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Pulmonary oxygen uptake kinetics are slowed in young patients with cystic fibrosis during very heavy but not moderate intensity cycling exercise

Saynor, Z.; Barker, A. R.; Oades, Patrick; Williams, Craig A. (LWW, 2016)
Characterization of human disease phenotypes associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, and IFIH1.

Crow, Y. J.; Chase, D. S.; Lowenstein Schmidt, J.; Szynkiewicz, M.; Forte, G. M. A.; Gornall, H. L.; Oojageer, A.; Anderson, B.; Pizzino, A.; Helman, G.; Abdel-Hamid, M. S.; Devriendt, K.; Di Rocco, M.; Fahey, M. C.; Fazzi, E.; Lim, M. J.; Ferrie, C. D.; Khan, N.; Gowrinathan, N. R.; McKee, S. A.; Gowrishankar, K.; Oades, Patrick; Hanrahan, D.; King, M. D.; Kirk, E. P.; Kumar, R.; Ramesh, V.; Lin, J.-P. S-M; Lagae, L.; Landrieu, P.; Lauffer, H.; Laugel, V.; Olivieri, I.; Roubertie, A.; Moroni, I.; Linnankivi, T.; Rasmussen, M.; Mackay, M. T.; Marom, D. R.; Morton, J. E. V.; Salvatici, E.; Moutard, M.-L.; Tacke, U.; Ostergaard, J. R.; Murray, K.; Nabbout, R.; Nampoothiri, S.; Nunez-Enamorado, N.; Tonduti, D.; Régal, L.; Pérez-Dueñas, B.; Tan, T. Y.; Prendiville, J. S.; Segers, K. A.; Ricci, F.; Valente, E. M.; Rio, M.; Webb, H. J.; Rodriguez, D.; Sinha, G. P.; Soler, D.; Spiegel, R.; Livingston, J. H.; te Water Naude, J.; Stödberg, T. I.; Straussberg, R.; Swoboda, K. J.; Suri, M.; Whitehouse, W. P.; Abdel-Salam, G. M.; Van Coster, R. N.; Wee Teik, K.; Rozenberg, F.; Thomas, M. M.; Till, M.; van der Knaap, M. S.; Ackroyd, S.; Vassallo, G.; Bahi-Buisson, N.; Whitney, R. N.; Vijzelaar, R.; Vogt, J.; Wallace, G. B.; Wassmer, E.; Bernard, G.; Lebon, P.; Zaki, M. S.; Bailey, K. M.; Zuberi, S. M.; Aeby, A.; Vanderver, A.; Bianchi, M.; Orcesi, S.; Cereda, C.; Rice, G. I.; Agosta, G.; Albin, C.; Allon-Shalev, S.; Crichiutti, G.; Barnerias, C.; Arellano, M.; Ariaudo, G.; Aswani, V.; Babul-Hirji, R.; Chandler, K. E.; Baildam, E. M.; Billette de Villemeur, T.; Barth, M.; Dabydeen, L.; Battini, R.; Beresford, M. W.; Blair, E. M.; Bloom, M.; Burlina, A. B.; Figueiredo, A.; Chitayat, D. A.; Carpanelli, M. L.; Carvalho, D. R.; Castro-Gago, M.; Cavallini, A.; Isidor, B.; Dale, R. C.; Collins, A. E.; Gener, B.; Sierra Corcoles, C.; Cordeiro, N.J. V.; D'Arrigo, S.; Kara, B.; De Goede, C. G E L; La Piana, R.; De Laet, C.; De Waele, L.M. H.; Denzler, I.; Desguerre, I.; Marques Lourenço, C.; Goizet, C. (Wiley, 2015-02)
Aicardi-Goutières syndrome is an inflammatory disease occurring due to mutations in any of TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR or IFIH1. We report on 374 patients from 299 families with mutations in these ...
Assessment of interferon-related biomarkers in Aicardi-Goutières syndrome associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, and ADAR: a case-control study

Rice, G. I.; Forte, G. M. A.; Szynkiewicz, M.; Chase, D. S.; Aeby, A.; Abdel-Hamid, M. S.; Ackroyd, S.; Allcock, R.; Bailey, K. M.; Balottin, U.; Barnerias, C.; Bernard, G.; Bodemer, C.; Botella, M. P.; Cereda, C.; Chandler, K. E.; Dabydeen, L.; Dale, R. C.; De Laet, C.; De Goede, C. G E L; del Toro, M.; Effat, L.; Enamorado, N. N.; Fazzi, E.; Gener, B.; Haldre, M.; Lin, J.-P. S-M; Livingston, J. H.; Lourenco, C. M.; Marques, W.; Oades, Patrick; Peterson, P.; Rasmussen, M.; Roubertie, A.; Schmidt, J. L.; Shalev, S. A.; Simon, R.; Spiegel, R.; Swoboda, K. J.; Temtamy, S.A.; Vassallo, G.; Vilain, C.N.; Vogt, J.; Wermenbol, V.; Whitehouse, W. P.; Soler, D.; Olivieri, I.; Orcesi, S.; Aglan, M. S.; Zaki, M. S.; Abdel-Salam, G. M. H.; Vanderver, A.; Kisand, K.; Rozenberg, F.; Lebon, P.; Crow, Y. J. (Elsevier, 2013-12)
Airflow limitation following cardiopulmonary exercise testing and heavy-intensity intermittent exercise in children with cystic fibrosis.

Stevens, D.; Oades, Patrick; Williams, Craig A. (Springer, 2015-02)
The clinical importance of exercise testing and training in the healthcare management of young patients with cystic fibrosis (CF) is growing. The aim of the present study was to determine the incidence of airflow limitation ...
Impaired aerobic function in patients with cystic fibrosis during ramp exercise.

Saynor, Z.; Barker, A. R.; Oades, Patrick; Williams, Craig A. (Wolters Kluwer, 2014-12)
This study aimed to document the matching of muscle O2 delivery to O2 use in young patients with cystic fibrosis (CF) from muscle deoxygenation (HHb) dynamics during ramp exercise.
Exercise metabolism during moderate-intensity exercise in children with cystic fibrosis following heavy-intensity exercise.

Stevens, D.; Oades, Patrick; Armstrong, N.; Williams, Craig A. (NRC Research Press, 2011-12)
Muscle metabolism is increased following exercise in healthy individuals, affecting exercise metabolism during subsequent physical work. We hypothesized that following heavy-intensity exercise (HIE), disease factors in ...
A protocol to determine valid V˙O2max in young cystic fibrosis patients.

Saynor, Z.; Barker, A. R.; Oades, Patrick; Williams, Craig A. (Elsevier, 2013-11)
Measuring aerobic fitness (V˙O2max) via a maximal cardiopulmonary exercise test is an important clinical tool in cystic fibrosis. This study sought to establish: (1) the validity of traditional criteria to verify maximal ...
Reproducibility of maximal cardiopulmonary exercise testing for young cystic fibrosis patients.

Saynor, Z.; Barker, A. R.; Oades, Patrick; Williams, Craig A. (Elsevier, 2013-12)
The reproducibility of cardiopulmonary exercise testing (CPET) has not been established in young cystic fibrosis (CF) patients using a valid protocol.
A survey of exercise testing and training in UK cystic fibrosis clinics.

Stevens, D; Oades, Patrick; Armstrong, N.; Williams, Craig A. (Elsevier, 2010-09)
Exercise testing is a valuable prognostic tool and exercise training has many health benefits in cystic fibrosis (CF). The objective of this study was to survey the provision of exercise testing and training in UK CF clinics.
Letter to the Editor: Validity and reliability concerns associated with cardiopulmonary exercise testing young people with cystic fibrosis. Response to: Statement on Exercise Testing in Cystic Fibrosis (Hebestreit et al., 2015 Respiration 90(4):332-51)

Saynor, Z.; Barker, A. R.; Oades, Patrick; Tomlinson, Owen; Williams, Craig A. (Karger, 2016)

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