粘着性小腸閉塞による家族性地中海熱 (FMF) - 診断上の課題
Tamer Hassan Hassan1, Karim Sourial2
1Colorectal, Nottingham University Hospitals NHS Trust-City Campus, Nottingham, Nottinghamshire, UK surgentamer1980@outlook.sa.
BMJ case reports
|September 3, 2025
まとめ
家族性地中海熱 (FMF) は,盲腸炎や小腸閉塞 (SBO) のような外科的緊急事態を模倣することができます. FMFの早期診断は,不必要な手術や合併症を防ぐために重要です.
科学分野:
- 医学について
- 遺伝学
- 免疫学
背景:
- 家族性地中海熱 (Family Mediterranean Fever,FMF) はオートソーム性後退性自己炎症性疾患である.
- この病気は主に地中海,中東,南アジア系の人々に影響します.
- FMFは,手術状態を模倣した急性腹痛の再発エピソードで表れます.
研究 の 目的:
- 手術による腹部の緊急事態を模倣する症状を呈する FMF の症例を報告する.
- FMF発作と急性腹部の外科的原因を区別する診断上の課題を強調する.
- リスク集団における再発性腹痛の微分診断において,FMFを考慮する重要性を強調する.
主な方法:
- 17歳の時にFMFと診断された男性患者の症例報告
- 患者の再発性腹痛と複数の手術歴のレビュー
- ラパロトミーと小腸切除を含む診断手順と結果の分析
主要な成果:
- 患者は FMFの診断を受ける前に2つの不必要な手術 (尾切除と探索性腹腔切除) を受けました.
- CTで確認された粘着性小腸阻害 (SBO) の第三の腹腔切除は小腸切除につながった.
- 術後の合併症には,腹痛の悪化と長時間の内症候群が含まれていた.
結論:
- このケースは FMFに関連した腹部発作と手術による腹部発作を区別する診断の困難さを強調しています.
- 誤った診断は不必要な手術と 関連疾患につながる可能性があります
- FMFの早期認識と診断は,適切な管理を導き,外科的合併症を回避するために不可欠です.
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