Wamasende feminization syndrome: ukwelashwa kanye nokuvimbela

Lesi sifo ka feminization wamasende - ukuphazamiseka ayeyivelakancane azalwa naso okuyinto kuhambisana ukwehla luvelo hormone ocansini besilisa. Ezimweni ezimbi kakhulu, umzimba kanye akusho babe buthuntu lapho imithelela androgens. Izimpawu zalesi sifo ungaba ntshashintshe of kobukhulu kanye nokwelashwa kwe-iziguli abelethwe ngamunye.

Yiqiniso, abantu ibhekene isifo esifanayo, banesithakazelo ukwaziswa okwengeziwe. Kuyini wamasende feminization syndrome? Kanjani kanjani lesi sifo futhi ukuthi kukhona izindlela eziphumelelayo ngempela ukwelashwa? Yini ebangela izifo? Kuyini isimo iziguli? Izimpendulo zale mibuzo athungatha eziningi.

Lesi sifo ka feminization wamasende - kuyini?

Okokuqala, ake ukubhekana ne lisho ukuthini igama elithi. Lesi sifo ka feminization wamasende - yesifo azalwa naso, okuyinto kubangelwe ngokuguqula izakhi zofuzo chromosome ocansini. Ukugula ephelezelwa ukulahlekelwa luvelo androgens, degree of nokuncipha ezifweni nama-hormone ocansini besilisa ingase ibe khona ehlukile - lokhu kuncike kobukhulu kanye nezimpawu.

Ngokwesibonelo, ngesikhathi ukumelana ngokulinganisela androgens ngaphandle umfana eba ngempela evamile. Nokho, umuntu omdala kungaba, ukuthi lo muntu oyinyumba, ngoba isidoda isidumbu sakhe nje hhayi kukhiqizwa.

ezahlukene ngokuphelele ubukeka ukulahlekelwa ephelele luvelo hormone yokulwa nezifo ezifana wamasende feminization syndrome. karyotype womuntu wesilisa. Nokho, abafana bazalwa okuthiwa hermaphroditism kwamanga, lapho kukhona ukwakheka Izitho zangasese sangaphandle uhlobo insikazi khona ngesikhathi esisodwa testes futhi ezingeni evamile kwe-testosterone egazini. Phakathi nenkathi yokuthomba kulezi abafana kwenza babe nezici ngokocansi owesifazane (isib, nezindlala mammary ukwandisa).

Lesi sifo ka feminization wamasende - a zokugula abavamile. ayizinkulungwane 50-70 izinsana zaba ingane 1 kuphela nale wezakhi. Uma sicabangela amacala hermaphroditism, cishe 15-20% eziguli imbangela khona Izitho zangasese atypical ehlangene STF. Phela, in the zokugula wezokwelapha livela ngamagama ahlukene - a syndrome ka insensitivity androgen Morris syndrome, pseudohermaphroditism besilisa.

STF e insikazi: kungenzeka?

Abantu abaningi banesithakazelo umbuzo ngabe wamasende feminization sifo ngo abesifazane kungenzeka. Kusukela zokugula lihlotshaniswa kuguquka ku-Y-chromosome, singakwazi ngokuqinisekile ithi ngokwencwadi owesilisa ngamunye kuphela.

Ngakolunye uhlangothi, abantu abahlushwa izifo ezifana, ngokuvamile ibukeke owesifazane. Ngaphezu kwalokho, lapho bebona ngokwabo ngokufanele. Ngokwezibalo, iziguli ezinemishini hermaphroditism kwamanga ngokuvamile ibukeke i, wesifazane okhangayo abade nge sibalo mafutha. Abantu ne kokutshelwa ngokukhubazeka kwengane ngisho udumo ezinye izici ekhethekile, kuhlanganise intelligence okunengqondo futhi kunembile, ikhono ngokushesha Orient e isimo, amandla, ukusebenza kahle kanye nezinye "owesilisa 'izimfanelo.

Kuvela elinye iqiniso elithakazelisayo - abesifazane abaningi abathintekayo kwezemidlalo, abe karyotype besilisa. Yingakho abadlali professional ukuthatha ngamathe ngoba i-DNA ukuhlaziywa - abesifazane (okungukuthi, amadoda) sifo Morris ayivunyelwe ukuncintisana.

By the way, khona isifo enjalo kuthiwa wenziwa izibalo eziningi zomlando, kuhlanganise Joan of Arc kanye Queen adumile of England U-Elizabeth Tudor.

Ziyini izimbangela eziyinhloko zalokhu yentuthuko isifo

Njengoba sekushiwo, Morris syndrome (wamasende feminization syndrome) kuwumphumela AR isakhi sofuzo isici. kuguquka okunjalo ezithinta ezenza umuntu ezwe hormone androgenic, ngenxa yalokho nje babe buthuntu. Ngokocwaningo olwenziwa, it is ngocansi ngalesi sifo X-exhumene imfanelo esikhubazayo, lapho othwala izakhi zofuzo ezilimele, ngokuvamile umama. Ngakolunye uhlangothi, kungenzeka, futhi kuguquka nokuguqulwa umntwana, ngubani wayezelwe abazali ababili ngempela enempilo, kodwa Ezimweni ezinjalo ezibikwa ngokuvamile kude esingaphansi.

Phakathi ukuthuthukiswa embryonic we gonads (nezindlala ngokobulili) akhiwa ngokuvumelana karyotype umbungu - ingane has amasende egcwele. Kodwa ngenxa umonakalo izicubu we isakhi sofuzo ingazikhathaleli (ngisaba nozwela) ukuze testosterone futhi degidrosteronu, okuyinto banesibopho ukwakheka umthondo, scrotum, urethra, wendlala. Ngesikhathi esifanayo walondoloza nozwela izicubu estrogen, elibangela sathuthukisa we zangasese yohlobo zesifazane (ngaphandle kwesibeletho, amashubhu fallopian nowesithathu engenhla sangasese).

Ifomu egcwele syndrome kanye nezici zayo

Wamasende feminization syndrome (Morris) kungase kuhambisane ukulahlekelwa ephelele yokwamukela luvelo testosterone. Ezimweni ezinjalo, ingane izalwe nge genotype besilisa (kukhona Y-chromosome), ubulili nezindlala wesilisa, kodwa owesifazane Izitho zangasese zangaphandle.

Izingane ezinjalo abanalo scrotum futhi umthondo futhi amasende futhi bahlale ngaphakathi komgodi ongaphakathi kwamathumbu. Kunalokho, kukhona labia emomozini futhi yangaphandle. Kaningi, odokotela ezimweni ezinjalo uthi ngokuzalwa amantombazane. Ukuze uthole usizo, iziguli babephathwa, njengoba umthetho, osemusha wakhala ukungabi khona esikhathini. By the way, ingane ukuthuthukisa izici ngokobulili yesibili uhlobo besifazane (ukuguquka kwezwi, izinwele ukukhula, webele ukukhulisa). Ngo uthole ukucwaningwa okuningiliziwe le udokotela eqinisekisa ukuthi gonads ka ubulili besilisa isethi ezithile zofuzo.

Ngokuvamile, sitholakele "feminization wamasende syndrome 'ababeki abesifazane omdala abaphendukela onguchwepheshe mayelana amenorrhea kanye nenzalo.

Amafomu engagcwalisiwe ngokuphelele yalesi sifo Morris kanye degree sokuthuthuka

Lesi sifo ka feminization wamasende ezingabantu kungenziwa kuphela ephelezelwa ukuncipha isikhashana esinqunyiwe yokwamukela luvelo testosterone. Ezimweni ezinjalo, iqoqo izimpawu kungenzeka wehlukile. ngezigaba uhlelo wadalwa 1996, kukhona ngaphansi okuyinto izinhlobo ezinhlanu eziyinhloko zesifo.

• degree Okokuqala, noma uhlobo besilisa. Ingane ine phenotype owesilisa evelele futhi ithuthukisa ngaphandle ngeziphambeko emakiwe. Ngezikhathi ezithile usakhula waphawula webele ukukhulisa futhi ushintsho uncharacteristic kwezwi. Kodwa iziguli bezilokhu kahle spermatogenesis, okuholela nenzalo.
• degree Okwesibili (uhlobo mhlawumbe besilisa). NeTuthuko ohlobo wesilisa, kodwa ngokuhlukahluka okuthile. Ngokwesibonelo, kungenzeka ukwakheka micropenis futhi hypospadias (offset meatus). Ngokuvamile, iziguli uhlushwa gynecomastia. sebethule Kubhekiwe futhi engalingani fat ongama.
• Izinga lesithathu, noma ukuthuthukiswa uhlobo banemizwa engqubuzanayo. Iziguli waphawula ukuncipha wawubona wenduku. Kushintshwe futhi scrotum - ngezinye izikhathi efomini ke ungathi labia yangaphandle. Kukhona displacement we urethra, futhi amasende ngokuvamile akazange ozakwehlela scrotum. izici Make kanye nobuntu abesifazane - nokwengezwa webele, sibalo ejwayelekile (okhalweni olubanzi, emahlombe ewumngcingo).
• degree Okwesine (uhlobo ikakhulukazi female). Iziguli kuleli qembu abe phenotype zesifazane. Amasende bahlale ngaphakathi komgodi ongaphakathi kwamathumbu. Thuthukisa kwezitho zobulili besifazane, kodwa ukwahluka. Ngokwesibonelo, bakha ingane nge emfushane "abayizimpumputhe" sangasese kanye clitoris ngokuvamile hypertrophied nezobakhumbuza micropenis.
• degree Okwesihlanu, noma uhlobo besifazane. Leli fomu sifo ephelezelwa ukwakheka izimpawu zabesifazane - ingane ibelethwe intombazane. Nokho, kukhona ezinye ngeziphambeko. Ikakhulukazi, iziguli kaningi waphawula kweNdikimba clitoris.

It kuhambisana izimpawu of wamasende feminization syndrome. Ukuveza nge zokugula efanayo akunakwenzeka - umzimba lesiguli akusho ukukhiqiza amaseli ubulili besilisa, kanye nezinhlaka zesifazane azitholakali noma abumane ngokugcwele.

Ngokwezibalo, abantu kokutshelwa ngokukhubazeka kwengane ngokuvamile uhlushwa hernias inguinal, ngenxa ukuhlukumeza ukudlula emasendeni we-canal inguinal. Ngenxa displacement we ukuvulwa kwangaphandle urethra engozini izifo ezihlukahlukene uhlelo urinary (isib, pyelonephritis, urethritis, kanye nezinye izifo ukuvuvukala).

izindlela zokuxilonga

Ukuxilongwa lesi sifo - inqubo eside. Kuhlanganisa iqoqo izinqubo:

• Ukuze uqale, udokotela iqoqa umlando. Ngokuhamba inhlolovo kudingeka sithole ukuthi ngabe ingane anezinye iziyaluyalu zentuthuko emva kokuzalwa noma phakathi nokuthomba. Sikuhlaziye umlando womndeni (ukuthi kuke kwaba khona ngeziphambeko enjalo esuka izihlobo).
• Isinyathelo esibalulekile nokuthi behlolwa, phakathi kwesikhathi lapho Uchwepheshe ungaphawula khona kwezinso ku Ukuklanywa komzimba futhi we zangasese zangaphandle, uhlobo izinwele ukusatshalaliswa, nokunye. D. Labetibambile yokukala ukuphakama nesisindo isiguli. ucwaningo okuningi kuyadingeka ukuze abone comorbidities.
• Ngokuzayo labetibambile karyotyping - inqubo evumela ukucacisa nangohlobo lwezinto Ama-chromosomes e, okuyinto yena, wenza sikwazi ukuthola ucansi isiguli.
• Uma kudingeka, eyenziwa yamangqamuzana ukuhlolwa kwezakhi zofuzo, futhi phakathi naleso sikhathi uzama ukuthola inombolo futhi ezihlukahlukene zofuzo ezilimele.
• Kuyinto luhlolo saziso ngumuntu Urologist - udokotela ofunda netimphawu we zangasese zangaphandle, palpate lo wendlala yabesilisa, njll ...
• Ngemva kokuhlaziya, igazi kuthathwa ukuhlola amazinga ama-hormone.
• Kuyinto ulwazi kanye ultrasound luhlolo. Le nqubo yenza kube lula ukubona anomalies kule nokwakheka kwezitho zokuzala yangaphakathi, testes Undescended ukuhlonza, ukuxilonga comorbidities.
• Ulwazi olunembile kakhulu isakhiwo izibilini ingatholakala ngesikhathi resonance kazibuthe noma tomography ngekhompyutha.

Lesi sifo ka feminization wamasende: ukwelashwa

Ukwelashwa, kulesi simo incike yobudala isiguli kanye degree of insensitivity kuya androgen yokwamukela i-hormone. Ngempela babelwe yokwelapha hormone, okuvumela ukuba kuqedwe ukuntula androgen, ukusiza ukudala ilungelo yesibili ubulili izici, ukuqeda anomalies kungenzeka ekuthuthukiseni.

Kuyaqondakala ukuthi yisinyathelo esibalulekile kakhulu kuyinto kwengqondo - Isiguli kumele njalo uthintane onguchwepheshe. Phela, ngokusho izibalo, i-hermaphroditism kwamanga ngokuvamile kuholela ukuthuthukiswa lwesifo sokucindezeleka. Uma kuguquka sitholakale ngengozi ngesikhathi zokuba umuntu omdala (sikhuluma a insensitivity ephelele testosterone receptor), udokotela angase anqume ukuba angazami uyibike kowesifazane ophila impilo ephelele futhi ezibiza ngokuthi kummeleli ubulili fair.

Lapho ukuhlinzwa kuyadingeka?

Izinkinga eziningi zingaxazululwa ngosizo izinqubo ezikhethekile. Ezigulini ezine-phenotype besifazane ibonisa ukususwa kwezinto testes. Le nqubo kubalulekile ukuvikela hernias ngoba ukuthuthukiswa futhi ukuqhubeka izici kobulili besilisa. Ngaphezu kwalokho, inqubo nokuvimbela nomdlavuza wamasende.

Uma umzimba eba uhlobo besifazane, kwesinye isikhathi kuqakathekile plastic emomozini futhi ngemomozi, okuyinto wenza sikwazi ukuba nobuhlobo bobulili. Surgically ungakwazi ukuqeda offset i-canal urinary.

With ukuthuthukiswa iphethini lesiguli besilisa kwesinye isikhathi kuqakathekile ukuletha Amapayipi kwesidoda ku scrotum. Njengoba amadoda amaningi nge kokutshelwa ngokukhubazeka kwengane uhlushwa gynecomastia, ngokuvamile kwenziwa webele ukuhlinzwa plastic, ezisiza ukubuyisela komzimba zemvelo.

Onalesi sifo iziguli kanye yezinkinga kungenzeka

Lesi sifo ka feminization wamasende (Morris) akuyona usongo ngqo ukuphila. Umzimba imisebenzi evamile ngisho nalapho insensitivity ngokugcwele androgen hormone. Ngemva nesineke yezokwelapha futhi kuhlinzwa angaphila ukuphila ngokugcwele owesifazane, kokuba karyotype besilisa. Kodwa kukhona ingozi yokuba nomdlavuza wamasende ukuthi akazange ozakwehlela scrotum - ezimweni ezinjalo kubalulekile ukuthatha isinyathelo. Nokuvinjelwa umdlavuza wenziwa ukususwa kuhlinzwa we testes kule scrotum (uma isiguli sifuna phenotype besilisa) noma ukususwa ephelele indlala (uma phenotype nabesimame).

Ngokuphathelene nezinye izinkinga kungenzeka ukuthi uhlu yabo ukungabi nakwenzeka sobuhlobo bobulili (malformation of umthondo), uchama (e inqubo ukuthuthukiswa kohlelo urogenital urinary isiteshi eshintshashintshayo). Iziguli kukhona nenzalo kungakhathaliseki phenotype. Ungakhohlwa mayelana nobunzima kwezenhlalo, njengoba akuzona zonke ingane futhi osemusha kungenzeka engaphezulu ukuqonda izici umzimba wayo siqu. Yiqiniso, inkinga sphere ngokobulili, kanye nezifo ze-uhlelo excretory, luqedwe phakathi nokuhlinzwa. Onalesi sifo ngoba iziguli evumayo noma kunjalo.

Zikhona izinyathelo?

Ngeshwa, azikho izindlela ukwazi ukuvimbela isifo ezinjalo. Kodwa njengoba wamasende feminization syndrome yisimo zokugula zofuzo, ingozi yomdlavuza ekuthuthukiseni kungatholakala at the stage of ukuhlela okukhulelwa - abazoba abazali kudingeka ukuthi uhlolwe.

Ezigulini kakade ukuthi nginesifo iziyaluyalu, badinga usizo olufanelekile wezokwelapha kanye nokuhlolwa odokotela njalo, ukwelashwa kwama-hormone.