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ID 48814
タイトルヨミ
シンキンショウ ノ ビョウリ : Duchenneガタ キンジストロフィー ブケンレイ ニオケル シンゾウ ビョウヘン ノ ケントウ
タイトル別表記
Pathology of the dilated cardiomyopathy : autopsy study of the cardiac involvement in Duchenne muscular dystrophy
著者
香川, 典子 徳島大学医学部保健学科検査技術科学専攻形態系検査学講座 徳島大学 教育研究者総覧 KAKEN研究者をさがす
キーワード
DMD
cardiac involvement
pathology
cell adhesion molecules
資料タイプ
学術雑誌論文
抄録
Dystrophinopaties are due to mutations in the dystrophin gene on chromosome Xp21.1 and comprise the allelic entities Duchenne muscular dystrophy (DMD), Becker muscular dystrophy and X-linked dilated cardiomyopathy. In all three entities, the heart is affected to various degrees. The cardiac involvement in autopsy cases with DMD is described.
The cardiac weight of DMD varied widely. Of 285 hearts, 109 were atrophic, 107 were within normal range, and 69 were hypertrophic. The incidence of dilated cardiomyopathy, was highest in hypertrophic group. The posterior and lateral left ventricular wall were most extensively replaced by scar tissue, especially, the outer wall was more affected.
The microscopic characteristics of cardiac involvement in DMD was the replacement of myocardium by connective tissue. The degenerative changes of myocardial fiber and fatty infiltration were also noticed, but no regenerative myocardial fiber was observed.
Expression of NCAM, N-cadherin, β-catenin and connexin 43 were investigated immunohisohchemically. The immunoreactivity of these cell adhesion molecules was recognized at the intercalated disc of myocardial cell, although, in DMD the reactivity was weaker than in control cases. In DMD, reduced expression of these cell adhesion molecules can result in slowed ventricular conduction, which may contribute to the development of arrhythmia and heart failure.
掲載誌名
四国医学雑誌
ISSN
00373699
cat書誌ID
AN00102041
出版者
徳島医学会
61
5-6
開始ページ
121
終了ページ
125
並び順
121
発行日
2005-12-20
備考
EDB ID
フルテキストファイル
言語
jpn
部局
医学系