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

Rodr�guez-Cal�, Fidel; Su�rez-Medina, Ram�n; Josefina Venero-Fern�ndez, Silvia; Smyth, Alan; Carr, Siobh�n B.; Fogarty, Andrew W.

Abstract

Background: We aimed to establish a national cystic fibrosis (CF) registry for Cuba, a developing country.
Methods: Regional centres that deliver care for all CF patients provided information for a national database.
Findings: The 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.
Interpretation: These 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.

Citation

Rodríguez-Calá, F., Suárez-Medina, R., Josefina Venero-Fernández, S., Smyth, A., Carr, S. B., & Fogarty, A. W. (2019). The prevalence, clinical status and genotype of cystic fibrosis patients living in Cuba using national registry data. Journal of Cystic Fibrosis, 18(4), 522-524. https://doi.org/10.1016/j.jcf.2018.10.007

• Journal Article Type: Article
• Acceptance Date: Oct 12, 2018
• Online Publication Date: Oct 23, 2018
• Publication Date: 2019-07
• Deposit Date: Oct 18, 2018
• Publicly Available Date: Oct 24, 2019
• Journal: Journal of Cystic Fibrosis
• Print ISSN: 1569-1993
• Electronic ISSN: 1873-5010
• Publisher: Elsevier
• Peer Reviewed: Peer Reviewed
• Volume: 18
• Issue: 4
• Pages: 522-524
• DOI: https://doi.org/10.1016/j.jcf.2018.10.007
• Keywords: cystic fibrosis, Cuba, prevalence
• Public URL: https://nottingham-repository.worktribe.com/output/1174294
• Publisher URL: https://www.sciencedirect.com/science/article/pii/S1569199318308592
• Contract Date: Oct 18, 2018