Functional ability and muscle force in healthy children and ambulant Duchenne muscular dystrophy patients

2005 ◽  
Vol 9 (6) ◽  
pp. 387-393 ◽  
Author(s):  
Ernesto A.C. Beenakker ◽  
Natalia M. Maurits ◽  
Johanna M. Fock ◽  
Oebele F. Brouwer ◽  
Johannes H. van der Hoeven
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Functional Ability ◽  
Muscle Force ◽  
Healthy Children

scholarly journals Pain Characteristics Among Individuals with Duchenne Muscular Dystrophy According to Their Clinical Stage

2022 ◽  
Author(s):  
Aram Kim ◽  
Mina Park ◽  
Hyung-Ik Shin
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Functional Ability ◽  
Assessment Tool ◽  
Clinical Stage ◽  
Pain Interference ◽  
Lumbosacral Region ◽  
Cross Sectional ◽  
Pain Frequency ◽  
Pain Characteristics

Abstract Background: Assessment of pain is not routine, standardized, or well-understood in individuals with Duchenne muscular dystrophy (DMD), even though pain is a common problem reported by more than half of DMD patients. Previous studies in this area included multiple neuromuscular diseases with highly variable phenotypes. Therefore, our aim was to focus on DMD specifically and evaluate comprehensive pain characteristics according to the disease stages, from ambulatory to late non-ambulatory. Methods: This was a cross-sectional study conducted in an out-patient pediatric rehabilitation clinic. Participants were 148 males with confirmed DMD, 14.5±5.3 years of age. Face-to-face interviews were conducted using a structured questionnaire regarding pain frequency, duration, intensity, location, aggravating/relieving factors, pain interference (Brief Pain Inventory), pain quality (PainDETECT Questionnaire), and functional ability (DMD Functional Ability Self-Assessment Tool). Pain characteristics were analyzed according to the clinical stage: ambulatory (Amb), early non-ambulatory (ENA), and late non-ambulatory (LNA).Results: Of the 148 participants who completed the assessment, 66 (44.6%) reported pain during the previous 4 weeks. There were no differences in the pain duration or intensity among the three groups. Pain location (Amb: calf, ENA: knee, LNA: lumbosacral region), aggravating factor (Amb: ambulation, ENA: transfer, LNA: sitting), and relieving factor (Amb: rest and massage, ENA and LNA: positional change) differed according to the clinical stage. Individuals in the LNA stage reported an increase in the frequency of pain and number of pain sites. The effect of pain on mood was also found to be greater in the LNA group than in the other clinical stages. Conclusion: Pain characteristics including location, aggravating/relieving factors, pain frequency, and pain interference change as the disease progresses in patients with DMD. Clinicians could more efficiently and critically assess and manage the patients’ pain aspect, based on these findings.

Absence of Major Side Effects of Nifedipine Following Treatment of Duchenne Dystrophy

PEDIATRICS ◽  
1985 ◽  
Vol 75 (6) ◽  
pp. 1168-1169
Author(s):  
RICHARD T. MOXLEY
Keyword(s):  
Skeletal Muscle ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Functional Ability ◽  
Laboratory Data ◽  
Blocking Agent ◽  
Duchenne Dystrophy ◽  
Double Blind ◽  
Channel Blocking ◽  
Sarcolemmal Integrity

Abnormal calcium accumulation in skeletal muscle due to altered sarcolemmal integrity may be important in the pathogenesis of Duchenne muscular dystrophy. To evaluate the potential beneficial effect of a calcium channel-blocking agent on the course of Duchenne muscular dystrophy, 105 patients were randomly selected for a double-blind 18-month study utilizing treatment with nifedipine (n = 54) or placebo (n = 51). Muscle strength, contractures, functional ability, cardiopulmonary changes, and laboratory data were monithred; the detailed results are published elsewhere.

scholarly journals Upper Limb Evaluation in Duchenne Muscular Dystrophy: Fat-Water Quantification by MRI, Muscle Force and Function Define Endpoints for Clinical Trials

PLoS ONE ◽  
2016 ◽  
Vol 11 (9) ◽  
pp. e0162542 ◽  
Author(s):  
Valeria Ricotti ◽  
Matthew R. B. Evans ◽  
Christopher D. J. Sinclair ◽  
Jordan W. Butler ◽  
Deborah A. Ridout ◽  
...  
Keyword(s):  
Clinical Trials ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Upper Limb ◽  
Muscle Force ◽  
Water Quantification ◽  
And Function

Muscle Pathology and Functional Ability in Children with Duchenne Muscular Dystrophy

2010 ◽  
Vol 42 ◽  
pp. 815-816
Author(s):  
Jasjit K. Deol ◽  
Ilona Schmalfuss ◽  
Donovan J. Lott ◽  
Sean A. Germain ◽  
Sunita Mathur ◽  
...  
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Functional Ability ◽  
Muscle Pathology

scholarly journals Motor assessment in patients with Duchenne muscular dystrophy

2012 ◽  
Vol 70 (6) ◽  
pp. 416-421 ◽  
Author(s):  
Gabriela Palhares Campolina Diniz ◽  
Laura Maria de Lima Belizário Facury Lasmar ◽  
Juliana Gurgel Giannetti
Keyword(s):  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Motor Function ◽  
Muscular Strength ◽  
Muscle Force ◽  
Medical Research Council ◽  
Significant Loss ◽  
Strong Correlations ◽  
Function Measure

OBJECTIVE: Evaluate muscle force and motor function in patients with Duchenne muscular dystrophy (DMD) in a period of six months. METHOD: Twenty children and adolescents with diagnosis of DMD were evaluated trough: measurement of the strength of the flexors and extensors of the shoulder, elbow, wrist, knee and ankle through the Medical Research Council (MRC), and application of the Motor Function Measure (MFM). The patients were evaluated twice within a six-month interval. RESULTS: Loss of muscle strength was identified in the MRC score for upper proximal members (t=-2.17, p=0.04). In the MFM, it was noted significant loss in the dimension 1 (t=-3.06, p=0.006). Moderate and strong correlations were found between the scores for muscular strength and the MFM dimensions. CONCLUSION: The MFM scale was a useful instrument in the follow up of patients with DMD. Moreover, it is a more comprehensive scale to assess patients and very good for conducting trials to evaluate treatment.

scholarly journals First manifestation and evolution of early left ventricular dysfunction in children with Duchenne muscular dystrophy

2021 ◽  
Vol 64 (5) ◽  
pp. 62-69
Author(s):  
Iulia Rodoman ◽  
◽  
Alexandr Dorif ◽  
Ina Palii ◽  
Victoria Sacara ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Working Group ◽  
Systolic Function ◽  
Rna Extraction ◽  
Left Ventricular ◽  
Control Group ◽  
Healthy Children ◽  
Creatine Kinase Mb

Background: Standard pediatric cardiology examinations and echocardiography fail to discover when the cardiomyopathy will occur in patient with Duchenne muscular dystrophy (DMD). Noninvasive markers are needed to fill this gap. Material and methods: This cohort study included a total number of 30 children (21 children (70%) with DMD and 9 (30%) healthy children. Blood samples were used for biochemical (level of creatine kinase, creatine kinase-MB, lactate dehydrogenase) and miRNA (presence of miR133a 3p, miR133b 3p, miR206 3p, miR208a 3p, miR208b 3p) analysis. All patients underwent partial conventional echocardiography ECOCG and Speckle Tracking. Results: The children in the working group presented compared to healthy children: FCC values increased by 15 (71%) vs 2 (22%), high levels of CK, CK-MB, LDH, which is characteristic for the disease and reflects its stage. Also, there is a decrease in systolic function indicators in the working group: mean FE 59 ± 3.8 %, and GLS: -16.2 ± 3.1%. MiRNA analyses confirmed the presence of miR133a 3p, miR133b 3p, miR206 3p, miR208a 3p, miR208b 3p in both working and control group. Conclusions: For the first time in the Republic of Moldova, we developed and adapted protocols for RNA extraction from human blood, performing screening of specific miRNA in the serum of patients with DMD and healthy children. Also, altered LV strain notwithstanding a normal or mildly modified LVEF represents an essential viewpoint for prospective pediatric drug trials in DMD-related cardiomyopathy prevention.

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