Abstract

CF lung disease frequently has intermittent episodes of acute worsening of symptoms (PEx) responsible for much of the morbidity and mortality. Objective To determine frequency and associated risk factors of PEx and to report the proportion of patients (pt) who do not recover the pulmonary function (PF). Material and Methods Observational, cohort, retrospective study. Medical records of CFpt treated in 2013 were reviewed and divided in two groups: G1 (requiring intravenous antibiotic treatment) and G2 (not exacerbated). Current age, gender, p.F508 del, baseline FEV1% and BMI Z score, % of P. aeruginosa (PA), MRSA and BCC chronic colonization, % of CFRD, FEV1% at the end of the PEx and % pt who recovered the baseline FEV1 were recorded. The relative risk (RR) of the associations between the incidence of PEx and risk factors was estimated. Results 117 pt (54% male) were included, 50 pt in G1 and 67 pt in G2. Statistically significant differences between both groups were: age (10 vs 7.7 years, p=0.03), BMI z score (–0.65 vs –0.049, p=0.007), p.F508 del (93% vs 71%, p=0.007), FEV1% (102% vs 68.5%, p=0.000), CFRD (20% vs 0%, p=0.000) and BCC chronic colonization (10% vs 0%, p=0.000), PA (40% vs 11%, p=0.001), MRSA (40% vs 13%, p=0.002). The characteristics associated with the incidence of PEx were BMI Z score (RR 0.69, p=0.002), p.F508 del (RR 3.23, p=0.05) and BCC chronic colonization (RR 3.69, p=0.002), PA (RR 1.89, p=0.01) and MRSA (RR 2.32, p=0.002). 23% of pt did not recover their previous lung function. Conclusions Worst nutritional status, p.F508 del, chronic BCC, PA and MRSA colonization were risk factors for PEx. A quarter of the patients did not recover their baseline PF.

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