Kuberekwa adrenal hyperplasia

Congenital adrenal hyperplasia izita rinopihwa kune boka rezvinetso zvakagara nhaka zveadrenal gland.

Vanhu vane 2 adrenal gland. Imwe iri pamusoro peimwe yeitsvo dzayo. Aya ma gland anoita mahormone, akadai secortisol uye aldosterone, ayo akakosha pahupenyu. Vanhu vane congenital adrenal hyperplasia vanoshaya enzyme idrenal gland inoda kugadzira mahormone.

Panguva imwecheteyo, muviri unoburitsa yakawanda androgen, mhando yechirume yebonde mahormone. Izvi zvinokonzeresa kuti hunhu hwechirume huonekwe kare (kana zvisina kukodzera).

Congenital adrenal hyperplasia inogona kukanganisa vese vakomana nevasikana. Vanenge 1 mu10,000 kusvika 18,000 vana vanozvarwa vaine congenital adrenal hyperplasia.

Zviratidzo zvinosiyana, zvinoenderana nerudzi rwekuberekwa adrenal hyperplasia mumwe munhu anaro, uye nezera ravo apo chirwere ichi chikaonekwa.

• Vana vane mafomu akaomarara vanogona kunge vasina zviratidzo kana zviratidzo zvekuberekwa adrenal hyperplasia uye vangangotadza kuwanikwa kusvika nguva yekuyaruka.
• Vasikana vane fomu yakaoma kwazvo vanowanzove nemitezo yekubereka pakuzvarwa uye vanogona kuongororwa zviratidzo zvisati zvaonekwa.
• Vakomana vanozoonekwa vakajairika pakuzvarwa, kunyangwe kana vaine fomu yakaoma.

Muvana vane chimiro chakakomba chechirwere, zviratidzo zvinowanzo kukura mukati memasvondo maviri kana matatu mushure mekuzvarwa.

• Kudya zvisina tsarukano kana kurutsa
• Kupera mvura mumuviri
• Electrolyte shanduko (isina kujairika mazinga e sodium uye potasium muropa)
• Abnormal moyo mutinhimira

Vasikana vane chimiro chakapfava vanowanzo kuve nemitezo yevakadzi yekubereka (ovaries, chibereko, uye fallopian machubhu). Vanogona zvakare kuve neshanduko dzinotevera:

• Nguva isina kujairika yekuenda kumwedzi kana kutadza kuenda kumwedzi
• Kutaridzika kwekutanga kwepubic kana armpit bvudzi
• Bvudzi rakawandisa kukura kana bvudzi kumeso
• Kumwe kuwedzera kwecitoris

Vakomana vane chimiro chakapfava vanowanzo kuoneka senguva dzose pakuzvarwa. Nekudaro, ivo vanogona kuita sevanopinda mukuyaruka nguva isati yakwana. Zviratidzo zvinogona kusanganisira:

• Kudzamisa inzwi
• Kutaridzika kwekutanga kwepubic kana armpit bvudzi
• Yakawedzera penis asi akajairwa testes
• Tsandanyama dzakasimudzwa mushe

Vese vakomana nevasikana vanenge vakareba sevana, asi vakapfupika pane zvakajairwa sevakuru.

Mutarisiri wehutano wemwana wako achaodha zvimwe bvunzo. Kuongororwa kweropa kwakajairika kunosanganisira:

• Serum electrolytes
• Aldosterone
• Renin
• Cortisol

X-ray yeruoko rweruboshwe neruoko zvinogona kuratidza kuti mapfupa emwana anoita kunge emumwe munhu mukuru kupfuura zera ravo chairo.

Genetic bvunzo dzinogona kubatsira kuongorora kana kusimbisa kusagadzikana, asi hazviwanzo kudikanwa.

Chinangwa chekurapa ndechekudzosera mahormone kune akajairwa, kana padhuze neyakajairika. Izvi zvinoitwa nekutora chimiro checortisol, kazhinji hydrocortisone. Vanhu vangangoda mamwe madosi emishonga panguva yekushushikana, senge chirwere chakanyanya kana kuvhiyiwa.

Iye mupi anoona hutachiona hwepabonde hwemwana ane genitalia isina kujairika nekutarisa ma chromosomes (karyotyping). Vasikana vane nhengo dzinoratidzika sevanhurume vanogona kuvhiyiwa genitalia yavo panguva yehucheche.

Steroid inoshandiswa kurapa congenital adrenal hyperplasia haiwanzo kukonzera mhedzisiro senge kufutisa kana mapfupa asina kusimba, nekuti madosi anotsiva mahormone ayo muviri wemwana usingakwanise kuita. Zvakakosha kuti vabereki vataure zviratidzo zvehutachiona uye kushushikana kune mupi wemwana wavo nekuti mwana anogona kuda mumwe mushonga. Steroid haigone kumiswa kamwe-kamwe nekuti kuita izvi kunogona kutungamira kukundikana kweadrenal.

Aya masangano anogona kubatsira:

• National Adrenal Zvirwere Foundation - www.nadf.us
• Iyo MAGIC Foundation - www.magicfoundation.org
• Iyo CARES Foundation - www.caresfoundation.org
• Adrenal Insufficiency United - aiunited.org

Vanhu vane chirwere ichi vanofanira kutora mushonga hupenyu hwavo hwese. Ivo kazhinji vane hutano hwakanaka. Zvisinei, vanogona kunge vari vapfupi pane vanhu vakuru, kunyangwe nekurapwa.

Mune zvimwe zviitiko, kuberekwa adrenal hyperplasia kunogona kukanganisa kubereka.

Zvinetso zvinogona kusanganisira:

• Kuwedzera kweropa
• Shuga shoma yeropa
• Yakaderera sodium

Vabereki vane nhoroondo yemhuri yekuzvarwa adrenal hyperplasia (yerudzi rupi zvarwo) kana mwana ane chirwere ichi anofanirwa kufunga nezvekupiwa mazano.

Kuongororwa kwepamuviri kunowanikwa kune mamwe marudzi ekuberekwa adrenal hyperplasia. Kuongororwa kunoitwa mune yekutanga trimester ne chorionic villus sampling. Kuongororwa mune yechipiri trimester kunoitwa nekuyera mahormone senge 17-hydroxyprogesterone mune amniotic fluid.

Kuongororwa kwekuzvarwa patsva kunowanikwa kune yakajairika fomu yekuberekwa adrenal hyperplasia. Izvo zvinogona kuitwa pachitsitsinho kamuti ropa (sechikamu cheyakajairwa kuongororwa kunoitwa kune vachangozvarwa). Uyu muedzo uri kuitwa parizvino mumatunhu mazhinji.

Adrenogenital syndrome; 21-hydroxylase kushomeka; CAH

• Zvinyorwa zveAdrenal

Donohoue PA. Kukanganisika kwekusimudzira bonde. Mune: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Nelson Chinyorwa chePediatrics. 21st ed. Philadelphia, PA: Elsevier; 2020: chap 606.

Yau M, Khattab A, Pina C, Yuen T, Meyer-Bahlburg HFL, Nyowani MI. Kukanganisa kwe andrenal steroidsidogenesis. Muna: Jameson JL, De Groot LJ, de Kretser DM, et al, eds. Endocrinology: Vakuru uye Chiremba. 7th ed. Philadelphia, PA: Elsevier Saunders; 2016: chits. 104.