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Longitudinal pulmonary function testing outcome measures in Duchenne muscular dystrophy: Long-term natural history with and without glucocorticoids. Neuromuscular disorders : NMD McDonald, C. M., Gordish-Dressman, H., Henricson, E. K., Duong, T., Joyce, N. C., Jhawar, S., Leinonen, M., Hsu, F., Connolly, A. M., Cnaan, A., Abresch, R. T., CINRG investigators for PubMed, Dubrovsky, A., Kornberg, A., Ryan, M., Webster, R., Biggar, W. D., McAdam, L. C., Mah, J. K., Kolski, H., Vishwanathan, V., Chidambaranathan, S., Nevo, Y., Gorni, K., Carlo, J., Tulinius, M., Lotze, T., Bertorini, T. E., Day, J. W., Karachunski, P., Clemens, P. R., Abdel-Hamid, H., Teasley, J., Kuntz, N., Driscoll, S., Bodensteiner, J. B., Connolly, A. M., Pestronk, A., Abresch, R. T., Henricson, E. K., Joyce, N. C., McDonald, C. M., Cnaan, A., Morgenroth, L. P., Leshner, R., Tesi-Rocha, C., Thangarajh, M., Duong, T. 2018; 28 (11): 897–909

Abstract

We describe changes in pulmonary function measures across time in Duchenne muscular dystrophy patients treated with glucocorticoids (GCs)?>?1 year compared to GC naive patients in the Cooperative International Research Group Duchenne Natural History Study, a multicenter prospective cohort study. 397 participants underwent 2799 pulmonary function assessments over a period up to 10 years. Fifty-three GC naive participants (?1 year cumulative GC treatment. Forced vital capacity (FVC), peak expiratory flow rate (PEFr), maximal inspiratory and expiratory pressures were performed and calculated as a percent predicted (%p). GC treatment slowed the rate of pulmonary decline as measured by FVC%p, in patients aged 7-9.9 years. GC treatment slowed 12 and 24-month progression of percent predicted spirometry to a greater degree in those with baseline FVC%p from?

View details for DOI 10.1016/j.nmd.2018.07.004

View details for PubMedID 30336970