Kuyini - a syndrome Rokitansky-Otto Küstner? Syndrome Rokitansky-Otto Küstner: izimbangela, izimpawu, ukwelashwa

Surrogacy evumela izingane omama ngenxa yezizathu zempilo hhayi ukucabanga kwengane bebodwa. Inani labo, ngokuvumelana izibalo, kukhuphuke iminyaka ngeminyaka. Esinye sezizathu zalokho kudluliswa kwesinqumo ukuthola usizo kukamama wesivumelwano kuyinto Rokitansky-Otto Küstner syndrome. Izimbangela lesi sifo, izimpawu kanye nezindlela eziyisisekelo ukuphathwa uzobe okuxoxwe ngazo kulesi sihloko. Uyofunda ukuthi owesifazane yilesi sifo babe nabantwana.

zokugula isici

Syndrome Rokitansky-Otto Küstner-Meyer-Hauser - ongavamile isifo izifo zabesifazane. unamagama ososayensi obuze incazelo eningiliziwe zokugula egameni layo. Futhi kule izincwadi zezokwelapha ezisekelwe ekwazisweni kukhona omqondofana zesifo: atresia lwesitho sangasese sowesifazane, aplasia kwesibeletho. Noma kunjalo, azibonisi nakho konke ukwephulwa emzimbeni, okuvame ukuhlotshiswa ngayo lesi sifo. Ngaphezu kwalokho, atresia lwesitho sangasese sowesifazane kungenzeka ngenxa yisihlungu noma awe ukuhlinza. Ngokuvamile, igama eligcwele lesi sifo ukunciphisa amagama ezimbili zokuqala ososayensi noma elithi "MRKH syndrome."

I zokugula libhekene ukungabi okuphelele noma okuyingxenye kwesibeletho, kanye 2/3 sangasese. Kulokhu, kukhona ukuthuthukiswa egcwele amaqanda nezitho zangasese. Futhi egcinwe izici ngokobulili yesibili (umzimba izinwele ngohlobo zesifazane, esifubeni). Rokitansky-Otto Küstner sifo sitholakale akuvamile (Kwesinye isenzakalo ngasinye inkulungwane 5. Girls izinsana).

Imininingwane zomlando

Letinchazelo lokuqala usuku isifo emuva ngonyaka 1829. udokotela German Carl Mayer kuqashelwe ukuthi aplasia lwesitho sangasese sowesifazane uvame ukuhambisana ezihlukahlukene kwezinso zentuthuko. Ngemva nje kancane kwalokho, ngo-1838, i-sezifo kusuka Austria Karl von Rokitansky incazelo syndrome wanezela. Wanquma ukuthi uma lesi sifo futhi kwesibeletho ngingekho, amaqanda kodwa ukugcina ikhono ukusebenza ngokugcwele. Ngo-1910, udokotela German-German Kyustner wafingqa ulwazi olutholakala kwi isifo, unezela esakubona siqu. I wezifo zabesifazane echazwe izibonelo inhlanganisela njalo nge aplasia lesizalo kombungu ukuthuthukiswa izinso. Ngo-1961, uProfesa Zhorzh Hauzer baseSwitzerland wasikisela ukuthi igama elithi "u-Otto Küstner Rokitansky-Mayer-syndrome." Usosayensi eshicilelwe imibono yakhe eminingi iziguli nale Ihlahlubo, okuyinto yabhekisela insensitivity nama-hormone abesilisa phakathi iziguli. Ngo-1977, Houser eyokuqala incazelo okufana atypical zokugula. Banikezwe umnikelo enkulu ososayensi, lesi sifo laqanjwa ngokuthi i-ukuthi bangabavumbululi amagama amane.

Izimbangela isifo

Ngisho e -embryonic period fetus ukukhubazeka labhubhisa yokwakheka agcwele Otto Küstner-Rokitansky syndrome. Izithombe izingane nale Ihlahlubo emva kokuzalwa musa zihlukile ontanga yabo inempilo. Ngokusho theory Muller sika, i-fetus ubulili izitho ukuthuthukisa enyangeni yesibili ngemva kokubamba. Umthelela yezici omubi isidumbu abakhulelwe ngalesi sikhathi kungabangela izifo ezihlukahlukene izingane. On okuvela a syndrome sithinta zezinzwa izicubu dysplasia umbungu lapho ukuphazamiseka yokulandelana kwayo ngokohlu kwenzeka. izinguquko sokugembula kuholele ukukhula kwezitho onesici, loluvetwa okuyinto Otto Küstner-Rokitansky syndrome.

Yiziphi izici nomthelela nesenzakalo lesi sifo ingane ngesikhathi sokukhulelwa?

1. toxicosis eqinile.
2. HIV komama.
3. Ngengozi isisu.
4. Ukuthatha imithi ethile (ezifana corticosteroids).
5. Ukweqisa ophuzweni oludakayo.

Lesi yisifo hhayi labizwa ngokuthi izifo sitholwa njengefa. Nokho, umkhuba emtholampilo kukhona izimo lapho zokugula kutholakala ukuthi uphethwe yini abesifazane abaningana emndenini owodwa.

ukubonakaliswa Clinical lesi sifo

Otto Küstner-Rokitansky syndrome iyaqala ukubonisa ukuthomba lapho ukuthomba umzimba. Amantombazane kancane kancane babanjwa amabele, ukushintsha sibalo, kukhona umzimba izinwele ezindaweni ezibucayi. Njengoba kuthiwa izici ngokobulili yesibili musa ukuvumela esolwa izinkinga zezempilo. Amantombazane amaningi azizwa izinhlungu gqwa kule aphansi endaweni esiswini, ukusinda ngemuva aphansi. Nokho, ukungabi zokwehlukaniswa kuholela ukudinga udokotela wezifo zabesifazane. Esinye isizathu ukuba kudokotela wezifo kungaba inkinga ye-imvelo obuseduze. imizamo Akuphumelelanga sobuhlobo bobulili ngokuvamile kuholele ukuhlukumezeka kuya perineum noma ukudabuka lwesinye. Ukubonakaliswa emizimbeni nokungathuthuki singasebenza njengesisekelo esiqinile sokufinyelela kwesibeletho esingesihle nge normal fallopian amashubhu. Cishe u-40% ukujabulisa ubulili MRKH syndrome kuhlangene kombungu kohlelo urinary.

Ukwahlukaniswa izifo

Njengezinye anomalies eziningi ukuthuthukiswa embryonic, Rokitansky-Otto Küstner syndrome has ngamazinga ahlukene zaba namandla kakhulu. Amandla ngamunye wabo kunqunywa kobukhulu kwezinso fetus. Kukhona izinhlobo ezintathu zesifo:

1. Ejwayelekile MRKH syndrome (64% azo zonke izimo). It libhekene aplasia kwesibeletho futhi esithweni sakho sangasese.
2. Atypical MRKH syndrome (24% azo zonke izimo). Kulesi sifo, amantombazane ulahlekile kwesibeletho futhi sangasese, kukhona ukungasebenzi ezikhiqiza amaqanda.
3. MURCS-inhlangano (12% azo zonke izimo). Lona fomu olunzima kunalo lonke ake lesi sifo, okuyinto libhekene iziyaluyalu engenhla nangezimpendulo is kwahambisana by an ukuthuthukiswa okungavamile lwamathambo.

Ifomu lesi sifo inquma isu zokwelapha kanye kusiza odokotela ongenza isibikezelo zokululama.

izindlela ukuxilongwa

Yini ukuhlolwa kuyadingeka ukuqinisekisa syndrome Rokitansky-Otto Küstner? Ngezimpawu zesifo ungeyena umbandela kuphela uphethwe. luhlolo nesineke iqala kuhlolwe ngamehlo. Ngo-75% izimo, akazivezi yikuphi ukuphulwa, ngoba izici yesibili zivumelana kwezinkambiso. Khona-ke, isheke esitulweni izifo zabesifazane. Amantombazane kanye uphethwe ihlo libonakala eyenziwa esebenzisa abathola. Ukuze ejwayelekile iDemo syndrome emomozini (kufika ku 2 cm), engesiyo ekupheleni isiteshi wesibeletho. Enye zokuxilonga kuyinto luhlolo rectal, phakathi kwesikhathi lapho udokotela Uyaphenya tube fallopian kanye lesizalo ingaphakathi. Le ndlela yenza kube lula ukuhlola degree of kumiswa izitho.

nesilinganiso njalo lokushisa basal kusiza ukucacisa isigaba umjikelezo wokuya esikhathini uwubufakazi ukuqinisekisa ukusebenza ngokugcwele ezikhiqiza amaqanda. Ngaphezu kwalokho, udokotela ekunika ukuhlolwa kwegazi-hormone, i-MRI kanye ultrasound okhalo. Ezimweni sina ngokukhethekile kungadinga uphethwe laparoscopic.

Izindlela yokwelashwa

Yinye kuphela indlela ukwelapha Rokitansky-Otto Küstner syndrome - plastic obuseduze. Umsebenzi kumiswa sangasese ngokuthi colpopoiesis ukuba ukunakekelwa odokotela wakhe aye ayisebenzisa ekhulwini XIX. Ekuqaleni, ukusebenza esetshenziswa iphathe abesifazane ngenhlonipho walondoloza kwesibeletho imisebenzi. Kulezi iziguli ngegazi ayivelanga umzimba, futhi kancane kancane zanqwabelana komgodi ongaphakathi kwamathumbu. Ngenxa yalokho, baye kwatholakala ukuthi une-izinqubo neoplastic, okuyinto ayehambisana nezinhlungu ezinzima kanti yezinkinga ezihlukahlukene.

Okwamanje ubambe colpopoiesis ngezindlela ezimbili:

1. Ukunwetshwa sangasese ngosizo i ibhaluni inflatable. Ukusebenza kuthatha isikhathi eside. nokuqaliswa kwalo kungenzeka kuphela uma ubude lwesitho sangasese sowesifazane akuyona esingaphansi 4 cm.
2. Ukwakhiwa ingxenye neovaginy kapopopo sigmoid noma isikhumba esiswini. ubuchwepheshe besimanje ukuvumela ukulungisa izici ngaphandle sebeziqhumbuza besisu - laparoscopically. Odokotela bavame uthanda le ndlela, kumiswa i emomozini yokufakelwa.

Umnyaka ekahle colpopoiesis ziyinkathi kusukela engu-16 kuya ku-21. Kulokhu, lo nokuvuthwa yokugcina umzimba. Ngenhloso yokwenza plastic sowesifazane sangasese - unike amandla abesifazane ukuba ngijabulele ukuphila ubulili egcwele. Ukuvimbela imperforate zayo kufanele uhlukane nale ucansi njalo, njalo ukudlula bougienage inqubo.

Izinkinga emva kokuhlinzwa

Yiziphi lezo zinkinga ngemuva kokuhlinzwa ungase uhlangane iziguli kwathiwa unesifo "Rokitansky-Otto Küstner syndrome"? Ukwelashwa sifo ngezinye izikhathi zihambisana yilutho olubi. Colpopoiesis - lena kokuhlinzwa. Njenganoma iyiphi enye indlela ukuhlinzwa, kungenzeka ukuthuthukiswa yezinkinga. Phakathi kwabo, lo ezivamile sepsis futhi peritonitis. Futhi, iziguli eziningi ngemva imithi oyinikiwe, odokotela kutholakala ukuthi uphethwe i-fusion neovagina izindonga. Lokhu zokugula eba ngenxa yokuntuleka ukuphila ocansini njalo. Ngakho-ke, odokotela batusa kakhulu ukuthi abesifazane ukuba bangakushayi indiva ocansini.

Isibikezelo ngemva yokutakula

Izinguquko emzimbeni ezenzeka abesifazane kwathiwa unesifo "Rokitansky-Otto Küstner syndrome" ayikuvumeli ukuba afeze umsebenzi zokuzala ngendlela engokwemvelo. Nokho, izindlela zesimanje of IVF nosizo surrogacy iziguli enjalo unezingane begazi. muva Ososayensi baye imemezele imiphumela emihle donor kwesibeletho ukufakelwa imisebenzi. Ngemva kwesikhashana, mhlawumbe, ukufakelwa kwezitho zomzimba kuzosiza abesifazane nalesi sifo esikhohlisayo bebodwa bazale abantwana.

Okungavundile kanye syndrome Rokitansky-Mayer-Otto Küstner

Can Nginezingane nalesi sifo? Lo mbuzo uphakama kusukela besifazane abaningi ababekhuphuke abhekene egunjini lokuhlolwa kwezifo. Ngokuhamba kwesikhathi, yilowo nalowo kubo ofuna ukuzama ku indima kamama. Iningi labesifazane nale syndrome ungethele ingane. Uma amaqanda kuthiwa usebenza kahle, ngakho ochwepheshe basikisela iziguli babe Sokuzivikela ezinsizeni kukamama wesivumelwano. Kulokhu ingane izalwe?

Ngo esigabeni sokuqala uhlelo umama ngokwesivumelwano kanye owesifazane ogulayo ezinqunyiwe izidakamizwa hormone. Ukusetshenziswa kwezidakamizwa ngaphakathi kwenkathi ethile ikuvumela ukuvumelanisa imijikelezo kokuya kwawo esikhathini. Khona-ke iqanda syndrome MRKH sowesifazane eyayenziwe inseminated nesidoda somyeni wakhe. Ezinsukwini ezimbalwa zabo 'ziye zaba "e konemimoya ukuthi ulingisa izakhiwo amashubhu fallopian. Ngemva kwezinsuku ezintathu, ochwepheshe sebeqala oyi-yenzeka nokulungiselela: ukunquma ocansini umbungu, khona chromosomal zikhubazeke futhi zibe nezifo sitholwa njengefa. Ngosuku lwesihlanu owasungula i-embryo ibizwa sidluliselwe esibelethweni kukamama wesivumelwano. Kwezinye izimo, podsazhivanie double, isibonelo, ngosuku lwesithathu futhi yesihlanu.

Inombolo zemibungu idluliselwe ingase yehluke kuye ngesistimu izinga izinhlayiya eziyisisekelo zofuzo, iminyaka yobudala isiguli. Ngesicelo owesifazane, iqanda ubuningi ibhekane inqubo cryopreservation. Ngenxa yalokhu nje ebamba isisu ezweni izinyanga 9 kukhona ingane ephile saka. Nokho, le nqubo has kokubili abasekeli kanye nezitha.

Eceleni kufanele ucabangele surrogacy. Emitholampilo eminingi ezikhethekile kukhona base okubizwa ngokuthi zabesifazane, abazimisele ukunikeza inkonzo efanayo. Ngokuvamile kuba abasha, amantombazane enempilo. Esinye sezizathu i asesebenzisa umtholampilo esinjalo syndrome Rokitansky-Otto Küstner-Meyer-Hauser. Izithombe esizayo omama ngokwesivumelwano futhi zingatholakala kuleli egciniwe. Yonke inqubo, kusukela ukukhethwa ngisabone biomamy nokukhulelwa ophela, esilawula abengamele. Ngakho kungcono ukuxhumana ochwepheshe, kunokuba bahlanganyele yokusesha self. Kuphela ngale ndlela ingaqhubeka beqiniseka ngekusasa lempilo yesintu ingane yakho.

Kufinyeta

Syndrome Rokitansky-Mayer-Otto Küstner - kuyinto isifo esidluliselwa ngofuzo libhekene ukungabi noma hypoplasia kwesibeletho futhi esithweni sakho sangasese. Amantombazane amaningi ngaphambi ukuthomba alazi ngezinkinga zempilo. Amenorrhea ebangela ukuba ubone udokotela wezifo zabesifazane. Kwakungaleso reception ngesikhathi uchwepheshe beqala bati khona syndrome ezinjalo. Ngakolunye uhlangothi, kuba ejwayelekile ukuba maldevelopment kwesibeletho futhi sangasese, futhi ngakolunye - khona yesibili ubulili izici. Namuhla, kunamabandla amaningana zokwelapha izifo. Bonke njalo ngcono futhi kulekelelwa. Yokwelapha lesi sifo ukuba asuse isici by amapulasitiki obuseduze. Ngenxa yalokho, isiguli balahle izinkinga buyinkimbinkimbi, ukujabulela ukuphila futhi ngisho unezingane. Ngokuphathelene iphuzu yokugcina, idinga usizo kukamama wesivumelwano.