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Epidemiology of cystic fibrosis in Latin America: preliminary communication
C N Macri1, A S de Gentile, A Manterola
1Department of Pediatrics, Hospital de Niños Ricardo Gutierrez, Buenos Aires, Argentina.
Insights
Cystic Fibrosis (CF) affects many in Latin America, with survival rates varying by country. More research is needed to understand the full scope of CF in the region.
Area of Science:
- Medical Research
- Pediatrics
- Pulmonology
Background:
- Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
- Limited epidemiological data exists for CF in Latin America (LA).
Purpose of the Study:
- To evaluate the clinical features and survival of CF patients in LA.
- To provide insights into the prevalence and characteristics of CF in the region.
Main Methods:
- Retrospective study of 743 CF patients from 1979-1989 across four LA institutions.
- Diagnosis confirmed via sweat test.
- Analysis of patient demographics, age at diagnosis, and survival rates.
Main Results:
- A slight male predominance (61.0%) was observed, particularly in Brazil.
- Mean age at diagnosis exceeded 3 years.
- Survival rates varied significantly, with Chile showing the highest proportion of living patients (71.4%) and Argentina the lowest (33.8%).
- Median survival post-diagnosis was 3 years in Brazil, 4-5 years in Mexico and Chile, and 6-7 years in Argentina.
- Argentinian patients had longer survival curves, with 18% reaching 15 years of age.
Conclusions:
- CF is not rare in Latin America.
- Significant regional variations in CF clinical features and survival exist.
- Extensive epidemiological studies are crucial for accurate CF prevalence data in LA.
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.