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Revista Cubana de Pediatría

versão On-line ISSN 1561-3119

Resumo

VAZQUEZ GUTIERREZ, Giselle Lucila et al. Survival in Patients with Cystic Fibrosis. Rev Cubana Pediatr [online]. 2023, vol.95  Epub 24-Abr-2023. ISSN 1561-3119.

Introduction:

Cystic fibrosis is no longer a pediatric disease but a chronic adult disease. There are multiple factors that condition the survival of these patients.

Objective:

To determine the survival of pediatric patients with cystic fibrosis hospitalized in a specialized center.

Methods:

Retrospective cohort study whose universe consisted of 27 patients included in the Granma provincial cystic fibrosis registry, 2003-2018. Variables were analyzed: current age and gender, genetic variant, presence of germs, clinical forms and survival after diagnosis and treatment. The Kaplan-Meier method and Log-Rank Test were used, for a significance level of 95%.

Results:

40.7% of patients are currently over 15 years of age, male sex predominates (63%) and the F508del mutation in its heterozygous genetic variant (44.4%). The average survival is 21.4 years, there were no significant differences according to gender, clinical forms and types of germs.

Conclusions:

The average survival rate of patients with cystic fibrosis in Granma, Cuba, is 21.4 years, lower than that documented in developed countries. The Cuban State guarantees resources for the care of these patients, despite the country's economic difficulties.

Palavras-chave : cystic fibrosis; genetic diseases; survival analysis; survival; mean survival time; mutation.

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