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

Svetlana V Glinianaia, Judith Rankin, Joachim Tan, Maria Loane, Ester Garne, Clara Cavero‑Carbonell, Hermien EK de Walle, Miriam Gatt, Mika Gissler, Kari Klungsøyr, Nathalie Lelong, Amanda J Neville, Anna Pierini, David Tucker, Stine K Urhoj, Diana Wellesley, Joan K Morris

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Abstract

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
Article numberarchdischild-2022-325068
Pages (from-to)1-7
Number of pages8
JournalArchives of disease in childhood
Volume108
Issue number6
Early online date7 Mar 2023
DOIs
Publication statusPublished online - 7 Mar 2023

Bibliographical note

Funding Information:
The European Union’s Horizon 2020 research and innovation programme under grant agreement No. 733 001 (January 2017–May 2022) https://ec.europa.eu/programmes/horizon2020/en ).

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

Keywords

  • Child Health
  • Mortality
  • Syndrome
  • Genetics
  • Epidemiology

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