Kei roto i tō whānau, inā koa he tamaiti, he mate homoni whānau? Tērā pea kua mōhio koe ki ngā raruraru pēnei i te whakaroa o te tipu, te pakeketanga wawe, me ngā huringa paheketanga koretake i roto i ngā wāhine. Ko tētahi āhuatanga ira onge ka puta ai ēnei ko te 'Congenital Adrenal Hyperplasia'. Ka kiia e mātou ko CAH. I tēnei rā, ka kōrero poto mātou mō tēnei me tētahi maimoatanga hou kua puta mai mōna.
He aha te CAH?
He mate ira te CAH e ārai ana i ngā repe adrenal, arā, ngā repe iti e rua kei runga ake i ngā whatukuhu, kei whakaputa i te cortisol, he homoni he mea nui mō ō tātou oranga.
Whakaarohia ō tātou tinana he wheketere. Ina iti te nui o tētahi o ngā hua (cortisol) i roto i tēnei wheketere, ka raru te pokapū whakahaere i roto i te roro (te repe pituitary) ka tukuna ngā tohu (ACTH) ki ngā repe adrenal kia kaha ake te mahi. Nā tēnei whakaohooho nui rawa, ahakoa kāore e piki ake te hanga cortisol, ka tīmata te whakaputa i ngā homoni tāne e kiia nei ko ngā 'androgens' .
Ka taea e tēnei koretake o te taurite o ngā homoni te whakararu i te maha o ngā raruraru:
- Ngā pēpi wahine: I te whānautanga, ka rite te āhua o ngā ira tangata o waho ki ō te pēpi tāne.
- Mō ngā taha e rua: ka pā mai pea ngā raruraru pēnei i te whakaroa o te tipu, te pakeketanga wawe, me te kore tamariki i muri mai i te ao.
Hei tāpiritanga, tera pea ka iti ake te hanga o tētahi atu homoni nui e kiia nei ko te aldosterone i ētahi o ngā tūroro CAH. Ki te kore tēnei homoni, ka tino kino te CAH, ka arahi atu ki te ohorere, te koma, tae noa ki te mate.
Te maimoatanga 70-tau te pakeke me te tumanako hou
Mō ngā tekau tau, ko te rongoā anake mō te CAH ko te whakakapi i ngā taumata iti o te homoni cortisol i roto i te tinana ki ngā horopeta nui o ngā steroids , pērā i te hydrocortisone. Heoi, mā te tango i ēnei horopeta nui o ngā steroids mō te wā roa ka pā mai he maha ngā pānga taha. Ko ngā mea tino kitea ko te taumaha, te toto toto tiketike, te ngoikore o ngā wheua, me te tipu puhoi o ngā tamariki.
Engari he rongo pai tēnei! I muri i te 70 tau, kua whakaaetia e te FDA te rongoā tuatahi, ko te rongoā anake hoki i hangaia mō te CAH, ko te crinecerfont (ingoa waitohu: Crenessity).
He pēhea te mahi a tēnei rongoā hou (Crinecerfont)?
Koinei te mea tino nui. He rerekē te mahi a tēnei rongoā hou, he rerekē i te maimoatanga tawhito.
Kāore a 'Crinesurfont' e tāpiri i ngā homoni mai i waho. Engari, ka haere tika atu tēnei rongoā ki te pokapū whakahaere o te roro (te repe pituitary) ka aukati i te tukunga o te tohu kāore e hiahiatia (ACTH) e whakaoho ana i ngā repe adrenal.
He rite tēnei ki te whakaweto i te 'pana' o tētahi pūnaha kāore e hiahiatia. Mā tēnei ka whakahaere te tinana i te whakaputanga o ngā homoni 'androgen' kāore e hiahiatia.
Ko te painga nui o tēnei, nā te mea kua whakahaeretia ngā taumata o te androgen, ka taea te whakaiti i te horopeta o ngā steroids e hiahiatia ana hei pupuri i te taurite o ngā homoni. Ko te tikanga o tēnei ka taea hoki te whakaiti i ngā pānga taha e puta mai ana i ngā steroids.
| Āhuatanga | Maimoatanga tawhito (ngā horopeta steroid teitei) | Maimoatanga hou (Crinecerfont + horopeta iti o te steroid) |
|---|---|---|
| Mahi | Te whakarato i te homoni cortisol kua whakaitihia ki waho. | Te aukati i ngā tohu kāore e hiahiatia (ACTH) mai i te roro me te whakahaere i te hanga androgen. |
| Whāinga matua | E hiahiatia ana ngā horopeta teitei hei whakahaere i ngā taumata homoni. | Me whakaiti i ngā taumata o te androgen me te whakaiti i te horopeta o ngā steroids. |
| Ngā pānga taha | Ngā raruraru roa pēnei i te taumaha nui, te koretake o te tipu, me te ngoikore o ngā wheua. | Te kaha ki te whakaiti i ngā pānga taha e pā ana ki te steroid. Kua pūrongohia ngā pānga taha iti (mamae upoko, ngenge). |
Mā wai tēnei rongoā e tino pai?
Kua whakaaetia te 'Crinesurfont' mō ngā tūroro CAH neke atu i te 4 tau te pakeke . Ehara i te mea hei whakakapi mō ngā steroids, engari he tāpiritanga ki ngā steroids.He maimoatanga tāpiri.
- Mō ngā tamariki: I te mea kei te tipu tonu ngā tamariki, ka pā nui te nui o ngā steroids ki tō rātou tipu. Nō reira, he tino whai hua tēnei rongoā, ka taea te whakaiti i te horopeta o ngā steroids, mō rātou. He mea nui rawa tēnei i te wā o te taiohitanga, ina uaua ki te whakahaere i ngā homoni.
- Mō ngā pakeke: Ka taea hoki e tēnei maimoatanga te whakaora i ngā pakeke e pāngia ana e ngā pānga taha o te tango steroids mō te wā roa, e uaua ana rānei ki te pupuri i ngā taumata homoni pumau.
Akohia ngā pānga taha me te haumaru
I roto i ngā whakamātautau haumanu, he torutoru noa iho ngā pānga taha iti, rangitahi kua pūrongohia mō tēnei rongoā.
- Mō ngā pakeke: ngenge, ānini, whanoke, mamae hononga.
- Mō ngā tamariki: te ānini, te mamae o te puku, te ngenge, te ihu pupuhi.
He mea nui, he ngawari te nuinga o ēnei tohu, ā, he iti noa iho te tokomaha o ngā tūroro i mutu te tango i te rongoā.
Whakatūpato Motuhake: Kia tūpato ki te raruraru o te adrenal!
I te wā e tango ana i tēnei rongoā, he mea tino nui kia mōhio koe ki tētahi mate kino e kiia nei ko te 'raruraru adrenal'. He māmā noa iho, ka puta tēnei mate mēnā kāore te tinana e whiwhi i te nui o te 'cortisol' e tika ana i te wā e tino taumaha ana (hei tauira, he mate kino, he pokanga, he aituā).
Nō reira, kōrero ki tō tākuta, kia tino mōhio hoki koe ki te horopeta steroid tāpiri me tango e koe i roto i te āhuatanga ohorere ("horopeta ahotea") . Kia tūpato hoki ki ngā tohu o te 'raruraru adrenal' (hei tauira, te ruaki nui, te korere, te whanoke, te ngaro o te māramatanga) ā, kia mahara ki te haere tonu ki te hōhipera, ki te rūma ohorere rānei (ETU) mēnā ka puta tēnei āhuatanga.
Me pēhea taku mōhio mēnā he tika tēnei rongoā mōku/mō taku tamaiti?
Me whakatau tēnei me te kōrero tahi me tō tākuta, inā koa he tākuta endocrinologist . Ka whakaarohia e tō tākuta ngā āhuatanga maha, tae atu ki ngā hua o ō whakamātautau toto, te tere tipu, te taumaha, te toto toto tiketike, me ngā taumata homoni o nāianei, hei whakatau mēnā he tika tēnei rongoā māu.
Ehara tēnei i te rongoā mō te CAH, engari he nui te tūmanako ka āwhina i te whakapai ake i te kounga o te oranga mō ngā tūroro, ka whakaiti i ngā pānga taha o te steroids, ā, ka māmā ake te whakahaere i ngā homoni.
Karere Tangohia ki te Kāinga
- Ko te tikanga o te CAHHe āhuatanga ira e iti ake ai te cortisol e whakaputaina ana e ngā repe adrenal, engari he nui ake ngā homoni androgen e whakaputaina ana.
- He maimoatanga hou a Crinecerfont i hangaia mō te CAH, ko te tuatahi tēnei o tōna momo i roto i te 70 tau.
- Ehara tēnei i te rongoā hei whakakapi i ngā steroids. He rongoā tēnei e hoatu ana hei tāpiri atu ki ngā steroids, ā, ka āwhina i te whakaiti i te horopeta o ngā steroids e hiahiatia ana.
- Ko te painga matua ko te kaha ki te whakaiti i ngā pānga taha roa o ngā steroids.
- Me pātai atu ki tō tākuta mō te āhuatanga ohorere e kiia nei ko te 'raruraru adrenal' me te "horopeta ahotea" me tango e koe i taua wā.
- He mea nui kia kōrero koe ki tētahi tākuta e tohunga ana ki ngā mate homoni kia mōhio ai koe mēnā he tika tēnei maimoatanga mōu, mō tāu tamaiti rānei.
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