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Abstract

Background Exercise and physical activity (PA) are essential components of the care of cystic fibrosis (CF) patients. Lower PA levels have been associated with worse pulmonary function, aerobic fitness, glycemic control, and bone mineral density. Most people with CF do not engage in the recommended amounts of PA. Objective To determine the level of PA in children and adolescents with CF. Methods A systematic review with meta‐analysis was conducted without language restrictions in five databases. Were included studies that analyzed PA measured by objective and subjective instruments in children and adolescents with CF. Two independent reviewers analyzed the studies, extracted the data, and assessed the quality of evidence. The risk of bias of the included studies was assessed with the National Heart, Lung, and Blood Institute's risk‐of‐bias tool. Results Of the 1,535 reports returned by the initial search, 20 articles reporting on 785 patients were included in the data synthesis. The forest plot showed that the CF group had a similar moderate‐to‐vigorous PA (MVPA) (mean difference −7.79, 95%CI −15.65 to 0.08 min/day; p = 0.05) and sedentary time (mean difference −50.81, 95%CI −109.96 to 8.35 min/day; p = 0.09) to the control group. Conclusion Children and adolescents with CF have a similar MVPA and sedentary time compared to controls. There are many options, subjective and objective, for assessing PA in this population. Optimal tool selection should guarantee more valid results.

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... Some studies detected only differences in vigorous activities, with CF children being less active than the healthy groups [30], but not in sedentary time [39]. However, as in this study, a recent systematic review and meta-analysis [40] concluded that CF children and adolescents have similar MVPA and sedentary time as controls. In fact, this study also did not find any differences between percentages of accomplishment between CF children (36.4%) and the CG (42.2%). ...
... The percentage of accomplishment in the CF group is very close to the 38.9% found in British children with CF [19], but far from the 2.1% reported in a Spanish sample [18], and from the 90% detected in another sample of British CF patients [41]. Although these studies used the same objective method of measuring PA levels (accelerometers), few studies have been conducted on this population, with small samples and high heterogeneity related to the different instruments used, to obtain properly robust information when comparing studies and samples [40,42]. In spite of the scarcity of studies, we must not lose sight of how CF young patients should engage in PA and sports as part of their therapy. ...
... In the study of Selvadurai et al. [14], only pubescent CF boys were more active than pubescent girls, but no differences by gender were found in prepubescent children. Along the same lines, Puppo et al. [40] highlighted the fact that healthy girls tend to have less PA than healthy boys, as in CF children. According to these authors, one reason could be related to the fact that parents and teachers have lower expectations of girls engaging in regular PA than boys. ...
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The purpose of this study was to examine differences in sports participation and the levels of physical activity (PA) and sedentary behaviour (SB) between schoolchildren with cystic fibrosis (CF) and a healthy control group (CG) taking into account the gender variable. PA and SB were measured with an accelerometer for 7 consecutive days in 44 children (24 girls; 11.0 (3.2) years) with CF and 45 age-, sex-, and socioeconomic status-matched controls (24 girls; 11.1 (3.0) years). CF patients and CG did not differ in moderate-to-vigorous PA (54 (31) vs. 59 (27) min/day respectively) or in SB (558 (106) vs. 553 (92) min/day respectively). There were no differences in meeting the PA guidelines between both groups (CF: 36.4% vs. CG: 42.4%). Gender analysis revealed that boys were more active and met more PA guidelines than girls regardless of the group (CF or CG), girls with CF being the least active group (only 16.7% met PA guidelines). A possible compensatory effect was found between SB and PA only in the CF sample, as for each minute/day spent in SB the odds of meeting PA guidelines decreased by 34%. These findings suggest that promoting a reduction in SB is as important as promoting PA in the CF population, especially in girls. Health caregivers, coaches, teachers, or parents could offer appealing supervised and unsupervised physical activities, foster the adoption of active lifestyles, or incorporate PA into daily routines to improve the health of CF schoolchildren.
... The outcomes of a recent patient-driven research priority partnership [10], highlighted the need for research to advance our understanding of the benefits of PA and exercise [11], and simplify treatment burden in CF [10]. Previously, the time spent being physically active [12,13], as well as the associations between PA and health [13] and the effect of PA [14] and structured exercise interventions [15] for CF, has been systematically reviewed. These reviews have concluded that individuals with CF spent a similar amount of time being physically active relative to non-CF peers [12,13], and that despite heterogeneity in study designs, interventions and outcomes, there was no evidence to actively discourage PA or exercise in CF [15]. ...
... Previously, the time spent being physically active [12,13], as well as the associations between PA and health [13] and the effect of PA [14] and structured exercise interventions [15] for CF, has been systematically reviewed. These reviews have concluded that individuals with CF spent a similar amount of time being physically active relative to non-CF peers [12,13], and that despite heterogeneity in study designs, interventions and outcomes, there was no evidence to actively discourage PA or exercise in CF [15]. ...
... Exercise is a structured subcomponent of PA conducted for the inherent health associations. Nonetheless, evidence suggests that all PA, irrespective of purpose or intensity, is associated with improved health status in CF [12][13][14][15]. Therefore, both PA and exercise must be considered when attempting to integrate activity into the daily lives of those with CF, and not solely the prescription of structured exercise per se. ...
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Background Cystic fibrosis (CF) is a genetically inherited, life-limiting condition, affecting ~90,000 people globally. Physical activity (PA) and exercise form an integral component of CF management, and have been highlighted by the CF community as an area of interest for future research. Previous reviews have solely focused on PA or structured exercise regimens independent of one another, and thus a comprehensive assessment of the physical health benefits of all PA, including exercise, interventions, is subsequently warranted. Therefore, the purpose of this review is to evaluate the effects of both PA and exercise upon outcomes of physical health and healthcare utilisation in people with CF. Methods A systematic review has been registered and reported in line with Preferred Reporting Items for Systematic Reviews and Meta-Analysis-P guidelines. This will include randomised control trials on the effects of PA and exercise, relative to usual treatment, upon people with CF. Primary outcomes will include variables associated with fitness, PA, lung health, inflammation, body composition, glycaemic control and patient-reported outcomes. Secondary outcomes will include adverse events and healthcare utilisation. Searches will be undertaken in Ovid MEDLINE, OVID EMBASE, PsychINFO, ERIC, SPORTDiscus, ASSIA, CCTR, CINHAL and Web of Science databases, and will be searched from date of inception onwards. Two reviewers will independently screen citations and abstracts, and full-texts, for inclusion and data extraction, respectively. Methodological quality will be assessed using the Cochrane Risk of Bias-2 tool. If feasible, random-effects meta-analyses will be conducted where appropriate. Additional analyses will explore potential sources of heterogeneity, such as age, sex, and disease severity. Discussion This systematic review will build on previous research, by comprehensively assessing the impact of both PA and exercise upon physical health and healthcare utilisation in people with CF. Results of this review will be utilised to inform discussions that will ultimately result in a consensus document on the impact of physical activity and exercise for people with CF. Systematic review registration PROSPERO CRD42020184411
... Although the reasons for these differences are not fully comprehended, we hypothesize that physical conditioning may play a role to explain the maintenance of VO 2 peak in patients with CF. A recent systematic review and metaanalysis concluded that CF children and adolescents have similar moderate-to-vigorous physical activity and sedentary time as healthy controls 29 . European Cystic Fibrosis Society states that physical activity and exercise must be integral to the overall physiotherapy management suggested for every individual with CF, irrespective of age and disease severity 30 . ...
Preprint
Introduction . Exercise intolerance is common in chronic respiratory diseases (CRD), but its mechanisms are still poorly understood. The aim of this study was to evaluate exercise capacity and its association with lung function, ventilatory limitation, and ventilatory efficiency in children and adolescents with cystic fibrosis (CF) and asthma when compared to healthy controls. Methods . Cross‐sectional study including patients with mild‐to‐moderate asthma, CF and healthy children and adolescents. Anthropometric data, lung function (spirometry) and exercise capacity (cardiopulmonary exercise testing) were evaluated. Primary outcomes were peak oxygen consumption (VO peak), forced expiratory volume in 1 second (FEV ), breathing reserve (BR), ventilatory equivalent for oxygen consumption (V /VO ) and for carbon dioxide production (V /VCO ), both at the ventilatory threshold (VT ) and peak exercise. Results . Mean age of 147 patients included was 11.8±3.0 years. There were differences between asthmatics and CF children when compared to their healthy peers for anthropometric and lung function measurements. Asthmatics showed lower VO peak when compared to both healthy and CF subjects, although no differences were found between healthy and CF patients. A lower BR was found when CF patients were compared to both healthy and asthmatic. Both CF and asthmatic patients presented higher values for V /VO and V /VCO at VT when compared to healthy individuals. For both V /VO and V /VCO at peak exercise CF patients presented higher values when compared to their healthy peers. Conclusion . Patients with CF achieved good exercise capacity despite low ventilatory efficiency, low BR, and reduced lung function. However, asthmatics reported reduced cardiorespiratory capacity and normal ventilatory efficiency at peak exercise. These results demonstrate differences in the mechanisms of ventilatory limitation to maximum exercise testing in children and adolescents with CRD.
... pwCF participated in lower moderate-to-vigorous PA versus controls, but this effect was only evident after puberty [123] . A recent systematic review and metaanalysis reported that children and adolescents with CF had differences in the time spent in moderate-vigorous physical activity versus age-matched controls [124] . These contrasting findings demonstrate the importance of separately evaluating the developmental and age-related changes in pwCF, and does support some earlier research that the gender gap in PA levels occurs more in girls after puberty [102] . ...
Article
Exercise intolerance is common in people with CF (pwCF), but not universal among all individuals. While associated with disease prognosis, exercise intolerance is not simply a reflection of the degree of lung disease. In people with severe CF, respiratory limitations may contribute more significantly to impaired exercise capacity than in those with mild-moderate CF. At all levels of disease severity, there are peripheral factors e.g., abnormal macro- and micro-vascular function that impair blood flow and reduce oxygen extraction, and mitochondrial defects that diminish metabolic efficiency. We discuss advances in understanding the central and peripheral mechanisms underlying exercise intolerance in pwCF. Exploring both the central and peripheral factors that contribute to exercise intolerance in CF can help inform the development of new therapeutic targets, as well as help define prognostic criteria.
... Some authors have suggested that children and adolescents with CRDs are less physically active; 12,13 however, other authors have shown that there is no difference between these two groups. [14][15][16] Our objectives were (1) to determine the PA levels measured through accelerometers in children and adolescents with CRDs and (2) to compare them with their healthy peers. ...
Article
Background: The literature is unclear as to whether children and adolescents with chronic respiratory diseases (CRDs) differ from their healthy peers in physical activity (PA). Objective: To determine the PA levels measured through accelerometers in children and adolescents with CRDs. Methods: The authors conducted a systematic review using five databases. The authors included studies that assessed the PA measured by accelerometers in children and adolescents with CRDs. Two independent reviewers analyzed the studies, extracted the data, and assessed the quality of evidence. Results: From 11,497 reports returned by the initial search, 29 articles reporting on 4381 patients were included. In the sensitivity analysis, the authors found that children and adolescents with CRDs had a moderate-to-vigorous PA (MVPA) of -0.08 hours per day (95% confidence interval [CI], -0.12 to -0.03 h/d; P = .001), which was lower than the healthy controls; the values for sedentary time (mean difference -0.47 h/d; 95% CI, -1.29 to 0.36 h/d; P = .27) and steps/d (mean difference 361 steps/d; 95% CI -385 to 1707 steps/d; P = .45) were similar for both. Conclusion: Children and adolescents with CRDs have a slight reduction in MVPA in comparison with healthy controls, but sedentary time and steps/d were similar for both.
Article
Attainment and maintenance of good nutrition has been an important aspect of management in cystic fibrosis (CF) for decades. In the era of highly effective modulator therapy for CF, the quality of the nutrients we recommend is increasingly important. Our therapy must support our patients' health for many years beyond what we previously thought. Preventing cardiovascular disease, reducing hyperlipidemia, and optimizing lean body mass for active, longer lives now join the long-standing goal of promoting lung function through nutrition. This chapter summarizes recent developments in nutrition in people with CF, with an eye to the evolution of our practice.
Article
Background: Physical activity (including exercise) may form an important part of regular care for people with cystic fibrosis (CF). This is an update of a previously published review. Objectives: To assess the effects of physical activity interventions on exercise capacity by peak oxygen uptake, lung function by forced expiratory volume in one second (FEV1), health-related quality of life (HRQoL) and further important patient-relevant outcomes in people with cystic fibrosis (CF). Search methods: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings. The most recent search was on 3 March 2022. We also searched two ongoing trials registers: clinicaltrials.gov, most recently on 4 March 2022; and the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP), most recently on 16 March 2022. SELECTION CRITERIA: We included all randomised controlled trials (RCTs) and quasi-RCTs comparing physical activity interventions of any type and a minimum intervention duration of two weeks with conventional care (no physical activity intervention) in people with CF. Data collection and analysis: Two review authors independently selected RCTs for inclusion, assessed methodological quality and extracted data. We assessed the certainty of the evidence using GRADE. MAIN RESULTS: We included 24 parallel RCTs (875 participants). The number of participants in the studies ranged from nine to 117, with a wide range of disease severity. The studies' age demographics varied: in two studies, all participants were adults; in 13 studies, participants were 18 years and younger; in one study, participants were 15 years and older; in one study, participants were 12 years and older; and seven studies included all age ranges. The active training programme lasted up to and including six months in 14 studies, and longer than six months in the remaining 10 studies. Of the 24 included studies, seven implemented a follow-up period (when supervision was withdrawn, but participants were still allowed to exercise) ranging from one to 12 months. Studies employed differing levels of supervision: in 12 studies, training was supervised; in 11 studies, it was partially supervised; and in one study, training was unsupervised. The quality of the included studies varied widely. This Cochrane Review shows that, in studies with an active training programme lasting over six months in people with CF, physical activity probably has a positive effect on exercise capacity when compared to no physical activity (usual care) (mean difference (MD) 1.60, 95% confidence interval (CI) 0.16 to 3.05; 6 RCTs, 348 participants; moderate-certainty evidence). The magnitude of improvement in exercise capacity is interpreted as small, although study results were heterogeneous. Physical activity interventions may have no effect on lung function (forced expiratory volume in one second (FEV1) % predicted) (MD 2.41, 95% CI ‒0.49 to 5.31; 6 RCTs, 367 participants), HRQoL physical functioning (MD 2.19, 95% CI ‒3.42 to 7.80; 4 RCTs, 247 participants) and HRQoL respiratory domain (MD ‒0.05, 95% CI ‒3.61 to 3.51; 4 RCTs, 251 participants) at six months and longer (low-certainty evidence). One study (117 participants) reported no differences between the physical activity and control groups in the number of participants experiencing a pulmonary exacerbation by six months (incidence rate ratio 1.28, 95% CI 0.85 to 1.94) or in the time to first exacerbation over 12 months (hazard ratio 1.34, 95% CI 0.65 to 2.80) (both high-certainty evidence); and no effects of physical activity on diabetic control (after 1 hour: MD ‒0.04 mmol/L, 95% CI ‒1.11 to 1.03; 67 participants; after 2 hours: MD ‒0.44 mmol/L, 95% CI ‒1.43 to 0.55; 81 participants; moderate-certainty evidence). We found no difference between groups in the number of adverse events over six months (odds ratio 6.22, 95% CI 0.72 to 53.40; 2 RCTs, 156 participants; low-certainty evidence). For other time points (up to and including six months and during a follow-up period with no active intervention), the effects of physical activity versus control were similar to those reported for the outcomes above. However, only three out of seven studies adding a follow-up period with no active intervention (ranging between one and 12 months) reported on the primary outcomes of changes in exercise capacity and lung function, and one on HRQoL. These data must be interpreted with caution. Altogether, given the heterogeneity of effects across studies, the wide variation in study quality and lack of information on clinically meaningful changes for several outcome measures, we consider the overall certainty of evidence on the effects of physical activity interventions on exercise capacity, lung function and HRQoL to be low to moderate. Authors' conclusions: Physical activity interventions for six months and longer likely improve exercise capacity when compared to no training (moderate-certainty evidence). Current evidence shows little or no effect on lung function and HRQoL (low-certainty evidence). Over recent decades, physical activity has gained increasing interest and is already part of multidisciplinary care offered to most people with CF. Adverse effects of physical activity appear rare and there is no reason to actively discourage regular physical activity and exercise. The benefits of including physical activity in an individual's regular care may be influenced by the type and duration of the activity programme as well as individual preferences for and barriers to physical activity. Further high-quality and sufficiently-sized studies are needed to comprehensively assess the benefits of physical activity and exercise in people with CF, particularly in the new era of CF medicine.
Article
Little is known about motor competence and the longitudinal development of motor performance among youth with cystic fibrosis (CF). In this study, we assessed aspects of motor performance in different age groups of young patients with CF and compared them with a healthy reference group of same aged children. We also examined the development of motor performance among different age groups of these children with CF, using The Deutscher Motorik Test (DMT) to assess attributes of health-related and motor performance-related fitness. We used an incremental ergometer cycle test to determine maximal exercise capacity (expressed as peak workload). We evaluated and recorded habitual physical activity (PA) as measured by the number of steps per day and the time spent in different PA intensities (expressed in metabolic equivalents). In total, 31 children and adolescents with CF agreed to participate (13 girls,18 boys) aged 6–17 years ( M = 11.3, SD =3.3 years); they had a mean one second forced expiratory volume (expressed as a percentage of predicted value [% pred]) of 87.2% ( SD = 22.3%). We found their values of health-related and motor performance-related fitness to be significantly lower ( p < 0.05) than those of their healthy peer participants. In contrast to the reference group, participants with CF up to 14 years of age showed a linear improvement in these values and in their PA, followed by a plateau or even a nonsignificant decrease after age 14. These findings have important implications for the development and prescription of exercise programs for children with CF. Besides aerobic and strength exercises, we recommend that neuromuscular training be integrated into exercise programs to improve the coordinative abilities of youth with CF. More attention should be paid to vulnerable older adolescents to ensure their long-term motivation to maintain exercise participation.
Article
The outlook for those with cystic fibrosis (CF) has never been brighter with ever increasing life expectancy and the approval of the highly effective CFTR modulators, such as elexacaftor/tezacaftor/ivacaftor. With that being said, the progressive pulmonary decline and importance of lung health, infection, and inflammation in CF remains. This review is the second part in a three‐part CF Year in Review 2020. Part one focused on the literature related to CFTR modulators while part three will feature the multisystem effects related to CF. This review focuses on articles from Pediatric Pulmonology, including articles from other journals that are of particular interest to clinicians. Herein, we highlight studies published during 2020 related to CF pulmonary disease, infection, treatment, and diagnostics.
Article
Background In children and adolescents with asthma, the disease may reduce the perceived capability to participate in physical activity (PA) contributing to an increase in the sedentary lifestyle. The literature is unclear as to whether children and adolescents with asthma differ their PA levels from their healthy peers. Objective Our objective was to describe the different methods and instruments used to measure PA and to compare the PA levels of children and adolescents with asthma with those of their healthy peers. Study Design We conducted a systematic review of five databases. We included studies that compared the PA measured by objective and subjective instruments in children and adolescents with asthma versus controls. Two independent reviewers analyzed the studies, extracted the data, and assessed the quality of evidence. Results Of the 5966 reports returned by the initial search, 28 articles reporting on 3184 patients were included in the data synthesis. A forest plot showed that both groups had similar values of moderate to vigorous PA (MVPA; mean difference, −0.05 h/day; 95% confidence interval [CI], −0.11–0.01; p = .13), sedentary time (mean difference 0.00 h/day; 95% CI, −0.22–0.23 h/day; p = .99) and steps/day (mean difference 354 steps/day; 95% CI, −563–1270 steps/day; p = .45). Conclusion Children and adolescents with asthma have similar MVPA, steps/day, and sedentary time compared to the controls. The main instruments used were questionnaires and accelerometers.
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The revised edition of the Handbook offers the only guide on how to conduct, report and maintain a Cochrane Review. The second edition of The Cochrane Handbook for Systematic Reviews of Interventions contains essential guidance for preparing and maintaining Cochrane Reviews of the effects of health interventions. Designed to be an accessible resource, the Handbook will also be of interest to anyone undertaking systematic reviews of interventions outside Cochrane, and many of the principles and methods presented are appropriate for systematic reviews addressing research questions other than effects of interventions. This fully updated edition contains extensive new material on systematic review methods addressing a wide-range of topics including network meta-analysis, equity, complex interventions, narrative synthesis, and automation. Also new to this edition, integrated throughout the Handbook, is the set of standards Cochrane expects its reviews to meet. Written for review authors, editors, trainers and others with an interest in Cochrane Reviews, the second edition of The Cochrane Handbook for Systematic Reviews of Interventions continues to offer an invaluable resource for understanding the role of systematic reviews, critically appraising health research studies and conducting reviews.
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Background and Objective This review is an update of a previous review published in 2010, and aims to summarize the available studies on the measurement properties of physical activity questionnaires for young people under the age of 18 years. Methods Systematic literature searches were carried out using the online PubMed, EMBASE, and SPORTDiscus databases up to 2018. Articles had to evaluate at least one of the measurement properties of a questionnaire measuring at least the duration or frequency of children’s physical activity, and be published in the English language. The standardized COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN) checklist was used for the quality assessment of the studies. Results This review yielded 87 articles on 89 different questionnaires. Within the 87 articles, 162 studies were conducted: 103 studies assessed construct validity, 50 assessed test–retest reliability, and nine assessed measurement error. Of these studies, 38% were of poor methodological quality and 49% of fair methodological quality. A questionnaire with acceptable validity was found only for adolescents, i.e., the Greek version of the 3-Day Physical Activity Record. Questionnaires with acceptable test–retest reliability were found in all age categories, i.e., preschoolers, children, and adolescents. Conclusion Unfortunately, no questionnaires were identified with conclusive evidence for both acceptable validity and reliability, partly due to the low methodological quality of the studies. This evidence is urgently needed, as current research and practice are using physical activity questionnaires of unknown validity and reliability. Therefore, recommendations for high-quality studies on measurement properties of physical activity questionnaires were formulated in the discussion. PROSPERO Registration Number CRD42016038695.
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Recibido el 4 de diciembre de 2017; aceptado el 13 de julio de 2018 Palabras clave: Fibrosis quística; Niños; Capacidad física; Consumo pico de oxígeno Resumen Introducción: La fibrosis quística (FQ) es una enfermedad multisistémica hereditaria y progresiva. Una mejor capacidad física puede retardar la progresión de la enfermedad, mejorando así el pronós-tico y la supervivencia. El objetivo de esta investigación fue evaluar la capacidad física de los niños ad-mitidos en el programa nacional de FQ de la Región Metropolitana, Chile. Pacientes y Métodos: Se utilizó un diseño de estudio transversal multicéntrico. Los criterios de inclusión fueron: niños de 6 a 12 años de edad, incluidos en el Programa Nacional de FQ, madurez sexual Tanner I, ausencia de exacerbaciones respiratorias en los últimos 30 días y ausencia de enfermedades musculoesqueléticas. La capacidad aeróbica máxima fue evaluada a través del consumo pico de oxígeno (VO 2 pico) y se determinó con un protocolo incremental en un cicloergómetro magnético conectado a un ergoes-pirómetro en el que paralelamente se analizaron los gases respiratorios: valores de consumo de oxí-geno y producción de dióxido de carbono cada 30 segundos, umbral anaeróbico y carga máxima de trabajo. Además, se evaluaron los valores de capacidad vital forzada (CVF), volumen espiratorio al primer segundo (VEF 1), relación VEF 1 /CVF y los flujos espiratorios forzados entre el 25 y 75% de la capacidad vital. Durante la prueba se registró: saturación arterial de oxígeno, frecuencia respiratoria, Versión in press ID 517 ARTÍCULO ORIGINAL Introducción La Fibrosis Quística (FQ) es una enfermedad here-ditaria, autosómica recesiva causada por una mutación del gen que codifica para la proteína de regulación de transmembrana de la fibrosis quística (CFTR) 1. Actual-mente, a nivel global existen más de 1900 mutaciones de la CFTR y cerca de 2/3 de los casos corresponden a la mutación p.F508del 1. En Chile, esta mutación repre-senta menos del 40% de todos los casos 2. Esta patología genera diferentes alteraciones en distintos órganos, principalmente en los pulmones y a nivel gastrointestinal 3 generando, en muchos casos, secuelas que afectan la calidad de vida y la sobrevida de estos pacientes, que gracias a los avances en el desa-rrollo de nuevos tratamientos, principalmente farma-cológicos, han mejorado el pronóstico de esta enfer-medad 4,5. La actividad física y el ejercicio son ampliamente aceptados como parte de las estrategias terapéuticas en el manejo de la FQ 6-8 siendo parte fundamental del crecimiento y desarrollo de los niños 9. Existen distintas formas para medir de forma objetiva la capacidad físi-ca, siendo el gold standard el consumo pico de oxígeno Fibrosis quística-H. Puppo et al frecuencia cardíaca, presión arterial, volumen corriente y se consultó la percepción de fatiga de extre-midades inferiores y disnea a través de la escala de Borg modificada. La duración aproximada del test fue alrededor de 10 minutos. Resultados: Se revisaron los registros clínicos de 43 niños, recogidos en seis centros de salud. Veintinueve niños cumplieron los criterios de inclusión siendo 23 reclutados. Dos niños no pudieron participar, reduciendo el grupo final de sujetos a 21 (13:8 varones:mujeres). La edad media fue de 8,8 ± 2 años; el peso fue de 30,5 ± 10,9 kg; la talla fue de 1,32 ± 0,11 m y el índice de masa corporal fue de 17,1 ± 3,5 (z-score 0,01 ± 1,34). Más de la mitad (61%) de los niños estaba eutrófico. El VO 2 pico obtenido fue de 43,7 ± 6,5 ml/min/kg (106,7 ± 19,8% de los valores teóricos de referencia). Sólo el 10% de los niños tenían valores inferiores a los valores teóricos esperados para población normal, ajustados por sexo y edad. No se encontraron correlaciones entre el VO 2 pico y las variables antropométricas y de función pulmonar. Conclusión: La mayoría de los niños evaluados (90%) tenían capacidad física similar a los valores teóricos de referencia para niños sanos ajustados por sexo y edad. Abstract Introduction: Cystic fibrosis (CF) is an inherited, progressive, multisystem disease. Better physical capacity may slow disease progression, thus improving prognosis and survival. The objective of this research was to evaluate the physical capacity of children admitted to the National CF Program of the Metropolitan Region, Chile. Patients and Methods: A multicenter, cross-sectional study design was used. The inclusion criteria were children aged 6 to 12 years enrolled in the National CF Program; Tanner sexual maturity stage I, no respiratory exacerbations in the last 30 days, and no musculoskeletal pathologies. The maximum aerobic capacity was assessed through the peak oxygen uptake (VO 2 peak) and determined with an incremental protocol in a magnetic cycle er-gometer connected to an ergo-spirometer with which, at the same time, respiratory gases, oxygen consumption and carbon dioxide production values every 30 seconds, anaerobic threshold, and maximum workload were analyzed. The values of forced vital capacity (FVC), forced expiratory volume in 1 second (FEV 1), FEV 1 /FVC ratio, and forced expiratory flows between 25% and 75% of vital capacity were assessed through ergo-spirometry. At the beginning of the ergo-spirometry, arterial oxygen saturation, respiratory rate, heart rate, blood pressure, tidal volume and the perception of lower extremity fatigue and dyspnea were recorded using the modified Borg scale. The test lasted approximately 10 minutes. Results: The clinical records of 43 children collected from six health centers were reviewed. Out of these, 29 children met inclusion criteria, and 23 were recruited. Two children were unable to participate, reducing the final subject group to 21 (13 males, 8 females). The mean age was 8.8 ± 2 years; weight 30.5 ± 10.9 kg; height 1.32 ± 0.11 m; and body mass index 17.1 ± 3.5 (z-score 0.01 ± 1.34). More than half of the children (61%) had normal weight. The obtained VO 2 peak was 43.7 ± 6.5 ml/min/kg (106.7 ± 19.8% of the predictive values). Only 10% of the children had values lower than those predicted by sex and age. No correlations were found between VO 2 peak and anthropometric and pulmonary function variables. Conclusion: Most of the evaluated children (90%) had physical capacity similar to healthy subjects by sex and age.
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The prevalence and mortality of chronic obstructive pulmonary disease (COPD) is increasing in Chile, constituting a public health problem. Pulmonary and systemic consequences of COPD affect physical activity, as the disease progresses. There are multiple means for physical activity assessment, from low cost and easily applicable questionnaires to sophisticated laboratory tests. Physical inactivity is a modifiable risk factor for morbidity and mortality in patients with COPD. Physical activity interventions not only contribute to decrease the likelihood of mortality, but also protect from comorbidities, especially cardiovascular ones. It also plays a major role avoiding functional limitations of these subjects. Dyspnea and fatigue render exercise as an unpleasant activity for most patients with COPD. If psychological alterations such as anxiety and depression are summed, these patients drift towards an inactive lifestyle. This article analyzes several tools available to assess physical activity is patients with COPD, useful in clinical practice. (Rev Med Chile 2017; 145: 1588-1596)
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Background: Physical activity is recommended as a component of the cystic fibrosis (CF) treatment regimen. However, to date, there is limited research examining the effects of behavioral counseling interventions aimed at increasing physical activity. The aim of this study was to assess the feasibility of a theoretically informed, parent-mediated counseling intervention in increasing habitual physical activity and quality of life among children and youth with CF. Methods: Participants were recruited from the pediatric respirology clinic at the Children's Hospital of Winnipeg. Participants ranged in age between 8 and 18 y. A randomized control feasibility trial was implemented, and participants were randomized to the intervention (n = 7) or control group (n = 6). Intervention group participants engaged in 4 counseling sessions to examine the acceptability and feasibility of physical activity counseling in the care of children with CF. The primary outcome was study feasibility, as measured by recruitment, retention, adherence, acceptability, and the frequency of adverse events. Secondary outcomes included physical activity and quality of life, as measured by accelerometry and the Pediatric Quality of Life Inventory. Results: Thirteen subjects completed the study. No adverse events were found in this trial. The intervention was found to be feasible and acceptable with good recruitment, retention, adherence, and acceptability. Positive trends were also reported in terms of increases in physical activity, reductions in time spent being sedentary, and improvements in most dimensions of quality of life pre- to post-intervention. Conclusions: The findings suggest that counseling is feasible for the CF community. An appropriately powered randomized controlled trial is required in the future to investigate the utility of counseling as a means to enhance quality of life and physical activity behavior.
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Objectives Study of currently approved drugs and exploration of future clinical development pipeline therapeutics for cystic fibrosis, and possible limitations in their use. Methods Extensive literature search using individual and a combination of key words related to cystic fibrosis therapeutics. Key findingsCystic fibrosis is an autosomal recessive disorder due to mutations in CFTR gene leading to abnormality of chloride channels in mucus and sweat producing cells. Respiratory system and GIT are primarily involved but eventually multiple organs are affected leading to life threatening complications. Management requires drug therapy, extensive physiotherapy and nutritional support. Previously, the focus was on symptomatic improvement and complication prevention but recently the protein rectifiers are being studied which are claimed to correct underlying structural and functional abnormalities. Some improvement is observed by the corrector drugs. Other promising approaches are gene therapy, targeting of cellular interactomes, and newer drugs for symptomatic improvement. Conclusions The treatment has a long way to go as most of the existing therapeutics is for older children. Other limiting factors include mutation class, genetic profile, drug interactions, adverse effects, and cost. Novel approaches like gene transfer/gene editing, disease modeling and search for alternative targets are warranted.
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Objective: Cystic fibrosis (CF), caused by mutations in the CF transmembrane conductance regulator (CFTR) gene, continues to present diagnostic challenges. Newborn screening and an evolving understanding of CF genetics have prompted a reconsideration of the diagnosis criteria. Study design: To improve diagnosis and achieve standardized definitions worldwide, the CF Foundation convened a committee of 32 experts in CF diagnosis from 9 countries to develop clear and actionable consensus guidelines on the diagnosis of CF and to clarify diagnostic criteria and terminology for other disorders associated with CFTR mutations. An a priori threshold of ≥80% affirmative votes was required for acceptance of each recommendation statement. Results: After reviewing relevant literature, the committee convened to review evidence and cases. Following the conference, consensus statements were developed by an executive subcommittee. The entire consensus committee voted and approved 27 of 28 statements, 7 of which needed revisions and a second round of voting. Conclusions: It is recommended that diagnoses associated with CFTR mutations in all individuals, from newborn to adult, be established by evaluation of CFTR function with a sweat chloride test. The latest mutation classifications annotated in the Clinical and Functional Translation of CFTR project (http://www.cftr2.org/index.php) should be used to aid in diagnosis. Newborns with a high immunoreactive trypsinogen level and inconclusive CFTR functional and genetic testing may be designated CFTR-related metabolic syndrome or CF screen positive, inconclusive diagnosis; these terms are now merged and equivalent, and CFTR-related metabolic syndrome/CF screen positive, inconclusive diagnosis may be used. International Statistical Classification of Diseases and Related Health Problems, 10th Revision codes for use in diagnoses associated with CFTR mutations are included.
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PURPOSE: Glucose abnormality and diabetes are the most common comorbidities in cystic fibrosis (CF). Combined (aerobic and resistance) exercise program in type 2 patients with diabetes demonstrated an improvement of glycemic control. The aim of the study was to determine whether a combined exercise program is beneficial to improve plasma glucose at 2 h of the oral glucose tolerance test in CF. METHOD: Eighteen adults with CF with glucose abnormality were recruited (Clinicaltrial.gov: NTC02127957), and 17 were randomly assigned to a control or exercise group for 12 wk. V˙O2max, oral glucose tolerance test, muscular endurance and strength, and quality of life were measured pre- and postintervention. RESULTS: Fourteen participants completed the protocol. Patients in the exercise group improved significantly their 2-h plasma glucose values (-2.34 ± 1.26 mmol·L, P < 0.007, confidence interval = 99.22%) and presented a reduction of 17.2% (P < 0.05) in total glucose excursion. No significant change for other parameters was observed. CONCLUSION: A combined exercise program improves glycemic control in CF.
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Background Synthesis of multiple randomized controlled trials (RCTs) in a systematic review can summarize the effects of individual outcomes and provide numerical answers about the effectiveness of interventions. Filtering of searches is time consuming, and no single method fulfills the principal requirements of speed with accuracy. Automation of systematic reviews is driven by a necessity to expedite the availability of current best evidence for policy and clinical decision-making. We developed Rayyan (http://rayyan.qcri.org), a free web and mobile app, that helps expedite the initial screening of abstracts and titles using a process of semi-automation while incorporating a high level of usability. For the beta testing phase, we used two published Cochrane reviews in which included studies had been selected manually. Their searches, with 1030 records and 273 records, were uploaded to Rayyan. Different features of Rayyan were tested using these two reviews. We also conducted a survey of Rayyan’s users and collected feedback through a built-in feature. Results Pilot testing of Rayyan focused on usability, accuracy against manual methods, and the added value of the prediction feature. The “taster” review (273 records) allowed a quick overview of Rayyan for early comments on usability. The second review (1030 records) required several iterations to identify the previously identified 11 trials. The “suggestions” and “hints,” based on the “prediction model,” appeared as testing progressed beyond five included studies. Post rollout user experiences and a reflexive response by the developers enabled real-time modifications and improvements. The survey respondents reported 40% average time savings when using Rayyan compared to others tools, with 34% of the respondents reporting more than 50% time savings. In addition, around 75% of the respondents mentioned that screening and labeling studies as well as collaborating on reviews to be the two most important features of Rayyan. As of November 2016, Rayyan users exceed 2000 from over 60 countries conducting hundreds of reviews totaling more than 1.6M citations. Feedback from users, obtained mostly through the app web site and a recent survey, has highlighted the ease in exploration of searches, the time saved, and simplicity in sharing and comparing include-exclude decisions. The strongest features of the app, identified and reported in user feedback, were its ability to help in screening and collaboration as well as the time savings it affords to users. Conclusions Rayyan is responsive and intuitive in use with significant potential to lighten the load of reviewers.
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Non-communicable disease is a leading threat to global health. Physical inactivity is a large contributor to this problem; in fact, the WHO ranks it as the fourth leading risk factor for overall morbidity and mortality worldwide. In Canada, at least 4 of 5 adults do not meet the Canadian Physical Activity Guidelines of 150 min of moderate-to-vigorous physical activity per week. Physicians play an important role in the dissemination of physical activity (PA) recommendations to a broad segment of the population, as over 80% of Canadians visit their doctors every year and prefer to get health information directly from them. Unfortunately, most physicians do not regularly assess or prescribe PA as part of routine care, and even when discussed, few provide specific recommendations. PA prescription has the potential to be an important therapeutic agent for all ages in primary, secondary and tertiary prevention of chronic disease. Sport and exercise medicine (SEM) physicians are particularly well suited for this role and should collaborate with their primary care colleagues for optimal patient care. The purpose of this Canadian Academy and Sport and Exercise Medicine position statement is to provide an evidence-based, best practices summary to better equip SEM and primary care physicians to prescribe PA and exercise, specifically for the prevention and management of non-communicable disease. This will be achieved by addressing common questions and perceived barriers in the field.
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Systematic reviews should build on a protocol that describes the rationale, hypothesis, and planned methods of the review; few reviews report whether a protocol exists. Detailed, well-described protocols can facilitate the understanding and appraisal of the review methods, as well as the detection of modifications to methods and selective reporting in completed reviews. We describe the development of a reporting guideline, the Preferred Reporting Items for Systematic reviews and Meta-Analyses for Protocols 2015 (PRISMA-P 2015). PRISMA-P consists of a 17-item checklist intended to facilitate the preparation and reporting of a robust protocol for the systematic review. Funders and those commissioning reviews might consider mandating the use of the checklist to facilitate the submission of relevant protocol information in funding applications. Similarly, peer reviewers and editors can use the guidance to gauge the completeness and transparency of a systematic review protocol submitted for publication in a journal or other medium.
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The aim of this position statement was to inform the choice of physical activity tools for use within CF research and clinical settings. A systematic review of physical activity tools to explore evidence for reliability, validity, and responsiveness. Narrative answers to "four key questions" on motion sensors, questionnaires and diaries were drafted by the core writing team and then discussed at the Exercise Working Group in ECFS Lisbon 2013. Our current position is that activity monitors such as SenseWear or ActiGraph offer informed choices to facilitate a comprehensive assessment of physical activity, and should as a minimum report on dimensions of physical activity including energy expenditure, step count and time spent in different intensities and sedentary time. The DigiWalker pedometer offers an informed choice of a comparatively inexpensive method of obtaining some measurement of physical activity. The HAES represents an informed choice of questionnaire to assess physical activity. There is insufficient data to recommend the use of one diary over another. Future research should focus on providing additional evidence of clinimetric properties of these and new physical activity assessment tools, as well as further exploring the added value of physical activity assessment in CF. Copyright © 2015. Published by Elsevier B.V.
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Despite the benefits of physical activity for youth living with cystic fibrosis (CF), the majority of patients are insufficiently active to meet physical activity recommendations. Existing physical activity interventions are atheoretical and tend to prescribe standardized exercise regimes which are often not appealing for children and compromise long term adherence. Following recent calls for counselling based physical activity approaches in the CF population, this study describes the development of a theoretically informed, parent-mediated, behavioural counselling intervention for CF youth―“CF Chatters”. We first a) describe the development of a grounded theory of physical activity in youth with CF; b) explain how this theory informed the development and implementation of the CF Chatters program and c) reflect on the findings of our pilot intervention using a case study research design. CF Chatters participants demonstrated self reported increases in physical activity and quality of life. While further development and more robust objective measures are needed to extend this investigative pilot work, our findings suggest that behavioural counselling is an effective modality for enhancing physical activity participation and quality of life in this life limited group of children and youth.
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Background. Patients with cystic fibrosis (CF) are observed to have diminished lung function, nutritional status, and aerobic exercise capacity. All three parameters are related to prognosis and survival. However, there is little information regarding these parameters in prepubertal patients. Methods. Our study groups consisted of sixteen patients with CF (7 girls) and 99 healthy volunteers (52 girls). Subjects performed spirometry and a progressive exercise test to exhaustion on a cycle ergometer. Leg muscle strength was measured using an isokinetic dynamometer. Physical activity was examined using the modifiable activity questionnaire and accelerometer. Results. Nutritional status was similar between groups (BMI—boys control versus CF 18.5 versus 17.9, girls control versus CF 19.5 versus 17.4). Girls with CF were significantly smaller and lighter than controls. Lung function was significantly reduced in CF groups (FEV1—boys control versus CF 91% versus 84%, girls control versus CF 90% versus 82%). Patients with CF were as active and as fit as their healthy controls. Conclusion. In this group of prepubertal children with CF, nutritional status was comparable to healthy children of the same age. Their aerobic exercise tolerance and peripheral muscle strength were also relatively well preserved despite significantly lower lung function.
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Exercise is beneficial for patients with Cystic Fibrosis (CF) but long term effects of physical activity on lung function evolution are unknown. We evaluated the longitudinal relationship between changes in habitual physical activity (HPA) and rate of decline in lung function in patients with CF.We tracked HPA with the Habitual Activity Estimation Scale, FEV1 and Stage I exercise tests in 212 patients with CF over a nine year period.Adjusting for gender, baseline age and FEV1, mucoid Pseudomonas aeruginosa and CF related diabetes, FEV1 % predicted decreased by -1.63%/year (SD=0.08 p<0.0001) while HPA increased by 0.28 hours·day(-1)·year(-1) (SD=0.03 p<0.0001), over the study period. A greater increase in HPA was associated with a slower rate of decline in FEV1 (r=0.19, p<0.0069). Dividing into HIGH and LOW activity, (above or below the mean rate of change of activity respectively), a steeper rate of FEV1 decline was observed for LOW (-1.90%/year) compared to HIGH (-1.39%/year; p=0.002).Increases in HPA are feasible despite progression of lung disease and are associated with a slower rate of decline in FEV1, highlighting the benefit of regular physical activity, and its positive impact on lung function in patients with CF.
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Physical activity (PA) is recommended as part of the treatment regimen for cystic fibrosis (CF) although objective methods have been scarcely used to monitor achievement of PA guidelines. PA was measured by accelerometer in outpatient CF children (n=47) and results were compared with those obtained in age- and gender-matched healthy controls (n=39). 2.1% of the outpatients fulfilled PA guidelines (i.e. ≥60min·day(-1) of moderate-to-vigorous PA (MVPA)) vs. 34.2% of controls. Overall, lower MVPA levels were observed in CF patients than controls despite the former undergoing more light or total PA. Peak oxygen uptake was also lower in the CF group than in controls (37.5±7.8 vs. 43.5±7.6ml·kg(-1)·min(-1), p=0.002) and was correlated with MVPA and vigorous PA in the former. These findings support a need to promote PA interventions (including MVPA) targeted at improving cardiorespiratory fitness in CF children.
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Purpose To investigate (1) the contributions of sex, age, nutritional status- and physical-fitness-related variables on health-related quality of life (HRQOL) in Spanish children with cystic fibrosis, and (2) the agreement on HRQOL between children and their parents. Methods In 28 children aged 6–17 years, body mass index percentile, percentage body fat, physical activity, pulmonary function, cardiorespiratory fitness, functional mobility, and dynamic muscle strength were determined using objective measures. HRQOL was measured using the revised version of the cystic fibrosis questionnaire. Simple and multiple linear regression analyses were performed to determine the variables associated with HRQOL. To assess the agreement on HRQOL between children and parents, intra-class correlation coefficients (ICCs) were calculated. Results Girls reported worse emotional functioning, a higher treatment burden, and more respiratory problems than boys. Greater functional mobility appeared associated with a less favourable body image and more eating disturbances. Agreement on HRQOL between children and parents was good to excellent, except for the domain of treatment burden. Conclusions Sex and age were stronger predictors of HRQOL than nutritional status- or physical-fitness-related variables. Children reported a lower treatment burden than their parents perceived them to have.
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Daily physical activity (PA) may be an excellent tool for the maintenance of bone health in patients with cystic fibrosis (CF). The aim of this study was to analyze the possible association between physical capacity and activity and bone mineral density (BMD) in young adults with CF. A secondary goal was to evaluate vertebral fractures in this population. A cross-sectional study was conducted in 50 patients with CF who were clinically stable and aged > 16 years but not lung transplant recipients. PA was quantified with a portable motion monitor (BodyMedia Fit Armband). Cardiopulmonary exercise and 6-min walk tests were used to assess exercise capacity. BMD was obtained from dual-energy x-ray absorptiometry of the lumbar column, hip, and whole body. To analyze vertebral fractures and deformity, we performed the Genant and Cobb methods. Daily PA time at low (3-4.8 metabolic equivalent tasks [METs]) and moderate (4.8-7.2 METs) intensity, respectively, was correlated with Z score (BMD) of the lumbar column (r = 0.36, P < .01 and r = 0.59, P < .001), the neck of femur (r = 0.51, P < .001 and r = 0.72, P < .001), and the total hip (r = 0.54, P < .001 and r = 0.74, P < .001). PA, BMI, age, and sex were predictors of BMD. Vertebral fractures correlated with kyphosis (r = 0.42, P = .02), but not with BMD. Patients who were mildly and severely affected differed in vertebral fracture rate and kyphosis prevalence (P = .002 and P = .013, respectively). The most active patients with better exercise capacity had higher BMD. Those with more affected pulmonary function had a greater prevalence of vertebral fractures and dorsal kyphosis.
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The purpose of this study was to develop an activity energy expenditure (AEE) prediction equation for the Actiheart activity monitor for use in children with chronic disease. In total, 63 children, aged 8-18 years with different types of chronic disease (juvenile arthritis, hemophilia, dermatomyositis, neuromuscular disease, cystic fibrosis or congenital heart disease) participated in an activity testing session, which consisted of a resting protocol, working on the computer, sweeping, hallway walking, steps and treadmill walking at three different speeds. During all activities, actual AEE was measured with indirect calorimetry and the participants wore an Actiheart on the chest. Resting EE and resting heart rate were measured during the resting protocol and heart rate above sleep (HRaS) was calculated. Mixed linear modeling produced the following prediction equation: This equation results in a nonsignificant mean difference of 2.1 J/kg/min (limits of agreement: -144.2 to 148.4 J/kg/min) for the prediction of AEE from the Actiheart compared with actual AEE. The Actiheart is valid for the use of AEE determination when using the new prediction equation for groups of children with chronic disease. However, the prediction error limits the use of the equation in individual subjects.
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The purpose was to: 1) perform a systematic review of studies examining the relation between physical activity, fitness, and health in school-aged children and youth, and 2) make recommendations based on the findings. The systematic review was limited to 7 health indicators: high blood cholesterol, high blood pressure, the metabolic syndrome, obesity, low bone density, depression, and injuries. Literature searches were conducted using predefined keywords in 6 key databases. A total of 11,088 potential papers were identified. The abstracts and full-text articles of potentially relevant papers were screened to determine eligibility. Data was abstracted for 113 outcomes from the 86 eligible papers. The evidence was graded for each health outcome using established criteria based on the quantity and quality of studies and strength of effect. The volume, intensity, and type of physical activity were considered. Physical activity was associated with numerous health benefits. The dose-response relations observed in observational studies indicate that the more physical activity, the greater the health benefit. Results from experimental studies indicate that even modest amounts of physical activity can have health benefits in high-risk youngsters (e.g., obese). To achieve substantive health benefits, the physical activity should be of at least a moderate intensity. Vigorous intensity activities may provide even greater benefit. Aerobic-based activities had the greatest health benefit, other than for bone health, in which case high-impact weight bearing activities were required. The following recommendations were made: 1) Children and youth 5-17 years of age should accumulate an average of at least 60 minutes per day and up to several hours of at least moderate intensity physical activity. Some of the health benefits can be achieved through an average of 30 minutes per day. [Level 2, Grade A]. 2) More vigorous intensity activities should be incorporated or added when possible, including activities that strengthen muscle and bone [Level 3, Grade B]. 3) Aerobic activities should make up the majority of the physical activity. Muscle and bone strengthening activities should be incorporated on at least 3 days of the week [Level 2, Grade A].
Article
Purpose: Physical activity is well known to improve or maintain exercise capacity and overall health in patients with cystic fibrosis (CF). However, many patients do not meet recommended guidelines for physical activity. The purpose of this study was to determine if individualized, targeted coaching could improve the levels of physical activity and quality of life in children and adolescents with CF. Subjects: Twelve children with CF, ages 7 to 17, participated in this study.Method: Each participant completed a physical activity questionnaire (PAQ) and a disease-specific quality of life questionnaire (CFQ) at baseline and three months later. The participants were given pedometers and a 10,000 step per day target. Participants also participated in discussions regarding ways to increase physical activity appropriate for their interest, abilities and age. Weekly follow-up phone calls were made to obtain pedometer counts and discuss physical activities performed. Results: Physical activity scores (PAQ) improved in 6 of 12 participants. Quality of life improved in many dimensions of the CFQ, particularly in vitality (3 of 5 improved); emotional (8 of 12 improved) and respiratory (7 of 12 improved). Conclusions: The results of this small pilot study suggest that an individualized coaching approach to physical activity in children and adolescents with CF may improve physical activity levels and improve important aspects of quality of life in some children with CF.
Article
Objectives: Cardiovascular disease prevention should begin in childhood. However, the influence of physical activity on cardiovascular health in early childhood is unknown. Our purpose in this study was to determine the effect of physical activity on trajectories of cardiovascular health indicators during early childhood. Methods: This prospective, observational cohort study (Health Outcomes and Physical Activity in Preschoolers) enrolled 418 3- to 5-year-olds with annual assessments for 3 years. Total physical activity (TPA) and moderate-to-vigorous physical activity (MVPA) were measured over 7 days via accelerometry. Cardiovascular health indicators included cardiovascular fitness (exercise time on a maximal treadmill test [treadmill time] and 1-minute heart rate recovery), resting arterial stiffness (whole-body pulse wave velocity and carotid β stiffness index), and seated systolic blood pressure. Data were analyzed by using linear mixed-effects modeling; effects are reported as unstandardized estimates (Est). Results: There were main effects of TPA and MVPA on treadmill time (Est = 0.004 [P = .005] and 0.008 [P = .001], respectively) and heart rate recovery (Est = 0.05 [P < .001] and 0.08 [P < .001], respectively). There was a main effect of TPA on pulse wave velocity (Est = -0.001; P = .02) and an MVPA × time interaction (Est = -0.002; P = .01). For carotid β stiffness index, the effect of a TPA × time interaction was not significant (Est = -0.002; P = .051); however, there was a significant MVPA × time interaction (Est = -0.003; P = .03). MVPA was associated with a slower rate of change in systolic blood pressure for girls (Est = 0.06; P = .009). Conclusions: Children who engage in higher levels of physical activity during early childhood have better cardiovascular health indicators, with more intense physical activity (ie, MVPA) attenuating the stiffening of arteries.
Article
Physical activity (PA) is a key element in Cystic Fibrosis (CF) treatment strategies, yet little is known as to whether activity compensation occurs. This study examined whether PA and/or sedentary time on one day were temporally associated with time spent in these intensities the following day in youth with CF. Time spent sedentary and in different PA intensities were objectively-measured for seven consecutive days in 50 youth (22 boys; 12.0 ± 2.7 years); 25 with mild-to-moderate CF and 25 age- and sex-matched controls. Multilevel analyses (day and child) were conducted using generalised linear latent and mixed models. On any given day, every additional 10 minutes spent in sedentary time or moderate-to-vigorous physical activity (MVPA) were associated with 1.9 (95%CI: −3.6 to −1.2) and 12.4 (95%CI: −22.1 to −2.9) minutes less sedentary time the following day, respectively. These temporal associations were also observed when split by group (3.1 vs. 1.9 minutes for healthy and CF, respectively). These findings indicate that youth do not compensate their PA, irrespective of disease status, between days, but may compensate their sedentary time between days. Experimental studies are warranted to fully elucidate whether compensatory responses to PA and sedentary time occur, which is fundamental for informing PA promotion strategies.
Article
Prognostic factors are associated with the risk of future health outcomes in individuals with a particular health condition or some clinical start point (eg, a particular diagnosis). Research to identify genuine prognostic factors is important because these factors can help improve risk stratification, treatment, and lifestyle decisions, and the design of randomised trials. Although thousands of prognostic factor studies are published each year, often they are of variable quality and the findings are inconsistent. Systematic reviews and meta-analyses are therefore needed that summarise the evidence about the prognostic value of particular factors. In this article, the key steps involved in this review process are described.
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In this review, we develop a blueprint for exercise biology research in the new millennium. The first part of our plan provides statistics to support the contention that there has been an epidemic emergence of modern chronic diseases in the latter part of the 20th century. The health care costs of these conditions were almost two-thirds of a trillion dollars and affected 90 million Americans in 1990. We estimate that these costs are now approaching $1 trillion and stand to further dramatically increase as the baby boom generation ages. We discuss the reaction of the biomedical establishment to this epidemic, which has primarily been to apply modern technologies to stabilize overt clinical problems (e.g., secondary and tertiary prevention). Because this approach has been largely unsuccessful in reversing the epidemic, we argue that more emphasis must be placed on novel approaches such as primary prevention, which requires attacking the environmental roots of these conditions. In this respect, a strong association exists between the increase in physical inactivity and the emergence of modern chronic diseases in 20th century industrialized societies. Approximately 250,000 deaths per year in the United States are premature due to physical inactivity. Epidemiological data have established that physical inactivity increases the incidence of at least 17 unhealthy conditions, almost all of which are chronic diseases or considered risk factors for chronic diseases. Therefore, as part of this review, we present the concept that the human genome evolved within an environment of high physical activity. Accordingly, we propose that exercise biologists do not study “the effect of physical activity” but in reality study the effect of reintroducing exercise into an unhealthy sedentary population that is genetically programmed to expect physical activity. On the basis of healthy gene function, exercise research should thus be viewed from a nontraditional perspective in that the “control” group should actually be taken from a physically active population and not from a sedentary population with its predisposition to modern chronic diseases. We provide exciting examples of exercise biology research that is elucidating the underlying mechanisms by which physical inactivity may predispose individuals to chronic disease conditions, such as mechanisms contributing to insulin resistance and decreased skeletal muscle lipoprotein lipase activity. Some findings have been surprising and remarkable in that novel signaling mechanisms have been discovered that vary with the type and level of physical activity/inactivity at multiple levels of gene expression. Because this area of research is underfunded despite its high impact, the final part of our blueprint for the next millennium calls for the National Institutes of Health (NIH) to establish a major initiative devoted to the study of the biology of the primary prevention of modern chronic diseases. We justify this in several ways, including the following estimate: if the percentage of all US morbidity and mortality statistics attributed to the combination of physical inactivity and inappropriate diet were applied as a percentage of the NIH's total operating budget, the resulting funds would equal the budgets of two full institutes at the NIH! Furthermore, the fiscal support of studies elucidating the scientific foundation(s) targeted by primary prevention strategies in other public health efforts has resulted in an increased efficacy of the overall prevention effort. We estimate that physical inactivity impacts 80–90% of the 24 integrated review group (IRG) topics proposed by the NIH's Panel on Scientific Boundaries for Review, which is currently directing a major restructuring of the NIH's scientific funding system. Unfortunately, the primary prevention of chronic disease and the investigation of physical activity/inactivity and/or exercise are not mentioned in the almost 200 total subtopics comprising the IRGs in the Panel's proposal. We believe this to be a glaring omission by the Panel and contend that the current reorganization of NIH's scientific review and funding system is a golden opportunity to invest in fields that study the biological mechanisms of primary prevention of chronic diseases (such as exercise biology). This would be an investment to avoid US health care system bankruptcy as well as to reduce the extreme human suffering caused by chronic diseases. In short, it would be an investment in the future of health care in the new millennium.
Article
Background: Physical exercise training may form an important part of regular care for people with cystic fibrosis. This is an update of a previously published review. Objectives: To assess the effects of physical exercise training on exercise capacity by peak oxygen consumption, pulmonary function by forced expiratory volume in one second, health-related quality of life and further important patient-relevant outcomes in people with cystic fibrosis. Search methods: We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group Trials Register which comprises references identified from comprehensive electronic database searches and handsearches of relevant journals and abstract books of conference proceedings.Date of the most recent search: 04 May 2017.We searched ongoing trials registers (clinicaltrials.gov and the WHO ICTRP). Date of most recent search: 10 August 2017. Selection criteria: All randomised and quasi-randomised controlled clinical trials comparing exercise training of any type and a minimum duration of two weeks with conventional care (no training) in people with cystic fibrosis. Data collection and analysis: Two authors independently selected studies for inclusion, assessed methodological quality and extracted data. The quality of the evidence was assessed using the GRADE system. Main results: Of the 83 studies identified, 15 studies which included 487 participants, met the inclusion criteria. The numbers in each study ranged from nine up to 72 participants; two studies were in adults, seven were in children and adolescents and six studies included all age ranges. Four studies of hospitalised participants lasted less than one month and 11 studies were outpatient-based, lasting between two months and three years. The studies included participants with a wide range of disease severity and employed differing levels of supervision with a mixture of types of training. There was also wide variation in the quality of the included studies.This systematic review shows very low- to low-quality evidence from both short- and long-term studies that in people with cystic fibrosis aerobic or anaerobic physical exercise training (or a combination of both) has a positive effect on aerobic exercise capacity, pulmonary function and health-related quality of life. No study reported on mortality; two studies reported on adverse events (moderate-quality evidence); one of each study reported on pulmonary exacerbations (low-quality evidence) and diabetic control (very low-quality evidence). Although improvements were not consistent between studies and ranged from no effects to clearly positive effects, the most consistent effects of the heterogeneous exercise training modalities and durations were found for maximal aerobic exercise capacity (in four out of seven studies) with unclear effects on forced expiratory volume in one second (in two out of 11 studies) and health-related quality of life (in two out of seven studies). Authors' conclusions: Evidence about the efficacy of physical exercise training in cystic fibrosis from 15 small studies with low to moderate methodological quality is limited. Exercise training is already part of regular outpatient care offered to most people with cystic fibrosis, and since there is some evidence for beneficial effects on aerobic fitness and no negative side effects exist, there is no reason to actively discourage this. The benefits from including physical exercise training in an individual's regular care may be influenced by the type and duration of the training programme. High-quality randomised controlled trials are needed to comprehensively assess the benefits of exercise programmes in people with cystic fibrosis and the relative benefits of the addition of aerobic versus anaerobic versus a combination of both types of physical exercise training to the care of people with cystic fibrosis.
Article
Background: Regular physical activity (PA) is increasingly recognised as important in the care of patients with Cystic Fibrosis (CF) but there is a dearth of evidence regarding physical activity levels (PAL) or how these are accrued in those with CF. Methods: Physical activity was measured by a hip-worn accelerometer for seven consecutive days by eighteen children (10 boys; 12.4 ± 2.8 years) with mild to moderate CF and eighteen age- and sex-matched controls (10 boys; 12.5 ± 2.7 years). Results: Both CF and healthy children demonstrated similar PAL and patterns of accumulation across the intensity spectrum, with higher levels of PA during weekdays in both groups. FEV1 was predicted by high-light PA in CF compared to low-light PA in healthy children. Conclusion: These findings highlight weekends and light PA as areas warranting further research for the development of effective intervention strategies to increase PA in the youth CF population.
Article
There is clear and consistent evidence that regular physical activity is an important component of healthy lifestyles, and fundamental to promoting health and preventing disease. Despite the known benefits of physical activity participation, many people in the United States (US) remain inactive. More specifically, physical activity behavior is socially patterned with lower participation rates among women, racial/ethnic minorities, sexual minority youth, individuals with less education, persons with physical, mental and cognitive disabilities, individuals > 65 years of age, and those living in the southeast region of the US. Many health-related outcomes follow a pattern that is similar to physical activity participation. In response to the problem of inequities in physical activity and overall health in the US, the American College of Sports Medicine (ACSM) has developed a national roadmap that supports achieving health equity through a physically active lifestyle. The actionable, integrated pathways that provide the foundation of ACSM's roadmap include: (1) communication: raising awareness of the issue and magnitude of health inequities, and conveying the power of physical activity in promoting health equity; (2) education: developing educational resources to improve cultural competency for healthcare providers and fitness professionals as well as developing new community-based programs for lay health workers; (3) collaboration: building partnerships and programs that integrate existing infrastructures and leverage institutional knowledge, reach, and voices of public, private and community organizations; and (4) evaluation: ensuring that ACSM attains measurable progress in reducing physical activity disparities to promote health equity. This paper provides a conceptual overview of these four pathways of ACSM's roadmap, an understanding of the challenges and advantages of implementing these components as well as the organizational and economic benefits of achieving health equity.
Article
Objective: To determine the face, content, and construct validity of the Stages of Exercise Scale (SOES) in children with rheumatologic conditions [juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM)], and if the validity of the SOES differs by disease type by comparing it with a disease control with a chronic respiratory illness [cystic fibrosis (CF)]. Methods: Sixty-seven children and adolescents (43 female) ages 11 to 18 years with a diagnosis of either JDM (n = 15), JIA (n = 39), or CF (n = 13) completed the SOES; scales of sensibility, process of change, decisional balance, and self-efficacy; the Child Health Assessment Questionnaire; and patient/physician ratings of disease severity. Physical activity was measured by an accelerometer. Relationships among SOES and measured constructs were determined by ANOVA and with logistical modeling. Results: SOES, decisional balance, and self-efficacy as well as behavioral and cognitive processes from the process of change demonstrated significant differences across the staging subgroups. Disease groups did not significantly differ on the scoring across the SOES. Children and adolescents in higher stages participated in more minutes of vigorous physical activity compared with those in the lower stages. Conclusion: The SOES demonstrated good face, content, and construct validity in children and adolescents with rheumatic disease.
Article
Objectives: The aim of this study was to compare habitual physical activity between cystic fibrosis (CF) patients and healthy controls and to investigate possible predictors for reduced physical activity in CF patients. Methods: Sixty-six patients with CF (3-44 years) and 65 healthy controls (3-55 years) were asked to wear an accelerometer (Actigraph GT1M) for 9 days (at least 10 hr/day). Physical activity was classified in five categories from very low to very strenuous. Results: In general, there was no difference in physical activity between CF patients and healthy controls. However, young school-aged 6-13 years old children with CF spent less time with strenuous and very strenuous activity than healthy controls (adjusted difference in activity -0.43 (-0.69, -0.17)). Patients with very low lung function were significantly less active, but other CF-associated conditions did not affect physical activity. Conclusions: While we found similar levels of physical activity measured by accelerometry in patients with CF compared to healthy controls in general, young school-aged children showed less engagement in strenuous activities than their healthy counterparts. As the reduced physical activity in young school children was not likely to be explained by the disease state, strenuous physical activity may be enhanced by advocating exercise and sport. Pediatr Pulmonol. 2016; 9999:1-11. © 2016 Wiley Periodicals, Inc.
Article
In this review, we develop a blueprint for exercise biology research in the new millennium. The first part of our plan provides statistics to support the contention that there has been an epidemic emergence of modern chronic diseases in the latter part of the 20th century. The health care costs of these conditions were almost two-thirds of a trillion dollars and affected 90 million Americans in 1990. We estimate that these costs are now approaching $1 trillion and stand to further dramatically increase as the baby boom generation ages. We discuss the reaction of the biomedical establishment to this epidemic, which has primarily been to apply modern technologies to stabilize overt clinical problems (e.g., secondary and tertiary prevention). Because this approach has been largely unsuccessful in reversing the epidemic, we argue that more emphasis must be placed on novel approaches such as primary prevention, which requires attacking the environmental roots of these conditions. In this respect, a strong association exists between the increase in physical inactivity and the emergence of modern chronic diseases in 20th century industrialized societies. Approximately 250,000 deaths per year in the United States are premature due to physical inactivity. Epidemiological data have established that physical inactivity increases the incidence of at least 17 unhealthy conditions, almost all of which are chronic diseases or considered risk factors for chronic diseases. Therefore, as part of this review, we present the concept that the human genome evolved within an environment of high physical activity. Accordingly, we propose that exercise biologists do not study "the effect of physical activity" but in reality study the effect of reintroducing exercise into an unhealthy sedentary population that is genetically programmed to expect physical activity. On the basis of healthy gene function, exercise research should thus be viewed from a nontraditional perspective in that the "control" group should actually be taken from a physically active population and not from a sedentary population with its predisposition to modern chronic diseases. We provide exciting examples of exercise biology research that is elucidating the underlying mechanisms by which physical inactivity may predispose individuals to chronic disease conditions, such as mechanisms contributing to insulin resistance and decreased skeletal muscle lipoprotein lipase activity. Some findings have been surprising and remarkable in that novel signaling mechanisms have been discovered that vary with the type and level of physical activity/inactivity at multiple levels of gene expression. Because this area of research is underfunded despite its high impact, the final part of our blueprint for the next millennium calls for the National Institutes of Health (NIH) to establish a major initiative devoted to the study of the biology of the primary prevention of modern chronic diseases. We justify this in several ways, including the following estimate: if the percentage of all US morbidity and mortality statistics attributed to the combination of physical inactivity and inappropriate diet were applied as a percentage of the NIH's total operating budget, the resulting funds would equal the budgets of two full institutes at the NIH! Furthermore, the fiscal support of studies elucidating the scientific foundation(s) targeted by primary prevention strategies in other public health efforts has resulted in an increased efficacy of the overall prevention effort. We estimate that physical inactivity impacts 80-90% of the 24 integrated review group (IRG) topics proposed by the NIH's Panel on Scientific Boundaries for Review, which is currently directing a major restructuring of the NIH's scientific funding system. Unfortunately, the primary prevention of chronic disease and the investigation of physical activity/inactivity and/or exercise are not mentioned in the almost 200 total subtopics comprising t
Article
An understanding of the habitual physical activity levels of children with chronic disorders is an important consideration relevant to both treatment and clinical monitoring (8). However, a feasible and useful tool for measuring the physical activity levels of children with chronic illness in clinical settings is not readily available. In this article, we review the development and initial psychometric testing (i.e., construct and reliability) of the Habitual Activity Estimation Scale (HAES), a measure developed for use in clinical research. A summary of these investigations suggests that the HAES appears both valid and reliable as a measure of activity in pediatric populations.
Article
The objectives of this study were to (i) assess sedentary time and prevalence of screen-based sedentary behaviours of children with a chronic disease and (ii) compare sedentary time and prevalence of screen-based sedentary behaviours to age- and sex-matched healthy controls. Sixty-five children (aged 6-18 years) with a chronic disease participated: survivors of a brain tumor, haemophilia, type 1 diabetes mellitus, juvenile idiopathic arthritis, cystic fibrosis and Crohn's disease. Twenty-nine of these participants were compared to age- and sex-matched healthy controls. Sedentary time was measured objectively by an ActiGraph GT1M or GT3X accelerometer worn for 7 consecutive days and defined as <100 counts per minute. A questionnaire was used to assess screen-based sedentary behaviours. Children with a chronic disease engaged in an average of 10.2±1.4 hours of sedentary time per day, which comprised 76.5±7.1% of average daily monitoring time. There were no differences between children with a chronic disease and controls in sedentary time (adjusted for wear time, p=0.06) or in the prevalence of TV watching, and computer or video game usage for varying durations (p=0.78, p=0.39 and, p=0.32 respectively). Children with a chronic disease, though relatively healthy, accumulate high levels of sedentary time, similar to those of their healthy peers.
Article
IntroductionRespiratory therapy is a keystone of the treatment for cystic fibrosis (CF) lung disease, but it is time consuming. Objectives We aimed to assess the total time spent on respiratory therapy, including chest physiotherapy (CPT) and physical activity (PA), as well as inhalation therapy (IT) and maintenance of materials (MM) to rationalise and optimise treatment. MethodsA cross-sectional prospective study in a paediatric CF cohort. A questionnaire was developed to look at the time spent on respiratory care over 3 months. Enrolled in this study are all CF patients aged from 6 to 16 years (the exclusion criterion was lung transplantation). ResultsOf the 40 enrolled patients, 22 participated (13 boys and 9 girls), with a mean age of 11 years. The patients spent approximately 19.46h per week (standard deviation7.53, 8.00-35.25h) on therapy: CPT (30.58%), IT (15.11%), PA (50%) and MM (4.32%), without statistical significance between sexes. Conclusion In our cohort, CF patients spent an average of nearly 20h a week in respiratory therapy, within a wide range of between 8h to almost 36h a week. PA consumes almost half of the time. Physicians have to take into consideration the burden of the treatment, to optimise the therapy.
Article
The use of physical activity monitors in population-based research has increased dramatically in the past decade. In this report, we review the major purpose for using physical activity monitors in different types of population-based studies (i.e., surveillance, intervention, association studies) and discuss the strengths and weaknesses for the various behavioral outcomes derived from monitors for each study type. We also update and extend previous recommendations for use of these instruments in large-scale studies, particularly with respect to selecting monitor systems in the context of technological advances that have occurred in recent years. The current state of the science with respect to optimal measurement schedules for use of physical activity monitors is also discussed. A checklist and flowchart are provided so that investigators have more guidance when reporting key elements of monitor use in their studies.
Article
Cystic fibrosis (CF) lung disease leads to progressive deterioration in exercise capacity. Because physical activity has been shown to improve lung function and quality of life (QoL), developing routine exercise programs can benefit this patient population. Lung function, nutritional status, and exercise capacity and assessments of habitual activity and QoL were measured before and after a two-month, subject-designed exercise regimen based on self-reported activity assessment. Statistical analysis included Wilcoxon signed-rank, Wilcoxon rank sum, and Fisher's exact tests. Subjects completing the study demonstrated significant improvement in exercise capacity and body image perception, a CF-specific QoL measure (p<0.001). In secondary analyses, subjects improving exercise capacity showed significant increases in lung function and self-reported habitual activity. Increases in exercise capacity over a two-month period resulted in significantly improved lung function and self-reported habitual activity. Longer, controlled trials are needed to develop individualized exercise recommendations.
Article
Regular exercise and habitual physical activity are important for patients with cystic fibrosis (CF). Research has demonstrated the benefits of aerobic, anaerobic, and strength exercise training programs for health and quality of life, however, the CF patient is faced with unique barriers and challenges to participation. Recently, increased levels of habitual physical activity have been shown to slow the decline in lung function in patients with CF, and regular participation in a variety of activities may result in greater adherence in the long term. Research is now available to justify the incorporation of exercise into the routine care of patients with CF. This paper provides the background and rationale for the implementation of exercise and habitual physical activity recommendations by the health care team. Education of health care providers regarding the importance of exercise and habitual physical activity for patients with CF is needed in order for exercise and physical activity to be incorporated as key components of clinical practice and into the lives of patients with CF.
Article
The aim of the present study was to investigate the prevalence of muscle weakness and the importance of physical inactivity in cystic fibrosis (CF), and its relationship to exercise tolerance and muscle strength. Exercise tolerance, skeletal and respiratory muscle strength were studied in a group of 64 adults with CF (age 26+/-8 yrs, FEV(1 % predicted) 65+/-19) and in 20 age-matched controls. Physical activity (PA) was assessed in 20 patients and all controls. Quadriceps muscle weakness was present in 56% of the patients. Peak oxygen uptake and 6-min walking distance were below normal in 89 and 75% of patients, respectively. Respiratory muscle strength was normal. The differences remained after correcting for PA. Quadriceps force was correlated to the 6-min walking distance but not to peak oxygen uptake. "Mild" PA (>3 metabolic equivalents (METS)) and the number of steps overlapped with controls, but CF patients had less moderate PA (>4.8 METS). Moderate PA was related to peak oxygen uptake and quadriceps force. Skeletal muscle weakness and exercise intolerance are prevalent in cystic fibrosis. Physical inactivity is a factor significantly contributing to exercise tolerance and skeletal muscle force in adults with cystic fibrosis, but these impairments are in excess to that expected from physical inactivity only.
Article
Sex differences, age at diagnosis, type and severity of symptoms at presentation, pulmonary function, and radiologic findings are prognostic factors that enable the clinician to place patients in various risk groups. The definition and characteristics of these groups will aid in the assessment, counseling, and follow up of patients with cystic fibrosis.
Article
Previous studies have shown female sex, impaired pulmonary function, older age, malnutrition, and colonization of the respiratory tract with Pseudomonas cepacia to be associated with a poor prognosis in patients with cystic fibrosis. We sought to determine the prognostic value of exercise testing in addition to the other prognostic factors. A total of 109 patients with cystic fibrosis, 7 to 35 years old, underwent pulmonary-function and exercise testing in the late 1970s. They were followed for eight years to determine the factors associated with subsequent mortality. Survival rates were calculated with standard life-table methods. Cox proportional-hazards regression models were used to determine crude relative risks of mortality and relative risks adjusted for age, sex, body-mass index, forced expiratory volume in one second (FEV1) end-tidal partial pressure of carbon dioxide (PCO2) at peak exercise, and oxygen consumption at peak exercise (VO2 peak). Patients with the highest levels of aerobic fitness (VO2 peak, > or = 82 percent of predicted) had a survival rate of 83 percent at eight years, as compared with rates of 51 percent and 28 percent for patients with middle (VO2 peak, 59 to 81 percent of predicted) and lowest (VO2 peak, < or = 58 percent of predicted) levels of fitness, respectively. After adjustment for other risk factors, patients with higher levels of aerobic fitness were more than three times as likely to survive than patients with lower levels of fitness. Colonization with P. cepacia was associated with a risk of dying that was increased fivefold. Age, sex, body-mass index, FEV1, and end-tidal PCO2 at peak exercise were not independently correlated with mortality. Higher levels of aerobic fitness in patients with cystic fibrosis are associated with a significantly lower risk of dying. Although better aerobic fitness may simply be a marker for less severe illness, measurement of VO2 peak appears to be valuable for predicting prognosis. Further research is warranted to determine whether improving aerobic fitness through exercise programs will result in a better prognosis.
Article
There was a need to design a questionnaire that could accurately assess the activity patterns of Native Americans to evaluate the relationship between physical activity and diabetes. Such a questionnaire was developed and implemented into the data collection scheme of the prospective Pima Indian Study of Arizona. The questionnaire, which assesses historical, past-year, and past-week leisure and occupational activity, was examined in 29 Pima individuals aged 21-36 yr and was shown to be reliable with test-retest correlations (rank-order correlations ranged from 0.62 to 0.96 for leisure and occupational activity). Reproducibility of the past-year leisure physical-activity estimate was determined in 69 participants aged 10-59 yr and was found to be reliable in all age-groups with the exception of the 10- to 14-yr-old age-group (rank-order correlations were 0.31 in the 10- to 14-yr-old age-group compared to 0.88 to 0.92 in those greater than 20 yr of age). Validity of the current-activity section of the questionnaire was demonstrated indirectly through comparisons with activity monitors. The past-week leisure-activity estimate was related to the Caltrac activity monitor counts per hour (rho = 0.62, P less than 0.05, n = 17). In summary, a physical-activity questionnaire has been developed that is both reliable and feasible to use in the Pima Indian population to evaluate the relationship of physical activity to non-insulin-dependent diabetes mellitus.
Article
Traditional outcome measures in CF include PFTs, exercise tests, and several scoring systems that depend on pulmonary status and are largely subjective. The Quality of Well-being scale (QWB) is a widely used tool for measuring quality of life by three subscales: mobility, physical activity, and social activity, with points assigned within each subscale. The QWB has been shown to be valid in patients with COPD. We administered the QWB scale to 44 patients with CF, aged 7 to 36 years, and examined the relationship between QWB and PFTs, and in 15 patients the QWB vs exercise performance (peak VO2) on a progressive cycle ergometer test. QWB was significantly correlated with each variable examined: QWB vs FEV1, r=0.5518 (p<.0001); QWB vs FEF25-75%, r = 0.4793 (p<.001); QWB vs PEFR, r=0.4018 (p<.01); QWB vs peak Vo2, r=0.5778 (p<.01). The QWB scale is an objective measure that is significantly correlated with measures of performance and pulmonary function in CF. The relationship is not one of identity; further, the QWB is broad based and takes into account general well-being, not just pulmonary health, adding an important dimension to the care of patients with CF. (Chest 1989; 95:344-47) QWB = quality of well-being; SEP = Sickness Impact Profile
Article
"Physical activity," "exercise," and "physical fitness" are terms that describe different concepts. However, they are often confused with one another, and the terms are sometimes used interchangeably. This paper proposes definitions to distinguish them. Physical activity is defined as any bodily movement produced by skeletal muscles that results in energy expenditure. The energy expenditure can be measured in kilocalories. Physical activity in daily life can be categorized into occupational, sports, conditioning, household, or other activities. Exercise is a subset of physical activity that is planned, structured, and repetitive and has as a final or an intermediate objective the improvement or maintenance of physical fitness. Physical fitness is a set of attributes that are either health- or skill-related. The degree to which people have these attributes can be measured with specific tests. These definitions are offered as an interpretational framework for comparing studies that relate physical activity, exercise, and physical fitness to health.
Article
Increased energy expenditure, poor dietary intake, and fat malabsorption in patients with cystic fibrosis (CF) frequently lead to growth failure and malnutrition, which are associated with pulmonary failure and decreased survival. The study purpose was to understand better the energy expenditure and requirements in the mild pulmonary disease state in children. Resting and total energy expenditure were measured in 6- to 9-yr-old, pancreatic-insufficient children with CF (n = 25) and control children (n = 25) of similar age, gender, and weight. The effect of the most common genotype, homozygous delta F508, on energy expenditure was also investigated. Dietary intake, degree of fat malabsorption, body composition, physical activity, and clinical status were determined. The CF group had a 9% increase in resting energy expenditure, which was not related to genotype or severity of lung disease. Both CF genotype subgroups (delta F508 homozygous and all others) had a similar, modest resting energy expenditure increase. Total energy expenditure was increased by 12% in the entire CF group and by 23% in the delta F508 homozygous CF subgroup compared with controls. The total energy expenditure increase in delta F508 homozygous children may be related to increased voluntary physical activity, reflecting no activity reduction associated with lung disease, or to an unidentified genotype-related mechanism. The clinical implication is that a detailed physical activity assessment should be evaluated along with resting energy expenditure, either measured or estimated by equations, when daily energy needs are being determined for children with CF.
Article
The mechanisms underlying the initiation of lung disease and early respiratory morbidity in cystic fibrosis (CF) are poorly understood. By identifying infants with CF through a statewide neonatal screening program, we investigated whether airway inflammation was present in these infants, with the goal of furthering our understanding of the early events in this lung disease. Bronchoalveolar lavage fluid (BALF) from 16 infants with CF (mean age, 6 mo) and 11 disease control infants (mean age, 12 mo) was examined for the following inflammatory parameters: (1) neutrophil count; (2) activity of free neutrophil elastase; (3) elastase/alpha 1-antiprotease inhibitor complexes; and (4) the level of interleukin-8 (IL-8). We also quantified the spontaneous level of expression of IL-8 mRNA transcripts by airway macrophages. Each index of airway inflammation was increased in the BALF of infants with CF as compared with control infants. In addition, both the number of neutrophils and IL-8 levels were increased in infants with CF who had negative cultures (n = 7) for common bacterial CF-related pathogens, as well as for common respiratory viruses and fungi at the time of bronchoalveolar lavage (BAL). These findings suggest that airway inflammation is already present in infants with CF who are as young as 4 wks. Furthermore, although many different cell types (e.g., epithelial cells) may express IL-8, airway macrophages appear to be a source of this chemokine, and may thus play a prominent role in early neutrophil influx into the lung.