Testicular syndrome feminization: maimoatanga me te ārai

Ko te syndrome o feminization testicular - he mate congenital ngā onge tahi nei e te hekenga i roto i te tūtohutanga ki hormones tane. I roto i te wā atu nui, e te tinana, ka meinga kiriuka ki nga pānga o androgens. Ka taea e whai taumata o te pakeke nga tohu o tenei mate, me te maimoatanga mō tūroro mau takitahi.

O te akoranga, e fehangahangai iwi ki te mate rite, e hiahia i roto i ētahi atu mōhiohio. He aha te mea testicular feminization syndrome? Me pēhea te ki te hamani i te mate, me te mehemea reira he ara tino whai hua o te maimoatanga? He aha ai te mate? He aha te mea te outlook mō ngā tūroro? Kei te titiro pahonoraa ki enei pātai mō te tokomaha.

Ko te syndrome o feminization testicular - he aha te mea te reira?

Tuatahi, kia mahi a ki te tikanga o te wā. Ko te syndrome o feminization testicular - he mate congenital, meinga nei e te irakētanga i roto i te pūira sex. Mate tahi e te mate o te tūtohutanga ki androgens, kia rerekē te tohu o te whakaiti i roto i te susceptibility ki hormones tane - tenei tei runga i te pakeke me te tohu.

Hei tauira, i te ātete āhua ki androgens waho whakawhanake tamaiti tino tikanga. Heoi, ka taea e te pakeke e, e te tangata he horomata, no te mea te parāoa te kore noa hua tona tinana.

Mutu rerekē titiro oti mate o te tūtohutanga ki hormones ki taua mate rite syndrome feminization testicular. karyotype tangata ko te tane. Heoi, kei te whanau tamariki ki te hermaphroditism teka pera-ka karanga, i roto i nei i reira ko te hanganga o nga taihemahema waho o te momo wahine ki te aroaro tukutahi o nga testes me te taumata noa o testosterone i roto i te toto. I roto i pūhuruhurutanga i roto i enei tamariki ahu ki te whakawhanake i ngā āhuatanga fakasekisualé wahine (hei tauira, ake i repe mammary).

Ko te syndrome o feminization testicular - he māuiuitanga āhua onge. kiia 50-70 mano hou mō te 1 anake tamaiti ki tenei irakētanga. Ki te whakaaro tatou ki nga take o te hermaphroditism, āhua 15-20% o tūroro hono te take o te aroaro o te taihemahema mohiotia te ki te STF. Ratau, i roto i te māuiuitanga hauora puta i raro i ngā ingoa rerekē - he syndrome o oreraa androgen Morris syndrome, pseudohermaphroditism toa.

STF i roto i te wahine: ko reira taea?

He hiahia i roto i te pātai o mehemea he taea te syndrome feminization testicular i roto i nga wahine tokomaha te iwi. Mai e pā ana te māuiuitanga e ki te irakētanga i roto i te Y-pūira, taea tino tatou te mea e nei hoki e ngawari anake ki te tane takitahi.

I te tahi atu te ringa, iwi e mate ana i te mate taua, maha titiro rite te wahine. Ano, kite ratou ratou ia au i. E ai ki ngā tatauranga, tūroro ki hermaphroditism teka titiro maha rite te ātaahua, te wahine roroa ki ahua hiroki. Iwi ki te tātaritanga taua ara kiia ki etahi āhuatanga motuhake, tae atu maramarama arorau, me te tika, te kaha ki te hohoro takoto i roto i te āhuatanga, te pūngao, te kakama, me te tahi atu mau huru maitatai "tane".

Ko tētahi atu meka ngā - tokomaha nga wahine whai wāhi i roto i hākinakina, i te karyotype tane. Ko te aha kaitäkaro ngaio tango huware mō te DNA tātaritanga - wahine (e ko, te tangata) ki te syndrome kore te Morris whakaaetia ki te whakataetae.

E te ara, pēnei te aroaro o taua mate kei te ki maha whika hītori, tae atu Joan o Arc me te Kuini rongonui o Ingarangi Elizabeth Tudor.

Ko te take matua o te whanaketanga mate

Ka rite ki kua whakahuatia, Morris syndrome (testicular syndrome feminization) ko te hua o te koha AR ira. he irakētanga taua e pa i te mau farii ohipa e whakautu ki hormones androgenic, rite ki te hua ratou hei noa e teoteo. E ai ki ngā akoranga, kua tuku ai i runga i te huru ngoikore syndrome X-hono, ai te kawe o ira hapa, ko te nuinga o te whaea. I te tahi atu te ringa, ko reira taea, me te irakētanga noa i roto i te tamaiti, i hapu e rua ngā mātua rawa hauora, engari kua korerotia take pērā tawhiti iti pinepine.

I roto i te whanaketanga embryonic o nga gonads (repe fakasekisualé) e hanga e rite ki te karyotype kukune - He komurumurua tonu te tamaiti. Otiia no te mea kahore he tairongo o te kino kikokiko o te ira (hia-) ki testosterone me degidrosteronu, e he kawenga mō te hanganga o te ure, raho, taiawa mimi, repeure. I te wa ano i tiaki te tūtohutanga o te kopa ki te estrogen, e ai te whanaketanga atu o nga taihemahema o te momo wahine (anake kōpū, korere kākano me te tuatoru o runga o te tenetene).

Ko te ahua tonu o te syndrome me ona āhuatanga

Testicular syndrome feminization (Morris) kia haere tahi e oti mate o receptor tūtohutanga ki te testosterone. I roto i ngā take pēnei i, whanau he tamaiti e ki te tohuira toa (i reira ko Y-pūira), repe tane, engari nga taihemahema uha waho.

Taua tamariki kahore Tunaroa me ure me i romia, me noho i roto i te kōhao puku. Engari, i reira ko te labia tenetene me waho. Tino maha, tākuta i roto i ngā take pērā mea e pā ana ki te whanautanga o ngā kōtiro. Hoki te tauturu, i tukinotia tūroro, rite ki te tikanga, amuamu te taiohi o te ngaro o te paheketanga. E te ara, te tamaiti te whakawhanake Tuarua āhuatanga fakasekisualé o momo wahine (kahore irakētanga o te reo, tupu makawe, whakarahi uma). I roto i te whakamātautau taipitopito te tākuta whakatau e nga gonads o tane, me te huinga motuhake o pūira.

E mea pinepine, te tātaritanga o "syndrome feminization testicular" hoatu he wahine pakeke e tahuri ki te mātanga e pā ana ki amenorrhea me te pākoko.

momo oti o te syndrome Morris me te tohu o tona whanaketanga

taea anake te haere tahi te syndrome o feminization testicular i roto i nga tangata e te whakaiti wāhanga i roto i te receptor tūtohutanga ki te testosterone. I roto i te take i taua, kia waiho atu kanorau he huinga o ngā tohu. I hanga pūnaha whakarōpū i roto i 1996, i raro i nei i reira e rima ngā matua o te mate.

• tohu Tuatahi, ranei momo tane. He te tamaiti he tohuāhua tane korerotia me te whanake i waho i tetahi ine i tohua. Taime te taiohitanga kitea whakarahi uma me te huringa uncharacteristic o reo. Ko tūroro tonu kua waimaero spermatogenesis, hua i roto i te pākoko.
• tohu Tuarua (momo pai tane). Ko te whanaketanga o te momo tane, engari ki etahi rerekētanga. Hei tauira, te taea micropenis hanganga me te hypospadias (wāhikē meatus). E mea pinepine, mamae tūroro i te gynecomastia. waipara ua faariro me te pāhiwihiwi o te ngako subcutaneous.
• Ko te toru o tohu, te whanaketanga o te momo neki ranei. kite tūroro he whakaiti i tohua o te ure. Whakarerekē me te Tunaroa - ētahi wā i roto i te puka rite reira te labia waho. He he he displacement o te taiawa mimi, me i romia kihai maha i heke ki te Tunaroa. Tohua a āhuatanga āhuatanga o te wahine - whakanui uma, he ahua angamaheni (hope whanui, pokohiwi kuiti).
• tohu te wha (momo nuinga wahine). Tūroro i roto i tenei rōpū i te tohuāhua wahine. noho komurumurua i roto i te kōhao puku. Te whakawhanake wahine whekau moepuku, engari ki etahi rerekētanga. Hei tauira, i hanga te tamaiti e te poto "matapo" te tenetene me te tonetone maha hypertrophied me fakamanatu micropenis.
• tohu tuarima, ranei momo wahine. haere tahi ana tēnei puka o te mate e te hanganga o tohumate o te wahine - whanau te tamaiti kei te he kotiro. Heoi, i reira e te tahi mau ine. I roto i ngā, kei te maha kitea tūroro whakarahi o te tonetone.

Kei te haere tahi te reira e tohu o syndrome feminization testicular. e kore he Reproduction ki rite māuiuitanga taea - e kore e tinana o te manawanui hua pūtau tane, a kahore e wātea whekau wahine ranei kahore e tino i hanga.

E ai ki ngā tatauranga, mamae maha iwi ki te tātaritanga taua i hernias inguinal, e tika ana ki te tūkino o te wahi o nga komurumurua o te awa inguinal. Nā ki te displacement o te whakatuwheratanga waho o te taiawa mimi i nui ake te mōrea o ngā mate o te pūnaha mimi (hei tauira, pyelonephritis, urethritis, me ētahi atu mate inflammatory).

ngā tukanga tātaritanga

Tātaritanga o tenei mate - he tukanga roa. Kei roto i te huinga o ngā tukanga:

• Hei timata, kohikohi te rata hītori. I roto i te akoranga o te rangahau e hiahia ana ki te kitea i roto i mehemea kua etahi disorders whanaketanga i muri i whanau ranei i roto i pūhuruhurutanga o te tamaiti. Tātarihia me te aamu utuafare (mehemea reira i kua taua ine i whanaunga).
• He taahiraa nui ko me te whakamātautau tinana, i roto i nei te mātanga e taea mōhio te aroaro o abnormalities i te hanganga o te tinana, me te o nga taihemahema waho, te momo o te makawe tohatoha, me pera i runga i. Held D. inenga o te tiketike, me te taimaha o te manawanui. hiahiatia rangahau atu te ki te tāutu i ngā comorbidities.
• I roto i te heke mai tū karyotyping - he tukanga e taea ki te whakatau i te nui, me te kounga o ngā pūira, i, i roto i te tahuri, hanga ana e ia taea ki te kitea i roto i te takoto o te manawanui.
• Ki te tika, kawea atu whakamātautau ira rāpoi, i roto i nei te wa e ngana ana ki te kitea i roto i te maha, me te momo o ira pakaru.
• Ko reira whakamātautau whakahauanga e te roma mimi - te ako i te tākuta te hanganga me ngā āhuatanga o te taihemahema waho, palpate te repeure, aha ...
• I te tātari, toto tangohia te ki tirohia taumata taiki.
• Ko te whakamātautau kōrero me te ultrasound. hanga ana tēnei tukanga taea reira ki te kitea kōhikohiko i roto i te hanganga o nga whekau whakaputa uri ā-, testes Undescended kite, whakatau comorbidities.
• Ka taea te whiwhi te mōhiohio tika te nuinga e pā ana ki te hanganga o te whekau i roto i te te kōwarotanga autō tomography computed ranei.

Ko te syndrome o feminization testicular: maimoatanga

Therapy, i roto i tenei take, tei runga i te tau o te manawanui me te tohu o te oreraa ki te taiaki receptor androgen. Tika whakaritea taiki whakakapinga therapy, e taea ana ki te whakakore i hē androgen, ki te āwhina i te hanga i te tika ngā āhuatanga sex tuarua, ki te whakakore kōhikohiko taea i roto i te whanaketanga.

Kei te matau te reira e te taahiraa tino nui ko te hinengaro - te manawanui me kōrero tonu te mātanga. I muri i te katoa, rite ki tatauranga, maha arata'i te hermaphroditism teka ki te whanaketanga o te whārua haumanu. Ki te pāngia tetahi irakētanga e tupono noa i roto i te pakeketanga (e korero tatou e pā ana ki te oreraa oti ki receptors testosterone), kia whakatau te tākuta e kore ki te pūrongo i te reira ki te wahine e ora i te ora tonu, me te tāutu ia rite te māngai o te takoto ataahua.

A, no te he e tika ana pokanga?

e taea te whakatika maha raruraru ki te awhina o ngā tukanga motuhake. Hoki tūroro ki tohuāhua wahine whakaatu tango o nga testes. Ko te tika tenei tukanga ki te ārai i hernias no te whanaketanga me te whanaketanga atu o ngā āhuatanga fakasekisualé tane. I tua atu, ko te tikanga ko te ārai o te mate pukupuku testicular.

Ki te whakawhanake i te tinana i roto i te momo wahine, he reira te tahi mau taime tenetene kirihou tika, me te teke, e hanga ana e ia taea ki te whai takoto. Hāparapara taea te whakakore wāhikē awa mimi.

Ki te whanaketanga o te tauira toa o te manawanui ko ētahi wā e tika ana ki te kawe i te aerodynamics huero i roto i te Tunaroa. Mai mamae maha nga tangata ki te tātaritanga taua i gynecomastia, kua maha whakamana reira uma pokanga kirihou, e tauturu i whakahoki āhua tinana maori.

Ko te waitohunga mō tūroro me pōauautanga taea

E kore te mea te syndrome o feminization testicular (Morris) he riri tika ki te ora. Ko te tinana Ko te mahi noa noa, no te ki te tino oreraa androgen hormones. I muri i manawanui hauora me te hāparapara taea ora te ora tonu o te wahine, he karyotype tane. Ko reira te mea he mōrea o te whakawhanake mate pukupuku testicular i kore e heke ana ki te Tunaroa - i roto i te wā, pērā he reira e tika ana ki te tango i mahi. Hoki te ārai o te mate pukupuku i mahi tango hāparapara o te testes i roto i te Tunaroa (ki te ko te manawanui tohuāhua toa) ranei oti tango o te repe (ki te tohuāhua wahine).

Ki te whakaaro ki ētahi atu pōauautanga taea e ratou rārangi ko te eita o takoto (malformation o kikokiko), Manakore (i roto i te tukanga o te whanaketanga o te pūnaha urogenital mimi whakaneke hongere). He o Apimereke, tūroro ahakoa o tohuāhua. Kaua e wareware e pā ana ki te mau fifi pāpori, rite kore nga tamariki, me te kuao tangata te atu taea ki te matau i te āhuatanga o ona ake tinana. O te akoranga, te raruraru o te ao fakasekisualé, me te mate o te pūnaha puha, e taea te to'o i roto i te pokanga. Ko te waitohunga mō tūroro pai tonu.

He reira āraihia?

Kia aroha mai, i reira e kore rawa e taea ki te aukati i taua mate. Engari kia rite ki syndrome feminization testicular ko te māuiuitanga ira, e taea te kitea te mōrea o te whakawhanake i mate pukupuku i te wā o te whakamahere o te hapūtanga - Me mātua tūmanako ki te kia whakamatautauria.

Hoki tūroro kua pāngia ki disorders, e hiahia ana ratou āwhina takuta me te whakamātautau hauora auau, therapy hormonal.