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Survival of children with Down syndrome in Italy
P Mastroiacovo1, R Bertollini, C Corchia
1Pediatric Department, Catholic University, Rome, Italy.
Insights
Survival rates for children with Down syndrome (DS) were analyzed. Congenital heart disease, low birth weight, and Southern Italy birth significantly impacted survival, possibly due to healthcare disparities.
Area of Science:
- Pediatrics
- Genetics
- Public Health
Background:
- Down syndrome (DS) is a genetic condition associated with increased health risks.
- Survival rates for individuals with DS have improved over time, but disparities may exist.
- Understanding prognostic factors is crucial for improving care and outcomes for children with DS.
Purpose of the Study:
- To investigate survival rates through age 8 in a cohort of Italian children with Down syndrome.
- To identify factors associated with mortality in children with DS.
- To explore potential reasons for geographical disparities in survival.
Main Methods:
- A cohort study of 917 children with Down syndrome born in Italy (1978-1984).
- Survival analysis through age 8, with univariate and Cox proportional hazard modeling.
- Analysis of factors including congenital heart disease, birth weight, maternal age, parity, socioeconomic status, sex, and region of birth.
Main Results:
- Overall survival at 8 years was approximately 76%.
- Congenital heart disease (CHD), low birth weight (<2,500g), and birth in Southern Italy were independently associated with significantly lower survival.
- No significant differences in survival were observed based on sex or socioeconomic status.
Conclusions:
- CHD, low birth weight, and geographical region are significant prognostic factors for survival in children with Down syndrome.
- Disparities in survival, particularly linked to birth region in Italy, suggest potential differences in healthcare quality.
- These findings highlight the need to address regional healthcare inequalities to improve outcomes for children with DS.
Abstract:
A cohort of 917 Down syndrome (DS) children born in Italy between 1978 and 1984 was studied for survival through the age of 8 years. The highest mortality occurred in the first month of life (7.9%); survival was about 80% at 1 year, 78% at 2 years, and 76% at 5 years, with small decreases thereafter. At the univariate analysis, survival was lower for subjects with congenital heart disease (CHD), birth weight less than 2,500 g, parity of 3 or plus, maternal age greater than or equal to 35 years, and for those born in Southern Italy compared with Northern Italy. No differences in survival were observed by sex and by socioeconomic status. The Cox proportional hazard model was used to evaluate the effect of each variable adjusted for all the others present in the model. Presence of CHD (odds ratio = 3.27; 95% confidence interval (C.I.) 2.31-4.63), birth in the South (odds ratio = 2.69; 95% C.I. 1.91-3.79), and low birth weight (odds ratio = 1.87; 95% C.I. 1.29-2.72) were independently associated with survival. None of the other variables emerged as a statistically significant prognostic factor. Various hypotheses were considered to interpret the unexpected effect of place of birth on survival. Quality of medical care provided in the South of Italy is the most likely determinant of the high mortality observed among children with DS born in that area of Italy. Such differences in survival within the same country could occur in other developed nations as well.