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Sökning: WFRF:(Hek K) > (2023)

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  • Glinianaia, SV, et al. (författare)
  • Ten-year survival of children with trisomy 13 or trisomy 18: a multi-registry European cohort study
  • 2023
  • Ingår i: Archives of disease in childhood. - : BMJ. - 1468-2044 .- 0003-9888. ; 108:6, s. 461-467
  • Tidskriftsartikel (refereegranskat)abstract
    • To investigate the survival to 10 years of age of children with trisomy 13 (T13) and children with trisomy 18 (T18), born 1995–2014.DesignPopulation-based cohort study that linked mortality data to data on children born with T13 or T18, including translocations and mosaicisms, from 13 member registries of EUROCAT, a European network for the surveillance of congenital anomalies.Setting13 regions in nine Western European countries.Patients252 live births with T13 and 602 with T18.Main outcome measuresSurvival at 1 week, 4 weeks and 1, 5 and 10 years of age estimated by random-effects meta-analyses of registry-specific Kaplan-Meier survival estimates.ResultsSurvival estimates of children with T13 were 34% (95% CI 26% to 46%), 17% (95% CI 11% to 29%) and 11% (95% CI 6% to 18%) at 4 weeks, 1 and 10 years, respectively. The corresponding survival estimates were 38% (95% CI 31% to 45%), 13% (95% CI 10% to 17%) and 8% (95% CI 5% to 13%) for children with T18. The 10-year survival conditional on surviving to 4 weeks was 32% (95% CI 23% to 41%) and 21% (95% CI 15% to 28%) for children with T13 and T18, respectively.ConclusionsThis multi-registry European study found that despite extremely high neonatal mortality in children with T13 and T18, 32% and 21%, respectively, of those who survived to 4 weeks were likely to survive to age 10 years. These reliable survival estimates are useful to inform counselling of parents after prenatal diagnosis.
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