Testicular feminization chirwere: kurapwa uye kudzivirira

The urwere pakati testicular feminization - munhu dererei vachiberekwa nyonganyonga iri pamwe vasanyanye aikanganiswa vanhurume mahormone. Muzviitiko zvakaipisisa, muviri uye anoita kutindivara kuti zvinokonzerwa androgens. Zviratidzo chirwere ichi chinogona kuva kunosiyana inosiyana zvakaoma kwazvo, uye kurapwa varwere vakanonga nomumwe.

Chokwadi, vanhu vari nematambudziko akafanana chirwere, vanofarira mamwe mashoko. Chii testicular feminization chirwere? Sei kubata chirwere uye kana pane here nzira inobudirira kurapwa? Chii chinokonzera chirwere? Chii nemaonero varwere? Mhinduro dzemibvunzo iyi vari kutsvaga zvakawanda.

The urwere pakati testicular feminization - chii?

Chokutanga, ngationei kubata zvinoreva izwi. The urwere pakati testicular feminization - munhu vachiberekwa chirwere, izvo yakakonzerwa mutation ari bonde chromosome. Kurwara aine kurasikirwa aikanganiswa androgens, ukuru zvinoderedza susceptibility kune vanhurume mahormone zvingave zvakasiyana - ichi zvinoenderana kukuvadza uye zviratidzo.

Somuenzaniso, pane imwe mwero kuramba androgens externally mukomana hwapedyo zvikuru kupengereka. Zvisinei, munhu mukuru anogona kuva, kuti murume chena, nekuti urume muviri wake hadzisi akabudiswa.

Zvakakwana akasiyana anotarisa kurasikirwa zvakakwana aikanganiswa mahormone zvirwere zvakadai testicular feminization syndrome. karyotype kwavanhu munhurume. Zvisinei, vakomana vanoberekwa vaine anonzi hermaphroditism kwenhema, umo paumbwe kwokunze nhengo dzokubereka nadzo munhukadzi mhando pamwe mawa pamberi testes uye chakaipa pamwero testosterone muropa. Pakuyaruka mune vakomana izvi vanowanzopedzisira vava mukadzi pabonde maitiro (semuenzaniso, mumasero mammary wedzera).

The urwere pakati testicular feminization - munhu dererei zvokurwara. 50-70 zviuru vachangoberekwa chakaita kuti chete 1 mwana pamwe mutation uyu. Kana isu funga zviitiko hermaphroditism, anenge 15-20% wevarwere mhaka pamberi atypical nhengo dzokubereka nadzo chokuita STF. Sezvineiwo, vanachiremba zvokurwara rinowanika pasi mazita akasiyana - munhu urwere pakati androgen kusava Morris urwere, mukono pseudohermaphroditism.

STF ari munhukadzi, zvinobvira?

Vanhu vazhinji vanofarira mubvunzo kana testicular feminization chirwere vakadzi zvinokwanisika. Sezvo zvokurwara ari kusonganirana mutation muna Y-chromosome, tinogona zvachose kutaura kuti zviri chete pasi wechirume mumwe.

Ukuwo, vanhu vanotambura nezvirwere zvakadai, kazhinji kutarira somukadzi. Uyezve, vakaona pachavo maererano. Maererano nenhamba, varwere hermaphroditism kwenhema kazhinji seungano, mukadzi anoyevedza akareba pamwe dzakaonda nhamba. Vanhu vane chirwere yakadaro kunyange ndiye dzimwe tsika, kusanganisira musoro uye yakarurama ungwaru, kukwanisa kukurumidza dzokumabvazuva mumamiriro ezvinhu, simba, kushanda zvakanaka nevamwe "munhurume" unhu.

Chimwe dzinonakidza chokwadi - Vakadzi vazhinji munyaya mitambo, vane murume karyotype. Ndicho chikonzero vatambi vari nyanzvi tora mate kuti DNA ongororo - vakadzi (ndiko, varume) pamwe chirwere Morris haabvumirwi kukwikwidzana.

Nenzira, kuvapo chirwere hwakadaro kunoitwa evanhu mazhinji, kusanganisira Joan pamusoro Arc uye yakakurumbira Mambokadzi England Elizabeth Tudor.

The kukonzera chirwere kukura

Sezvambotaurwa, Morris syndrome (testicular feminization chirwere) mugumisiro AR Gene kuremara. mumwe mutation kwakadaro kukanganisa receptors kuti sei androgenic mahormone, somugumisiro vanongovhura kutindivara. Maererano zvidzidzo, zviri chinopfuudzwa pamusoro chirwere X-kwakabatana recessive unhu, sezvamakaropafadzwa mutakuri chakaremara majini, kazhinji amai. Ukuwo, zvinokwanisika, uye kwakanga kwatangwa mutation ari mwana, uyo akazvarwa nevabereki vaviri zvikuru utano, asi vari Pakadaro kushumwa kure zvishoma kakawanda.

Munguva embryo Kugadzirwa gonads (mumasero pabonde) vari kuumbwa maererano mudumbu karyotype - mwana ane azere manhu. Asi nokuda zvinyama kuzotadzira jini haana kugumbuka (chakagomadzwa) kuti testosterone uye degidrosteronu, izvo basa paumbwe yemukomana, scrotum, urethra, yeprostate. Panguva iyoyo irambe nehanya kuti munyama kuti estrogen, iyo hunokonzera pakuzadzisa ari nhengo dzokubereka nadzo munhukadzi mhando (kunze dumbu, fallopian sepombi uye yokumusoro rechitatu nyinyo).

The azere chimiro chirwere uye mamiriro ayo

Testicular feminization syndrome (Morris) angaperekedzwa kurasikirwa zvakakwana Kanotokonya aikanganiswa testosterone. Muzviitiko zvakadaro, mwana anozvarwa aine murume genotype (pane Y-chromosome), vanhurume omumuviri, asi hadzi kwokunze nhengo dzokubereka nadzo.

Vana vakadaro havana scrotum uye mboro uye manhu Ndaindogara abdominal mhango. Asi, pane nyinyo dzokunze labia. Kazhinji, vanachiremba Pakadaro vanoti pamusoro pakazvarwa vasikana. Rubatsiro, varwere vakanga kubatwa, sezvo mutemo, wechiduku chema kusavapo kumwedzi. Nenzira, mwana kuva yechipiri pabonde zvechadenga hadzi mhando (hapana mutation nenzwi, bvudzi kukura, chipfuva nedumbu). Mune hwakadzama kuongororwa chiremba kunotema kuti gonads ose vanhurume uye yakananga vanochengeta okutodza.

Kazhinji, zvokuongorora kuti "testicular feminization chirwere" isa munhu mukuru vakadzi vanoenda nyanzvi pamusoro amenorrhea uye kushaya mbereko.

Kukwana siyana ari chirwere Morris uye ukuru kwayo budiriro

The urwere pakati testicular feminization nevanhu vanogona chete pamwe zvishomanana zvinoderedza Kanotokonya aikanganiswa testosterone. Muzviitiko zvakadaro, chine zvingaratidza kuva siyana. kupatsanura hurongwa akasikwa 1996, pasi rine chikuru zvimiro mashanu chirwere.

• First dhigirii, kana varume mhando. Mwana ane raidudzwa mukono phenotype uye anova asina mucherechedzo zvarakasiya. Apo neapo kuyaruka akacherechedza zamu akawedzera uye uncharacteristic kuchinja inzwi. Asi varwere kwagara vasingadzoreki spermatogenesis, zvichiguma mbereko.
• dhigirii Second (zvimwe makono mhando). Development ari munhurume mhando, asi vamwe zvakasiyana. Somuenzaniso, zvimwe inotungamirwa micropenis uye hypospadias (zvaizopedza meatus). Kazhinji, varwere kutambura gynecomastia. Akacherechedza uye gogonera deposition pamusoro subcutaneous mafuta.
• dhigirii rechitatu, kana pave havana chokwadi mhando. Varwere vakachengeta anyatsoona rutapudzo pakati yemukomana. Modified uye scrotum - dzimwe nguva ari muchimiro afanane rwokunze labia. Pane misha yacho urethra, uye manhu kazhinji haana uchaburukira scrotum. Kunoonekwa uye maitiro zviri vakadzi - zamu augmentation, chaiwo mufananidzo (rakafara hudyu, yakamanikana pamafudzi).
• dhigirii chechina (kunyanya vechikadzi mhando). Varwere vari muboka iri vane hadzi phenotype. Manhu kuramba ari abdominal mhango. Iva munhukadzi pabonde nhengo, asi vamwe kushanduka. Somuenzaniso, mwana ari inoumbwa pfupi "bofu" ari nepanhengo uye clitoris kazhinji hypertrophied uye anoyeuchidza micropenis.
• Wechishanu dhigirii, kana hadzi mhando. Ichi chimiro chirwere achabatsirwa paumbwe kwevanhukadzi zviratidzo - mwana anozvarwa ari musikana. Zvisinei, pane dzimwe zvarakasiya. Zvikurukuru, varwere kazhinji vakachengeta akawedzera clitoris.

Zvinonzi pamwe zviratidzo testicular feminization syndrome. Kubereka pamwe zvokurwara yakafanana hazvibviri - muviri murwere Haibudisi vanhurume masero, uye hadzi zvomukati havasi kuwanika kana vasiri zvakazara kuumbwa.

Maererano nenhamba, vanhu vane chirwere chakadai kazhinji kutambura inguinal hernias, nokuda kutyora ndima manhu ari inguinal mugero. Nokuda misha iri kwokunze Huro urethra panjodzi zvirwere zvakasiyana-siyana nezvezvimwe zvinetso zvedundira (semuenzaniso, pyelonephritis, urethritis, uye zvimwe zvirwere anogumbura).

chirwere dzokuvhiya

Chirwere ichi - yakareba kwacho. Rinosanganisira boka maitirwo:

• Chokutanga, chiremba anounganidza nhoroondo. In weboka muongororo vanofanira kuwana kana mwana ane zvimwe zvokukura nepfungwa azvarwa kana pakuyaruka. Anoongororwa nhoroondo yemhuri (kana pakanga zvarakasiya dzakadaro kubva kuhama).
• Danho rinokosha riri uye panyama kuongororwa, umo nyanzvi anogona kuona pamberi abnormalities marongerwo mumuviri uye kwokunze nhengo dzokubereka nadzo, kuti mhando nokuparadzirwa bvudzi, uye zvichingodaro. D. Ndakasimudza nokuyerwa kureba uye uremu murwere. More tsvakurudzo kunodiwa kuziva comorbidities.
• Mune remangwana akabata karyotyping - muitiro kuti anobvumira kuziva uwandu uye unhu okutodza, izvo zvinozoita zvinoita kuti azive bonde murwere.
• Kana zvakakodzera, akaita samolecular nemagene yekuedzwa, panguva iyo nguva kuedza kuziva nhamba uye dzakasiyana-siyana zvaparadzwa majini.
• Hazvina fanira kuongorora kuburikidza chiremba wezvedundira - chiremba ari kudzidza marongerwo uye zvaidiwa chokunze nhengo dzokubereka nadzo, palpate kweprostate, etc ...
• On pachisara ropa anoendeswa kuongorora mahomoni.
• It chinodzidzisa uye Ultrasound ongororo. nzira Izvi zvinoita kuti vanzwe anomalies ari nemamiriro womukati nhengo dzokubereka nadzo, testes Undescended kuzviona, rokuongorora comorbidities.
• Mashoko echokwadi kwazvo nemamiriro zvomukati kunogona kuwanikwa munguva rinobva resonance kana computed tomography.

The urwere pakati testicular feminization: kurapwa

Zvipatara, muchiitiko ichi kunoenderana makore murwere uye mwero kusava kune androgen Kanotokonya kemikari. Hako basa kemikari yokutsiva kurapa, izvo anobvumira kubvisa androgen kushaikwa, kubatsira angagadzirira yechipiri pabonde unhu, kubvisa zvinobvira anomalies kukura.

Zvinonzwisiswa kuti idanho zvikuru chinokosha psychotherapy - The murwere unofanira kugara kubvunza nyanzvi. Pashure pezvose, maererano nenhamba, mumwe hermaphroditism kwenhema zvinowanzoita kuti pave depression. Kana mutation kunokwanisa kuonekwa nemasanga panguva akura (tiri kutaura pamusoro kusava wakakwana kuna testosterone receptors), chiremba anogona kusarudza arege kuvazivisa mukadzi anogara upenyu huzere uye raanozvizivisa somumiririri Fair pabonde.

Kana Kuvhiya chinodiwa?

Zvinetso zvakawanda zvinogona kugadziriswa nokubatsira dzinokosha dzokuvhiya. Nokuti varwere hadzi phenotype kunoratidza nokubviswa testes. nzira Izvi zvakakodzera kudzivirira hernias nokuti kukura uye pakuzadzisa munhurume dzepabonde zvatariswa. Uyezve, nzira iri kudzivirira testicular kenza.

Kana muviri ikabuda hadzi mhando, dzimwe nguva zvakakodzera epurasitiki nepanhengo uye vulva, izvo zvinoita kuti tikwanise kuva zvepabonde. Surgically anogona rai kurega zvaizopedza Urinary Canal.

With kukura murwere varume muenzaniso dzimwe nguva zvakakodzera kuunza seminal ducts ari scrotum. Sezvo vanhu vakawanda vane chirwere chakadai achitambura gynecomastia, chinowanzoonekwa akaita zamu mapurasitiki pokuvhiyiwa, izvo kubatsira nokudzosa muviri wechisikigo chimiro.

The fungidziro varwere uye zvichiita yematambudziko

The urwere pakati testicular feminization (Morris) haisi zvakananga upenyu. Muviri kuipa mabasa kunyange kana zvakazara kusava kuti androgen mahormone. Mushure zvokurapa uye kuvhiya murwere anogona kurarama upenyu huzere mukadzi, ane murume karyotype. Asi pane pangozi yokuita testicular kenza kuti haana uchaburukira scrotum - Muzviitiko zvakadaro zvakafanira kuita. Nokuti kudzivirira kenza akaita zvokuvhiyiwa nokubviswa testes vari scrotum (kana murwere ari munhurume phenotype) kana nokubviswa shwanu rakakwana (kana mukadzi phenotype).

Panyaya zvimwe zvinoita yematambudziko vavo mazita ndiwo hazvibviri zvepabonde (malformation kuti sikarudzi), voiding (mu nedanho kukura urogenital gadziriro Urinary gwara-chinja). Varwere vari mbereko zvisinei phenotype. Usakanganwa pamusoro evanhu matambudziko, sezvo asiri ose mwana uye wechiduku zvikuru zvinoita kuti kunzwisisa hunhu muviri wayo. Chokwadi, dambudziko racho pabonde bhora, uyewo zvirwere excretory ezvinhu, inogona kubviswa pakuvhiya. The fungidziro varwere zvakanaka zvakadaro.

Pane matanho chokudzivirira?

Zvinosuruvarisa hapana nzira kudzivisa chirwere chakadai. Asi sezvo testicular feminization chirwere chinhu nemagene zvokurwara, ngozi yokuva nekenza kunogona kuonekwa panguva padariro kwekuronga pamuviri - vanotarisira vabereki vanofanira kuti kuedzwa.

Varwere kare nechirwere zvinetso, vanoda vanokwanisa kurapwa rubatsiro uyewo kugara zvokurapa bvunzo, hormone kurapa.