Cardiac abnormalities in a follow-up study on carriers of Duchenne and Becker muscular dystrophy.

Autor:	Schade van Westrum SM; Department of Neurology, Academic Medical Centre, University of Amsterdam, Amsterdam, the Netherlands., Hoogerwaard EM, Dekker L, Standaar TS, Bakker E, Ippel PF, Oosterwijk JC, Majoor-Krakauer DF, van Essen AJ, Leschot NJ, Wilde AA, de Haan RJ, de Visser M, van der Kooi AJ
Jazyk:	angličtina
Zdroj:	Neurology [Neurology] 2011 Jul 05; Vol. 77 (1), pp. 62-6. Date of Electronic Publication: 2011 Jun 22.
DOI:	10.1212/WNL.0b013e318221ad14
Abstrakt:	Objectives: Cardiac involvement has been reported in carriers of dystrophin mutations giving rise to Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD). The progress of these abnormalities during long-term follow-up is unknown. We describe the long-term follow-up of dilated cardiomyopathy (DCM) in DMD/BMD carriers. Methods: A long-term follow-up study was performed among Dutch DMD/BMD carriers first analyzed in 1995. A cardiac history was taken, and all carriers were assigned a functional score to assess skeletal muscle involvement. Electrocardiography and M-mode and 2-D echocardiography were performed. DCM was defined as an enlarged left ventricle with a global left ventricle dysfunction or fractional shortening less than 28%. Slow vital capacity of the lung was measured by a hand-held spirometer. Results: Ninety-nine carriers were monitored with a median follow-up of 9 years (range 7.0-10.6 years). Eleven carriers with DCM (10 DMD, 1 BMD) were identified. Nine of them developed DCM in the follow-up period. One of the patients with DCM reported in the 1995 study died of cardiac failure at age 57 years. DCM was more frequently found in carriers who were functionally symptomatic. Conclusion: Cardiac abnormalities in DMD/BMD carriers are progressive, as in patients with DMD/BMD.
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu