Survival Analysis of Down Syndrome Cohort in a Tertiary Health Care Center in India
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To identify the major causes of death in Down Syndrome (DS), the ages at which mortality rates are the highest and recognize factors associated with it.
The prospective cohort-based study was carried out in a tertiary health care center. Children with DS (n = 543) counseled at the Center of Medical Genetics, Sir Ganga Ram Hospital from 2005 through 2009 were followed up in year 2010. Survival curves and Cox’s proportional hazards regression analysis were used to determine the effect of different variables on survival.
Total mortality was 13 %, of which 80.3 % was in children less than 2 y of age. Presence of congenital heart disease (CHD) increased the risk of mortality by 5.7 folds (p = 0.001). A definitive survival benefit after cardiac intervention was noted, although it differed with the type of CHD. Sex, maternal age at time of birth and karyotypes did not show a significant correlation with survival.
The higher DS infant mortality observed in the present study could be attributed to financial constraints of the families and misconceptions amongst health professionals. It is recommended that a nation-wide DS registry be created to study the morbidity and mortality in Down syndrome from birth. The findings of this study will help clinicians in making management decisions and enable better counseling.
KeywordsDown syndrome Mortality Survival Congenital heart disease India
The authors would like to thank Ashish Upadhyay for his assistance with statistical analysis.
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