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

Lookup NU author(s): Dr Svetlana Glinianaia, Professor Judith RankinORCiD

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This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC 4.0).


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.


Publication metadata

Author(s): Glinianaia SV, Rankin J, Tan J, Loane M, Garne E, Cavero-Carbonell C, de Walle HEK, Gatt M, Gissler M, Klungsøyr K, Lelong N, Neville A, Pierini A, Tucker DF, Urhoj SK, Wellesley DG, Morris JK

Publication type: Article

Publication status: Published

Journal: Archives of Disease in Childhood

Year: 2023

Volume: 108

Issue: 6

Pages: 461-467

Online publication date: 07/03/2023

Acceptance date: 03/02/2023

Date deposited: 08/03/2023

ISSN (print): 0003-9888

ISSN (electronic): 1468-2044

Publisher: BMJ Group

URL: https://doi.org/10.1136/archdischild-2022-325068

DOI: 10.1136/archdischild-2022-325068

ePrints DOI: 10.57711/w3ba-8746

PubMed id: 36882305


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Funding

Funder referenceFunder name
733001Commission of the European Communities

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