INTRODUCTION: Cystic Fibrosis is usually diagnosed in childhood. In Brazil, few studies have approached CF diagnosed in adulthood. OBJECTIVE: The aim of this study was to describe demographic and clinical characteristics and spirometric data of patients with CF diagnosed in adulthood (over 16 years of age) in Bahia, Brazil. METHODS: Twenty-eight patients with cystic fibrosis diagnosed in adulthood were evaluated at the Reference Center of Cystic Fibrosis of the state of Bahia. Variables of interest were age, gender, race, body mass index (BMI), sputum culture, presence of sinusitis and bronchiectasis, spirometric tests with the best values performed for forced expiratory volume in the first second (% FEV1), forced vital capacity (FVC) and response to bronchodilators. RESULTS: Mean age of diagnosis was 31.1 ± 12.4 years. Of the patients, 53.4% were black and mulattos and the mean BMI was 18.7 ± 3.0 kg/m2. P. aeruginosa was present in 12 (43%) of the total patients. The mean percentages ± SD of FVC and FEV1 were 58.9 ± 21.6% and 44.1 ± 23%, respectively. In the group of patients with presence of P. aeruginosa in the sputum cultures mean of the spirometry parameters were lower than those of the P. aeruginosa-free group. However, this difference achieved a statistically significant difference (p = 0.0007) only in FVC. CONCLUSIONS: In agreement with many authors, this study strengthen the point of view that CF must be investigated in patients with recurrent infections, sinusitis and bronchiectasis even in adulthood. Values of the percentages of FVC and FEV1 in relation to those foreseen were lower in patients bearers of P.aeruginosa thereby showing a higher deterioration of the pulmonary function.
Cystic fibrosis; Adult; Spirometry
