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Spirometry and peak cough flow testing (PCF) are commonly used in the respiratory assessment of children with a neuromuscular disorder (NMD). Testing uses two different machines, increases laboratory time, costs and resource utilisation. No studies have assessed the correlation between peak expiratory flow (PEF) obtained from spirometry and PCF in children with NMD using one device. An audit of children with a NMD managed at the Children's Hospital at Westmead in 2022-2024 aged < 20 years who performed spirometry and PCF testing on the same device (Vyaire Body BoxTM, Ultrasonic flow meter-based, or Vyaire PneumotachographTM, Pneumotach flow meter-based; Germany) was conducted to assess the correlation between PCF and PEF. Fifty-one sets of testing were identified, and 40 subjects (9F) had reproducible testing and were included. Median (range) age was 14.95 (7.20-19.00) years. Median PEF (L/min) was 4.05 (1.22-10.26) and median PCF (L/min) was 4.29 (1.69-10.82). PEF and PCF had a strong Pearson's correlation coefficient, (R = 0.97, p = 0.03). The coefficient of determination was 0.93. If laboratory resources permit, spirometry should be the test of choice for children with NMD. On average, spirometry required multiple practices to achieve reproducibility to meet ATS/ERS standards. PCF testing can be utilised for children where performing technically acceptable spirometry is not possible.

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With increasing competitiveness across the sporting landscape, there is a need for more research into monitoring and managing the young athlete, as the needs of a young athlete are vastly different to those of an older athlete who is already established in their respective sport. As the age of sports specialisation seems to decrease, exercise testing in the younger cohort of athletes is crucial for safety and long-term success. This article provides a comprehensive summary of available testing and monitoring methods that can be used to assist young athletes as they mature and attempt to excel in their chosen sport.

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BACKGROUND: Clinical and prognostic value of preschool Multiple Breath Washout (MBW) remains unclear. METHODS: Initial MBW results (Exhalyzer® D, EcoMedics AG) in preschool Cystic Fibrosis (CF) subjects (age 2-6 years) at a time of clinical stability were compared to (1) concurrent clinical status measures and (2) later spirometry outcomes. Abnormal Lung Clearance Index (LCI) was defined using published reference data (ULN for LCI 8.0). RESULTS: LCI was abnormal in 56% (28/50), with mean (SD) LCI 8.61(1.85) at age 4.71(1.3) years. Abnormal LCI was associated with higher dornase alfa use, previous positive bacterial cultures and pF508.del homozygous genotype. Later spirometry (n = 44; mean (SD) 2.3(0.5) years after MBW) demonstrated that abnormal initial preschool LCI was a strong predictor of lower later spirometry outcomes. CONCLUSION: Abnormal preschool LCI was associated with concurrent measures of clinical status and later spirometry deficits, suggesting early prognostic utility of MBW testing in this age range.

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OBJECTIVES: To evaluate the feasibility of measuring habitual physical activity (HPA) in children with cystic fibrosis (CF) and to assess the relation between HPA and the rate of decline in FEV1 over a period of 2 years. STUDY DESIGN: At regular clinic visits, 109 patients (7 to 17 years; 56 girls) performed pulmonary function testing and completed the Habitual Activity Estimation Scale (HAES). Patients were divided into quartiles, based on activity levels derived from the HAES. RESULTS: Girls in the two lowest activity quartiles had a more rapid rate of decline FEV1 (-3.40% and -3.05% predicted, respectively) than girls in the two highest activity quartiles (-0.93% and +1.17% predicted, respectively) (P = .02). In boys, the rate of decline of FEV1 was similar in all activity quartiles (-1.95% predicted). Patients reported significantly more activity in summer compared with spring, winter, and fall (P < .0001), and boys reported greater activity than girls (6.5 +/- 2.9 vs 5.4 +/- 2.5 h/d, P < .05). CONCLUSIONS: The annual rate of change of FEV1 was related to activity quartile in girls but not in boys. This research suggests that an inactive lifestyle may partially explain the poorer survival of female patients with CF. The HAES is a feasible tool for routine follow-up of HPA in our CF clinic.

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BACKGROUND: Carbon dioxide (CO2) retention during exercise is uncommon in mild to moderate lung disease in cystic fibrosis (CF). The ability to deal with increased CO2 is dependent on the degree of airflow limitation and inherent CO2 sensitivity. CO2 retention (CO2R) can be defined as a rise in P(ET)CO2 tension of > or =5 mm Hg with exercise together with a failure to reduce P(ET)CO2 tension after peak work by at least 3 mm Hg by the termination of exercise. AIM: To ascertain if carbon dioxide retention during exercise is associated with more rapid decline in lung function. METHODS: Annual spirometric and exercise data from 58 children aged 11-15 years, with moderate CF lung disease between 1996 and 2002 were analysed. RESULTS: The mean FEV1 at baseline for the two groups was similar; the CO2R group (n = 15) was 62% and the non-CO2 retention group (CO2NR) was 64% (n = 43). The decline in FEV1 after 12 months was -3.2% (SD 1.1) in the CO2R group and -2.3% (SD 0.9) in the CO2NR group. The decline after 24 months was -6.3% (SD 1.3) and -1.8% (SD 1.1) respectively. After 36 months, the decline in FEV1 was -5.3% (SD 1.2) and -2.6% (SD 1.1) respectively. The overall decline in lung function was 14.8% (SD 2.1) in the CO2R group and 6.7% (SD 1.8) in the CO2NR group. Using the primary outcome measure as a decline in FEV(1) of >9%, final multivariate analysis showed that the relative risks for this model were (95% CIs in parentheses): DeltaP(ET)CO2 11.61 (3.41 to 24.12), peak VO2 1.23 (1.10 to 1.43), and initial FEV(1) 1.14 (1.02 to 1.28). CONCLUSION: Results show that the inability to defend carbon dioxide during exercise is associated with a more rapid decline in lung function.

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AIMS: (1) To compare habitual activity levels in prepubescent and pubescent boys and girls with different degrees of CF lung disease severity and healthy controls. (2) To assess the relation between habitual activity levels and measures of fitness, lung function, nutrition, pancreatic status, and quality of life. METHODS AND RESULTS: A total of 148 children (75 girls and 73 boys) with CF and matched controls were studied. Regardless of disease severity, there were no differences in habitual activity between prepubescent boys and girls with CF. Pubescent boys with CF were significantly more active than girls with the same degree of disease severity. There were no significant differences in habitual activity between prepubescent children with CF and controls. Pubescent children with mild CF were significantly more active than controls, but those with moderate to severe disease were less active than controls. The best correlates with habitual activity levels were anaerobic power, aerobic capacity, and quality of life. In children with moderate to severe disease, nutrition status correlated significantly with activity levels. The impact of pancreatic status on activity levels and other measures of fitness was most apparent in pubescent girls. CONCLUSION: Gender differences in habitual activity were evident only after the onset of puberty. The impact of pancreatic insufficiency on measures of fitness and habitual activity was greatest in pubescent females. The reason for this gender difference may be an interplay of genetic, hormonal, and societal factors and is the focus of a longitudinal study.

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BACKGROUND: Non-invasive positive pressure ventilation (NPPV) has a beneficial effect on nocturnal hypoventilation and hospitalisation rates in adults with static or slowly progressive neuromuscular disease and respiratory failure. Its role in children affected with similar disease processes, however, remains unclear. AIMS: To investigate the impact of NPPV on hospitalisations and sleep related respiratory parameters in children with neuromuscular disease. METHODS: Fifteen children (mean age 11.7, range 3.4-17.8 years) diagnosed with neuromuscular disease who had been started on nocturnal NPPV and had at least one year of follow up since the initiation of such therapy were studied. Patients served as their own controls and comparison was made of the years preceding and following the initiation of NPPV. RESULTS: Children spent 85% fewer days in hospital (mean pre-NPPV 48.0 days, mean post-NPPV 7.0 days) and 68% less days in intensive care after initiation of NPPV (mean pre-NPPV 12.0 days, mean post-NPPV 3.9 days). Sleep study parameters including number of desaturations, apnoea-hypopnoea index and transcutaneous pCO2 levels improved after initiation of NPPV. CONCLUSIONS: NPPV can decrease hospitalisations for children with neuromuscular disease and improves sleep related respiratory parameters. A prospective study is now needed to further delineate the role of NPPV in this population of children.

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The aim of this study was to compare aerobic and resistance training in children with cystic fibrosis (CF) admitted to hospital with an intercurrent pulmonary infection with a control group. The subjects were randomized into three groups on the first day of admission. The fat-free mass (FFM) was calculated, using the skin fold thickness from four sites (biceps, triceps, subscapular, and iliac crest). Pulmonary function tests were performed within 36 hr of admission and repeated on discharge from the hospital, and again at 1 month after discharge. All subjects performed an incremental treadmill exercise test, using a modified Bruce protocol. Lower limb strength was measured using a Cybex dynamometer. An assessment of quality of life was made using the Quality of Well Being Scale, as previously reported. Activity levels were measured using a 7-day activity diary, and subjects also wore an accelerometer on their hips. There were no significant differences between the three groups in terms of disease severity, and length of stay in hospital. Subjects in all three groups received intravenous antibiotics and nutritional supplementation as determined by the physician. Children randomized to the aerobic training group participated in aerobic activities for five sessions, each of 30-min duration, a week. The children randomized to the resistance training group exercised both upper and lower limbs against a graded resistance machine. Subjects in the control group received standard chest physiotherapy. Our study demonstrated that children who received aerobic training had significantly better peak aerobic capacity, activity levels, and quality of life than children who received the resistance training program. Children who received resistance training had better weight gain (total mass, as well as fat-free mass), lung function, and leg strength than children who received aerobic training. A combination of aerobic and resistance training may be the best training program, and future studies to assess optimal training programs for CF patients are indicated.

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Leukotriene antagonists are a new class of anti-inflammatory drugs which have shown clinical efficacy in the management of asthma. However, their role in paediatric asthma is still unclear. In essence, while there are theoretical reasons as to why leukotriene antagonists would be of use in the management of childhood asthma, there is little clinical data on their use in this patient group. Studies with leukotriene antagonists to date have been performed in children with chronic 'undertreated' asthma which, under current recommendations, should be treated with inhaled corticosteroids. Furthermore, the magnitude of effect of leukotriene antagonists on lung function (forced expiratory volume in one second of less than 5% better than placebo) and daily symptoms (0.23 puffs per day of B2 agonist less than placebo), while reaching statistical significance, is unlikely to be of clinical significance in children with chronic undertreated asthma. There is, however, good evidence for leukotriene antagonist use in exercise induced asthma in children. We conclude that although leukotriene antagonists may play an important role in the management of childhood asthma in the future, particularly as corticosteroid sparing agents and in exercise induced asthma, clinical data in paediatric asthma is poor.

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The organs of a higher plant show two fundamental axes of asymmetry: proximodistal and dorsoventral. Dorsoventrality in leaves, bracts, and petal lobes of Antirrhinum majus requires activity of the PHANTASTICA (PHAN) gene. Conditional mutants revealed that PHAN is also required for earlier elaboration of the proximodistal axis. PHAN was isolated and shown to encode a MYB transcription factor homolog. PHAN mRNA is first detected in organ initials before primordium initiation. The structure and expression pattern of PHAN, together with its requirement in two key features of organ development, are consistent with a role in specifying lateral organ identity as distinct from that of the stem or meristem. PHAN also appears to maintain meristem activity in a non-cell-autonomous manner.

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