Summary Objective: to compare the characteristics of cystic fibrosis patients treated in two reference centers in southern Brazil in order to observe trends in the treatment and clinical outcomes that may produce changes in clinical conduct. Methods: cross-sectional, retrospective study with 83 patients diagnosed with cystic fibrosis, aged one month to eighteen years. The variables analyzed were obtained through review of medical records, including: demographic and clinical characteristics, socioeconomic status, pulmonary function test, bacterial colonization profile, medication and physiotherapy. Results: between the two centers there was significant difference in the variables, including admissions in the previous year (p<0.001), lifetime hospital admissions (p<0.001), use of Dornase alpha (p=0.003) and inhaled antibiotic therapy (p=0.006), which were higher at the Santo Antônio Children’s Hospital (HCSA), while age at first colonization with Staphylococcus aureus (p=0.008), maternal age (p=0.030), clinical score (p=0.001), socioeconomic score (p=0.021) and use of hypertonic saline (p<0.001) were lower at HCSA compared to São Lucas Hospital (HSL). Conclusion: the study centers seem to receive a different population of patients, both in socioeconomic terms, as well as disease severity, which interferes with the choice of medication treatment. At the HCSA, preventive actions against infection due to the high incidence of Burkholderia cepacia Complex and careful research into early lung changes will be encouraged.
Frequency of CFTR variants in southern Brazil and indication for modulators therapy in patients with cystic fibrosis
