I naute serh hi mipa nge hmeichhia tih chiang taka hriat a har em? Hei hi ‘Atypical Genitalia’ a ni thei.

I naute piang thar chu i kuah a, mipa nge hmeichhia tih i hre nghal thei lo a nih chuan engtin nge i awm ang? Ngaihtuah harsa tak a ni a, a buaithlak thei hle a, a buaithlak thei hle a, a lungchhiatthlak hle thei bawk. Hetiang hunah hian zawhna tam tak rilruah a lo lang thin. "Eng nge ka naute hi a tihsual?" "Hei hi thil pangngai a ni em?" "Tunah enge ka tih ang?" Chutiang thil chu i rilruah a kal zel mai thei. Hei hi damdawi lam hawi chuan ‘Atypical Genitalia’ kan tih chu a ni. Tun hma chuan ‘Ambiguous Genitalia’ an ti bawk. A awlsam zawngin sawi ila, hei hi naute pawn lam serh chu mipa leh hmeichhia a ni lo tih a chiang lo tihna a ni. Naute serh hi a lo thang kim lo thei a, a nih loh leh mipa leh hmeichhia inzawmkhawm an nei thei bawk. Hei hi Difference of Sexual Development (DSD) tia hriat a ni bawk.

He natna hi engtiang chiahin nge ‘Atypical Genitalia’ tia koh a nih?

Hei hi a awm lo hle a ni . Statistics atanga a lan dan chuan nau piang thar 1,000 atanga 4,500 zinga pakhat chauh hi he natna hian a vei a ni. Chuvangin, hriat loh tak tak ni mahse, hei hi hriat chian a pawimawh hle.

Naute serh hi engtin nge a tlangpuiin a lo thanglian?

Hei hi hrethiam tur chuan nausen pum chhunga sex organs lo thanglian dan hi tlem tal i hriat a ngai a ni. Ngaihtuah teh, hei hi thil kalphung buaithlak tak leh mak tak a ni. Hei hi step lian pathum-ah a thleng thin: 1. Genetic sex determination: Thil thleng hmasa ber chu naute genetic sex tihfel a ni. Hei hi pa atanga sperm lo chhuak chu nu atanga egg nen an inzawm khawm hian a thleng thin. Nu hian X chromosome a dawng fo thin. Pa hian X emaw Y chromosome emaw a dawng thei a ni. Chuvangin, nu atanga X leh pa atanga X an inzawm (XX) chuan hmeichhia a lo piang ta a ni. Nu atanga X leh pa atanga Y inkawp (XY) chuan mipa naupang a lo piang ta a ni. 2. Gonad formation: A dawtah chuan naute gonads te hi he genetic sex ang hian a lo thang lian a. Naute hmeichhiaah chuan ovary a lo thang lian a, mipaah ​​chuan testes a lo thang lian bawk. 3. Internal leh external genitalia thanlenna: A tawp berah chuan heng gonads atanga hormone siam chhuah te hian naute chhungril leh pawn lam genitalia lo thanglian turin mawhphurtu an ni. Chuvangin, he thil kalphung hmun eng emaw zatah hian inthlak danglamna a awm a nih chuan Dissociative Developmental Disorder (DSD), emaw ‘Atypical Genitalia’ tih ang chi natna a awm thei a ni. A tam zawkah chuan hei hi hormone lama harsatna vang a ni. Heng hormone influence te hi nu leh pa atanga genes kaltlangin a kal thei a, a nih loh leh chhan hriat lohvin a thleng thei bawk.

‘Atypical Genitalia’ awm chhante chu engte nge ni?

A tam zawkah chuan hei hi a chhan ber chu naupai laia naute taksaa hormone mumal lo awm vang a ni.Heng hormone te hian nausen sex organs than chhoh dan an thunun a. Chuvangin, heng hormone-te hi inthlauhna a awm chuan naute sex organs lo thanglian dan a nghawng dawn a ni. Heng chhante hi naute sex chromosome a zirin a danglam thei.

46 XX DSD (Hmeichhia chromosome pattern nei DSD) .

Hetiang dinhmunah hian naute genetic makeup chu hmeichhia (XX) a ni. Chumi awmzia chu naute hian ovary leh a chhungah uterus a nei tihna a ni. Mahse, pawn lam serh chu mipa ang maiin a lang thei a, penis leh testicles a nei bawk. Hei hi naute pawn lam taksa peng hrang hrangte lo thanglian zelnaah mipa hormone (androgens) level sang zawka an awm chuan a thleng thin. Hei hi a chhan langsar ber chu ‘Congenital Adrenal Hyperplasia’ (CAH) an tih hi a ni. Hei hi naute adrenal glands-in mipa hormone tam lutuk a siam chhuah hun a ni.

46 XY DSD (DSD mipa chromosome pattern nei) 1.1.

Hetiang a nih chuan naute genetic makeup chu mipa (XY) a ni. Chumi awmzia chu naute hian a chhungah testicles (testes) a nei tur a ni. Mahse, pawn lam sengte hian mipa zia chiang takin a lantir lo va, hmeichhe mizia nen pawh a inang thei hle. Hei hi a chhan lian tak tak engemaw zat a awm a:

• Nausen testicles te chu a thang tha lo hle.
• Testicles hian mipa hormone testosterone hi a siam chhuak tling lo.
• Testosterone siam chhuah ni mah se, naute taksain a hmang dik thei lo.

Gonadal Differentiation-a harsatna awm thei

Hetiang a nih chuan naute gonads te hi testes emaw ovary emaw ah a lo thang kim lo tihna a ni.

• Mixed Gonadal Dysgenesis: Hetah hian gonads (testes emaw ovaries ) te hi a lo thang kim lo.
• Partial Gonadal Dysgenesis: Hetah hian naute gonads-ah hian testicular tissue thenkhat lo awm tawh mahse, chu testicular te chu a thawk tha lo hle.
• Gonadal Dysgenesis: Hetah hian gonad pahnih hi a hmaa an awm a, chu chu testicles emaw ovary emaw-ah an lo thang lian lo tihna a ni.

Ovotesticular DSD (ovum-testicular DSD) tih a ni a, a chhuahna tur hmun hi a awm lo.

Hei hi thil awm lo tak a ni. Chutiang a nih chuan naute gonads-ah hian ovarian tissue leh testicular tissue te pawh a awm thei. A nih loh leh, a sir khatah ovary a awm thei a, a sir lehlamah testicle a awm thei bawk.

Pawn lam atang chuan ‘Atypical Genitalia’ natna hi eng ang nge a nih?

He natna lan dan hi a nghawng chromosome leh hormone dinhmun a zirin a inang lo. Naute genetically female (XX chromosomes)-a atypical genitalia hian a hnuaia thil awmte hi a nei thei a ni:

• Clitoris chu a lian a, penis te tak te ang maiin a lang.
• Urethral opening hi a awmna pangngai ni lovin scrotum hnuaiah emaw, vagina chhungah emaw a awm a.
• Labia te chu a inzawm khawm a, scrotum ang maiin a lang.
• Labia inkarah hian tissue lump a awm thei a, chu chu testicles awmna pouch ang mai a ni thei.

Naute genetically male (XY chromosomes)-a atypical genitalia-ah hian:

• Penis hi a awm lo vek emaw, a te hle emaw a, penis lian zawk (micropenis) ang mai a ni.
• Urethral opening hi penis bul lamah emaw, scrotum inkarah emaw a awm a, a tip-ah ni lovin (he dinhmun hi ‘Hypospadias’ an ti bawk).
• Scrotum hi a te a, hmun hnih ah a inthen a, labia ang mai a ni.
• Testicles chu pum chhung atanga lo chhuk a ni tawh a, scrotum ( undescended testicles ) ah a awm tawh lo.

‘Atypical Genitalia’ natna lan chhuahna dangte chu engte nge ni?

Pawn lam mipat hmeichhiatna lan dan hi a zia ber a ni. Chu bâkah, hetiang thilte hi i hmu thei bawk:

• Hormone inthlauhna (hormone inthlauhna) a awm.
• Hmeichhe naupang tan chuan thlasik a intan hma hle thei a, a tlai thei a, a thleng lo thei bawk.
• Hypospadias chu urethral opening chu penis tip-ah a awm loh chuan a ni.

Engtin nge doctor-te hian he natna hi an hriat theih?

Doctor te chuan naute a pian veleh he natna hi an hmuchhuak nghal fo thin . A nih loh leh, naute well-child exam hmasa ber lai khan. Naute hi mipa nge hmeichhia tih a chiang lo a nih chuan naute doctor chuan test eng emaw zat tih belh leh turin a ti ang.

I fa medical checkup neih chhung hian eng nge beisei tur?

Doctor chuan i chhungkaw medical history a zawt hmasa ang che. Tichuan, naute pawn lam serh chu uluk takin a enfiah ang. Diagnosis dik tak siam nan a hnuaia test te hi tih theih a ni:

• Thisen test: Naute hormone level leh chromosomes enfiah nan.
• Imaging test: Hengte hian naute chhungril lam a en thei a, chu chu ultrasound, X-ray emaw MRI (Magnetic Resonance Imaging) scan emaw a ni.
• Biopsy emaw Laparoscopy: Naute gonads atanga tissue te tak te lak chhuah a, enfiah tur.

A châng chuan diagnosis siam thuai theih a ni. Mahse, a chang chuan hun rei tak a daih thei. Diagnosis dik tak neih hi a pawimawh hle. Chutiang chuan naute sex assignment chu i ruat thei a, enkawlna dik tak i ruahman thei bawk.

Engtin nge ‘Atypical Genitalia’ hi enkawl a nih?

Enkawl tan hmain a hmasa ber atan he natna hi a chhan dik tak hriat chhuah a ni. Tichuan, hormone test leh test dang result a\angin medical team chuan naute enkawlna tur ruahmanna leh sex assignment dik tak chu an rel ang. Hei hi thil buaithlak leh sensitive tak a nih avangin multidisciplinary team an awm ang che. Hetiang dinhmunah hian thiamna nei mi zawn a pawimawh hle. Thurawn tha ber ber pe theitu tur che an ni ang. He team-ah hian specialist te pawh an tel thei a, chungte chu:

• Nausen piang thar (Neonatologist) a ni
• Geneticist lam thiam a ni
• Endocrinologist a ni
• Surgeon a ni
• Urologist (Urologist) a ni
• Psychologist a ni

Nang leh he medical team te hian in fate hun rei tak chhunga a dam theih dan tur te, nakin lawka a mipat hmeichhiatna hman dan tur te, leh nau neih theihna tur te chungchangah thutlukna tha ber ber in siam ang. Enkawlna chu thil tam takah a innghat ang a, chung zingah chuan:

• Naute dinhmun chhan.
• Nausen serh a lan dan.
• I chhungte duhdan leh nunphung rin dan.

Enkawlna kawng hrang hrangte chu:

• Hormone Replacement Therapy (HRT): Hormone inthlauhna a awm chuan hormone therapy chauh hmangin he natna hi control theih a ni.
• Reconstructive surgery: A châng chuan, medical team-te chuan an sawiho thei che a, i naute chu a la naupan laiin operation neih an rawt thei bawk. Mahse, ‘Atypical Genitalia’ tana he reconstructive surgery chungchangah hian ngaihdan hrang hrang a awm. A chang chuan, entirnan, naute hian zun kalna tur kawngkhar a neih loh chuan damdawi lamah chuan zai a ngai thin. Mahse, surgery hi a lan dan tihchangtlun nan a ni ber a nih chuan nu leh pa thenkhat chuan naute a lo puitlin hma leh anmahni taksa chungchangah thutlukna an siam theih hma chu nghah an thlang thin. Hei hi thutlukna pawimawh tak leh ngaihtuah fel tak a ni.

Hriat reng tur: He surgical decision hian i fa hmalam hun nunah nghawng lian tak a nei thei a, chuvangin he lama thiamna nei counselor nena inbiakna neih a tha.

Ka fa hian ‘Atypical Genitalia’ a nei a nih chuan eng nge ka beisei ang?

‘Atypical Genitalia’ natna hian naupang taksaah chauh ni lovin, khawtlang leh rilru lamah pawh nghawng thui tak a nei a ni.Mipat hmeichhiatna (genitals) awm dan avanga lo awm thei a ni a, chu chuan naupangin a hriatna a nghawng thei a ni. Surgery neih tura thutlukna siam hian naupang nunphung a nghawng thei a ni. Hei vang hian hemi chungchangah hian mithiamte thurawn lak a pawimawh hle. ‘Atypical Genitalia’ tih hmuhchhuah hian lungngaihna nasa tak thlen thei mah se, i medical team chuan enkawlna tha ber tur rel turin an pui ang che.

Engtikah nge ka fa ‘Atypical Genitalia’ natna chungchangah hian doctor thurawn ka lak ang?

Doctor chuan he natna hi a pian tirh emaw, naute damdawi lam enfiah hmasak ber emaw a nih laiin a hmuchhuak tlangpui. Chumi hnuah chuan doctor chuan diagnosis leh treatment options te chu i hnenah a sawipui ang che.

Ka fa doctor hnenah eng zawhna nge ka zawh ang?

Hetiang hunah hian i rilruah zawhna tam tak i nei mai thei. Hêng zawhnate hian a ṭanpui thei che a ni:

• Ka fa hian enkawlna a mamawh nghal em, a nih loh leh rei vak lo kan nghak a, nakinah thutlukna kan siam thei ang em?
• Ka fa hian eng specialist nge a hmuh ang?
• Genetic counselor kan hmu tur em ni?
• Ka fa hmalam hun chu eng ang nge ni ang?
• ‘Atypical Genitalia’ vei mi leh chhungkaw tan support group a awm em?

Ka fa hian nakin lawkah fa a nei thei ang em?

Nakin lawka nau neih theihna leh mipat hmeichhiatna hman dan tur chu he natna thlentu, Atypical Genitalia-ah a innghat a ni. Zirna thenkhatah chuan:

• Hmeichhe naupang Congenital Adrenal Hyperplasia (CAH) vei te chu hormone hmanga enkawlna an dawn hnuah nau an pai thei a ni.
• Mipa naupang pakhat tal chu normally functioning testicle nei chuan fa an nei thei a rinawm.
• Ovotesticular DSD vei, ovary hnathawh dan pangngai nei te chuan fa an nei thei mai thei.
• Testicular dysgenesis awm mahse, nausen pum tha tak nei te chuan implanted embryo chu an chhawm nung thei a ni.

A tawp berah chuan hriat reng tur (Take-Home Message) .

I naute piang thar hian ‘Atypical Genitalia’ a nei tih hriat chu nang leh i chhungte tan chuan thil mak tak, lungchhiatthlak leh buaithlak tak a ni thei tih kan hrethiam. I chhungte leh medical team te nen medical, ethical leh psychological issue tam tak i sawiho a ngai dawn a ni.

Mahse khawngaihin hre reng ang che, he zinkawngah hian nangmah chauh i ni lo. I medical team chuan i mamawh information, support leh advice an pe ang che. An puihnain i naute sex leh enkawl dan tur chungchangah thutlukna tha ber ber i siam thei a, i naute chu nun tha ber i pe thei a, thil tha ber ber i hmu thei bawk. Huaisenna hloh suh. Hriatna dik leh thlawpna i neih chuan he harsatna hi hmachhawn turin chakna i nei ang.

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`Atypical Genitalia, Ambiguous Genitalia, DSD, Mipat hmeichhiatna, Hormone, Chromosomes, Naupang hriselna, Genetic Conditions, Congenital Adrenal Hyperplasia, Sexual Development