V. Rodriguez

189 Airway clearance techniques currently used in a cystic fibrosis center in Argentina

Introduction: Nowadays modern physiotherapy has proven to be an effective approach to Cystic Fibrosis treatment although many patients still had a tendency to continue with conventional therapies. Objective: To describe which method of the Airway Clearance Techniques (ACT) patients perform and to know if they had incorporated exercise as a part of their treatment. Methods: Cross sectional, observational study. Data of physiotherapy techniques (ACT, exercise and Inhaled Therapy) were collected. Results: There were 110 patients attended in our clinic. From these, 101 patients (92%) perform some kind of ACT. 30 (27.2%) perform MPD /C (Modified Postural Drainage and Chest Compression), 29 (26.3%) prefer PD/Pc (postural drainage and percussion), 36 (32.7%) ACBT (active cycle of breathing technique) and 12 (10.9%) PEP (Positive Expiratory Pressure) and Osc. PEP (Oscillating Positive Expiratory Pressure) with other techniques. Only 39 patients (35.4%) were daily assisted by a physiotherapist. 88 patients were able to perform exercise according to their age, although 70 (79.5%) usually do. Related to inhaled therapy, 84 of 110 patients (76.3%) use DNase and 42 (38.1%) Hypertonic Saline. Conclusions: Although many patients still prefer PD/ Pc, a considerable number of them had incorporated modern physiotherapy. A great number of patients had understood the importance of an active life and they perform some type of sport or exercise according to the child’s age and abilities.

187 Risk factors for pulmonary exacerbations (PEx) in pediatric cystic fibrosis (CF) patients

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.

184 Is chronic infection with methicillin resistant Staphylococcus aureus (MRSA) worse than methicillin sensitive Staphylococcus aureus (MSSA) in CF patients

Objectives: Literature reviews have identified a lack of consensus about the definition of an exacerbation in CF. We sought to determine which exacerbation definitions are most commonly used in CF clinical trials, and which signs/symptoms are most commonly included in exacerbation definitions across all CF clinical trials. Method: Eligible randomised trials were those in people with CF aged over 6 years, where a sign/symptom-based definition was used to identify exacerbations as an outcome. Eligible trials were identified from reference lists of Cochrane Reviews in CF. Identified trials underwent citation tracking for further eligible trials. The number of times a definition was used across all trials was tallied. The number of times each symptom was included in any trial’s definition was also tallied. Results: The searches identified 36 trials that had clearly specified the exacerbation definition used. The most commonly used definitions were from the DNase trial by Fuchs (1994), used in 11 trials; the US CFF Conference (1994), used in 6 trials; and the EPIC study (2009), used in 3 trials. The symptoms most commonly used among all trials were: sputum (used in 36 trials), cough (35), spirometry (34), weight/appetite (33), chest sounds (29), CXR changes (29), fever (27) and dyspnoea (24). The remaining 13 signs/symptoms were each used in 18 or fewer trials. Conclusion: Researchers may use these results to guide selection of a definition for their research. Clinicians may use these results to consider whether the definition used in a particular trial includes signs and symptoms that are generally accepted as representing an exacerbation by most clinical trialists in CF.

195 Pulmonary function in cystic fibrosis children younger than 3 years old

Studies suggest that the decline in pulmonary function begins early in CF infants even in those patients without respiratory symptoms. Objective: to describe pulmonary function in CF children younger than 3 years old and to determine if there is any association between pulmonary function and clinical features. Methods: observational and retrospective, study. We collected data from CF patients who underwent an Infant Pulmonary Function Test (IPFT) before the age of three years. IPFTs were assessed by partial flow/volume curves with the rapid thoracicabdominal compression technique to obtain the maximal flow at the functional residual capacity (V′maxFRC). Clinical issues recorded were: age, gender, genetics, pancreatic sufficiency, nutritional status and microbiological findings. Results: 46 patients (31 males) with a median (interquartile range) age of 8 months (5 to 15) were included. Overall V′maxFRC Z score was −0.8 (−1.4 to 0.3), −0.1 (−1.1 to 0.7) in males and −0.9 (−1.8 to 0.3) in females (p =NS). V′maxFRC Z score was −0.3 (−1.1 to 0.2) in children younger than 6 months and −0.1 (−1.3 to 1) in older ones (p =NS). In patients homozygous p.508del V′maxFRC Z score was −0.5 (−1.2 to 0.8) while in patients with other mutations it was −0.2 (−1.2 to 0.6) (p =NS). There was also no statistical difference in V′maxFRC between well-nourished and bad-nourished patients, and between patients with and without Pseudomonas aeruginosa (PA) in sputum. Conclusion: IPFT assessed by partial flow/volume curves was normal in our CF population less than 3 years old. We have not found any difference in pulmonary function comparing CF children with different clinical features.

199 Pulmonary function and clinical aspects in cystic fibrosis (CF) patients chronically colonized with Pseudomonas aeruginosa

Chronic colonization with Pseudomonas aeruginosa (PA) in CF patients shows more rapidly decline in pulmonary function (PF). Objective: To compare the PF and clinical characteristics of CF patients according to the colonization with PA. Methods: Observational cross-sectional study. We collected data from CF patients who were performed at least four sputum cultures from September 2009 to September 2010. We divided them into four groups according to Leeds Criteria: Chronic infection, Intermittent colonization, Free and Never. We considered: age, genetics, pulmonary function, nutritional status, intravenous (IV) antibiotics and PA antibodies. Results: We evaluated 97 CF patients: 26% Chronic, 25% Intermittent, 27% Free and 19 % Never. The current age (median and interquartile range) was 10.7 (8.5 to 16.2), 5.8 (2.78 to 7.67), 5.5 (4.7 to 8.9) and 4.2 (0.97 to 5.3), respectively (p< 0.001). The homozygous p.508 del was present in 34%, 56%, 29%, and 31% of patients, respectively (p =NS). PA antibodies was 7.6 (4.5 to 16.5), 1.6 (1 to 1.8), 1.5 (1to 2.6) and 0.8 (0.6 to 1), respectively (p< 0.001). The Zscore BMI was −0.39 (−1.14 to 0.13), −0.1 (−0.81 to 0.31), 0.19 (−0.28 to 0.83) and 0.11 (−0.94 to 0.37) in each group (p =NS). The FEV1 was 63 (47.5 to 92.2), 79 (54.7 to 108), 99 (85 to 105) and 109 (105.7 to 112), respectively (p = 0.06). (Chronic vs. Free p = 0.007 and Chronic vs. Never p< 0.001). Iv Antibiotic treatments were required in 1 (0−1), 1 (1−2), 0 (0−1) and 0 (0−0), respectively (p< 0.01). Conclusion: Chronic infection with PA determined lower pulmonary function, more iv antibiotics treatments and had higher level of antibodies than the others groups.