نبذة مختصرة : Trabalho Final do Mestrado Integrado em Medicina apresentado à Faculdade de Medicina ; Introduction: Cystic Fibrosis is a recessive autossomic disease. The most frequent related mutation is F508del. In Portugal there are an estimated 338 patients. The average age of adult patients in the outpatient clinic is 31.23 years old. The lung is the main organ that conditions morbidity and mortality. Its gradual function loss is aggravated by multiple factors such as colonizing agents, frequent exacerbations, other organs involvement and Diabetes mellitus. The aim of this study is to correlate the cycles of antibiotic therapy with current microbiology status and lung function. In addition, a demographic characterization of the adult patient population of the Centre of Portugal was carried out. Methods: In this five-year retrospective study we included adult patients from the CF outpatient clinic of Centro Hospitalar e Universitário de Coimbra. Every patient was over 18 years old and transplanted ones were excluded. Demographic data, mutational profile, best annual lung function measurement and its corresponding weight, annual cumulative microbiology, number of intravenous and oral antibiotic cycles and associated comorbidities of every patient were assessed. BMI was also calculated. The statistical analysis included quantitative variable normality tests and correlation tests between LF, microbiology and number of antibiotic cycles. Results: 55.2% of the 29 registered patients were female, median age was 31.6±11.8 years and median height was 1.64 m. 86.20% of the 29 patients had at least one F508del mutation and 37.93% were F508del homozygotes. The second most common mutation was R3334W. 7% of the patients were underweight, 66% were normal weight, 21% were over-weight and 7% were obese. The most common microbiological agent found was Staphylococcus aureus. The highest number of antibiotic oral and intravenous cycles was 7. 75.9% of the patients had pancreatic insufficiency and 13.8% had Diabetes mellitus. A positive ...
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