精巣性女性化症候群
WordNet
- a pattern of symptoms indicative of some disease
- a complex of concurrent things; "every word has a syndrome of meanings"
- the process of becoming feminized; the development of female characteristics (loss of facial hair or breast enlargement) in a male because of hormonal disorders or castration (同)feminisation
- of or involving the testes; "testicular cancer"
PrepTutorEJDIC
- (疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態
UpToDate Contents
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English Journal
- Timing of gonadectomy in adult women with complete androgen insensitivity syndrome (CAIS): patient preferences and clinical evidence.
- Deans R, Creighton SM, Liao LM, Conway GS.SourceUniversity of New South Wales and Royal Hospital for Women, Sydney Australia, NSW, Australia Department of Women's Health, Elizabeth Garrett Anderson UCL Institute of Women's Health, University College Hospital, London, UK.
- Clinical endocrinology.Clin Endocrinol (Oxf).2012 Jun;76(6):894-8. doi: 10.1111/j.1365-2265.2012.04330.x.
- Objective Adult women with complete androgen insensitivity syndrome (CAIS) are increasingly likely to defer or decline gonadectomy despite counselling about malignancy risk. The objectives of this study were to review the evidence on the risk of gonadal malignancy in adult women with CAIS and to
- PMID 22211628
- Male patients with partial androgen insensitivity syndrome: a longitudinal follow-up of growth, reproductive hormones and the development of gynaecomastia.
- Hellmann P, Christiansen P, Johannsen TH, Main KM, Duno M, Juul A.SourceDepartment of Growth and Reproduction, GR-5064, Copenhagen University Hospital, Blegdamsvej 9, DK-2100 Copenhagen, Denmark; anders.juul@rh.regionh.dk.
- Archives of disease in childhood.Arch Dis Child.2012 May;97(5):403-9. Epub 2012 Mar 12.
- OBJECTIVE: To describe the natural history of phenotype, growth and gonadal function in patients with partial androgen insensitivity syndrome.SETTING: Tertiary paediatric endocrine centre.METHODS: Retrospective evaluation of 14 male patients with partial androgen insensitivity syndrome (PAIS) with v
- PMID 22412043
Japanese Journal
- Vaginoplasty with a Pudendal-Thigh Flap in Intersexuals
- Namba Yuzaburo,Sugiyama Narushi,Yamashita Shuji,Hasegawa Kenjiro,Kimata Yoshihiro,Nakatsuka Mikiya
- Acta Medica Okayama 62(6), 415-419, 2008-12
- … One was a female with testicular feminization syndrome for whom we reconstructed the total vagina with a pudendal-thigh flap, and the other was a female with an adrenogenital syndrome for whom we enlarged the introitus of the vagina with the same approach. …
- NAID 120002305474
- P-243 腹腔鏡下鼡径ヘルニア根治術を契機に発見された精巣性女性化症候群の1例(ポスター 臍・鼠径ヘルニア・精巣2,Better Life for Sick Children, Better Future for Pediatric Surgery,第45回日本小児外科学会学術集会)
- 石川 暢己,奥山 宏臣,窪田 昭男,長谷川 利路,川原 央好,上原 秀一郎,三谷 泰之,島田 憲次,松本 富美,松井 太
- 日本小児外科学会雑誌 44(3), 552, 2008-05-20
- NAID 110006834703
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★リンクテーブル★
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- 英
- 精巣性女性化症候群 testicular feminization syndrome
- 同
- アンドロゲン不応症？ androgen insensitivity syndrome アンドロゲン不感性症候群 アンドロゲン非感受性症候群、アンドロゲン抵抗性症候群 androgen resistance syndrome
- 関
- アンドロゲン受容体。ライフェンスタイン症候群
[show details]
概念
- 性染色体は46XYであり精巣が存在するにもかかわらず、男性化が生じず表現型が完全に女性である病態の総称。
- アンドロゲン不応症のうち特に完全型を指していうことがある。
病型
完全型
不完全型
- 精巣性女性化症候群にあてはまるが、外陰部に軽度の男性化が見られる。
- 外陰部：軽度男性化
部分型
病因
- ヌル変異：完全型
- 点変異 ：完全型～部分型。(アンドロゲン受容体の温度不安定性が生じる)
病態
- アンドロゲン受容体異常によるアンドロゲン不応症
- 外性器：女性、内性器：男性
- SRYを有するため、未分化性腺は精巣となり、セルトリ細胞からはMISが分泌されミュラー管を退縮させる。ライディッヒ細胞はテストステロンを分泌し、ウォルフ管を精巣上体、精管、精嚢、射精管に分化させる。ジヒドロテストステロンを受容する受容体に異常があるため、外性器は男性化することができず、女性化する。
症候
検査
- YN.D-80 QB.D-294 G9M.52
- FSH,LH：高値
- 血中エストロゲン：正常男性より多い。
- 血中アンドロゲン：正常男性と同じかや上昇
- 血中テストステロンレベル：健常男性と同レベル
- 組織5α-redactase：正常～上昇
参考
- http://www.nurs.or.jp/~academy/igaku/s3/s35221.htm
国試
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- 関
- testes、testicle、testis
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- 関
- feminize
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精巣性女性化症候群