Cardiac dysfunction with Becker muscular dystrophy

Saito, Miho; Kawai, Hisaomi; Akiyama, Masashi; Adachi, Kazutaka; Nishida, Yoshihito; Saito, Shiro

doi:10.1016/s0002-8703(96)90250-1

Cited by 80 publications

(62 citation statements)

References 21 publications

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“…In a series of 328 patients longitudinally studied for more than a decade the clinical incidence of cardiomyopathy increased steadily over the teen years with approximately one third of patients being affected by the age of 14, one half of patients by age of 18 and all adult patients (4). Patients with Becker muscular dystrophy (BMD), who have residual expression of dystrophin and a milder skeletal muscle involvement are at high risk of developing cardiomyopathy which often causes death due to heart failure (25). Given the technological improvement of respiratory management via various assist devices in recent years (26); it is predicted that abnormal cardiac function will emerge as an even more common feature of DMD.…”

Section: Discussionmentioning

confidence: 99%

Myostatin does not regulate cardiac hypertrophy or fibrosis

Cohn

Liang

Shetty

et al. 2007

Neuromuscular Disorders

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Myostatin is a negative regulator of muscle growth. Loss of myostatin has been shown to cause increase in skeletal muscle size and improve skeletal muscle function and fibrosis in the dystrophindeficient mdx mouse, an animal model for Duchenne muscular dystrophy. We evaluated whether lack of myostatin has an impact on cardiac muscle growth and fibrosis in vivo. Using genetically modified mice we assessed whether myostatin absence induces similar beneficial effects on cardiac function and fibrosis. Cardiac mass and ejection fraction were measured in wild type, myostatin-null, mdx and double mutant mdx/myostatinnull mice by high resolution echocardiography. Heart mass, myocyte area and extent of cardiac fibrosis were determined post mortem. Myostatin-null mice do not demonstrate ventricular hypertrophy when compared to wild type mice as shown by echocardiography (ventricular mass 0.69± 0.01 g vs.0.69±0.018 g, P = 0.75, respectively) and morphometric analyses including heart/body weight ratio (5.39±0.45mg/g vs. 5.62±0.58mg/g, P = 0.59 respectively) and cardiomyocyte area 113.67±1.5μm 2 , 116.85±1.9μm 2 ; P = 0.2). Moreover, absence of myostatin does not attenuate cardiac fibrosis in the dystrophin deficient mdx mouse model for DMD (12.2% vs. 12% respectively, P = 0.88). The physiological role of myostatin in cardiac muscle appears significantly different than that in skeletal muscle as it does not induce cardiac hypertrophy and does not modulate cardiac fibrosis in mdx mice.

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Section: Discussionmentioning

confidence: 99%

Myostatin does not regulate cardiac hypertrophy or fibrosis

Cohn

Liang

Shetty

et al. 2007

Neuromuscular Disorders

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“…There is no correlation between CI and the severity of skeletal muscle manifestations (14,16). There are also no correlations between the severity of CI and the deleted exons (4,17).…”

Section: Pathoanatomical Background Of CI In Bmdmentioning

confidence: 94%

“…CI in BMD may be subclinical (asymptomatic) and detectable only by instrumental investigations, or symptomatic. Asymptomatic CI occurs in most cases, and up to one-third of patients develop dilative cardiomyopathy (dCMP) with concomitant heart failure (4,5). The degree of symptomatic CI in BMD varies greatly between no (or hardly any) cardiac abnormalities to severe arrhythmias, hypertrophic cardiomyopathy (hCMP), dCMP, heart failure or sudden cardiac death (3,6).…”

mentioning

confidence: 99%

Cardiac involvement in Becker muscular dystrophy

Finsterer¹,

Stöllberger²

2008

Canadian Journal of Cardiology

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“…Olgumuzda H+E rutin boyasõnõn yanõ sõra uygulanan PAS, Van Gieson, Mason Trikom histokimyasal boyalarõ ve ABC immŸnoenzimatik yšntemi ile uygulanan Anti-Distrofin monoklonal antikoru doÛru tanõya ulaßõl-masõ amacõyla kullanõlmõßtõr. Becker tipi PMDÕde (18) kalp tulumun nadir olmasõ, Emery-Dreifuss MuskŸler Distrofilerinin (10) ise ilk semptomlarõn adšlesan dš-nemde ortaya •õkmasõ ve Anti-Distrofin monoklonal antikorunda izlenen boyama šzelliÛi nedeni ile Duchenne tipi PMD olarak yorumlandõ (3)(4)(5).…”

Section: Tartiþmaunclassified

Kardiyak Tutulumlu Progresif Musküler Distrofi Bir Olgu Sunumu

Gülmen¹,

Bilgin²,

Çekin³

et al. 2004

Bull Leg Med

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Ani beklenmedik ölüm nedenlerinin ülkelerin ulaştığı sağlık hizmetlerinin kalitesi ve yaygınlığına bağlı olarak çeşitlilik gösterdiği belirtilmektedir. Progresif Musküler Distrofi olgusu ani beklenmedik şüpheli ölüme ilginç bir örnek oluşturması nedeni ile sunulmak istendi. Adana Adli Tıp Kurumu Morg İhtisas Dairesi Başkanlığına gönderilen ölü muayene ve otopsi tutanağında, evinde ölmesi üzerine savcılığa haber verildiği ve Devlet Hastanesi morgunda otopsi yapıldığı, dış muayenesinde ve organların makroskobik incelenmesinde özellik tespit edilmediği histopotoljik ve toksikolojik inceleme için örneklerin alındığı kayıtlıdır. Çukurova Tıp Fakültesi Adli Tıp Anabilim Dalında yapılan histopatolojik değerlendirilmesinde; kalp kasmda Duchenne tipi Musküler Distrofi ile uyumlu myokardial bulgular saptandı. Olguda Progresif Musküler Distrofi (PMD) seyri sırasında ortaya çıkabilen kardiyak tutulum ölüm nedeni olarak kabul edildi. Anabilim dalında ilk kez saptanan PMD olgusu adli tıp çalışanlarının dikkatini çekmek amacıyla kaynaklar ışığında tartışılmaktadır.Anahtar kelimeler: progresif musküler distrofi; ani ölüm; adli patoloji; otopsi

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Cardiac dysfunction with Becker muscular dystrophy

Cited by 80 publications

References 21 publications

Myostatin does not regulate cardiac hypertrophy or fibrosis

Myostatin does not regulate cardiac hypertrophy or fibrosis

Cardiac involvement in Becker muscular dystrophy

Kardiyak Tutulumlu Progresif Musküler Distrofi Bir Olgu Sunumu

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