Kodi pali aliyense m'banja mwanu, makamaka mwana, amene ali ndi vuto la mahomoni obadwa nalo? Mwina mukudziwa mavuto monga kuchedwa kukula, kutha msinkhu msanga, komanso kusakhazikika kwa msambo mwa akazi. Matenda osowa omwe angayambitse izi amatchedwa 'Congenital Adrenal Hyperplasia'. Tidzawatcha mwachidule kuti CAH. Lero, tikambirana mwachidule za izi komanso chithandizo chatsopano chomwe chabwera chifukwa cha izi.
Mwachidule, kodi CAH ndi chiyani?
CAH ndi matenda a majini omwe amalepheretsa ma adrenal glands, omwe ndi ma glands awiri ang'onoang'ono omwe ali pamwamba pa impso, kuti asapange cortisol, mahomoni ofunikira kwambiri pa moyo wathu.
Taganizirani matupi athu ngati fakitale. Pamene chimodzi mwa zinthu (cortisol) mu fakitale iyi chatsika, malo olamulira muubongo (pituitary gland) amakwiya ndipo amatumiza zizindikiro (ACTH) ku ma adrenal glands kuti agwire ntchito molimbika. Chifukwa cha kusonkhezeredwa kotereku, ngakhale kuti kupanga cortisol sikukwera, m'malo mwake kumayamba kupanga mahomoni ambiri achimuna otchedwa 'androgens' .
Kusalinganika kwa mahomoni kungayambitse mavuto ambiri:
- Makanda achikazi: Akabadwa, ziwalo zoberekera zakunja zimatha kuwoneka ngati za mwana wamwamuna.
- Kwa onse awiri: mavuto monga kuchedwa kukula, kutha msinkhu msanga, komanso kusabereka angachitike mtsogolo.
Kuphatikiza apo, odwala ena a CAH angakhalenso ndi kuchepa kwa kupanga kwa mahomoni ena ofunikira otchedwa aldosterone. Popanda mahomoni awa, CAH ikhoza kukhala yoopsa kwambiri, zomwe zimapangitsa kuti munthu agwe ndi mantha, chikomokere, kapena imfa.
Chithandizo cha zaka 70 ndi chiyembekezo chatsopano
Kwa zaka zambiri, chithandizo chokha cha CAH chinali kusintha kuchuluka kwa cortisol m'thupi ndi kuchuluka kwa ma steroid , monga hydrocortisone. Komabe, kumwa ma steroid ambiri kwa nthawi yayitali kungayambitse zotsatirapo zingapo. Zofala kwambiri mwa izi ndi kunenepa kwambiri, kuthamanga kwa magazi, mafupa ofooka, komanso kukula movutikira kwa ana.
Koma tsopano pali nkhani yabwino! Patatha zaka 70, FDA yavomereza mankhwala oyamba komanso okhawo omwe adapangidwa makamaka a CAH, crinecerfont (dzina la kampani: Crenessity).
Kodi mankhwala atsopanowa (Crinecerfont) amagwira ntchito bwanji?
Ichi ndiye chinthu chofunikira kwambiri. Mosiyana ndi mankhwala akale, mankhwala atsopanowa amagwira ntchito mosiyana kwambiri.
'Crinesurfont' siwonjezera mahomoni ochokera kunja. M'malo mwake, mankhwalawa amapita mwachindunji ku malo olamulira ubongo (pituitary gland) ndikuletsa kutulutsidwa kwa chizindikiro chosafunikira (ACTH) chomwe chimalimbikitsa ma adrenal glands.
Mwachidule, izi zili ngati kuzimitsa 'switch' ya dongosolo losafunikira. Izi zimalamulira kupanga kwa mahomoni osafunikira a 'androgen' m'thupi.
Ubwino waukulu wa izi ndikuti popeza kuchuluka kwa androgen kumayendetsedwa, mlingo wa ma steroid ofunikira kuti mahomoni azikhala bwino ukhoza kuchepetsedwa kwambiri . Izi zikutanthauza kuti zotsatira zoyipa zomwe zimayambitsidwa ndi ma steroid zimathanso kuchepetsedwa kwambiri.
| Khalidwe | Chithandizo chakale (mankhwala ambiri a steroid) | Chithandizo chatsopano (Crinecerfont + mlingo wochepa wa steroid) |
|---|---|---|
| Magwiridwe antchito | Kupereka hormone yochepetsedwa ya cortisol kunja. | Kuletsa zizindikiro zosafunikira (ACTH) kuchokera muubongo ndikuwongolera kupanga kwa androgen. |
| Cholinga chachikulu | Mlingo wokwera umafunika kuti muchepetse kuchuluka kwa mahomoni. | Kuchepetsa milingo ya androgen ndi kuchepetsa mlingo wa ma steroids kumafunika. |
| Zotsatirapo zoyipa | Mavuto a nthawi yayitali monga kunenepa, kulephera kukula bwino, komanso mafupa ofooka. | Kuthekera kwa kuchepetsa zotsatira zoyipa zokhudzana ndi steroid. Zotsatirapo zazing'ono (kupweteka mutu, kutopa) zanenedwa. |
Kodi mankhwala awa ndi abwino kwa ndani?
'Crinesurfont' pakadali pano yavomerezedwa kwa odwala a CAH azaka zopitilira 4. Siyolowa m'malo mwa ma steroids, koma yowonjezera ma steroids.Chithandizo chowonjezera.
- Kwa ana: Popeza ana akukulabe, kumwa mankhwala ambiri a steroid kungathandize kwambiri pakukula kwawo. Chifukwa chake, mankhwalawa, omwe amachepetsa mlingo wa steroid, angathandize kwambiri kwa iwo. Izi ndizofunikira kwambiri makamaka paunyamata, pamene zimakhala zovuta kulamulira mahomoni.
- Kwa akuluakulu: Mankhwalawa angathandizenso akuluakulu omwe amavutika ndi zotsatirapo zoyipa chifukwa chomwa mankhwala a steroids kwa nthawi yayitali, kapena omwe amavutika kukhala ndi mahomoni okhazikika.
Dziwani za zotsatirapo zake komanso chitetezo chake
Mu mayeso azachipatala, zotsatirapo zochepa zazing'ono komanso zakanthawi chabe zanenedwa ndi mankhwalawa.
- Kwa akuluakulu: kutopa, mutu, chizungulire, kupweteka kwa mafupa.
- Kwa ana: mutu, kupweteka m'mimba, kutopa, mphuno yotsekeka.
Chofunika kwambiri n’chakuti, zizindikiro zambirizi zinali zochepa, ndipo chiwerengero cha odwala omwe anasiya kumwa mankhwala chinali chochepa kwambiri.
Chenjezo Lapadera: Dziwani Mavuto a Adrenal!
Mukamamwa mankhwalawa, ndikofunikira kwambiri kudziwa za vuto lalikulu lotchedwa 'adrenal crisis'. Mwachidule, vutoli lingachitike ngati thupi sililandira 'cortisol' yofunikira panthawi ya nkhawa yayikulu (monga matenda oopsa, opaleshoni, ngozi).
Choncho, lankhulani ndi dokotala wanu ndipo onetsetsani kuti mukudziwa bwino mlingo wa steroid wowonjezera womwe muyenera kumwa pakagwa mwadzidzidzi ("mlingo wopsinjika") . Komanso, dziwani zizindikiro za 'vuto la adrenal' (monga kusanza kwambiri, kutsegula m'mimba, chizungulire, kutaya chidziwitso) ndipo kumbukirani kupita kuchipatala kapena kuchipinda chodzidzimutsa (ETU) nthawi yomweyo ngati zoterezi zitachitika.
Ndingadziwe bwanji ngati mankhwalawa ndi oyenera ine/mwana wanga?
Chisankhochi chiyenera kupangidwa pokambirana ndi dokotala wanu, makamaka katswiri wa matenda a endocrinologist . Dokotala wanu adzaganizira zinthu zambiri, kuphatikizapo zotsatira za mayeso anu a magazi, kuchuluka kwa kukula, kulemera, kuthamanga kwa magazi, ndi kuchuluka kwa mahomoni omwe alipo, kuti adziwe ngati mankhwalawa ndi oyenera kwa inu.
Iyi si mankhwala a CAH, koma pali chiyembekezo chachikulu kuti ithandiza kukonza moyo wa odwala, kuchepetsa zotsatira zoyipa za steroid, ndikupangitsa kuti kusamalira mahomoni kukhale kosavuta.
Uthenga Wopita Nawo Kunyumba
- CAH amatanthauzaMatenda a majini omwe ma adrenal glands amapanga cortisol yochepa komanso mahomoni ambiri a androgen.
- Crinecerfont ndi mankhwala atsopano opangidwa makamaka a CAH, oyamba a mtundu wake m'zaka 70.
- Iyi si mankhwala omwe amalowa m'malo mwa ma steroids. Ndi mankhwala omwe amaperekedwa kuwonjezera pa ma steroids ndipo amathandiza kuchepetsa mlingo wa ma steroids omwe amafunikira.
- Ubwino waukulu ndi kuthekera kochepetsa zotsatira zoyipa za steroid kwa nthawi yayitali .
- Onetsetsani kuti mwafunsa dokotala wanu za vuto ladzidzidzi lotchedwa 'adrenal crisis' ndi "mlingo wopsinjika maganizo" womwe muyenera kumwa panthawiyo.
- Ndikofunikira kufunsa dokotala yemwe ndi katswiri wa matenda a mahomoni kuti adziwe ngati chithandizochi chili choyenera kwa inu kapena mwana wanu.











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