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書籍詳細

未契約
書籍名 新女性医学大系 17 性の分化とその異常
出版社 中山書店
発行日 2002-02-28
著者
  • 武谷雄二(総編集)
  • 青野敏博(編集)
  • 麻生武志(編集)
  • 中野仁雄(編集)
  • 野澤志朗(編集)
  • 藤本征一郎(担当編集)
ISBN 4521544312
ページ数 397
版刷巻号 第1版第1刷
分野
シリーズ 新女性医学大系
閲覧制限 未契約

性分化異常の基礎から臨床までを、1冊で記述した日本初の成書である。本書を通じて、性分化異常の診断から外科的・内科的治療、治療後の身体的・精神的ケアのすべてを修得できる。巻頭では性分化の最新の基礎知識を詳説し、啓蒙書の役割も担っている。

目次

  • 表紙
  • 執筆者一覧
  • 目次
  • 生殖器系の発生・解剖・機能
    • I 生殖器系の発生と解剖
      • 1. 三胚葉の発生
      • 2. 性腺の発生
      • 3. 精巣の発生
      • 4. 卵巣の発生
      • 5. 生殖管の発生
      • 6. 生殖管系の分化
      • 7. 腟の発生
      • 8. 外性器の分化
      • 9. 男性外性器の発生
      • 10. 女性外性器の発生
      • 11. 付属性腺の分化
      • 12. 鼠径管の発生
      • 13. 精巣下降
      • 14. 卵巣下降
    • II 性決定・分化と分子機構
      • A. 正常性分化のプロセス
        • 1. 性腺形成
          • a. ステップ1 : 接合体から未分化性腺が形成される過程
          • b. ステップ2 : 未分化性腺の性特異的分化が選択される過程
          • c. ステップ3 : 未分化性腺から胎児精巣が形成される過程
          • d. ステップ4 : 未分化性腺から胎児卵巣が形成される過程
          • e. ステップ5 : 胎児精巣から成人精巣が形成される過程
          • f. ステップ6 : 胎児卵巣から成人卵巣が形成される過程
        • 2. 性管および外性器分化
      • B. 生殖細胞の発生を司る遺伝子機構
        • 1. 生殖細胞の発生・分化機構
          • a. マウスPGCの起源と形成機構
          • b. PGCの移動
          • c. PGCの増殖, 分化, 細胞死
        • 2. 性腺の形成機構
          • a. SRY
          • b. WT1
          • c. SOX-9
          • d. DAX-1
          • e. Ad4BP/SF-1
          • f. Wnt-4
          • g. MIS
      • C. X・Y染色体の進化
        • 1. X染色体とY染色体
        • 2. 哺乳類のX染色体 ― 大野の法則
        • 3. 単孔類, 有袋類のX染色体
        • 4. ヒト性染色体の進化
        • 5. X染色体の不活性化
      • D. ゲノムインプリンティング
        • 1. 卵ゲノムと精子ゲノムの違い
        • 2. インプリンティングを受ける遺伝子
        • 3. DNAメチル化の役割
        • 4. ゲノムインプリンティングの起源
      • E. 性決定・分化に関与する遺伝子とその異常
        • i. 性の分化と染色体・遺伝子
          • 1. 性の決定・分化機構の探索の歴史的背景
            • a. 性の決定と分化の仕組み
            • b. Y染色体と精巣決定因子
            • c. Y染色体以外の精巣決定因子
          • 2. 性腺の性決定にかかわるSRYの上位遺伝子
          • 3. 性腺の性決定にかかわる候補遺伝子
        • ii. 中枢の性分化 ― 女の脳・男の脳
          • 1. 脳の性分化とアンドロゲンの芳香化 ― ラットなどの実験動物の場合
          • 2. ヒトの脳の性分化とアンドロゲン
          • 3. 脳内におけるアンドロゲン受容体の分布
          • 4. 発生過程における性ホルモンのニューロンへの作用 ― 神経回路形成の調節
          • 5. 性ホルモンによるニューロン数の調節
          • 6. ヒトの脳における性的二型神経細胞グループ
          • 7. 性的指向とジェンダーアイデンティティとアンドロゲン
          • 8. 脳の認知機能の性差
          • 9. 子供の遊びのパターンや自由画の性差
          • 10. 脳重や脳の部位的な男女差
          • おわりに ― 脳の性分化と遺伝子のかかわり
        • iii. 内性器の分化
          • 1. 性腺の分化・発達
          • 2. 内性器の分化
          • 3. 内性器の分化・発達誘導因子
            • a. アンドロゲン
            • b. アンドロゲン受容体 (AR)
            • c. Muller管 (傍中腎管) 抑制物質 (因子)
        • iv. 外性器の分化
          • 1. 外性器の分化における内分泌学的影響
          • 2. 外性器の分化に関与する遺伝子
            • a. 5α-還元酵素に関与する遺伝子
            • b. ARに関与する遺伝子
            • c. その他の外性器の発生に関与する遺伝子
  • 性分化異常の臨床
    P.119閲覧
    • I 性分化異常総論
      • A. 早期診断と取り扱い
        • 1. 性の分化
        • 2. 性分化異常症
          • a. XX男性
          • b. XY女性
          • c. 真性半陰陽
          • d. 副腎性器症候群
          • e. 精巣性女性化症候群
          • f. Turner症候群
        • 3. 性成熟の異常
          • a. 思春期早発症
          • b. 思春期遅発症
      • B. 性染色体の構造異常と臨床
        • 1. 性染色体と遺伝子
        • 2. Y染色体構造異常と臨床
        • 3. X染色体構造異常と臨床
        • 4. 性染色体の構造異常の種類と対応
          • a. 染色体構造異常
          • b. 過剰マーカー染色体
          • c. 遺伝相談と実際
          • d. 染色体異常の告知
    • II 性分化異常各論
      • A. 性腺分化異常
        • i. Turner症候群
          • 1. 病因
            • a. 45, Xの核型
            • b. モザイク
            • c. 構造異常
          • 2. 頻度
          • 3. 症状
            • a. 低身長
            • b. 性腺機能不全
            • c. 外表奇形
            • d. 心奇形, 腎奇形
            • e. 耐糖能異常, 糖尿病
          • 4. 診断
          • 5. 治療
            • a. 成長ホルモン治療
            • b. エストロゲン治療
            • c. 成人Turner症候群女性のケア
        • ii. Noonan症候群
          • 1. 定義, 概念
          • 2. 病因
          • 3. 頻度
          • 4. 症状
          • 5. 診断, 鑑別診断
          • 6. 治療, 予後
        • iii. XY型性腺形成異常症
          • 1. 定義, 概念
          • 2. 病因
          • 3. 症状, 臨床像
          • 4. 診断, 鑑別診断
            • a. 内分泌検査成績
            • b. 染色体分析
            • c. PCR-SSCP法による解析
            • d. 直接塩基配列決定法による解析
            • e. XY型性腺形成異常症における遺伝子異常
          • 5. 治療
        • iv. XX型性腺形成異常症
          • 1. XX型性腺形成異常症
          • 2. XX男性
          • 3. 真性半陰陽
          • 4. 性腺形成への考察
        • v. 混合型性腺形成異常症
          • 1. 定義
          • 2. 染色体構成
          • 3. 臨床症状および身体所見
          • 4. 診断
          • 5. 治療
        • vi. ポリソミーX女性 (superfemale)
          • 1. 定義
          • 2. 病因
          • 3. 頻度
          • 4. 症状
            • a. トリプルX
            • b. テトラソミーX
            • c. ペンタソミーX
          • 5. 診断
          • 6. 治療
        • vii. Klinefelter症候群
          • 1. 歴史, 定義
          • 2. 病因
          • 3. 頻度
          • 4. 臨床所見
            • a. 臨床症状
            • b. 性腺機能
            • c. 知能, 性格
            • d. 合併症
            • e. Klinefelter症候群の亜型
          • 5. 診断
          • 6. 治療
        • viii. XYY個体
          • 1. 概念, 病因
          • 2. 頻度
          • 3. 臨床像
          • 4. 診断
          • 5. 今後の課題
        • ix. 精子形成不全とAZF
          • 1. 精子形成不全と遺伝子異常
          • 2. 精子形成関連遺伝子AZFとは
          • 3. 男性不妊症患者におけるAZF遺伝子異常の頻度
          • 4. AZF遺伝子の子孫への伝播
          • 5. AZF遺伝子異常検索のためのDNA検査の実際
          • 6. AZF遺伝子検索とインフォームドコンセント
          • 7. AZF遺伝子解明と男性不妊症治療
      • B. 生殖管・外性器分化異常
        • i. 真性半陰陽
          • 1. 概念
          • 2. 頻度・染色体構成からみた病因
          • 3. 臨床所見, 臨床検査所見
          • 4. 診断, 鑑別診断
            • a. 診断, 病理組織学的検査
            • b. 性腺の存在部位と性腺の存在様式
          • 5. 治療の概略
        • ii. 副腎性器症候群
          • 1. 臨床像
            • a. 21-水酸化酵素 (21-OH) 欠損症
            • b. 11β-水酸化酵素欠損症
          • 2. 治療
            • a. グルココルチコイド療法
            • b. 外科的治療法
          • 3. 症例
        • iii. 非副腎性女性半陰陽
          • 1. アロマターゼ (エストロゲン合成酵素) 欠損症
          • 2. アンドロゲン作用のある薬剤による女性仮性半陰陽
          • 3. 母体のアンドロゲン産生腫瘍による女性仮性半陰陽
          • 4. 尿生殖器系の形成異常による女性仮性半陰陽
        • iv. アンドロゲン合成障害
          • 1. アンドロゲン合成経路の概略
          • 2. 性分化過程におけるアンドロゲンの生理作用
          • 3. アンドロゲン合成障害の病因と症状
            • a. 主に未分化性腺期に関連するアンドロゲン合成障害
            • b. 主に性決定期に関連すると考えられるアンドロゲン合成障害
            • c. 主に内・外性器形成期あるいはそれ以降に関連するアンドロゲン合成障害
          • 4. アンドロゲン合成障害治療原則
        • v. アンドロゲン不応症
          • 1. 疾患の概要
          • 2. アンドロゲンの作用機序
          • 3. アンドロゲン不応症候群
          • 4. 5α-還元酵素欠損症
        • vi. 先天性副腎リポイド過形成
          • 1. ステロイドホルモンとStARタンパク質
          • 2. 先天性副腎リポイド過形成
            • a. 先天性副腎リポイド過形成の臨床
            • b. 遺伝子変異
            • c. 病因・病態生理
            • d. 実験動物モデル
          • 3. StARタンパク質
            • a. 細胞内のコレステロール輸送機序
            • b. 下垂体ホルモンのステロイドホルモン産生細胞への作用
            • c. StARタンパク質クローニングの経緯
            • d. StARタンパク質の機能および発現
            • e. StARタンパク質のホモログ
            • f. StARタンパク質の構造と機能
            • g. StAR遺伝子の発現
        • vii. 内性器の奇形・位置異常
          • 1. 内性器の奇形
          • 2. 内性器の位置異常
        • viii. 外因性ホルモンによる異常
          • 1. 薬剤の胎児への影響
          • 2. 性腺, 内・外性器の分化
          • 3. 外因性ホルモンによる性分化異常および外性器異常
            • a. 外因性プロゲスターゲン, アンドロゲンによる女性仮性半陰陽
            • b. 外因性プロゲスターゲンによる男性仮性半陰陽
            • c. 外因性エストロゲンによる外性器異常
            • d. その他
          • 4. 内分泌攪乱 (化学) 物質
            • a. 作用機序
            • b. 内分泌攪乱物質の精子数への影響
            • c. 内分泌攪乱物質の外性器への影響
      • C. 性同一性障害
        • i. 病態と診断
          • 1. 今, なぜ性同一性障害なのか
          • 2. 性同一性障害とは
          • 3. 性同一性障害でみられる症状
            • a. 反対の性別に対して強く惹かれる
            • b. 自分の身体の性を強く嫌う
            • c. 日常生活で反対の性別役割をとろうとする
          • 4. 性同一性障害の診断
            • a. 生物学的性別の決定
            • b. 性の自己意識 (ジェンダー) の決定
            • c. 除外診断
            • d. 診断の手続き
            • e. 診断に際して配慮すべきこと
          • 5. 性同一性障害の症状と他症候との違い
            • a. 性嗜好障害との違い
            • b. 服装倒錯症との違い
            • c. 性同一性障害と性別違和症候群, 性転換症
            • d. 性同一性障害と性対象
            • e. 性同一性障害の症状にみられる性差
          • 6. 治療
            • a. 第1段階 (精神療法)
            • b. 第2段階 (ホルモン療法)
            • c. 第3段階 (手術療法)
          • 7. 性同一性障害の成因
            • a. 性の分化とジェンダーの確立
            • b. 脳の構造の性差と性同一性障害
        • ii. 治療
          • 1. 治療の原則
          • 2. 精神療法
          • 3. ホルモン療法
            • a. ホルモン療法の開始条件
            • b. 男性から女性へ
            • c. 女性から男性へ
          • 4. 手術療法
            • a. 性別適合手術の定義
            • b. 女性から男性への性別適合手術
            • c. 男性から女性への性別適合手術
    • III 性分化異常症の治療
      • A. 薬物療法
        • i. 小児
          • 1. 性決定の基本法則
          • 2. 薬物療法
            • a. 先天性副腎皮質過形成の治療
            • b. 矮小陰茎に対する治療
            • c. 男性ホルモン補充療法
            • d. 女性ホルモン補充療法
        • ii. 成人
          • 1. 成人における先天性副腎皮質過形成の概略
            • a. リポイド副腎過形成 (Prader症候群)
            • b. 21-ヒドロキシラーゼ欠損症
            • c. 11β-ヒドロキシラーゼ欠損症
            • d. 3β-ヒドロキシステロイドデヒドロゲナーゼ (3β-HSD) 欠損症
            • e. 17α-ヒドロキシラーゼ / 17,20-リアーゼ欠損症
            • f. 成人CAHの治療
          • 2. 男性化症候群
            • a. 男性化の症候
            • b. 成熟女性におけるアンドロゲンの産生源
            • c. 病因
            • d. 診断
          • 3. 治療
      • B. 外科的治療
        • i. 婦人科領域
          • 1. 腟欠損症
            • a. 腟形成術施行の時期
            • b. 造腟法の種類
            • c. 腹腔鏡併用低侵襲性造腟術
            • d. Davydov法
          • 2. 腟横中隔
          • 3. 腟縦中隔
          • 4. 双角子宮, 中隔子宮
          • 5. 性腺形成異常症における性腺摘除術
        • ii. 泌尿器科・外科領域
          • 1. 外科的治療の対象疾患
          • 2. 性腺・付属器の生検・切除
          • 3. 男性化外陰形成術
          • 4. 女性化外陰形成術
            • a. 陰核形成術
            • b. 陰唇形成術
            • c. 腟形成術
      • C. 心理的ケア
        • i. 小児
          • 1. Turner症候群
            • a. 診断時のケア
            • b. 本人への説明
            • c. 本人, および家族の会
          • 2. 21-水酸化酵素欠損症
            • a. 小児期の心理的ケア
            • b. 思春期の心理的ケア
            • c. 本人, および家族の会
          • 3. その他の性分化異常症
            • a. 診断時の両親への説明
            • b. 本人への説明
            • c. 本人, および家族の会
        • ii. 成人
          • 1. Turner症候群
          • 2. アンドロゲン不応症候群
          • 3. 真性半陰陽, 先天性副腎皮質過形成
          • 4. 産科婦人科疾患と関連せずに見出された性分化異常
          • 5. 遺伝カウンセリングにあたっての医療サイドの立場
  • Glossary
    P.363閲覧
  • 索引
    P.369閲覧
    • 和文索引
    • 欧文索引
  • 奥付

参考文献

生殖器系の発生・解剖・機能

P.15 掲載の参考文献

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