Duchenne Muscular Dystrophy

McDonald, Craig; Abresch, Richard T.; Carter, G. T.; Fowler, William M.; Johnson, Ernest H.; Kilmer, David D.; Sigford, B. J.

doi:10.1097/00002060-199509001-00003

Cited by 497 publications

(55 citation statements)

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“…This finding is consistent with the results of a previous study by McDonald et al [9]. However, unlike their results, lower extremity contractures were common in the ambulatory patients in our study, especially those involving the ankle joint.…”

Section: Discussionsupporting

confidence: 93%

“…Unlike previous studies, all of the patients in the present study were prescribed deflazacort. However, our results are similar to those obtained when steroids were not widely used [9]. Mendell et al [18] also suggested that although prednisone treatment improves the strength and functioning of patients with DMD, it has no significant effect on major joint ROM.…”

Section: Discussionsupporting

confidence: 89%

“…The severities of hip flexion (HF), knee flexion (KF), and ankle plantarflexion (APF) contractures were classified, based on a previous study [9], as mild (1°–19°), moderate (20°–40°), and severe (> 40°) for HF contractures; mild (1°–14°), moderate (15°–40°), and severe (> 40°) for KF contractures; and mild (1°–14°), moderate (15°–30°), and severe (> 30°) for APF contractures.…”

Section: Methodsmentioning

confidence: 99%

“…McDonald et al [9] reported that lower extremity contractures were rare in patients able to maintain an upright posture, but developed soon after the patients became confined to a wheelchair for the majority of the day. However, these authors did not describe any differences between the major lower extremity joints, i.e., the hip, knee, and ankle.…”

Section: Introductionmentioning

confidence: 99%

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Lower extremity joint contracture according to ambulatory status in children with Duchenne muscular dystrophy

Choi

Chun²,

Kim

et al. 2018

BMC Musculoskelet Disord

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BackgroundLower extremity joint contractures have negative effects on gait in children with Duchenne muscular dystrophy (DMD). Thus, contracture prevention is essential for maintaining a patient’s functional ability and an acceptable quality of life. This study investigated hip flexion (HF), knee flexion (KF), and ankle joint plantar flexion (APF) contractures among male patients with DMD, based on the patients’ ambulatory status. Differences in major joint contractures, based on passive stretching exercise participation, were also investigated.MethodsA total of 128 boys with DMD, followed at the DMD clinic of a tertiary care hospital, were included in this cross-sectional study. The passive ranges-of-motion of the hip, knee, and ankle joints were measured, in the sagittal plane, using a goniometer. The Vignos Scale was used to grade ambulatory function. Boys with DMD who performed stretching exercises for more than 5 min/session, > 3 sessions/week, were classified into the stretching group.ResultsThe HF (23.5o), KF (43.5o), and APF (34.5o) contracture angles in the non-ambulatory group were more severe than those in the ambulatory group. APF contractures (41 patients, 52.6%) were more frequently observed early, even within the ambulatory period, than were hip (8 patients, 10.3%), and knee joint (17 patients, 21.8%) contractures. Passive stretching exercises > 3 sessions/week were not associated with the degree of lower extremity joint contractures in the ambulatory or non-ambulatory group.ConclusionHF, KF, and APF contractures are more common and severe when there is deterioration of ambulatory function. Stretching exercises alone are unlikely to prevent lower extremity joint contractures.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionsupporting

confidence: 89%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Lower extremity joint contracture according to ambulatory status in children with Duchenne muscular dystrophy

Choi

Chun²,

Kim

et al. 2018

BMC Musculoskelet Disord

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“…Although DMD is present at birth, clinical symptoms are not evident until 3-5 years of age (10). Initial symptoms include leg weakness and increasing convex curvature of the spine muscles and results in progressive weakness, usually leaving DMD patients wheelchair bound by age 11 or 12 years (11). …”

Section: Introductionmentioning

confidence: 99%

Erythropoietin reduces the expression of myostatin in mdx dystrophic mice

Feder

Rugollini

Santomauro

et al. 2014

Braz J Med Biol Res

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Erythropoietin (EPO) has been well characterized as a renal glycoprotein hormone regulating red blood cell production by inhibiting apoptosis of erythrocyte progenitors in hematopoietic tissues. EPO exerts regulatory effects in cardiac and skeletal muscles. Duchenne muscular dystrophy is a lethal degenerative disorder of skeletal and cardiac muscle. In this study, we tested the possible therapeutic beneficial effect of recombinant EPO (rhEPO) in dystrophic muscles in mdx mice. Total strength was measured using a force transducer coupled to a computer. Gene expression for myostatin, transforming growth factor-β1 (TGF-β1), and tumor necrosis factor-α (TNF-α) was determined by quantitative real time polymerase chain reaction. Myostatin expression was significantly decreased in quadriceps from mdx mice treated with rhEPO (rhEPO=0.60±0.11, control=1.07±0.11). On the other hand, rhEPO had no significant effect on the expression of TGF-β1 (rhEPO=0.95±0.14, control=1.05±0.16) and TNF-α (rhEPO=0.73±0.20, control=1.01±0.09). These results may help to clarify some of the direct actions of EPO on skeletal muscle.

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