International Journal of Epidemiology, Vol 26, 822-829, Copyright © 1997 by International Epidemiological Association
C Hayes, Z Johnson, L Thornton, J Fogarty, R Lyons, M O'Connor, V Delany and K Buckley
OBJECTIVE: To determine the survival status of children with Down syndrome
(DS), and to document factors influencing survival. DESIGN: Follow-up study
of cases identified from the Dublin European Register of Congenital
Anomalies and Twins (EUROCAT) Register. Follow-up was attempted for each
case until death or 1992 or until the date last known to be alive. SETTING:
Eastern Health Board, Dublin. SUBJECTS: In all, 389 DS children, born
between 1 January 1980 and 31 December 1989 were followed up. RESULTS:
Survival rates of 88% at one year and 82% at 10 years were found. There was
a non-significant improvement in survival between the cohort born in
1980-1984 and that born in 1985- 1989. Congenital heart defects reduced
survival to 72% and complete atrio-ventricular canal defects (CAVD) had the
poorest prognosis (58% survival at 10 years). Cases with CAVD showed a
trend towards improved survival when surgically treated. Maternal age
mother's county of residence, sex of infant, season of birth and presence
of additional non-cardiac congenital anomalies had no impact on survival.
CONCLUSIONS: Four out of five DS children now survive at least 10 years.
Adequate educational and health service provision needs to be made for
them, especially those with congenital heart defects. The need for studies
which compare survival and quality of life in DS children with CAVD who
undergo cardiac surgery versus those who do not, taking account of various
selection factors, is identified.
ARTICLES
Ten-year survival of Down syndrome births
Health Information Unit, Eastern Health Board, Dr Steevens Hospital, Dublin, Ireland.
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