妊娠および胎児の長鎖3-ヒドロキシアシル共酵素A脱水素酵素欠乏症
B Wilcken1, K C Leung, J Hammond
1Oliver Latham Laboratory, New South Wales Health Department, Sydney, Australia.
Lancet (London, England)
|February 13, 1993
まとめ
胎児のロングチェーン3ヒドロキシアシル共酵素A脱水素酶 (LCHAD) 欠乏によって合併された妊娠は,脂肪肝やHELLP症候群などの母親の肝臓問題を引き起こす可能性があります. 影響を受けていない赤ちゃんは,これらの合併症のない母親から生まれ,胎児のLCHAD状態が母親の健康に影響することを示唆しています.
科学分野:
- バイオケミストリー バイオケミストリー
- 遺伝学 遺伝学とは
- 妊産婦・胎児医学について
背景:
- 長鎖3ヒドロキシアシル共酵素A脱水素酵素 (LCHAD) 欠乏症は遺伝性代謝障害である.
- 脂肪肝やHELLP症候群などの妊娠中の母親の合併症は,重大なリスクをもたらす可能性があります.
研究 の 目的:
- 胎児のLCHAD欠乏症と妊娠中の母親の肝臓合併症の関連性を調査する.
- 胎児のLCHAD状態が母親の不良結果の発生に影響するかどうかを判断する.
主な方法:
- 5人の母親の11人の妊娠を報告したケースシリーズ.
- 肝機能と特定の症候群の発達を含む,母と胎児の健康状態の臨床観察.
主要な成果:
- LCHAD欠乏症の胎児を含む6つの妊娠は,母親の脂肪肝とHELLP症候群によって複雑になりました.
- 3つの妊娠は,影響を受けていない赤ちゃんをもたらし,これらの妊娠はほとんど合併症がない.
- 胎児のLCHAD欠乏状態と分離された母親への有害な影響,母親のヘテロジゴシティのみではありません.
結論:
- 胎児LCHAD欠乏症は,妊娠中に母親の肝機能に悪影響を及ぼす可能性があります.
- 母親の異卵性性だけでは,観察された有害効果を説明することはできません;胎児の状態は重要です.
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