UNDER-RECOGNIZED PROBLEM IN CYSTIC FIBROSIS (CF): URINARY INCONTINENCE (UI)

2014 Cystic Fibrosis Conference kg/m 2 ). Following discussion and demonstration of the knack technique, 58% of the patients reported an improvement of their urinary incontinence symptoms. However, 17% also admitted that they were nonadherent with using the technique. (Three have not returned to the clinic to assess their response to the technique.) When compared to the control group of 13 without UI, there was no difference in age, lung function, and frequency of hospitalizations (Table). Conclusions: About half of the CF subjects screened suffered from stress UI and 75% of these experienced symptoms every day. There does not seem to be a correlation with FEV1%, CF-related diabetes, BMI, hospi- talizations, genotype, and the presence of significant microbial pathogens. The knack technique is an appropriate and effective intervention for the treatment of stress urinary incontinence with females with cystic fibrosis as young as 11 years old. Clinical Implications: Further studies are needed to determine the etiology of UI in the CF population. It does not appear related to the sever- ity of lung disease nor with the BMI. Frequent screening and education for patients who suffer from urinary incontinence can reduce symptoms and improve quality of life. Pelvic floor exercise and techniques should be incorporated into routine CF care. Conclusion: Children and adolescents with CF present changes in inspiratory muscle endurance that are distinct at different levels of pulmo- nary impairment. Furthermore, the significant endurance associations are better related to airway resistance. IDENTIFICATION OF SUBCLINICAL CARDIAC DYSFUNCTION IN CYSTIC FIBROSIS ADULTS Sellers, , Z. 1 ; McGlocklin, L. 2 ; Brasch, A. 2 1. Pediatrics, Stanford University, Palo Alto, CA, USA; 2. Heart and Cardiovascular Center, Carle Foundation Hospital, Urbana, IL, USA Background and Aims: Cystic fibrosis (CF) is known to cause right ventricular (RV) dysfunction secondary to chronic obstructive lung disease, however, whether CF individuals are at risk for primary left ventricular (LV) dysfunction remains unknown. CFTR is expressed in cardiac myo- cytes, and studies by Sellers et al. in mice show that lack of CFTR alters cardiomyocyte contraction, Ca 2+ signaling, and decreases in vivo cardi- ac reserve. Prior echocardiographic studies in CF have been difficult to interpret due to confounding comorbidities (i.e. poor lung function, RV V abnormalities) and inherent limitations of conventional echocardiography due to tethering from neighboring segments. To determine if CF individuals display primary LV dysfunction, we undertook a pilot study using strain and strain rate echocardiography, an imaging tool less influenced by neigh- boring segments, to examine LV function in CF adults. Methods: Transthoracic echocardiography with conventional tissue Doppler, along with strain and strain rate imaging were performed in 8 adults with CF. Additionally, brain natriuretic peptide (BNP) levels were measured. Results were compared to literature values obtained from healthy control subjects. To determine if there was any positive correlation between low strain and strain rate values and poor lung function, we calculated Pear- son correlation coefficients for strain and strain rate vs. FEV1. Results: Conventional echocardiography showed normal systolic func- tion with LV ejection fraction of 61(4% (normal *55%) and BNP was normal in all individuals with a mean of 20(11 pg/mL (normal 0.05). Conclusions: In this pilot study, we show that strain and strain rate echocardiography can identify LV systolic abnormalities in CF individuals not detected by conventional echocardiography. We propose that this echo- cardiography modality may identify subclinical cardiac dysfunction in CF and allows for evaluation of LV function independent of lung function. This study, coupled with prior animal studies, provides evidence for primary cardiac abnormalities in CF, independent of lung disease. Early diagnosis of f cardiac dysfunction may result in better outcomes for individuals with CF. Table: Numbers given are mean (standard deviation) COMPARISON OF INSPIRATORY MUSCLE ENDURANCE BETWEEN CHILDREN AND ADOLESCENTS WITH CYSTIC FIBROSIS AND HEALTHY INDIVIDUALS Vendruculo, F.M. 1 ; Heinzmann-Filho, J.P. 1 ; Piva, T.C. 1 ; Marostica, P.J. 2 ; Donadio, , M.V. 1 1. Centro Infant - IPB, Pontificia P ontificia Universidade Catolica do Rio Grande do Sul - PUCRS, Porto P orto Alegre, Brazil; 2. Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil Introduction: Mechanisms of pulmonary changes that occur in cystic fibrosis (CF) may influence the inspiratory muscle endurance (IME) in CF patients. However, several studies were conducted to evaluate inspiratory muscle strength and less information is available about endurance levels in these patients. Objective: To evaluate inspiratory muscle endurance in children and adolescents with CF in comparison with healthy subjects and correlate with pulmonary function tests. Methods: This cross-sectional observational study evaluated CF patients (6 to 18 years old) and healthy subjects paired in a 1:2 proportion, using sex, age, height and weight. Spirometry, impulse oscillometry, pleth- ysmography, manovacuometry and a protocol of IME were performed. To determine IME a commercially-available threshold-loading device was used during an incremental loading procedure. In order to obtain pressures over 41 cmH 2 0 the spring p g was modified. Patients started inspiring p g from a (MIP) threshold-loading g device set at 30% of maximal inspiratory p y pressure p for 2 minutes. The threshold load was then increased every 2 minutes in increments of 10% of MIP. IME was defined by the maximal inspirato- ry pressure sustained for at least 1 minute and results were expressed in percentage of MIP. Data were analyzed using Student’s t-test and Pearson correlation coefficient. Results: Thirty-four patients with CF were included in the study with a mean age of 12.6(2.9 years and twenty of them were boys (58.8%). The mean IME was 60.9(13.3. Sixty-eight healthy subjects were included and they presented a mean endurance of 65.3(12.3. No significant difference in the IME between CF patients and healthy subjects was found. When restricting the analysis to CF patients without colonization by Pseudomonas aeruginosa (PA) or with forced expiratory volume in one second (FEV 1 ) greater than 80% of predicted values, IME were significantly lower in com- parison to the control group (p=0.02 and p=0.05, respectively). There was no significant differences in the endurance of patients colonized by PA or with FEV 1 <80% when compared to their healthy controls. Endurance cor- related better with both total airway resistance (r=0.35, p=0.04) and central airway resistance (r=0.48, p=0.04). THE RELATIONSHIP BETWEEN EXERCISE CAPACITY AND GLUCOSE TOLERANCE IN A PEDIATRIC CF POPULATION NOT DIAGNOSED WITH CYSTIC FIBROSIS-RELATED DIABETES Foster, K.E.; McPhail, G.L.; Crisalli, J.; Chini, B.A. Pulmonary Medicine, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, USA Background: Reduced exercise capacity (EC) and cystic fibrosis-re- lated diabetes mellitus (CFRD) have been associated with a greater decline in lung function, nutritional status, and life expectancy in people with CF. Some research has shown that patients with CFRD have reduced EC, but to our knowledge no information is available on the relationship between EC and glucose tolerance in patients without CFRD. Methods: In a retrospective study, we evaluated 70 maximal cardio- pulmonary exercise tests (CPET) of 50 patients not diagnosed with CFRD.