The Influence of Acute Hypoxia on Oxygen Uptake and Muscle Oxygenation Kinetics During Cycling Exercise in Prepubertal Boys.

PURPOSE: To examine the effect of normobaric hypoxia on pulmonary oxygen uptake (V˙O2) and muscle oxygenation kinetics during incremental and moderate-intensity exercise in children. METHODS: Eight prepubertal boys (9-11 y) performed incremental cycle tests to exhaustion in both normoxia and hypoxia (fraction of inspired O2 of 15%) followed by repeat 6-minute transitions of moderate-intensity exercise in each condition over subsequent visits. RESULTS: Maximal oxygen uptake (V˙O2max) was reduced in hypoxia compared with normoxia (1.69 [0.20] vs 1.87 [0.26] L·min-1, P = .028), although the gas exchange threshold was not altered in absolute terms (P = .33) or relative to V˙O2max (P = .78). During moderate-intensity exercise, the phase II V˙O2 time constant (τ) was increased in hypoxia (18 [9] vs 24 [8] s, P = .025), with deoxyhemoglobin τ unchanged (17 [8] vs 16 [6], P ≥ .28). CONCLUSIONS: In prepubertal boys, hypoxia reduced V˙O2max and slowed V˙O2 phase II kinetics during moderate-intensity exercise, despite unchanged deoxyhemoglobin kinetics. These data suggest an oxygen delivery dependence of V˙O2max and moderate-intensity V˙O2 kinetics under conditions of reduced oxygen availability in prepubertal boys.

Predatory publishing in medical education: a rapid scoping review.

BACKGROUND: Academic publishing is a cornerstone of scholarly communications, yet is unfortunately open to abuse, having given rise to 'predatory publishers'- groups that employ aggressive marketing tactics, are deficient in methods and ethics, and bypass peer review. Preventing these predatory publishers from infiltrating scholarly activity is of high importance, and students must be trained in this area to increase awareness and reduce use. The scope of this issue in the context of medical students remains unknown, and therefore this sought to examine the breadth of the current literature base. METHODS: A rapid scoping review was undertaken, adhering to adapted PRISMA guidelines. Six databases (ASSIA, EBSCO, Ovid, PubMed, Scopus, Web of Science) were systematically searched for content related to predatory publishing and medical students. Results were single-screened, facilitated by online reviewing software. Resultant data were narratively described, with common themes identified. RESULTS: After searching and screening, five studies were included, representing a total of 1338 students. Two predominant themes- understanding, and utilisation- of predatory publishers was identified. These themes revealed that medical students were broadly unaware of the issue of predatory publishing, and that a small number have already, or would consider, using their services. CONCLUSION: There remains a lack of understanding of the threat that predatory publishers pose amongst medical students. Future research and education in this domain will be required to focus on informing medical students on the issue, and the implication of engaging with predatory publishers.

Validity and repeatability of cardiopulmonary exercise testing in interstitial lung disease.

BACKGROUND: Cardiopulmonary exercise testing (CPET), and its primary outcome of peak oxygen uptake (VO2peak), are acknowledged as biomarkers in the diagnostic and prognostic management of interstitial lung disease (ILD). However, the validity and repeatability of CPET in those with ILD has yet to be fully characterised, and this study fills this evidence gap. METHODS: Twenty-six people with ILD were recruited, and 21 successfully completed three CPETs. Of these, 17 completed two valid CPETs within a 3-month window, and 11 completed two valid CPETs within a 6-month window. Technical standards from the European Respiratory Society established validity, and repeatability was determined using mean change, intraclass correlation coefficient and typical error. RESULTS: Every participant (100%) who successfully exercised to volitional exhaustion produced a maximal, and therefore valid, CPET. Approximately 20% of participants presented with a plateau in VO2, the primary criteria for establishing a maximal effort. The majority of participants otherwise presented with secondary criteria of respiratory exchange ratios in excess of 1.05, and maximal heart rates in excess of their predicted values. Repeatability analyses identified that the typical error (expressed as percent of coefficient of variation) was 20% over 3-months in those reaching volitional exhaustion. CONCLUSION: This work has, for the first time, fully characterised how patients with ILD respond to CPET in terms of primary and secondary verification criteria, and generated novel repeatability data that will prove useful in the assessment of disease progression, and future evaluation of therapeutic regimens where VO2peak is used as an outcome measure.

The Exeter Activity Unlimited statement on physical activity and exercise for cystic fibrosis: methodology and results of an international, multidisciplinary, evidence-driven expert consensus.

BACKGROUND: The roles of physical activity (PA) and exercise within the management of cystic fibrosis (CF) are recognised by their inclusion in numerous standards of care and treatment guidelines. However, information is brief, and both PA and exercise as multi-faceted behaviours require extensive stakeholder input when developing and promoting such guidelines. METHOD: On 30th June and 1st July 2021, 39 stakeholders from 11 countries, including researchers, healthcare professionals and patients participated in a virtual conference to agree an evidence-based and informed expert consensus about PA and exercise for people with CF. This consensus presents the agreement across six themes: (i) patient and system centred outcomes, (ii) health benefits, iii) measurement, (iv) prescription, (v) clinical considerations, and (vi) future directions. The consensus was achieved by a stepwise process, involving: (i) written evidence-based synopses; (ii) peer critique of synopses; (iii) oral presentation to consensus group and peer challenge of revised synopses; and (iv) anonymous voting on final proposed synopses for adoption to the consensus statement. RESULTS: The final consensus document includes 24 statements which surpassed the consensus threshold (>80% agreement) out of 30 proposed statements. CONCLUSION: This consensus can be used to support health promotion by relevant stakeholders for people with CF.

Desaturation during exercise is not a sufficient mechanism for prediction of osteoporosis in non-cystic fibrosis bronchiectasis.

BACKGROUND: Recent research has proposed an association between desaturation during a six minute walking test (6MWT) and osteoporosis in an elderly group of individuals with non-cystic fibrosis bronchiectasis. A causative pathway through activation of hypoxia-inducible factor 1-alpha (HIF-1α) has been proposed. COMMENTARY: Queries regarding the statistical approaches used are identified and discussed within this correspondence. These predominate around the use of linear regression models to predict osteoporosis in a group that is already osteoporotic, presenting with extreme values for bone mineral density (BMD). Further queries are raised regarding the HIF-1α pathway, and physical activity (PA) is proposed as an upstream mechanism for both reduced exercise tolerance and low BMD. CONCLUSIONS: It is suggested that osteoporosis cannot be predicted in a group that is already osteoporotic, and that PA is likely to be the causative mechanism between desaturation in the 6MWT and low BMD in non-cystic fibrosis bronchiectasis.

A web-based intervention to promote physical activity in adolescents and young adults with cystic fibrosis: protocol for a randomized controlled trial.

BACKGROUND: Regular participation in physical activity by people with cystic fibrosis (CF) promotes positive clinical and health outcomes including reduced rate of decline in lung function, fewer hospitalizations and greater wellbeing. However adherence to exercise and activity programs is low, in part due to the substantial daily therapy burden for young people with CF. Strict infection control requirements limit the role of group exercise programs that are commonly used in other clinical groups. Investigation of methods to promote physical activity in this group has been limited. The Active Online Physical Activity in Cystic fibrosis Trial (ActionPACT) is an assessor-blinded, multi-centre, randomized controlled trial designed to compare the efficacy of a novel web-based program (ActivOnline) compared to usual care in promoting physical activity participation in adolescents and young adults with CF. METHODS: Adolescents and young adults with CF will be recruited on discharge from hospital for a respiratory exacerbation. Participants randomized to the intervention group will have access to a web-based physical activity platform for the 12-week intervention period. ActivOnline allows users to track their physical activity, set goals, and self-monitor progress. All participants in both groups will be provided with standardised information regarding general physical activity recommendations for adolescents and young adults. Outcomes will be assessed by a blinded assessor at baseline, after completion of the intervention, and at 3-months followup. Healthcare utilization will be assessed at 12 months from intervention completion. The primary outcome is change in moderate-to-vigorous physical activity participation measured objectively by accelerometry. Secondary outcomes include aerobic fitness, health-related quality of life, anxiety and depression and sleep quality. DISCUSSION: This trial will establish whether a web-based application can improve physical activity participation more effectively than usual care in the period following hospitalization for a respiratory exacerbation. The web-based application under investigation can be made readily and widely available to all individuals with CF, to support physical activity and exercise participation at a time and location of the user's choosing, regardless of microbiological status. TRIAL REGISTRATION: Clinical trial registered on July 13, 2017 with the Australian and New Zealand Clinical Trials Register at (ACTRN12617001009303).

Validity of the Supramaximal Test to Verify Maximal Oxygen Uptake in Children and Adolescents.

Purpose: This study had 2 objectives: (1) to examine whether the validity of the supramaximal verification test for maximal oxygen uptake ( V˙O2max ) differs in children and adolescents when stratified for sex, body mass, and cardiorespiratory fitness and (2) to assess sensitivity and specificity of primary and secondary objective criteria from the incremental test to verify V˙O2max . Methods: In total, 128 children and adolescents (76 male and 52 females; age: 9.3-17.4 y) performed a ramp-incremental test to exhaustion on a cycle ergometer followed by a supramaximal test to verify V˙O2max . Results: Supramaximal tests verified V˙O2max in 88% of participants. Group incremental test peak V˙O2 was greater than the supramaximal test (2.27 [0.65] L·min-1 and 2.17 [0.63] L·min-1; P < .001), although both were correlated (r = .94; P < .001). No differences were found in V˙O2 plateau attainment or supramaximal test verification between sex, body mass, or cardiorespiratory fitness groups (all Ps > .18). Supramaximal test time to exhaustion predicted supramaximal test V˙O2max verification (P = .04). Primary and secondary objective criteria had insufficient sensitivity (7.1%-24.1%) and specificity (50%-100%) to verify V˙O2max . Conclusion: The utility of supramaximal testing to verify V˙O2max is not affected by sex, body mass, or cardiorespiratory fitness status. Supramaximal testing should replace secondary objective criteria to verify V˙O2max .

Analysis of oxygen uptake efficiency parameters in young people with cystic fibrosis.

PURPOSE: This study characterised oxygen uptake efficiency (OUE) in children with mild-to-moderate cystic fibrosis (CF). Specifically, it investigated (1) the utility of OUE parameters as potential submaximal surrogates of peak oxygen uptake ([Formula: see text]), and (2) the relationship between OUE and disease severity. METHODS: Cardiopulmonary exercise test (CPET) data were collated from 72 children [36 CF, 36 age- and sex-matched controls (CON)], with OUE assessed as its highest 90-s average (plateau; OUEP), the gas exchange threshold (OUEGET) and respiratory compensation point (OUERCP). Pearson's correlation coefficients, independent t tests and factorial ANOVAs assessed differences between groups and the use of OUE measures as surrogates for [Formula: see text]. RESULTS: A significant (p < 0.05) reduction in allometrically scaled [Formula: see text] and all OUE parameters was found in CF. Significant (p < 0.05) correlations between measurements of OUE and allometrically scaled [Formula: see text], were observed in CF (r = 0.49-0.52) and CON (r = 0.46-0.52). Furthermore, measures of OUE were significantly (p < 0.05) correlated with pulmonary function (FEV1%predicted) in CF (r = 0.38-0.46), but not CON (r = -0.20-0.14). OUEP was able to differentiate between different aerobic fitness tertiles in CON but not CF. CONCLUSIONS: OUE parameters were reduced in CF, but were not a suitable surrogate for [Formula: see text]. Clinical teams should, where possible, continue to utilise maximal CPET parameters to measure aerobic fitness in children and adolescents with CF. Future research should assess the prognostic utility of OUEP as it does appear sensitive to disease status and severity.

Getting Your First Publication in Medical Education-Why? What? Where? How?

The process of getting one's work published is a major milestone for many in their early academic and clinical careers. However, this process can be confusing and overwhelming for many who have yet to publish themselves. There are differing motivators for publishing work in our early career stages, alongside considerations, such as what we publish, where we decide to submit work, and how we logistically undertake the submission process. This commentary provides a holistic overview for the early career medical educator, empowering them to take the bold steps toward "getting published."

Comparison of Reporting Quality in National Cystic Fibrosis Patient Registries: Implications for Identifying Use of Novel CFTR Modulators.

INTRODUCTION: Advances in development of cystic fibrosis transmembrane conductance regulator modulator (CFTRm) therapies mean that now people who are heterozygous (instead of having to be homozygous) for the common F508del variant can benefit from these therapies. Recent economic estimates suggest only approximately 15% of the global population have CFTRm access, yet it is unknown how prevalence of F508del and economic factors may affect this availability. METHODS: Data related to prevalence of cystic fibrosis (CF), CFTRm usage, and prevalence of F508del in 10 countries were extracted from publicly accessible registry reports from 2021. National gross domestic product (GDP) was obtained via open access World Bank data. Descriptive statistics and correlation coefficients assessed relationships. RESULTS: Notable discrepancies were noted in the equity of availability of data between national registries-only four countries reported number of patients eligible for CFTRm. Registry data represented 70,694 patients, with 42,858 found to be using CFTRm (60.6%). Prevalence of CFTRm usage ranged from 1.8% to 76.7% and prevalence of F508del ranged from 35.2% to 94.4%. The correlation between prevalence of CFTRm usage and F508del is positive (r = 0.56, p = 0.10), and the correlation between CFTRm usage and GDP (per capita) was also positive, and significant (r = 0.72, p = 0.02). CONCLUSION: Both F508del prevalence and GDP are associated with variable CFTRm usage rates, although a predominant reason is unclear as a result of poor consistency in registry reporting. Urgent action is needed to create uniform reporting of registry data and increase availability of novel CFTRm therapies to the global CF population.

Adapting, restarting, and terminating a randomised control trial for people with cystic fibrosis: Reflections on the impact of the COVID-19 pandemic upon research in a clinical population.

Background: Habitual physical activity (PA) and exercise form a cornerstone of the management of cystic fibrosis (CF), a genetically inherited pulmonary and digestive condition - whereby telehealth platforms have been proposed as a mechanism to engage remotely people with CF in PA and exercise. Methods: To test this, in early 2020, the 'ActivOnline: Physical Activity in Cystic Fibrosis Trial' (ActiOn PACT) randomised control trial was established to examine whether an online intervention was effective at increasing PA in adolescents and adults with CF. Results: The emergence of the COVID-19 pandemic in 2020 forced this trial to be paused and modified, with the adoption of online recruitment and remote assessment of outcome measures. Despite such adaptations in accord with frameworks developed by the National Institute for Health Research, this trial failed to recruit and was subsequently terminated. Conclusions: This article details the authors reflections upon the proposed reasons for lack of recruitment, including improved technology and medications for people with CF, and contextualises this finding in relation to the wider issue of non-reporting of trial results in clinical research.

Middle cerebral artery blood velocity and end-tidal carbon dioxide responses to moderate intensity cycling in children, adolescents, and adults.

This study investigated the middle cerebral artery blood velocity (MCAv) response to constant work-rate moderate-intensity cycling exercise in 21 children (9.3 ± 0.8 yr), 17 adolescents (12.3 ± 0.4 yr), and 20 young adults (23.6 ± 2.4 yr). Participants completed an incremental ramp test to exhaustion on a cycle ergometer to determine maximal oxygen uptake and gas exchange threshold (GET) before completing three 6-min transitions at a moderate intensity (90% GET) on separate visits. On each visit, bilateral MCAv was measured by transcranial Doppler ultrasonography and breath-by-breath end-tidal carbon dioxide ([Formula: see text]) via a metabolic cart. Data were ensemble-averaged for each participant and analyzed using a monoexponential model. Absolute MCAv was significantly higher throughout exercise in children and adolescents compared with adults (P < 0.001). Children had a significantly lower relative increase in MCAv from baseline (∼12%) compared with adolescents (∼20%) and adults (∼18%, P < 0.040). All adolescents and adults had a monoexponential rise in MCAv and [Formula: see text], but this was observed in only eight children. Children and adolescents had a significantly faster MCAv time constant (τ, 12 ± 6 and 14 ± 8 s, respectively) compared with adults (27 ± 9 s, P < 0.001). MCAv τ was positively associated with faster [Formula: see text] τ in adolescents (r = 0.70, P = 0.002) but not in children (r = -0.20, P = 0.640). Time- and amplitude-based response parameters of MCAv kinetics were significantly associated with [Formula: see text] kinetics in adults (r = 0.50-0.74, P ≤ 0.025), but not in children (r = -0.19 to -0.48, P > 0.227). These findings suggest that the transition from childhood to adulthood impacts the MCAv response to exercise and the relationships between [Formula: see text] and MCAv kinetics during exercise.NEW & NOTEWORTHY This is the first study to find that children have smaller increases in Δ%MCAv (∼12%) during moderate-intensity exercise compared with adolescents and adults (∼18%-20%). Furthermore, MCAv kinetics were significantly faster in children and adolescents, compared with adults. MCAv kinetic responses were significantly and positively associated with [Formula: see text] kinetics in adults, but not in children. These novel data also suggest that the regulatory role of [Formula: see text] on MCAv during exercise begins to strengthen during adolescence.

Percentage Predicted Peak Oxygen Consumption in People With Fontan Circulation: A Rapid Systematic Scoping Review and Validation Study.

BACKGROUND: Peak oxygen consumption (peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$) is routinely measured in people who have congenital heart disease and is reported as a percentage of predicted value, based upon age- and sex-matched normative reference values (NRVs). This study aimed to identify which NRVs are being used, assess whether NRVs are being applied appropriately, and evaluate if recommended NRVs are valid when applied to people with congenital heart disease. METHODS AND RESULTS: A systematic scoping review identified studies that reported peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ percentage of predicted value in people with congenital heart disease. A modified risk of bias tool evaluated the included studies. Forty-five studies reported peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ percentage of predicted value, and only 21 (47%) studies described or provided a reference on how their percentage of predicted value was calculated. The most cited NRVs were from Wasserman (n=12) and Cooper and Weiler-Ravell (n=7). Risk of bias analysis judged 63% of studies as having some concerns. The NRVs recommended by the American Heart Association were applied to participants with a Fontan circulation (n=70; aged 26.5±6.4 years; 59% women) to examine validity. Predicted peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ values from the Wasserman NRV was not significantly associated to measured peak V̇O2$$ \dot{\mathrm{V}}{\mathrm{O}}_2 $$ values (men: b=0.31, R2≤0.01; women: b=0.07, R2=0.02). CONCLUSIONS: Numerous NRVs have been applied to individuals with congenital heart disease and are often poorly reported and inappropriately matched to participants. The Wasserman NRV was the most cited but showed poor validity when applied to a Fontan cohort.

Cardiopulmonary Exercise Testing Provides Prognostic Information in Advanced Cystic Fibrosis Lung Disease.

Rationale: Cardiopulmonary exercise testing (CPET) provides prognostic information in cystic fibrosis (CF); however, its prognostic value for patients with advanced CF lung disease is unknown. Objectives: To determine the prognostic value of CPET on the risk of death or lung transplant (LTX) within 2 years. Methods: We retrospectively collected data from 20 CF centers in Asia, Australia, Europe, and North America on patients with a forced expiratory volume in 1 second (FEV1) ⩽ 40% predicted who performed a cycle ergometer CPET between January 2008 and December 2017. Time to death/LTX was analyzed using mixed Cox proportional hazards regression. Conditional inference trees were modeled to identify subgroups with increased risk of death/LTX. Results: In total, 174 patients (FEV1, 30.9% ± 5.8% predicted) were included. Forty-four patients (25.5%) died or underwent LTX. Cox regression analysis adjusted for age, sex, and FEV1 revealed percentage predicted peak oxygen uptake ([Formula: see text]o2peak) and peak work rate (Wpeak) as significant predictors of death/LTX: adjusted hazard ratios per each additional 10% predicted were 0.60 (95% confidence interval, 0.43-0.90; P = 0.008) and 0.60 (0.48-0.82; P < 0.001). Tree-structured regression models, including a set of 11 prognostic factors for survival, identified Wpeak to be most strongly associated with 2-year risk of death/LTX. Probability of death/LTX was 45.2% for those with a Wpeak ⩽ 49.2% predicted versus 10.9% for those with a Wpeak > 49.2% predicted (P < 0.001). Conclusions: CPET provides prognostic information in advanced CF lung disease, and Wpeak appears to be a promising marker for LTX referral and candidate selection.

A systematic review to explore how exercise-based physiotherapy via telemedicine can promote health related benefits for people with cystic fibrosis.

To conduct a systematic review to evaluate the effects of physiotherapy exercises delivered via telemedicine on lung function and quality-of-life in people with Cystic Fibrosis (CF). The databases AMED, CINAHL and MEDLINE were searched from December 2001 until December 2021. Reference lists of included studies were hand-searched. The PRISMA 2020 statement was used to report the review. Studies of any design reported in the English language, included participants with CF, and within outpatient settings were included. Meta-analysis was not deemed appropriate due to the diversity of interventions and heterogeneity of the included studies. Following screening, eight studies with 180 total participants met the inclusion criteria. Sample sizes ranged from 9 to 41 participants. Research designs included five single cohort intervention studies, two randomised control trials and one feasibility study. Telemedicine-based interventions included Tai-Chi, aerobic, and resistance exercise delivered over a study period of six to twelve weeks. All included studies which measured percentage predicted forced expiratory volume in one second found no significant difference. Five studies measuring the Cystic Fibrosis Questionnaire-Revised (CFQ-R) respiratory domain found improvements, however, did not meet statistical significance. For the CFQ-R physical domain, measured by five studies, two studies found an improvement, although not statistically significant. No adverse events were reported across all studies. The included studies indicate that telemedicine-based exercise over 6-12 weeks does not significantly change lung function or quality-of-life in people with CF. Whilst the role of telemedicine in the care of pwCF is acceptable and promising; further research with standardised outcome measures, larger sample sizes and longer follow-up are required before clinical practice recommendations can be developed.

Expert guidelines on exercise and physical activity for people with cystic fibrosis.

Research has shown that there is a lack of confidence and understanding in how to use exercise for managing cystic fibrosis. This editorial discusses the key points of a consensus statement that highlights what is and is not known about the relationship between cystic fibrosis and exercise.

Agreement between left and right middle cerebral artery blood velocity responses to incremental and constant work-rate exercise in healthy males and females.

Objective.To quantify the agreement between left and right middle cerebral artery blood velocity (MCAv) responses to incremental and constant work-rate exercise in adults.ApproachSeventeen healthy adults (23.8 ± 2.4 years, 9 females) completed a ramp incremental test to exhaustion on a cycle ergometer, three 6-minute transitions at a moderate-intensity, and three at a heavy-intensity, all on separate days. Bilateral MCAv was measured throughout using transcranial Doppler ultrasonography, with left and right MCAv data analysed separately. Data were analysed at baseline, gas exchange threshold, respiratory compensation point and exhaustion during ramp incremental exercise. MCAv responses to constant work-rate exercise were analysed using a mono-exponential model, to determine time- and amplitude-based kinetic response parameters.Main ResultsLeft and right MCAv responses to incremental and constant work-rate exercise were significantly, strongly and positively correlated (r≥ 0.61,P< 0.01). Coefficient of variation (left versus right) ranged from 7.3%-20.7%, 6.4%-26.2% and 5.9%-22.5% for ramp, moderate and heavy-intensity exercise, respectively. The relative change in MCAv from baseline was higher in the right compared to left MCAv during ramp, moderate and heavy-intensity exercise (allP< 0.05), but the effect sizes were small (d≤ 0.4). Small mean left-right differences were present during ramp incremental exercise at all time-points (<6 cm s-1; <4%), and for all kinetic parameters during moderate and heavy-intensity exercise (<3 cm s-1, <3%, <4 s).SignificanceThese findings demonstrate similarities between left and right MCAv responses to incremental and constant-work rate exercise in adults on a group-level, but also highlight individual variation in the agreement between left and right MCAv exercise responses.

Normal reference values for aerobic fitness in cystic fibrosis: a scoping review.

Objective: The importance of aerobic fitness (VO2peak) in cystic fibrosis (CF) is well established, and regular exercise testing is recommended. To standardise VO2peak, a 'percentage of predicted' (%pred) derived from normative reference values (NRV), as promoted by the 2015 European Cystic Fibrosis Society Exercise Working Group (ECFS EWG), can be reported. However, the NRVs used in CF and their relative frequency is unknown. Method: A scoping review was performed via systematic database searches (PubMed, Embase, Web of Science, SciELO, EBSCO) and forward citation searches for studies that include people with CF and report VO2peak as %pred. Studies were screened using Covidence, and data related to patient demographics, testing modality and reference equations were extracted. Additional analyses were performed on studies published in 2016-2021, following the ECFS EWG statement in 2015. Results: A total of 170 studies were identified, dating from 1984 to 2022, representing 6831 patients with CF, citing 34 NRV. Most studies (154/170) used cycle ergometry, 15/170 used treadmills, and the remainder used alternative, combination or undeclared modalities. In total, 61/170 failed to declare the NRV used. There were 61 studies published since the ECFS EWG statement, whereby 18/61 used the suggested NRV. Conclusion: There is a wide discrepancy in NRV used in the CF literature base to describe VO2peak as %pred, with few studies using NRV from the ECFS EWG statement. This high variance compromises the interpretation and comparison of studies while leaving them susceptible to misinterpretation and limiting replication. Standardisation and alignment of reporting of VO2peak values are urgently needed.

The Effect of Dysglycaemia on Changes in Pulmonary and Aerobic Function in Cystic Fibrosis.

Cross-sectional studies have reported lower pulmonary and aerobic function during exercise in people with cystic fibrosis-related diabetes (CFRD) compared to non-CFRD counterparts. However, this association has yet to be longitudinally investigated. Therefore, this study examines these differences over time between people with cystic fibrosis (CF) of differing glycaemic status. Annual review data, including cardiopulmonary exercise tests and pulmonary function tests, were retrospectively analysed at baseline (T0, n = 82) and at a one-year follow-up (T1, n = 54). Data was analysed in three groups: normal glucose tolerance (NGT), impaired glucose tolerance (IGT), and CFRD. Further analyses were undertaken, with a dichotomous split of NGT and a combined IGT/CFRD group. At baseline, a significant reduction in the majority of variables, including forced expiratory volume in one second (FEV1) and maximal oxygen uptake (VO2max), was observed in the CFRD (n = 19) group compared to NGT (n = 58). At follow-up, no significant differences were observed, and no interaction effect between CFRD status and time was identified. FEV1 and VO2max presented with varying directions and magnitudes of change within patients. In summary, patients with CFRD have a reduced aerobic and pulmonary function compared to non-CFRD counterparts, although such changes disappeared at follow up. Varying responses for FEV1 and VO2max highlight the need to consider both variables as independent markers of function in CF.

The impact of COVID-19 upon the delivery of exercise services within cystic fibrosis clinics in the United Kingdom.

OBJECTIVES: The COVID-19 pandemic has resulted in unprecedent changes to clinical practice, and as the impact upon delivery of exercise services for people with cystic fibrosis (CF) in the United Kingdom was unknown, this was characterised via a national survey. METHODS: An electronic survey was distributed to healthcare professionals involved in the exercise management of CF via established professional networks. RESULTS: In total, 31 CF centres participated. Findings included significant reductions in exercise testing and widespread adaptation to deliver exercise training using telehealth methods. Promisingly, 71% stated that they would continue using virtual methods of engaging patients in future practice. CONCLUSION: These findings highlight adaptation to the COVID-19 pandemic and the need to develop sustainable and standardised telehealth services to manage patients moving forwards.