Descriptive analysis of cystic fibrosis patients followed by the Pediatric Pulmonology Unit of a university hospital in Porto Alegre-RS <b>[Abstract in English]</b>

Abstract

Aims: To provide a descriptive analysis of patients followed in a local cystic fibrosis treatment center and compare the collected data with those found in the literature.
Methods: Relevant data for clinical follow-up of cystic fibrosis patients were used for analysis and correlation.
Results: Twenty six male and twenty two female patients were studied. From 48 patients, 89,6% presented with pancreatic insufficiency and 29,1% had at least one positive culture to Pseudomonas aeruginosa in the last 6 months. The patients colonized by P. aeruginosa and the ones with pancreatic insufficiency presented with worse values for forced expiratory volume in one second (FEV1). The pancreatic insufficiency group (84 versus 112%; p = 0,005) and the patients colonized by P. aeruginosa (75 versus 85%; p = 0,006) had lower pulmonary function parameters.
Conclusions: The data from our study show that this group of patients presents in accordance with data available from literature, considering the analysed features of the disease. However, there is a marked exception in the time of diagnosis, which is still very late in Brazil.
KEY WORDS: CYSTIC FIBROSIS/diagnosis; CYSTIC FIBROSIS/dietoterapia; CHILD; INFANT; ADOLESCENT; SPIROMETRY; PSEUDOMONAS.