フォンタン に 関する 肝臓 疾患: 検診,治療,移植 の 検討
Juliet Emamaullee1,2, Ali N Zaidi3,4, Thomas Schiano5
1Liver Transplant Center, Children's Hospital-Los Angeles, CA (J.E.).
Circulation
|August 11, 2020
まとめ
フォントンの手術で生き残った成人は 肝硬変や肝硬変を含む肝臓の合併症に直面します このレビューは,これらの患者におけるフォンタン関連肝疾患と移植の検討に焦点を当てています.
科学分野:
- 心臓病科
- ヘパトロジー
- 移植医学
背景:
- 心臓の先天性病を患っている子供たちは 手術の進歩のおかげで 大人になるまで生き延びています
- およそ7万人を対象に実施されたフォントン手術は,ほとんどの患者にフォントン関連肝疾患 (FALD) を引き起こす.
- FALDは進行し,心臓と肝臓の移植が必要になります.
研究 の 目的:
- フォントン関連肝疾患に関する文献をレビューする.
- FALDの診断とモニタリング戦略に重点を置く
- フォントン患者の心臓移植と肝臓移植の組み合わせについて検討する.
主な方法:
- フォントンの循環と肝疾患に関する文献レビュー
- FALDの進行を示す監視バイオプシーのデータ分析.
- 移植を受ける患者のアウトカムの検討
主要な成果:
- ほぼ全てのフォントンの生存者は 青春期までに臨床的に無音な肝繊維症を発症する.
- 肝硬変と肝細胞癌を含む進行したFALDは,ますます報告されています.
- 末期FALDでは心臓と肝臓を併用した移植が 広く行われています
結論:
- FALDの管理と移植の決定を導くためのコンセンサスガイドラインが不足しています.
- FALDの早期発見と管理は長期的な成果の改善に不可欠です.
- 肝臓疾患のフォントンの生存者のケアを最適化するには 多分野間の協力が不可欠です
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