Kwiimpawu zesondo eziprayimari nezesekondari, ii-hormone zinoxanduva, ezinye zazo ziveliswa kwiingqungquthela ze-adrenal. Kukho isifo esingazalwa esichazwe ngokungafezeki kwalezi zilonda ze-endocrine kunye nokukhululwa ngokugqithiseleyo kwe-androgens. Ukugqithisa kwe-hormone yesini emzimbeni kubangela ukuba utshintsho olukhulu kwisakhiwo somzimba.
I-Adrenogenital Syndrome - Izizathu
Ubuthakathaka obucatshungulwayo bubangelwa ukuguquka komzimba ozalwe ngumntwana. Akunqabile ukufunyaniswa, iziganeko ze-adrenogenital syndrome yile-1 kwi-5000-6500. Ukutshintsha kwikhowudi yemfuza kubangela ukwanda kwindinganiso kunye nokuchithwa kwe-correnx ye-adrenal. Ukuveliswa kwee-enzyme ezizodwa ezithatha inxaxheba ekuveliseni i-cortisol kunye ne-aldosterone kuncitshiswa. Ukungabikho kwabo kukhokelela ekunyuseni kwiinkxwaba zesini zesini.
I-Adrenogenital syndrome - isigaba
Ngokuxhomekeke kwinqanaba lokukhula kancinci kunye nobunzima beempawu, kuchazwa isifo kwiimeko ezininzi. Iifom ze-adrenogenital syndrome:
- ityuwa-ukulahleka;
- i-viril (elula);
- post-pubertal (nonclassical, atypical).
I-Adrenogenital syndrome - ifomu yetyuwa
Uhlobo oluqhelekileyo lwe-pathology, olufunyanwa kwiintsana okanye abantwana bonyaka wokuqala wokuphila. Ngohlobo lokulahlekelwa ngetyuwa lwe-adrenogenital syndrome, ibhalansi ye-hormone iyaphazamiseka kwaye umsebenzi we-correx ene-adrenal ayanele. Olu hlobo lwesifo luhambelana nokuxinwa kwe-aldosterone. Kubalulekile ukugcina umlenze wetyuwa wamanzi emzimbeni. I-adrenogenital syndrome ibangela ukuphulwa komsebenzi wentliziyo kunye nokuxhuma kwixinzelelo legazi. Oku kwenzeka ngokumalunga nemvelaphi yokuqokelela i-salts ezintso.
I-Adrenogenital syndrome yifom
Uhlobo olulula okanye oluqhelekileyo lwekhosi yokukhubazeka aluhambisani neziganeko zokungakwazi ukufumana izifo. I-adrenogenital syndrome echazwe (ifom ye-ACS viril) ikhokelela kuphela kutshintsho kwii-genitalia zangaphandle. Olu hlobo lwesifo lufunyenwe kwakhona kwintsana okanye emva kokubeleka. Ngaphakathi kwenkqubo yokuzala ihlala isesiqhelo.
Ifom ye-Postpubertate ye-adrenogenital syndrome
Olu hlobo lwesifo lubizwa ngokuba yi-atypical, efunyenwe kwaye engaqhelekanga. I-adrenogenital syndrome enjalo ibonakala kuphela kwabasetyhini abanomdla wobomi obusebenzayo. Isizathu sokuphuhliswa kwezilwanyana sinokuguquka komzimba ngokwasemzimbeni, kunye nesifo se-correl . Esi sifo sisoloko sihamba kunye nokungasebenzi, ngoko ke, ngaphandle kwonyango olwaneleyo, i-adrenogenital syndrome kunye nokukhulelwa ayingqinelaniyo. Ngaphandle kokuveliswa ngempumelelo, umngcipheko wokuphuphuma kwesisu uphakamileyo, umntwana uyabulawa ngisho naseziqaleni zokuqala (iiveki ezi-7-10).
I-Adrenogenital syndrome - iimpawu
Umfanekiso wekliniki wendlela echazwe ngayo i-genetic ihambelana nobudala kunye nesimo sesifo. I-Adrenogenital syndrome kwiintsana eziza kutshatyalaliswa ngamanye amaxesha ayinakunqunywa, ngenxa yokuba isondo lomntwana singakwazi ukuchonga ngokungalunganga. Izibonakaliso ezithile zempilo zibonakaliswa kwiminyaka emi-2-4, kwezinye iimeko zibonakaliswa kamva, ebusheni okanye ekukhuleni.
I-Adrenogenital syndrome kubafana
Ngetyuwa-ukulahlekelwa kweso sifo, ukuphazamiseka kwengxolo yeryuwa yamanzi kuyabonwa:
- isifo sohudo;
- ukuhlanza okukhulu;
- uxinzelelo lwegazi oluphantsi;
- zixhala;
- tachycardia;
- ukulahleka kwesisindo.
I-adrenogenital syndrome elula kubantwana besilisa inempawu ezilandelayo:
- ipenisi ekhulisiweyo;
- ukugqithisa kwesikhumba se-scrotum;
- i-epidermis emnyama ejikeleze i-anus.
Amakhwenkwe amakhwenkwe awanakuthi afunyanwe ngenxa yokuba umfanekiso weklinikhi useneminyaka engaphantsi. Kamva (ukususela kwiminyaka emi-2) i-adrenogenital syndrome ibonakala ngakumbi:
- ukukhula kweenwele emzimbeni, kubandakanywa nezitho zangasese;
- ilizwi eliphantsi, elibi;
- i-hormone acne ( acne vulgaris);
- u masculini;
- ukukhawuleza kwendlela yokwenza amathambo;
- lifutshane.
I-Adrenogenital syndrome kumantombazana
Ukuchaza ukugula okucatshangelwayo kwiintsana zamabhinqa kulula, kuhamba kunye neempawu ezinjalo:
- i-clitoris ye-hypertrophic, ekhangeleka kakhulu njengepenis;
- i-labia enkulu, ibonakala njenge-scrotum;
- ibhinqa kunye ne-urethra zidibaniswa zibe sinus urogenital.
Ngokwemvelaphi yeempawu zintsana, ngamantombazana ngamanye amaxesha aphosakeleyo ngamakhwenkwe aze akhuliswe ngokuhambelana nesini esingalunganga. Ngenxa yoko, esikolweni okanye ebusheni, aba bantwana kaninzi banengxaki yengqondo. Ngaphakathi kwenkqubo yokuzala yintombazana ihambelana ngokupheleleyo ne-female genotype, yingakho evakalelwa kukuba ngumfazi. Umntwana uqala ukungqubuzana kwangaphakathi kunye nobunzima ngokulungelelanisa kuluntu.
Emva kweminyaka emi-2, i-congenital adrenogenital syndrome ibonakaliswe yimpawu ezilandelayo:
- Ukukhula kwangaphambili kweenwele ze-pubic kunye neempondo;
- imilenze emfutshane kunye neengalo;
- i-muscularity;
- ukubonakala kweenwele zobuso (ngo-8 ubudala);
- yomzimba womntu (amahlombe avulekileyo, i-pelvis encinci);
- ukungabikho kokukhula kweentlanzi zamathambo;
- Ukukhula okuphantsi kunye nomsizi omkhulu;
- ilizwi elibi;
- isifo sesifo;
- ukuqala kwexesha lokuya esikhathini (kungekho ngaphaya kweminyaka eyi-15-16);
- umjikelo ongazinzileyo, ukulibaziseka ngokukhawuleza kwimihla;
- i-amorrhea okanye i-oligomenorrhea;
- ukungabikho;
- uxinzelelo lwegazi luya
- ukugqithisa ngokugqithisileyo kwe-epidermis.
I-Adrenogenital syndrome - ukuxilongwa
Izifundo ze-Instrumental kunye neelabhoratri zinceda ukufumanisa i-hyperplasia kunye nokungasebenzi kwe-correx ye-adrenal. Ukufumanisa i-adrenogenital congenital syndrome kwiintsana, uvavanyo olupheleleyo lwezitho zangasese kunye ne-tomography (okanye i-ultrasound) eyenziwa. Uvavanyo lwe-hardware luyakwazi ukubona ama-ovari kunye nesisu kwiintombazana kunye namalungu omzimba wesini.
Ukuqinisekisa ukuxilongwa kwesigxina, uhlalutyo lwebhoratri lwe-adrenogenital syndrome lwenziwa. Iquka ukuhlolisisa umchamo kunye negazi kwi-hormone:
- 17-OH-progesterone;
- aldosterone;
- cortisol;
- 17-ketosteroids.
Ukongezwa:
- biochemistry ;
- vavanyo lwegazi;
- uviwo oluqhelekileyo lomchamo.
Unyango lwe-adrenogenital syndrome
Akunakwenzeka ukukrazula i-pathology ye-genetic pathology, kodwa izibonakaliso zayo zekliniki zinokupheliswa. I-Adrenogenital syndrome - iingcebiso zeklinikhi:
- Ukufumana ixesha lokuphila kwamachiza e-hormonal. Ukuqhelanisa umsebenzi we-correnx ye-adrenal kwaye ulawule ukulinganisela kwe-endocrine, kuya kufuneka uhlale usela i-glucocorticoids. Inketho ekhethiweyo yiDexamethasone. Isisombululo sibalwa ngabanye kwaye sisuka kwi-0.05 ukuya kwi-0.25 mg ngosuku. Ngotyuwa-ukulahlekelwa uhlobo lwesifo, kubalulekile ukuthatha i-corticoids yamaminerali ukuze kugcinwe i-ityuwa yamanzi ityuwa.
- Ukulungiswa kwembonakalo. Izigulane ezine-diagnostic ezichazweyo zicetyiswa ukuba zibe neplastiki ye-vaginal, i-clitorectomy kunye namanye amanyathelo okucatshungulwa ukuqinisekisa ukuba izitho zangasese zineendlela ezichanekileyo kunye nobukhulu.
- Ukubonisana rhoqo kunye nengcali yengqondo (ngesicelo). Ezinye izigulana zifuna uncedo ekulungiseni uluntu kunye nokwamkelwa kwabo njengabantu abagciniweyo.
- Ukukhuthazwa kwe-ovulation. Abasetyhini abafuna ukukhulelwa badinga ukufumana iyeza ezizodwa eziqinisekisa ukulungiswa komjikelezo wesini kunye nokunciphisa imveliso ye-androgen. I-Glucocorticoids ithathwa kwixesha lokugaya.