過剰な亜鉛は,クリ族とオジボワ・クリ族の子どもにおける重度の進行性コレスタシスと関連している
M J Phillips1, C A Ackerley, R A Superina
1Department of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
Lancet (London, England)
|March 30, 1996
まとめ
慢性コレステティック性肝疾患のカナダ先住民の6人の子どもには,肝臓の銅と亜鉛が過剰に含まれていた. これは,金属代謝の潜在的な遺伝的障害や,肝臓の深刻な状態に寄与する環境要因を示唆している.
科学分野:
- 小児肝臓病理学 小児肝臓病理学
- 医学遺伝学 医学遺伝学
- トレースエレメントの代謝
背景:
- 高濃度の肝臓銅は,様々な肝臓疾患と関連しています.
- カナダ先住民の6人の子供たちが,重度の慢性コレステア性肝疾患を呈した.
- これらの子供たちは,肝臓の銅と亜鉛の濃度が過剰であった.
研究 の 目的:
- 重度の慢性コレステティック性肝疾患の小児における肝組織の元素組成を調べる.
- 肝臓の銅と亜鉛の濃度を,患児と対照群の患児で比較する.
主な方法:
- 6人の子供 (22ヶ月から8歳までの年齢) の肝臓検体または生検材料の分析.
- スキャニング・トランスミッション・電子顕微鏡 (STEM) のX線基本微分析を活用した.
- 元素の定量化のために原子吸収スペクトロフォトメトリーを使用した.
- 結果を4つの対照サンプルと比較した.
主要な成果:
- 肝臓のエクスプラントは,リゾソームとサイトプラズマに濃厚な堆積物と,進んだ胆動脈硬化症を示した.
- 肝臓の銅濃度は,対照群 (2.3-2.9μg/g) と比較して,患者 (47.6-56.9μg/g乾燥量) で有意に上昇した.
- 肝臓における亜鉛濃度は,患者 (104〜128μg/g乾燥重量) と対照群 (1.9〜3.2μg/g) よりも著しく高かった.
結論:
- 肝臓の余分な銅は,慢性コレスタシスに起因する可能性があります.
- 肝臓における過剰な亜鉛の原因は未だに解明されていない.
- 金属代謝の遺伝的障害は,家族関係によるものと考えられていますが,環境要因は除外されていません.
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