Steroid therapy is associated with decreased numbers of dendritic cells and fibroblasts, and increased numbers of satellite cells, in the dystrophic skeletal muscle

Hussein, Mahmoud R.; Abu-Dief, Eman E.; Kamel, Nageh F.; Mostafa, Mohammed Mahmoud

doi:10.1136/jcp.2010.078204

Cited by 16 publications

(10 citation statements)

References 33 publications

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“…It is therefore likely that the anti‐inflammatory effects of corticosteroid treatment, by way of nuclear factor‐kappa B inhibition, may indirectly improve the myogenic potential of muscle stem cells. Interestingly, recent reports also suggest that steroid therapy may increase the number of muscle satellite cells within dystrophic skeletal muscle . In fact, we recently found that mTOR activity was markedly increased in the skeletal muscles of dKO mice, suggesting that its upregulation may be responsible for the rapid depletion of muscle stem cells.…”

Section: Paradigm Shiftsmentioning

confidence: 82%

Evolving paradigms in clinical pharmacology and therapeutics for the treatment of Duchenne muscular dystrophy

Huard

Lü

2016

Clin Pharma and Therapeutics

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Progressive muscle weakness and degeneration due to the lack of dystrophin eventually leads to the loss of independent ambulation by the middle of the patient's second decade, and a fatal outcome due to cardiac or respiratory failure by the third decade. More specifically, loss of sarcolemmal dystrophin and the dystrophin-associated glycoprotein (DAG) complex promotes muscle fiber damage during muscle contraction. This process results in an efflux of creatine kinase (CK), an influx of calcium ions, and the recruitment of T cells, macrophages, and mast cells to the damaged muscle, causing progressive myofiber necrosis. For the last 20 years, the major goal in the development of therapeutic approaches to alleviate muscle weakness in DMD has been centered on the restoration of dystrophin or proteins that are analogous to dystrophin, such as utrophin, through a variety of modalities including cell therapy, gene therapy, gene correction, and the highly promising techniques utilizing CRISPR/Cas9 technology. Despite the development of new therapeutic options, there still exist numerous challenges that we must face with regard to these new strategies and, consequently, we still do not have any feasible options available to ultimately slow the progression of this devastating disease. The purpose of this article is to highlight the current knowledge and advancements in the evolving paradigms in clinical pharmacology and therapeutics for this devastating musculoskeletal disease.

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Section: Paradigm Shiftsmentioning

confidence: 82%

Evolving paradigms in clinical pharmacology and therapeutics for the treatment of Duchenne muscular dystrophy

Huard

Lü

2016

Clin Pharma and Therapeutics

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“…Very few DC expressing CD1a were detected: in 1 of 2 DM specimens and in 6 of 11 PM specimens. It is noteworthy that in Duchenne muscular dystrophy and in Becker muscular dystrophy, prednisone treatment resulted in reduced DC among the infiltrating cells in the dystrophic muscles 34,35 .…”

Section: Discussionmentioning

confidence: 97%

“…One example is the 2000 report of DC-expressing factor XIIIa and HLA-DR in Duchenne muscular dystrophy and limb-girdle muscular dystrophy 36 . The identification of DC in Duchenne muscular dystrophy and in Becker muscular dystrophy, by means of electron microscopy with euchromatic nuclei and multiple long dendrites, was described in 2010 34 .…”

Section: Rheumatologymentioning

confidence: 99%

Fascin-expressing Dendritic Cells Dominate in Polymyositis and Dermatomyositis

Gendek-Kubiak

Gendek²

2012

J Rheumatol

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“…The most effective treatments for DMD may be molecular therapies, such as therapies that induce expression of the missing dystrophin protein, increase muscle strength, or reduce muscle fibrosis [4]; most of these therapies have been used in experimental or preclinical settings, with results comparable or superior to those obtained with corticosteroids [5][6][7][8]. Corticosteroids are considered the only currently available, gold-standard treatment for DMD; they enhance muscle strength and delay disease progression, probably by inhibiting myofibril degeneration and inflammation [9,10]. The most commonly used corticosteroid is prednisone or its oxazoline derivative, deflazacort.…”

Section: Introductionmentioning

confidence: 98%

A novel treatment regimen for Duchenne muscular dystrophy

Cai²,

Zhong³

et al. 2013

NeuroReport

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Duchenne muscular dystrophy (DMD) is the most common, X-linked genetic, skeletal muscle disease, with various regimens of treatment. The objective of this study was to determine the safety and efficacy of a novel treatment regimen for this disease. Thirty boys with DMD were administered prednisone according to the following regimen: in the first year, 1.5 mg/kg/day for the first 3 months, 1.0 mg/kg/day for the next 3 months, 0.75 mg/kg/day for the next 3 months, and 0.5 mg/kg/day for the last 3 months. In the second year, prednisone was administered 0.5 mg/kg on the alternate day for 12 months. The muscle strength (Medical Research Council sum score and Gower's sign), serum enzymes (creatine kinase, creatine kinase isoenzyme-2, and lactate dehydrogenase), pulmonary function (forced vital capacity, maximum voluntary ventilation), body weight, height, and BMI were determined before treatment and 3, 6, 9, 12, and 24 months after treatment. The results showed that the patients' mean Medical Research Council sum score increased from 46.1 at the baseline to 53.6 at 12 months and was maintained at 24 months. Gower's sign disappeared in 22 (73.3%) patients at 12 months and 21 (70.0%) at 24 months. The serum levels of creatine kinase, creatine kinase isoenzyme-2, and lactate dehydrogenase decreased and pulmonary function improved after 24 months of treatment. Significantly increased weight gain, osteoporosis, and cushingoid features were not observed. Our results suggested that this novel prednisone regimen for DMD has similar efficacy and safety as other regimens.

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Steroid therapy is associated with decreased numbers of dendritic cells and fibroblasts, and increased numbers of satellite cells, in the dystrophic skeletal muscle

Abstract: The ability of steroids to induce ultrastructural features of improvement supports the notion that they have beneficial therapeutic role. The clinical ramifications of these observations mandate further studies.

Cited by 16 publications

References 33 publications

Evolving paradigms in clinical pharmacology and therapeutics for the treatment of Duchenne muscular dystrophy

Evolving paradigms in clinical pharmacology and therapeutics for the treatment of Duchenne muscular dystrophy

Fascin-expressing Dendritic Cells Dominate in Polymyositis and Dermatomyositis

A novel treatment regimen for Duchenne muscular dystrophy

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