DUCHENNE MUSCULAR DYSTROPHY AND STEROID THERAPY: EVALUATION OF PATIENTS' MOTOR PERFORMANCE

2006 ◽  
Vol 37 (S 1) ◽  
Author(s):  
SL Parreira ◽  
MBD Resende ◽  
R Cardoso Alves ◽  
MDC Peduto ◽  
MS Carvalho ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Steroid Therapy ◽  
Motor Performance ◽  
Therapy Evaluation

T.P.49 Low-dose steroid therapy for cardiomyopathy in Duchenne Muscular Dystrophy patients

2012 ◽  
Vol 22 (9-10) ◽  
pp. 866
Author(s):  
K. Ishigaki ◽  
T. Murakami ◽  
T. Saito ◽  
T. Sato ◽  
S. Kajino ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Steroid Therapy ◽  
Low Dose

Vitamin D in the prevention and treatment of comorbid conditions in Duchenne muscular dystrophy

2021 ◽  
Vol 2 (1) ◽  
pp. 38-50
Author(s):  
Tatiana A. Gremiakova ◽  
Vasiliy M. Souslov ◽  
Gulzhan E. Sakbaeva ◽  
Andrey A. Stepanov
Keyword(s):  
Vitamin D ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Steroid Therapy ◽  
Motor Development ◽  
Smooth Muscles ◽  
Comorbid Conditions ◽  
Increased Risk ◽  
Positive Effect ◽  
Tubular Bones

Duchenne muscular dystrophy (DMD) is an X-linked recessive degenerative neuromuscular disorder due to a deficiency of dystrophin protein. This protein is most common in skeletal and cardiac muscles, to a lesser extent in smooth muscles and the brain. With DMD, progressive damage and muscle degeneration, a delay in motor development, and respiratory cardiac disorders are progressing. Patients with DMD have an increased risk of developing osteoporosis, fractures of the tubular bones and vertebrae, and neurocognitive impairment. Vitamin D is recommended prophylactically for DMD since many studies have shown its deficiency. The purpose of this work is to consolidate the literature data on the vitamin D deficiency in DMD patients and its effects on the development of concurrent comorbid conditions of the musculoskeletal, endocrine, and nervous systems. The authors discuss data concerning the appropriate level of vitamin D throughout the life span of DMD has a positive effect on the course of the disease patients’ quality of life ends. Primary clinical outcomes of vitamin D normalization include prevention of the development of osteoporosis (especially after the start of steroid therapy), fractures of the tubular bones and vertebrae, prolonged ability to walk, more effective treatment with bisphosphonates, including a decrease in the number of complications during initial use and lower jaw necrosis, positive effect on the expression of autistic spectrum symptoms. For patients with long-term steroid therapy, metabolic and liver disorders, calcidiol could be used, allowing quick deficiency compensation instead of standard vitamin D preparations.

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.

Steroid therapy in duchenne muscular dystrophy (DMD)

1992 ◽  
Vol 8 (5) ◽  
pp. 353
Author(s):  
Alberto L. Dubrovsky ◽  
Lilia Mesa ◽  
José Corderi ◽  
Patricia Marco ◽  
Daniel Flores
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Steroid Therapy

Trends of steroid therapy for Duchenne muscular dystrophy in Japan

2015 ◽  
Vol 25 ◽  
pp. S199
Author(s):  
F. Takeuchi ◽  
H. Komaki ◽  
H. Nakamura ◽  
N. Yonemoto ◽  
K. Kashiwabara ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Steroid Therapy

Correlation between motor performance scales, body composition, and anthropometry in patients with duchenne muscular dystrophy

2012 ◽  
Vol 113 (2) ◽  
pp. 133-137 ◽  
Author(s):  
Erhan Bayram ◽  
Yasemin Topcu ◽  
Pakize Karakaya ◽  
Meral Torun Bayram ◽  
Ebru Sahin ◽  
...  
Keyword(s):  
Body Composition ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Motor Performance

Steroid Therapy and Cardiac Function in Duchenne Muscular Dystrophy

2005 ◽  
Vol 26 (6) ◽  
pp. 768-771 ◽  
Author(s):  
L.W. Markham ◽  
R.L. Spicer ◽  
P.R. Khoury ◽  
B.L. Wong ◽  
K.D. Mathews ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Cardiac Function ◽  
Steroid Therapy

scholarly journals All-Cause Mortality and Cardiovascular Outcomes With Prophylactic Steroid Therapy in Duchenne Muscular Dystrophy

2013 ◽  
Vol 61 (9) ◽  
pp. 948-954 ◽  
Author(s):  
Gernot Schram ◽  
Anne Fournier ◽  
Hugues Leduc ◽  
Nagib Dahdah ◽  
Johanne Therien ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Steroid Therapy ◽  
Cardiovascular Outcomes ◽  
All Cause Mortality
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