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Dystrophin analysis in idiopathic dilated cardiomyopathy.
  1. V V Michels,
  2. G M Pastores,
  3. P P Moll,
  4. D J Driscoll,
  5. F A Miller,
  6. J C Burnett,
  7. R J Rodeheffer,
  8. J A Tajik,
  9. A H Beggs,
  10. L M Kunkel
  1. Department of Medical Genetics, Mayo Clinic/Foundation, Rochester, Minnesota 55905.

    Abstract

    Idiopathic dilated cardiomyopathy (DCM) is characterised by ventricular dilatation and impaired systolic function resulting in congestive heart failure and frequently death. A dilated cardiomyopathy is common in patients with symptomatic Duchenne/Becker muscular dystrophy, a disease caused by dystrophin gene defects. However, cardiomyopathy is rarely the predominant clinical feature of this form of muscular dystrophy. To determine whether dystrophin gene defects might account for a significant number of patients with apparently isolated idiopathic DCM, we performed dystrophin gene analysis in 27 DCM patients, who were ascertained as part of a prospective study on idiopathic DCM. No dystrophin gene defects were found in our patients, whose average age was 50 years. These data suggest that dystrophin defects are not a common cause of idiopathic DCM in this age group in the absence of skeletal muscle cramps or weakness.

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