Androgen lefu la ho se tsotelle

Androgen insensitivity syndrome (AIS) ke ha motho ea nang le liphatsa tsa lefutso (ea nang le X le e le 'ngoe ea chromosome) a hanana le lihormone tsa banna (tse bitsoang androgens). Ka lebaka leo, motho o na le tse ling tsa litšobotsi tsa 'mele tsa mosali, empa liphatsa tsa lefutso tsa monna.

AIS e bakoa ke bofokoli ba liphatsa tsa lefutso ho X chromosome. Liphoso tsena li etsa hore 'mele o sitoe ho arabela lihormone tse hlahisang ponahalo ea monna.

Syndrome e arotsoe ka mekhahlelo e 'meli e meholo:

• Tlatsa AIS
• Karolo ea AIS

Ho AIS e felletseng, botona le likarolo tse ling tsa 'mele tsa banna li hloleha ho hola. Ha a hlaha, ngoana o shebahala joaloka ngoanana. Mofuta o felletseng oa lefu lena o hlaha ho ba 1 ho ba 20 ba tsoaloang ba phela.

Ho AIS e sa fellang, batho ba na le lipalo tse fapaneng tsa litšobotsi tsa banna.

Karolo ea AIS e ka kenyelletsa mathata a mang, a joalo ka:

• Ho hloleha ha testes e le 'ngoe kapa ka bobeli ho theohela mokhoeng kamora ho hlaha
• Hypospadias, boemo boo ho bona ho buloang ha urethra ho ka tlas'a botona, ho fapana le ntlheng
• Reifenstein syndrome (e tsejoang hape e le lefu la Gilbert-Dreyfus kapa lefu la Lubs)

Infertile male syndrome le eona e nkuoa e le karolo ea karolo e itseng ea AIS.

Motho ea nang le AIS e felletseng o bonahala e le mosali empa ha a na popelo. Ba na le moriri o monyane haholo oa sephakeng le oa pepeneneng. Ha u kena bohlankaneng kapa boroetsaneng, litšoaneleho tsa botona le botšehali (joalo ka matsoele) lia hlaha. Leha ho le joalo, motho ha a ilele khoeli ebe oa emola.

Batho ba nang le karolo ea AIS ba ka ba le litšobotsi tsa 'mele tsa banna le tsa basali. Ba bangata ba koaloa ka karolo e itseng ea botshehadi ba ka ntle, selelo se holisitsoeng le botshehadi bo bokhuts'oane.

Ho ka ba le:

• Lera la botshehadi empa ha ho na popelo kapa popelo
• Inguinal hernia e nang le li-testes tse ka utluoang nakong ea tlhahlobo ea 'mele
• Mabele a tloaelehileng a basali
• Liteko ka mpeng kapa libakeng tse ling tse sa tloaelehang 'meleng

AIS e felletseng ha e fumanehe hangata bongoaneng. Ka nako e 'ngoe, kholo e utloahala ka mpeng kapa bothateng bo fetohang testicle ha e hlahlojoa ka ts'ebetso ea bongaka. Batho ba bangata ba nang le boemo bona ha ba fumanoe ho fihlela ba sa ilele khoeli kapa ba ba le bothata ba ho ima.

Karolo e 'ngoe ea AIS e fumaneha khafetsa nakong ea bongoana hobane motho a ka ba le litšobotsi tsa' mele tsa banna le tsa basali.

Liteko tse sebelisetsoang ho fumana boemo bona li ka kenyelletsa:

• Mosebetsi oa mali ho lekola maemo a testosterone, hormone ea luteinizing (LH), le hormone e hlohlelletsang follicle (FSH)
• Ho etsa liphatsa tsa lefutso (karyotype) ho fumana hore na motho o entsoe joang
• Pelvic ultrasound

Liteko tse ling tsa mali li ka etsoa ho thusa ho tseba phapang lipakeng tsa khaello ea AIS le androgen.

Likokoana-hloko tse sebakeng se fosahetseng li kanna tsa se ke tsa tlosoa ho fihlela ngoana a qeta ho hola le ho kena bohlankaneng. Nakong ena, li-testes li ka tlosoa hobane li ka ba le mofetše, joalo ka testicle efe kapa efe e sa rateheng.

Phetoho ea estrogen e ka laeloa kamora bocha.

Kalafo le kabelo ea bong e ka ba taba e thata haholo, 'me e tlameha ho lebisoa ho motho ka mong.

Pono ea AIS e felletseng e ntle haeba lisele tsa testicle li tlosoa ka nako e nepahetseng ho thibela mofetše.

Mathata a kenyelletsa:

• Ho hloka thari
• Litaba tsa kelello le sechaba
• Kankere ea testicular

Bitsa mofani oa tlhokomelo ea bophelo haeba uena kapa ngoana oa hau a e-na le matšoao a lefu lena.

Testicular bosali

• Anatomy ea banna ea ho ikatisa
• Anatomy ea basali ea ho ikatisa
• Anatomy ea basali ea ho ikatisa
• Ho ngola litlhaku

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Donohoue PA. Mathata a kholo ea kholo ea thobalano. Ka: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, bahlophisi. Buka ea Nelson ea Pediatrics. La 21 Ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 606.

Yu RN, DA DA. Mathata a kholo ea kholo ea thobalano: etiology, tekolo le tsamaiso ea bongaka. Ka: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, bahlophisi. Campbell-Walsh-Wein Urology. 12th ed. Philadelphia, PA: Elsevier; 2021: khaolo ea 48.