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.

Gender differences in lung volumes and flow rates, and in respiratory control have been documented previously. How these gender differences affect exercise responses in normal subjects is less clear, particularly as many studies involved highly fit subjects. This study aimed to investigate potential gender differences occurring during progressive exercise in healthy males and females of average fitness. Fourteen males and ten females of mean (SD) age 23 (0.35) years completed a progressive exercise test to exhaustion on a cycle ergometer, with a ramp increase of 15 W min(-1) (female) or 20 W min(-1) (male). All females were studied during the follicular phase of their menstrual cycle. Cardiorespiratory variables were measured, breath by breath, and values were compared at rest, at 40 W, at physiologically equivalent workloads below, at and above the gas exchange threshold and at peak oxygen uptake (VO(2peak)). Mean VO(2peak) (SEM) was 32.4 (2.01) ml kg(-1) min(-1) for the females and 41.9 (1.80) ml kg(-1) min(-1) for the males. Females had a significantly lower end-tidal partial CO(2) pressure at rest and throughout exercise. Increases in exercise minute ventilation were achieved by a significantly greater tidal volume in males, whereas females adopted a significantly greater breathing frequency. Ratings of respiratory discomfort were significantly greater in the male group at physiologically equivalent workloads compared to the female group. This study shows gender differences exist in the ventilatory and sensory response to progressive exercise in untrained subjects. Further work is required to ascertain if these effects are altered during the luteal phase of the menstrual cycle.

Nasal nitric oxide (nNO) is extremely low in most people with primary ciliary dyskinesia (PCD) and its measurement is an important contributor to making the diagnosis. Existing guidelines and technical standards focus on nNO measurements in older, cooperative children using chemiluminescent analysers. However, measurements of nNO in pre-school children (2–5 years) may facilitate early diagnosis, and electrochemical rather than chemiluminescence analysers are widely used. Pre-schoolers often need different methods to be employed when measuring nNO. Hence a European Respiratory Society Task Force has developed this technical standard as the first step towards standardising sampling, analysis, and reporting of nNO measured as part of the diagnostic testing for PCD in all age groups including preschool-age children. Furthermore, we considered both chemiluminescence and electrochemical analysers that are in use worldwide. There was paucity of quality evidence for electrochemical analysers and sampling methods used in young children, and this manuscript proposes future research priorities to allow updates of this technical standard.

Airway nitric oxide (NO) is a ubiquitous signaling molecule with bronchoprotective, anti-inflammatory, and anti-infective roles. Cystic fibrosis (CF) is a chronic lung condition associated with deceased exhaled NO. Strategies to increase exhaled NO in CF have yielded inconsistent results. A potential new method of increasing systemic NO involves ingestion of dietary, inorganic nitrate which is reduced to nitrite and NO. We present the case of a 12-year-old, athletic boy with CF who demonstrated acute but marked increases in exhaled NO following dietary nitrate consumption compared to placebo

Nasal nitric oxide (nNO) measurements are routinely used in the assessment of patients suspected to have primary ciliary dyskinesia (PCD), but recommendations for performing such measurements have not focused on children and do not include all current practices. To guide the development of a European Respiratory Society-supported technical standard for nNO measurements in children, an international online survey was conducted to better understand current practices for measuring nNO among providers involved in PCD diagnostics.Seventy-eight professionals responded, representing 65 centres across 18 countries, mainly located in Europe and North America. Nearly all centres measured nNO in children and more than half of them performed measurements before 5-years of age. The test was often postponed in children with signs of acute airway infection. In Europe, the electrochemical technique was more frequently used than chemiluminescence. A similar proportion of centres performed measurements during exhalation against a resistance (49/65) or during tidal breathing (50/65) with 15 centres using only exhalation against a resistance and 15 centres using only tidal breathing. The cut-off values used to discriminate PCD was consistent across centres using chemiluminescence analyzers and these centres reported results as an output (nL.min−1). However, cut-off values were highly variable across centres using electrochemical devices, and nNO concentrations were typically reported as ppb.This survey represents the first to determine real-world use of nNO measurements worldwide and revealed remarkable variability in methodology, equipment, and interpretation. These findings will be useful to standardise methods and training.