The prevalence, clinical status and genotype of cystic fibrosis patients living in Cuba using national registry data

Abstract

BackgroundWe aimed to establish a national cystic fibrosis (CF) registry for Cuba, a developing country. MethodsRegional centres that deliver care for all CF patients provided information for a national database. FindingsThe prevalence of CF in Cuba is 26.3 cases per 1,000,000 population. The median age at diagnosis is 2 years, and the median age of the total population was 15 years. Of those aged 16 years or older, the prevalence of Pseudomonas aeruginosa infection was 46%, the prevalence of Staphylococcus aureus infection was 36%, and 80% of individuals were receiving oral azithromycin. The commonest gene mutation was F508del which was observed in 50% of patients. InterpretationThese data demonstrate that it is possible to establish a national CF registry in a developing country such as Cuba. This provides baseline data to permit evaluation of health care delivery enable the spread of good clinical practice nationally.