Longitudinal relationship between physical activity and lung health in patients with cystic fibrosis.

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 using the Habitual Activity Estimation Scale, forced expiratory volume in 1 s (FEV₁) and Stage I exercise tests in 212 patients with CF over a 9-year period.

Physiological correlates of pulmonary function in children with cystic fibrosis.

Rationale: Although peak aerobic capacity (VO(2peak)) has been linked to outcome in patients with cystic fibrosis (CF), measuring is time consuming, and requires expensive equipment and expertise that is not readily available in all centers. Other fitness parameters such as peak anaerobic power, measures of power and strength may be simpler to deliver in the clinic. The relationship between these measures and established outcomes such as forced expiratory volume in one second (FEV(1)) and peak aerobic power (VO(2peak)) in CF remains unclear.

Skeletal muscle metabolism in cystic fibrosis and primary ciliary dyskinesia.

Previous studies have reported differences in muscle function and metabolism between patients with cystic fibrosis (CF) and healthy controls (HC), but it is currently unknown whether these abnormalities are specific to CF or also seen in other airway diseases. In this study, we used magnetic resonance spectroscopy (MRS) during exercise to assess muscle metabolism in CF patients. Twenty patients with CF and 20 age, gender, and habitual activity-matched HCs and a respiratory disease comparison group with primary ciliary dyskinesia (PCD; n = 10) were studied.

Exercise and physical activity in children with cystic fibrosis.

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.

Assessment of body composition in pediatric patients with cystic fibrosis.

Rationale: Cystic fibrosis (CF) leads to pathological changes in organs that express the cystic fibrosis transmembrane conductance regulator (CFTR), including secretory cells of the digestive tract and the pancreas. Maintaining nutritional sufficiency is challenging for CF patients and therefore accurate monitoring is important for their clinical management.

Purpose: The objectives of this study were to evaluate the effectiveness of skinfold measurements as an accurate method for determining body composition (fat mass (FM) and lean body mass (LBM)) of this population, using dual-energy X-ray absorptiometry (DEXA) as a gold standard comparison and to determine the most accurate equation for this calculation in children with CF.

Reliability and validity of the habitual activity estimation scale (HAES) in patients with cystic fibrosis.

Purpose: To understand potential benefits of exercise in the cystic fibrosis (CF) population, there needs to be accurate methods to quantify it. The Habitual Activity Estimation Scale (HAES) questionnaire has been shown to be a feasible tool to measure physical activity however the reliability and validity have yet to be determined in the CF population.

Methods: Fourteen (seven male, seven female) patients aged 16.

Fetal response to maternal exercise in pregnancies with uteroplacental insufficiency.

Objective: The purpose of this study was to determine the fetal response to submaximal maternal exercise at 22 to 26 weeks in pregnancies with abnormal uterine artery Doppler.

Study Design: This was a prospective comparison of singleton pregnancies with uteroplacental vascular insufficiency (UPVI) (mean uterine pulsatility index [PI] values >1.45 [n = 12]) and those with normal uterine artery Doppler (n = 23).

Determination of maximal voluntary ventilation in children with cystic fibrosis.

Maximal voluntary ventilation (MVV) may be determined directly by the sprint method or calculated from pulmonary function data, using the functions MVV = forced expired volume in 1 sec (FEV(1)) x 35 or MVV = FEV(1) x 40. The purpose of this paper was to test the validity of the equation over a wide range of lung function in children. Cystic fibrosis (CF), a chronic lung disease where children typically have a wide range of pulmonary function, was chosen as the study requirement.