Mwana wako ari kutambura nechirwere chakadai here? Ngatidzidzei nezve Congenital Adrenal Hyperplasia (CAH)!

Dzimwe nguva paunotarisa mwana achangoberekwa, kana mwana ari kukura zvishoma, ungave wakaona shanduko. Pamwe chiremba akakuudza kuti mwana wako ane chirwere chinonzi `(Congenital Adrenal Hyperplasia)` kana `(CAH)`. Usatye paunonzwa zita iri. Kunyangwe rakaoma zvishoma, ngatitaure nezvazvo muchiSinhala chiri nyore kunzwisisa. Sekutaura kwaunoita neshamwari yako.

Chii chinonzi congenital adrenal hyperplasia (CAH)?

Zvichitaurwa zviri nyore, CAH chirwere chekuzvarwa nacho, kana kuti genetic, chinonyanya kukanganisa ma adrenal glands mumiviri yedu. Unoyeuka here kuti kune ma adrenal glands maviri madiki pamusoro peitsvo dzedu? Ndizvo zvatinodaidza kuti adrenal glands . Kunyangwe ari madiki, anogadzira mahormone akati wandei akakosha pakushanda zvakanaka kwemiviri yedu.

Ngationei kuti mahormone aya chii:

• Cortisol: Homoni iyi inobatsira muviri wedu kutsungirira kushushikana, kurwara, uye kukuvara. Inobatsirawo pakugadzirisa zvinhu zvakaita seBP, simba, uye shuga muropa.
• Aldosterone: Hormone iyi inobatsira kuchengetedza huwandu hwakakwana hwemunyu (sodium) nemvura mumiviri yedu. Inodiwawo kudzora BP uye huwandu hweropa.
• Androgens: Aya ndiwo mahormone echirume (semuenzaniso, testosterone). Mahormone aya akakosha pakuyaruka, kukura zvakanaka, uye kukura.

Zvino, zvinoitika kumunhu ane `(CAH)` ndechekuti rimwe kana akawanda ema enzyme aya anobatsira ma adrenal glands kugadzira mahormone akakosha anodzikira kana kushaikwa. Pasina iyi enzyme, ma adrenal glands haagone kushanda zvakanaka. Zvadaro:

• Pamwe hausi kugadzira cortisol yakakwana.
• Zvinogoneka kuti hapana aldosterone yakakwana inogadzirwa.
• Pamwe androgen yakawandisa inogadzirwa.

Kusarongeka kwemahormone uku ndiko kunonyanya kuitika kana munhu arwara nechirwere che `(CAH)`.

Ndedzipi mhando huru dze `(CAH)`?

Kune mhando mbiri huru dzeCAH. Mhando mbiri idzi dzinoita 95% yezvirwere.

1. Classic CAH: Iyi ndiyo mhando yeCAH yakanyanya kuoma uye yakakomba . Inogona kukonzera matambudziko akakomba anosanganisira adrenal glands, akadai sekuvhunduka uye kutoita coma. Inogona kuisa hupenyu panjodzi kana ikasaonekwa uye ikarapwa nekukurumidza. Iyi classic CAH chirwere chinowanzoonekwa pakuzvarwa.

Izvi zvinewo marudzi maviri emhando iyi:

• CAH Inopedza Munyu: Izvi ndizvo zviri mu (CAH)Chimiro chakanyanya kuipa . Muchiitiko ichi, adrenal glands inoburitsa aldosterone, iyo inenge yakaderera zvikuru. Kana aldosterone yakarasika, muviri haugone kudzora huwandu hwemunyu (sodium) muropa. Izvi zvinoita kuti sodium yakawandisa ibudiswe muweti. Pamusoro pezvo, cortisol inogadzirwawo zvishoma, asi androgens inogadzirwa yakawanda.
• CAH inongoerekana yawedzera simba: Iyi imhando yeCAH isina kusimba . Muchiitiko ichi, kushomeka kwealdosterone hakuna kuipa zvakanyanya. Saka, hapana zviratidzo zvinotyisidzira hupenyu. Zvisinei, kugadzirwa kwecortisol kushoma uye kugadzirwa kweandrogen kwakawanda. Kugadzirwa kweandrogen kwakawandisa uku kunogona kukonzera zviratidzo zvine chekuita nekukura pabonde.

2. Nonclassic CAH: Iyi ndiyo mhando yeCAH isina kunyanya kuoma . Inowanzoonekwa muhudiki, kuyaruka, kana kuti kukura. Zviratidzo zvinogona kunge zviripo kana kusavapo. Izvi zvinogonawo kukonzerwa nekugadzirwa kwakawandisa kwe androgen, izvo zvinogona kutungamira kuzviratidzo zvine chekuita nekukura pabonde.

Kuwedzera kune marudzi makuru aya, kune mamwe marudzi akawanda asingawanzoonekwi eCAH, rimwe nerimwe rine zviratidzo zvakasiyana.

Ndiani anogona kuwana CAH? Yakajairika zvakadii?

Chirwere ichi chinonzi ``(CAH)`` chinogona kuitika kune chero ani zvake. Izvi zvinoreva kuti chinogona kubata vacheche, vana, vakuru, chero ani zvake.

Munyika dzakadai seAmerica neEurope, chirwere chekare cheCAH chinokanganisa munhu mumwe chete pavanhu zviuru gumi kusvika kuzviuru gumi nezvishanu. Chirwere chekare cheCAH chinowanzoonekwa zvishoma, chinokanganisa munhu mumwe chete pavanhu zana kusvika mazana maviri. Mhando dzose dziri mbiri dzinowanikwa pasi rese.

Ndezvipi zviratidzo zve `(CAH)`?

Zviratidzo zveCAH zvinogona kusiyana zvichienderana nerudzi uye murume kana mukadzi. Semuenzaniso, vamwe vacheche vanoberekwa vaine zviratidzo, nepo vamwe vachikura.

Zviratidzo zveClassic CAH

MuCAH yekare, huwandu hwe androgen hwakakwira zvikuru. Izvi zvinogona kukonzera zviratidzo zvine chekuita nemahormone epabonde. CAH inopedza munyu uye inongowedzera hutachiona inogona kukonzera zviratidzo zvakaita se:

• Kusajeka kwenhengo dzesikarudzi muvana vechikadzi: Izvi zvinoreva kuti nhengo dzesikarudzi dzekunze dzemwana dzinogona kutaridzika sedzemwana wechirume. Zvisinei, nhengo dzesikarudzi dzemukati dzemukadzi (dzakadai semazai nechibereko) dzakajairika.
• Nhengo yemurume yakakura muvana vechirume.
• Zviratidzo zvekuyaruka usati wasvika zera: Semuenzaniso, kuchinja kwezwi, mapundu akakomba, uye kukura kwebvudzi muhapwa, nzvimbo dzakavanda, uye kumeso.
• Kubuda kwehunhu hwevarume muvasikana: kukura kwetsandanyama, kudzika kwezwi, uye kukura kwebvudzi rechiso risingadiwe.
• Kukura nekukurumidza zvikuru (mumatanho ekutanga).
• Kuenda kumwedzi kusina kurongeka.
• Matumbu ematesticular asina kunaka.
• Kusabereka.

Pamusoro pezvo, kunyanya kuvanhu vane CAH inorasikirwa nemunyu , kusawirirana kwemahormone kunogona kukonzera zviratidzo zvakakomba zveadrenal. Izvi zvine njodzi zvikuru uye zvinoda kurapwa nekukasika :

• Kushaya mvura mumuviri.
• Kudzikira kwehuwandu hwe sodium muropa (Hyponatremia).
• Kuderera kweropa (Hypotension).
• Kurova kwemoyo kusina kurongeka (Arrhythmia).
• Kuderera kweshuga muropa (Hypoglycemia).
• Kuwedzera kweacidity muropa (metabolic acidosis).
• Kurutsa.
• Manyoka.
• Kuderedza huremu.
• Mamiriro ekuvhunduka `(Kuvhunduka)`.

Zvakakosha: Zviratidzo izvi zveCAH inorasika munyu, kunyanya mumwana achangoberekwa, idambudziko. Unofanira kuona chiremba nekukasika.

Zviratidzo zveNonclassic CAH

CAH isingawanzoitiki chirwere chisina kunyanya kuipa. Ungasatomboziva kuti unacho. Zviratidzo zvacho zvinowanzova zvishoma, asi zvinogona kusanganisira:

• Kukura nekukurumidza zvikuru (mumatanho ekutanga).
• Mapundu.
• Kuyaruka usati wasvika panguva yekuyaruka.
• Kukura kwebvudzi risingadiwe pachiso kana mumuviri wemukadzi.
• Kuenda kumwedzi kusina kurongeka.
• Kusabereka.
• Kuve nemhanza yemurume (pakutanga).
• Varume vane nhengo huru asi machende madiki.

Chii chinokonzera `(CAH)`?

Kazhinji, chikonzero chikuru cheCAH kushomeka kana kusavapo kwe enzyme 21-hydroxylase. Kuchinja kwemajini kunokanganisa huwandu hwe enzyme iyi. Kashoma, kushomeka kwe enzyme 11-hydroxylase kunogonawo kukonzera CAH.

CAH chirwere che autosomal recessive disorder . Unoziva here kuti izvozvo zvinorevei? Tinowana makopi maviri ejini rega rega - rimwe kubva kuna amai vedu nerimwe kubva kuna baba vedu. CAH inoitika chete kana mwana akagara nhaka kopi yejini rinokonzera kushomeka kwema enzyme kubva kuvabereki vese. Kana munhu aine jini rimwe chete rakachinja, anogona kunge ari mutakuri wechirwere asi asingaratidze zviratidzo.

Vanachiremba vanoongorora sei chirwere chinonzi `(CAH)`?

Classic CAH

Mwana wako asati adzoka kumba kubva kuchipatara, vanachiremba vachaongorora mwana kuti haana chirwere here kana kuti kwete (CAH). Izvi zvakajairika.Chikamu chekuongororwa kwemwana achangoberekwa, izvi zvinoitwa nekutora donhwe diki reropa kubva pachitsitsinho chemwana. Izvi zvinogona kuona kana uine CAH yekare.

Kana uine pamuviri uye watova nemwana ane CAH, unogona kuongororwa majini emwana asati aberekwa panguva yekuzvitakura . Chiremba wako anogona kuita amniocentesis (kuyedzwa kwemvura iri mudumbu mako) kana chorionic villus sampling (kuyedzwa kwechikamu cheplacenta) kuti aone kana uine CAH.

CAH isiri yekare

Nonclassic CAH inowanzoonekwa mushure mekunge iwe kana mwana wako vatanga kuratidza zviratidzo. Dzimwe nguva, zvinogonawo kuitika pakukura. Chiremba wako achaita zvinotevera kuti aongorore chirwere chacho:

• Kuongororwa kwemuviri.
• Kuongororwa kweropa.
• Kuongororwa kweweti.
• Kuongororwa kwemajini.

CAH inorapwa sei?

Kurapwa kweCAH kunoenderana nerudzi uye kuoma kwezviratidzo. Hapana mushonga weCAH. Zvisinei, mishonga nemimwe mishonga inogona kubatsira kudzora zviratidzo uye kurarama hupenyu hwakanaka.

Kurapa kwekare kweCAH

Chiremba wako achagara achitarisa mamiriro ako ezvinhu. Vachagara vachiongorora ropa rako kuti vaone huwandu hwemahormone ako. Chinangwa chikuru chekurapa ndechekuona kukura kwakajairika uye kukura pabonde.

Chiremba wako anogona kukupa mishonga yakaita seiyi yekurapa zviratidzo zvako:

• Mishonga yemunyu: Vana vacheche vangada mishonga yekuwedzera sodium chloride (kunyanya mishonga inopedza munyu).
• Glucocorticoids: Izvi zvinoshanda nekutsiva hormone cortisol, iyo muviri usingagadzire. Ungangoda kuwedzera mushonga uyu paunenge uchirwara, wakasuruvara, kana kuti wakaneta.
• Mineralocorticoids: Izvi zvinoshandiswa kutsiva hormone inonzi aldosterone, iyo isingagadzirwe mumuviri.

Kana uine Classic CAH, unofanira kunwa mishonga iyi zuva nezuva kweupenyu hwako hwese. Kana ukarega kunwa mushonga, zviratidzo zvako zvichadzoka.

Imwe nzira yekurapa ndeyekuvhiyiwa kwevana vechikadzi vane nhengo dzepabonde dzisinganyatsooneki. Kuvhiyiwa uku kunogona kuitwa pakati pemwedzi miviri kusvika mitanhatu mushure mekuzvarwa kuti kudzoreredze chitarisiko uye mashandiro enhengo dzekunze dzemuviri. Mune zvimwe zviitiko, kuvhiyiwa kunogona kunonoka kwemakore akati wandei. Izvi zvinofanirwa kukurukurwa nachiremba wako zvakanaka usati waita sarudzo.

Kurapa kweCAH kusiri kwekare

Kana usina zviratidzo, ungasada kurapwa. Kana uine zviratidzo zvishoma, ungangopihwa mishonga mishoma yeglucocorticoids. Vanhu ava havawanzoda kurapwa kwehupenyu hwese.

Kana iwe kana mwana wako aine CAH, zvakakosha kutsvaga rubatsiro rwehutano hwepfungwa. Izvi zvinodaro nekuti matambudziko akawanda anouya nechirwere ichi (senge shanduko dzemuviri, kushaya mbereko, nezvimwewo) anogona kukanganisa pfungwa. Nokudaro, rutsigiro rwehutano hwepfungwa chikamu chakakosha chekurapa CAH. Inogona kuvandudza hupenyu.

Ndezvipi zvinetso zvinogona kuitika nekuda kweCAH?

MuCAH yekare, kunyanya rudzi rwemunyu unorasika, mvura yakawandisa nemunyu zvinorasika muweti. Izvi zvinogona kukonzera matambudziko akakomba akadai sekusawirirana kwema electrolyte (semuenzaniso potassium) . Kana zvikasarapwa, kusawirirana uku kunogona kukonzera:

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

Kana CAH isingarapwe inogona kukonzerawo matambudziko. Kune varume:

• Kuyaruka kwekutanga.
• Kupfupika kwehurefu (kuderera kwehurefu).

Kune vakadzi:

• Hunhu hwemuviri wemurume hunogara huripo.
• Kuenda kumwedzi kusingaenderane nenguva.
• Kusabereka.

Kunewo njodzi yemamwe matambudziko (akadai sekutapukirwa nehutachiona, kubuda ropa, uye mavanga) kubva mukuvhiyiwa kunoitwa panhengo dzesikarudzi dzisinganyatsooneki.

`(CAH)` inogona kudzivirirwa here?

Nekuti CAH chirwere chemajini, hachigone kudzivirirwa. Kana mumwe munhu mumhuri mako aine CAH, kana kuti kana iwe uchitova nemwana ane CAH, funga nezvemajini ekupa mazano uye/kana kuongororwa kwemajini . Izvi zvichakubatsira kunzwisisa njodzi yekuti vana vako vagare nhaka chirwere ichi.

Mamiriro ezvinhu akaita sei mushure mekurapwa kwe `(CAH)`?

Kana vanhu vane CAH vakaongororwa nekukurumidza uye vakarapwa nemazvo, vanogona kurarama hupenyu hune hutano uye hune zvibereko. Vanhu vane CAH yekare vachafanira kutora mishonga zuva nezuva kweupenyu hwavo hwese. Zviratidzo zvinogona kudzoka kana mishonga ikamiswa.

Vanhu vazhinji vane CAH vane hutano hwakanaka, asi vanogona kunge vapfupi pane vamwe vakuru. Mune zvimwe zviitiko, CAH inogona kukanganisa kubereka. Kana wakazvarwa uine nhengo dzepabonde dzisinganyatsooneki, ungangoda rubatsiro rwepfungwa. Kana uine matambudziko mushure mekurapwa, usatya kutaura nachiremba wako.

Nzira yekuderedza uremu uchishandisa `(CAH)`?

Mimwe mishonga inoshandiswa kurapa CAH (kunyanya glucocorticoids) inogona kukonzera kuwedzera uremu. Taura nachiremba wako nezvehuremu hwako. Iye anogona kuona kana kuwedzera uremu kuri kukonzerwa nemushonga kana chimwe chirwere. Muyero wemushonga wako ungangoda kugadziriswa. Iye anogonawo kukubatsira kugadzira chirongwa chekudya uye kurovedza muviri kuti akubatsire kuchengetedza huremu hwakanaka.

Ko `(CAH)` kuremara here?

Mamwe masangano anogona kufunga nezvezvirwere zveadrenal gland sekuremara. Kana CAH inokodzera sekuremara zvinoenderana nematambudziko anokonzerwa nechirwere ichocho.

Pakupedzisira, zvinhu zvekurangarira (Mharidzo Yekuenda Kumba)

Zvakajairika kuti unzwe wakaremerwa paunoziva kuti mwana wako ane chirwere chemajini. Zvisinei, vana vanozvarwa vaine Congenital Adrenal Hyperplasia (CAH) vane ramangwana rakanaka kana chirwere ichi chikaonekwa nekukurumidza uye chikarapwa nemazvo.

Rangarira zvinhu izvi:

• (CAH) chirwere chemajini chinokanganisa kugadzirwa kwemahormone muadrenal glands.
• CAH yekare ndiyo mhando yakanyanya kuoma uye inogona kuonekwa kuburikidza nekuongororwa kwemwana achangoberekwa.
• Kuongororwa kwechirwere nekukurumidza uye mishonga yehupenyu hwese (yeCAH yekare) zvakakosha zvikuru.
• Kurapwa kunogona kudzora zviratidzo uye kubvumira kukura kwakajairika.
• Vamwe vanhu vangada rubatsiro rwehutano hwepfungwa uye mazano.
• Kana uchironga kubata pamuviri uye uine nhoroondo yemhuri yeCAH, taura nachiremba wako nezvemazano ezvemajini.

Usazvidya moyo, hausi wega. Kana uine mimwe mibvunzo nezve `(CAH)`, bvunza chiremba wako. Vanogona kukubatsira.

` Congenital Adrenal Hyperplasia, CAH, Adrenal glands, Cortisol, Aldosterone, Androgen, Hormone kusawirirana, Genetic disorder, Kuongororwa kwevana vacheche, Hormone, Zvirwere zveGenetic, Adrenal glands