UNASSIGNED: Pulmonary function loss in @entity1 with @entity1555 ( @entity1555 ) is progressive and leads to @entity338 . The purpose of this study in 10-18 year old @entity1 with @entity1555 is the assessment of the inter-correlation between pulmonary function tests (PFTs), their reliability and the association with the general disease stage measured by the Brooke score. Dynamic PFTs (peak expiratory flow [PEF], forced vital capacity [FVC], forced expiratory volume in one second [FEV1]) and @entity551 ( @entity551 , MEP) were prospectively collected from 64 @entity1555 @entity1 enrolled in the DELOS trial (ClinicalTrials.gov, number NCT01027884). Baseline PEF percent predicted (PEF%p) was <80% and @entity1 had stopped taking glucocorticoids at least 12 months prior to study start. At baseline PEF%p, FVC%p and FEV1%p correlated well with each other (Spearman's rho: PEF%p-FVC%p: 0.54; PEF%p-FEV1%p: 0.72; FVC%p-FEV1%p: 0.91). @entity551 %p and MEP%p correlated well with one another ( @entity551 %p-MEP%p: 0.71) but less well with PEF%p ( @entity551 %p-PEF%p: 0.40; MEP%p-PEF%p: 0.41) and slightly better with FVC%p ( @entity551 %p-FVC%p: 0.59; MEP%p-FVC%p: 0.74). The within-subject coefficients of variation (CV) for successive measures were 6.97% for PEF%p, 6.69% for FVC%p and 11.11% for FEV1%p, indicating that these parameters could be more reliably assessed compared to @entity551 (CV for @entity551 %p: 18.00%; MEP%p: 15.73%). Yearly rates of PFT decline (placebo group) were larger in dynamic parameters (PEF%p: -8.9% [SD 2.0]; FVC%p: -8.7% [SD 1.1]; FEV1%p: -10.2% [SD 2.0]) than static airway pressures ( @entity551 %p: -4.5 [SD 1.3]; MEP%p: -2.8 [SD 1.1]). A considerable drop in dynamic pulmonary function parameters was associated with loss of upper limb function (transition from Brooke score category 4 to category 5). In conclusion, these findings expand the understanding of the reliability, correlation and evolution of different pulmonary function measures in @entity1555 @entity1 who are in the pulmonary function decline phase.

Characterization of pulmonary function in 10-18 year old XXXX with @entity1555 .

@entity1 :: ('9606', 'Species') :: ['patients'] @entity1555 :: ('MESH:D020388', 'Disease') :: ['Duchenne muscular dystrophy', 'DMD'] @entity338 :: ('MESH:D011665', 'Disease') :: ['pulmonary insufficiency'] @entity551 :: ('MESH:D007022', 'Disease') :: ['maximum static airway pressures', 'MIP']
@entity1 :: (9606,Species) :: ['patients']
