterm infants with low birth weight. The mortality rates were not significantly higher for infants after 28 days with sickle cell disease than for all New York births, but they were 2.7-8.4 times higher for children 1 through 9 years old with homozygous sickle cell disease than for those of all non-Hispanic black or Hispanic children born to New York residents.
  
  
  CONCLUSIONS
  Estimated mortality risk in children with homozygous sickle cell disease remains elevated even after adjustment for maternal race/ethnicity. These results provide evidence regarding the current burden of child mortality among children with sickle cell disease despite newborn screening.Genet Med 17 6, 452-459.
authors.affiliation: 1] School of Public Health, University at Albany, State University of New York, Albany, New York, USA [2] Division of Data Management and Research, Office of Primary Care and Health Management System, New York State Department of Health, Albany, New York, USA.
authors.forename: Ying
authors.identifier: 0000000158482688
authors.initials: Y
authors.lastname: Wang
authors.affiliation: School of Public Health, University at Albany, State University of New York, Albany, New York, USA.
authors.forename: Gang
authors.initials: G
authors.lastname: Liu
authors.affiliation: 1] School of Public Health, University at Albany, State University of New York, Albany, New York, USA [2] Newborn Screening Program, Wadsworth Center, New York State Department of Health, Albany, New York, USA.
authors.forename: Michele
authors.initials: M
authors.lastname: Caggana
authors.affiliation: Office of Vital Statistics, New York City Department of Health and Mental Hygiene, New York, New York, USA.
authors.forename: Joseph
authors.initials: J
authors.lastname: Kennedy
authors.affiliation: Office of Vital Statistics, New York City Department of Health and Mental Hygiene, New York, New York, USA.
authors.forename: Regina
authors.initials: R
authors.lastname: Zimmerman
authors.affiliation: Department of Pediatrics, Albert Einstein College of Medicine/Children's Hospital Montefiore, New York, New York, USA.
authors.forename: Suzette O
authors.initials: SO
authors.lastname: Oyeku
authors.affiliation: Division of Blood Diseases and Resources, National Heart, Lung and Blood Institute, Bethesda, Maryland, USA.
authors.forename: Ellen M
authors.initials: EM
authors.lastname: Werner
authors.affiliation: Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
authors.forename: Althea M
authors.initials: AM
authors.lastname: Grant
authors.affiliation: Department of Pediatrics, Columbia University Medical Center, New York, New York, USA.
authors.forename: Nancy S
authors.initials: NS
authors.lastname: Green
authors.affiliation: Division of Blood Disorders, National Center on Birth Defects and Developmental Disabilities, Centers for Disease Control and Prevention, Atlanta, Georgia, USA.
authors.forename: Scott D
authors.initials: SD
authors.lastname: Grosse
mesh_terms: D000755:Anemia, Sickle Cell / Q000175:diagnosis / Q000453:epidemiology / Q000401:mortality*; D002423:Cause of Death; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006451:Hemoglobin, Sickle / Q000235:genetics; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009026:Mortality; D015997:Neonatal Screening; D009518:New York / Q000453:epidemiology; D010641:Phenotype; D011159:Population Surveillance; D012307:Risk Factors
chemical_list: D006451:Hemoglobin, Sickle