Whakaarohia te mamae ohorere o te puku, kāore he take e kitea ana, me te wiriwiri, te kore rānei o ō waewae, te ngoikore o ngā uaua, me te whakapairuaki. Ahakoa kua kite koe i ētahi tākuta me te whai whakamātautau maha, kāore tonu i te mārama te pūtake o te mate. Kua pāngia koe, tētahi atu rānei e mōhio ana koe e tēnei? He mate ira onge pea tēnei e warewarehia ana. I tēnei rā, kei te tūhuratia e mātou tēnei mate: Acute Hepatic Porphyria , AHP rānei.
He aha rawa te Acute Hepatic Porphyria (AHP)?
Ko te AHP e pā ana ki tētahi whānau o ngā mate ira tuku iho onge e pā ana ki tō pūnaha io , me tō kiri i ētahi wā. He tino onge, e pā ana ki te rima i roto i te 100,000 tāngata. Ka taea e tēnei āhuatanga te whakaoho i ngā raruraru hauora ohorere, kino, ā, ka morearea pea ki te ora.
Kia mārama ai tātou ki te take, me titiro tātou ki ō toto. Kei roto i ō tātou toto whero he pūmua e kiia nei ko te Hemoglobin . Ko tana mahi matua he tango i te hāora mai i ō pukahukahu ka tuku ki ō whekau me ō kiko—pēnei i tētahi ratonga tuku hāora motuhake.
Hei hanga i tēnei pūmua, me whai wāhanga nui tō tinana e kiia nei ko te Heme .
Mō te hunga whai AHP, he hapa, he ngaro rānei tētahi whākōkī motuhake e hiahiatia ana mō te hanga Heme. Whakaarohia he rite ki te ngana ki te tunu i tētahi tunu kai engari kua ngaro tētahi o ngā kai matua; kāore e taea te hanga tika i te hua whakamutunga.
Ko te nuinga o te wā ka puta tēnei ngoikoretanga whākōkī i roto i te ate , e kiia ana hoki ko te pūnaha ate . Koinei tonu te take i tapaina ai te mate ko te Acute Hepatic Porphyria .
Ina kore e taea e tō tinana te whakaputa i te Heme, ka tīmata te kohikohi o ngā matū hangahanga - ina koa ko ngā matū paitini pēnei i te Porphobilinogen (PBG) me te waikawa Aminolevulinic (ALA) - ki roto i te ate. Ka uru ēnei paitini ki roto i te toto, ka taea te haere puta noa i te tinana, ka kino rawa atu tō pūnaha io. Ko tēnei kino o te io ka hua ake ngā tohu pēnei i te mamae nui , te ngoikore , me te whakapairuaki .
He aha ngā momo matua o te AHP?
E whā ngā momo matua o te AHP. Ahakoa he ngoikore o tētahi whākōkī rerekē i roto i te tukanga whakaputa Heme i roto i ia momo, he pānga nui ēnei momo e whā ki te ate me te pūnaha io.
Te Porphyria Wawe Koretake (AIP)
Koinei te momo tino noa, e pāngia ana e te 80% o ngā tūroro AHP. Nā te ngoikore o te whākōkī Hydroxymethylbilane synthase (HMBS) i puta mai i te whakarerekētanga ira i roto i te ira HMBS. Ka taea tēnei te tuku iho mai i tētahi mātua, ka taea rānei te puta hei huringa ira matapōkere, kāore he hītori whānau o mua.
He aha te āhua o te AHP?
He rerekē ngā tohu o te AHP. Tērā pea ētahi tāngata kāore e pāngia e ngā tohu, ko ētahi ia ka pāngia e ngā whakaekenga maha, kino hoki. He mea nui te mōhio ki ēnei tohu.
| Pūnaha Tinana e Pāngia ana | Ngā Tohu Noa |
|---|---|
| Puku | Mamae nui te puku (te tohu matua ), te whakapairuaki, te ruaki, me te kōroke. |
| Pūnaha Io | Te ngenge, te mākūkū rānei o ngā waewae, te ngoikore o ngā uaua, te mamae o ngā uaua, ā, i ētahi wā, ka pāngia e te pararutiki. |
| Hauora Hinengaro | Te kore okiokinga, te manukanuka, te pōhēhē, me ngā moemoeā. |
| Manawa me te Toto Toto | Te tere o te manawa (tachycardia) me te toto toto tiketike. |
| Ētahi atu | He mimi pōuri, whero, parauri rānei (ina koa i te wā e pā mai ana te whakaeke). |
Nā te mea he onge te AHP, he maha ngā wā ka pohehetia ōna tohu mō ngā mate noa ake, ka whakaroa pea i te tautuhi mate.
He aha te mea e whakaoho ana i te whakaeke?
He maha ngā tāngata whai AHP e ora noa ana, engari ka taea e ētahi mea whakaoho te whakaputa i ngā whakaeke ohorere. He mea nui te mōhio ki ēnei:
- Ngā Rongoā Motuhake: Ina koa ko ngā barbiturates me ngā paturopi sulfonamide. Mena kei a koe te AHP, me kōrero ki tō tākuta i mua i te tīmatanga o tētahi rongoā hou.
- Waipiro: He mea nui hei whakaoho i ngā whakaekenga AHP.
- Te Whakawhāititanga Kaora/Te Nohopuku: Ka taea e ngā kai tino iti i roto i ngā kaora me ngā warowaihā te whakaoho i ngā tohu.
- Ngā Huringa Homoni: Ina koa ko ngā mea e pā ana ki te huringa paheketanga i roto i ngā wāhine.
- Te ahotea me ngā mate urutā: Ka taea e te ahotea tinana, te kirika, ngā mate noa rānei pēnei i te rewharewha te whakaoho i tētahi wā.
Āhea koe me toro atu ki tētahi tākuta?
Ki te pāngia koe e ēnei e whai ake nei, kaua e whakarērea. Rapua he tohutohu hauora mēnā:
- He mamae puku nui tōu e hokihoki mai ana, kāore he whakamāramatanga.
- Ka pāngia koe e ngā tohu e pā ana ki ngā io pērā i te ngau, te ngoikore rānei o ngā peka.
- Kei a koe tētahi hītori whānau e mōhiotia ana mō te Porphyria.
- Ka kite koe kua huri tō mimi ki te whero pouri, ki te parauri rānei.
Ki te pāngia koe e tētahi āhuatanga ohorere, pērā i te mamae nui, te uaua ki te manawa, te pōhēhē ohorere, te kore rānei e taea te neke i ō ringaringa me ō waewae, rapua tonutia te āwhina i te tari ohorere tata ki a koe, waea atu rānei ki te 911.
He rongoā kei te wātea mō te AHP?
Ahakoa kāore he rongoā mō tēnei mate ira, he tino whai hua ngā maimoatanga e wātea ana hei whakahaere me te ārai i ngā whakaekenga.
Ko ngā wā tino kino me whakauru ki te hōhipera kia tau ai te pumau.
- Ko te huarahi matua ko te tautuhi me te tango i te pūtake.
- Ka whakamahia te rongoā mamae kaha hei whakahaere i te mamae nui.
- Ka tohua ngā rongoā hei whakahaere i te whakapairuaki me te ruaki.
- Ka taea e te werohanga huka mā roto i te uaua te āwhina i te pehi i te hanga o ngā matū paitini i roto i te ate.
- Maimoatanga Motuhake: Ka mahi ngā werohanga Hemin hei whakakapinga tika, hei tohu i te ate kia mutu te whakaputa nui rawa i ngā matū kino o te Heme.
- Hei tāpiritanga, mō te hunga e pāngia ana e ngā whakaekenga auau, kei te wātea inaianei ngā maimoatanga whakamōmona ira matatau.
Mā tō tākuta e whakatau te mahere maimoatanga e tino tika ana mō ō hiahia.
Karere Tangohia ki te Kāinga
- He mate ira onge te Acute Hepatic Porphyria (AHP), engari mā te whakahaere i tēnei mate, ka ora noa te tini o te tangata i ngā oranga noa, whai hua.
- Ko te tohu matua ko te mamae nui o te puku, e hokihoki mai ana, me ngā raruraru pūnaha io (ngoikore, ngoikore).
- He mea nui te karo i ngā mea whakaohooho—pērā i ētahi rongoā, te waipiro, me te nohopuku roa.
- Ki te whakaaro koe kei a koe ngā tohu, kei a koe rānei tētahi hītori whānau, me toro atu ki tō tākuta i te wā tonu.
- Kei reira ngā maimoatanga whai hua hei whakahaere i ngā tohu me te ārai i ngā whakaekenga a meake nei. Kāore he take e mataku ai; mahi tahi me tō kaiwhakarato hauora.
AHP, Porphyria Hepatic Koi, Porphyria, mamae puku, mate pūnaha io, mate ira, heme, hemoglobin, porphyrin, mate onge
