SURVIVAL OF CHILDREN WITH DOWN-SYNDROME IN SOUTH-AMERICA

The first step of all healthcare actions aimed at promoting an appropr iate quality of life for infants affected by Down syndrome (DS) is to ensure their survival. This investigation was aimed at estimating the infant mortality rate of infants affected with DS in urban populations of South America. Thirty-three hospitals included in the Latin Americ an Collaborative Study of Congenital Malformations (ECLAMC) distribute d in 23 cities of 5 South American countries followed 360 liveborn DS cases born during the 1988-1992 period. Families were recontacted afte r the infant should have reached the age of one year. The collected da ta included information about health status; i.e., frequency and dates of diagnosed illnesses and hospital admissions, and, in case of death , information on date, place and cause of death, and illness immediate ly before death. Information about the interviews included place, date , and name of the interviewer. A closed questionnaire was employed by the interviewers, mostly physicians, nurses, and social workers. Life table analysis up to the age of one year was performed by the actuaria l survival method. The overall mean survival at age one year was 0.736 (SE = 0.023). Thirty-three (9.2%) of the 360 cases died neonatally, a nd 62 (17.2%) within the remaining 2-to-12-month interval. The probabi lity of survival at one year of age did not differ between public (209 cases; mean 0.718; SE = 0.031) and private (151 cases; mean: 0.762; S E = 0.035) (chi(2):0.87; df:1; P >0.05) health systems. The 150 DS cas es with a congenital heart defect (CHD) had a significantly lower Prob ability of survival at the age of one year (mean: 0.660; SE: 0.039) th an did the 210 cases without CHD (mean: 0.790; SE: 0.028) (chi(2):6.67 ; df:1; P <0.01). The death rate in the first year of life for DS case s without a detected cardiac defect (21%) is significantly higher than that reported in developed countries; namely, 16% from Italy, 11% fro m Canada, 10% from England, and 7% from Denmark. Am. J. Med. Genet. 79 :108-111, 1998. (C) 1998 Wiley-Liss, Inc.