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. 1993 Nov;30(11):955–957. doi: 10.1136/jmg.30.11.955

Dystrophin analysis in idiopathic dilated cardiomyopathy.

V V Michels 1, G M Pastores 1, P P Moll 1, D J Driscoll 1, F A Miller 1, J C Burnett 1, R J Rodeheffer 1, J A Tajik 1, A H Beggs 1, L M Kunkel 1, et al.
PMCID: PMC1016607  PMID: 8301653

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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Selected References

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