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Longitudinal changes in respiratory function in SBMA.
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posted on 2016-12-22, 19:06 authored by Shinichiro Yamada, Atsushi Hashizume, Yasuhiro Hijikata, Tomonori Inagaki, Keisuke Suzuki, Naohide Kondo, Kaori Kawai, Seiya Noda, Hirotaka Nakanishi, Haruhiko Banno, Akihiro Hirakawa, Haruki Koike, Katherine Halievski, Cynthia L. Jordan, Masahisa Katsuno, Gen SobueLongitudinal changes in %PEF (A), %VC (B), and FEV1.0/FVC (C) as a function of disease duration were analyzed. The solid lines indicate representative disease progression over disease duration calculated using a marginal model under an unstructured covariance matrix. The broken curvilinear line demonstrates the 95% confidence interval of these models. We calculated the estimated values at clinical onset (intercepts) and the change values per year (D). In an analysis using the marginal model, a generalized estimating equation (shown by the solid lines) identified that %PEF (A) and %FVC (B) demonstrated a slowly but with steady progression. PEF, peak expiratory flow; FVC, forced vital capacity; FEV1.0, forced expiratory volume in 1 s.
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trunk subscoresRT-PCRperoxisome proliferator-activated receptor deltamitochondrial biogenesis-relatedBulbar Muscular Atrophyfunction profilemotor functiongrip powercontrol subjectsDecreased Peak Expiratory Flow AssociatedMuscle Fiber-Type SwitchingmRNA levelsSBMA subjectsdisease durationPEFfast-twitch fibersADLexpression levelsALSmotor function testsperoxisome proliferator-activated receptor gamma coactivator 1-muscle strengthgenes encodingPredicted valuestongue pressureExplosive muscle strength
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