Introduction:

Patients with cystic fibrosis have had a greater survival and life quality in the last decades; this is due to a precocious diagnostic, a better nutrition state, and diminution of respiratory infections.

Objective:

To describe the characteristics of cystic fibrosis patients and follow up process to survivors for the last 40 years.

Methods:

Descriptive, longitudinal and retrospective study in 96 patients diagnosed with cystic fibrosis in “William Soler” Pediatric University Hospital (1977-2017). Data was obtained from clinical records. Demographic, temporary, clinical, genetic and microbiological variables were analized.

Results:

From the sample, 27 patients keep under medical follow up; 9 patients older than 19 years old were remitted to the Neumology consultation; 30 died; and 30 patients are not followed up at the hospital. 60.4 % of the patients was diagnosed before the first year of life, t was predominant the male sex (62.5 %) and most of them were white skin (88.5 %). Diagnosis suggested; the respiratory clinical symptoms, 39.6 %; mixed, 38.5 %; digestive symptoms, 19.8 %; salt lost, 2.1 %. Out of the 27 cases followed at present, 74.1 % are eutrophics, and there are not patients with malnutrition. In the first microbiological isolation, Pseudomonas aeruginosa prevailed in patients under 1-year-old and Staphilococcus aureus in patients between 1 and 4 years old. More than 37.0 % of patients had respiratory complications, 14.8 % had hepatic complications, and 40.7 % had no complications. The genetic characterization was mostly of ∆F508 (59.2 %).

Conclusions:

Pseudomonas aeruginosa is frequently in patients under 1-year-old. Almost half of the patients has no complications; and it is improved their nutritional state and mortality decreased.

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