Testicular feminization ciwo: jiyya da kuma rigakafin

A ciwo na testicular feminization - a gwada rare nakasar cuta wadda aka tare da wani karu a ji na ƙwarai to namiji ji ba gani. A mafi tsanani lokuta, cikin jiki da kuma ba zama m da sakamakon androgens. A bayyanar cututtuka da wannan cuta za su iya samun sãɓãwar launukansa digiri na tsanani, da kuma magani ga marasa lafiya tsince akayi daban-daban.

Hakika, mutane suna fuskantar wani irin cuta, suna sha'awar ƙarin bayani. Mene ne testicular feminization ciwo? Yadda za mu bi da cutar da kuma ko akwai sosai tasiri hanyoyi da magani? Abin da ya haddasa cuta? Mene ne gaba ga marasa lafiya? Amsoshin wadannan tambayoyi suna neman yawa.

A ciwo na testicular feminization - abin da yake da shi?

Da farko, bari mu magance da ma'anar kalma. A ciwo na testicular feminization - wani nakasar da cutar, wanda aka sa ta maye gurbi a cikin wani jima'i chromosome. Rashin lafiya tare da asarar ji na ƙwarai to androgens, da mataki na raguwa a laulayi da ɗa namiji hormones iya zama daban-daban - wannan ya dogara da tsanani kuma bayyanar cututtuka.

Alal misali, a wata matsakaici jure androgens externally yaro tasowa quite al'ada. Duk da haka, ya fara tasawa iya zama, cewa mutumin rama, saboda maniyyi jikinsa ne kawai ba samar.

Mabanbanta dubi cikakken asarar ji na ƙwarai to hormones da irin wannan cuta a matsayin testicular feminization ciwo. mutum karyotype ne namiji. Duk da haka, boys an haife tare da abin da ake kira ƙarya hermaphroditism, a cikin abin da akwai samuwar na waje genitalia na mace irin tare da na lokaci daya gaban testes da kuma al'ada matakin na testosterone a cikin jini. A lokacin balaga, a wadannan yara maza sukan wajen samar da mace jima'i halaye (misali, mammary gland kara).

A ciwo na testicular feminization - a gwada rare Pathology. 50-70 dubu jarirai lissafta kawai 1 yaro tare da wannan maye gurbi. Idan muka yi la'akari da lokuta na hermaphroditism, kamar 15-20% da marasa lafiya cikin hanyar gaban atypical genitalia an haɗa da jami'an rundunar STF. Ba zato ba tsammani, a cikin likita Pathology bayyana a karkashin sunaye daban-daban - a ciwo na androgen insensitivity Morris ciwo, namiji pseudohermaphroditism.

Da jami'an rundunar STF a mace: shi ne zai yiwu?

Mutane da yawa suna sha'awar wannan tambaya na ko testicular feminization ciwo a mata mai yiwuwa ne. Tun da Pathology ake dangantawa da maye gurbi a cikin Y-chromosome, za mu iya shakka cewa shi ne kawai batun wani mutum namiji.

A daya hannun, mutanen da fama da irin wannan cuta, sau da yawa kama da wata mace. Haka kuma, sun gane kansu yadda ya kamata. Bisa kididdigar da, marasa lafiya da ƙarya hermaphroditism sau da yawa kama da wani m, m mace da ramammu adadi. Mutane da irin wannan ganewar asali ko yaba da wasu na musamman halaye, ciki har da ma'ana da kuma daidai da hankali, da ikon da sauri Orient a cikin halin da ake ciki, makamashi, yadda ya dace da kuma sauran "namiji" halaye.

Wani mai ban sha'awa Gaskiyar - mata da yawa da hannu a wasanni, da namiji karyotype. Shi ya sa masu sana'a da 'yan wasa dauki yau ga DNA analysis - mata (cewa shi ne, maza) tare da ciwo Morris ba a yarda ya gasa.

Af, gaban irin wannan cuta da aka dangana wa da yawa na tarihi Figures, ciki har da Joan na Arc kuma sanannen Sarauniyar Ingila Elizabeth Tudor.

Babban Sanadin cutar ci gaba

Kamar yadda aka ambata riga, Morris ciwo (testicular feminization ciwo) ne sakamakon AR gene lahani. Irin wannan maye gurbi da shafi da rabe cewa amsa Androgenic ji ba gani, a sakamakon su kawai zama m. A cewar nazarin, shi ne daukar kwayar cutar a kan ciwo X-nasaba recessive hali, a cikinsa wani m of m kwayoyin halitta, shi ne yawanci uwar. A daya hannun, shi ne zai yiwu, da kuma wani yunkri maye gurbi a cikin yaro, wanda aka ɗauki cikinsa ta biyu iyaye quite lafiya, amma irin haka ne ya ruwaito nisa kasa sau da yawa.

A lokacin da amfrayo ci gaban da gonads (jima'i gland) suna kafa bisa ga tayin karyotype - yaro yana da cikakken golaye. Amma saboda nama lalacewar na gene ba m (desensitized) zuwa testosterone da degidrosteronu, wanda ke da alhakin samuwar azzakari, Kwalatai (Scrotum), mafitsara, prostate. A lokaci guda kiyaye ji na ƙwarai kyallen takarda zuwa estrogen, wanda ya haddasa kara ci gaban da genitalia na mace irin (sai mahaifa, fallopian shambura da kuma na sama na uku daga cikin farji).

Cikakken tsari na ciwo, kuma da fasali

Testicular feminization ciwo (Morris) iya a tare da cikakken asarar tsoka mai amsa sigina ji na ƙwarai to testosterone. A irin haka ne, yaro da aka haife tare da wani namiji genotype (akwai Y-chromosome), da ɗa namiji gland, amma mace waje genitalia.

Irin yara da babu Kwalatai (Scrotum) da kuma azzakari da golaye da kuma zama a cikin kogon ciki. A maimakon haka, akwai farji da kuma m labia. Popular sau da yawa, likitoci a irin haka ce game da haihuwar yara mata. Domin taimako, marasa lafiya da aka bi, kamar yadda mai mulkin, wani saurayi koka da rashi na haila. Af, da yaro ci gaba sakandare jima'i halaye na mace irin (babu maye gurbi na murya, gashi girma, ƙirjinka kara girma). A wani cikakken jarrabawa likita kayyade cewa gonads na namiji jima'i da wani takamaiman sa na chromosomes.

Sau da yawa, da ganewar asali "testicular feminization ciwo" sa ya fara tasawa mata suka juya zuwa ga wani gwani game amenorrhea da kuma rasa haihuwa.

Bai cika siffofin na ciwo Morris da kuma mataki na cin gaban

A ciwo na testicular feminization a maza iya kawai a tare da wani m raguwa a tsoka mai amsa sigina ji na ƙwarai to testosterone. A irin haka ne, a sa na bayyanar cututtuka na iya zama mafi bambancin. rarrabuwa tsarin da aka halitta a shekara ta 1996, a karkashin abin da akwai biyar babban siffofin da cutar.

• Da farko mataki, ko namiji type. The yaro yana da wani pronounced namiji phenotype kuma tasowa ba tare da wani alama sabawa. Lokaci-lokaci samartaka lura da nono kara girma da kuma uncharacteristic canji na murya. Amma marasa lafiya ko da yaushe aka sosai spermatogenesis, sakamakon rashin haihuwa.
• Na biyu digiri (zai fi dacewa namiji irin). Development ne na namiji type, amma tare da wasu bambancin. Alal misali, zai yiwu samuwar micropenis da hypospadias (biya diyya meatus). Sau da yawa, marasa lafiya da wahala daga gynecomastia. Ya lura da m shaida na subcutaneous mai.
• The uku digiri, ko da ci gaban ambivalent irin. Marasa lafiya lura wani alama rage daga cikin azzakari. Modified da Kwalatai (Scrotum) - wani lokacin a cikin nau'i yake kama da matsanancin labia. Akwai hijirar na mafitsara, da kuma golaye sau da yawa bai sauka a cikin Kwalatai (Scrotum). Alama da kuma halayyar fasali na mata - nono augmentation, wani hali adadi (m kwatangwalo, kunkuntar kafadu).
• Fourth digiri (yafi mace irin). Marasa lafiya a cikin wannan kungiyar da mace phenotype. Golaye kasance a cikin kogon ciki. Ci gaba mace jima'i gabobin, amma tare da wasu bambancin. Alal misali, yaro da aka kafa ta a takaice "makafi" farji da kuma shasshafa dan tsakar ta sau da yawa hypertrophied da kuma tunatar da micropenis.
• Biyar digiri, ko mace irin. Wannan nau'i na cutar da ake tare da samuwar mata bayyanar cututtuka - da yaro da aka haife wata yarinya. Duk da haka, akwai wasu sabawa. A musamman, marasa lafiya sukan lura kara girma da shasshafa dan tsakar ta.

Yana yana tare da bayyanar cututtuka na testicular feminization ciwo. Sake bugun tare da kama Pathology ba zai yiwu - haƙuri ta jiki ba ya nuna ɗa namiji Kwayoyin, kuma mace gabobin ne ba samuwa ko ba su cikakken kafa.

Bisa kididdigar da, mutane da irin wannan ganewar asali sau da yawa wahala daga inguinal hernias, saboda da take hakkin da nassi daga cikin golaye na inguinal canal. Saboda hijirar na waje bude daga cikin mafitsara a ƙãra hadarin cututtuka daban-daban na urinary tsarin (misali, pyelonephritis, urethritis, da kuma sauran kumburi cututtuka).

bincike da hanyoyin

Ganewar asali da wannan cuta - mai tsawo tsari. Yana hada da wani sa na hanyoyin:

• Don fara, da likita tattara tarihi. A cikin shakka daga binciken bukatar gano ko yaro yana da wasu na cin gaba cuta bayan haihuwa ko a lokacin balaga. Bincikar da iyali tarihi (ko ya kasance wannan sabawa daga dangi).
• An muhimmanci mataki ne da kuma ta jiki jarrabawa, a lokacin da gwani zai iya lura gaban munanan a cikin tsarin da jiki da kuma na waje genitalia, da irin gashi rarraba, da sauransu. D. Gudanar ji na tsawo da kuma nauyi na haƙuri. More bincike ake bukata don gane comorbidities.
• A nan gaba da aka gudanar karyotyping - wata hanya da damar domin sanin yawa da kuma ingancin chromosomes, wanda, bi da bi, ya sa ya yiwu don gano jima'i na haƙuri.
• Idan ya cancanta, da za'ayi kwayoyin kayyade gwaji, lokacin da lokacin da kokarin gano adadin kuma iri-iri na lalace genes.
• Shi ne m jarrabawa da wani urologist - likita na nazarin tsarin da kuma fasali na waje genitalia, tausa prostate, da dai sauransu ...
• A analysis, jini yana dauka duba hormone matakan.
• Shi ne m da kuma duban dan tayi jarrabawa. Wannan hanya ya sa ya yiwu don gane anomalies a cikin tsarin da ciki haihuwa gabobin, testes Undescended gane, gane asali comorbidities.
• A mafi m bayanai game da tsarin da kayan ciki za a iya samu a lokacin da wani Magnetic rawa ko lissafta tomography.

A ciwo na testicular feminization: magani

Far, a cikin wannan yanayin ya dogara da shekaru na haƙuri, kuma mataki na insensitivity da androgen tsoka mai amsa sigina hormone. Dole sanya hormone maye far, wanda damar kawar da androgen kasawa, don taimaka haifar da dama sakandare jima'i halaye, don kawar da yiwu anomalies a ci gaba.

An fahimci cewa wani musamman muhimmanci mataki ne psychotherapy - The haƙuri dole ne ko da yaushe tuntubar wani gwani. Bayan duk, bisa ga statistics, da ƙarya hermaphroditism sau da yawa take kaiwa zuwa cin gaban asibiti ciki. Idan wani maye gurbi da aka yi fama da damar a lokacin} uruciyarsa (muna magana ne game da wani cikakken insensitivity zuwa testosterone rabe), likita zai iya yanke hukunci ba da rahoto da shi ga wata mace da suka zaune a cika rai da kuma gano kansa a matsayin wani wakilin adalci jima'i.

Lokacin da tiyata wajibi ne?

Mutane da yawa matsalolin da za a iya warware da taimako na musamman hanyoyin. Ga marasa lafiya da mace phenotype nuna kau na testes. Wannan hanya wajibi ne don hana hernias domin ci gaba da kuma kara ci gaba da namiji jima'i halaye. Bugu da ƙari kuma, da hanya ne rigakafin testicular ciwon daji.

Idan jiki tasowa a cikin mace irin, shi ne wani lokacin dole roba farji da kuma vulva, wanda ya sa ya yiwu a yi jima'i. Surgically iya kawar da biya diyya urinary canal.

Tare da ci gaban da haƙuri da namiji juna ne wani lokacin dole kawo seminal ducts a Kwalatai (Scrotum). Tun da yawa maza da irin wannan ganewar asali sha daga gynecomastia, shi ne sau da yawa yi nono roba tiyata, wadda taimaka mayar jikin siffar.

A hangen nesa ga marasa lafiya da kuma zai yiwu rikitarwa

A ciwo na testicular feminization (Morris) ba kai tsaye barazana ga rayuwa. The jiki ne na al'ada ayyuka har a lõkacin da cikakken insensitivity zuwa androgens. Bayan likita da m haƙuri iya zama cikakken rai mace, ciwon namiji karyotype. Amma akwai wani hadarin tasowa testicular ciwon daji cewa bai sauka a cikin Kwalatai (Scrotum) - a irin haka wajibi ne a dauki mataki. Domin rigakafin ciwon daji yi m kau na testes a cikin Kwalatai (Scrotum) (idan haƙuri ne namiji phenotype) ko cikakken kau da gland shine yake (idan mace phenotype).

Game da wasu yiwu rikitarwa cewa su jerin ne da rashin yiwuwar jima'i (malformation na al'aurar), voiding (a aiwatar da ci gaban da urogenital tsarin urinary tashar canjawa). Marasa lafiya ne infertile ko da phenotype. Kada ka manta game da zamantakewa matsaloli, kamar yadda ba kowane yaro da kuma matashi da mafi yiwu ga fahimtar halaye na da kansa jiki. Hakika, matsalar na jima'i Sphere, kazalika da cututtuka na excretory tsarin, za a iya shafe ta a lokacin tiyata. A hangen nesa ga marasa lafiya m wata hanya.

Shin akwai m matakan?

Abin baƙin ciki, akwai wani wajen iya hana irin wannan cuta. Amma kamar yadda testicular feminization ciwo ne a kayyade Pathology, hadarin tasowa ciwon daji za a iya gano a mataki na shiryawa da ciki - mai yiwuwa iyaye bukatar samun gwada.

Ga marasa lafiya riga kamu da cuta, suna bukatar m likita taimako, kazalika da likita Nazarin, hormonal far.