Life spans of Duchenne muscular dystrophy patients in the hospital care program in Japan

Mukoyama, Masakuni; Kondo, Kiyotaro; Hizawa, Kazuo; Nishitani, Hiroshi

doi:10.1016/0022-510x(87)90092-x

Cited by 43 publications

(24 citation statements)

References 3 publications

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“…There was no difference in baseline characteristics between the treatment groups. At the end of phase I, mean LVEF was 60.7 Ϯ 7.6% in group 1 versus 64.4 Ϯ 9.8% in group 2, and was Ͻ45% in a single patient in each group (p ϭ NS Duchenne muscular dystrophy (DMD), an inherited X-linked disease associated with absence of dystrophin (1), is characterized by progressive muscle weakness and inescapable cardiac involvement (1,2), fatal in approximately 40% of patients (3,4). Angiotensin-converting enzyme inhibitors (ACEI) are effective in overt congestive heart failure (CHF) (5) and have prophylactic effects in the Syrian hamster, an experimental model of beta-sarcoglycanopathy phenotypically similar to DMD (6,7).…”

Section: Resultsmentioning

confidence: 99%

Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy

Duboc¹,

Meune²,

Lerebours

et al. 2005

Journal of the American College of Cardiology

354

177

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Section: Resultsmentioning

confidence: 99%

Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy

Duboc¹,

Meune²,

Lerebours

et al. 2005

Journal of the American College of Cardiology

354

177

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“…, and death usually occurs before 25 years of age (5,6). However, the ages of these lifetime events have changed as various new forms of medical support have become available; ventilator support, active rehabilitation, physiotherapy for airway clearance, and so on (7)(8)(9)(10).…”

Section: Duchenne Muscular Dystrophy (Dmd) Is An X-linked Recessive Dmentioning

confidence: 99%

Mental Retardation and Lifetime Events of Duchenne Muscular Dystrophy in Japan

Mochizuki

Miyatake²,

Suzuki³

et al. 2008

Intern. Med.

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“…Ultimately progression toward heart failure, characterized by pronounced ventricular fibrosis, will occur in 40% of patients between 10 and 30 years of age [16,17,21,22].…”

Section: Characteristics Of the Dystrophic Cardiomyopathy In Dmdmentioning

confidence: 99%

Alterations in mitochondrial function as a harbinger of cardiomyopathy: Lessons from the dystrophic heart

Burelle

Khairallah

Ascah

et al. 2010

Journal of Molecular and Cellular Cardiology

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While compelling evidence supports the central role of mitochondrial dysfunction in the pathogenesis of heart failure, there is comparatively less information available on mitochondrial alterations that occur prior to failure. Building on our recent work with the dystrophin-deficient mdx mouse heart, this review focuses on how early changes in mitochondrial functional phenotype occur prior to overt cardiomyopathy and may be a determinant for the development of adverse cardiac remodelling leading to failure. These include alterations in energy substrate utilization and signalling of cell death through increased permeability of mitochondrial membranes, which may result from abnormal calcium handling, and production of reactive oxygen species. Furthermore, we will discuss evidence supporting the notion that these alterations in the dystrophin-deficient heart may represent an early "subclinical" signature of a defective nitric oxide/cGMP signalling pathway, as well as the potential benefit of mitochondria-targeted therapies. While the mdx mouse is an animal model of Duchenne muscular dystrophy (DMD), changes in the structural integrity of dystrophin, the mutated cytoskeletal protein responsible for DMD, have also recently been implicated as a common mechanism for contractile dysfunction in heart failure. In fact, altogether our findings support a critical role for dystrophin in maintaining optimal coupling between metabolism and contraction in the heart.

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Life spans of Duchenne muscular dystrophy patients in the hospital care program in Japan

Cited by 43 publications

References 3 publications

Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy

Effect of perindopril on the onset and progression of left ventricular dysfunction in Duchenne muscular dystrophy

Mental Retardation and Lifetime Events of Duchenne Muscular Dystrophy in Japan

Alterations in mitochondrial function as a harbinger of cardiomyopathy: Lessons from the dystrophic heart

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