Maonekedwe a chiwerewere ndi apachilendo, mahomoni ali ndi udindo, ndipo zina mwazimene zimapangidwa m'matenda a adrenal. Pali matenda obadwa nawo omwe amadziwika ndi kusagwirizana kwa mapuloteni oterewa komanso kutuluka kwa androgens. Kuchuluka kwa mahomoni amtundu wamwamuna mu thupi kumabweretsa kusintha kwakukulu mu thupi la thupi.
Adrenogenital Syndrome - Zimayambitsa
Matenda omwe amalingalira amachokera ku chibadwidwe cha chibadwa cha genetic mutation. Kawirikawiri sapezeka, matenda a adrenogenital ndi 1 vuto pa 5000-6500. Kusintha kwa ma geneti kumapangitsa kuwonjezeka kwa kukula ndi kuwonongeka kwa adrenal cortex. Kupangidwa kwa michere yapadera yomwe ikugwira nawo ntchito yopanga cortisol ndi aldosterone yafupika. Kuperewera kwawo kumapangitsa kuwonjezeka kwa mahomoni achiwerewere.
Matenda a Adrenogenital - Mndandanda
Malingana ndi mlingo wa kukula kwa adrenocortical ndi kuopsa kwake kwa zizindikiro, matendawa akupezeka m'mitundu yosiyanasiyana. Mitundu ya matenda a adrenogenital:
- mchere-kutaya;
- chosavuta (chophweka);
- pos-pubertal (nonclassical, atypical).
Matenda a Adrenogenital - mawonekedwe a mchere
Mtundu wambiri wa matenda, umene umapezeka mwa ana obadwa kumene kapena ana a chaka choyamba cha moyo. Ndi mtundu wodwala wamchere wa adrenogenital, mphamvu ya mahomoni imasokonezeka ndipo ntchito ya adrenal cortex imakhala yochepa. Matenda amtunduwu amatsagana ndi aldosterone. Ndikofunika kusunga mchere wa madzi m'thupi. Matenda a adrenogenital amachititsa kuphwanya ntchito ya mtima ndikudumpha m'magazi. Izi zimachitika motsutsana ndi msinkhu wa kusungunula mchere mu impso.
Matenda a Adrenogenital ndi mawonekedwe abwino
Kusiyana kosavuta kapena kosiyana pakati pa kachitidwe ka matenda sikukuphatikiza ndi zochitika zosavomerezeka. Matanthauzo a adrenogenital syndrome (ACS mafilimu mawonekedwe) amatsogolera kusintha kokha kumtundu wakunja. Matenda amtundu umenewu amapezeka kuti ali aang'ono kapena atangobereka kumene. M'kati mwa njira yoberekera imakhala yachilendo.
Matenda a adrenogenital postpubertate
Mtundu uwu wa matenda umatchedwanso wotchuka, wopangidwa ndi wosakhala wachikale. Matenda a adrenogenital amenewa amapezeka mwa amayi omwe ali ndi moyo wogonana. Chifukwa cha kukula kwa matenda angakhale onse obadwa mwa majini, ndi chifuwa cha adrenal cortex . Matendawa nthawi zambiri amakhala ndi matenda osabereka, choncho popanda mankhwala okwanira, matenda a adrenogenital komanso mimba ndizosiyana. Ngakhalenso pokhapokha atakhala ndi pakati, maopsezo a kuperewera padera ndi aakulu, mwanayo amafa ngakhale kumayambiriro (masabata 7-10).
Matenda a Adrenogenital - zizindikiro
Chithunzi cha kachipatala cha zolakwika za genetic zikugwirizana ndi msinkhu komanso mtundu wa matendawa. Matenda a Adrenogenital m'mimba mwachindunji nthawi zina sangazindikire, chifukwa cha zomwe mwana wamwamuna amatha kuzizindikira molakwika. Zizindikiro zenizeni za matendawa zimaonekera kuchokera pa zaka 2 mpaka 4, nthawi zina zimawonetseratu mtsogolo, msinkhu kapena kukula msinkhu.
Matenda a Adrenogenital kwa anyamata
Ndi matenda omwe amatha kutaya mchere, zizindikiro za mchere wa madzi zimasokonezeka:
- kutsekula m'mimba;
- kusanza kwakukulu;
- kuthamanga kwa magazi;
- phokoso;
- tachycardia;
- kulemera.
Matenda ovuta a adrenogenital pakati pa ana aamuna ali ndi zizindikiro zotsatirazi:
- mbolo yowonjezereka;
- kupweteka kwa khungu khungu ka scrotum;
- mdima wakuda pafupi ndi anus.
Anyamata obadwa kumene samapezeka kawirikawiri chifukwa chithunzi chachipatala ali wamng'ono chimafotokozedwa bwino. Pambuyo pake (kuyambira zaka 2) adrenogenital syndrome imaonekera kwambiri:
- kukula kwa tsitsi pa thupi, kuphatikizapo ziwalo zoberekera;
- mawu otsika, owopsa;
- hormonal acne ( acne vulgaris);
- masculinization;
- kupititsa patsogolo fupa;
- msinkhu.
Matenda a Adrenogenital kwa atsikana
Kutanthauzira matenda omwe amaganiziridwa mwa ana aakazi ndi osavuta, amatsatiridwa ndi zizindikiro zotere:
- hypertrophic clitoris, yomwe imawoneka ngati mbolo;
- lalikulu labiya, amawoneka ngati chotupa;
- chiberekero ndi mitsempha zimagwirizanitsidwa kukhala uchirogenital sinus.
Posiyana ndi zizindikiro za makanda, atsikana nthawi zina amalakwitsa anyamata ndipo amaleredwa molingana ndi kugonana kolakwika. Chifukwa cha izi, kusukulu kapena msinkhu, ana awa nthawi zambiri amakhala ndi mavuto a maganizo. M'kati mwa chiberekero cha msungwana wa msungwanayo mofanana ndi mawonekedwe a akazi, ndicho chifukwa chake amadzimverera kuti ndi mkazi. Mwanayo amayamba kutsutsana ndi mavuto ndi kusintha kwa anthu.
Pambuyo pa zaka ziwiri, congenital adrenogenital syndrome imakhala ndi zizindikiro zotsatirazi:
- kukula msanga kwa ubweya wa pubic ndi zipsera;
- miyendo yochepa ndi manja;
- mchitidwe;
- maonekedwe a tsitsi la nkhope (ndi zaka 8);
- thupi la munthu (mapewa akulu, mapepa apang'ono);
- kusowa kwa kukula kwa mapira a mammary;
- Kukula kwakukulu ndi msambo waukulu;
- mawu amwano;
- chithandizo;
- kumayambiriro kumapeto kwa msambo (palibe kale kuposa zaka 15-16);
- kusinthasintha kosasinthasintha, kuchedwa kawirikawiri kumwezi;
- amenorrhea kapena oligomenorrhea;
- kusowa;
- kuthamanga kwa magazi kudumpha;
- mimba yambiri ya epidermis.
Matenda a Adrenogenital - matenda
Maphunziro a sayansi ndi ma laboratory amathandiza kuzindikira hyperplasia ndi kusagwirizana kwa adrenal cortex. Kuti mudziwe kuti adrenogenital congenital syndrome ndi ana, kufufuza bwino mazira ndi kuwerengera tomography (kapena ultrasound) kumachitika. Kufufuza kwa hardware kumatha kuzindikira mazira ndi chiberekero mwa atsikana ndi ziwalo zoberekera.
Pofuna kutsimikizira kuti matendawa ndi otani, kafukufuku wa laboratori wa matenda a adrenogenital akuchitika. Zimaphatikizapo kuphunzira mkodzo ndi magazi pa mahomoni:
- 17-OH-progesterone;
- pulogalamu;
- cortisol;
- 17-ketosteroids.
Kuonjezeredwa:
- biochemistry ;
- kuyesa magazi;
- Mtsinje waukulu.
Kuchiza kwa matenda a adrenogenital
N'zosatheka kuchotsa machitidwe a ma genetic, koma mawonetseredwe ake a kuchipatala akhoza kuthetsedwa. Matenda a Adrenogenital - Zokuthandizani kuchipatala:
- Moyo wothandizira mankhwala osokoneza bongo. Kuonetsetsa kuti ntchito ya adrenal cortex ikuyendetsedwa bwino ndikuyendetsa bwino tsamba la endocrine, muyenera kumamwa mowa glucocorticoids. Njira yosankhika ndi Dexamethasone. Mlingo umawerengedwa payekha ndipo umakhala wochokera ku 0.05 mpaka 0.25 mg pa tsiku. Ndi mchere wotaya mchere, ndikofunika kutenga mchere wa corticoids kuti musunge mchere wa madzi.
- Kukonzekera kwa maonekedwe. Odwala omwe ali ndi matendawa akulimbikitsidwa kukhala ndi pulasitiki ya vaginal, clitorectomy ndi njira zina zochitiramo opaleshoni kuti ziwalozo zikhale ndi mawonekedwe abwino komanso kukula.
- Kuyankhulana nthawi zonse ndi katswiri wa zamaganizo (pa pempho). Odwala ena amafunika kuthandizidwa pa kusintha kwa anthu ndi kuvomereza okha ngati munthu wamphumphu.
- Kulimbikitsidwa kwa ovulation. Azimayi omwe akufuna kutenga pakati ayenera kupita kuchipatala chokhazikika kuti athe kukonzekera kusamba ndi kuchotsedwa kwa torogen. Glucocorticoids imatengedwa nthawi yonse ya mimba.