S.P.23 Percent predicted forced vital capacity is a viable outcome measure in Laminin alpha 2 – Deficient congenital muscular dystrophy

2012 ◽  
Vol 22 (9-10) ◽  
pp. 893-894
Author(s):  
J. Collins ◽  
R. Vandyke ◽  
M. Fenchel ◽  
M. McCallum ◽  
S. Volker ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Outcome Measure ◽  
Congenital Muscular Dystrophy

P.1.3 Postural changes in forced vital capacity characterize congenital muscular dystrophy subtypes

2013 ◽  
Vol 23 (9-10) ◽  
pp. 742
Author(s):  
K.G. Meilleur ◽  
M.M. Linton ◽  
A. Rutkowski ◽  
M.E. Leach ◽  
M. Jain ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Congenital Muscular Dystrophy ◽  
Postural Changes

Performance of the Upper Limb Module—A Reliable and Valid Evaluation for Chinese Patients with Duchenne Muscular Dystrophy

2020 ◽  
Author(s):  
Yuen Yee Alice Chiu ◽  
Chun Wai Lo ◽  
Chi Kuk Connie Hui ◽  
Wai Chong Susanna Choi ◽  
So Lun Lee ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Forced Vital Capacity ◽  
Motor Performance ◽  
Vital Capacity ◽  
High Reliability ◽  
Reliability And Validity ◽  
Chinese Patients ◽  
Rater Reliability

Abstract Background Duchenne muscular dystrophy is a genetic disease leading to progressive muscle weakness and degeneration. Effective assessment tool is needed to allow monitoring of progress to guide the management. This study assessed the reliability and validity of the Performance of Upper Limb (PUL) Module when used in patients with Duchenne Muscular Dystrophy (DMD). MethodsTotal thirty-three Chinese DMD patients were included. Twenty-five video-recorded PUL Module version 1.3 assessments were performed for the recruited patients with three raters evaluated the same recorded video for inter-rater reliability and evaluated the same performance one month later for intra-rater reliability. Construct validity was assessed correlating the PUL Module scores with the patients’ age, their forced vital capacity (N=25) and their Hammersmith motor scale scores (N=25) performed on the same day. ResultsThe intra-rater and inter-rater reliability (ICC 0.92 - 0.99), internal consistency (Cronbach’s alpha 0.97 - 0.99) and known groups validity (AUC 0.97) of PUL module were excellent. PUL was negatively correlated with age (r = -0.912), and positively correlated with the forced vital capacity (r = 0.87) and the Hammersmith motor scale (r = 0.84). The findings confirm the high reliability and validity of PUL module, and its high clinical relevancy in monitoring the deteriorating upper limb motor performance that strongly correlated with the lung function and generalized motor performance as age increased in DMD. ConclusionThis first study of PUL module in Chinese patients with DMD confirmed that it is a reliable valid tool to monitor clinical progress and outcome for DMD.

P4.57 Effects of air stacking on peak cough flows and forced vital capacity in patients with muscular dystrophy and spinal muscular atrophy

2011 ◽  
Vol 21 (9-10) ◽  
pp. 721 ◽  
Author(s):  
T.B. Carvalho ◽  
M.B. Resende ◽  
E. Zanoteli ◽  
J.M. Salge ◽  
A.S. Nakashima ◽  
...  
Keyword(s):  
Muscular Dystrophy ◽  
Spinal Muscular Atrophy ◽  
Forced Vital Capacity ◽  
Vital Capacity ◽  
Muscular Atrophy

A longitudinal study of forced vital capacity in Duchenne muscular dystrophy

1997 ◽  
Vol 7 (6-7) ◽  
pp. 435
Author(s):  
M.F. Phillips ◽  
R. Quinlivan ◽  
R.H.T. Edwards ◽  
P.M.A. Calverley
Keyword(s):  
Longitudinal Study ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Forced Vital Capacity ◽  
Vital Capacity
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