Uyini Kálmán syndrome? Kallman sika Syndrome: symptomatology, ukuxilongwa kanye nokwelashwa kwemiphumela ethile eyisiSekelo

"Ukugqashula" isakhi esisodwa kungaholela izifo ezingathí sina, ikhambi kubo kungenzeki ngokuphelele. Enye yalezi pathologies kuyinto Kallman Syndrome. Lesi sifo kwenzeka ogxile ezingabantu, nakuba abameleli besifazane ngezinye izikhathi nabo.

incazelo Jikelele lesi sifo

Kallman Syndrome - a angahlangani zofuzo okuyinto idluliselwa njengenhlangano autosomal esikhubazayo, autosomal isakhi sofuzo ephezulu kanye X-ezixhunyiwe.

Libhekene isifo iziyaluyalu hypothalamus futhi pituitary indlala. Kulokhu-ke eyokuqala ngokungalungile ukhiqiza ukukhulula isici. Yena, kusiza ukunciphisa ukukhiqizwa gonadotropin kule ndlala pituitary. Ngakho-ke, ukusebenza evamile gonads kusuka kube nzima.

Izimbangela ukuthuthukiswa isifo

Kallman syndrome - isifo esidluliselwa ngofuzo kungenziwa senziwa lutho. Imbangela eyinhloko sifo uxhumano okungalungile lamaseli owesilisa nowesifazane ngesikhathi ebamba isisu. Okungukuthi, e kwesinye sezitokisi futhi aqukethe "ile" ezakhi zofuzo enikeza awokuthi ukubonakaliswa kwaleso sifo ezinganeni.

Ngokwemvelo, Ukuwohloka izinga kwezakhi ikhenela okuthintekayo iziphi izici zangaphandle. The main wabo isimo kwemvelo. ukuvezwa kwesikhathi eside amakhemikhali angase futhi kuguqulwe izakhi zofuzo ezihlukahlukene. Kallman syndrome kungaba ukuzibonakalisa ngokukhanyayo ivezwa, noma ngeziphambeko encane evela kuyinsakavukela.

symptomatology lesi sifo

Omelelwa isifo inezimpawu ezithile, ngakho-ke kuyinto esihlukile kalula kusuka kwamanye pathologies. Uma isiguli kutholakala Kallman Syndrome, izimpawu zingase zihlanganise:

• ukuthomba Akuphelele. Ngezinye izikhathi iyivelakancane ngokuphelele. Ngokwesibonelo, amadoda wabonisa inani elincane wamasende - 3 kuphela ml (ngokuvamile kufanele kube 12 ml).
• lesikhumba Hyperplastic futhi ezibuthakathaka scrotum.
• A hypoplasia wawubona wenduku kanye endlaleni engasesinyeni.
• Distribution kwezicubu adipose njengomuntu wesifazane.
• ukungabi ngokugcwele noma kancane ngezici ngokobulili yesibili. Ngokwesibonelo, kungenzeka ukuthi amadoda ulahlekile izinwele emzimbeni.
• passivity ngokocansi.
• kombungu System: ulwanga emfantwini, lip emfantwini, gothic esibhakabhakeni.
• Ukungakwazi azibonayo iphunga. Ngakho ukunambitha bezibona uhlala.

Lezi zimpawu singawathola noma kakhulu ababuthakathaka. Konke kuncike degree of engavamile hypothalamus futhi pituitary.

ukuhlola Izici

Kallman syndrome (izithombe iziguli okufakiwe kuze kube izinsiza ezikhethekile ngezihloko yezokwelapha, bonisa ukuhlukahluka osobala Ukuklanywa komzimba besilisa) akusona isifo evame kakhulu. Nokho, ukuze aphathe ekuxilonga kufanele kube ukunakekela okukhethekile. It sihlinzekela ukuqaliswa izenzo ezinjalo:

1. Ihlola amazinga ayisisekelo hormone emzimbeni: estradiol, testosterone, prolactin, ozokusebenzisa. Hlola isikhathi eside futhi kuyinkomba. Kunenkolelo okungenani ngesonto.
2. ucwaningo engeziwe kuzosiza ochwepheshe ukuhlukanisa sifo kusukela ukubambezeleka ngokomthethosisekelo ukuthuthukiswa ngokobulili.
3. Hlola luvelo iphunga. Lokhu kwenziwa izinto kakhulu ukumane, isiguli kunikezwa (insipho, amakha), kube luqhatshi eqinile. Ngokusho kocwaningo uyaphetha: umqondo ejwayelekile iphunga abantu noma cha.
4. US izinso kanye testis.
5. MRI we pituitary futhi ngxenye. Ngaphezu kwalokho, phakathi tomograph luhlolo kuyadingeka ukuze unaka noma ikuphi izimila ekhanda ukuthi kungabangela ukubonakala izimpawu zesifo ezichazwe ngenhla.
6. Ukuqoqa umlando womndeni. Kusukela zokugula yetfulwe ngofuzo, ukuthola ulwazi mayelana amacala lesi sifo emkhayeni lesiguli kuyokwenzeka ukuletha ukusazi ngokunembile isifo esikuphethe kakhulu.

Izici yokwelashwa

Njengoba lesi sifo zofuzo, khona-ke ayibulale ngeke kwenzeke. Nokho, kumelwe ukwelashwa eyenziwa. Ngokushesha ngemva kokuvela kwaleso sifo, isiguli iqondiswa testosterone. Inqubo kwenziwa ezinyangeni ezimbalwa.

Ngenxa ezifana yokwelapha ochwepheshe ingafinyelela ngcono isimo lesiguli jikelele impilo, ukuthuthukisa isifiso sobulili. Ngaphezu kwalokho, verilizatsiya izuzwe. Ngemva kwalokho, odokotela bayaqhubeka yokwelapha, okuhloswe ngaso ekubuyiseleni kungenzeka isizukulwane kwesidoda ovuthiwe. Kulokhu, wawungasasebenzi testosterone futhi gonadotropins.

Qaphela ukuthi uma indoda inezinwele kwatholakala ukuthi une-"Kálmán syndrome", ukuphathwa kuyodingeka ukuqhuba ukuphila. Izosiza nokugcinwa izici ngokobulili yesibili kanye nokubuyiselwa waphila ukuphila okuvamile. Nokho, uma isiguli nale Ihlahlubo ngeke alinge unezingane, kufanele ngaso sonke isikhathi xhumana zofuzo. Ingozi yokuba ingane nge zokugula efanayo uhlala eliphezulu kakhulu. Ngo lonke - ne kancane izimpawu kanye nokwelashwa okufanele - isiguli Ungaphila impilo evamile kakhulu.

Ngokuphathelene zokuvimbela, yavusa alikho. Ngokwemvelo, kuyinto efiselekayo ukugwema lezo zici engaholela "break" ezakhi zofuzo. Hlala uphilile!