Dambudziko remahormone here? Ngatidzidzei nezve Congenital Adrenal Hyperplasia (CAH) nenzira iri nyore.

Wakaona here shanduko kana kunetseka nezvekuonekwa kwenhengo dzesikarudzi dzemwanasikana wako achangoberekwa? Kana kuti mwanakomana wako mudiki ari kutanga kuratidza zviratidzo zvekuyaruka asati asvika zuva rake rekuzvarwa? Pamwe mwana wako ari kurutsa, kuzvimba, kana kuti aneta? Nhasi tichataura nezvechirwere chemajini chinogona kukonzera zvinhu izvi. Tinodaidza izvi kuti Congenital Adrenal Hyperplasia, kana kuti CAH muchidimbu. Usazvidya mwoyo, zita racho rinogona kunzwika serakaoma, asi ngatirambei takariverenga.

Zvinorevei kuti CAH?

Zvakanaka, ngatitangei tatarisa kuti CAH chii. Tose tine magland maviri, akaita sengowani diki, pamusoro peitsvo dzedu. Aya ndiwo atinodaidza kuti magland eadrenal. Magland maviri aya madiki anoburitsa mahormone akakosha akakosha pakushanda kwemuviri wedu.

• Cortisol: Iyi ndiyo hormone huru yekushushikana mumuviri wedu. Inogadzirira muviri kuti ugadzirire zvirwere, njodzi, uye kushushikana. Inobatsirawo kudzora BP, simba, uye shuga muropa.
• Aldosterone: Homoni iyi inobatsira kuchengetedza munyu (sodium) nemvura mumiviri yedu, nokudaro ichidzora BP nehuwandu hweropa.
• Androgens: Aya ndiwo mahormone echirume. Testosterone ndeimwe yemahormone akadaro. Mahormone aya akakosha zvikuru pakutanga kwekuyaruka, kukura zvakanaka uye kukura.

Zvino, zvinoitika kumunhu ane CAH ndezvekuti enzyme chaiyo inodiwa kugadzira mahormone aya haisipo kana kuti inoderera mumuviri. Kungofanana nekunge spice isipo mundiro, kuravira kwendiro kunochinja, kana enzyme iyi isipo, adrenal glands hadzikwanisi kugadzira mahormone aya zvakanaka.

Saka chii chinoitika?

• Pamwe hormone inonzi cortisol haisi kugadzirwa zvakanyanya sezvainodiwa.
• Pamwe hormone inonzi aldosterone haisi kugadzirwa zvakanyanya sezvainodiwa.
• Pane kudaro, mahormone echirume anonzi androgens anotanga kugadzirwa zvakanyanya .

Kusarongeka kwemahormone uku ndiko kunokonzera matambudziko ese anokonzerwa neCAH.

Kune mhando huru dzeCAH here?

Ehe, kune mhando mbiri huru dzeCAH. 95% yevarwere vakaonekwa kuti vane chirwere ichi vanowira mumhando mbiri idzi.

1. CAH yekare: Iyi ndiyo mhando yeCAH yakanyanya kuoma uye yakakomba. Inowanzoonekwa pakuzvarwa. Kana ikasarapwa zvakanaka, inogona kukonzera shock, coma, uye kunyange rufu. Saka, zvakakosha zvikuru kuongorora nekurapa chirwere ichi nekukurumidza.Kune mhando mbiri dzerudzi urwu.

• CAH inopedza munyu: Iyi ndiyo mhando yeCAH yakanyanya kuipa. Muchiitiko ichi, hormone yealdosterone inogadzirwa nehuwandu hushoma. Izvi zvinoita kuti muviri usakwanise kudzora huwandu hwemunyu (sodium). Nekuda kweizvozvo, munyu wakawandisa unoburitswa muweti. Panguva imwe chete, hormone yecortisol inodzikirawo, uye hormone yeandrogen inogadzirwa yakawandisa.
• CAH isingabudisi munyu zvakanyanya (isina munyu, rudzi rwakajairika): Ichi chirwere chisina kunyanya kuipa. Pano, kushomeka kwehomoni ye aldosterone hakuna kuipa zvakanyanya. Nokudaro, zviratidzo zvinotyisidzira hupenyu hazvionekwi. Zvisinei, homoni ye cortisol ichiri shoma uye homoni ye androgen yakakwira. Izvi zvinokonzera matambudziko ane chekuita nekukura kwepabonde.

2. Nonclassic CAH: Iyi ndiyo mhando yeCAH isina kunyanya kuipa. Inowanzoonekwa muhudiki, kuyaruka, kana kuti vanhu vakuru. Vamwe vanhu vanogona kunge vasina zviratidzo zvachose. Pano zvakare, zvimwe zviratidzo zvine chekuita nekukura pabonde zvinogona kuitika nekuda kwekugadzirwa kwakanyanya kwemahormone e androgen.

Ndezvipi zviratidzo zvinogona kukonzera chirwere cheCAH?

Zviratidzo zveCAH zvinogona kusiyana zvichienderana nerudzi uye murume kana mukadzi. Ngatitarisei tafura iri pazasi kuti tinzwisise izvi zvakajeka.

Sei CAH ichikura? Chii chinokonzera?

Zvichitaurwa zviri nyore, CAH chirwere chinokonzerwa nemajini . Izvi zvinoreva kuti chinhu chatinogara nhaka kubva kuvabereki vedu. Hachisi chirwere chinotapukira.

Pahunhu hwese huri mumuviri wedu, pane majini maviri, rimwe kubva kuna amai vedu nerimwe kubva kuna baba vedu. Kuti CAH iitike, mwana anofanira kugara nhaka majini asina kunaka kubva kuna amai vake nababa vake . Chirwere ichi chinonzi autosomal recessive disorder.

CAH inowanzo kukonzerwa nekushaikwa kwe enzyme 21-hydroxylase. Inokonzerwa nekuchinja kwemajini kunobva enzyme iyi.

Ungaziva sei kana uine CAH?

Classic CAH (rudzi rwakaoma)

Aya ndiwo mashoko akanakisa. Zvipatara zvakawanda muSri Lanka zvino zvinosanganisira bvunzo yekuongorora yeClassic CAH pakati pemiedzo inoitwa pakuzvarwa. Izvi zvinoitwa mazuva mashoma mwana aberekwa, uchishandisa donhwe diki reropa rakatorwa kubva pachitsitsinho chemwana . Iyi bvunzo (kuongorora kwemwana achangoberekwa) inogona kuona nekukurumidza kana mwana aine Classic CAH. Izvi zvinoreva kuti kurapwa kunogona kutanga zviratidzo zvisati zvaonekwa, zvichiponesa hupenyu hwemwana.

Kana mhuri ine mwana ane CAH yatova nemwana mumwe, zvinokwanisika kuyedza mwana wacho kuti aone kana aine chirwere ichi panguva yekuzvitakura. Kune bvunzo dzakakosha dzeizvi, dzakadai seamniocentesis. Taura nachiremba wako nezveizvi.

CAH isina kujairika (yakapfava)

Chimiro ichi chinyoro chinogona kunonoka kuoneka nekuti zviratidzo zvinozoonekwa gare gare. Kana iwe kana mwana wako akatanga kuona zviratidzo zvataurwa pamusoro apa, munofanira kuona chiremba. Chiremba acha:

• Kuongororwa kwemuviri kunoitwa.
• Kuongororwa kweropa uye kuongororwa kweweti kunoongorora huwandu hwemahormone.
• Dzimwe nguva kuongororwa kwemajini kunogona kusimbisa chirwere.

Ndezvipi zvinorapwa izvi?

CAH haigone kurapwa zvachose. Zvisinei, kana ukarapa zvakanaka, inogona kudzorwa zvakanaka uye unogona kurarama hupenyu hwakanaka uye hwakajairika . Kurapwa kunoenderana nerudzi rwechirwere uye kuoma kwezviratidzo.

Kurapa kweClassic CAH

Vanhu ava vanofanira kutora mishonga zuva nezuva kwehupenyu hwavo hwese . Chinangwa chikuru chekurapa ndechekuenzanisa huwandu hwemahormone nekupa mahormone asina kukwana mumuviri. Izvi zvinobatsira kuona kukura kwakajairika uye kukura pabonde.

• Glucocorticoids: Mishonga iyi inotsiva hormone yecortisol isingagadzirwe nemuviri. Muyero wemishonga iyi ungangoda kuwedzerwa kana mwana aine chirwere chakadai sefivha, hutachiona, kana panguva yekushungurudzika kwakadai sekuvhiyiwa.
• Mineralocorticoids: Izvi zvinopihwa kutsiva hormone aldosterone isingagadzirwe mumuviri.
• Mishonga yekuwedzera munyu: Vana vacheche vane chirwere chekurasikirwa nemunyu vanopihwa sodium chloride kuti itsive munyu unorasika mumuviri.

Imwe nzira yekurapa ndeyekuvhiyiwa kwekugadzirisa nhengo dzesikarudzi dzisina kunyatsojeka muvasikana. Izvi zvinowanzoitwa pakati pemwedzi miviri kusvika mitanhatu mushure mekuzvarwa kwemwana.

Kurapa kwenonclassic CAH

Vanhu vane chimiro ichi chisina kusimba vanogona kusada kurapwa kana vasina zviratidzo. Kana vaine zviratidzo zvisina kusimba, vanogona kupihwa mishonga mishoma yeglucocorticoids. Vanhu ava havawanzoda kurapwa kwehupenyu hwavo hwese.

Mune chero mamiriro ezvinhu akadai, kutsvaga mazano ehutano hwepfungwa kuti utaure nezvekushushikana nematambudziko ari kusangana nemwana nevabereki chikamu chakakosha chekurapa.

Ndezvipi zvinetso zvinogona kuitika kana zvikasarapwa?

Kana ikasarapwa, kunyanya rudzi rweClassic CAH runopedza munyu, inogona kuva nengozi zvikuru. Kurasikirwa nemunyu wakawandisa mumuviri kunogona kukonzera matambudziko anogona kutokonzera njodzi kuhupenyu. Kana mwana achirutsa zvakanyanya, achikamhina, kana kuti asiri kuyamwa , anofanira kuendeswa kuchipatara nekukurumidza.

Chii chinogona kuitika kana chikasarapwa:

• Kurova kwemoyo kusina kurongeka (Arrhythmia)
• Kusungwa kwemwoyo
• Kunyangwe rufu runogona kuitika.

CAH isingawanzoitiki inogona kukonzera matambudziko kunyangwe ikasarapwa. Semuenzaniso, kusabereka, kutenderera kwemwedzi zvisina kurongeka, uye hunhu hwechirume hwechigarire muvakadzi.