He rerekētanga kei roto i te whanaketanga ā-ira tangata o tāu tamaiti? Me kōrero tātou mō te Androgen Insensitivity Syndrome!

Ina tupu te tamaiti i roto i te kōpū, i ētahi wā kāore ngā mea iti e rite ki te mea e tumanakohia ana. Tērā pea he raruraru iti ki ō rātou ira tangata i te whānautanga mai o ētahi tamariki. Kāore rānei ō rātou tinana e rerekē pērā i te mea e tumanakohia ana ina tae ki te pakeketanga. I ēnei wā, ka taea e tātou te whakaaro ki tētahi āhuatanga e kiia nei ko te `(Androgen Insensitivity Syndrome)`. Kaua e manukanuka, ka kōrerohia e mātou tēnei mea kia māmā noa iho, kia mārama ai koe.

He aha te mate kore aro o te Androgen?

Ki te kī māmā noa, ko te Androgen Insensitivity Syndrome (AIS) he āhuatanga e puta ai he tamaiti tāne ira (arā, he ira X tōna, he ira Y tōna), engari kāore tōna tinana e aro tika ana ki ngā homoni tāne, arā, ngā androgen . Whakaarohia pēnei: ​​he iti ngā kuaha i roto i ō tātou tinana e tuku karere ana ki ngā pūtau, ā, ka aro atu rātou ki ēnei homoni. I roto i te tangata whai AIS, kāore ēnei kuaha e mahi tika ana.

E kiia ana tēnei e ngā tākuta he mate rerekētanga ira tangata . I mua ko te mate whakawahinetanga o te pēpi te ira tāne, engari kāore taua ingoa e whakamahia ana i ēnei rā. Ka pā tēnei āhuatanga ki te whanaketanga o ngā whekau ira tangata o te pēpi i a rātou e noho ana i roto i te kōpū. Ka pā hoki ki te whanaketanga o ngā āhuatanga ira tangata e tika ana kia puta i te wā o te pakeketanga. He maha ngā wā, kāore e taea e te hunga whai mate pēnei te whānau tamariki i te pakeketanga (kore tamariki) .

He momo rerekē tēnei?

Āe, e toru ngā momo matua o te 'AIS'. He rerekē ngā āhuatanga o ia momo.

1. Te Mate Pāwera o te Androgen Katoa (CAIS):

• He rite tonu te āhua o waho o ēnei tāngata ki ngā kōtiro. Ara, he rite tonu ō rātou ira tangata ki ō te kōtiro.
• Heoi, kāore i a rātou ngā ira tāne o roto o te wahine (arā, ngā ovaries, ngā ngongo fallopian, te kōpū).
• I te nuinga o te wā, ka whakatipuria te hunga whai `CAIS` i roto i te whānautanga o ngā kōtiro.

2. Te Mate Pāwera o te Androgen Wāhanga (PAIS):

• Kāore pea ō rātou ira tangata o waho i tino whanake pērā i tō te tama, tērā pea he āhua rite ki tō te kōtiro, tērā pea he ranunga o ngā mea e rua, ā, he uaua ki te tautuhi mārama he tāne, he wahine rānei rātou (ira tangata koretake).
• Ko te hunga whai `PAIS` ka whakatipuria i ētahi wā he tama, i ētahi wā he kōtiro.

3. Te Mate Pāwera o te Androgen Ngāwari (MAIS):

• He rite te āhua o ō rātou ira tangata ki ō te tamaiti tāne.
• Heoi anō, kāore e taea e rātou te whānau tamariki (kore tamariki). E whakaaro ana ētahi tākuta ko te `MAIS` he wāhanga o te `PAIS` tonu.

Ko wai ka pāngia e tēnei mate? He pēhea te noa o tōna pānga?

Ko tēnei mate `AIS` i puta mai i te hapa o te `Androgen Receptor (AR) ira` , kei runga i te `X` chromosome` e tukuna ana mai i te whaea ki te tamaiti. Nā te mea he ira `X-linked` tēnei, ki te mea he kawe te whaea i tēnei ira hapa, 1 i roto i te 4 te tūponotanga o tana tamaiti tāne ki te whakawhanake i tēnei mate `AIS`. Ka taea hoki e ngā tamariki wahine te whiwhi i tēnei ira hapa, ā, ka noho hei kawe hoki rātou, engari kāore e whanaketia he `AIS`.

He tino onge te mate AIS. Tata ki te kotahi i roto i te 99,000 pēpi tāne ka pāngia e te PAIS. E rua ki te rima i roto i te 100,000 pēpi ka pāngia e te CAIS.

He aha tēnei e tupu nei? He aha te take?

E ai ki tā mātou i kōrero ai i mua ake nei, ko te take matua mō tēnei he āhuatanga rerekē o te ira `AR` i runga i te ira `X`. Ina kore e mahi tika tēnei ira, kāore e taea e te tinana te hanga i ngā kaiwhakawhiwhi androgen. Ko ēnei kaiwhakawhiwhi androgen, i te whakarapopototanga, ko ngā mea e āwhina ana i ngā pūtau o te tinana ki te urupare ki ngā homoni tāne e kiia nei ko ngā androgen (hei tauira , te testosterone) . Nō reira, ina ngaro ēnei kaiwhakawhiwhi, kāore te tinana e "mōhio" ki ngā homoni tāne, ko te tikanga ahakoa kei reira aua homoni, kāore e mahi i tā rātou e tika ana kia mahi.

He aha ngā tohu o tēnei?

He rerekē ngā tohu o te AIS i runga i te momo. Heoi, ko te āhuatanga matua e kitea ana i ngā momo katoa ko te kore tamariki .

Kāore e taea e te hunga whai `CAIS` te hapu, te whakahāwea rānei i tā rātou hoa. Ahakoa te āhua o ō rātou ira tangata ki ō te wahine, kāore he whekau whakaputa uri wahine o roto i a rātou. Kāore hoki te hunga whai `PAIS`, `MAIS` rānei e taea te whānau tamariki. Tērā pea he iti rawa te ure o ā rātou, ā, he iti rawa, he kore rānei te whakaputanga parāoa.

Ko ētahi atu tohu ka pā ki te hunga whai CAIS ko:

• Te roa haere i te kotiro noa i te wā o te pakeketanga.
• Te korenga o te paheketanga (Amenorrhea) .
• I te wā o te pakeketanga , he iti noa iho, he kore rānei he huruhuru i ngā kēkē me te wāhi ira tangata.
• Te whaiti, te papaku rānei o te tenetene .
• Kei roto tonu ngā rauropi i te kōhao puku (kāore i te heke iho).

Ko ētahi atu tohu ka pā ki te hunga whai PAIS ko:

• Ko te bifid scrotum he āhuatanga e wehea ai te rae kia rua .
• Te whakanui ake i te kiore (Clitoromegaly) .
• Te whakanui ake i te kiko o te ū i roto i ngā tama (Gynecomastia) .
• Ko te hypospadias te wāhi kei raro i te ure te pūwaha o te ure, ehara i te pito .
• Ngā piri o te waha .
• Te ure iti rawa (Micropenis) .
• Ngā rauropi kāore anō kia heke iho i te wāhanga .

Ko ētahi atu tohu ka pā ki te hunga whai `MAIS`:

• Te whakanui i ngā ū `(Gynecomastia)` .
• Ure iti (Micropenis).
• He iti noa ngā huruhuru o te tinana .

Me pēhea koe e mōhio ai ki tēnei?

Ka taea e te tākuta te kite i te PAIS i te whānautanga mā te titiro ki ngā ira tangata o te pēpi. Heoi, ki te mea he CAIS, he MAIS rānei tō te pēpi, kāore pea e kitea kia tae rā anō ki te 11, 12 rānei tau te pakeke o te tamaiti, arā, i te wā e pakeke haere ana te pēpi . Koinei te wā ka taea e te tākuta te kī mēnā he raruraru.

Hei tauira, ko te tamaiti whai CAIS, tērā pea kāore he paheketanga, kāore rānei he huruhuru tūtae. Ko te tamaiti whai MAIS, tērā pea he iti rawa te ure, he ū rānei. I te wā o te pakeketanga, ka taea e ngā rauropi kāore i heke iho te hernia mā roto i tētahi wāhi ngoikore o te pakitara puku. I ētahi wā, ka kitea e ngā tākuta he rauropi kāore i heke iho te tamaiti i te wā e pokangahia ana mō te hernia inguinal .

He aha ngā momo whakamātautau e mahia ana?

Hei whakaū i tēnei mate `AIS`, me mahi e te tākuta ētahi whakamātautau:

• Ngā whakamātautau toto: Ka tirohia e ēnei ngā taumata homoni , ngā ira tangata, me ngā hapa ira .
• Ngā whakamātautau whakaahua: Ka taea e ngā whakamātautau pēnei i te ultrasound te whakaū mēnā kua hangaia ngā whekau whakaputa uri wahine.

Mena kua pāngia tētahi o tō whānau e te AIS, ā, kei te whakaaro koe kia whānau tamariki, he mea pai kia whakamātauria he ira . Mā tēnei whakamātautau ka taea e koe te mōhio mēnā he kaikawe koe i tēnei ira rerekē.

He aha ngā maimoatanga mō tēnei?

Ko te maimoatanga mō te AIS ka whakatauhia e te ira tangata i tohua i te whānautanga. Ko te nuinga o ngā maimoatanga ka tīmata i muri i te pakeketanga o te tamaiti. Mā tēnei ka wātea te wā mō te tinana o te tamaiti ki te pāngia e ngā huringa whanaketanga, ā, ka taea ai e te tamaiti te whai wāhi nui atu ki ā rātou whakataunga maimoatanga.

Heoi, e whakapono ana ētahi tohunga hauora me mahi ētahi maimoatanga, pērā i te tango i ngā ure, i mua i te pakeketanga nā te mea kei te tūpono te whakawhanake i tētahi momo mate pukupuku e kiia nei ko te gonadoblastomas i roto i ngā ure kāore anō kia heke. E kī ana hoki rātou ka taea te mahi i ētahi atu maimoatanga i muri i te mutunga o te pakeketanga.

Ngā kōwhiringa maimoatanga mō ngā tamariki e whakatipuria ana hei tama:

• He pokanga hei whakatika i ngā ira tāne. Hei tauira, ko te 'whakatika hypospadias' (te whakarite anō i te whakatuwheratanga o te urethra) ko te 'orchiopexy' rānei (te whakarite anō i ngā rauropi kāore i heke ki roto i te scrotum).
• Te pokanga whakaiti ū hei tango i te kiko nui o te ū.
• Ko te pokanga whakatika hernia he tikanga hei kati i te kiko tuwhera, ngoikore rānei o te pakitara o te puku.
• Te rongoā homoni testosterone .

Ngā kōwhiringa maimoatanga e wātea ana mō ngā tamariki kua whāngaihia hei kōtiro:

• He pokanga hei tango i te ira tāne, i te kiko nui rānei o te kōpū.
• Ngā tikanga whakawhanui i te tara kore-pokanga hei whakahōhonu ake i te tara.
• Te rongoā homoni estrogen .

Ka taea te ārai i tēnei?

Kāore he huarahi hei ārai i te AIS. Heoi, ki te pāngia tētahi o tō whānau e te mate, ā, kei te āwangawanga koe kei riro i tāu tamaiti tēnei ira rerekē, ka taea te whakamātautau ira kia kitea ai mēnā he kaikawe koe.

Kei te pēhea te noho a te hunga me tēnei mate? (Te Tirohanga ā-Mārama)

Ka taea e te hunga whai AIS te ora ora, te ora hauora . He pai te urupare a te tini ki te rongoā homoni me te pokanga. Heoi, nā te mea he maha ngā wā ka kore tamariki te hunga e pāngia e te AIS, ka uaua pea te hinengaro o te tini tāngata. Ka pā nui hoki ki te hauora hinengaro o ngā tamariki me ngā taiohi.

Ki te kore koe e tangohia te gonad , ka piki ake tō tūponotanga ki te pāngia e te mate pukupuku testicular mā te 30%.

Mena kei taku tamaiti te AIS, me pēhea taku āwhina?

"He wāhanga nui o te whakahaere i te AIS te tiaki i te hauora hinengaro o te tamaiti."

Mena he AIS tō tāu tamaiti, he mea nui kia whai pūnaha tautoko kaha hei mārama ki ngā tākuta, ki ngā hoa, me te whānau. Mā te whakauru atu ki ngā rōpū tautoko ka taea hoki e tāu tamaiti te whakapuaki i ō rātou wheako me ngā wero ki ētahi atu e pāngia ana e te taua āhuatanga.

Ina tae tāu tamaiti ki te pakeketanga, ā, ka tīmata ia ki te kite kāore tōna tinana e rerekē ana pērā i te mea e tumanakohia ana, he mea tino nui kia kōrero koe ki a ia mō te AIS .

Me pēhea te whakahaere i te AIS hei pakeke?

He uaua te noho me te AIS hei pakeke. He uaua pea te whai i tētahi oranga moepuku noa, te kimi i tētahi hoa e mārama ana, e whakaae ana ki tō āhua, ā, ka pā kino te kore tamariki ki te hinengaro o te tini tāngata.

Mena kei a koe te AIS, he mea āwhina pea te kōrero ki tētahi kaiwhakatakoto whakaaro, ki tētahi kaiwhakaora hinengaro rānei mō ō wheako. Ka taea hoki e koe te hono atu ki ētahi atu kei a rātou te AIS i roto i ngā rōpū tautoko.

Hei whakamutunga, ngā mea hei maumahara

Ko te Androgen Insensitivity Syndrome (AIS) he āhuatanga e pā ana ki te tangata he tāne ira engari kāore e aro ki ngā homoni tāne. Nō reira, ka rite pea ō rātou ira ki ō te wahine, ka rite rānei ō rātou āhuatanga tāne, wahine hoki. Ka puta he maha ngā wero i tēnei āhuatanga. Nō reira, he mea tino nui kia kōrero tonu te hunga whai AIS ki ā rātou tākuta, kia whiwhi tautoko hinengaro kaha hoki. Mā te tohutohu hauora tika me te tautoko a te hunga e arohaina ana, ka taea hoki e ēnei tāngata te noho angitu.

` Te mate kore aro o te androgen, AIS, te whanaketanga ā-ira tangata, ngā homoni, ngā mate ira, ngā chromosomes, te kore tamariki, ngā ira tāne, te pakeketanga, te whakamātautau ira