Cardiac involvement in Fukuyama muscular dystrophy is less severe than in Duchenne muscular dystrophy

Tetsushi Yamamoto, Mariko Taniguchi-Ikeda, Hiroyuki Awano, Masaaki Matsumoto, Tomoko Lee, Risa Harada, Takamitsu Imanishi, Nobuhide Hayashi, Yoshitada Sakai, Ichiro Morioka, Yasuhiro Takeshima, Kazumoto Iijima, Jun Saegusa, Tatsushi Toda

研究成果: Article査読

3 被引用数 (Scopus)

抄録

Background One of the main complications in patients with muscular dystrophies is cardiac dysfunction. The literature on cardiac involvement in patients with Fukuyama congenital muscular dystrophy (FCMD) is limited. Aim To compare cardiac involvement between patients with FCMD and Duchenne muscular dystrophy (DMD). Methods We compared cardiac involvement between 30 patients with FCMD and 181 patients with DMD using echocardiography and serum biomarkers. All patients were receiving regular checkups at Kobe University Hospital. We used single regression analysis to compare echocardiographic parameters, age, and serum biomarkers. Results Almost all clinical and echocardiographic parameters were lower in patients with FCMD than DMD. The brain natriuretic peptide concentration in patients with FCMD showed no correlation with age or left ventricular ejection fraction (r = 0.231, p = 0.22 and r = 0.058, p = 0.76, respectively). A log-rank test revealed that the risk of left ventricular systolic dysfunction was lower in patients with FCMD than DMD (p = 0.046, hazard ratio = 0.348). Conclusion The clinical progression of cardiac dysfunction is significantly milder in patients with FCMD than DMD, while skeletal muscle involvement is significantly worse in patients with FCMD. These data suggest that the pathophysiological findings of FCMD can be explained by less severe cardiac dysfunction in FCMD than DMD.

本文言語English
ページ(範囲)861-868
ページ数8
ジャーナルBrain and Development
39
10
DOI
出版ステータスPublished - 11-2017
外部発表はい

All Science Journal Classification (ASJC) codes

  • 小児科学、周産期医学および子どもの健康
  • 発達神経科学
  • 臨床神経学

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