まとめ
胞性線維症の患者は,肝硬変や水性出血を含む肝疾患を頻繁に発症する. アスピリンは避けるべきであり,注射性硬化療法は,システィック線維症肝疾患の出血の管理に好ましいかもしれません.
科学分野:
- 小児胃腸内科 小児胃腸内科
- 肝臓病理学 肝臓病理学
- 肺内科 肺内科 肺内科
背景:
- 囊性線維症 (Cystic fibrosis,CF) は,多システム遺伝疾患である.
- 肝臓の関与は,CF患者の重要な合併症です.
- 肝臓の症状は,異常な肝機能検査から肝硬変まで様々です.
研究 の 目的:
- 小児性システィック線維症患者の肝臓の関与を将来的に評価するために.
- CFにおける肝疾患の発生率と進行を評価する.
- CFにおける水出血の最適な管理戦略を特定する.
主な方法:
- 74人のCF患者 (1~19歳) を対象に1~7年間にわたる見通し観察研究.
- 評価には,肝機能検査,肝硬変の画像検査,水性出血や水性の増加などの合併症のモニタリングが含まれていました.
- の出血に対する治療結果 (シャント手術 vs. 注射性硬化療法) を比較した.
主要な成果:
- 20人は肝臓に問題があり,18人は肝硬変 (4~13歳) を発症した.
- 6人の患者で出血が発生し,その50%はアスピリンの使用に関連しており,アスピリンの服用を避けるのが推奨されています.
- 注射式スクレロセラピーは,水出血制御のためのシャント手術と比較して,有効性とよりよい耐性を示しました.
結論:
- 肝硬変や水性出血を含む肝臓疾患は,性線維症の一般的な重篤な合併症です.
- アスピリンの摂取は,水出血を誘発する危険性があるため,CF患者では絶対に避けるべきです.
- 注射性硬化療法は,CFにおけるの出血の管理に有望でよく耐える代替手段であり,従来のシャント手術を上回る可能性がある.
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