Androgen matenda osamva

Androgen insensitivity syndrome (AIS) ndi pamene munthu yemwe ali wamwamuna (yemwe ali ndi X imodzi ndi Y chromosome) amalimbana ndi mahomoni amphongo (otchedwa androgens). Zotsatira zake, munthuyo ali ndi zina mwakuthupi zamunthu wamkazi, koma chibadwa chamunthu.

AIS imayambitsidwa ndi zolakwika zamtundu pa X chromosome. Zolakwika izi zimapangitsa thupi kulephera kuyankha mahomoni omwe amatulutsa mawonekedwe achimuna.

Matendawa agawika m'magulu awiri akulu:

• Malizitsani AIS
• Tsankho AIS

Mu AIS wathunthu, mbolo ndi ziwalo zina zamwamuna zimalephera kukula. Pobadwa, mwanayo amawoneka ngati mtsikana. Mtundu wathunthu wamatendawa umapezeka mwa ana 1 mwa 20,000 obadwa amoyo.

Mwapang'ono pa AIS, anthu ali ndi mitundu yosiyanasiyana yamikhalidwe yamwamuna.

Tsankho AIS lingaphatikizepo zovuta zina, monga:

• Kulephera kwa mayeso amodzi kapena onse awiri kuti abwere m'matumba atabadwa
• Hypospadias, vuto lomwe kutsegula kwa mtsempha kuli pansi pamunsi pa mbolo, m'malo mofikira kumapeto
• Matenda a Reifenstein (amadziwikanso kuti Gilbert-Dreyfus syndrome kapena matenda a Lubs)

Matenda aamuna osabereka amawerengedwanso kuti ndi gawo limodzi la AIS.

Munthu yemwe ali ndi AIS wathunthu amawoneka ngati wamkazi koma alibe chiberekero. Ali ndi tsitsi lalifupi komanso lapakhosi. Pakutha msinkhu, zikhalidwe zogonana zazimayi (monga mabere) zimayamba. Komabe, munthu samasamba ndikukhala wachonde.

Anthu omwe ali ndi tsankho la AIS atha kukhala ndi mawonekedwe amuna ndi akazi. Ambiri amatseka pang'ono kumaliseche akunja, chikumbu chokulitsidwa, ndi nyini yayifupi.

Pakhoza kukhala:

• Nyini koma yopanda chiberekero kapena chiberekero
• Matenda a Inguinal ndi ma testes omwe amatha kumveka mukamayesa thupi
• Mabere achikazi abwinobwino
• Kuyesedwa pamimba kapena malo ena onyansa mthupi

AIS wathunthu samapezeka nthawi zambiri ali mwana. Nthawi zina, kukula kumamveka m'mimba kapena kubuula komwe kumadzakhala chikumbu chikafufuzidwa ndi opaleshoni. Anthu ambiri omwe ali ndi vutoli sapezeka mpaka atayamba kusamba kapena zimawavuta kutenga pakati.

Partial AIS imapezeka nthawi yaubwana chifukwa munthuyo amatha kukhala ndi zikhalidwe za amuna ndi akazi.

Mayeso omwe amagwiritsidwa ntchito kuti apeze vutoli atha kukhala:

• Magazi amagwira ntchito kuti aone kuchuluka kwa testosterone, mahomoni a luteinizing (LH), ndi mahomoni othandizira (FSH)
• Kuyesedwa kwa majini (karyotype) kuti mudziwe mtundu wa munthuyo
• Pelvic ultrasound

Mayeso ena amwazi angapangidwe kuti athandize kusiyanitsa pakati pa kuchepa kwa AIS ndi androgen.

Machende omwe ali pamalo olakwika sangathe kuchotsedwa mpaka mwana atamaliza kukula ndikudutsa msinkhu. Pakadali pano, ma testes amatha kuchotsedwa chifukwa amatha kukhala ndi khansa, monganso machende osavomerezeka.

Estrogen amatha kusintha m'malo mwake atatha msinkhu.

Chithandizo ndi kutumizidwa pakati pa amuna ndi akazi imatha kukhala nkhani yovuta kwambiri, ndipo imayenera kulunjika kwa munthu aliyense payekha.

Maganizo a AIS athunthu ndi abwino ngati minyewa ya machende imachotsedwa nthawi yoyenera kupewa khansa.

Zovuta zimaphatikizapo:

• Kusabereka
• Nkhani zamaganizidwe ndi chikhalidwe
• Khansa ya testicular

Itanani foni kwa omwe akukuthandizani ngati inu kapena mwana wanu muli ndi zizindikiro za matendawa.

Mayeso achikazi

• Kutengera kwamwamuna kubereka
• Matupi achikazi oberekera
• Matupi achikazi oberekera
• Zojambula

Chan YM, Hannema SE, Achermann JC, Hughes IA. Zovuta zakukula kwakugonana. Mu: Melmed S, Auchus RJ, Goldfine AB, Koenig RJ, Rosen CJ, olemba. Buku la Williams la Endocrinology. Wolemba 14th. Philadelphia, PA: Elsevier; 2020: chap 24.

Donohoue PA. Zovuta zakukula kwakugonana. Mu: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, olemba. Nelson Textbook of Pediatrics. Wolemba 21. Philadelphia, PA: Elsevier; 2020: chap 606.

Yu RN, Daimondi DA. Zovuta zakukula kwakugonana: etiology, kuwunika, ndi kasamalidwe ka zamankhwala. Mu: Partin AW, Dmochowski RR, Kavoussi LR, Peters CA, olemba. Campbell-Walsh-Wein Urology. Wolemba 12. Philadelphia, PA: Elsevier; 2021: mutu 48.