Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy
Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
Standard
Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy. / Sveen, Marie-Louise; Thune, Jens Jakob; Køber, Lars; Vissing, John.
I: Archives of Neurology, Bind 65, Nr. 9, 2008, s. 1196-201.Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › fagfællebedømt
Harvard
APA
Vancouver
Author
Bibtex
}
RIS
TY - JOUR
T1 - Cardiac involvement in patients with limb-girdle muscular dystrophy type 2 and Becker muscular dystrophy
AU - Sveen, Marie-Louise
AU - Thune, Jens Jakob
AU - Køber, Lars
AU - Vissing, John
N1 - Keywords: Adolescent; Adult; Aged; Cardiovascular Diseases; Female; Humans; Male; Middle Aged; Muscular Dystrophies, Limb-Girdle; Muscular Dystrophy, Duchenne; Prospective Studies
PY - 2008
Y1 - 2008
N2 - OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology at Rigshospitalet. Patients One hundred one patients with LGMD2A-I and BMD and 29 patients with LGMD2 and no molecular diagnosis. MAIN OUTCOME MEASURES: Clinical investigation, echocardiography, and electrocardiographic findings. RESULTS: Cardiac involvement was present in 24 of 100 patients (24%) with LGMD2A-I and in 14 of 30 patients (47%) with BMD. Only a few patients with LGMD2A and unclassified LGMD2 had mild cardiac involvement, whereas 29% and 67% of patients with LGMD2I and LGMD2E, respectively, had cardiac involvement. Cardiac involvement was not correlated with age, muscle strength, or the level of dystrophic changes on muscle biopsy. CONCLUSIONS: This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.
AB - OBJECTIVE: To investigate the extent of cardiac involvement in patients with 1 of the 12 groups of recessively inherited limb-girdle muscular dystrophy type 2 (LGMD2A-L) and Becker muscular dystrophy (BMD). DESIGN: Prospective screening. SETTING: Neuromuscular Clinic and Department of Cardiology at Rigshospitalet. Patients One hundred one patients with LGMD2A-I and BMD and 29 patients with LGMD2 and no molecular diagnosis. MAIN OUTCOME MEASURES: Clinical investigation, echocardiography, and electrocardiographic findings. RESULTS: Cardiac involvement was present in 24 of 100 patients (24%) with LGMD2A-I and in 14 of 30 patients (47%) with BMD. Only a few patients with LGMD2A and unclassified LGMD2 had mild cardiac involvement, whereas 29% and 67% of patients with LGMD2I and LGMD2E, respectively, had cardiac involvement. Cardiac involvement was not correlated with age, muscle strength, or the level of dystrophic changes on muscle biopsy. CONCLUSIONS: This study demonstrates a high prevalence of cardiac involvement in patients with LGMD2I, LGMD2E, and BMD. Patients with LGMD2A, LGMD2D, and unclassified LGMD2 have a much lower and milder prevalence of cardiac involvement.
U2 - 10.1001/archneur.65.9.1196
DO - 10.1001/archneur.65.9.1196
M3 - Journal article
C2 - 18779423
VL - 65
SP - 1196
EP - 1201
JO - JAMA Neurology
JF - JAMA Neurology
SN - 2168-6149
IS - 9
ER -
ID: 17395164