Kana mwana ari mudumbu achikura, dzimwe nguva zvinhu zvidiki hazvifambi sezvaitarisirwa. Vamwe vana vanogona kunge vaine matambudziko madiki nenhengo dzavo dzesikarudzi pavanozvarwa. Kana kuti, miviri yavo ingasachinja sezvaitarisirwa pavanosvika pazera rekuyaruka. Panguva dzakadaro, tinogona kufunga nezvechirwere chinonzi `(Androgen Insensitivity Syndrome)`. Musazvidya mwoyo, tichataura nezvazvo zviri nyore, nenzira yamunonzwisisa.
Chii chinonzi Androgen Insensitivity Syndrome?
Zvichitaurwa zviri nyore, Androgen Insensitivity Syndrome (AIS) chirwere umo mwana anoberekwa ari murume (kureva kuti, ane X chromosome uye Y chromosome), asi muviri wake haupindure nemazvo kumahormone echirume, androgens . Funga nezvazvo nenzira iyi: kune magedhi madiki mumiviri yedu anotumira mameseji kumasero, uye anopindura kumahormone aya. Mumunhu ane AIS, magedhi aya haashande nemazvo.
Vanachiremba vanoti izvi chirwere chekusiyana kwepabonde . Chaimbonzi testicular feminization syndrome, asi zita iroro harichashandiswi. Chirwere ichi chinokanganisa kukura kwenhengo dzepabonde dzemwana achiri mudumbu. Chinokanganisawo kukura kwehunhu hwepabonde hunofanira kuitika panguva yekuyaruka. Kazhinji, vanhu vane chirwere ichi havakwanisi kuva nevana sevanhu vakuru (kusabereka) .
Kune mhando dzakasiyana dzeizvi here?
Ehe, kune mhando nhatu huru dze'AIS'. Rudzi rumwe nerumwe rune hunhu hwakasiyana zvishoma.
1. Complete Androgen Insensitivity Syndrome (CAIS):
- Vanhu ava vanotaridzika sevasikana chete. Kureva kuti, nhengo dzavo dzesikarudzi dzinoita sedzemusikana.
- Zvisinei, havana nhengo dzemukati dzevakadzi (semuenzaniso maovari, machubhu ekutapira, chibereko).
- Kazhinji, vanhu vane `CAIS' vanorerwa vari vasikana.
2. Partial Androgen Insensitivity Syndrome (PAIS):
- Nhengo dzavo dzekunze dzinogona kunge dzisina kukura zvakakwana sedzemukomana, kana kuti dzinogona kutaridzika sedzemusikana, kana kuti dzinogona kunge dziine musanganiswa wezvose zviri zviviri, zvichiita kuti zviome kuziva kuti ndedzemurume kana mukadzi (dzimwe nhengo dzemuviri dzisinganyatsooneki).
- Vanhu vane `PAIS` dzimwe nguva vanorerwa vari vakomana uye dzimwe nguva sevasikana.
3. Mild Androgen Insensitivity Syndrome (MAIS):
- Nhengo dzavo dzesikarudzi dzinoita sedzemwana wechirume.
- Zvisinei, kazhinji havakwanisi kuva nevana (kusabereka). Vamwe vanachiremba vanoona `MAIS` sechikamu che`PAIS` pachayo.
Ndiani anogona kuva nechirwere ichi? Chinowanzoitika sei?
Dambudziko iri re'AIS' rinokonzerwa nekusakwana kwejini re'Androgen Receptor (AR)' , riri pa'X' chromosome inopfuudzwa kubva kuna amai kuenda kumwana. Sezvo iri jini re'X-linked', kana amai vari ivo vanotakura jini iri risina kunaka, mwana wavo wechirume ane mukana we1 pa4 wekuva nechirwere ichi che'AIS'. Vana vechikadzi vanogonawo kugara nhaka jini iri risina kunaka, uye ipapo vachavawo vanotakura, asi havazove ne'AIS'.
AIS chirwere chisingawanzoitiki . PAIS inobata anenge mwana mumwe chete pavana 99,000 vechirume. CAIS inobata pakati pevana vaviri kusvika vashanu pavana 100,000.
Sei izvi zvichiitika? Chikonzero chacho ndechei?
Sezvatakambotaura, chikonzero chikuru cheizvi kusarongeka kwejini re'AR' pa chromosome ye'X'. Kana jini iri rikasashanda zvakanaka, muviri haugone kugadzira ma androgen receptors. Aya ma androgen receptors, muchidimbu, zvinhu zvinobatsira masero emuviri kupindura kumahormone echirume anonzi androgens (semuenzaniso , testosterone) . Saka, kana ma receptors aya asipo, muviri hau "zivi" mahormone echirume, zvinoreva kuti kunyangwe mahormone iwayo aripo, haaite zvaanofanira kuita.
Ndezvipi zviratidzo zveizvi?
Zviratidzo zveAIS zvinosiyana zvichienderana nerudzi rwechirwere ichi. Zvisinei, chinhu chikuru chinowanikwa mumarudzi ese kusabereka .
Vanhu vane `CAIS` havagone kubata pamuviri kana kuita kuti mumwe wavo abate pamuviri. Kunyange zvazvo nhengo dzavo dzepabonde dzichiita sedzemukadzi, havana nhengo dzemukati dzevakadzi dzekubereka nadzo. Vanhu vane `PAIS` kana `MAIS` havakwanisiwo kuva nevana. Vanogona kunge vaine nhengo diki yemurume, uye kubereka kwembeu kunogona kunge kushoma kana kusavapo.
Zvimwe zviratidzo zvinogona kuonekwa nevanhu vane CAIS zvinosanganisira:
- Kureba kupfuura musikana wemazuva ose panguva yekuyaruka.
- Kusaenda kumwedzi (Amenorrhea) .
- Munguva yekuyaruka , bvudzi harina kana kuti harina kana kutombova nebvudzi muhapwa nenzvimbo dzepabonde.
- Kutetepa kana kusadzika kwenhengo yemukadzi .
- Machende anoramba ari mudumbu (haana kudzika).
Zvimwe zviratidzo zvinogona kuonekwa nevanhu vane PAIS zvinosanganisira:
- Bifid scrotum chirwere apo testicle inokamurwa kuita zvikamu zviviri .
- Kukura kweclitoris (Clitoromegaly) .
- Kukura kwenyama yemazamu muvakomana (Gynecomastia) .
- Hypospadias inguva apo buri remuuro riri pasi penhengo yemurume, kwete pamucheto .
- Kunamatira kwemazino .
- Nhengo diki isina kujairika (Micropenis) .
- Machende asina kukwira zvishoma .
Zvimwe zviratidzo zvinogona kuonekwa nevanhu vane `MAIS`:
- Kuwedzera mazamu `(Gynecomastia)` .
- Nhengo diki (Micropenis).
- Kuva nebvudzi shoma mumuviri .
Unozviziva sei izvi?
Chiremba anogona kuona PAIS pakuzvarwa nekutarisa nhengo dzesikarudzi dzemwana. Zvisinei, kana mwana aine CAIS kana MAIS, inogona kusaonekwa kusvika mwana ava nemakore angangoita 11 kana 12 ekuberekwa, mwana paanenge ava kuyaruka . Apa ndipo chiremba anogona kuona kana paine dambudziko.
Semuenzaniso, mwana ane CAIS anogona kunge asina kuenda kumwedzi kana kuti asina bvudzi repanhengo yake. Mwana ane MAIS anogona kunge aine nhengo diki kana kuti anogona kuita mazamu. Munguva yekuyaruka, machende asina kukwira anogona kubuda nepanzvimbo isina kusimba mudumbu. Dzimwe nguva, vanachiremba vanoona kuti mwana ane machende asina kukwira pavanovhiyiwa hernia yegumbo .
Ndedzipi mhando dzebvunzo dzinoitwa?
Kuti asimbise mamiriro aya e'AIS', chiremba achafanirwa kuita bvunzo dzinoverengeka:
- Kuongororwa kweropa: Izvi zvinoongorora huwandu hwemahormone , machromosome ebonde, uye kusarongeka kwemajini .
- Miedzo yekufungidzira: Miedzo yakaita se ultrasound inogona kusimbisa kana nhengo dzekubereka dzechikadzi dzakaumbwa.
Kana mumwe munhu mumhuri mako akambova neAIS, uye uri kuronga kuva nevana, zvakanaka kuti uedzerwe majini . Kuongororwa uku kunogona kukuudza kana uri mutakuri wejini iri risingawanzoitiki.
Ndezvipi zvinorapwa izvi?
Kurapwa kweAIS kunoenderana nebonde rakapihwa pakuzvarwa. Kurapwa kwakawanda kunotanga mwana asvika pazera rekuyaruka. Izvi zvinopa nguva yekuti muviri wemwana ushanduke uye zvinoita kuti mwana anyanye kubatanidzwa musarudzo dzekurapa kwake.
Zvisinei, vamwe nyanzvi dzezvehutano vanotenda kuti mamwe marapirwo, akadai sekubvisa machende, anofanira kuitwa munhu asati asvika pazera rekuyaruka nekuda kwenjodzi yekubatwa nechirwere chegomarara chinonzi gonadoblastomas mumachende asina kukwira. Vanotiwo mamwe marapirwo anogona kuitwa mushure mekunge kuyaruka kwapera.
Nzira dzekurapa vana vanorerwa vari vakomana:
- Kuvhiyiwa kwekugadzirisa nhengo dzepabonde dzevarume. Semuenzaniso, `hypospadias repair` (kuronga patsva nzvimbo yekuvhura urethra) kana `orchidopexy` (kuronga patsva machende asina kukwira mudumbu).
- Kuvhiyiwa kwekubvisa mazamu akawandisa.
- Kuvhiyiwa kwekugadzirisa hernia inzira yekuvhara nyama yakavhurika kana isina simba mumadziro edumbu.
- Kurapa kwemahormone eTestosterone .
Nzira dzekurapa dziripo kune vana vanotorwa sevasikana:
- Kuvhiyiwa kubvisa nhengo dzepabonde dzevarume kana nhengo dzepabonde dzakawandisa.
- Nzira dzekukudza nhengo dzesikarudzi dzisingashandiswe pakuvhiya.
- Kurapa neestrogen hormone .
Izvi zvinogona kudzivirirwa here?
Hapana nzira yekudzivirira AIS. Zvisinei, kana mumwe munhu mumhuri yako aine chirwere ichi uye uchinetseka kuti mwana wako anogona kugara nhaka jini iri risingawanzoitiki, kuongororwa kwemajini kunogona kuitwa kuti uone kana uri mutakuri wechirwere ichi.
Vaya vanorarama nechirwere ichi vari sei? (Chiratidzo Cheramangwana)
Vanhu vane AIS vanogona kurarama hupenyu hwakakwana, hune hutano . Vazhinji vanobvuma kurapwa nemahormone nekuvhiyiwa. Zvisinei, nekuti AIS inowanzo kukonzera kushaya mbereko, inogona kuomera vanhu vazhinji mupfungwa. Inogonawo kukanganisa hutano hwepfungwa hwevana nevechidiki.
Kana usina kuvhiyiwa gonadectomy , njodzi yako yekubatwa nekenza yematesticular inowedzera neinenge 30%.
Kana mwana wangu aine AIS, ndingamubatsira sei?
"Kutarisira hutano hwepfungwa hwemwana chikamu chikuru chekutarisira AIS."
Kana mwana wako aine AIS, zvakakosha kuva nerutsigiro rwakasimba rwekunzwisisa vanachiremba, shamwari, nemhuri. Kubatana nemapoka ekutsigirana kunogonawo kubatsira mwana wako kugovana zvakaitika kwaari nematambudziko ake nevamwe vari kusangana nedambudziko rimwe chete.
Mwana wako paanosvika pazera rekuyaruka, otanga kuona kuti muviri wake hausi kuchinja sezvaitarisirwa, zvakakosha zvikuru kutaura naye nezveAIS .
Unoita sei neAIS semunhu mukuru?
Kugara neAIS semunhu mukuru kunogona kuva kwakaoma. Zvinogona kuoma kuva nehupenyu hwepabonde hwakajairika, kuwana mumwe munhu anonzwisisa uye anogamuchira dambudziko rako, uye kushaya mbereko kunogona kukanganisa zvakanyanya pfungwa dzevanhu vazhinji.
Kana uine AIS, zvinogona kubatsira kutaura neanopa mazano kana nyanzvi yezvehutano nezvezvawakasangana nazvo. Unogona zvakare kubatana nevamwe vane AIS mumapoka ekutsigirana.
Pakupedzisira, zvinhu zvekurangarira
Androgen Insensitivity Syndrome (AIS) chirwere chinokonzerwa nekuchinja kwemajini emunhurume asi asingapindure mahormone emurume. Nekuda kweizvozvo, nhengo dzemuviri wake dzingataridzika sedzemukadzi, kana kuti dzinogona kunge dziine hunhu hwemurume nemukadzi. Chirwere ichi chinogona kukonzera matambudziko akasiyana-siyana. Saka, zvakakosha kuti vanhu vane AIS vataure navanachiremba vavo nguva dzose uye vawane rutsigiro rwakasimba rwepfungwa. Nemazano ekurapa akakodzera uye rutsigiro rwevadikanwi, vanhu ava vanogonawo kurarama hupenyu hunobudirira.
` Androgen insensitivity syndrome, AIS, kukura pabonde, mahormone, zvirwere zvemajini, machromosome, kusabereka, nhengo dzepabonde, kuyaruka, kuyedzwa kwemajini











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