Subclinical cardiological involvement in Sicilian patients with pure congenital muscular dystrophy

Congenital muscular dystrophies (CMD) are a heterogeneous group of diseases characterized by a marked weakness, generalized hypotonia, joint contractures and a muscle biopsy suggestive of dystrophic changes. The aim of this study has been to assess the prevalence of heart involvement in merosin-posi...

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Bibliographic Details
Published inMinerva pediatrica Vol. 57; no. 5; p. 275
Main Authors Falsaperla, R, Romeo, G, Mattia, C, Pavone, P, Quattrocchi, S
Format Journal Article
LanguageEnglish
Published Italy 01.10.2005
Subjects
Child
Child, Preschool
Data Interpretation, Statistical
Diastole
Echocardiography, Doppler
Female
Heart Diseases - diagnosis
Heart Diseases - epidemiology
Heart Diseases - etiology
Humans
Infant
Italy
Male
Muscular Dystrophies - complications
Muscular Dystrophies - congenital
Prevalence
Stroke Volume
Ultrasonography, Doppler, Color
Italy
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Summary:Congenital muscular dystrophies (CMD) are a heterogeneous group of diseases characterized by a marked weakness, generalized hypotonia, joint contractures and a muscle biopsy suggestive of dystrophic changes. The aim of this study has been to assess the prevalence of heart involvement in merosin-positive CMD patients examined by echocardiography and Doppler. A total of 10 merosin-positive CMD patients and 100 healthy children were evaluated by echocardiography and Doppler examinations and the results were compared. Three out of 10 patients showed a reduction of the ejection fraction and of the shortening fraction; in 1 case, a dilatation of left ventricular diastolic diameter was observed. In the 10 patients examined by echocardiography and Doppler, a heart involvement has been observed in 25% of cases.
ISSN:0026-4946