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Keywords:

  • Epidemiology;
  • cystic fibrosis;
  • Latin America

Abstract

We conducted a retrospective study of 743 patients with cystic fibrosis (CF), covering the 1979–89 period in 4 Latin American (LA) institutions to evaluate the clinical features of the disease. The review, although dealing only with patients referred to, or diagnosed at these institutions may be considered to comprise the majority of patients living in these areas. Diagnosis was confirmed by the sweat test in all cases. There was a slight predominance of males, (61.0%) mainly in the Brazilian group. The mean age at diagnosis was over 3 years. This was similar to the mean age at admission. Almost half of the patients were still alive at follow-up, but their mean age was low (6.4 years in Brazil, 7.4 in Chile, 9.6 in Mexico, and 11.3 in Argentina). Mean age at death ranged from 3.1 years (Chile) to 8.7 years (Argentina). The highest proportion of living patients was in Chile (71.4%) and the lowest in Argentina (33.8%). Survival curve comprised 15 years after diagnosis for the Argentinian group, compared to 6 years for the remaining groups. Fifty percent of the patients lived 6–7 years after diagnosis in Argentina, 4–5 years in Mexico and Chile, and 3 years in Brazil. Among Argentine patients 39.2% survived to 10 years of age, and 18% to 15 years. These findings suggest that CF is not rare in LA but extensive epidemiological studies are needed to obtain accurate figures on the magnitude of the problem. Pediatr Pulmonol 1991; 10:249–253.