Kusarura uyerogenensitivity syndrome

Partial uyerogenensitivity syndrome (PAIS) chirwere chinoitika muvana kana muviri wavo usingakwanise kupindura nenzira kwayo kune echirume mahormone (androgens). Testosterone ihomoni yebonde yechirume.

Ichi chirwere rudzi rwe androgen insensitivity syndrome.

Mumwedzi miviri kusvika mitatu yekubata pamuviri, vacheche vese vane nhengo dzakaenzana dzakaenzana. Sezvo mwana achikura mukati mechibereko, nhengo yechirume kana yechikadzi inokura zvichienderana neaviri echromosomes ebonde kubva kuvabereki. Izvo zvakare zvinoenderana nematanho e androgens. Mune mwana ane XY chromosomes, akakwirira mazinga e androgens anoitwa mumayestes. Mwana uyu anozoita sikarudzi yechirume. Mune mwana ane ma chromosomes XX, hapana ma testes uye iwo mazinga e androgens akaderera kwazvo. Mwana uyu anozoita sikarudzi yechikadzi. MuPAIS, pane shanduko mune iyo geni iyo inobatsira muviri kuziva nekushandisa mahormone echirume nemazvo. Izvi zvinotungamira kumatambudziko nekuvandudzwa kwenhengo dzechirume. Pakazvarwa, mwana anogona kunge aine nhengo dzakavandika dzisina kujeka, izvo zvinotungamira mukukanganiswa pamusoro pebonde remwana.

Iyo chirwere chinopfuudzwa kuburikidza nemhuri (nhaka). Vanhu vane maX chromosomes maviri havana kukanganiswa kana kopi imwe chete yeX chromosome iine hutachiona hwekuchinja. Varume vanowana nhaka kubva kuvanaamai vavo vanozove nemamiriro acho. Iko kune 50% mukana wekuti mukomana mukomana waamai ane jeni achabatwa. Mwana wese wechikadzi ane 50% mukana wekutakura geni. Nhoroondo yemhuri yakakosha mukumisikidza njodzi zviitiko zvePAIS.

Vanhu vane PAIS vanogona kuve vese vari vaviri murume nemukadzi hunhu hunhu. Izvi zvinogona kusanganisira:

• Nhengo dzisina kujairika dzevanhurume, senge urethra iri pazasi pepenhengo, mudiki mbichana, diki chikanda (ine tambo pasi nepakati kana isina kuvharwa zvakakwana), kana machende asingadiwe.
• Kukura kwemazamu muvanhurume panguva yekuyaruka. Yakadzikira bvudzi remuviri uye ndebvu, asi zvakajairika pubic uye armpit bvudzi.
• Kusagadzikana pabonde nekushaya mbereko.

Anopa hutano anoita bvunzo dzepanyama.

Miedzo inogona kusanganisira:

• Kuongororwa kweropa kutarisa huwandu hwehomoni yechirume neyechikadzi
• Genetic bvunzo senge karyotyping kutarisa ma chromosomes
• Kuverenga kwehurume
• Testicular biopsy
• Pelvic ultrasound kutarisa kana nhengo dzevakadzi dzekubereka dziripo

Vacheche vane PAIS vanogona kupihwa murume kana murume zvichienderana nehukuru hwekusaziva. Nekudaro, kupihwa kwevanhukadzi inyaya yakaoma uye inofanira kutariswa zvakanyatsonaka. Zvinogona kurapwa zvePAIS zvinosanganisira:

• Kune avo vanopihwa sevanhurume, kuvhiyiwa kunogona kuitwa kudzikisa mazamu, kugadzirisa machende asina kukodzera, kana kuumbazve nhengo yemurume. Ivo vanogona zvakare kugamuchira androgens kubatsira kumeso bvudzi kukura uye kudzamisa izwi.
• Kune avo vakapihwa semadzimai, kuvhiya kunogona kuitwa kubvisa machende nekumisazve nhengo dzesikarudzi. Iyo yechikadzi hormone estrogen inozopihwa panguva yekuyaruka.

Aya mapoka anotevera anogona kupa rumwe ruzivo paPAIS:

• Intersex Sangano reNorth America - www.isna.org/faq/conditions/pais
• NIH Genetic uye Rare Zvirwere Ruzivo Ruzivo - rarediseases.info.nih.gov/diseases/5692/partial-androgen-insensitivity-syndrome

Androgens inonyanya kukosha panguva yekutanga kukura mudumbu. Vanhu vane PAIS vanogona kuva neupenyu hwakajairika uye kuva neutano hwakakwana, asi vanogona kunetseka kubata mwana. Muzviitiko zvakanyanyisa, vakomana vane nhengo dzekunze dzevakadzi kana sikarudzi diki kwazvo vanogona kuve nezvinetso zvepfungwa kana zvepfungwa.

Vana vane PAIS nevabereki vavo vanogona kubatsirwa nekupangwa mazano uye kupihwa rubatsiro kubva kuchikwata chekuchengetedza hutano chinosanganisira nyanzvi dzakasiyana.

Fonera mupi wako kana iwe, mwanakomana wako, kana nhengo yemhuri yechirume uine kushaya mbereko kana kukwana kukwana kwehurume sikarudzi. Kuedzwa kwegenetic uye kuraira zvinokurudzirwa kana PAIS ichifungidzirwa.

Kuongororwa kwepamuviri kunowanikwa. Vanhu vane nhoroondo yemhuri yePAIS vanofanirwa kufunga nezvekupiwa mazano.

PAIS; Androgen insensitivity syndrome - zvishoma; Isina kukwana testicular feminization; Type I yemhuri isina kukwana murume pseudohermaphroditism; Lubs chirwere; Reifenstein chirwere; Rosewater chirwere

• Chekubereka murume

Achermann JC, Hughes IA. Matambudziko evana ekukura pabonde. Mune: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, eds. Williams Chinyorwa cheEndocrinology. 13 ed. Philadelphia, PA: Elsevier; 2016: chits. 23.

Shnorhavorian M, Fechner PY. Kukanganiswa kwekusiyana pabonde. Mune: Gleason CA, Juul SE, eds. Zvirwere zveAvery zveMucheche. 10th ed. Philadelphia, PA: Elsevier; 2018: chits. 97.