Daniela Savi

Bone marrow-derived progenitors are greatly reduced in patients with severe COPD and low-BMI

Chronic obstructive pulmonary disease (COPD) patients have reduced circulating hemopoietic progenitors. We hypothesized that severity of COPD parallels the decrease in progenitors and that the reduction in body mass index (BMI) could be associated with more severe bone marrow dysfunction. We studied 39 patients with moderate to very severe COPD (18 with low-BMI and 21 with normal-BMI) and 12 controls. Disease severity was associated to a greater reduction in circulating progenitors. Proangiogenetic and inflammatory markers correlated with disease severity parameters. Compared to normal-BMI patients, low-BMI patients showed: greater reduction in circulating progenitors; higher VEGF-A, VEGF-C, HGF, Ang-2, TNF-alpha, IL-6 and MCP-1 levels. Furthermore, among patients with similar pulmonary impairment, those who displayed low-BMI had a more markedly reduced number of CD34(+) cells and late endothelial progenitors. We show that the reduction in hematopoietic and endothelial progenitor cells correlates with COPD severity. Our findings also indicate that, in severe low-BMI COPD patients, bone marrow function seems to be further impaired and may lead to reduced reparative capacity.

Relationship between daily physical activity and aerobic fitness in adults with cystic fibrosis.

BackgroundThe best clinical practice to investigate aerobic fitness includes measurements obtained during cardiopulmonary exercise testing (CPET), however it remains an underutilised clinical measure in cystic fibrosis (CF). To investigate this further, different methods of quantifying exercise capacity in CF are required. The possibility that measuring physical activity (PA) by a portable accelerometer could be used to assess the CF aerobic state and could be added among the CPET surrogates has not been investigated. The aim of this study was to examine the relationship between PA and exercise fitness both at submaximal and maximal levels in clinically stable adults with CF.MethodsThirty CF patients (FEV1 71 ± 19% predicted) and fifteen healthy controls undertook an incremental CPET on a cycle ergometer. CPET-related measurements included: oxygen uptake (V’O2), carbon dioxide production (V’CO2), ventilatory profile, heart rate (HR) and oxygen pulse (V’O2/HR) throughout exercise and at lactic threshold (LT) and peak. LT measures represent submaximal exercise related data. PA was assessed using the accelerometer SenseWear Pro3 Armband.ResultsModerate (>4.8 metabolic equivalents (METS)) and moderate + vigorous (>7.2 METS) PA was related to V’O2 (p = 0.005 and p = 0.009, respectively) and work rate (p = 0.004 and p = 0.002, respectively) at LT. Moderate PA or greater was positively related to peak V’O2 (p = 0.005 and p = 0.003, respectively). Daily PA levels were similar in CF and healthy controls. Except for peak values, V’O2 profile and the V’O2 at LT were comparable between CF and healthy controls.ConclusionsIn adult CF patients daily PA positively correlated with aerobic capacity. PA measurements are a valuable tool in the assessment of exercise performance in an adult CF population and could be used for interventional exercise trials to optimize exercise performance and health status. PA levels and parameters obtained at submaximal exercise are similar in CF and in healthy controls.

Physical activity patterns and clusters in 1001 patients with COPD

We described physical activity measures and hourly patterns in patients with chronic obstructive pulmonary disease (COPD) after stratification for generic and COPD-specific characteristics and, based on multiple physical activity measures, we identified clusters of patients. In total, 1001 patients with COPD (65% men; age, 67 years; forced expiratory volume in the first second [FEV1], 49% predicted) were studied cross-sectionally. Demographics, anthropometrics, lung function and clinical data were assessed. Daily physical activity measures and hourly patterns were analysed based on data from a multisensor armband. Principal component analysis (PCA) and cluster analysis were applied to physical activity measures to identify clusters. Age, body mass index (BMI), dyspnoea grade and ADO index (including age, dyspnoea and airflow obstruction) were associated with physical activity measures and hourly patterns. Five clusters were identified based on three PCA components, which accounted for 60% of variance of the data. Importantly, couch potatoes (i.e. the most inactive cluster) were characterised by higher BMI, lower FEV1, worse dyspnoea and higher ADO index compared to other clusters (p < 0.05 for all). Daily physical activity measures and hourly patterns are heterogeneous in COPD. Clusters of patients were identified solely based on physical activity data. These findings may be useful to develop interventions aiming to promote physical activity in COPD.

WS04.2 Growth hormone deficiency (GHD) in adult patients (pts) with cystic fibrosis (CF)

Introduction CF adult pts complain signs and symptoms that overlap with the GHD: loss of muscle mass, physical function, occurrence of bone fragility, cardiovascular complications and lower stress tolerance. Aim To assess the prevalence of GHD in a cohort of CF adults. Methods 41 pts (30M) with a mean age of 36.9±10.7 yrs were enrolled. Exclusion criteria: liver or lung transplantation, age Results Enrolled subjects had the following characteristics: BMI:21.7±2.8 kg/m 2 FEV1: 61.9±18.3%, FEF25–75 39±23.7%, CVF 78.7±19.5%. All pts with GHD have a severe genotype: have both alleles with mutations of the first 3 classes. Evaluation of the GH-IGF1 axis revealed 34.2% with a GHD: severe in 12.2% and partial in 22%. In severe GHD, mean GH peak was 9.2±1.3 ng/ml and mean IGF1 185.4±51.5 ng/ml, in partial GHD mean GH peak 13.2±1.5, mean IGF1 208±39.7, and in pts with normal GH response, mean GH peak was 51±38.6, mean IGF1 210.6±77.5. All pts with severe GHD showed a ΔF508 mutation (60% in homozygous). We found a significant difference in GHD pts than no GHD: in BMI (23.4±2.4 vs 20.9±2.7) and fasting glucose (115.9±29.1 vs 87.7±13.7) and that these variables are inversely correlated with the GH peak (BMI: R=−0.33, p = 0.04; fasting glucose: R=−0.31, p = 0.05). Discussion To our knowledge there are no studies on the GHD in adults pts CF. You have to follow these pts over time to assess the impact of GHD on general health; should be increased cases in the study to see if this data is confirmed.

WS9.5 The role of daily physical activity on exercise performance in adults with cystic fibrosis

WS9.5 The role of daily physical activity on exercise performance in adults with cystic fibrosis D. Savi1, M. Di Paolo2, N.J. Simmonds3, T. Perelli1, M. Varchetta1, S. Bertasi1, G. Cimino1, P. Troiani1, V. D’Alù1, S. Quattrucci1, S. Cucchiara1, P. Palange2. 1Sapienza University of Rome, Department of Pediatrics and Pediatric Neurology, Cystic Fibrosis Center, Rome, Italy; 2Sapienza University of Rome, Department of Public Health and Infectious Diseases, Rome, Italy; 3Royal Brompton Hospital and Imperial College, Department of Cystic Fibrosis, London, United Kingdom

234 Cystic fibrosis: new trends in ophthalmological evaluation

Objective: Advances in cystic fibrosis (CF) management over the past 20 years have lead to an increase in life expectancy such that CF is emerging as a chronic disease requiring complex treatment strategies. In this study, we investigated the relationship between daily treatment activities and perceived treatment burden in CF adults from a single UK centre. Methods: A questionnaire was designed using the CF Questionnaire-Revised (a CF-specific measure of healthcare-related quality of life) to measure self-reported daily treatment activities and percentage perceived treatment burden score (PPTBS), with higher scores representing greater perceived treatment burden. Results: Among the twenty respondents, the mean reported time to complete daily therapies was 130 minutes (range 0–480 minutes) and the median total number of daily medications was 5 (range 1−8). The mean PPTBS was 60% (SD 16%), with no significant difference in PPTBS based on gender or age. In a multivariable model, PPTBS trended towards a negative association with FEV1 (p = 0.08) however was not significantly influenced by the total time spent on treatments or number of daily medications (total, nebulised or oral). Conclusion: Daily treatment activities exhibit a broad range which may reflect variability in disease severity and/or treatment adherence in the respondent group. Interestingly, perceived treatment burden did not appear to associate with the total time spent on daily treatments. The observed trend towards a negative association with FEV1 suggests that factors other than the time burden of treatment regimen act to increase PPTBS in CF adults with more severe lung disease.