<i>CFTR</i> Genotype and Maximal Exercise Capacity in Cystic Fibrosis. A Cross-Sectional Study

Radtke, Thomas; Hebestreit, Helge; Gallati, Sabina; Schneiderman, Jane E.; Braun, Julia; Stevens, Daniel; Hulzebos, Erik H.J.; Takken, Tim; Boas, Steven R.; Urquhart, Don S.; Lands, Larry C.; Tejero, Sergio; Sovtić, Aleksandar; Dwyer, Tiffany; Petrović, Miloš; Harris, Ryan A.; Karila, C.; Savi, Daniela; Usemann, Jakob; Mei-Zahav, Meir; Hatziagorou, Elpis; Ratjen, Félix; Kriemler, Susi; Wilkes, Donna L.; Wells, Greg D.; Solomon, Melinda; Ebidia, Jen; Trgachef, Stephanie; Woloschuk, Alexandra; Quintana‐Gallego, Esther; Cejudo, Pilar; Junge, S.; Smaczny, Christina; Fall, A.; Blacklock, Sarah; Tsanakas, John; Kaluza, Ingrid

doi:10.1513/annalsats.201707-570oc

Cited by 32 publications

(27 citation statements)

References 42 publications

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“…Flow-volume loops during exercise were not performed in our study. Recent multicenter study from the CFTR exercise group use similar approach for evaluating breathing reserve [35]. As expected, we found high incidence of respiratory limitation, represented as low BR and high ventilatory equivalents ( _ VE= _ VCO 2 ), in both patient groups, which was statistically different from the control group.…”

Section: Discussionsupporting

confidence: 74%

Exercise capacity in patients with cystic fibrosis vs non-cystic fibrosis bronchiectasis

Bar‐Yoseph¹,

Ilivitzki²,

Gur³

et al. 2018

Clinical Respiratory Physiology, Exercise and Functional Imaging

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Background Bronchiectasis is associated with morbidity, low exercise capacity and poor quality of life. There is a paucity of data on exercise capacity using cardiopulmonary exercise test (CPET) in non-cystic fibrosis (CF) bronchiectasis. Our aim was to compare exercise capacity using CPET in CF and non-CF bronchiectasis patients. Methods Cross-sectional retrospective/prospective controlled study assessing CPET using cycle ergometer. Exercise parameters and computed tomography (CT) findings were compared. Results: Hundred two patients with bronchiectasis and 88 controls were evaluated; 49 CF (age 19.7 ± 9.7 y/o, FEV 1 %predicted 70.9 ± 20.5%) and 53 non-CF (18.6 ± 10.6 y/o, FEV 1 % predicted 68.7 ± 21.5%). Peak oxygen uptake (peak _ VO 2) was similar and relatively preserved in both groups (CF 1915.5±702.0; non-CF 1740±568; control 2111.0±748.3 mL/min). Breathing limitation was found in the two groups vs. control; low breathing reserve (49% in CF; 43% non-CF; 5% control) and increased _ VE= _ VCO 2 (CF 31.4±4.1, non-CF 31.7±4.1 and control 27.2 ± 2.8). Oxygen pulse was lower in the non-CF; whereas a linear relationship between peak _ VO 2 vs. FEV 1 and vs. FVC was found only for CF. CT score correlated with _ VE= _ VCO 2 and negatively correlated with _ VO 2 =kg and post exercise oxygen saturation (SpO 2). Conclusions CPET parameters may differ between CF and non-CF bronchiectasis. However, normal exercise capacity may be found unrelated to the etiology of the bronchiectasis. Anatomical

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Section: Discussionsupporting

confidence: 74%

Exercise capacity in patients with cystic fibrosis vs non-cystic fibrosis bronchiectasis

Bar‐Yoseph¹,

Ilivitzki²,

Gur³

et al. 2018

Clinical Respiratory Physiology, Exercise and Functional Imaging

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“…Muscle abnormalities also may occur due to a dysfunction in the sarcoplasmic reticulum disrupting calcium homeostasis and increased systemic inflammation (Radtke et al, 2018). CFTR protein abnormalities in cystic fibrosis can also affect action potentials and electromechanical coupling during muscle contraction (Divangahi et al, 2009).…”

Section: Discussionmentioning

confidence: 99%

Tissue oxygenation in peripheral muscles and functional capacity in cystic fibrosis: a cross‐sectional study

Coelho

Aquino²,

Diniz

et al. 2020

Experimental Physiology

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New Findings What is the central question of this study?How do peripheral muscle tissue oxygenation and physical conditioning levels of children and adolescents with cystic fibrosis compare to demographically matched controls? What is the main finding and its importance?Children and adolescents with cystic fibrosis consumed more oxygen, more quickly and exhibited slower recovery, demonstrating that there may have been deficiencies in oxygen supply related to both oxygen uptake and oxygen transport. Abstract Cystic fibrosis affects skeletal muscle performance and functional capacity. However, it is currently unclear how peripheral muscle behaviour is affected, especially in children and adolescents. To examine this, we compared tissue oxygenation of children and adolescents with cystic fibrosis against healthy volunteers. We also evaluated the functional capacity of participants via the modified shuttle test (MST) and assessed for associations between performance and near‐infrared spectroscopy. A total of 124 participants enrolled. Participants were divided into either the cystic fibrosis group (CFG) or the healthy group (HG). Statistical comparisons between groups were evaluated with the Mann–Whitney U test and associations with functional capacity were evaluated using Spearman's correlation coefficient. CFG volunteers scored lower on the MST compared to the HG. They walked shorter distances (P = 0.001) with less efficiency because they performed the tests with a less efficient walking economy (P = 0.001) and a greater deoxyhaemoglobin concentration (P = 0.001). Further, they experienced reduced tissue oxygen saturation (P = 0.037) faster than the HG. As a result, they presented lower respiratory (P = 0.001) and lower heart (P = 0.001) rate values at the end of the MST, with a longer post‐test heart rate recovery time (P = 0.005). There was a significant association between deoxygenation time and functional capacity. The CFG consumed more oxygen, more quickly, with a slower recovery, reflecting impairments in the dynamics of muscle oxygen extraction. The results suggest differences in functional capacity and haemodynamic recovery in children and adolescents with cystic fibrosis.

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“…In the present study, predicted peak work rate is only associated with nutritional status, airflow obstruction, and P. aeruginosa chronic infection. Previous studies have already documented that pulmonary function limitation, inadequate nutrition and P. aeruginosa infection contribute to exercise intolerance in patients with CF . Indeed malnutrition can lead to the loss of body fat and free fat mass, with muscle mass as the main part of it.…”

Section: Discussionmentioning

confidence: 99%

“…Previous studies have already documented that pulmonary function limitation, inadequate nutrition and P. aeruginosa infection contribute to exercise intolerance in patients with CF. 17 Indeed malnutrition can lead to the loss of body fat and free fat mass, with muscle mass as the main part of it. Low BMI in CF is known to be closely implicated in worsening lung function and predicts worse outcome over a 4-year period.…”

Section: Discussionmentioning

confidence: 99%

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

Gambazza

Guarise

Carta

et al. 2020

Pediatric Pulmonology

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Background Lung clearance index (LCI2.5) is a marker of overall lung ventilation inhomogeneity and has proven to be able to detect early peripheral damage in subjects with cystic fibrosis (CF), with greater sensitivity than conventional spirometry. Combining its sensitivity with the output of an incremental exercise testing, we hypothesized that any sign of ventilation inhomogeneity in subjects without severe airflow obstruction and with a normal exercise tolerance could be relevant for the CF team, tracking early lung disease and potential exercise limiting factors. Methods Patients with CF in clinical stable conditions were recruited between 2015 and 2017. Available spirometry, nitrogen multiple‐breath washout test and symptoms‐limited exercise testing performed as parts of patients' annual routine evaluation were considered for this cross‐sectional study. To describe the relationship between exercise intensity and ventilation inhomogeneity, a linear regression analysis was performed using backward elimination based on Akaike information criteria. Results Seventy‐seven patients (38 females) were included. Sacin and LCI2.5 were significantly higher in patients with an overall reduced exercise tolerance. Peak work developed during exercise was associated with body mass index (b = 5.25; 95% confidence interval [CI] = 1.53‐8.98), forced expiratory volume in 1 second (FEV1; b = 3.71; 95% CI = 1.96‐5.46), Pseudomonas aeruginosa chronic infection (b = −8.84; 95% CI = −15.84 to −1.84) but not with LCI2.5. Conclusion Exercise capacity and airflow obstruction are associated in this Italian CF cohort. Considering the greater discriminatory power of LCI2.5 over FEV1 and peak work, the Godfrey protocol without gas analysis cannot provide detailed information about lung function or efficiency. However, this incremental protocol without gas exchange measures can still provide the CF team with information about exercise tolerance and disability.

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CFTR Genotype and Maximal Exercise Capacity in Cystic Fibrosis. A Cross-Sectional Study

Abstract: CFTR functional genotype class was not associated with maximal exercise capacity in patients with cystic fibrosis overall, but those with at least one copy of a F508del-CFTR mutation and a single class V mutation had lower maximal exercise capacity.

Cited by 32 publications

References 42 publications

Exercise capacity in patients with cystic fibrosis vs non-cystic fibrosis bronchiectasis

Exercise capacity in patients with cystic fibrosis vs non-cystic fibrosis bronchiectasis

Tissue oxygenation in peripheral muscles and functional capacity in cystic fibrosis: a cross‐sectional study

Exercise capacity and ventilation inhomogeneity in cystic fibrosis: A cross‐sectional study

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