What is the clinical evidence from this study?

Study design: Review. Population: Dystrophinopathies.

What question did this study set out to answer?

To review the cardiac pathology and clinical manifestations of dystrophin deficiency, highlighting protocols for cardiac screening and monitoring across dystrophinopathies.

January 1, 2008Journal of Pediatric Rehabilitation Medicine

Cardiac involvement in the dystrophinopathies

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Key result

This review details the cardiac manifestations of dystrophinopathies and outlines principles for screening and monitoring cardiac status in patients with myopathies and muscular dystrophies.

Population

Patients with dystrophinopathies, including Duchenne Muscular Dystrophy, Becker Muscular Dystrophy, and…

Design

Review

Authors

MRMark W. RussellPediatric Cardiology DTDeWayne TownsendUniversity of Minnesota JMJoseph M. MetzgerHeart Failure / Cardiomyopathy

Discussion

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Implication

May guide cardiac screening in dystrophinopathies; leaves open prospective validation of protocols.

Key Points

To review the cardiac pathology and clinical manifestations of dystrophin deficiency, highlighting protocols for cardiac screening and monitoring across dystrophinopathies.
Narrative synthesis of clinical features and cardiac involvement across dystrophinopathies, including Duchenne muscular dystrophy, Becker muscular dystrophy, and X-linked dilated cardiomyopathy.
Examination of clinical screening and cardiac surveillance strategies applicable to patients with inherited myopathies.
Deficiency or absence of functional dystrophin drives progressive striated muscle degeneration, culminating in cardiorespiratory failure typically by the second to third decade in Duchenne muscular dystrophy.
Cardiac manifestations manifest across a spectrum of dystrophin disorders, presenting as milder involvement in Becker muscular dystrophy and as the predominant phenotype in X-linked dilated cardiomyopathy.

Structured PICO

Population

Patients with dystrophinopathies, including Duchenne Muscular Dystrophy (DMD), Becker Muscular Dystrophy (BMD), and X-linked dilated cardiomyopathy (XLDCM).

This review summarizes the cardiac manifestations of dystrophinopathies and discusses the principal elements of screening and monitoring cardiac status in these patients.

Cite This Study

Russell et al. (2008) conducted a review in Dystrophinopathies. This review details the cardiac manifestations of dystrophinopathies and outlines principles for screening and monitoring cardiac status in patients with myopathies and muscular dystrophies.

synapsesocial.com/papers/6a930b0b90d586981c41a646 https://doi.org/10.3233/prm-2008-00037

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Cardiac involvement in the dystrophinopathies

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Cardiac involvement in the dystrophinopathies

Discussion

Member takes

Implication

Key Points

Structured PICO

Cite This Study

Also Consider

Also Consider