長期QT症候群のプロバンドの影響を受けた両親や兄弟姉妹に対する臨床的影響
J Kimbrough1, A J Moss, W Zareba
1Department of Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
Circulation
|August 2, 2001
まとめ
Proband LQTSの重症度は,親戚の心臓発作を予測するものではありません. 1度目の親戚の臨床評価は,長QT症候群のリスクを評価し,管理を導くために重要である.
科学分野:
- 心臓病学 心臓病学
- 遺伝学 遺伝学とは
- クリニカル・メディシン 臨床医学
背景:
- ロングQT症候群 (LQTS) のような遺伝性心疾患は,家族のスクリーニングを必要とします.
- LQTS検査者の一級親族は,しばしばこの疾患を患っている.
- 家族を評価することは,LQTSの総合的な管理の鍵です.
研究 の 目的:
- LQTSプロバンドの重度が,一級親族における心臓発作リスクを予測するかどうかを評価する.
- LQTS被験者の影響を受けた両親や兄弟姉妹の臨床経過を分析する.
主な方法:
- 211人のLQTS受験者および791人の一級親族の臨床およびEKGデータを分析した.
- 罹患した親戚の心臓発作のリスク要因が特定されました.
- 臨床的重度の比較は,試験受験者とその影響を受けた親族の間で行われました.
主要な成果:
- Proband LQTSの重症度は,親や兄弟姉妹の心臓発作リスクと有意に相関していません.
- 女性親は,男性親よりも最初の心臓発作の累積リスクが高かった.
- QTcの持続時間と女性の性別は,親の心臓発作の危険因子であり,兄弟姉妹のQTcは危険因子であった.
結論:
- プロバンドのLQTSの臨床的重症度は,罹患した一級親族の重症度を予測するための信頼できる指標ではありません.
- 親族に対する個々のリスク評価は,効果的なLQTSケアにとって不可欠です.
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