Ngaba kukho umntu kusapho lwakho, ingakumbi umntwana, onengxaki yehomoni yokuzalwa? Usenokuba uqhelene neengxaki ezifana nokulibaziseka kokukhula, ukufikisa kwangethuba, kunye nemijikelo yokuya exesheni engaqhelekanga kwabasetyhini. Imeko engaqhelekanga yemfuza enokubangela oku ibizwa ngokuba yi-'Congenital Adrenal Hyperplasia'. Siza kuyibiza ngokuba yi-CAH ngamafutshane. Namhlanje, siza kuthetha ngokufutshane ngale nto kunye nonyango olutsha oluye lwavela ngayo.
Ngamafutshane, yintoni i-CAH?
I-CAH yimeko yemfuza ethintela amadlala e-adrenal, amadlala amabini amancinci abekwe ngaphezulu kwezintso, ekuveliseni i-cortisol, i-hormone ebalulekileyo ebomini bethu.
Cinga ngemizimba yethu njengomzi-mveliso. Xa enye yeemveliso (i-cortisol) kule mveliso iphelelwa ngamandla, iziko lolawulo ebuchotsheni (i-pituitary gland) liyaphazamiseka lize lithumele imiqondiso (ACTH) kumadlala e-adrenal ukuba asebenze nzima. Ngenxa yolu khuthazo lugqithisileyo, nangona imveliso ye-cortisol inganyuki, endaweni yoko iqala ukuvelisa iihomoni ezininzi zamadoda ezibizwa ngokuba yi -'androgens' .
Oku kungalingani kwe-hormone kunokubangela iingxaki ezininzi:
- Iintsana ezingamantombazana: Xa zizalwa, amalungu esini angaphandle anokubonakala ngathi ngawomntwana oyinkwenkwe.
- Kumacala omabini: iingxaki ezinje ngokulibaziseka ekukhuleni, ukufikisa kwangethuba, kunye nokungafumani bantwana zinokwenzeka kamva ebomini.
Ukongeza, abanye abaguli be-CAH basenokuba nokuveliswa okunciphileyo kwenye i-hormone ebalulekileyo ebizwa ngokuba yi-aldosterone. Ngaphandle kwale hormone, i-CAH inokuba yingozi kakhulu, nto leyo ekhokelela ekuthukeni, ekuqhawukeni, okanye ekufeni.
Unyango lweminyaka engama-70 kunye nethemba elitsha
Kangangamashumi eminyaka, unyango olulodwa lwe-CAH yayikukutshintsha amanqanaba aphantsi e-hormone cortisol emzimbeni ngeedosi eziphezulu ze-steroids , ezifana ne-hydrocortisone. Nangona kunjalo, ukuthatha ezi dosi ziphezulu ze-steroids ixesha elide kunokubangela iziphumo ezininzi ebezingalindelekanga. Ezona zixhaphakileyo zezi kukwanda kobunzima, uxinzelelo lwegazi oluphezulu, amathambo abuthathaka, kunye nokukhula okungalindelekanga ebantwaneni.
Kodwa ngoku kukho iindaba ezimnandi! Emva kweminyaka engama-70, i-FDA ivumile iyeza lokuqala nelokuphela kwalo elenzelwe ngokukodwa i-CAH, i-crinecerfont (igama lophawu: iCrenessity).
Isebenza njani le chiza litsha (iCrinecerfont)?
Le yeyona nto ibalulekileyo. Ngokungafaniyo nonyango lwakudala, eli yeza litsha lisebenza ngendlela eyahlukileyo ngokupheleleyo.
I-'Crinesurfont' ayongezi iihomoni ezivela ngaphandle. Endaweni yoko, eli yeza liya ngqo kwiziko lolawulo lobuchopho (i-pituitary gland) kwaye lithintele ukukhululwa kwesignali engafunekiyo (ACTH) evuselela ii-adrenal glands.
Ngamafutshane, oku kufana nokucima 'iswitshi' yenkqubo engeyomfuneko. Oku kulawula ukuveliswa komzimba kweehomoni 'ze-androgen' ezingafunekiyo.
Eyona nzuzo inkulu yoku kukuba ekubeni amanqanaba e-androgen elawulwa, umthamo wee-steroids ezifunekayo ukugcina ibhalansi yeehomoni unokuncipha kakhulu . Oku kuthetha ukuba iziphumo ebezingalindelekanga ezibangelwa zii-steroids nazo zinokuncitshiswa kakhulu.
| Uphawu | Unyango lwakudala (iidosi eziphezulu ze-steroid) | Unyango olutsha (iCrinecerfont + iidosi eziphantsi ze-steroid) |
|---|---|---|
| Ukusebenza kakuhle | Ukubonelela nge-cortisol hormone enciphileyo ngaphandle. | Ukuthintela imiqondiso engafunekiyo (ACTH) engqondweni nokulawula ukuveliswa kwe-androgen. |
| Injongo ephambili | Iidosi eziphezulu ziyafuneka ukulawula amanqanaba eehomoni. | Ukunciphisa amanqanaba e-androgen kunye nokunciphisa umthamo wee-steroids ezifunekayo. |
| Imiphumela | Iingxaki zexesha elide ezifana nokutyeba, ukukhula okukhawulezileyo, kunye nobuthathaka bamathambo. | Kusenokwenzeka ukuba iziphumo ebezingalindelekanga eziphantsi ezinxulumene neesteroid. Iziphumo ebezingalindelekanga ezincinci (intloko ebuhlungu, ukudinwa) zibikwe. |
Ngubani ofanele eli yeza?
I-'Crinesurfont' okwangoku ivunyiwe kwizigulana ze-CAH ezingaphezulu kweminyaka emi-4 ubudala . Ayithathi indawo yee-steroids, kodwa yongezwa kwii-steroids.Unyango olongezelelweyo.
- Kubantwana: Ekubeni abantwana besakhula, iidosi eziphezulu zee-steroids zinokuba nefuthe elikhulu ekukhuleni kwabo. Ke ngoko, eli yeza, elinokunciphisa idosi yee-steroids, linokuba luncedo kakhulu kubo. Oku kubaluleke kakhulu ngexesha lokufikisa, xa kunzima ukulawula ii-hormone.
- Kwabantu abadala: Olu nyango lunokubonelela ngoncedo nakubantu abadala abanengxaki yeziphumo ebezingalindelekanga zokuthatha ii-steroids ixesha elide, okanye abanobunzima bokugcina amanqanaba e-hormone ezinzile.
Funda ngemiphumo emibi kunye nokhuseleko
Kwizilingo zeklinikhi, zimbalwa nje iziphumo ebezingalindelekanga ezincinci nezexeshana eziye zaxelwa ngeli yeza.
- Kwabantu abadala: ukudinwa, intloko ebuhlungu, isiyezi, iintlungu zamalungu.
- Kwabantwana: intloko ebuhlungu, isisu esibuhlungu, ukudinwa, impumlo exineneyo.
Okubalulekileyo kukuba uninzi lwezi mpawu bezincinci, kwaye inani lezigulana ekwafuneka ziyeke ukusebenzisa amayeza laliphantsi kakhulu.
Isilumkiso Esikhethekileyo: Lumka ngeNgxaki ye-Adrenal!
Xa usebenzisa eli yeza, kubaluleke kakhulu ukuqaphela imeko enzima ebizwa ngokuba yi-'adrenal crisis'. Ngamafutshane, le meko inokwenzeka ukuba umzimba awufumani isixa esifunekayo se-'cortisol' ngexesha loxinzelelo olukhulu (umz., usulelo olunzima, utyando, ingozi).
Ngoko ke, thetha nogqirha wakho kwaye uqiniseke ukuba uyazi kakuhle ukuba yeyiphi idosi eyongezelelweyo ye-steroid ekufuneka uyithathe xa kukho imeko engxamisekileyo ("idosi yoxinzelelo") . Kwakhona, qaphela iimpawu 'zengxaki ye-adrenal' (umz., ukuhlanza kakhulu, urhudo, isiyezi, ukulahlekelwa zingqondo) kwaye ukhumbule ukuya esibhedlele okanye kwigumbi likaxakeka (ETU) ngoko nangoko ukuba into enjalo iyenzeka.
Ndingazi njani ukuba eli yeza lifanelekile kum/kumntwana wam?
Esi sigqibo kufuneka senziwe ngokubonisana nogqirha wakho, ingakumbi ingcali ye-endocrinologist . Ugqirha wakho uza kuqwalasela izinto ezininzi, kuquka iziphumo zovavanyo lwakho lwegazi, izinga lokukhula, ubunzima, uxinzelelo lwegazi, kunye namanqanaba e-hormone akhoyo, ukuze amisele ukuba eli yeza lifanelekile na kuwe.
Olu asilonyango lwe-CAH, kodwa kukho ithemba elikhulu lokuba luya kunceda ukuphucula umgangatho wobomi bezigulane, lunciphise iziphumo ebezingalindelekanga ze-steroid, kwaye lwenze kube lula ukulawula iihomoni.
Umyalezo Wokuya Ekhaya
- I-CAH ithetha ukuthiniImeko yemfuza apho ii-adrenal glands zivelisa i-cortisol encinci kunye nee-androgen hormones ezininzi.
- I-Crinecerfont lunyango olutsha oluphuhliselwe ngokukodwa i-CAH, olokuqala lohlobo lwayo kwiminyaka engama-70.
- Eli asiloyeza elithatha indawo yee-steroids. Liyeza elinikwa ukongeza kwii-steroids kwaye linceda ekunciphiseni umthamo wee-steroids ezifunekayo.
- Inzuzo ephambili kukukwazi ukunciphisa iziphumo ebezingalindelekanga zexesha elide ze-steroids.
- Qiniseka ukuba ubuza ugqirha wakho ngemeko engxamisekileyo ebizwa ngokuba yi-'adrenal crisis' kunye "nomthamo woxinzelelo" omele uwuthathe ngelo xesha.
- Kubalulekile ukubonisana nogqirha ogxile kwizifo zamahomoni ukuze ubone ukuba olu nyango lufanelekile na kuwe okanye kumntwana wakho.
💬 Comments (0)
Akukho zimvo zithunyelweyo okwangoku. Faka uluvo lwakho apha okokuqala.
Faka uluvo lwakho