Kana iwe kana mumwe munhu mumhuri yako aine chirwere chinonzi Congenital Adrenal Hyperplasia (CAH), iyi inhau inokosha zvikuru kwauri. Mushure memakore makumi manomwe, mushonga mutsva wechirwere ichi wabvumidzwa. Iyi itarisiro huru kune vanhu vanorarama neCAH. Saka ngatitarisei kuti mushonga mutsva uyu chii, kuti unoshanda sei, uye mabhenefiti awo ndeapi.
Kutanga, ngatinzwisisei, chii chinonzi CAH?
Zvichitaurwa zviri nyore, CAH chirwere chemajini chinokanganisa ma adrenal glands edu. Ma adrenal glands aya ari pamusoro peitsvo dzedu. Anogadzira mahormone akati wandei akakosha mumuviri wedu.
Funga nezve adrenal gland sefekitori. Chinhu chikuru chinogadzirwa nefekitori iyi ihormone inonzi cortisol. Iyi ihormone inokosha zvikuru inobatsira miviri yedu kukunda kushushikana, kurwara, uye njodzi.
Kana huwandu hwe cortisol mumunhu ane hutano hwadzikira, uropi (semutariri wefekitori) hunotumira chiratidzo (order) chinonzi ACTH kuadrenal gland kuti "igadzire cortisol." Asi mumunhu ane CAH, adrenal gland haina enzyme inokosha, kana kuti 'mushandi', inodiwa kugadzira cortisol. Saka zvisinei nekuti uropi hunotumira ACTH yakawanda sei, fekitori haigone kugadzira cortisol.
Izvi zvinomutsa mibvunzo miviri:
1. Muviri haugadziri cortisol inodiwa .
2. Sezvo zviratidzo zveACTH zvichibva kuuropi zvichiramba zvichiyerera, imwe mhando yehomoni inogadzirwa neadrenal gland , inonzi androgens, inogadzirwa yakawandisa .
Ma androgens aya (semuenzaniso testosterone) mahormone echirume. Varume nevakadzi vanoda mamwe acho, asi muCAH, anokwira zvakanyanya. Izvi zvinogona kukonzera kuyaruka, kushaya mbereko, uye mamwe matambudziko mukukura kwemwana .
Kwemakumi emakore, mushonga mukuru weizvi wave uri wekupa ma steroids (glucocorticoids) anoshanda se cortisol kunze. Izvi hazvingotsivi cortisol iyo muviri usina, asiwo zvinodzora chiratidzo cheACTH kubva muuropi, zvichideredza kugadzirwa kwe androgens. Zvisinei, dambudziko nderekuti izvi zvinoda kutora ma steroids akawanda kupfuura akajairwa kwenguva yakareba. Izvi zvinogona kukonzera migumisiro yakawanda, yakadai sekuwedzera uremu, kutetepa kwemapfupa, uye matambudziko ekurara .
Wakawana sei mushonga mutsva uyu?
Masayendisiti akashamisika kana paine imwe mhinduro yedambudziko iri remasteroid. Pane kupa masteroids akawanda kunze, ko kana tikawana nzira yekuderedza zvakananga chiratidzo cheACTH chinobva muuropi?
Ndipo pavakawana chinangwa chitsva. Chaive chinhu chinogamuchira muuropi chinonzi CRF1. Kana huwandu hwecortisol hwadzikira, chiratidzo cheACTH chinotanga kushanda neCRF1. MuCAH, maitiro aya anokanganiswa.
Saka masayendisiti akafunga kuti, kana tikavhara CRF1 iyi, tinogona kuderedza kugadzirwa kweACTH, handiti? Kana izvozvo zvikaitika, kugadzirwa kweandrogens kuchadzorwawo, uye huwandu hwema steroid hunofanirwa kutorwa nemurwere huchaderedzwawo.
Pfungwa itsva iyi ndiyo yakakonzera kugadzirwa kwemushonga mutsva unonzi Crinecerfont. Uyu mushonga wakagadzirirwa kugadzirisa dambudziko guru reCAH nekudzivisa CRF1.
Kuongororwa kunoitwa vanhu vasati vapa mushonga
Mushonga mutsva haugone kupihwa vanhu nekukurumidza kana wangowanikwa. Unofanirwa kuongororwa kakawanda kuti uone kana wakachengeteka uye uchishanda. Mushonga weCrinecerfont wakapinda mumatanho ese aya.
1. Kuongororwa kwemurabhoritari nemhuka: Danho rekutanga nderekuyedza mushonga uyu pamasero nemhuka dziri murabhoritari. Izvi ndezvekuona kana mushonga wacho une chepfu kana kuti uchakuvadza nhengo dzemuviri. Fungidzira, mushonga mumwe chete pamishonga mitsva gumi ndiyo inokunda zvipingamupinyi izvi uye inotenderwa.
2. Miedzo yekiriniki: Mushure mekunge bvunzo dzemhuka dzabudirira, miedzo yevanhu inoitwa muzvikamu zvakasiyana-siyana.
Mhedzisiro yekuyedzwa kweCrinecerfont yakadini?
Miedzo yakaitwa pamushonga uyu yaburitsa mhedzisiro yakanaka kwazvo. Kunyanya miedzo yePhase II nePhase III yakakosha zvikuru.
Chikamu chechipiri - Mhedzisiro yevana vadiki
Pano, boka diki revana vadiki (vane makore 14-17) vane CAH vakapihwa mushonga uyu kwemazuva gumi nemana uye shanduko mumazinga avo emahormone dzakaonekwa. Mhedzisiro yacho yaishamisa.
- ACTH (chiratidzo cheuropi) chakaderera ne57% .
- 17-OHP (chiratidzo chekugadzirwa kwe androgen) yakaderera ne69% .
- Androstenedione (rudzi rwe androgen) yakaderera ne58% .
Chikamu Chechitatu - Mhedzisiro yemapoka makuru
Vakurudzirwa nemhedzisiro iyi, masayendisiti akaita miedzo mikuru miviri vachishandisa vakuru nevana, uko vamwe vakapihwa mushonga weCrinecerfont uye vamwe vakapihwa placebo yaitaridzika semushonga asi isina zvayakaita.
| Chikwata | Mhedzisiro huru | Tsananguro |
|---|---|---|
| Vakuru (vachitora Crinecerfont) | Kuderedza chipimo chemushonga westeroid | Nekudzora huwandu hwemahormone, zvaikwanisika kuderedza huwandu hwemasteroids ezuva nezuva ne27% . |
| Vakuru (vakatora placebo) | Kuderedza chipimo chemushonga westeroid | Chipimo che steroid chakaderedzwa ne10% chete. |
| Vana (vachitora Crinecerfont) | Mazinga eAndrogen uye steroid dosage | Mushure memavhiki mana, huwandu hwe androgen hwakaderera zvakanyanya. Pakupera kwekuyedzwa, dhigirii re steroid rakaderedzwa ne18% . |
| Vana (vakatora placebo) | Mazinga eAndrogen uye steroid dosage | Mazinga eAndrogen akawedzera. Chipimo chesteroid chaifanira kuwedzerwa neinenge 6%. |
Mhedzisiro iyi inoratidza zvakajeka kuti Crinecerfont inobatsira kudzora huwandu hwe androgens, ukuwo ichideredzawo huwandu hwe steroid dzinogona kukuvadza varwere kwenguva refu.
Mushonga uyu wakachengeteka zvakadii?
Ongororo dzakaratidza kuti mushonga uyu hauna migumisiro yakaipa yakawanda. Migumisiro yakawanda inonzi idiki uye ndeyenguva pfupi.
- Kuneta
- Musoro
- Mhino inotonhora kana kuti yakazadza
- Kurwadziwa kwetsandanyama kana mudumbu
- Kurwadziwa kwemajoini
Nyevero huru chete inouya nemushonga uyu ndeyekuti kana ukasatora mushonga chaiwo wemasteroid waunonyorerwa nachiremba , huwandu hwecortisol mumuviri wako hunogona kudzikira zvine njodzi, zvichikonzera chirwere chinotyisidzira hupenyu chinonzi adrenal insufficiency. Zvisinei, hapana munhu akatora mushonga uyu nemazvo muzvidzidzo izvi akambosangana nechirwere ichi . Saka, zvakakosha kutevedzera mirairo yachiremba wako.
Tariro itsva yekurapwa kweCAH
Nekuda kwekubudirira uku, FDA (sangano guru rinobvumidza mishonga) muUnited States rakapa mvumo yakakosha yemushonga uyu. Crinecerfont (zita rekuti Crenessity) haisi mushonga weCAH. Zvisinei, idanho guru mukurapa CAH.
Vanachiremba vanogona kukupa mushonga uyu pamwe chete nekurapa kwauri kuita iye zvino kwemishonga ye steroid (kuwedzera kurapwa) . Izvi zvinogona kubatsira kuderedza huwandu hwe steroid yako. Izvi zvinoreva kuti unogona kuwana migumisiro mishoma kubva ku steroid, kudzora huwandu hwe hormone yako zviri nani, uye kuva nehupenyu hwakanaka.
Mharidzo Yekuenda Nayo Kumba
- CAH (Congenital Adrenal Hyperplasia) chirwere chinokonzerwa nemajini chinokanganisa adrenal glands uye chinokonzera kusawirirana kwemahormone.
- Crinecerfont (Crenessity) mushonga uchangobva kubvumidzwa weCAH, unopiwa pamwe chete nekurapwa ne steroid.
- Izvi zvinoderedza huwandu hwakawanda hwemahormone e androgen mumuviri uye zvinogona kubatsira kuderedza huwandu hwema steroid anotorwa zuva nezuva.
- Nekuderedza huwandu hwema steroid, pane mukana wekuderedza migumisiro inokonzerwa nekushandiswa kwenguva refu kwe steroid.
- Kunyangwe izvi zvisiri mushonga wechirwere ichi, idanho guru mukuvandudza hupenyu hwevarwere veCAH.
- Kana iwe kana mwana wako aine CAH, zvakakosha kutaura nachiremba we endocrinologist kana wemhuri kuti uone kana mushonga mutsva uyu wakakunakira .
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