TY - JOUR
T1 - Dystrophin analysis in idiopathic dilated cardiomyopathy
AU - Michels, Virginia V.
AU - Pastores, Gregory M.
AU - Moll, Patricia P.
AU - Driscoll, David J.
AU - Miller, Fletcher A.
AU - Burnett, John C.
AU - Rodeheffer, Richard J.
AU - Tajik, Jamil A.
AU - Beggs, Alan H.
AU - Kunkel, Louis M.
AU - Thibodeau, Stephen N.
PY - 1993
Y1 - 1993
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=0027422079&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0027422079&partnerID=8YFLogxK
M3 - Article
C2 - 8301653
AN - SCOPUS:0027422079
SN - 0022-2593
VL - 30
SP - 955
EP - 957
JO - Journal of medical genetics
JF - Journal of medical genetics
IS - 11
ER -