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子どもにおける拡張性心筋症の発生率,原因,結果
Jeffrey A Towbin1, April M Lowe, Steven D Colan
1Texas Children's Hospital, Baylor College of Medicine, Houston, USA.
JAMA
|October 19, 2006
まとめ
拡張性心筋病 (DCM) は子供に影響し,発症率は年齢,性別,人種によって異なります. 結果は,診断時の原因,年齢,心不全の状態に依存し,多くの症例は原因が不明である.
科学分野:
- ペディアトリック・カルディオロジー
- 心血管伝染病学 心血管伝染病学
- 心臓病の遺伝学について
背景:
- 拡張性心筋症 (DCM) は,小児における心臓移植の主な原因であるが,その流行病学と臨床経過は依然として十分に理解されていない.
- 小児の集団におけるDCMの明確な理解を確立することは,患者のアウトカムを改善し,治療戦略を導くために不可欠です.
研究 の 目的:
- 子どもにおけるDCMに関連した発生率,病因,結果,およびリスク要因を包括的に記述する.
- 小児DCMの詳細な疫学プロフィールを提供する.
主な方法:
- 89の小児心臓センターからの前向きな,人口ベースのコホートを使用した縦断的な研究で,三等医療センターからの遡及的なコホートで補完されました.
- 北米で18歳未満でDCMと診断された1426人の子供を含む,疾患の二次原因を除く.
- 主要なDCMは,厳格なエコーカルディオグラフィックおよび/または病理学的基準を使用して確認されました.
主要な成果:
- 小児性DCMの全年発生率は10万人に0.57であったが,男の子,黒人,および1歳未満の乳児ではより高い.
- Idiopathic DCMは66%の症例を占め,心筋炎と神経筋疾患が最も一般的な既知の原因でした.
- 1年および5年後の死亡率または心臓移植率はそれぞれ31%と46%でした. 副作用の危険因子には,高齢化,閉塞性心不全,左心室機能の低下などがある.
結論:
- 小児のDCMは,結果が根本的な原因,患者の年齢,心不全の発症時の重症度によって著しく影響される異質な状態です.
- 発生率は人種,性別,年齢によって影響を受け,人口格差を強調しています.
- イディオパシー症例の割合が高いことは,標的型療法を開発するための基礎的なメカニズムに関するさらなる研究が必要であることを強調しています.
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