@article{RufBeerKoestleretal.2019, author = {Ruf, Katharina and Beer, Meinrad and K{\"o}stler, Herbert and Weng, Andreas Max and Neubauer, Henning and Klein, Alexander and Platek, Kathleen and Roth, Kristina and Beneke, Ralph and Hebestreit, Helge}, title = {Size-adjusted muscle power and muscle metabolism in patients with cystic fibrosis are equal to healthy controls - a case control study}, series = {BMC Pulmonary Medicine}, volume = {19}, journal = {BMC Pulmonary Medicine}, doi = {10.1186/s12890-019-1039-8}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-200981}, pages = {269}, year = {2019}, abstract = {Background Skeletal muscle function dysfunction has been reported in patients with cystic fibrosis (CF). Studies so far showed inconclusive data whether reduced exercise capacity is related to intrinsic muscle dysfunction in CF. Methods Twenty patients with CF and 23 age-matched controls completed an incremental cardiopulmonary cycling test. Further, a Wingate anaerobic test to assess muscle power was performed. In addition, all participants completed an incremental knee-extension test with 31P magnetic resonance spectroscopy to assess muscle metabolism (inorganic phosphate (Pi) and phosphocreatinine (PCr) as well as intracellular pH). In the MRI, muscle cross-sectional area of the M. quadriceps (qCSA) was also measured. A subgroup of 15 participants (5 CF, 10 control) additionally completed a continuous high-intensity, high-frequency knee-extension exercise task during 31P magnetic resonance spectroscopy to assess muscle metabolism. Results Patients with CF showed a reduced exercise capacity in the incremental cardiopulmonary cycling test (VO2peak: CF 77.8 ± 16.2\%predicted (36.5 ± 7.4 ml/qCSA/min), control 100.6 ± 18.8\%predicted (49.1 ± 11.4 ml/qCSA/min); p < 0.001), and deficits in anaerobic capacity reflected by the Wingate test (peak power: CF 537 ± 180 W, control 727 ± 186 W; mean power: CF 378 ± 127 W, control 486 ± 126 W; power drop CF 12 ± 5 W, control 8 ± 4 W. all: p < 0.001). In the knee-extension task, patients with CF achieved a significantly lower workload (p < 0.05). However, in a linear model analysing maximal work load of the incremental knee-extension task and results of the Wingate test, respectively, only muscle size and height, but not disease status (CF or not) contributed to explaining variance. In line with this finding, no differences were found in muscle metabolism reflected by intracellular pH and the ratio of Pi/PCr at submaximal stages and peak exercise measured through MRI spectroscopy. Conclusions The lower absolute muscle power in patients with CF compared to controls is exclusively explained by the reduced muscle size in this study. No evidence was found for an intrinsic skeletal muscle dysfunction due to primary alterations of muscle metabolism.}, language = {en} } @article{NeubauerWirthRufetal.2012, author = {Neubauer, Henning and Wirth, Clemens and Ruf, Katharina and Hebestreit, Helge and Beer, Meinrad}, title = {Acute Muscle Trauma due to Overexercise in an Otherwise Healthy Patient with Cystic Fibrosis}, series = {Case Reports in Pediatrics}, volume = {2012}, journal = {Case Reports in Pediatrics}, number = {527989}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-123967}, year = {2012}, abstract = {Cystic fibrosis (CF) is one of the most common inherited diseases and is caused by mutations in the CFTR gene. Although the pulmonary and gastrointestinal manifestations of the disease remain in the focus of treatment, recent studies have shown expression of the CFTR gene product in skeletal muscle cells and observed altered intramuscular \(Ca^{2+}\) release dynamics in CFTR-deficient animal models. Physical exercise is beneficial for maintaining fitness and well-being in CF patients and constitutes one aspect of modern multimodal treatment, which has considerably increased life span and reduced morbidity. We report on a case of acute muscle trauma resulting from excessive dumbbell exercise in a young adult with cystic fibrosis and describe clinical, laboratory and imaging characteristics of acute exercise-induced muscle injury.}, language = {en} } @article{KaireitSorrentinoRenneetal.2017, author = {Kaireit, Till F. and Sorrentino, Sajoscha A. and Renne, Julius and Schoenfeld, Christian and Voskrebenzev, Andreas and Gutberlet, Marcel and Schulz, Angela and Jakob, Peter M. and Hansen, Gesine and Wacker, Frank and Welte, Tobias and T{\"u}mmler, Burkhard and Vogel-Claussen, Jens}, title = {Functional lung MRI for regional monitoring of patients with cystic fibrosis}, series = {PLoS ONE}, volume = {12}, journal = {PLoS ONE}, number = {12}, doi = {10.1371/journal.pone.0187483}, url = {http://nbn-resolving.de/urn:nbn:de:bvb:20-opus-172457}, year = {2017}, abstract = {Purpose To test quantitative functional lung MRI techniques in young adults with cystic fibrosis (CF) compared to healthy volunteers and to monitor immediate treatment effects of a single inhalation of hypertonic saline in comparison to clinical routine pulmonary function tests. Materials and methods Sixteen clinically stable CF patients and 12 healthy volunteers prospectively underwent two functional lung MRI scans and pulmonary function tests before and 2h after a single treatment of inhaled hypertonic saline or without any treatment. MRI-derived oxygen enhanced T1 relaxation measurements, fractional ventilation, first-pass perfusion parameters and a morpho-functional CF-MRI score were acquired. Results Compared to healthy controls functional lung MRI detected and quantified significantly increased ventilation heterogeneity in CF patients. Regional functional lung MRI measures of ventilation and perfusion as well as the CF-MRI score and pulmonary function tests could not detect a significant treatment effect two hours after a single treatment with hypertonic saline in young adults with CF (p>0.05). Conclusion This study shows the feasibility of functional lung MRI as a non-invasive, radiation-free tool for monitoring patients with CF.}, language = {en} }