Testicular feminization syndrome: mankhwala ndi kapewedwe

The matenda a feminization testicular - ndi n'chosoŵa kobadwa nako matenda omwe limodzi ndi kuchepa kudziwa mahomoni chiwalo chachimuna. Zikavuta kwambiri, thupi ndi chiyani kugwira zotsatira za androgens. Zizindikiro za matenda angathandize osiyana a mwamphamvu, ndi kuchiza odwala anatola payekha.

Kumene, anthu akukumana ndi matenda ofanana, ndi chidwi zambiri. Kodi testicular feminization syndrome? Kodi kuchiza matenda ndiponso ngati pali njira kwenikweni bwino chithandizo? Kodi chimayambitsa matenda? ndi maganizo odwala chiyani? Mayankho a mafunso amenewa akufunafuna ambiri.

The matenda a feminization testicular - ndichiyani?

Choyamba, tiyeni kuthana ndi mawu akuti. The matenda a feminization testicular - matenda obadwa, amene amayambitsidwa ndi kusintha mu khromozomu kugonana. Matenda limodzi ndi imfa ya kudziwa androgens, mlingo wa kuchepetsa chiwopsezo kwa mahomoni kugonana mwamuna angakhale osiyana - izi zidalira pa mwamphamvu ndi zizindikiro.

Mwachitsanzo, pa kukana zolimbitsa kuti androgens kunja mnyamata akufotokozera ndithu bwinobwino. Komabe, munthu wamkulu akhoza kukhala kuti munthu ndi wosabala, chifukwa umuna thupi lake si opangidwa.

Kwathunthu osiyana akuyang'ana imfa kwathunthu kwa kudziwa mahomoni ndi matenda monga testicular syndrome feminization. karyotype munthu wamwamuna. Komabe, anyamata amabadwa ndi otchedwa hermaphroditism onyenga, mmene muli mapangidwe wa maliseche kunja mtundu yaikazi ndi kukhalapo munthawi yomweyo testes ndi kambiri wa testosterone m'magazi. Panthawi yakutha msinkhu anyamata awa amakhala wamkazi makhalidwe a chiwerewere (mwachitsanzo, zopangitsa mammary kuonjezera).

The matenda a feminization testicular - ndi n'chosoŵa kudwala. 50-70 zikwi akhanda nkhani mwana 1 yekha ndi kusintha zimenezi. Ngati tikulingalira milandu hermaphroditism pafupifupi 15-20% odwala chifukwa cha kukhalapo maliseche atypical chikugwirizana ndi STF lapansi. Zodabwitsa ndizakuti, mu kudwala zachipatala amapezeka pansi maina osiyana - ndi matenda a androgen kukakala Morris matenda, mwamuna pseudohermaphroditism.

STF mu yaikazi: kodi n'zotheka?

Anthu ambiri amachita chidwi ndi funso ngati testicular feminization syndrome mu akazi ndi zotheka. Popeza kudwala amagwirizana ndi kusintha mu Y-khromozomu, tikhoza anena kuti ndi nkhani yokhayo mwamuna aliyense.

Komano, anthu odwala ngati matenda, nthawi zambiri mukuwoneka ngati mkazi. Ndiponso, kuzindikira okha mogwirizana. Malinga ndi kafukufuku, odwala hermaphroditism onyenga kawirikawiri amaoneka ngati wokongola, wamtali mkazi Taphunzira chithunzi. Anthu ndi matenda umenewu amatamanda ndi makhalidwe ena apadera, kuphatikizapo zomveka zolondola nzeru, luso mwamsanga oyendetsa zimenezi, mphamvu Mwachangu ndi ena "mwamuna" makhalidwe.

chidwi ina - akazi ambiri nawo masewera, ndi karyotype wamwamuna. Ndicho chifukwa Akatswiri kutenga malovu kuti DNA kusanthula - akazi (kutanthauza amuna) ndi matenda Morris sikuloledwa kupikisana.

Mwa njira, pamaso pa matenda otere zimachitika kanjedza ambiri mbiri, kuphatikizapo Joan waku Arc ndi Mfumukazi zotchuka England Elizabeth Tudor.

Zimayambitsa chachikulu cha chitukuko matenda

Monga tanenera kale, Morris syndrome (testicular feminization syndrome) chifukwa cha chilema AR mbewu. Chotero masinthidwe okhudza maselo amene amathandiza kuti mahomoni androgenic, chifukwa cha amangoika kugwira ntchito. Malinga ndi maphunziro, imafalikira pa matenda X-zogwirizana recessive khalidwe, mmene chonyamulira cha chibadwa zosalongosoka, kawirikawiri mayi. Komano, n'zotheka, ndipo ndi kusintha mowiriza mu mwana amene anabadwa mwa makolo awiri athanzi ndithu, koma Zikatero amafotokozedwa kutali kwambiri.

Pa embryonic chitukuko cha gonads ndi (zopangitsa kugonana) anapanga malinga ndi karyotype mwana wosabadwayo - mwana ali maliseche zonse. Koma chifukwa cha minofu kuwonongeka kwa mbewu si tcheru (wopanda chifundo) kuti testosterone ndi degidrosteronu, amene ali ndi udindo kwa mapangidwe mbolo, scrotum, mkodzo, prostate. Pa nthawi yomweyo anasunga tilinazo zimakhala kwa esitirojeni, zomwe zimabweretsa pofuna ya maliseche a mtundu yaikazi (kupatula chiberekero, chiberekero ndi chachitatu chapamwamba nyini).

Mawonekedwe wodzala ndi syndrome ndi zochititsa

Testicular feminization syndrome (Morris) akhozanso kukopeka ndi imfa wathunthu wa cholandilira kudziwa testosterone. Zikatero, mwana wabadwa ndi genotype wamwamuna (pali Y-khromozomu), zopangitsa kugonana wamwamuna, koma wamkazi maliseche kunja.

Ana otere alibe scrotum ndi mbolo komanso machende ndi kukhalabe zonse m'mimba. M'malo mwake, pali nyini ndi akunja labia. Nthawi zambiri, madokotala Zikatero kunena za kubadwa kwa atsikana. Thandizo, odwala ankapatsidwa, monga ulamuliro, mnyamata anadandaula kulibe kusamba. Mwa njira, mwana kukhala sekondale makhalidwe kugonana mtundu wamkazi (palibe kusintha mawu, kukula tsitsi, chifuwa kukulitsa). Mu tinafotokoza mwatsatanetsatane dokotala alinga kuti gonads kugonana wamwamuna, komanso achindunji DNA.

Nthawi zambiri, matenda a "testicular feminization syndrome" kuyika akazi wamkulu amene amapita kwa katswiri za amenorrhea ndi osabereka.

mitundu chosakwanira cha matenda Morris ndi digiri ya chitukuko

The matenda a feminization testicular anthu akhoza limodzi ndi tsankho kuchepetsa cholandilira kudziwa testosterone. Zikatero, ya zizindikiro mwina osiyanasiyana. dongosolo gulu analengedwa mu 1996 umene pali njira zazikulu zisanu za matendawa.

• digiri Choyamba, kapena mtundu wamwamuna. The mwana ali ndi kutchulidwa wamwamuna fenotayipi ndipo akufotokozera popanda zolakwika aliyense n'kuchiumba. Nthawi zina unyamata anati bere kukulitsa ndi kusintha uncharacteristic mawu. Koma odwala nthawizonse wakhala chakhungu spermatogenesis, chifukwa mu osabereka.
• digiri yachiwiri (makamaka wamwamuna mtundu). Development ndi a mtundu wamwamuna, koma ndi ena yofanana. Mwachitsanzo, n'zotheka mapangidwe micropenis ndi hypospadias (kuchepetsa meatus). Nthawi zambiri, odwala amadwala gynecomastia. Ananena ndi m'goli mafunsidwe a subcutaneous mafuta.
• Mlingo lachitatu, kapena chitukuko cha mtundu okayikira. Odwala anati kuchepetsa Amowabu la mbolo. Kusinthidwa ndi scrotum - nthawizina mu mawonekedwe izo akufanana labia akunja. Pali kusamutsidwa a mkodzo, ndi machende zambiri sanali ukutsika mu scrotum lapansi. Amalembedwa ndi khalidwe mbali ya akazi - chifuwa augmentation, chithunzi lililonse (m'chiuno lonse, mapewa yopapatiza).
• digiri wachinayi (makamaka wamkazi mtundu). Odwala gulu ili ndi fenotayipi wamkazi. Machende kukhalabe zonse m'mimba. Mukhale wamkazi ziwalo kugonana, koma ndi ena kusintha. Mwachitsanzo, mwana aumbike mwa kanthawi "akhungu" nyini ndi nkongo zambiri hypertrophied ndipo akukumbutsa micropenis.
• Chachisanu digiri, kapena mtundu wamkazi. Izi mawonekedwe a matenda limodzi ndi mapangidwe zizindikiro akazi - mwanayo amabadwa ali mtsikana. Komabe, pali ena zolakwika. Makamaka, odwala zambiri anati kukulitsa wa nkongo ndi.

Iwo limodzi ndi zizindikiro za testicular syndrome feminization. Kubereka kudwala ofanana sizingatheke - m'thupi lake sikuti Maselo chiwalo chachimuna ndi ziwalo wamkazi palibe kapena ayi athanzi.

Malinga ndi kafukufuku, anthu ndi matenda zimenezi nthawi amadwala hernias inguinal chifukwa cha kuphwanya ndime ya maliseche a ngalande inguinal. Chifukwa cha kusamuka kwa kutsegula kunja mkodzo pa chiopsezo matenda osiyanasiyana a dongosolo mkodzo (mwachitsanzo, pyelonephritis, urethritis, ndi matenda ena yotupa) chinawonjezeka.

njira yodziwira

Matenda a matenda - njira yaitali. Pamafunikanso ya ndondomeko:

• Kuyamba, dokotala likamva mbiri. Mu njira ya kafukufuku ayenera kupeza ngati mwanayo ali ndi matenda kakulidwe atangobadwa kapena pa unamwali. Kusanthula ndi mbiri banja (ngati panali ngati zolakwika pakati pa abale).
• Chinthu chofunika zoona thupi, imene katswiri angaone pamaso pa kuvutika mu kapangidwe ka thupi la maliseche kunja, mtundu wa yogawa tsitsi, ndi zina zotero. D. Analemekeza muyeso wa msinkhu ndi thupi la wodwalayo. kafukufuku More chofunika kudziwa comorbidities.
• M'tsogolo unachitikira karyotyping - ndondomeko zimene zimathandiza kudziwa kuchuluka ndi khalidwe DNA, amene nawonso, limachititsa tikupeza kugonana wodwala.
• Ngati ndi kotheka, ikuchitika kuyezetsa maselo chibadwa, imene nthawi akuyesera kuti apeze nambala ndipo zosiyanasiyana majini kuonongeka.
• Ndi kuvomerezedwa Kupenda ndi urologist ndi - dokotala akuphunzira kapangidwe ndi mbali ya maliseche kunja, palpate prostate, etc ...
• Pa kusanthula magazi amatengedwa kukayendera timadzi ta m'thupi.
• Ndi nkhani ndi ultrasound bwino. njirayi limachititsa kuti azindikire anomalies mu Kapangidwe ka ziwalo zoberekera, testes Undescended kudziwa, ama comorbidities.
• The mfundo zolondola kwambiri zokhudza kapangidwe ka ziwalo mkati akhoza kuwapeza pa kumveka maginito kapena yopangidwa tomography.

The matenda a feminization testicular: chithandizo

Therapy, mu nkhani iyi zimadalira m'badwo wa mtima ndi digiri ya kukakala kwa androgen lilime lathu. Kwenikweni anatumizidwa timadzi m'malo mankhwala amene amalola kuthetsa akusowa androgen, kuthandiza kulenga ufulu sekondale makhalidwe kugonana, kuthetsa zotheka anomalies mu chitukuko.

Iwo ankadziwa kuti chinthu chofunikira kwambiri ndi misala - Wodwala ayenera kukaonana ndi katswiri. Ndipotu, malingana ndi kuwerengera, ndi hermaphroditism onyenga kawirikawiri zimabweretsa chitukuko cha matenda a maganizo. Ngati kusintha ndi amawapeza mwangozi pamene akula (tikulankhula za kukakala wathunthu zolandilira testosterone), adokotala akhoza kugamula kuti kukanena kwa mkazi amene amakhala moyo wonse ndi akudzitchulanso monga nthumwi ya kugonana chilungamo.

Pamene opaleshoni m'pofunika?

mavuto ambiri angathe kuthetsedwa ndi thandizo la njira yapadera. Kwa odwala ndi fenotayipi wamkazi limasonyeza kuchotsa testes lapansi. njirayi m'pofunika kuteteza hernias chifukwa chitukuko ndi pofuna kukwaniritsa khalidwe lachimuna kugonana. Komanso, zimatenga kupewa khansa testicular.

Ngati thupi akufotokozera mu mtundu chachikazi, ndi zina zofunika pulasitiki nyini ndi akumaliseche, zomwe zimathandiza kuti mukugonana. Opaleshoni akhoza kuthetsa kuchepetsa ngalande mkodzo.

Ndi kukula chitsanzo wodwalayo mwamuna zina zomuthandiza pa ducts seminal mu scrotum lapansi. Popeza anthu ambiri ndi matenda amenewa amadwala gynecomastia, nthawi zambiri anachita m'mawere opaleshoni pulasitiki, zomwe zimathandiza kubwezeretsa mawonekedwe thupi.

Kuti madokotala ananena zawo odwala ndi mavuto zotheka

The matenda a feminization testicular (Morris) si kuopseza mwachindunji moyo. thupi ndi ntchito yachibadwa ngakhale pamene kwathunthu kukakala kwa androgen mahomoni. Pambuyo wodwala kuchipatala ndi opaleshoni akhoza kukhala moyo wathunthu wa mkazi, kukhala karyotype wamwamuna. Koma pali chiopsezo cha khansa testicular kuti sanali ukutsika mu scrotum kwa - mu Zikatero m'pofunika kuchitapo kanthu. Kupewa khansa anachita kuchotsa opaleshoni ya testes mu scrotum (ngati wodwalayo wamwamuna fenotayipi) kapena kuchotsedwa kwathunthu kwa England (ngati mkazi fenotayipi).

Poganizira mavuto ena zotheka kuti mndandanda awo ndi chosatheka kugonana (malformation a kumaliseche), voiding (mu njira ya chitukuko cha dongosolo urogenital mkodzo njira kulakwa). Odwala n'ngosabereka kaya fenotayipi. Musaiwale za mavuto ena, si monga mwana aliyense ndi wachinyamata kwambiri zotheka kumvetsa makhalidwe a thupi lake. Kumene, vuto la dera kugonana, komanso matenda a dongosolo excretory, angathe kuthetsedwa pa opaleshoni. Kuti madokotala ananena zawo odwala yabwino mulimonse.

Kodi pali njira zodzitetezera?

Mwatsoka, palibe njira kuteteza matenda amenewa. Koma ngati testicular feminization syndrome ndi kudwala chibadwa, ngozi za khansa angathe kuonedwa pa siteji ya mapulani a mimba - makolo oyembekezera ayenera kuti anayesedwa.

Kwa odwala kale anapezeka ndi matenda, ayenera chithandizo choyenera zachipatala komanso mayeso zonse zachipatala, m'thupi mankhwala.