Testicular feminization maʻi pale: lapaau a me ka Kāohi

Ka maʻi pale o testicular feminization - he kŘpa a pohihihi keia congenital kāna hana i ua pu ma ka emi ma ka pā lonoaʻili i kane wahine hormones. I oi loa hoopii, i ke kino a me ka malama lilo insensitive i ka hopena o androgens. Nā symptoms o keia maʻi hiki i loli iki degere o ka oolea, a me ka lapaʻau no nā mea maʻi lalau pākahi.

O ka holo ana, eʻalo kanaka me ka like maʻi, e hoihoi i ka 'ike hou aku. He aha ka testicular feminization maʻi pale? Pehea e hana i ka maʻi, a ina paha he mea maoli ka hoʻokō 'ano o ka lapaau? He aha ke kumu maʻi? He aha ka ke Outlook no ka hoʻomanawanui iki mai? Pane i kēia mau nīnau e imi no na mea he nui.

Ka maʻi pale o testicular feminization - He aha ka mea?

Mua, e i ka hana me ka ano o ka makahiki. Ka maʻi pale o testicular feminization - he congenital maʻi, a ua kauoha ia e ka mutation i loko o ka wahine chromosome. Maʻi pu ma ka poho o ka pā lonoaʻili i androgens, ke degere o ka ho'ēmiʻana i loko o susceptibility i kane anal hormones paha e kekahi - kēia hilinaʻi nui ma luna o ka oolea a me ka symptoms.

No ka laʻana, ma ka lōpū ia'ku i androgens externally keiki e hoʻomōhala ana i he maʻamau. Eia naʻe, he kanaka makua ke e, i ke kanaka mea sterile, no ka mea, o ka sperm kona kino ua wale,ʻaʻole i lawe.

Loa okoa nana piha lilo o ka pā lonoaʻili i hormones ku e na ano maʻi like testicular feminization maʻi pale. kanaka karyotype mea he kane. Eia naʻe, i me ka ai-kapaia hermaphroditism wahahee, ma ka a he mea i ke kahua o ka mawaho genitalia o ka wahine 'ano me ka mea i ka wā hoʻokahi alo o na testes a me ka maʻamau nui o ka testosterone i loko o ke koko hanau keikikane. Ma puberty i loko o kēia mau keiki kāne i kamaʻilioʻana e hoʻoikaika i wahine moe ano (e like, mammary glands hoomahuahua).

Ka maʻi pale o testicular feminization - he kŘpa a ke nānā pathology. 50-70 tausani nā pēpē i hoike ia wale 1 keiki i keia mutation. Inā na hihia o ka hermaphroditism mākou noʻonoʻo, ka hola 15-20% o nā mea maʻi i ka aoao o ke alo o atypical genitalia ua pili i ka STF. Incidentally, i loko o ka 'inikua pathology akaka ma lalo o kekahi mau inoa - he maʻi pale o androgen insensitivity Morris maʻi pale, kane pseudohermaphroditism.

STF i loko o ka wahine: ka mea hiki?

He nui nā kanaka i hoihoi i loko o ka ninau o ina ka testicular feminization maʻi pale i ka wahine me ke kūʻokoʻa. Mai ka pathology ua pili me ka mutation i loko o ka Y-chromosome, ua hiki maopopo leʻa i aku nei au ia ia mea malalo wale i kekahi kanaka kāne.

Ma ka kekahi lima, kanaka e loaa ana mai ia i ka maʻi, pinepine nana e like me ka wahine. Eia hoʻi, ka mea, ike lakou ia lakou iho e like. Wahi a ka 'ikepili helu, nā mea maʻi me ka hermaphroditism wahahee pinepine nana me ka uʻi, he poe loihi e wahine me ka wīwī huahelu. People me ia i kaʻike, a hiki uku i kekahi kūikawā kāna moʻolelo, me Logical a me ka pololei manao, i ka hiki e koke orient ma ke kulana, ikehu, ka hoʻokōʻana a me nā "kane" ano.

Kekahi hana hoihoi 'mea - nui na wahine limahana i loko o haʻuki, i ke kane karyotype. A no ke aha la 'oihana' âlapa lawe tuha no ka DNA Ka Ikepili - wahine (ia mea, na kanaka) me ka maʻi pale Morris ua ole ae i ka hoʻokūkū.

Ma ke ala, i ke alo o ia maʻi ua wahi i na mea he nui mōʻaukala na kii, me ke komo pu Joan o piʻo a me ke kaulana Moiwahine oʻEnelani Elizabeth Tudor.

I ka papa kuhikuhiE kumu o ka maʻi ulu

E like me ua oleloia, Morris maʻi pale (testicular feminization maʻi pale) o ka hopena o ka Ara'ōewe kina. Oia ka mutation ka heluna o nā receptors e pane i ka androgenic hormones, like me ka hopena ka mea wale lilo insensitive. E like me ka haʻawina, ka mea, ua i hiki ma luna o ka maʻi pale X-ua hoʻopili recessive kāna moʻolelo, ai ka lawe ana i hemahema nā'ōewe, mea i hookuu i ka makuwahine. Ma ka kekahi lima, he mea hiki, a me kekahi noho'na makaainana i ulu mutation i loko o ke keiki, ka mea i hapai ia e na makua mau loa ai ola, akā, ua hai aku la ia mau hihia loihi emi pinepine.

Ma embryonic kūpono 'ana o nā gonads (' īnea i glands) e hana e like me ka fetus karyotype - he keiki i piha hoʻi. Akā, no ka mea, o ka mea'aʻaʻa pōʻino o ka'ōewe mea i ikepili koʻikoʻi (desensitized) e testosterone a me ka degidrosteronu, i mea kuleana no ka ho okumu ana o ka penis, scrotum, urethra, prostate. I ka Ia manawa i mālama i ka pā lonoaʻili o nā'aʻaʻa e estrogen, i ke kumu no ka hou 'ana o nā genitalia o ka wahine type (koe ka uterus, fallopian paipu a me ka hoaka o hapakolu o ka vagina).

Ka piha ano o ka maʻi pale a me kona hiʻona

Testicular feminization maʻi pale (Morris) i ke ukali e piha lilo o ka mea loaʻa pā lonoaʻili i ka testosterone. Ma ia hihia, ua hānauʻia ke keiki me ke kane genotype (ka mea, Y-chromosome), kane keka glands, akā, i ka wahine mawaho genitalia.

Na keiki i ole scrotum a penis, a hoʻi, a noho i loko o ka abdominal lua. Aka, aia no i ka vagina, a mawaho labia. Muli o kona kulana pinepine, kauka ma na hihia e olelo aku e pili ana i ka hanau ana o ke kaikamahine. No ke kōkua, ua malamaia lakou hoʻomanawanui iki mai, e like me ka rula, hoopiiia i ka teenager o ka wa e kaawale aku menstruation. Ma ke ala, i ke keiki hoʻomōhala kiʻekiʻe 'īnea i ano o ka wahine type (ʻaʻohe mutation o ka leo, ulu lauoho, umauma hoʻonui). I ka piha nana ke kauka hoʻoholo i nā gonads o kane, ke keka, a me ka ho'ākāka 'ia paha o nā chromosomes.

Pinepine, i kaʻike, o "testicular feminization maʻi pale" i kekahi kanaka makua wahine e huli ana i ka hoʻomalu e pili ana amenorrhea a me ka infertility.

Kāpili ano o ka maʻi pale Morris, a me ka degere o kona ulu ana

Ka maʻi pale o testicular feminization i loko o na kanaka hiki wale ke ukali ma kekahi hapa ka ho'ēmiʻana i ka mea loaʻa pā lonoaʻili i ka testosterone. Ma ia ano hihia, he lākou o nā symptoms i e oi like. Mea Maluna'ōnaehana i hanaia i ka 1996, ma lalo o a, aia no elime ka papa kuhikuhi ano o ka maʻi.

• First degere, a me kane type. Ke keiki i ka hai kane phenotype a me ka hoʻomōhala ai me kekahi hiohiona deviations. Kēlā wā i kēia wā adolescence mālama umauma hoʻonui a me uncharacteristic loli o ka leo. Akā, hoʻomanawanui iki mai mau, ua hihia hoʻi spermatogenesis, kūpono ma ka infertility.
• Lua o ka degere (kaʻoi loa kane type). Development mea o kaneʻano, akā, me kekahi likeʻole. No ka laʻana, ka mea hiki kahua micropenis a me ka hypospadias (offset meatus). Pinepine, nā mea maʻi e ae mai gynecomastia. Mālama a me ka haoʻa palapala moʻopane o subcutaneous momona.
• Ke kolu o ka degere, a me ka hooulu ana o ambivalent type. E hoomanawanui i malama i na hiohiona ka ho'ēmiʻana o ka penis. Hoʻololi 'a me ka scrotum - kekahi manawa i loko o ka palapala e kohu ai ka pā labia. Aia He He pohupani hale o ka urethra, a me ka hoʻi pinepine i ole iho i loko o ka scrotum. Kaha a me ka ano hiʻona o na wahine - umauma augmentation, he typical huahelu (ākea puhaka, puka poohiwi).
• Ha o ka papahelu (ʻano nui wahine type). Nā mea maʻi i loko o kēia hui i ka wahine phenotype. Hoʻi e noho i loko o ka abdominal lua. Ulu ka wahine moe nā loko, akā, me kekahi mea oko a. No ka laʻana, he keiki ua hana ia e kekahi pōkole "makapo" ka vagina, a me ka clitoris pinepine hypertrophied a me ka nani a micropenis.
• Lima o ka degere, a wahine type. Kēia palapala no ka maʻi ua pu ma ke kahua o ka wahine ka symptoms - ke keiki ua hanau he kaikamahine. Eia naʻe, loaʻa nō kekahi mau deviations. I ka pau, e hoomanawanui i ka pinepine malama hoʻonui o ka clitoris.

Ua Ua pu ma symptoms o testicular feminization maʻi pale. Nā'ōlelo me ka like pathology mea,ʻaʻole e hiki - ke hoʻomanawanui i ka ke kino, aole ia e paka kane ke keka keena, a me ka wahine nā loko mea,ʻaʻole i loaʻa 'ole ka mea, aole i hiki maopopo hana.

Wahi a ka 'ikepili helu, kanaka me ia i kaʻike, pinepine e ae mai inguinal hernias, ma muli o ia ka' aʻe o ka hooholo ana o ka hoʻi o ka inguinal Canal. Aie i ka pohupani hale o ka mea mawaho puka o ka urethra ma māhuahua pilikia o nā maʻi likeʻole o ka urinary'ōnaehana (e like, pyelonephritis, urethritis, a me nā hoʻoʻino maʻi).

PalekanaEND_LINK kaʻina hana

ʻike, o keia maʻi - he lengthy kaʻina. Ua nā He lākou o nā kaʻina hana:

• E hoʻomaka, ke kauka 'ohi i mōʻaukala. Ma ka papa o ka anamanaʻo Pono e loaʻa mai paha i ke keiki i kekahi developmental kāna hana ma hope o ka hānau 'ole i puberty. Ua kālailai a me ka ohana mōʻaukala (ina paha he nui, ua like deviations mai hoahanau).
• An ka hoʻokahuli mea a physical ninaninau, oiai i ka hoʻomalu ke nana i ke alo o abnormalities i loko o ka 'ole o ke kino, a o ka mea mawaho genitalia, ke' ano o ka lauoho o ka makana, a no laila, ma luna o. D. paa ana o ke kiʻekiʻe a me ke kaumaha o ka hoʻomanawanui. More noiʻi ua pono ai e kuhikuhi comorbidities.
• I ka wā e hiki mai paʻa karyotyping - he hana ia e leie aku e hooholo i ka nui a me ka pono o ka chromosomes, a, ma ka huli, i ka mea e hiki ai e loaʻa mai i ka wahine o ka hoʻomanawanui.
• Inā pono, lawe mai hakakino aaiaoe ikea ai, a ma ia manawa ho'āʻo nei e loaʻa mai i ka helu a me kaʻano o ka poino nā'ōewe.
• He OAXA ka ninaninau ma ka urologist - he kauka ua këia i ka 'ole a me nā hiʻona o ka mawaho genitalia, palpate ka prostate, a pela aku ...
• Ma Ka Ikepili, ua laweʻia ke koko, e nānā hōmona pae.
• He informative a me ka ultrasound ninaninau. Kēia kaʻina hana i ka mea e hiki ai e huai ae anomalies i loko o ka 'ole o ka na reproductive nā loko, testes Undescended huai ae, diagnose comorbidities.
• Ka loa pololei 'ike e pili ana i ka' ole o ka mea na nā loko hiki ke loaa ma kaʻume mākenēki resonance a Me tomography.

Ka maʻi pale o testicular feminization: lapaʻau

Inc, ma keia hihia hilinaʻi nui ma luna o ka makahiki o ke ahonui a me ka degere o ka insensitivity i ka androgen mea loaʻa hōmona. Pono ia hōmona hope Inc, e leie aku e hoʻopau androgen deficiency, e kōkua hana i ka pono haumāna ke keka ano, e hoʻopau hiki anomalies i loko o ka ulu ana.

Ma ka hoʻomaopopo aku i ka loa nui i koe i ka psychotherapy - Ka hoomanawanui pono mau nīnau i ka hoʻomalu. Ma hope o nā mea a pau, e like me ka 'ikepili helu, he hermaphroditism hoopunipuni pinepine hiki aku ai i ke kūpono' ana o ka lapaʻau kaumaha. Inā he mutation ua diagnosed wale i adulthood (mākou e kamaʻilio e pili ana i ka piha insensitivity e testosterone receptors), ke kauka e olelo ole, e hoike aku ia ia i kekahi wahine e ola i ka piha ola, a e hōʻike ana iā ia iho me kekahi 'elele o ka maikai ke keka.

I kaʻoki kino ka pono?

He nui nā pilikia hiki ke wehe pilikiaʻana me ke kōkua o kūikawā kaʻina hana. No nā mea maʻi me ka wahine phenotype hōʻike ana o na testes. Kēia ke kaʻina hana no ka pono, e pale aku hernias no ka mea, i ke ana a me ka hou 'ana o ke kane, ka moe' ano. Eia kekahi, i ke kaʻina hana o ka Kāohiʻana o testicular Ka maʻi 'aʻai.

Inā i ke kino e hoʻomōhala ana i loko o ka wahineʻano, ka mea i kekahi manawa e pono ai ea vagina a me ka vulva, aʻo ka mea e hiki ai i ka i moe ke keakea ole. Surgically hiki hoʻopau offset urinary Canal.

Me ka hooulu ana o ka hoʻomanawanui ka kāne kumu i kekahi manawa e pono e lawe mai i ka seminal ducts i loko o ka scrotum. No ia mea, na kanaka me ia i kaʻike, e ae mai oe mai ka gynecomastia, ka mea, ua pinepine hana umauma eaʻoki kino, a kōkua hoihoi maoli kino shape.

Ke prognosis no nā mea maʻi, a hiki complications

Ka maʻi pale o testicular feminization (Morris) mea,ʻaʻole he ala hoʻoweliweli 'ana i ke ola. Ke kino Ua maʻamau oihana i ka wā i hiki maopopo insensitivity e androgen hormones. Ma hope o 'inikua, aʻoki lāʻau lapaʻau hoomanawanui ke ola i ka piha ola o ka wahine, me ke kane karyotype. Akā, he mea i ka pilikia o ka ulu testicular Ka maʻi 'aʻai mea i ole iho i loko o ka scrotum - i loko o ia mau hihia ia mea e pono ke lawe hana. No ka Kāohiʻana o ka maʻi 'aʻai hanaʻoki lāʻau lapaʻau ana o na testes i loko o ka scrotum (ina o ka hoʻomanawanui mea kane phenotype) a piha ana o ka gland (ina wahine phenotype).

Me ka pili ana i ka 'ē hiki complications i kā lākou papa inoa o ka impossibility o moe ke keakea ole (malformation o ka wahine), voiding (i loko o ke kaʻina hana o ka? Acaeoey o ka urogenital'ōnaehana urinary Kanal' oni iki). Nā mea maʻi i infertile nānā 'ole o ka phenotype. Mai poina e pili ana i ka nohona pilikia, like ole i na keiki a me ka opiopio kanaka i ka nui loa, e maopopo i nā 'ano o kona kino iho. O ka holo ana, no ka pilikia o ka moe poepoe, e like me nā maʻi o ka excretory'ōnaehana, hiki ke eliminated iʻoki kino. Ke prognosis no nā mea maʻi maikaʻi Hehe.

He loaʻa kāohi ana?

Eia naʻe, loaʻa nō aohe mea hiki ke pale ia maʻi. Akā, me testicular feminization maʻi pale mea he aaiaoe pathology, o ka pilikia o ka ulu maʻi 'aʻai hiki ke' akomi i ma ke kahua o ka hoʻoneʻe o ka hāpai keiki - prospective makua Pono no ka ikea ai.

No nā mea maʻi ua diagnosed me kāna hana, ka mea e pono ai palapala hōʻoia no 'inikua kōkua like hoʻi me regular' inikua examinations, hormonal Inc.