正常及異常之性別發(fā)展

1、1正常及異常之性別發(fā)展正常及異常之性別發(fā)展區(qū)分及決定性別：1.基因性別2.性腺性別3.荷爾蒙性別4.內(nèi)生殖器5.外生殖器6.腦部之性別（CNSbrainsex）7.後天指派決定之性別（Assignmentrearingsex）8.性別鑑定或性別表現(xiàn)正常發(fā)展建立基因性別受孕性腺分化1.性腺分化發(fā)展在56週此時Primodialgermcell移行至gonadalridges如果失敗則造成streakgonad六週前為Bipotential

2、(indifferent)的性腺(包含germcellsspecialepithelialmesenchymemesonephricductinternalsexternalgenitaliaundifferentiated)2.接下來的分化由基因調(diào)控睪丸基因決定組（TDFgene）(SRYregionofYsexdeterminingregionoftheYchromosome)對睪丸分化是必須的如缺少TDFgene會造成gonada

3、ldysgenesisTDF或SRY蛋白質(zhì)基因?qū)m當?shù)男韵侔l(fā)展是需要的3.另外autosomegene(steroidogenicenzymes)也是必須的睪丸的形成睪丸的形成：67週:SertolicellsspermatogeniccdsseminiferoustubulesLeydigcells卵巢的發(fā)展：卵巢的發(fā)展：約於晚兩週發(fā)生Germcells於皮質(zhì)層增生卵細胞20週時五到七百萬出生時只剩一到兩百萬此時的卵細胞為propha

4、seI3性別的指派社會的interactiongenderroleisheavilyinfluencedbyassignmentofsexofrearingfollowedbysocialinteractionbasedupongenitalappearancethedevelopmentofsecondarysexualacteristics.異常性別發(fā)展A.胎兒內(nèi)分泌的異常（disdersoffetalendocrinology）M

5、ulinizedfemales(femalepseudohermaphroditism)1.Congenitaladrenalhyperplasia21hydroxylase(P450c21)deficiency11?hydroxylase(p450c11?)deficiency3?hydroxysteroiddehydrogenasedeficiency2.ElevatedrogensinthematernalcirculationD

6、rugintakeMaternaldisease3.Aromatase(P450arom)deficiencyIncompletelymulinizedmales(malepseudohermaphroditism)1.rogeninsensitivitysyndromes2.5?Reductasedeficiency3.Testosteronebiosynthesisdefects3?hydroxysteroiddehydrogena

7、sedeficiency17?hydroxylase(p450c17)deficiency17?hydroxysteroiddehydrogenasedeficiency4.Gonadotropinresistanttestes5.AntimullerianhmonedeficiencyB.異常性腺發(fā)展(disdersofgonadaldevelopment)MalepseudohermaphroditismPrimarygonadal