Nausen pum chhunga nausen a lo seilian hian a chang chuan thil tenawm tak tak chu beisei angin a kal lo. Naupang thenkhat chuan an pian tirh hian an taksa peng hrang hrangah harsatna tenau te an nei thei a. A nih loh leh, an taksa chu puitlin hun an thlen hunah beisei angin a danglam lo thei bawk. Chutiang hunah chuan `(Androgen Insensitivity Syndrome)` tih hi kan ngaihtuah thei a ni. Lungngai suh, he thu hi awlsam takin, i hriatthiam theih dan tur angin kan sawi dawn a ni.
Androgen Insensitivity Syndrome hi eng nge ni?
A awlsam zawngin, Androgen Insensitivity Syndrome (AIS) hi naupang chu genetically mipa (i.e., X chromosome leh Y chromosome nei) ni mahse a taksain mipa sex hormone, androgens te chu a chhang tha lo a ni. Hetiang hian han ngaihtuah teh: kan taksaah hian kawngkhar te tak te a awm a, chu chuan cell-te hnenah thuchah a thawn a, heng hormone te hi an chhang let thin. AIS vei miah chuan heng gate te hian hna an thawk tha lo.
Doctor te chuan hei hi sex differentiation disorder an ti a . Tun hma chuan testicular feminization syndrome an ti thin a, mahse chu hming chu hman a ni tawh lo. Hetiang dinhmun hian nausen pum chhunga a la awm laiin a sex organs lo thanglian dan a nghawng a ni. Tin, puitlin laia lo awm tur mipat hmeichhiatna lama mizia lo piang chhuak tur a nghawng bawk. Vawi tam tak chu hetiang natna vei te hian puitlingah fa an nei thei lo (infertility) .
Hei hi chi hrang hrang a awm em?
Ni e, `AIS` hi chi thum a awm a. Type tin hian mizia a inang lo deuh vek a.
1. Androgen Insensitivity Syndrome (CAIS) natna chi khat: 1. A rilru a hah lutuk chuan a rilru a buai em em a.
- Heng mite hi pawn lam atang chuan hmeichhe naupang ang maiin an lang. Chu chu an serh chu nula serh ang deuhin a lang tihna a ni.
- Mahse, hmeichhe chhungril lam (i.e. ovaries, fallopian tubes, uterus) an nei lo.
- A tam zawkah chuan `CAIS` vei te hi hmeichhe naupang anga seilian an ni.
2. Androgen Insensitivity Syndrome (PAIS) avanga natna hrik awm thei te:
- An pawn lam serh chu mipa serh angin a lo thang kim lo thei a, hmeichhe naupang ang maiin an lang thei a, an pahnih hian an inzawm khawm thei bawk a, chu chuan mipa emaw hmeichhia emaw tih chiang taka hriat a harsat phah (ambiguous genitalia).
- `PAIS` vei te hi a chang chuan mipa angin an seilian a, a chang chuan hmeichhia angin an seilian bawk.
3. Androgen Insensitivity Syndrome (MAIS) natna vei te:
- An serh chu naupang mipa serh ang mai a ni.
- Mahse, fa nei thei lo (infertility) an ni tlangpui bawk. Doctor thenkhat chuan `MAIS` hi `PAIS` ngeia tel ve niin an ngai.
He natna hi tuin nge nei thei ang? Engtiang chiahin nge a hluar?
He `AIS` dinhmun hi nu atanga naupang hnena kal `X` chromosome-a awm `Androgen Receptor (AR) gene` -a chhiatna avanga lo awm a ni. Hei hi `X-linked` gene a nih avangin nu chu he gene chhia hi kengtu a nih chuan a fa mipa hian he `AIS` natna hi a vei theihna chance 4 zinga 1 a nei a ni. Hmeichhia naupangte pawhin he gene chhia hi an rochun thei a, chutah chuan carrier an ni bawk ang a, mahse `AIS` an nei lo ang.
AIS hi a awm lo hle a ni . PAIS hian naute mipa 99,000 zinga pakhat vel a vei a ni. CAIS hian naute 100,000 zinga pahnih atanga panga inkar a vei a ni.
Engvangin nge hetiang hi a lo awm? Eng nge a chhan?
Kan sawi tawh ang khan a chhan ber chu `X` chromosome-a `AR` gene-a thil danglam vang a ni. He gene hian hna a thawh that loh chuan taksain androgen receptor a siam thei lo. Heng androgen receptor te hi, a awlsam zawngin, taksa cell-te’n mipa hormone androgens an tih (entir nan , testosterone) an dawn theihna tura puitu thilte an ni. Chuvangin, heng receptor te hi a awm loh chuan taksa hian mipa hormone te chu a "hriat" lo tihna a ni a, chu chu chu hormone te chu awm mahse an tih tur chu an ti lo tihna a ni.
Hei hi eng symptoms nge ni?
AIS natna lan chhuah dan hi a chi hrang hrangah a inang lo. Mahse, chi hrang hranga langsar ber chu nau pai theih lohna hi a ni .
`CAIS` vei te hian nau an pai thei lo va, an kawppui pawh an rai thei lo. An serh chu hmeichhe serh ang deuh mah se, chhungril lama hmeichhe chi thlahtu (internal female reproductive organs) an nei lo. `PAIS` emaw `MAIS` vei te pawhin fa an neih a rinawm loh hle. An zang te tak te an nei thei a, sperm siamchhuah pawh a tlem hle emaw, a awm lo emaw pawh a ni thei.
CAIS vei te hian symptom dang an neih theih te chu:
- Puitling laiin hmeichhe naupang pangngai aia sang danglam tak a nih .
- Thlasik awm lohna (Amenorrhea) .
- During puberty , armpits leh genital area-ah sam a tlem hle emaw, a awm lo emaw a ni .
- Vagina a tawi emaw a thuk lo emaw .
- Testicles chu pum chhungah a awm reng (a chhuk lo).
PAIS vei te hian symptom dang an neih theih te chu:
- Bifid scrotum hi testicle pahnih ah then a ni a .
- A rilru a buai em em a, a rilru a hah em em bawk a. clitoris (Clitoromegaly) .
- Mipa naupang hnute tissue lian (Gynecomastia) .
- Hypospadias hi urethral opening chu penis hnuai lamah a awm a, tip ah a awm lo .
- Labial adhesions te chu a awm a .
- Penis te tak te pangngai lo (Micropenis) .
- Testicles tla lo thenkhat .
`MAIS` vei te hian an tawn theih tur symptom dang:
- Hnute tihzauh `(Gynecomastia)` .
- Mipa zang te tak te (Micropenis).
- Taksa sam tlem lutuk neih .
Engtin nge hei hi i hriat chian?
Doctor chuan naute pian tirh lamah PAIS hi a hmuchhuak thei a, chu chu naute serh hmun enin a hmu thei a ni. Mahse, naute hian CAIS emaw MAIS emaw a neih chuan naupang kum 11 emaw 12 emaw vel a nih hma chuan hriat theih a ni lo thei a, chu chu naute chu a puitlin hunah a ni . Hei hi doctor-in harsatna a awm leh awm loh a hriat theih hun a ni.
Entirnan, CAIS vei naupang chu period a nei lo emaw, pubic hair a nei lo emaw pawh a ni thei. Naupang MAIS vei chuan a zang a te hle thei a, a hnute pawh a nei thei bawk. Puberty lai hian undescended testicles chu abdominal wall-a hmun chak lo tak hmangin herniate a ni thei. A châng chuan, doctor-te chuan naupangin inguinal hernia an zai lai hian undecended testicles a nei tih an hmu chhuak ṭhin .
Eng ang test nge tih thin?
He `AIS` dinhmun hi nemnghet tur chuan doctor chuan test engemaw zat a tih a ngai dawn a ni:
- Thisen test: Hengte hian hormone level , sex chromosomes, leh genetic abnormalities te an enfiah thin.
- Imaging test: Ultrasound ang chi test hian hmeichhe chi thlahtu (reproductive organs) a lo piang em tih finfiah theih a ni.
I chhungkuaa mi tu emaw chuan AIS a nei tawh a, fa neih i tum a nih chuan genetic testing neih hi a tha hle . He test hian he gene pangngai lo carrier i nih leh nih loh a hriattir thei che a ni.
Hei hi eng enkawlna nge ni?
AIS enkawl dan tur chu pian tirh atanga mipa leh hmeichhia ruat dan azirin a innghat a ni. Enkawlna tam zawk hi naupangin a puitlin hnuah a tan thin. Hei hian naupang taksain hmasawnna inthlak danglamna a neih theih nan hun a siamsak a, naupang chu an enkawl dan tur thutlukna siamah a inrawlh tam thei hle bawk.
Mahse, hriselna lama mithiam thenkhat chuan enkawlna thenkhat, testicles lakchhuah te hi puitlin hmaa tih tur niin an ngai a, a chhan chu undescended testicles-ah cancer chi khat gonadoblastomas an tih chu a vei thei a ni. Tin, puitlin hun a zawh hnuah pawh enkawl dan dang pawh tih theih a nih thu an sawi bawk.
Naupang mipa naupang anga seilian tan enkawlna kawng hrang hrang:
- Mipa serh siam that nan surgery neih a ni. Entirnan, `hypospadias repair` (urethral opening siam thar leh) emaw `orchiopexy` (undecended testicles te scrotum chhunga dah thar leh).
- Breast reduction surgery hmanga hnute tissue tam lutuk paih chhuah a ni.
- Hernia repair surgery hi pum chhunga tissue inhawng emaw chak lo emaw khar nan a ni .
- Testosterone hormone hmanga enkawl a ni .
Naupang hmeichhe naupang anga enkawl tan enkawlna kawng hrang hrang awmte:
- Mipa serh emaw, clitoral tissue tam lutuk emaw paih chhuah nan surgery neih a ni.
- Non-surgical vaginal dilation hmanga vagina thuk zawk siam dan.
- Estrogen hormone hmanga enkawlna .
Hei hi ven theih a ni em?
AIS ven dan tur a awm lo. Mahse, i chhungkuaa mi pakhatin he natna hi a neih a, i fain he gene danglam tak hi a rochun mai thei tih i hlauh chuan, i carrier i nih leh nih loh hriat nan genetic testing neih theih a ni.
Hetiang dinhmun neia khawsa te hi engtin nge an awm? (Hmalam hun thlirna)
AIS vei te hian nun famkim, hrisel takin an nung thei a ni . Mi tam tak chuan hormone therapy leh surgery hmangin an chhang tha hle. Mahse, AIS hian nau pai theih lohna a thlen fo avangin mi tam tak tan chuan rilru lamah a harsa thei hle. Naupang leh puitling tleirawlte rilru hriselnaah nghawng thui tak a nei thei bawk.
Gonadectomy i neih loh chuan testicular cancer vei theihna chu 30% velin a pung a ni.
Ka fa hian AIS a nei a nih chuan engtin nge ka puih theih ang?
"Naupang rilru hriselna enkawl hi AIS enkawlna atana thil pawimawh tak a ni."
I fain AIS a neih chuan doctor, ṭhiante leh chhungte hriatthiamna tur support system nghet tak neih a pawimawh. Support group- a tel hian i fate chu an thil tawn leh harsatnate chu chutiang bawka tawk mek midangte hnena hrilh turin a pui thei bawk.
I fain a puitlin hun a thlen chuan, leh an taksa chu beisei angin a danglam lo tih a hriat tan chuan, AIS chungchang sawipui a pawimawh hle .
Puitling i nih angin AIS hi engtin nge i hmachhawn thin?
Puitling anga AIS nena nun chu thil harsa tak a ni thei. Sex nun pangngai neih a harsa thei a, i dinhmun hrethiam leh pawmtu kawppui zawn a harsa thei a, nau pai theih lohna hian mi tam tak rilruah nghawng thui tak a nei thei bawk.
AIS i neih chuan counselor emaw therapist emaw nen i thil tawn te sawipui chu a \angkai thei ang. Support group-a AIS nei midangte nen pawh i inzawm thei bawk.
A tawp berah chuan thil hriat reng tur
Androgen Insensitivity Syndrome (AIS) hi mi pakhat chu genetically mipa ni mahse mipa hormone a chhan loh vang a ni. Chumi avang chuan an serh chu hmeichhia ang deuhin a lang thei a, mipa leh hmeichhia mizia an nei ve ve thei bawk. Hetiang dinhmun hian harsatna hrang hrang a thlen thei a ni. Chuvangin AIS vei te tan chuan doctor te nena inbiakna neih reng a pawimawh hle a, rilru lam tanpuina nghet tak an neih a pawimawh hle. Damdawi lam thurawn dik tak leh kan hmangaih tak takte \anpuina an neih chuan heng mite hian nun hlawhtling tak an nei thei bawk.
` Androgen insensitivity syndrome, AIS, mipat hmeichhiatna lama hmasawnna, hormone, genetic diseases, chromosomes, nau pai theih lohna, genitals, puberty, genetic testing











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