Uma i-Hypothalamus Yakho Ihluleka Ukusebenza Ngokufanele
I-Kallmann syndrome ithinta amadoda kaningi kunabesifazane, ethinta cishe 1 ku-8,000 kuya ku-10 000 abesilisa nabangu-1 ku-40,000 kuya ku-70,000 abesifazane emhlabeni wonke.
I-Kallmann syndrome ingase izuzwe njengefa noma ingenzeka kumuntu ongenalo umlando womndeni wesifo. Ifomu elivame kakhulu njengefa elihlobene ne- X. Isakhi se-KAL1 kwi-X chromosome sinomthwalo wale fomu.
Ifomu elilandelwayo elilandelwayo elilandelayo elidlula zonke ngenxa yezinguquko esakhiweni se-KAL2 ku-chromosome 8. Izuzwe njengefa elikhulu kakhulu. Ifomu lesithathu elizuzwe njengefa le-Kallmann syndrome libangelwa ukuguqulwa kwesakhi se-KAL3 futhi ifa njenge- disosomal disorder. Indawo eqondile yesakhi se-KAL3 asikatholakali.
Izimpawu
E-Kallmann syndrome, ingxenye yengqondo, i-hypothalamus, ayikwazi ukusebenza kahle. Lokhu kubangela izimpawu ezifaka:
- ukwehluleka ukungena ekukhuleni
- akukho ukuzwa iphunga (ismia) noma ikhono elibuthakathaka kakhulu lokunuka (hyposmia)
- Izivivinyo ezingenakunqunywa (cryptorchidism) kumadoda
- i-penis encane ( microphallus ) kubesilisa
- Ukuya esikhathini akuqali kwabesifazane (okuthiwa i-amenorrhea eyinhloko)
- Ukunyakaza kokubili kwezandla zombili (okuthiwa i-bimanual synkinesis) kuthinta cishe umuntu oyedwa-wesihlanu wabesilisa abane-disorder
Kunezinye izimpawu ezenzeka kaningi kangako, njengokuzalwa ngezinso ezilodwa kuphela noma ukuphefumula kwamathambo (amathambo abuthakathaka).
Ukuxilongwa
Uma umuntu ehluleka ukuphuma ebusheni, uhanjelwa kudokotela we-endocrinologist for diagnosis and care. Lolu hlobo lodokotela lugxile ekuhluphekeni kwe-hormone futhi lunganquma ukuthi kungani ukukhulelwa kungenzeki.
Kunezizathu eziningi zokuthi kungani umuntu engase angene ekukhuleni. Kodwa-ke, i-Kallmann syndrome yiyona kuphela ehlobene nokungakwazi ukuhogela.
Ngokuvamile kulula ukubona ukuthi i-Kallmann syndrome ingaba khona ngokuhlola iphunga.
Kunezinhlobo ezimbili zokuhlobisa iphunga. Omunye usebenzisa amabhodlela amancane anezinto ezihlukile kuzo; olunye uhlolo lusebenzisa "amakhadi okubamba futhi aphefumule". Zombili izivivinyo zisebenzisa izinto ezinomsoco oqaphela wonke umuntu owaziyo, njengekhofi. Uma umuntu ovivinywa enomqondo ojwayelekile wokuhogela, khona-ke esinye isifo ngaphandle kwe-Kallmann syndrome sikhona.
Ukuhlola okungokwenyama kuzokukhombisa ukuthi ngabe ama-testes angenasidingo noma ama-genitals amancane ayenesilisa. Umlando womndeni we-Kallmann syndrome uzoba yindlela ebalulekile yokuxilongwa. Kuhlolwa ukuhlolwa kwegazi ukukala amazinga we-hormone luteinizing (LH) ne-follicle-stimulating hormone (FSH), kanye ne-testosterone noma i-estrogen. Zonke lezi hormone zibalulekile ekuthuthukiseni ngokocansi okujwayelekile. Amazinga aphansi kakhulu ala mahomoni asho ukuthi kukhona inkinga nge-hypothalamus noma i-pituitary gland ebuchosheni.
E-Kallmann syndrome, ukungabi nomuzwa wokuhogela kungenxa yokungabikho kwezakhiwo ezibizwa ngokuthi ama-olfactory ama-brain ebuchosheni. Ukuskena kwe-imagination ye-resonance imaging (MRI) yekhanda kungabonisa ukuthi lezi zakhiwo zikhona noma cha.
Ukwelapha
Ukwelashwa kwe-Kallmann syndrome kugxile ekwenzeni i-hormone engekho.
Kwabesilisa, i-testosterone noma i-hCG inikwe umjovo esimisweni. Kwabesifazane, amaphilisi e-estradiol athathwa nsuku zonke. Amaphilisi e-progesterone nawo athathwa nsuku zonke ngezinsuku ezingu-14 zokuqala zenyanga ngayinye ukudala umjikelezo wesimiso. Enye indlela yokuthatha izinhlobo ezimbili zamathebulethi usebenzisa amaphilisi okuvimbela ukuzala okuqukethe i-estrogen ne-progesterone. Kwabesilisa nakwabesifazane, ezinye izindlela zokwelapha zitholakalayo ngenxa yokungasebenzi. Ukuphathwa kahle kwabantu abane-Kallmann syndrome kuvumela ukuba bathole impilo evamile yokuzala.
Imithombo:
> AbuJbara, Mousa, Hanan Hamamy, Nadim Jarrah, > Nadima >> Shegem >, & Kamel Ajlouni. "Amaphrofayli omtholampilo kanye nefa le-Kallmann syndrome eJordani." Impilo Yokuzala 1 (2004): iPub.
> "Kallmann Syndrome." Database Darease Database. Inhlangano Kazwelonke Yezinkinga Ezinzima. 6 Feb 2008
> Saunders, uMark. "Ukuqonda i-Kallmann's Syndrome - Izimpendulo Zemibuzo Yakho." HYPOHH.net. 1997. HYPOHH.net. 6 Feb 2008