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母親の抗ロ抗体と抗ラ抗体の関連性のある内臓筋縮症
Lynne E Nield1, Earl D Silverman, Glenn P Taylor
1Department of Paediatrics, Division of Cardiology, The Hospital for Sick Children, University of Toronto Faculty of Medicine, Toronto, Ontario, Canada.
Circulation
|February 21, 2002
まとめ
生まれながらの心臓閉塞に関連した母親の抗体は,乳児の内臓線維弾性症 (EFE) を引き起こす可能性があります. この状態は,治療を受けても高い死亡率を持っています.
科学分野:
- 心臓病学 心臓病学
- 免疫学 免疫学とは
- ネオナトロジーの新生児科
背景:
- 母親の抗Roおよび抗La抗体は,先天性心臓閉塞 (CHB) と関連しています.
- 内心線維弾性症 (EFE) は,自己抗体媒介性CHBではめったに記述されず,自然史と病原性は不明です.
研究 の 目的:
- 抗Roまたは抗La抗体を持つ母親から生まれたCHBの胎児および小児におけるEFEの自然史および病原性を調査する.
主な方法:
- 5つのセンターの臨床歴,心声検査,病理学の遡及的レビュー.
- 13人の胎児とEFEとCHBを患った母親から生まれた13人の胎児と子供の分析.アンチ-ロまたはアンチ-ラ抗体の陽性.
- 免疫ヒストロケミストは,心臓に感染した胎児の免疫反応を評価する.
主要な成果:
- 重度の心室機能不全は,すべての症例で認められた.
- EFEは,心室ペースにもかかわらず,いくつかのケースで,ポスト-CHB診断を開発しました.
- EFEによる高死亡率 (85%),死亡者9人,心臓移植2人.
- 病理学では,中等から重度のEFEが示され,主に左心室で示された.
- 免疫ヒストケミストリーは,IgG/IgMの蓄積とT細胞の浸透を示し,胎児の免疫反応を示した.
結論:
- 内心筋線維弾性症 (EFE) は,自己抗体媒介の先天性心臓阻害 (CHB) で,ペースをつけても発生します.
- オートアンチボディに関連したEFEは,胎児および産後の人生の両方で非常に高い死亡率を伴う.
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