PMID- 12671111
OWN - NLM
STAT- MEDLINE
DCOM- 20030418
LR  - 20041117
IS  - 1098-4275 (Electronic)
IS  - 0031-4005 (Linking)
VI  - 111
IP  - 4 Pt 1
DP  - 2003 Apr
TI  - Population-based analyses of mortality in trisomy 13 and trisomy 18.
PG  - 777-84
AB  - OBJECTIVE: Although trisomy 13 and trisomy 18 are generally considered to be
      lethal, long-term survival of patients has been reported. We sought to evaluate
      mortality in people with trisomy 13 or 18 using 2 population-based strategies.
      METHODS: In the first analysis, infants who had trisomy 13 or 18 and were born
      during 1968-1999 were identified using the Metropolitan Atlanta Congenital
      Defects Program, a population-based birth defects surveillance system. Dates of
      death were documented using hospital records, Georgia vital records, and the
      National Death Index. In the second analysis, we used the Multiple-Cause
      Mortality Files compiled from US death certificates from 1979 through 1997. Using
      these 2 analyses, we examined median survival time or median age at death,
      survival beyond 1 year of age, and factors associated with longer survival.
      RESULTS: Using Metropolitan Atlanta Congenital Defects Program, we identified 70 
      liveborn infants with trisomy 13 and 114 liveborn infants with trisomy 18. Median
      survival time was 7 days (95% confidence interval [CI]: 3-15) for people with
      trisomy 13 and 14.5 days (95% CI: 8-28) for people with trisomy 18. For each
      condition, 91% of infants died within the first year. Neither race nor gender
      affected survival for trisomy 13, but for trisomy 18, girls and infants of races 
      other than white seemed to survive longer. The presence of a heart defect did not
      seem to affect survival for either condition. Using MCMF, we identified 5515
      people with trisomy 13 and 8750 people with trisomy 18 listed on their death
      certificates. Median ages at death for people with trisomy 13 and trisomy 18 both
      were 10 days; 5.6% of people with trisomy 13 and 5.6% of people with trisomy 18
      died at age 1 year or greater. Race and gender seemed to affect survival in both 
      conditions, with girls and blacks showing higher median ages at death.
      CONCLUSIONS: Although survival is greatly affected by trisomy 13 and trisomy 18, 
      5% to 10% of people with these conditions survive beyond the first year of life. 
      These population-based data are useful to clinicians who care for patients with
      these trisomies or counsel families with infants or fetuses who have a diagnosis 
      of trisomy 13 or 18.
FAU - Rasmussen, Sonja A
AU  - Rasmussen SA
AD  - National Center on Birth Defects and Developmental Disabilities, Centers for
      Disease Control and Prevention, Atlanta, Georgia, USA. skr9@cdc.gov
FAU - Wong, Lee-Yang C
AU  - Wong LY
FAU - Yang, Quanhe
AU  - Yang Q
FAU - May, Kristin M
AU  - May KM
FAU - Friedman, J M
AU  - Friedman JM
LA  - eng
PT  - Journal Article
PT  - Multicenter Study
PL  - United States
TA  - Pediatrics
JT  - Pediatrics
JID - 0376422
SB  - AIM
SB  - IM
MH  - African Continental Ancestry Group/genetics
MH  - Chromosome Disorders/*epidemiology/genetics/*mortality
MH  - Chromosomes, Human, Pair 13/*genetics
MH  - Chromosomes, Human, Pair 18/*genetics
MH  - Cytogenetic Analysis/statistics & numerical data
MH  - Databases, Factual
MH  - Death Certificates
MH  - European Continental Ancestry Group/genetics
MH  - Female
MH  - Genes, Lethal/genetics
MH  - Genetics, Population/methods
MH  - Georgia
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Male
MH  - Sex Factors
MH  - Survival Rate
MH  - Trisomy/*genetics
EDAT- 2003/04/03 05:00
MHDA- 2003/04/19 05:00
CRDT- 2003/04/03 05:00
PHST- 2003/04/03 05:00 [pubmed]
PHST- 2003/04/19 05:00 [medline]
PHST- 2003/04/03 05:00 [entrez]
PST - ppublish
SO  - Pediatrics. 2003 Apr;111(4 Pt 1):777-84.