Introduction:

Cystic fibrosis is a disease that affects multiple body systems in both children and adults. It is the most common limiting genetic disorder in Caucasians.

Objective:

To characterize patients with cystic fibrosis (CF) hospitalized in the Pediatric Service of a Level IV Social Security Hospital in Paraguay during 2016-2019.

Materials and Methods:

this was an observational, descriptive and cross-sectional study, with non-probabilistic sampling, in children who presented to the Hospital between 2016 and 2019. The medical records of admitted patients who met the inclusion criteria were used.

Results:

52 patients were included, 32/52 (61.54%) were female. The median age was 3 years (1 month to 16 years). Of the total, 37/52 (71.2%) patients did not have a family history of CF and the most frequent time of diagnosis was between 0 and 8 months of age. The predominant clinical manifestation at the time of diagnosis was mixed (respiratory/digestive) in 38/52 patients (73.1%) and the most commonly isolated pathogen from tracheal secretions was Pseudomonas aeruginosa in 33 (63.5%) of them. they. 18 (34.6%) patients were at risk of malnutrition.

Conclusion:

a predominance of the female sex was observed, the predominant clinical form of debut was mixed, respiratory and digestive. One third of the patients were at risk of malnutrition.

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