新しい肝臓鉄過負荷症候群で,正常なトランスファーリン飽和度がある
R Moirand1, A M Mortaji, O Loréal
1Clinique des Maladies du Fole, Hôpital Póntchalliou, Rennes, France.
Lancet (London, England)
|January 11, 1997
まとめ
遺伝学とは無関係な新しい鉄過負荷症候群が,原因不明の肝臓鉄過負荷を有する患者で特定されました. これは,過剰な鉄と代謝障害との関連を示唆し,遺伝的血色腫症の現在の診断基準の見直しを促した.
科学分野:
- 肝臓病理学 肝臓病理学
- 遺伝学 遺伝学とは
- メタボリック障害 メタボリック障害
背景:
- 原因不明の肝臓の鉄過負荷と正常なトランスファーリン飽和度の患者が調査されました.
- 肝臓の鉄濃度が上昇したが,正常なトランスファーリン飽和度を持つ被験者.
研究 の 目的:
- 遺伝的ヘモクロマトーシスと異なる新しい鉄過負荷症候群を特定する.
- 鉄分過剰と代謝異常との関係を調査する.
主な方法:
- 原因不明の鉄過負荷を有する65人の患者を,遺伝性血色変色症 (GH) の対照群と比較した.
- 肝臓の鉄濃度,血清フェリチン,トランスフリン飽和度,HLA抗原周波数などを分析した.
- 肝臓の鉄濃度が上昇した患者の研究された親族の年齢比.
主要な成果:
- 患者は高齢で,GH患者より重度の鉄過負荷が軽かった.
- HLA-A3抗原の頻度は,GHと比較して,患者において有意に低かった.
- 患者の95%が肥満,高脂血症,異常なグルコース代謝,または高血圧を示した.
結論:
- HLAに関連していない新しい鉄過負荷症候群が特定されました.
- 過剰な鉄分と代謝障害との関連を示唆している.
- 遺伝的血色腫症の現在の診断基準の見直しを推奨する.
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