Matenda ochepera a androgen osazindikira

Matenda a androgen insensitivity syndrome (PAIS) ndimatenda omwe amapezeka mwa ana pomwe thupi lawo silingayankhe moyenera ma hormone amphongo achimuna (androgens). Testosterone ndimadzi ogonana amuna.

Matendawa ndi mtundu wa androgen insensitivity syndrome.

M'miyezi iwiri kapena itatu yoyambirira ya mimba, ana onse amakhala ndi maliseche ofanana. Mwana akamakula m'mimba, maliseche amphongo kapena achimuna amakula kutengera ma chromosomes ogonana ochokera kwa makolo. Zimadaliranso magawo a androgens. Mwa mwana yemwe ali ndi ma chromosomes a XY, mayendedwe ambiri a androgens amapangidwa mumayeso. Mwana uyu amakhala ndi maliseche. Mwa mwana yemwe ali ndi ma chromosomes XX, mulibe mayeso ndipo magawo a androgens ndiotsika kwambiri. Mwana uyu amakhala ndi maliseche achikazi. Mu PAIS, pali kusintha kwa jini komwe kumathandiza thupi kuzindikira ndikugwiritsa ntchito mahomoni achimuna moyenera. Izi zimabweretsa mavuto ndikukula kwamimba yamwamuna. Pobadwa, mwanayo akhoza kukhala ndi ziwalo zobisika, zomwe zimabweretsa chisokonezo pa kugonana kwa mwanayo.

Matendawa amapitilira kudzera m'mabanja (obadwa nawo). Anthu omwe ali ndi ma chromosome awiri X samakhudzidwa ngati mtundu umodzi wokha wa X chromosome ungakhale ndi kusintha kwa majini. Amuna omwe amalandira cholowa kuchokera kwa amayi awo adzakhala ndi vutoli. Pali mwayi wa 50% kuti mwana wamwamuna wa mayi yemwe ali ndi jiniyo adzakhudzidwa. Mwana aliyense wamkazi amakhala ndi mwayi 50% wonyamula jini. Mbiri yakubanja ndiyofunikira pozindikira zoopsa za PAIS.

Anthu omwe ali ndi PAIS atha kukhala ndi mawonekedwe amuna ndi akazi. Izi zingaphatikizepo:

• Ziwalo zoberekera zazimuna, monga urethra wokhala kumunsi kwa mbolo, mbolo yaying'ono, kachilombo kakang'ono (kokhala ndi mzere pakati kapena kutsekedwa kwathunthu), kapena machende osavomerezeka.
• Kukula kwa m'mawere mwa amuna panthawi yakutha msinkhu. Kutsika kwa tsitsi ndi ndevu, koma tsitsi labwinobwino komanso lapakhosi.
• Kulephera kugonana komanso kusabereka.

Wothandizira zaumoyo adzayesa.

Mayeso atha kuphatikiza:

• Kuyezetsa magazi kuti muwone kuchuluka kwa mahomoni achimuna ndi achikazi
• Mayeso a chibadwa monga karyotyping kuti awone ma chromosomes
• Kuwerengera kwa umuna
• Chidziwitso
• Pelvic ultrasound kuti muwone ngati ziwalo zoberekera zazimayi zilipo

Makanda omwe ali ndi PAIS atha kupatsidwa mwayi wogonana amuna kapena akazi kutengera kukula kwa kusamvana kwawo. Komabe, kugawana amuna ndi akazi ndi nkhani yovuta ndipo kuyenera kuganiziridwa mosamala. Chithandizo cha PAIS ndi monga:

• Kwa iwo omwe amapatsidwa mwayi wamwamuna, opareshoni amatha kuchitika kuti achepetse mawere, kukonza machende osavomerezeka, kapena kusintha mbolo. Amathanso kulandira ma androgens othandizira tsitsi lakumaso kukula ndikukulitsa mawu.
• Kwa iwo omwe apatsidwa ngati akazi, opareshoni itha kuchitidwa kuti ichotse machende ndikukhazikitsanso maliseche. Mahomoni aakazi a estrogen amapatsidwa nthawi yotha msinkhu.

Magulu otsatirawa atha kupereka zambiri ku PAIS:

• Intersex Society yaku North America - www.isna.org/faq/conditions/pais
• NIH Genetic and Rare Diseases Information Center - rarediseases.info.nih.gov/diseases/5692/partial-androgen-insensitivity-syndrome

Androgens ndiofunikira kwambiri pakukula msanga m'mimba. Anthu omwe ali ndi PAIS atha kukhala ndi moyo wathanzi komanso kukhala athanzi, koma atha kukhala ndi vuto lakutenga mwana. Pazovuta kwambiri, anyamata okhala ndi maliseche akunja kapena mbolo yaying'ono kwambiri amatha kukhala ndi mavuto amisala kapena malingaliro.

Ana omwe ali ndi PAIS ndi makolo awo atha kupindula ndi upangiri ndi chisamaliro kuchokera ku gulu lazachipatala lomwe limaphatikizapo akatswiri osiyanasiyana.

Itanani omwe akukuthandizani ngati inu, mwana wanu wamwamuna, kapena wachibale wamwamuna muli osabereka kapena osakwanira kukula kwa maliseche achimuna. Kuyesedwa kwa majini ndi upangiri kumalimbikitsidwa ngati PAIS akukayikiridwa.

Kuyezetsa asanabadwe kulipo. Anthu omwe ali ndi mbiri ya banja la PAIS ayenera kulingalira za upangiri wa majini.

PAIS; Androgen insensitivity syndrome - tsankho; Testicular ukazi wosakwanira; Mtundu I banja losakwanira pseudohermaphroditism; Matenda a Lubs; Matenda a Reifenstein; Matenda a Rosewater

• Njira yoberekera yamwamuna

Achermann JC, Hughes IA. Matenda a ana a chitukuko cha kugonana. Mu: Melmed S, Polonsky KS, Larsen PR, Kronenberg HM, olemba. Buku la Williams la Endocrinology. Wolemba 13. Philadelphia, PA: Elsevier; 2016: chap 23.

Shnorhavorian M, Fechner PY. Zovuta zakusiyanitsa kwakugonana. Mu: Gleason CA, Juul SE, olemba. Matenda a Avery a Mwana Wongobadwa kumene. 10th ed. Philadelphia, PA: Elsevier; 2018: mutu 97.