Pane mumwe munhu mumhuri yako here, kunyanya mwana, ane chirwere chemahormone chaakaberekwa nacho? Ungave uchiziva matambudziko akadai sekunonoka kukura, kutanga kuyaruka, uye kutenderera kwemwedzi kusina kurongeka muvakadzi. Chirwere chemajini chisingawanzoitiki chinogona kukonzera izvi chinonzi 'Congenital Adrenal Hyperplasia'. Tichachidana muchidimbu kuti CAH. Nhasi, tichataura muchidimbu nezveizvi uye mushonga mutsva wakauya nazvo.
Zvichitaurwa zviri nyore, chii chinonzi CAH?
CAH chirwere chemajini chinodzivirira magland eadrenal, magland maviri madiki ari pamusoro peitsvo, kuti asagadzire cortisol, hormone inokosha muhupenyu hwedu.
Funga nezvemiviri yedu sefekitori. Kana chimwe chezvigadzirwa (cortisol) mufekitori iyi chaderera, nzvimbo yekudzora iri muuropi (pituitary gland) inogumbuka uye inotumira masaini (ACTH) kumagland eadrenal kuti ashande nesimba. Nekuda kwekukurudzirwa uku kwakanyanya, kunyangwe kugadzirwa kwecortisol kusingawedzere, inotanga kugadzira mahormone echirume akawanda anonzi 'androgens' .
Kusawirirana kwemahomoni uku kunogona kukonzera matambudziko akawanda:
- Vacheche vechikadzi: Pakuzvarwa, nhengo dzekunze dzemuviri dzinogona kutaridzika sedzemwana wechirume.
- Kune vese vari vaviri: matambudziko akadai sekunonoka kukura, kuyaruka, uye kushaya mbereko zvinogona kuitika pazera rekukura.
Pamusoro pezvo, vamwe varwere veCAH vanogona kunge vasina kugadzirwa kwakawanda kweimwe hormone inokosha inonzi aldosterone. Pasina iyi hormone, CAH inogona kuva yakanyanya, zvichikonzera kuvhunduka, kukotama, kana kutofa.
Kurapwa kwemakore makumi manomwe uye tariro itsva
Kwemakumi emakore, mushonga weCAH chete waive wekutsiva huwandu hwakaderera hwehomoni yecortisol mumuviri nekuwedzera madhigirii esteroid , akadai sehydrocortisone. Zvisinei, kutora madhigirii aya akawanda kwenguva yakareba kunogona kukonzera migumisiro yakati wandei. Yakanyanya kuwanda ndeyekuwedzera uremu, BP, mapfupa asina simba, uye kukura kwevana vasingakure zvakanaka.
Asi ikozvino pane mashoko akanaka! Mushure memakore makumi manomwe, FDA yakabvumidza mushonga wekutanga uye wega wakagadzirwa zvakanangana neCAH, crinecerfont (zita rechiratidzo: Crenessity).
Mushonga mutsva uyu (Crinecerfont) unoshanda sei?
Ichi ndicho chinhu chakakosha. Kusiyana nekurapwa kwekare, mushonga mutsva uyu unoshanda nenzira yakasiyana zvachose.
'Crinesurfont' haiwedzere mahormone anobva kunze. Pane kudaro, mushonga uyu unoenda zvakananga kunzvimbo yekudzora yeuropi (pituitary gland) uye unodzivisa kuburitswa kwechiratidzo chisingadiwe (ACTH) chinosimudzira adrenal glands.
Zvichitaurwa zviri nyore, izvi zvakafanana nekudzima 'switch' yesystem isina basa. Izvi zvinodzora kugadzirwa kwehormone dze'androgen' dzisina basa mumuviri.
Chinhu chikuru cheizvi ndechekuti sezvo huwandu hwe androgens huchidzorwa, huwandu hwe steroid hunodiwa kuchengetedza huwandu hwemahormone hunogona kuderedzwa zvakanyanya . Izvi zvinoreva kuti mhedzisiro inokonzerwa ne steroid inogonawo kuderedzwa zvakanyanya.
| Hunhu | Kurapwa kwekare (ma steroid akawanda) | Kurapa kutsva (Crinecerfont + madhigirii e steroid akaderera) |
|---|---|---|
| Mashandiro | Kupa hormone ye cortisol yakaderera kunze. | Kuvhara zviratidzo zvisingadiwe (ACTH) kubva muuropi uye kudzora kugadzirwa kwe androgen. |
| Chinangwa chikuru | Mishonga yakawanda inodiwa kudzora huwandu hwemahormone. | Kuderedza huwandu hwe androgens uye kuderedza huwandu hwe steroid hunodiwa. |
| Migumisiro yakaipa | Matambudziko enguva refu akadai sekuwedzera uremu, kusakura zvakanaka, uye mapfupa asina simba. | Mikana yekuderedza migumisiro yemishonga ine chekuita nesteroid. Migumisiro midiki (musoro, kuneta) yakashumwa. |
Mushonga uyu wakakodzera ani?
'Crinesurfont' parizvino inotenderwa kuvarwere veCAH vane makore anopfuura mana . Haisi yekutsiva masteroids, asi kuwedzerwa kune masteroids.Kurapa kwekuwedzera.
- Kuvana: Sezvo vana vachiri kukura, mishonga yakawanda ye steroid inogona kukanganisa kukura kwavo. Saka, mushonga uyu, unogona kuderedza mishonga ye steroid, unogona kuvabatsira zvikuru. Izvi zvinonyanya kukosha panguva yekuyaruka, pazvinenge zvakaoma kudzora mahormone.
- Kuvanhu vakuru: Kurapa uku kunogonawo kubatsira vanhu vakuru vanotambura nemigumisiro yakaipa nekuda kwekutora mishonga yemasteroid kwenguva yakareba, kana kuti vanoomerwa nekuchengetedza huwandu hwakagadzikana hwemahormone.
Dzidza nezvemigumisiro uye kuchengetedzeka
Mumiedzo yekiriniki, migumisiro mishomanana yenguva pfupi chete ndiyo yakataurwa nemushonga uyu.
- Kuvanhu vakuru: kuneta, musoro, dzungu, kurwadziwa kwemajoini.
- Kuvana: musoro, kurwadziwa mudumbu, kuneta, mhino yakavharika.
Chinonyanya kukosha ndechekuti zviratidzo izvi zvakawanda zvaive zvishoma, uye huwandu hwevarwere vakarega kutora mushonga hwaive hwakaderera zvikuru.
Yambiro Yakakosha: Ngwarira Dambudziko reAdrenal!
Paunenge uchitora mushonga uyu, zvakakosha kuziva nezvechirwere chakakomba chinonzi 'adrenal crisis'. Zvichitaurwa zviri nyore, chirwere ichi chinogona kuitika kana muviri ukasawana huwandu hunodiwa hwe'cortisol' panguva yekushushikana kwakanyanya (semuenzaniso, hutachiona hwakakomba, kuvhiyiwa, tsaona).
Saka, taura nachiremba wako uye iva nechokwadi chekuti unoziva chaizvo kuti ndeipi mishonga yekuwedzera steroid yaunofanira kutora kana paine emergency ("stress dose") . Zvakare, ziva zviratidzo zve 'adrenal crisis' (semuenzaniso, kurutsa zvakanyanya, manyoka, dzungu, kurasikirwa nepfungwa) uye yeuka kuenda kuchipatara kana ku emergency room (ETU) nekukasika kana chinhu chakadaro chikaitika.
Ndingaziva sei kana mushonga uyu wakakodzera ini/mwana wangu?
Sarudzo iyi inofanira kuitwa mushure mekubvunzana nachiremba wako, kunyanya chiremba we endocrinologist . Chiremba wako achafunga nezvezvinhu zvakawanda, kusanganisira mhedzisiro yekuongororwa kweropa rako, mwero wekukura, huremu, BP, uye huwandu hwemahormone aripo iye zvino, kuti aone kana mushonga uyu wakakodzera iwe.
Uyu hausi mushonga weCAH, asi pane tariro huru yekuti zvichabatsira kuvandudza hupenyu hwevarwere, kuderedza migumisiro yema steroid, uye kuita kuti kushandiswa kwemahormone kuve nyore.
Mharidzo Yekuenda Nayo Kumba
- CAH zvinorevaChirwere chemajini apo adrenal glands inoburitsa cortisol shoma uye mahormone akawanda e androgen.
- Crinecerfont mushonga mutsva wakagadzirwa zvakanangana neCAH, wekutanga werudzi rwayo mumakore makumi manomwe.
- Uyu hausi mushonga unotsiva masteroids. Mushonga unopiwa pamwe chete nemasteroids uye unobatsira kuderedza huwandu hwemasteroids anodiwa.
- Chinhu chikuru chinobatsira ndechekuti vanogona kuderedza migumisiro yenguva refu yemishonga iyi.
- Iva nechokwadi chekubvunza chiremba wako nezvedambudziko re emergency rinonzi 'adrenal crisis' uye "stress dose" yaunofanira kutora panguva iyoyo.
- Zvakakosha kubvunza chiremba ane ruzivo nezvezvirwere zvemahormone kuti uone kana kurapwa uku kwakakunakira iwe kana mwana wako.
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