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Minerva Pediatrica 2005 October;57(5):275-80

language: English

Subclinical cardiological involvement in sicilian patients with pure congenital muscular dystrophy

Falsaperla R., Romeo G., Mattia C., Pavone P., Quattrocchi S.


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Aim. 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.
Methods. A total of 10 merosin-positive CMD patients and 100 healthy children were evaluated by echocardiography and Doppler examinations and the results were compared.
Results. 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.
Conclusion. In the 10 patients examined by echocardiography and Doppler, a heart involvement has been observed in 25% of cases.

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