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.
Renal function (eGFR)
Measure kidney function based on creatinine value
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).
Medical test equipment that can be used at home
Essential home medical devices including blood pressure monitors, oximeters and digital thermometers.
Check Availability →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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