Ten-year survival of children with trisomy 13 or trisomy 18: a multi-registry European cohort study

Research output: Contribution to journalJournal articleResearchpeer-review

  • Svetlana V Glinianaia
  • Judith Rankin
  • Joachim Tan
  • Maria Loane
  • Ester Garne
  • Clara Cavero-Carbonell
  • Hermien E K de Walle
  • Miriam Gatt
  • Mika Gissler
  • Kari Klungsøyr
  • Natalie Lelong
  • Amanda Neville
  • Anna Pierini
  • David F Tucker
  • Urhøj, Stine Kjær
  • Diana Gay Wellesley
  • Joan K Morris

OBJECTIVE: To investigate the survival to 10 years of age of children with trisomy 13 (T13) and children with trisomy 18 (T18), born 1995-2014.

DESIGN: Population-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.

SETTING: 13 regions in nine Western European countries.

PATIENTS: 252 live births with T13 and 602 with T18.

MAIN OUTCOME MEASURES: Survival 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.

RESULTS: Survival 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.

CONCLUSIONS: This 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.

Original languageEnglish
JournalArchives of Disease in Childhood
Volume108
Issue number6
Number of pages7
ISSN1743-0585
DOIs
Publication statusPublished - 2023

Bibliographical note

© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.

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