He aha te waikawa Carglumic: Ngā whakamahinga, te horopeta, ngā pānga taha me ētahi atu

Ko te waikawa Carglumic he rongoā motuhake e āwhina ana i tō tinana ki te tukatuka i te ammonia, he hua para paitini pea e hanga ana i te wā e pakaru ana ngā pūmua. Ko tēnei rongoā i runga i te whakahaunga i hangaia mō ngā tāngata e whai ana i ngā āhuatanga ira onge e aukati ana i ō rātou tinana ki te whakakore i te ammonia i te taiao.

Whakaarohia te waikawa carglumic hei āwhina e uru mai ana i te wā e kore e pai te mahi a te pūnaha whakawātea ammonia o tō tinana. Ki te kore tēnei rongoā, ka piki ake ngā taumata ammonia ki ngā taumata mōrearea me te pā ki te mahi roro, e hāngai ana tēnei maimoatanga ki ngā tāngata tūroro.

He aha te waikawa Carglumic e whakamahia ana mō?

Ka rongoā te waikawa Carglumic i te hyperammonemia, he āhuatanga e hanga ana te ammonia i roto i tō toto ki ngā taumata mōrearea. Ka whakahaungia tēnei rongoā mō ngā tāngata e whai ana i ngā mate ira motuhake e pā ana ki te tukatuka o ō rātou tinana i te hauota mai i ngā pūmua.

Ko ngā āhuatanga matua e hiahia ana ki te waikawa carglumic ko te ngoikoretanga o te N-acetylglutamate synthase me ētahi momo o te waikawa organic. He mate ira onge ēnei e kore ai e pai te mahi, e ngaro katoa rānei ngā enzyme e hiahiatia ana hei wawahi i te ammonia.

Ka whakahaungia pea e tō tākuta tēnei rongoā mō ētahi atu āhuatanga metabolic onge e pā ana ki te hanga ammonia. Ko te whāinga tonu ko te aukati i te kino o te roro me ētahi atu raruraru nui ka puta i te wā e noho tiketike rawa ana ngā taumata ammonia mō te wā roa.

Me pēhea te mahi a te waikawa Carglumic?

Ka mahi te waikawa Carglumic mā te whakakapi i tētahi pūhui ngaro e kīia ana ko te N-acetylglutamate i tō ate. He mea nui tēnei pūhui mō te whakahohe i tētahi enzyme e āwhina ana ki te huri i te ammonia paitini ki ngā matū iti ake te kino e taea ai e tō tinana te whakakore.

Inā ka tango koe i te waikawa carglumic, ka tīmata tō pūnaha tukatuka ammonia o te ate. Ka āwhina te rongoā i tō ate ki te whakaputa i ngā whākōrere e hiahiatia ana hei huri i te ammonia ki ngā pūhui pēnei i te glutamine, ā, i te mutunga ki te urea, ka taea e ō tākihi te tango marie mā te mimi.

E kiia ana he kaha te rongoā nei, he mea motuhake rawa. Ehara i te rongoā whānui engari he rongoā whāinga mō ngā mate pūkoro motuhake. Ko te tikanga ka tīmata ngā pānga i roto i ngā hāora i muri i te tango i te rongoā, ahakoa ka roa pea te rā ki te whakatutuki i ngā hua pai.

Me pēhea taku tango i te waikawa Carglumic?

Tangohia te waikawa carglumic kia rite ki te tohutohu a tō tākuta, ko te tikanga ka wehea ki ngā horopeta maha i te roanga o te rā. Me tango ngā papa ki te wai, ā, he mea nui kia mau tonu te wā i waenga i ngā horopeta kia mau tonu ngā taumata ammonia.

Ka taea e koe te tango i tēnei rongoā me te kore kai rānei, ahakoa ka āwhina pea te tango i te kai ki te whakaiti i te puku puku ki te pā koe. Ki te raru koe ki te horo i ngā papa, ka taea e tō kaipūtaiao rongoā te whakaatu ki a koe me pēhea te whakarite i te whakatārewa wai mā te whakaranu i ngā papa kuru ki te wai.

Kaua rawa e kuru, e ngaungau rānei i ngā papa ki te kore e tohutohutia e tō kaiwhakarato hauora. Ko ētahi tūroro me whakarite ā rātou papa hei whakatārewa wai, inā koa ngā tamariki, ērā rānei e raru ana ki te horo.

Ka hiahia pea tō tākuta ki te aroturuki i ō taumata ammonia i ngā wā katoa mā ngā whakamātautau toto kia mōhio ai kei te pai te mahi o te rongoā. Ka āwhina ēnei whakamātautau ki te whakatau mēnā me whakatika tō horopeta.

Kia pehea te roa e tango ai au i te waikawa Carglumic?

Ko te nuinga o te hunga e mate ana i ngā āhuatanga ira e hiahia ana ki te waikawa carglumic me tango tēnei rongoā mō te ora. I te mea he mate tūturu ēnei, kāore te raruraru i raro i te tukatuka ammonia e ngaro, e tika ana te rongoā wā roa.

Ka aroturukihia e tō tākuta tō urupare ki te rongoā, ā, ka whakatika pea i tō horopeta i te roanga o te wā i runga i ō taumata ammonia me te hauora whānui. Ko ētahi tāngata e hiahia ana ki ngā horopeta teitei ake i ngā wā o te mate, te pēhanga rānei, ka nui ake te ammonia e whakaputaina e ō rātou tinana.

Kaua rawa e mutu te tango i te waikawa carglumic ohorere, me te kore e kōrero tuatahi ki tō tākuta. Ko te whakamutu ohorere i tēnei rongoā ka arahi ki te hanga ammonia mōrearea, me ngā raruraru e mōrearea ana ki te ora.

He aha ngā pānga taha o te waikawa Carglumic?

He pai te whakamanawanui a te nuinga o te tāngata ki te waikawa carglumic, engari pērā i ngā rongoā katoa, ka pānga taha. Ko te rongo pai, he mea noa ngā pānga taha nui ina whakamahia te rongoā i runga i te tohutohu.

Anei ngā pānga taha tino noa ka pā ki a koe:

• Te pōhara o te puku, te whakapairuaki rānei
• Te korere, te tūtae rānei
• Te ānini
• Te ngenge, te ngenge rānei
• Te ngaro o te hiahia
• Te mamae o te puku

He ngawari noa iho ēnei pānga taha, ā, ka pai ake i te wā e urutau ana tō tinana ki te rongoā. Ko te tango i te rongoā me te kai ka āwhina i te whakaiti i ngā pānga taha e pā ana ki te puku.

Ko ngā pānga taha iti noa iho engari he nui ake te kino, ka uru pea ki ngā tauhohenga mate pāwera nui, te ruaki tonu, ngā tohu rānei o ngā raruraru ate. Whakapā atu ki tō tākuta tonu mēnā ka pā ki a koe te mamae nui o te puku, te kōwhai o te kiri, o ngā kanohi rānei, te uaua rānei ki te hā.

Ko ētahi pānga taha onge e hiahia ana ki te āwhina hauora tonu, ko te maroke nui mai i te ruaki tonu, te korere rānei, me ngā tohu o te taurite pūkoro, pērā i te rangirua, te ngoikore rānei.

Ko wai kāore e tika kia tango i te waikawa Carglumic?

He iti rawa te tāngata kāore e taea te tango i te waikawa carglumic, i te mea i hangaia motuhake mō ngā āhuatanga ira onge, kei te nuinga o te wā ka nui ake ngā painga i ngā tūponotanga. Heoi, ka āta arotakehia e tō tākuta tō āhuatanga takitahi i mua i te whakahau i tēnei rongoā.

Kāore e tika ana kia tango te rongoā nei e te hunga e mōhiotia ana he pānga mate ki te waikawa carglumic, ki ētahi atu rānei o ōna whakauru. Ka whakaaro anō tō tākuta ki te mahi o ō pūkahukahu me te ate, nā te mea he mahi nui ō ēnei okana ki te āhua o te mahi a te rongoā.

Ka taea e ngā wāhine hapū me te whāngai ū te tango i te waikawa carglumic ki te whai rātou i ngā āhuatanga ira e hiahiatia ana. Ko ngā tūponotanga o te hyperammonemia kāore i rongoatia te nuinga o te wā ka nui ake i ngā tūponotanga pea mai i te rongoā i te wā o te hapū.

Ka whakaaro anō tō tākuta ki ētahi atu rongoā e tango ana koe kia mōhio ai kāore he pānga kino. Me kōrero ki tō kaiwhakarato hauora mō ngā rongoā katoa, ngā tāpiringa, me ngā hua otaota e whakamahia ana e koe.

Ngā Ingoa Waitohu o te Waikawa Carglumic

E wātea ana te waikawa carglumic i raro i te ingoa waitohu Carbaglu i te United States me ētahi atu whenua. Koinei te āhua o te rongoā e tino tohua ana.

I ētahi rohe, ka kitea pea e koe te waikawa carglumic i raro i ētahi atu ingoa waitohu, engari ko te whakauru kaha tonu te mea kotahi. Ka taea e tō kai rongoā te āwhina i a koe ki te tautuhi i te waitohu motuhake e wātea ana i tō rohe.

Ka wātea pea ngā putanga generic o te waikawa carglumic i ētahi whenua, ahakoa he rongoā motuhake tonu tēnei, kāore i te whānui te hanga e te maha o ngā kaihanga.

Ngā Kōwhiringa kē mō te Waikawa Carglumic

He iti noa ngā kōwhiringa kē tika mō te waikawa carglumic mō te rongoā i ngā āhuatanga ira motuhake i hangaia ai hei whakatika. Mō te ngoikoretanga o te N-acetylglutamate synthase, ko te waikawa carglumic te nuinga o te wā te kōwhiringa rongoā tuatahi.

Ka whai hua pea ētahi tūroro mai i ngā whakarerekētanga kai, pērā i te whakawhāiti i te pūmua, i te taha o te rongoā waikawa carglumic. Ka tūtohu pea tō tākuta i ngā tāpiringa pērā i te arginine, ētahi atu waikawa amino rānei hei tautoko i ngā kaha o tō tinana ki te tukatuka i te ammonia.

I ngā tūāturutanga ohorere me te hyperammonemia kaha, ko ngā rongoā pērā i te dialysis, ētahi atu tikanga tango ammonia rānei, tērā pea e tika ana. Heoi anō, he tikanga rangitahi ēnei, ehara i te rongoā wā-roa hei whakakapi i te waikawa carglumic.

Ka mahi tō rōpū hauora ki a koe ki te whakatau i te huarahi rongoā pai rawa atu i runga i tō āhuatanga ira motuhake me ō hiahia takitahi.

He Pai Anei te Wawaikawa Carglumic i te Phenylbutyrate konutai?

He rerekē te mahi a te waikawa carglumic me te phenylbutyrate konutai ki te whakatika i te hyperammonemia, ā, ko te whiriwhiri i waenga i a rāua e whakawhirinaki ana ki tō āhuatanga ira motuhake. Ehara rāua i te kaiwhakataetae tika engari he rongoā whakakapi mō ngā take rerekē e takoto ana.

I hangaia te waikawa carglumic mō te ngoikoretanga o te N-acetylglutamate synthase, ā, ka mahi mā te whakakapi i te pūhui ngaro e hiahiatia ana hei whakahohe i ngā enzyme tukatuka ammonia. Ka mahi te phenylbutyrate konutai mā te whakarato i tētahi huarahi kē mō te whakakorenga hauota.

Tērā pea ka hiahia ētahi tūroro i ngā rongoā e rua, i te wā e pai ake ana ētahi ki tētahi, ki tērā rānei. Ka whakatau tō tākuta i te huarahi pai rawa atu i runga i ngā hua o tō whakamātautau ira me te pēhea te urupare a tō tinana ki te rongoā.

Kua whai hua ngā rongoā e rua i roto i ā rāua tono motuhake, ā, kāore tētahi i te "pai ake" i tērā atu. Ko te mea nui ko te kimi i te rongoā tika mō tō āhuatanga ira takitahi.

Ngā Pātai Auau mō te Wawaikawa Carglumic

He Haumaru Anei te Wawaikawa Carglumic mō ngā Tamariki?

Āe, he haumaru te waikawa carglumic mō ngā tamariki, ā, he maha ngā wā ka tohua mō ngā pēpi me ngā tamariki nohinohi me ngā āhuatanga ira e pā ana ki te hyperammonemia. Kua akona te rongoā i roto i ngā tūroro tamariki, ā, e kiia ana he rongoā nui mō ēnei āhuatanga onge.

Ko te tikanga ka whiwhi nga tamariki i te horopeta e pa ana ki te taumaha, ā, ka tino mātakitaki tō tākuta tamariki i te tipu me te whanaketanga o tō tamaiti i te wā e tango ana i tēnei rongoā. Ka taea te whakarite i ngā papa hei whakatārewatanga wai mō ngā tamariki e kore e taea te horo i ngā pire.

Me pēhea taku mahi mēnā ka whakamahi au i te nui rawa o te waikawa carglumic?

Mēnā ka nui ake te waikawa carglumic i a koe i te mea i whakaritea, whakapā atu ki tō tākuta, ki te pokapū whakahaere paitini rānei i te wā tonu. Ahakoa kāore i te pai te tuhinga o ngā tohumate o te horopeta nui nā te āhua motuhake o te rongoā, he mea nui kia rapua te tohutohu hauora i te wā tonu.

Ka pāngia pea e koe te pikinga o ngā pānga taha pēnei i te pōpō nui o te puku, te korere, te ānini rānei. Ka hiahia pea tō tākuta ki te mātakitaki i ō taumata ammonia me te āhua whānui mēnā ka puta he horopeta nui.

Me pēhea taku mahi mēnā ka ngaro i ahau tētahi horopeta o te waikawa carglumic?

Tangohia te horopeta i ngaro i te wā tonu ka mahara koe, ki te kore e tata ana te wā mō tō horopeta e whai ake nei. I taua wā, pekehia te horopeta i ngaro, ka haere tonu ki tō hōtaka horopeta auau.

Kaua rawa e tango i te horopeta rua hei utu mō tētahi i ngaro, nā te mea ka puta pea ngā pānga taha. Mēnā ka wareware koe i ngā horopeta i ngā wā katoa, whakaarohia te whakatakoto i ngā whakamaharatanga waea, te whakamahi rānei i tētahi kaiwhakarite pire hei āwhina i te pupuri i te wā tika o te rongoā.

Āhea e taea ai e au te whakamutu i te tango i te waikawa carglumic?

Kaua rawa koe e whakamutu i te tango i te waikawa carglumic me te kore e kōrero tuatahi ki tō tākuta. Mō te nuinga o te tangata e pāngia ana e ngā āhuatanga ira e hiahia ana ki tēnei rongoā, he roa te rongoā nā te mea kāore te raruraru pūkoro e whakatika.

Ka arotake auau tō tākuta i tō rongoā, ā, ka whakatika pea i tō horopeta i runga i ō taumata ammonia me te hauora whānui. Mēnā he āwangawanga ōna mō te whakamahi wā roa, kōrerohia ōna ki tō kaiwhakarato hauora.

Ka taea e au te tango i te waikawa carglumic me ētahi atu rongoā?

He iti noa iho ngā pānga o te waikawa carglumic ki ētahi atu rongoā, engari he mea nui kia kōrero koe ki tō tākuta mō ngā rongoā katoa e inu ana koe. Ka pā pea ētahi rongoā ki te pai o te mahi a te waikawa carglumic, ka whakanui rānei i te tūponotanga o ngā pānga taha.

Ka arotake tō tākuta i ō rongoā katoa kia mōhio ai kei te mahi ngātahi rātou i runga i te haumaru. Kei roto i tēnei ko ngā rongoā i runga i te whakahaunga, ngā rongoā ka taea te hoko, ngā huaora, me ngā tāpiringa otaota.