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

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

Promotion of exercise in the management of cystic fibrosis - summary of national meetings

This is the final version. Available from the publisher via the DOI in this recordRationale, aims and objectives: Physical activity (PA) and exercise are important in maintaining and improving health and wellbeing in people with cystic fibrosis (CF) and measures of exercise capacity are useful outcomes in monitoring disease progression. The roles and responsibilities of CF multi-disciplinary team (MDT) members in supporting PA and exercise have yet to be fully defined. This communication reports on national meetings of CF MDT staff whose interest is to improve and standardise person-centered exercise provision and testing as part of routine CF care. We also introduce the role of the physiotherapy technician in supporting PA interventions. Meetings: The two meetings covered a range of presentations, discussions and workshops, focusing on the role of exercise and PA in CF management. Forty people from 15 NHS Hospital Trusts and 3 universities were asked to provide feedback via a questionnaire. Results: The common roles and responsibilities of clinical staff involved in exercise testing and prescription are described, with a wide range of duties identified. In addition, physiotherapists were reported as the main MDT member responsible for exercise provision. The majority of teams reported discussing exercise at every clinical visit (57%) and felt confident in discussing exercise with patients (67%). Conclusions: While this report highlights the current provision of exercise in CF MDTs, it also provides insight into the resources MDTs may require in order to enhance the profile of exercise within CF services, including enhanced training, guidelines and standardised clinical roles.Royal Devon & Exeter NHS Foundation TrustUniversity of Exeter Open Innovation Link Fun

Severe mental illness and chronic kidney disease: a cross-sectional study in the United Kingdom.

OBJECTIVE: We investigated the burden of chronic kidney disease (CKD) among patients with severe mental illness (SMI). METHODS: We identified patients with SMI among all those aged 25-74 registered in the UK Clinical Practice Research Datalink as on March 31, 2014. We compared the prevalence of CKD (two measurements of estimated glomerular filtration rate <60 mL/min/1.73 m2 for ≥3 months) and renal replacement therapy between patients with and without SMI. For patients with and without a history of lithium prescription separately, we used logistic regression to examine the association between SMI and CKD, adjusting for demographics, lifestyle characteristics, and known CKD risk factors. RESULTS: The CKD prevalence was 14.6% among patients with SMI and a history of lithium prescription (n = 4,295), 3.3% among patients with SMI and no history of lithium prescription (n = 24,101), and 2.1% among patients without SMI (n = 2,387,988; P < 0.001). The prevalence of renal replacement therapy was 0.23%, 0.15%, and 0.11%, respectively (P = 0.012). Compared to patients without SMI, the fully adjusted odds ratio for CKD was 6.49 (95% CI 5.84-7.21) for patients with SMI and a history of lithium prescription and 1.45 (95% CI 1.34-1.58) for patients with SMI and no history of lithium prescription. The higher prevalence of CKD in patients with SMI may, in part, be explained by more frequent blood testing as compared to the general population. CONCLUSION: CKD is identified more commonly among patients with SMI than in the general population

Annual changes in aerobic fitness are biased between normative equations in children and adolescents with cystic fibrosis

This is the author accepted manuscript. The final version is available from Elsevier via the DOI in this recordPaper P298 presented at the the 43rd European Cystic Fibrosis Digital Conference, 24 - 25 September 202

Promotion of exercise in the management of cystic fibrosis - summary of national meetings

Rationale, aims and objectives: Physical activity (PA) and exercise are important in maintaining and improving health and wellbeing in people with cystic fibrosis (CF) and measures of exercise capacity are useful outcomes in monitoring disease progression. The roles and responsibilities of CF multi-disciplinary team (MDT) members in supporting PA and exercise have yet to be fully defined. This communication reports on national meetings of CF MDT staff whose interest is to improve and standardise person-centered exercise provision and testing as part of routine CF care. We also introduce the role of the physiotherapy technician in supporting PA interventions.Meetings: The two meetings covered a range of presentations, discussions and workshops, focusing on the role of exercise and PA in CF management. Forty people from 15 NHS Hospital Trusts and 3 universities were asked to provide feedback via a questionnaire.Results: The common roles and responsibilities of clinical staff involved in exercise testing and prescription are described, with a wide range of duties identified. In addition, physiotherapists were reported as the main MDT member responsible for exercise provision. The majority of teams reported discussing exercise at every clinical visit (57%) and felt confident in discussing exercise with patients (67%).Conclusions: While this report highlights the current provision of exercise in CF MDTs, it also provides insight into the resources MDTs may require in order to enhance the profile of exercise within CF services, including enhanced training, guidelines and standardised clinical roles

Fumarate drives EMT in renal cancer

Medical Research Counci

Skeletal muscle contributions to reduced fitness in cystic fibrosis youth

This is the final version. Available from Frontiers Media via the DOI in this record. Data availability statement: The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Background: Increased maximal oxygen uptake (V̇ O2max) is beneficial in children with cystic fibrosis (CF) but remains lower compared to healthy peers. Intrinsic metabolic deficiencies within skeletal muscle (muscle “quality”) and skeletal muscle size (muscle “quantity”) are both proposed as potential causes for the lower V̇ O2max, although exact mechanisms remain unknown. This study utilises gold-standard methodologies to control for the residual effects of muscle size from V̇ O2max to address this “quality” vs. “quantity” debate. Methods: Fourteen children (7 CF vs. 7 age- and sex-matched controls) were recruited. Parameters of muscle size – muscle cross-sectional area (mCSA) and thigh muscle volume (TMV) were derived from magnetic resonance imaging, and V̇ O2max obtained via cardiopulmonary exercise testing. Allometric scaling removed residual effects of muscle size, and independent samples t-tests and effect sizes (ES) identified differences between groups in V̇ O2max, once mCSA and TMV were controlled for. Results: V̇ O2max was shown to be lower in the CF group, relative to controls, with large ES being identified when allometrically scaled to mCSA (ES = 1.76) and TMV (ES = 0.92). Reduced peak work rate was also identified in the CF group when allometrically controlled for mCSA (ES = 1.18) and TMV (ES = 0.45). Conclusions: A lower V̇ O2max was still observed in children with CF after allometrically scaling for muscle size, suggesting reduced muscle “quality” in CF (as muscle “quantity” is fully controlled for). This observation likely reflects intrinsic metabolic defects within CF skeletal muscle.National Institute for Health ResearchRoyal Devon & Exeter CF Research Charitable FundUniversity of Exete

Mesenteric rheumatoid nodules masquerading as an intra-abdominal malignancy: a case report and review of the literature

<p>Abstract</p> <p>Background</p> <p>Rheumatoid nodules are the most common extra-articular findings in patients with rheumatoid arthritis. They occur most commonly at pressure points such as the extensor surfaces of the forearms, fingers, and occiput, but have also been reported to occur in unusual locations including the central nervous system, pericardium, pleura, and sclera. We present the unusual case of rheumatoid nodules in the small bowel mesentery masquerading as an intra-abdominal malignancy.</p> <p>Case presentation</p> <p>A 65-year-old-male with a known history of longstanding erosive, nodular, seropositive rheumatoid arthritis was incidentally found to have a mesenteric mass on computed tomography (CT) exam of the abdomen. This mass had not been present on prior imaging studies and was worrisome for a malignancy. Attempts at noninvasive biopsy were nondiagnostic but consistent with a "spindle" cell neoplasm. Laparotomy revealed extensive thickening and fibrosis of the small bowel mesentery along with large, firm nodules throughout the mesentery. A limited bowel resection including a large, partially obstructing, nodule was performed. Pathology was consistent with an unusual presentation of rheumatoid nodules in the mesentery of the small bowel.</p> <p>Conclusion</p> <p>Rheumatoid nodules should be considered in the differential diagnosis of a patient who presents with an intra-abdominal mass and a history of rheumatoid arthritis. Currently, no tests or imaging modality can discriminate with sufficient accuracy to rule out a malignancy in this difficult diagnostic delimma. Hopefully, this case will serve as impetus for further study and biomarker discovery to allow for improved diagnostic power.</p

Aerobic capacity and skeletal muscle characteristics in glycogen storage disease IIIa: an observational study

Background: Individuals with glycogen storage disease IIIa (GSD IIIa) (OMIM #232400) experience muscle weakness and exercise limitation that worsen through adulthood. However, normative data for markers of physical capacity, such as strength and cardiovascular fitness, are limited. Furthermore, the impact of the disease on muscle size and quality is unstudied in weight bearing skeletal muscle, a key predictor of physical function. We aim to produce normative reference values of aerobic capacity and strength in individuals with GSD IIIa, and to investigate the role of muscle size and quality on exercise impairment. Results: Peak oxygen uptake (V̇O2peak) was lower in the individuals with GSD IIIa than predicted based on demographic data (17.0 (9.0) ml/kg/min, 53 (24)% of predicted, p = 0.001). Knee extension maximum voluntary contraction (MVC) was also substantially lower than age matched predicted values (MVC: 146 (116) Nm, 57% predicted, p = 0.045), though no difference was found in MVC relative to body mass (1.88 (2.74) Nm/kg, 61% of predicted, p = 0.263). There was a strong association between aerobic capacity and maximal leg strength (r = 0.920; p = 0.003). Substantial inter-individual variation was present, with a high physical capacity group that had normal leg strength (MVC), and relatively high V̇O2peak, and a low physical capacity that display impaired strength and substantially lower V̇O2peak. The higher physical capacity sub-group were younger, had larger Vastus Lateralis (VL) muscles, greater muscle quality, undertook more physical activity (PA), and reported higher health-related quality of life. Conclusions: V̇O2peak and knee extension strength are lower in individuals with GSD IIIa than predicted based on their demographic data. Patients with higher physical capacity have superior muscle size and structure characteristics and higher health-related quality of life, than those with lower physical capacity. This study provides normative values of these important markers of physical capacity