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 ◽  
...  
2012 ◽  
Vol 22 (9-10) ◽  
pp. 893-894
Author(s):  
J. Collins ◽  
R. Vandyke ◽  
M. Fenchel ◽  
M. McCallum ◽  
S. Volker ◽  
...  

2020 ◽  
Author(s):  
Yuen Yee Alice Chiu ◽  
Chun Wai Lo ◽  
Chi Kuk Connie Hui ◽  
Wai Chong Susanna Choi ◽  
So Lun Lee ◽  
...  

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.


2011 ◽  
Vol 21 (9-10) ◽  
pp. 721 ◽  
Author(s):  
T.B. Carvalho ◽  
M.B. Resende ◽  
E. Zanoteli ◽  
J.M. Salge ◽  
A.S. Nakashima ◽  
...  

1997 ◽  
Vol 7 (6-7) ◽  
pp. 435
Author(s):  
M.F. Phillips ◽  
R. Quinlivan ◽  
R.H.T. Edwards ◽  
P.M.A. Calverley

Author(s):  
Longxiang Su ◽  
Yinghua Guo ◽  
Yajuan Wang ◽  
Delong Wang ◽  
Changting Liu

AbstractTo explore the effectiveness of microgravity simulated by head-down bed rest (HDBR) and artificial gravity (AG) with exercise on lung function. Twenty-four volunteers were randomly divided into control and exercise countermeasure (CM) groups for 96 h of 6° HDBR. Comparisons of pulse rate, pulse oxygen saturation (SpO2) and lung function were made between these two groups at 0, 24, 48, 72, 96 h. Compared with the sitting position, inspiratory capacity and respiratory reserve volume were significantly higher than before HDBR (0° position) (P< 0.05). Vital capacity, expiratory reserve volume, forced vital capacity, forced expiratory volume in 1 s, forced inspiratory vital capacity, forced inspiratory volume in 1 s, forced expiratory flow at 25, 50 and 75%, maximal mid-expiratory flow and peak expiratory flow were all significantly lower than those before HDBR (P< 0.05). Neither control nor CM groups showed significant differences in the pulse rate, SpO2, pulmonary volume and pulmonary ventilation function over the HDBR observation time. Postural changes can lead to variation in lung volume and ventilation function, but a HDBR model induced no changes in pulmonary function and therefore should not be used to study AG CMs.


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