Iyini i-MRKH syndrome (i-Mayer-Rokitansky-Küster-Hauser Syndrome)? Singaxoxa ngalokhu?

Ingabe indodakazi yakho, noma ingane encane oyaziyo, ayikaqali ukuya esikhathini ngesilinganiso esifanele sobudala? Noma, ingabe ngezinye izikhathi izwa ubuhlungu noma ukungakhululeki lapho izama ukuya ocansini? Namuhla sizokhuluma ngesimo sempilo esikhethekile esingabangela lezi zinto, kodwa esingavamile kakhulu futhi abantu abaningi abake bezwa ngaso. Lokhu kubizwa ngokuthi i-Meyer-Rokitansky-Küster-Hauser syndrome , noma i-"MRKH syndrome" ngamafuphi. Nakuba igama lingase libonakale lide kancane, lesi yisimo sofuzo esingavamile esithinta abesifazane. Ake sixoxe ngaso kalula, ngendlela ongayiqonda.

Iyini ngempela i-MRKH syndrome?

Kalula nje, i-MRKH syndrome yisimo lapho isitho sangasese sowesifazane kanye nesibeletho kungakhuli kahle noma kungakheki ngokugcwele lapho ezalwa. Cabanga ngakho njengendlu eyakhiwe ngamakamelo athile angakheki kahle. Kodwa-ke, ezimweni eziningi, ama-ovari namapayipi e-fallopian abantu abanalesi simo asebenza ngokujwayelekile . Futhi, izitho zangasese zangaphandle, okungukuthi, ingxenye engezansi yesitho sangasese sowesifazane, umgodi wesitho sangasese sowesifazane, i-labia, i-clitoris, kanye nokukhula kwezinwele ezizungezile, kuyinto evamile. I-urethra ayithinteki, ngakho-ke akukho nkinga yokuchama.

Kodwa-ke, kwezinye izinhlobo ze-MRKH syndrome, kungase kube nezinkinga ezithile zokukhula kwezinye izitho zomzimba, njengezinso nomgogodla.

Ngokuvamile, lesi simo sitholakala esemncane, okungukuthi, eneminyaka engu-15-16, lapho ukuya esikhathini kungaqali. Lokhu kungenxa yokuthi isibeletho asikakhuli ngokugcwele, ngakho ukuya esikhathini akwenzeki. Ngezinye izikhathi, lapho uzama ukuya ocansini, isitho sangasese sowesifazane singase sibe buhlungu noma sibe nzima ngoba sifushane futhi sincane.

Ngenxa yokuthi isibeletho asikakhuli ngokugcwele noma asikho, akunakwenzeka ukukhulelwa nokuzala umntwana ngaphandle kosizo lwezokwelapha kulesi simo. Kodwa-ke, uma ama-ovari esebenza kahle futhi kukhiqizwa amaqanda , izinketho ezifana "ne-IVF - ukukhulelwa kwe-in vitro" kanye "nokuzala ingane" zingacatshangelwa. Uma unethemba lokuba nezingane, kubaluleke kakhulu ukukhuluma nodokotela wakho ngakho. Bangakweluleka ngezinketho ezifanele wena.

Amanye amagama amaningana asetshenziswa ku-`(MRKH syndrome)`:

• Ukungabikho kwesibeletho kanye nesitho sangasese sowesifazane (CAUV)
• Ukuzala kwesitho sangasese sowesifazane
• I-Müllerian agenesis
• I-Müllerian aplasia (MA)
• I-Genital renal ear syndrome (GRES)

Ingabe zikhona izinhlobo ze-MRKH syndrome?

Yebo, kunezinhlobo ezimbili eziyinhloko ze-`(MRKH syndrome)`:

• Uhlobo 1:Kulolu hlobo, ama-ovari nama-fallopian tubes asebenza kahle, kodwa ingxenye engenhla yesitho sangasese sowesifazane, umlomo wesibeletho, kanye nesibeletho ivaliwe noma ayikho endaweni yayo. Kodwa-ke, lokhu akuthinti noma yiziphi ezinye izitho emzimbeni.

• Uhlobo 2: Kulolu hlobo, ngaphezu kokuthi ingxenye engenhla yesitho sangasese sowesifazane, umlomo wesibeletho, kanye nesibeletho ivaliwe noma ayikho endaweni yayo, kungase kube nezinkinga ezithile ngamapayipi e-fallopian, ama-ovari, umgogodla, izinso, noma ezinye izitho zomzimba.

Sivame kangakanani lesi simo?

I-MRKH syndrome iyisimo esingavamile kakhulu . Sithinta cishe intombazane eyodwa kwayi-4,500. Ngakho-ke akumangazi ukuthi asikaze sizwe okuningi ngayo.

Ziyini izimpawu ze-MRKH?

Izimpawu ze-MRKH syndrome zingahluka kuye ngohlobo onalo.

Ezimweni eziningi, uphawu oluyinhloko ukungabikho kokuya esikhathini (i-amenorrhea) lapho umuntu eneminyaka engu-16. Lokhu kuyisibonakaliso sokuthi isibeletho noma isitho sangasese sowesifazane asikakhuli kahle. Kodwa-ke, ngenxa yokuthi ama-ovari asebenza kahle, ezinye izimpawu ezihlobene nokuya esikhathini, njengokuqunjelwa kanye nokushintshashintsha kwemizwa, zingavela ngisho nangaphandle kokuphuma kwegazi.

Ngenxa yokuthi unama-chromosome nama-hormone abesifazane, uthola ukukhula okuvamile kocansi, njengokukhula kwamabele nokukhula kwezinwele emakhwapheni nasezindaweni zobulili. Kodwa-ke, ungase uzwe ubuhlungu noma ukungakhululeki lapho uqala ukuzama ukwenza ucansi lwesitho sangasese sowesifazane. Lokhu kungenxa yokuthi isitho sakho sangasese sowesifazane sincane, sincane, futhi sifushane kunokujwayelekile.

Uma une-Type 2 MRKH, ungase ube nezimpawu ezengeziwe ezifana nalezi:

• Izinkinga zezinso, noma ukwehluleka kwenso eyodwa noma zombili.
• Izinkinga ngamathambo omgogodla noma ezinye izinkinga zesistimu yamathambo.
• Ukukhubazeka kokuzwa.
• Ezinye izinkinga zenhliziyo.

Yini ebangela i-MRKH?

Abacwaningi abakayazi imbangela eqondile ye-MRKH syndrome. Kodwa-ke, bayazi ukuthi ihilela izinkinga ezithile ngama-gene nama-chromosome. Kodwa-ke, abakwazanga ukuyifaka ku-gene eyodwa ethize. Into ebalulekile ukuthi akuyona into eyenziwe noma engenziwanga ngumama ngesikhathi sokukhulelwa . Ngakho-ke ungakhathazeki ngayo.

Uhlelo lokuzala lowesifazane luqala ukukhula emasontweni ambalwa okuqala okukhulelwa. Amapayipi e-fallopian, isibeletho, umlomo wesibeletho, kanye nengxenye engenhla yesitho sangasese sowesifazane kwakheka ngento ebizwa ngokuthi amapayipi e-Müllerian . Kubantu abane-MRKH syndrome, lawa mapayipi e-Müllerian awakhuli ngokugcwele. Akukaziwa okwamanje ukuthi kungani lokhu kwenzeka kwabanye abantu. Kodwa-ke, ngenxa yokuthi ama-ovari akhula ngokwehlukana nohlelo lonke lokuzala, ama-ovari ngokuvamile awathinteki.

Wazi kanjani ukuthi unesiqiniseko sokuthi une-MRKH?

Odokotela bavame ukuthola i-MRKH syndrome lapho intombazane encane yehluleka ukuya esikhathini okokuqala.

Isinyathelo sokuqala ekuxilongeni lesi simo ukuhlolwa ngokomzimba . Udokotela wakho uzofaka umunwe ogqokwe igilavu ​​​​esithweni sakho sangasese sowesifazane bese elinganisa ukujula nobubanzi baso. Lokhu kuhlolwa kungabona i-MRKH ngoba isitho sangasese sowesifazane sifushane. Ngemuva kwalokho, ungase ucelwe ukuthi wenze izivivinyo zesithombe, njenge-ultrasound scan noma i-MRI (magnetic resonance imaging) scan, ukuze ubone ukuthi isibeletho sakho, amashubhu e-fallopian, izinso, noma ezinye izitho zomzimba zithintekile yini. Ngezinye izikhathi, ukuhlolwa kwegazi kungenziwa ukuhlola amazinga akho e-hormone.

Yiziphi izindlela zokwelapha i-MRKH?

Ukwelashwa kwe-MRKH syndrome kuyahlukahluka kuye ngemigomo yakho kanye nezimpawu. Kunezindlela zokwelapha ngokuhlinzwa kanye nezindlela zokwelapha ezingahlinzwa. Ezinye zalezi zindlela zifaka phakathi i-vaginoplasty, ukuvuleka kwesitho sangasese sowesifazane, kanye nokufakelwa kwesibeletho.

Ukwanda kwesitho sangasese sowesifazane

Enye indlela odokotela abayisebenzisayo ukwelula isitho sangasese sowesifazane ukusebenzisa amadivayisi abizwa ngokuthi ama-vaginal dilators . Lawa enziwe ngepulasitiki noma i-silicone futhi afika ngobude nobubanzi obuhlukahlukene. Lawa madivayisi afana ne-tube asiza ekwandiseni nasekunwebeni isitho sangasese sowesifazane kusuka ngaphakathi. Udokotela wakho uzochaza indlela engcono kakhulu yokusebenzisa i-vaginal dilator ngesimo sakho.

I-Vaginoplasty (ukuvuselelwa kabusha kwesitho sangasese sowesifazane)

I-Vaginoplasty inqubo yokuhlinzwa yokudala isitho sangasese sowesifazane . Odokotela abahlinzayo banezindlela eziningana zokwenza le nqubo. Ngokuvamile, benza imbobo bese beyimboza ngezicubu ezithathwe kwenye ingxenye yomzimba wakho. Lokhu kukuvumela ukuthi ube nocansi olujwayelekile.

Ukufakelwa kwesibeletho

Ukufakelwa kwesibeletho kunganikeza owesifazane onesifo se-MRKH ithuba lokuthwala umntwana. Lokhu kuwukuhlinzwa okukhulu. Kuhilela ukufakelwa kwesibeletho kusuka kumuntu onikelayo kuya kowesifazane ongenaso. Lokhu kunikeza abantu abane-MRKH ithuba lokuthwala nokubeletha ingane yabo. Ukufakelwa kwesibeletho akukakwenziwa kabanzi kuzo zonke izingxenye zomhlaba, kodwa kungaba ukwelashwa okuphumelelayo kakhulu esikhathini esizayo.

Ingabe lesi simo singavinjelwa?

Cha, ayikho indlela yokuvimbela i-MRKH syndrome . Ingenzeka kubantu abangakaze babe nayo ngaphambili, noma ingaba yizakhi zofuzo. Njengoba kushiwo ngaphambili, ayibangelwa yizakhi zofuzo ezithile.

Ingabe abantu abane-MRKH bangaba nomntwana?

Yebo, abantu abane-`(MRKH syndrome)` bangaba nomntwana!Uma amaqanda akho esebenza, amaqanda akho angahlanganiswa nesidoda bese evundiswa kusetshenziswa ukuvundiswa kwe-in vitro (IVF). Umbungu ube usudluliselwa kumthwali wokukhulelwa/kumama ozalelwe esikhundleni sowesifazane . Lokhu kusho ukuthi uzoba nomntwana onezakhi zofuzo zakho, okhula esibelethweni somunye wesifazane onempilo.

Yiziphi ezinye izinkinga zempilo ezingabangelwa yi-MRKH?

Sesixoxile ngaphambilini ukuthi abantu abane-"Type 2" MRKH bangaba nezinkinga nezinye izitho zomzimba, njengezinso, umgogodla, noma inhliziyo. Ngaphezu kwalokho, ezinye izinkinga zempilo ezivame kubantu abane-"Type 2" MRKH yilezi:

• Izinkinga ezihlobene namathambo omgogodla.
• I-Scoliosis.
• Ukukhubazeka kwezinso, njengokuhlanganiswa kwezinso noma ukungabikho kwenso eyodwa noma zombili (i-renal agenesis).
• Ingozi eyengeziwe yamatshe ezinso, izifo zomgudu womchamo, kanye nokuvaleka komgudu womchamo.
• Ukukhubazeka kokuzwa.
• Isifo senhliziyo.

Izinkinga zesimiso sokuzala zivame kakhulu kubantu abane-MRKH. Ngaphezu kokungakwazi ukukhulelwa noma ukuba nomntwana wabo, labo abanesibeletho esingakakhuli kahle bangaba nezimo ezifana ne-endometriosis.

Ngakho konke lokhu, kuyinto evamile ukuzwa ukucindezeleka ngokomzwelo . Udokotela wakho angase akuphakamise ukuthi ujoyine iqembu lokusekela noma ufune insizakalo yokwelulekwa kwengqondo, njengokwelashwa kokuziphatha kwengqondo (i-CBT), ukuze ikusize ubhekane nesimo. Lezi zinto zingaba usizo kakhulu.

Akumangalisi ukuthi ukuthola ukuthi unesifo i-Mayer-Rokitansky-Küster-Hauser (MRKH) kungaba yinto eshaqisayo kakhulu. Uma uzalwa unesibeletho noma isitho sangasese sowesifazane esingakakhuli kahle, ungase ungazazi uze ube semncane futhi usanda kuqala ukuya esikhathini. Nakuba kungesona isimo esisongela ukuphila, singabangela ukucindezeleka okukhulu, sithinte ikhono lowesifazane lokukhulelwa nokwenza ucansi. Kwezinye izimo, singathinta ezinye izitho futhi sandise ingozi yezifo ezithile.

Izinto ezibaluleke kakhulu okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Kulungile, manje usuqonda kangcono lokho ebesikhuluma ngakho, i-"MRKH syndrome." Into ebaluleke kakhulu okufanele uyikhumbule ukuthi awuwedwa . Kukhona abanye abantu emhlabeni abaphila nalezi zimo.

• Uma unezimpawu ezinjengalezi, kubaluleke kakhulu ukubona udokotela omuhle bese uthola ukuxilongwa okunembile .
• Kukhona ukwelashwa okutholakalayo. Khuluma nodokotela wakho bese ukhetha ukwelashwa okukulungele.
• Ungalilahli ithemba ngezingane. Ngobuchwepheshe obufana ne-'(IVF)' kanye ne-'(surrogacy)', kunamathuba okwenza lelo phupho lifezeke.
• Nakekela impilo yakho yengqondo futhi.Uma kudingeka, funa ukwelulekwa bese ujoyina amaqembu okusekelana nabantu abake baba nokuhlangenwe nakho okufanayo. Lokhu kuzokunika amandla amaningi.
• Akulona iphutha lakho leli. Lesi yisimo semvelo esingenzeka kunoma ubani.

Udokotela wakho, kanye nethimba lochwepheshe, uzokunikeza ukwesekwa nesiqondiso osidingayo ukuze uqhubekele phambili nalesi sifo (i-MRKH syndrome). Bathembe. Uma uneminye imibuzo mayelana nalokhu, ungesabi ukubabuza. Yiba nempilo enhle!

I- MRKH syndrome, i-Mayer-Rokitansky-Küster-Hauser Syndrome, i-vaginal agenesis, i-uterine agenesis, impilo yabesifazane, impilo yokuzala, i-amenorrhea