Mena kei a koe, kei tētahi rānei o tō whānau tētahi mate e kiia nei ko te Congenital Adrenal Hyperplasia (CAH), he mea nui tēnei māu. Whai muri i te 70 tau, kua whakaaetia he maimoatanga hou mō tēnei mate. He tino tumanako nui tēnei mō te hunga e noho tahi ana me te CAH. Nō reira, me titiro tātou ki tēnei rongoā hou, me pēhea te mahi, me ngā painga.
Tuatahi, kia mārama tātou he aha te CAH?
Ki te kī māmā noa, he mate ira te CAH e pā ana ki ō tātou repe adrenal. Kei runga ake i ō tātou whatukuhu ēnei repe. Ka whakaputahia e rātou ētahi homoni he mea nui mō tō tātou tinana.
Whakaarohia te repe adrenal hei wheketere. Ko te hua matua o tēnei wheketere he homoni e kiia nei ko te cortisol. He homoni tino nui tēnei e āwhina ana i tō tātou tinana ki te aro atu ki te ahotea, te mate, me te tūponotanga.
Ina heke te taumata o te cortisol i roto i te tangata hauora, ka tukuna e te roro (pērā i te kaiwhakahaere wheketere) he tohu (whakahau) e kīia nei ko te ACTH ki te repe adrenal hei "hanga cortisol." Engari i roto i te tangata whai CAH, kāore i te repe adrenal he whākōkī matua, he 'kaimahi' rānei, e hiahiatia ana hei hanga cortisol. Nō reira, ahakoa te nui o te ACTH e tukuna ana e te roro, kāore e taea e te wheketere te hanga cortisol.
E rua ngā pātai ka ara ake i tēnei:
1. Kāore te tinana e whakaputa i te cortisol e tika ana .
2. I te haere tonu o ngā tohu ACTH mai i te roro, ka nui rawa te whakaputanga o tētahi atu momo homoni e hangaia ana e te repe adrenal , e kiia nei ko ngā androgens .
He homoni tāne ēnei androgens (hei tauira, te testosterone). E hiahiatia ana ētahi o ēnei e ngā tāne me ngā wāhine, engari i roto i te CAH, ka nui rawa. Ka taea e tēnei te whakatōtō wawe i te pakeketanga, te kore tamariki, me ētahi atu raruraru ki te whanaketanga o te tamaiti .
Mō ngā tekau tau, ko te rongoā matua mō tēnei ko te hoatu i ngā steroids (glucocorticoids) e mahi ana pērā i te cortisol ki waho. Ehara i te mea ka whakakapi noa i te cortisol kāore i roto i te tinana, engari ka whakahaere hoki i te tohu ACTH mai i te roro, ka whakaiti i te hanga o ngā androgens. Heoi, ko te raruraru me tango i ngā horopeta steroids teitei ake i te tikanga mō te wā roa. Ka taea e tēnei te whakaputa i ngā pānga taha maha, pērā i te taumaha, te angiangi o ngā wheua, me ngā raruraru moe .
I pēhea koe i kitea ai tēnei rongoā hou?
I whakaaro ngā kaipūtaiao mēnā he otinga anō mō tēnei raruraru steroid. Engari i te hoatu i te maha o ngā steroid ki waho, me pēhea mēnā ka kitea he huarahi hei whakaiti tika i te tohu ACTH e puta mai ana i te roro?
Koinei te wāhi i kitea ai e rātou tētahi ūnga hou. He kaiwhakawhiwhi i roto i te roro e kiia nei ko te CRF1. Ina heke ngā taumata cortisol, ka whakaohohia te tohu ACTH e te CRF1. I roto i te CAH, ka whakararuhia tēnei tukanga.
Nō reira i whakaaro ngā kaipūtaiao, mēnā ka āraia tēnei CRF1, ka taea e tātou te whakaiti i te hanga o te ACTH, tika? Mēnā ka tupu tēnā, ka whakahaeretia hoki te hanga o ngā androgens, ā, ka whakaitihia hoki te horopeta o ngā steroids e tika ana kia tangohia e te tūroro.
Nā tēnei whakaaro hou i hua ake ai te whanaketanga o tētahi rongoā hou e kiia nei ko Crinecerfont. He rongoā tēnei i hangaia hei whakatika i tētahi raruraru nui o te CAH mā te aukati i te CRF1.
Ngā whakamātautau i mahia i mua i te hoatutanga o te rongoā ki te tangata
Kāore e taea te hoatu i tētahi rongoā hou ki te tangata i te wā tonu ka kitea. Me whai i ētahi whakamātautau hei whakaū i tōna haumaru, i tōna whai huatanga hoki. I whai te rongoā Crinecerfont i ēnei mahi katoa.
1. Whakamātautau taiwhanga me te kararehe: Ko te taahiraa tuatahi ko te whakamātautau i te rongoā ki ngā pūtau me ngā kararehe i roto i te taiwhanga. Hei whakatau mēnā he paitini te rongoā, mēnā rānei ka kino ki ngā whekau. Whakaarohia, kotahi noa iho i roto i te 10,000 ngā rongoā hou ka puta i ēnei arai katoa, ā, ka whakaaetia.
2. Ngā Whakamātautau Haumanu: Kia angitu te whakamātautau kararehe, ka whakahaerehia ngā whakamātautau tangata i roto i ētahi wāhanga.
Kei te pēhea ngā hua o te whakamātautau mō Crinecerfont?
He tino pai ngā hua o ngā whakamātautau i mahia ki tēnei rongoā. He mea tino nui ngā whakamātautau Wāhanga II me te Wāhanga III.
Wāhanga II - Ngā hua mō ngā tamariki nohinohi
I konei, i hoatu te rongoā ki tētahi rōpū iti o ngā tamariki nohinohi (14-17 tau te pakeke) he CAH tō rātou, mō ngā rā 14, ā, i kitea ngā panonitanga o ō rātou taumata homoni. He mea miharo ngā hua.
- Kua heke te ACTH (tohu roro) mā te 57% .
- Kua heke te 17-OHP (he tohu mō te hanga androgen) mā te 69% .
- I heke te Androstenedione (he momo androgen) mā te 58% .
Wāhanga III - Ngā hua o ngā rōpū nui
I te whakatenatenahia e ēnei hua, i whakahaerehia e ngā kaipūtaiao ētahi whakamātautau nui e rua mā te whakamahi i ngā pakeke me ngā tamariki, i hoatu ai te rongoā Crinecerfont ki ētahi, ā, i hoatu he placebo ki ētahi atu, he rite ki te rongoā engari kāore he pānga.
| Tīma | Hua matua | Whakaahuatanga |
|---|---|---|
| Ngā Pakeke (e tango ana i te Crinecerfont) | Te whakaiti i te horopeta o te steroid | Mā te whakahaere i ngā taumata homoni, i taea ai te whakaiti i te horopeta o ia rā o ngā steroids mā te 27% . |
| Ngā pakeke (i tango i te rongoā placebo) | Te whakaiti i te horopeta o te steroid | I whakaitihia te horopeta o te steroid mā te 10% anake. |
| Tamariki (e tango ana i te Crinecerfont) | Ngā taumata androgen me te horopeta steroid | I muri i ngā wiki e 4, i tino heke te nui o ngā androgen. I te mutunga o te whakamātautau, i whakaitihia te horopeta steroid mā te 18% . |
| Tamariki (i tango i te rongoā placebo) | Ngā taumata androgen me te horopeta steroid | I piki ake ngā taumata o te androgen. I whakapiki ake te horopeta o te steroid mā te 6%. |
E whakaatu mārama ana ēnei hua ka āwhina a Crinecerfont ki te whakahaere i ngā taumata androgen, me te whakaiti anō hoki i te horopeta o ngā steroids ka pā kino ki ngā tūroro i te wā roa.
He pēhea te haumaru o tēnei rongoā?
Kua whakaatuhia e ngā rangahau he iti noa ngā pānga kino kino o tēnei rongoā. Ko te nuinga o ngā pānga kino kua pūrongohia he iti noa iho, he rangitahi noa iho.
- Ngenge
- Ānini
- Ihu makariri, ihu pūngāwerewere rānei
- Mamae uaua, mamae puku rānei
- Mamae hononga
Ko te whakatūpato nui anake e pā ana ki tēnei rongoā , ki te kore koe e tango i te horopeta tika o ngā steroids e tohua ana e tō tākuta , ka taea e ō taumata cortisol te heke iho i te tino iti, ka arahi ki tētahi mate morearea e kiia nei ko te adrenal insufficiency. Heoi, kāore anō kia pāngia tētahi i tango tika i te rongoā i roto i ngā rangahau e tēnei mate . Nō reira, he mea tino nui kia whai i ngā tohutohu a tō tākuta.
He tumanako hou mō te maimoatanga CAH
Nā ēnei hua angitu, kua whakaaetia motuhaketia tēnei rongoā e te FDA (te tari matua e whakaae ana ki ngā rongoā) i te Hononga o Amerika. Ehara a Crinecerfont (ingoa waitohu Crenessity) i te rongoā mō te CAH. Heoi, he taahiraa nui whakamua tēnei i roto i te maimoatanga o te CAH.
Ka taea e ngā tākuta te tohu i tēnei rongoā hei tāpiri atu ki tō maimoatanga steroid o nāianei (whakaora tāpiri) . Ka āwhina tēnei ki te whakaiti i tō horopeta steroid. Ko te tikanga o tēnei ka iti ake pea ngā pānga taha o ngā steroid, ka pai ake te whakahaere i ō taumata homoni, ā, ka pai ake te kounga o te oranga.
Karere Tangohia ki te Kāinga
- Ko te CAH (Congenital Adrenal Hyperplasia) he mate ira e pā ana ki ngā repe adrenal, ā, ka puta te kore taurite o ngā homoni.
- Ko te Crinecerfont (Crenessity) he rongoā hou kua whakaaetia mō te CAH, e hoatu ana hei tāpiritanga ki te maimoatanga steroids.
- Ka whakaitihia e tēnei ngā taumata tiketike o te homoni androgen i roto i te tinana, ā, ka āwhina hoki ki te whakaiti i te horopeta o ngā steroids o ia rā.
- Mā te whakaiti i te horopeta o ngā steroids, ka taea te whakaiti i ngā pānga taha e pā ana ki te whakamahinga roa o ngā steroids.
- Ahakoa ehara tēnei i te rongoā mō te mate, he taahiraa nui tēnei ki te whakapai ake i te kounga o te oranga mō ngā tūroro CAH.
- Mena kei a koe, kei tāu tamaiti rānei te CAH, he mea nui kia kōrero koe ki tō tākuta endocrinologist, ki te tākuta whānau rānei, kia kite ai mēnā he tika tēnei maimoatanga hou māu .
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