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

Valentina C. B. Gava Chakr, Fernanda Maria Vendrusculo, Gabriela Tomedi Leites, Márcio Vinícius Fagundes Donadio, Taísa Frescura Paim, Paulo José Cauduro Marostica, Mara Rúbia Silveira

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.



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