Isini esingaqondakaliyo

I-genitalia engaqondakaliyo sisiphene sokuzalwa apho amalungu esini angaphandle engakhange abonakale njengenkwenkwe okanye intombazana.

Isini semfuza somntwana simiselwe ekukhawulweni. Iseli yeqanda likamama liqukethe i-chromosome engu-X, ngelixa imbewu yesidoda sikayise iqulethe i-X okanye i-Y chromosome. Ezi chromosomes X kunye no-Y zichonga isini semfuzo somntwana.

Ngokwesiqhelo, usana luzuza njengelinye isibini see-chromosomes zesini, i-1 X kumama kunye ne-1 X okanye enye kuY kutata. Utata "umisela" isini semfuza somntwana. Usana oluza ilifa i-X chromosome kuyise ngumntu obhinqileyo wemfuza kwaye une-2 X chromosomes. Usana oluza ilifa i-Y chromosome kuyise yindoda yemfuza kwaye ine-1 X kunye ne-1 y chromosome.

Ilungu lokuzala kunye nelungu lobufazi kunye nezitho zangasese zombini zivela kwisicubu esifanayo kumntwana ongekazalwa. Iimpawu ezingaqondakaliyo zesini zinokuphuhla ukuba inkqubo ebangela ukuba izicwili ezingekazalwa zibe "yindoda" okanye "ibhinqa" ziphazamiseke. Oku kwenza ukuba kube nzima ukuchonga ngokulula usana njengendoda okanye ibhinqa. Ubungakanani bokungaqondakali buyahluka. Kunqabile kakhulu, ukubonakala komzimba kunokukhula ngokupheleleyo njengokuchasene nesini semfuza. Umzekelo, indoda yemfuza inokuthi ikhule imbonakalo yomfazi oqhelekileyo.

Kwiimeko ezininzi, i-genitalia engacacanga kumabhinqa (abantwana abane-2 X chromosomes) banezi zinto zilandelayo:

• I-clitoris eyandisiweyo ebonakala ngathi yipenisi encinci.
• Ukuvulwa kwe-urethral (apho kuphuma khona umchamo) kunokuba naphina, ngaphezulu, okanye ngaphantsi komphezulu we-clitoris.
• Ilebhu inokuthi idibaniswe kwaye ijongeke njengesikram.
• Usana lunokucingelwa ukuba luyindoda enamatyhalarha angalindelwanga.
• Ngamanye amaxesha iqhuma leethishu livakala ngaphakathi kwelebia edibeneyo, ukuyenza ibonakale ngathi sisikram kunye namatyhalarha.

Kwindoda yemfuza (i-1 X kunye ne-1 y chromosome), amalungu esini afanayo ahlala edibanisa ezi zinto zilandelayo:

• Ilungu lobudoda elincinci (elingaphantsi kweesentimitha ezi-2 ukuya kwezi-3, okanye u-3/4 ukuya ku-1/4 ye-intshi) ebonakala ngathi ikhulisiwe (i-clitoris yebhinqa elisandula ukuzalwa liqhele ukwandiswa xa kuzalwa).
• Ukuvulwa kwe-urethral kunokuba naphi na, ngaphezulu, okanye ngaphantsi kwepenisi. Inokubekwa esezantsi njenge-perineum, isenza ukuba usana lubonakale njengowasetyhini.
• Kunokubakho iscrotum esincinci esahluliweyo kwaye esijongeka njengelebia.
• Amatyhalarha angachazwanga aqhele ukwenzeka ngamalungu esini adidayo.

Ngaphandle kwemeko ezimbalwa, amalungu omzimba angacacanga ahlala engasongeli ubomi. Nangona kunjalo, inokudala iingxaki zentlalo zomntwana kunye nosapho. Ngesi sizathu, iqela leengcali ezinamava, kubandakanya i-neonatologists, i-geneticists, i-endocrinologists, kunye noogqirha bengqondo okanye oonontlalontle baya kubandakanyeka kukhathalelo lomntwana.

Izizathu zokuba amalungu omzimba angacacanga aquka:

• Pseudohermaphroditism. Ilungu lesini lelinye lesini, kodwa ezinye iimpawu zomzimba wesinye isini zikhona.
• I-hermaphroditism yokwenyani. Le yimeko enqabileyo kakhulu, apho izicwili ezivela kuzo zombini ii-ovari kunye namasende zikhona. Umntwana unokuba neenxalenye zesini esini sobudoda nesobufazi.
• Umxube we-gonadal dysgenesis (MGD). Le yimeko ye-intersex, apho kukho ezinye izakhiwo zamadoda (gonad, testis), kunye nesibeleko, ubufazi, kunye neetyhubhu ze-fallopian.
• I-hyperplasia yokuzalwa ye-adrenal. Le meko ineendlela ezininzi, kodwa eyona ndlela iqhelekileyo ibangela ukuba umntu obhinqileyo wemfuza abonakale eyindoda. Amazwe amaninzi avavanya le meko inokubusongela ubomi ngexesha lokuhlolwa kovavanyo olusandul 'ukuzalwa.
• Ukuchaphazeleka kweChromosomal, kubandakanya iKlinefelter's syndrome (XXY) kunye neT Turner syndrome (XO).
• Ukuba umama uthatha amayeza athile (njenge-androgenic steroids), umntu obhinqileyo wemfuzo unokujongeka njengowesilisa.
• Ukunqongophala kwemveliso yehomoni ezithile kunokubangela ukuba umbungu ukhule ngohlobo lomzimba wabasetyhini, nokuba ungubani na ngokwesini.
• Ukunqongophala kwee-testosterone zeseli receptors. Nokuba umzimba wenza iihomoni ezifunekayo ukuze zikhule zibe yindoda ebonakalayo, umzimba awunakuphendula kula mahomoni. Oku kuvelisa uhlobo lomzimba wabasetyhini, nokuba isini semfuza siyindoda.

Ngenxa yeziphumo ezinokubakho kwezentlalo nangokwengqondo kule meko, abazali kufuneka benze isigqibo malunga nokuba bangamkhulisa njani umntwana oyindoda okanye umntu obhinqileyo kwangoko emva kokuxilongwa. Kungcono ukuba esi sigqibo senziwe kwiintsuku zokuqala zobomi. Nangona kunjalo, esi sisigqibo esibalulekileyo, ke abazali akufuneki basingxamele.

Nxibelelana nomboneleli wakho wokhathalelo lwempilo ukuba ukhathazekile malunga nokubonakala kwamalungu angaphandle omntwana wakho, okanye umntwana wakho:

• Ithatha ngaphezulu kweeveki ezi-2 ukubuyisa ubunzima bayo bokuzalwa
• Ngaba iyagabha
• Ijongeka ngathi yonakele emzimbeni (yomile ngaphakathi emlonyeni, akukho zinyembezi xa ulila, ngaphantsi kwe-4 diapers emanzi ngeyure ezingama-24, amehlo ajongeka ngathi atshonile)
• Unciphile ukutya
• Unobumba obuhlaza okwesibhakabhaka (amaxesha amafutshane xa inani elincitshisiweyo legazi lingena kwimiphunga)
• Unengxaki yokuphefumla

Zonke ezi zinto zinokuba ziimpawu zokuzalwa kwe-adrenal hyperplasia.

Isini esingaqondakaliyo sinokufunyanwa ngexesha lovavanyo lokuqala lomntwana olungileyo.

Umboneleli uya kwenza uvavanyo lomzimba olunokuthi luveze amalungu esini angabi "yindoda eqhelekileyo" okanye "ibhinqa eliqhelekileyo," kodwa kwindawo ethile phakathi.

Umboneleli uya kubuza imibuzo yembali yezonyango ukunceda ekuchongeni nasiphi na isifo se-chromosomal. Imibuzo inokubandakanya:

• Ngaba ikhona imbali yosapho yokuphuma kwesisu?
• Ngaba ikhona imbali yosapho yokuzalwa komntwana oswelekileyo?
• Ngaba ikhona imbali yosapho yokufa kwangoko?
• Ngaba kukho naliphi na ilungu losapho elineentsana ezisweleke kwiiveki ezimbalwa zokuqala zobomi okanye ezazinamalungu omzimba angathandekiyo?
• Ngaba ikhona nayiphi na imbali yosapho yazo naziphi na iingxaki ezibangela amalungu esini athandabuzekayo?
• Ngawaphi amayeza athathwe ngumama ngaphambi okanye ngexesha lokukhulelwa (ngakumbi ii-steroids)?
• Zeziphi ezinye iimpawu ezikhoyo?

Uvavanyo lwemfuzo lunokumisela ukuba ngaba umntwana uyindoda okanye libhinqa. Rhoqo isampulu encinci yeeseli inokukhutshwa ngaphakathi kwizidlele zomntwana kolu vavanyo. Ukuvavanywa kwezi seli kuhlala kunokwanela ukumisela isini semfuza sosana. Uhlalutyo lweChromosomal luvavanyo olubanzi ngakumbi olunokufuneka kwiimeko ezingathandabuzekiyo.

I-Endoscopy, i-x-ray esiswini, isisu okanye i-pelvic ultrasound, kunye neemvavanyo ezifanayo ezinokufuneka ukumisela ubukho okanye ukungabikho kwamalungu angaphakathi esini (anje ngee testes ezingafunekiyo).

Uvavanyo lweLabhoratri lunokunceda ukumisela ukuba zisebenza njani na izinto zokuzala. Oku kunokubandakanya uvavanyo lwe-adrenal kunye ne-gonadal steroids.

Kwezinye iimeko, i-laparoscopy, i-laparotomy yokuhlola, okanye i-biopsy ye-gonads inokufuneka ukuze kuqinisekiswe ukuphazamiseka okunokubangela amalungu esini afanayo.

Kuxhomekeka kwisizathu, utyando, ukutshintshwa kwehomoni, okanye ezinye iindlela zonyango zisetyenziselwa ukunyanga iimeko ezinokubangela amalungu esini afanayo.

Ngamanye amaxesha, abazali kufuneka bakhethe ukuba bakhulise umntwana njengendoda okanye umntu obhinqileyo (ngaphandle kwama chromosomes omntwana). Olu khetho lunokuba nefuthe elikhulu kwezentlalo nangokwengqondo emntwaneni, ke iingcebiso zihlala zicetyiswa.

Phawula: Ihlala ilula ngokunyanga ngobuchwephesha (kwaye ke ikhulise) umntwana njengowasetyhini. Kungenxa yokuba kulula kugqirha ukuba enze ilungu langasese labasetyhini kunokuba lenze amalungu esini obudoda. Ke ngoko, ngamanye amaxesha oku kuyacetyiswa nokuba umntwana ukwimfuza. Nangona kunjalo, esi sisigqibo esinzima. Kuya kufuneka uyixoxe nosapho lwakho, umboneleli womntwana wakho, ugqirha wotyando, ugqirha wakho we-endocrinologist, kunye namanye amalungu eqela lokhathalelo lwempilo.

Izitho zangasese - ingacaci

• Ukuphazamiseka kokukhula kwilungu lobufazi kunye nelungu lobufazi

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Rey RA, Josso N.Ukuchongwa kunye nokunyangwa kokuphazamiseka kuphuhliso lwezesondo. Ku: Jameson JL, De Groot LJ, de Kretser DM, et al, ii-eds. Endocrinology: Abantu abadala kunye nabantwana. Umhla wesi-7. IPhiladelphia, PA: Elsevier Saunders; Ngo-2016: isahluko 119.

IPc emhlophe. Ukuphazamiseka kuphuhliso lwezesondo. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Umhla wama-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahl 233.

IPc emhlophe. I-hyperplasia ye-adrenal ye-congenital kunye nokuphazamiseka okunxulumene noko. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 594.