Yintoni na - a syndrome Rokitansky-Otto Küstner? Syndrome Rokitansky-Otto Küstner: oonobangela, iimpawu, unyango

Surrogacy ivumela abantwana nabafazi ngenxa yezizathu zempilo akakwazi ukukhawula umntwana ngokwabo. Inani labo, ngokutsho-manani, lanyuka ukusuka komnye unyaka ukuya komnye. Esinye sezizathu ukuba isibheno iinkonzo umama ngokwesivumelwano yi Rokitansky-Otto Küstner syndrome. Ubangelwa sifo, iimpawu kunye iindlela ezisisiseko unyango eza kuxutyushwa kweli nqaku. Kwakhona uya kufunda ukuba umfazi lo syndrome babe nabantwana.

zokugula Uphawu

Syndrome Rokitansky-Otto Küstner-Meyer-Hauser - isifo esinqabileyo yamanina. iqulathe amagama iinzululwazi thaca inkcazelo eneenkcukacha malunga leengxaki egameni layo. Kwakhona kule ncwadi zonyango kukho ntonye esi sifo: atresia lobufazi, aplasia ye isibeleko. Noko ke, musa mayibonise zonke ukunyhashwa emzimbeni, lifana onaso. Ukongezelela, atresia lobufazi kunokwenzeka ngenxa yokukrala okanye utyando. Ngokuqhelekileyo, igama ngokupheleleyo esi sifo ukunciphisa amagama zokuqala ezimbini izazinzulu okanye elithi "MRKH syndrome".

Le ngezifo luphawulwa lokungabikho olupheleleyo okanye olungaphelelanga isibeleko, kwakunye 2/3 kwinxenye yelungu lobufazi. Kulo mzekelo, kukho uphuhliso ngokupheleleyo zamaqanda zangasese. Kwakhona agcinwe iimpawu zasesekondari ngokwesondo (uboya umzimba kwi kuhlobo nemazi, esifubeni). Rokitansky-Otto Küstner syndrome banesifo kunqabile (icala elinye ngalinye amawaka 5. Girls kubelekwa).

ulwazi zembali

Iinkcazelo lokuqala umhla sifo emva konyaka-1829. ugqirha German Carl Mayer waphawula ukuba aplasia kusisi idla sikhatshwe ezahlukeneyo ukungaqheleki yophuhliso. Kungekudala emva koko, ngo-1838, kukho sezifo evela Austria Karl von Rokitansky syndrome inkcazelo engakumbi. Wagqiba ukuba, ukuba esi sifo kwakhona wesibeleko ndingekho, amaqanda kodwa ukugcina ikhono ukusebenza ngokupheleleyo. Ngowe-1910, ugqirha waseJamani German Kyustner zishwankathelwe ulwazi luyafumaneka sifo, ukudibanisa Iziluleko zakhe. Le ugqirha wachaza imizekelo indibaniselwano rhoqo kunye aplasia zesibeleko malformations uphuhliso zezintso. Ngowe-1961, uNjingalwazi Zhorzh Hauzer ukusuka eSwitzerland lubonisa ukuba igama elithi "Otto Küstner Rokitansky-Mayer-syndrome". Scientist wapapasha Iziluleko zakhe ezininzi izigulane nale sifo, leyo wakhankanya ukuba novelwano hormone eyindoda phakathi izigulane. Ngowe-1977, kuqala Houser thaca inkcazelo uhlobo belungafani ngezifo. Ngenxa igalelo elikhulu wenzululwazi, esi sifo kuthiwa ngenxa discoverers lwamagama ezine.

Izinto ezibangela izifo

Nkqu kwixesha osandul foetal iziphene, babeka yokwakheka okuluphawu Otto Küstner-Rokitansky syndrome. Iifoto kids nalo unesifo emva kokuzalwa musa ayahluka kulawo noontanga babo usempilweni. Ngokutsho kwithiyori Muller kaThixo, amalungu sex olungekazalwa ngexesha ngenyanga yesibini emva kokukhulelwa. Nemiphumela yeemeko elibi emzimbeni okhulelwe ngalo eli xesha kungabangela zizifo ezahlukahlukeneyo ebantwaneni. Kwisehlo a syndrome kwakhona kuchaphazela nokuqina isicwili dysplasia Umbungu apho ukuphazamiseka lwesakhiwo yayo kwenzeka. utshintsho yezifo kukhokelela kuphuhliso amalungu enesiphako, omnye abonisa owona Otto Küstner-Rokitansky syndrome.

Ziziphi izinto eziphembelela kokuvela esi sifo kusana ngexesha ukhulelwe?

1. toxicosis olomeleleyo.
2. HIV unina.
3. Ingozi isisu.
4. Imithi ezithile (ezifana corticosteroids).
5. kakubi utywala.

Lesi sifo abangafakwanga kuhlelo lwee-izifo njengelifa. Noko ke, xa kusenziwa clinical kukho iimeko apho ngezifo bene abafazi eziliqela yentsapho efanayo.

ezibonisa kwezonyango sifo

Otto Küstner-Rokitansky syndrome oluqalayo ukuba wabonakalaliswa ufikisa xa kukho ukufikisa ephilayo. Amantombazana ngcembe elirhangqiweyo amabele, ukutshintsha nani, kukho unwele umzimba kwiindawo olusondeleyo. Luvakaliswe iimpawu zesini zasesekondari musa avumela okukrokrelwayo iingxaki zempilo. Amantombazana amaninzi baziva iintlungu ngamaxesha kwindawo osesiswini, ubunzima emva asezantsi. Noko ke, ukungabikho sexesheni kukhokelela kufuna uncedo ugqirha. Esinye isizathu sokuba kutyelelo ukuya ingcali inokuba ingxaki zohlobo osenyongweni. imizamo yokuba neentlobano zesini zisoloko zibangela trauma ukuya perineum okanye komsipha lwesinye. Ngokubonakala lwemizimba wokungaphuhliswa unako ukukhonza njenge wesibeleko oludala kunye tubes engaqhelekanga. Malunga nama-40% omboniso syndrome sex MRKH sikubandakanye malformations inkqubo womchamo.

nokubekwa kwezifo

Njengabanye mgaqweni ezininzi zophuhliso osandul, Rokitansky-Otto Küstner syndrome kunamanqanaba ezahlukileyo ubungnqongqo. Ubunzulu ngamnye kubo emiselwe ubuzaza ukungaqheleki Palsy. Kukho iintlobo ezintathu sifo:

1. Gumbini MRKH syndrome (64% yazo zonke iimeko). Lubonakala yi aplasia ye wesibeleko kunye lobufazi.
2. Nedlac MRKH syndrome (24% yazo zonke iimeko). Kulo sifo, amantombazana ezingekhoyo isibeleko kunye lobufazi, kukho kwekhono ye zamaqanda.
3. MURCS-umbutho (12% yazo zonke iimeko). Olu hlobo ngokuqatha onaso, esibonakala ngezidubedube ezidweliswe ngasentla kwaye zincediswe linyathelo okungaqhelekanga ngamathambo.

Ifomu kwesi sifo limisela icebo zonyango kwaye inceda oogqirha benza kwingqikelelo achache.

iindlela isifo

Yiyiphi ukuhlolwa kuyimfuneko ukungqinisisa syndrome Rokitansky-Otto Küstner? Iimpawu zezifo hayi kwiimpawu kuphela moshilongo. iimviwo nomonde iqala uhlolo olubonakalayo. Ngowe-75% amatyala, oko akuthethi phendla naziphi na ukunyhashwa, kuba iimpawu zasesekondari bahambelana nemigangatho. Emva koko, itsheki esitulweni yamanina. Amantombazana kunye uxilongo nenwebu ebonisa ubuntombi nto yenziwa ngokusebenzisa wakuqonda. Kuba yoqobo syndrome ikuku elifutshane (cm ukuya kwi-2), nto leyo ingeyo mpela ijelo wesibeleko. Enye indlela diagnostic i uviwo efakwa, apho ugqirha Uphanda tube Imibhobho kunye zesibeleko kwisisu. Le ndlela yenza kube lula ukuba ukuvavanya izinga ukubunjwa amalungu.

ukujongwa rhoqo lobushushu carcinomas inceda amisele sigaba somjikelo sexesheni ukuqinisekisa ukusebenza ngokupheleleyo zamaqanda. Ukongeza, ugqirha umisela iimvavanyo igazi emzimbeni, MRI ultrasound zangasese. Kwiimeko kuyingozi kakhulu zingafuna diagnosis laparoscopy.

Iindlela zonyango

Inye kuphela indlela kunyanga Rokitansky-Otto Küstner syndrome - yeplastiki banye. Ukusebenza ukuyilwa lobufazi ekuthiwa colpopoiesis ukuba amagqirha Lilinyamekela asebenzise kwinkulungwane XIX. Ekuqaleni, umsebenzi asetyenziswa ukunyanga abafazi kunye nemisebenzi esibelekweni igcinwe. Kwezi izigulane igazi zange ifike ivela emzimbeni, yaye ngokuthe ngcembe amaninzi kwisisu. Ngenxa yoko, ukuba sele kufunyaniswe iinkqubo neoplastic, leyo bephelekwa ubuhlungu obukhulu iingxaki ezahlukeneyo.

Okwangoku ubambe colpopoiesis ngeendlela ezimbini:

1. Ukwandiswa kwinxenye yelungu lobufazi kunye uncedo ibhaloni sawa. Olu tyando kuthatha ixesha elide. Ukuphunyezwa kwawo kunokwenzeka kuphela xa ubude kusisi akukho ngaphantsi kwe-4 cm.
2. Formation isabelo neovaginy ye ikholoni sigmoid okanye isikhumba esiswini. ubuchwepheshe bale mihla ukuvumela ukuba alungise iziphene ngaphandle ukusikwa esiswini - laparoscopically. Oogqirha bathanda le ndlela, ukuyilwa i ikuku ngumntu.

Ixesha elifanelekileyo ukuze colpopoiesis na ixesha ukususela kwiminyaka eli-16 ukuya kuma-21. Eli xesha, nokuvuthwa yokugqibela eziphilayo. Injongo kuphela kweplastiki langasese - ukuba abasetyhini ukuba ukunandipha ubomi sex ngokupheleleyo. Ukuthintela imperforate wayo kufuneka ungamshiyi ngesondo rhoqo, hlale kudlula bougienage nkqubo.

Iingxaki emva kotyando

Zeziphi iingxaki emva kotyando nazo izigulane unesifo "Rokitansky-Otto Küstner syndrome"? Unyango lwe esi sifo ngamanye amaxesha kunye naziphi na iziphumo ezingalunganga. Colpopoiesis - oku ngumsebenzi kakhulu. Njengazo naziphi na ezinye uhlobo kotyando, kunokwenzeka kuphuhliso iingxaki. Phakathi kwabo, aziwayo uninzi nokudumba kunye peritonitis. Kwakhona, izigulane ezininzi emva kwikhondo unyango, oogqirha kwafunyaniswa ukuba fusion iindonga neovagina. Le ngezifo eyenzeke ngenxa yokungabikho ubomi babantu bokwabelana ngesondo rhoqo. Ngoko ke, oogqirha sicebisa ngamandla ukuba abasetyhini ukuba bangawutyesheli ngesondo.

Sase- emva alulame

Utshintsho emzimbeni ukuba kwenzeka kwabasetyhini unesifo "Rokitansky-Otto Küstner syndrome" akavumi ukwenza umsebenzi okuzala ngendlela yemvelo. Noko ke, iindlela zale mihla IVF kunye noncedo surrogacy ezinjalo izigulane kufuneka abantwana begazi. kutshanje Izazinzulu ziye wayebhengeza iziphumo ezintle a imisebenzi yotyando onikelayo esibelekweni. Emva kwethutyana, mhlawumbi, abohlukeneyo umzimba kuya kunceda amabhinqa ngesi sifo singanyangekiyo ngokwabo ukuze bazale abantwana.

Ubudlolo kwakunye syndrome Rokitansky-Mayer-Otto Küstner

Ngaba mna nabantwana nesi sifo? Lo mbuzo kuvela abafazi abaninzi abajongene nale ngezifo. Maxa wambi, ngamnye kubo ufuna ukuzama indima kamama. abafazi abaninzi nale syndrome kukuthwala umntwana. Ukuba amaqanda abasebenza ngesiqhelo, ngoko iingcali bacebise izigulane ukufumana uncedo lokufumana iinkonzo umama ngokwesivumelwano. Kulo mzekelo umntwana yamitha?

Inxaxheba kwinqanaba lokuqala inkqubo umama ngokwesivumelwano kunye umfazi ogulayo imiselwe iziyobisi hormone. Ukusetyenziswa kweziyobisi kwisithuba sexesha elithile ikuvumela ukuba ukuzivumelanisa bophulwe sexesheni. Ke iqanda syndrome MRKH eli bhinqa madlozi ngokuzenzela senye yendoda yakhe. Kwiintsuku ezimbalwa zabo "ikhule" kwi eliphakathi olungele iimpawu tubes. Emva kweentsuku ezintathu, iingcali ekuqaleni amalungiselelo pre-umzila: amisele nesini ndiseyimbumba, ubukho iziphene chromosomal kunye nezifo njengelifa. Ngosuku lwesihlanu isuka umbungu idluliselwa isibeleko kamama ngokwesivumelwano. Kwezinye iimeko, umntu podsazhivanie kabini, umzekelo, ngomhla wesithathu kunye wesihlanu.

Inani ngeembumba zidluliselwe ingohluka ngokuxhomekeke umgangatho umbandela yemfuza, ubudala isigulane. Xa isicelo yomfazi, iqanda ongasetyenziswanga baye inkqubo cryopreservation. Ngenxa yale ukukhulelwa kulo ihlabathi iinyanga 9 kukho umntwana onempilo. Nangona kunjalo, le nkqubo iye zombini abaxhasi kunye nokuphikisa.

Ngokwahlukeneyo kufanele cinga surrogacy. Kwiiklinikhi ezininzi ezizodwa kukho isiseko ekuthiwa-yabafazi, abakulungeleyo ukunika inkonzo efanayo. Ngokuqhelekileyo abaselula, amantombazana esempilweni. Esinye sezizathu kokubhenela kwikliniki hlobo syndrome Rokitansky-Otto Küstner-Meyer-Hauser. Amafoto elizayo oomama yengqondo nayo ingafumaneka kolu luhlu. yonke le nkqubo, kukhetho into engaba biomamy nokukhulelwa nesiphelo, abaphathi bamaziko ulawulo. Ngoko ke kungcono ukuba uqhagamshelane iingcali, kunokuba nenxaxheba ekufuneni self. Kuphela ngale ndlela sinokuhlala eqinisekile impilo elizayo usana lwakho.

Ngamafuphi

Syndrome Rokitansky-Mayer-Otto Küstner - sisifo yemfuza esibonakala ukungabikho okanye hypoplasia ye wesibeleko kunye lobufazi. Amantombazana amaninzi phambi ukufikisa ayazi noyena malunga neengxaki zempilo. Amenorrhea ebangela ukuba ukubona ugqirha. Kwaba kwindawo yolwamkelo kwi ingcali kuqala bati bobukho onjalo syndrome. Kwelinye icala, oko enohlobo maldevelopment le wesibeleko kunye lobufazi, kwaye kwelinye - ubukho neempawu zesini eziziisekondari. Namhlanje, kukho iindlela zonyango eziliqela ukuba izifo. Bonke babo rhoqo ukuba eziphuculweyo eya kuthi yongezwe. Uthomalaliso esi sifo kukuba ukususa siphene yi iiplastiki banye. Ngenxa yoko, le isigulane kukuphelisa iingxaki zengqondo kunye komzimba, ukunandipha ubomi kunye nokuba babe nabantwana. Ngokubhekiselele kwindawo yesibini, oko kufuna uncedo kamama ngokwesivumelwano.