What question did this study set out to answer?

Assess the presence and extent of myocardial involvement across familial cases of Becker muscular dystrophy, facioscapulohumeral dystrophy, and Bethlem myopathy.

May 1, 1992Muscle & Nerve

The heart in becker muscular dystrophy, facioscapulohumeral dystrophy, and bethlem myopathy

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Population

91 patients with familial muscular dystrophies, including 33 cases of Becker muscular dystrophy, 31 cases of…

Design

Cross-sectional

Authors

WVWillem G. de VoogtSint Lucas Andreas Hospital GRGuusje V. la RivièreUniversity of Amsterdam

Discussion

Member takes

Implication

Supports cardiac evaluation in BMD; leaves open monitoring needs in FSHD and Bethlem myopathy.

Key Points

Assess the presence and extent of myocardial involvement across familial cases of Becker muscular dystrophy, facioscapulohumeral dystrophy, and Bethlem myopathy.
Evaluated 33 familial cases of Becker muscular dystrophy, 31 of facioscapulohumeral dystrophy, and 27 of Bethlem myopathy.
Conducted physical examinations, chest X-rays, standard electrocardiography (EKG), and echocardiography in all patients, supplemented by continuous 24-hour EKG Holter monitoring in facioscapulohumeral dystrophy.
Thirteen patients with Becker muscular dystrophy (45%) exhibited EKG abnormalities similar to Duchenne muscular dystrophy, with only 1 of the 13 being wheelchair-dependent.
No cardiac alterations were detected in patients with facioscapulohumeral dystrophy, while only 1 patient with Bethlem myopathy presented with asymmetrical septal hypertrophy.

Structured PICO

Population

91 patients with familial muscular dystrophies, including 33 cases of Becker muscular dystrophy (BMD), 31 cases of facioscapulohumeral (FSH) dystrophy, and 27 cases of Bethlem myopathy.

Outcome

Cardiac involvement assessed by physical examination, chest x-ray, electrocardiographic (EKG), and echocardiographic examination.surrogate

Cardiac involvement is common in Becker muscular dystrophy but rare or absent in facioscapulohumeral dystrophy and Bethlem myopathy.

Cite This Study

Voogt et al. (1992) studied this question.

synapsesocial.com/papers/6a857fec57a6378aebd0bdb7 https://doi.org/10.1002/mus.880150510

Topics

Echocardiography Cardiomyopathy

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The heart in becker muscular dystrophy, facioscapulohumeral dystrophy, and bethlem myopathy

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Topics

The heart in becker muscular dystrophy, facioscapulohumeral dystrophy, and bethlem myopathy

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Also Consider

Topics

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