Xa i-Hypothalamus yakho ayinakukwazi ukusebenza ngokufanelekileyo
I-Kallmann isifo sithinta amadoda ngokuphindaphindiweyo kunabesifazana, okuchaphazela malunga ne-1 ukuya kuma-8 000 ukuya ku-10,000 amadoda kunye no-40 kumawaka angama-40,000 ukuya kuma-70,000 amabhinqa emhlabeni jikelele.
I-Kallmann syndrome inokufumana ilifa okanye ingenzeka kumntu ongenalo nentsapho yembali yesifo. Ifom yefowuni eqhelekileyo ifom yefom ye-X edibeneyo . I-gene ye-KAL1 kwi-X ye-chromosome ityala le fomu.
Ifom elandelayo ezuzekileyo ifa ngenxa yokuguqulwa kwesigqabi se-KAL2 kwi-chromosome 8. Ifa ilifa kwi- autosomal indlela ephezulu . Ifomu lesithathu elizuze ilifa le-Kallmann syndrome libangelwa ukuguqulwa kwegciwane le-KAL3 kwaye ilifa njenge- autosomal disorder. Indawo ekhoyo yegciwane le-KAL3 ayifumananga.
Iimpawu
Kwi-Kallmann syndrome, inxalenye yengqondo, i-hypothalamus, ayikwazi ukusebenza ngokufanelekileyo. Oku kubangela iimpawu ezibandakanya:
- ukungaphumeleli ekugqibeleni
- akukho mvakalelo yokunuka (anosmia) okanye ubunamandla obuthakathaka kakhulu bokunuka (hyposmia)
- iimvavanyo ze-cryptorchidism ezingamadoda
- i-penis encinci ( microphallus ) kwindoda
- Ukuya esikhathini akuzange kuqale kwabesetyhini (ekuthiwa yi-amenorrhea yokuqala)
- Ukuhamba kwezandla zombini ngexesha elifanayo (elibizwa ngokuthi i-bimanual synkinesis) kuthintela malunga ne-5 kwindoda enesifo
Kukho ezinye iimpawu ezenzeka rhoqo ngaphantsi, ezifana nokuzalwa ngesifo esisodwa kuphela okanye ukuba ne-osteoporosis (amathambo abuthathaka).
Ukuxilongwa
Ukuba umntu uhluleka ukuhamba ebusheni, uyabhekiswa kwi-endocrinologist yokuxilongwa nokunyamekela. Olu hlobo logqirha lujolise kwiingxaki ze-hormone kwaye lunokucacisa ukuba kutheni ukungabikho kobutshatshaba akuzange kwenzeke.
Kukho izizathu ezininzi zokuba umntu angenakuhamba ngokutshatyalaliswa. Nangona kunjalo, i-Kallmann syndrome yodwa edibeneyo nekhono lokunuka.
Ngokuqhelekileyo kulula ukubona ukuba i-Kallmann syndrome ingaba khona ngokuqhuba uvavanyo lwevumba.
Kukho ezimbini iindidi zovavanyo lwevumba. Omnye usebenzisa amabhodlela amancinci anezinto ezahlukeneyo kuzo; olunye uvavanyo lisebenzisa "ukukhahlela kunye nokukhwaza" amakhadi. Zomibini iimvavanyo zisebenzisa izinto ezinomsila onamandla abantu abaninzi abazi, njengekhofi. Ukuba umntu ovavanywa unomqondo oqhelekileyo wokuvumba, ke enye ingxaki ngaphandle kwe-Kallmann syndrome ikhona.
Uvavanyo olubonakalayo luya kubonisa ukuba ngaba iimvavanyo zeengxaki ezingenakunqwenela okanye izitho zangasese ezincinci zikhoyo kwindoda. Imbali yentsapho ye-Kallmann syndrome iya kuba yinto ebalulekileyo yokuxilongwa. Ukuhlolwa kwegazi kwenziwa ukwenzela ukulinganisa amazinga okumisa i-hormone (LH) kunye ne-follicle-stimulating hormone (FSH), kunye ne-testosterone okanye i-estrogen. Zonke ezi i-hormone zibalulekile ekuphuculweni ngokwesondo ngokuqhelekileyo. Amanqanaba aphantsi kakhulu a ma hormone athetha ukuba kukho ingxaki kunye ne-hypothalamus okanye i-pituitary gland kwingqondo.
Kwi-Kallmann syndrome, ukungabi nento yokuphofisa kubangelwa ukungabikho kwezakhiwo ezibizwa ngama-bulfactory bulbs kwingqondo. Ukukhangela ukukhenketha kwe-imagination (MRI) yentloko kunokubonisa ukuba ngaba izakhiwo zikhoyo okanye cha.
Unyango
Ukwelashwa kwe-Kallmann syndrome kugxininisa ekutshintsheni amahomoni alahlekileyo.
Kubantu, mhlawumbi i-testosterone okanye i-hCG inikwe umjovo kwishedyuli. Kubafazi, iipilisi ze-estradiol zithathwa imihla ngemihla. Iipilisi zeprogesterone nazo zithathwa imihla ngemihla kwiintsuku ezili-14 zokuqala kwenyanga nganye ukudala umjikelezo wesini. Enye indlela yokuthatha ezimbini iintlobo zamathebhulethi usebenzisa iipilisi zokukhusela eziqukethe i-estrogen kunye neprogesterone. Kwababini nabasetyhini, ezinye iindlela zonyango zifumaneke ukungabikho komntwana. Uphulo olufanelekileyo lwabantu abane-Kallmann syndrome lubavumela ukuba bafikelele kwimpilo eqhelekileyo yokuzala.
Imithombo:
> AbuJbara, Mousa, Hanan Hamamy, Nadim Jarrah, > Nadima >> Shegem >, & Kamel Ajluni. "Iiklinikhi kunye nelifa leenkcukacha ze-Kallmann syndrome eJordan." Impilo yokuzala 1 (2004): iPub.
> "Kallmann Syndrome." Isiseko seDisase Database. I-National Organisation for Distress Disorders. 6 Feyi 2008
> Saunders, Mark. "Ukuqonda i-Kallmann's Syndrome - Iimpendulo Zemibuzo Yakho." HYPOHH.net. 1997. HYPOHH.net. 6 Feyi 2008