Exercise capacity following a percutaneous endoscopic gastrostomy in a young female with cystic fibrosis: a case report

This is the final version of the article. Available from the publisher via the DOI in this record.Physiological Reports published by Wiley Periodicals, Inc. on behalf of the American Physiological Society and The Physiological Society.Cystic fibrosis (CF) is a genetic condition affecting the respiratory and gastrointestinal systems, with patients experiencing problems maintaining weight, especially during rapid growth periods such as puberty. The aim of this case report was to monitor the effect of gastrostomy insertion and implementation of overnight supplemental feeding upon clinical outcomes, including body mass index (BMI), lung function (FEV1), and exercise-related variables (maximal oxygen uptake [VO2max] and ventilatory efficiency [VE/VO2]) in an 11-year-old female with CF. Combined incremental and supramaximal exercise testing to exhaustion was performed at four time points: 3 months prior to the procedure (T1), 2 days prior to (T2), 4 months (T3), and 1 year following the procedure (T4). Improvements following gastrostomy insertion were observed at the 1 year follow-up with regards to BMI (+20%); whereas absolute VO2max remained stable and lung function fluctuated throughout the period of observation. Declines in function with regards to body weight relative VO2max (−16.3%) and oxygen uptake efficiency (+7.5%) were observed during this period. This case report is the first to consider exercise-related clinical outcomes in assessing the effect of implementing gastrostomy feeding in CF. The varied direction and magnitude of the associations between variables shows that further investigations are required

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

This is the final version. Available on open access from BMJ publishing Group via the DOI in this recordData availability statement: All data relevant to the study are included in the article or uploaded as online supplemental information. Not applicable.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.Canon Medical Systems UK LtdUniversity of Exete

Validity and reliability concerns associated with cardiopulmonary exercise testing young people with cystic fibrosis

This is the author accepted manuscript. The final version is available from the publisher via the DOI in this record.Letter to the Edito

Cardiopulmonary responses to maximal aerobic exercise in patients with cystic fibrosis (article)

This is the final version. Available from Public Library of Science via the DOI in this record.The dataset associated with this article is located in ORE at: https://doi.org/10.24378/exe.1105Cystic fibrosis (CF) is a debilitating chronic condition, which requires complex and expensive disease management. Exercise has now been recognised as a critical factor in improving health and quality of life in patients with CF. Hence, cardiopulmonary exercise testing (CPET) is used to determine aerobic fitness of young patients as part of the clinical management of CF. However, at present there is a lack of conclusive evidence for one limiting system of aerobic fitness for CF patients at individual patient level. Here, we perform detailed data analysis that allows us to identify important systems-level factors that affect aerobic fitness. We use patients’ data and principal component analysis to confirm the dependence of CPET performance on variables associated with ventilation and metabolic rates of oxygen consumption. We find that the time at which participants cross the gas exchange threshold (GET) is well correlated with their overall performance. Furthermore, we propose a predictive modelling framework that captures the relationship between ventilatory dynamics, lung capacity and function and performance in CPET within a group of children and adolescents with CF. Specifically, we show that using Gaussian processes (GP) we can predict GET at the individual patient level with reasonable accuracy given the small sample size of the available group of patients. We conclude by presenting an example and future perspectives for improving and extending the proposed framework. The modelling and analysis have the potential to pave the way to designing personalised exercise programmes that are tailored to specific individual needs relative to patient’s treatment therapies.Wellcome TrustEngineering and Physical Sciences Research Counci

The efficacy of virtual reality interventions compared with conventional physiotherapy in improving the upper limb motor function of children with cerebral palsy: a systematic review of randomised controlled trials

This is the final version. Available on open access from Taylor & Francis via the DOI in this recordPURPOSE: Cerebral palsy (CP) is the commonest motor disability affecting children. This study reviewed the evidence for virtual reality (VR) intervention compared with conventional physiotherapy in upper limb function of children with CP. METHODS: Searches were undertaken in MEDLINE, EMBASE, PEDro, CENTRAL, Web of Science, CINAHL, ERIC, ICTRP, EU-CTR, ClinicalTrials.gov and EThOS databases. Only randomised-controlled trials (RCTs) were included. Two reviewers independently screened the search results, assessed full-text articles, extracted data and appraised the methodological quality by using the Cochrane collaboration's risk of bias (RoB2) tool. Albatross plots were used to synthesise the data. RESULTS: Seven RCTs, examining motor function in a total of 202 children with CP, included. Four trials used the Quality of Upper Extremity Skills Test (QUEST) as an outcome measure, and three trials used grip strength. These outcome measures were utilised to develop two Albatross plots. Data from the plots showed contradictory findings of the included studies. CONCLUSIONS: The effect of VR in the upper limb rehabilitation of children with CP remains unclear. All included studies used commercial non-immersive VR games. Future high-quality clinical research is needed to explore the extent to which non-immersive and immersive VR is feasible and effective with children and adolescents.IMPLICATIONS FOR REHABILITATIONThe current evidence supporting the use of VR as a rehabilitative tool is weak and uncertain.The current use of VR relies only on commercial non-immersive VR (off-shelf) games, which are not adjustable to meet the demands and goals of therapy programmes.Future research is needed to study the therapeutic feasibility of immersive VR with children and adolescents.Ministry of Education, Saudi Arabi