Te kikorangi metilīni mō te metahimokoropinemia: he aha i whakamahia ai i ngā hōhipera

Ka tae mai tētahi tūroro, he rite te tae o ōna ngutu ki te kōhatu papatahi hina, he tere tana hā, he pōturi tana whakautu. E ai ki te pūrere aroturuki, kei te 85 ōrau te kukūtanga hāora. Ka whakapiki te nēhi i te hāora. Kāore te tau e neke. Ka tangohia he toto, ā, he parauri tiakarete tōna tae; ka noho parauri tonu ahakoa ka pā ki te hau. Ko tēnei raupapa te tohu tīmatanga e tino mōhiotia ana mō te tiaki metahimokoropinemia mā te kikorangi metilīni, ā, e whakaatu ana he aha tēnei mate e whakapoauau ai i ngā whānau me ngā mīhini.

Ehara ngā pūkahukahu i te pūtake o te raruraru. Ko te rino kei roto i te himokoropini kē.

E rua ngā āhua o taua ngota rino kotahi

Kei ia rāpoi ngota himokoropini he rino i te āhua Fe2+. I taua āhua ka herea e ia te hāora i ngā pūkahukahu, ka tuku ai ki ngā kiko. Ka taea e ngā matū whakahāora te tango i tētahi atu irahiko i taua rino, ka huri ai ki te āhua Fe3+. Ko te matū tae ka puta ko te metahimokoropini. Kāore e taea e te rino Fe3+ te here hāora. Ko te mea kino ake, ka whakakaha ake i te pupuri hāora a ngā wāhi rino Fe2+ e toe ana i taua rāpoi ngota, nō reira ka iti iho te hāora ka tukuna ki ngā wāhi e hiahiatia ana.

I te toto hauora, ka noho te metahimokoropini ki raro iho i te 1 ōrau pea, nā te mea e mahi tonu ana tētahi whākōkī whakatika. Ka whakamahi te ara whakatika matua i te NADH me te whākōkī whakaiti cytochrome b5, ka whāngaihia e te wetewete kūhuka noa i te pūtau toto whero. Ka whakamahia te NADPH e tētahi ara tāpiri, engari he iti noa tāna mahi i ngā āhuatanga noa. Ka hāngai āhua ngā tohumate ki te ōrau, ahakoa ka mōrearea pea tētahi tau iti ake mēnā he anemia, he mate manawa, he mate pūkahukahu rānei. Ka kitea pea te kikorangi o te kiri me te ngenge i ngā uara tata ki te 10 ki te 20 ōrau. I runga ake i te 30 ōrau, ka puta noa te ānini, te hā poto, me te rangirua. He maha ngā wā ka mate te tangata i ngā uara tata ki te 70 ōrau. He awhe ēnei tau, ehara i te oati, nā te mea ko te tukunga hāora te mea nui, kaua ko te uara taiwhanga anake.

Ko ētahi pūtake noa o te mate ka riro mai ko te dapsone, ngā nitrite me ngā nitrate, ngā pūhui aniline, me ngā rongoā whakamoe wāhi pērā i te penetokēni me te prilocaine. Kei reira hoki ngā korenga whākōkī nō te whānautanga mai, engari he onge. Ko te nuinga o ngā take i te hōhipera he mate ka riro mai, nō reira ko te mahi maimoatanga tuatahi i ngā wā katoa, ko te whakamutu i te pānga o te matū.

Te waka whakawhiti e whakamahia ana e ngā hōhipera

Kāore te kikorangi metilīni e tuku hāora. Ka whakarato kē i te kawe irahiko. I roto i te pūtau toto whero, ka whakamahia te NADPH e tētahi whākōkī hei whakaiti i te waikano kikorangi ki tōna āhua kore tae, arā, te reuko-kikorangi metilīni. Kātahi ka tukuna e taua āhua kore tae ngā irahiko ki te rino Fe3+ me te kore whākōkī, ka whakahokia te himokoropini ki te āhua Fe2+, ā, ka hoki anō te waikano ki tōna āhua kikorangi. Ka huri haere tonu te waikano i tēnei huringa. Ka whakaahuatia taua tukunga irahiko i te whakamārama mō te whakawhiti irahiko a te kikorangi metilīni i waenga i ngā kaituku me ngā kaiwhiwhi, ā, ka whakamāramatia te āhua whakaiti kore tae i te whakataurite i te kikorangi metilīni me te reuko-kikorangi metilīni.

E whakamārama ana tēnei tukanga i ngā here e rua e ohorere ai te hunga kātahi anō ka ako. Tuatahi, e hiahiatia ana te NADPH mō te waka whakawhiti, ā, e whakawhirinaki ana te NADPH o ngā pūtau toto whero ki te glucose-6-phosphate dehydrogenase, arā, te G6PD. Ki te hapa te G6PD, kāore pea te waikano e huri ki tōna āhua hohe, me te tāpiri anō i tōna ake taumahatanga whakahāora. Koia te take ka whakarārangitia te korenga G6PD ki te tapanga hōhipera hei take kia kaua e whakamahia. Tuarua, he matū whakahāora tonu te kikorangi metilīni ina nui rawa te tapeke o ngā horopeta. Ki tua atu i te tapeke tata ki te 7 mg mō ia kg, ā, i ētahi wā ka iti ake mēnā ka tukurua ngā horopeta, ka whakapiki pea i te metahimokoropini, ka whakangaro hoki i ngā pūtau toto whero, kaua ko te whakatika. Ehara te nui ake o te waikano i te nui ake o te whakaora. He whāiti te awhe horopeta, ā, he take tō tēnā.

He aha te whakamātautau mārama tonu e whakapohehe ai

He maha ngā whānau e rapu ana i tētahi whakamātautau kikorangi metilīni ka taea ki te kāinga. Kāore he whakamātautau pērā. I te nuinga o te wā, e tohu ana taua kīanga ki te raupapa whakamātautau hei tātaritanga mō te metahimokoropinemia e whakapaetia ana, ā, me whakahaere taua raupapa i te taiwhanga.

E rua noa ngā roangaru mārama ka pānuihia e ngā pūrere ine hāora ā-patumanawa noa. Ina piki te metahimokoropini, ka tata haere ngā taumata ngongo mārama i aua roangaru e rua, ā, ka neke te kukūtanga e whakaaturia ana ki te takiwā o te 85 ōrau, ahakoa he aha te āhua tūturu. Kāore te hāora tāpiri e whakatika i te tau, nā te mea ehara te mea hē i te mea e inehia ana e te pūoko. I taua wā tonu, ka āhua noa pea te pēhanga hāora i te toto ia-tuku, arā, te PaO2, nā te mea e ine ana tēnei i te hāora kua memeha ki te wē toto, kaua ko te hāora e kawea ana e te himokoropini. Nō reira ka pūrongotia pea e tētahi pūrere tātari hau toto, e tātai ana i te kukūtanga mai i te PaO2, tētahi tau whakamauru, ahakoa kei te kikorangi tonu te tūroro. Ko te wehenga i waenga i te pūoko matimati me te uara toto ia-tuku kua tātaihia, he nui ake i te 5 ira ōrau i te nuinga o te wā, e kīia ana ko te āputa kukūtanga. He tohu tēnei, ehara i te tātaritanga whakaū.

Hei whakaū, me ine tika te metahimokoropini i tētahi tauira toto mā te ine hāora maha-roangaru. Ka tohu anō te toto parauri tiakarete, kāore e mārama ake tōna tae ina tāpirihia te hāora, ki taua mate. Ko te pūtake o ngā whakataunga maimoatanga katoa mō te metahimokoropinemia ko taua inenga me ngā tohumate, kaua rawa ko te pūrere ine hāora ā-patumanawa anake.

Te maimoatanga i raro i te tirotiro ngaio e whakaahuatia ana i te tapanga

E whakaahua ana tēnei wāhanga i tētahi werohanga rongoā whakahaunga ka tukuna e ngā kaimahi haumanu. Ehara tēnei i te aratohu mō te tango mā te waha, ā, kāore tētahi o ēnei taipitopito e hāngai ki ngā pata, ngā kāpene rānei e hokona ana mā te kiritaki.

E ai ki ngā mōhiohio whakahaunga o nāianei mō te werohanga kikorangi metilīni, ko te maimoatanga mō te metahimokoropinemia kua riro mai i ngā pakeke me ngā tamariki he 1 mg mō ia kg, ka tukuna ki roto i te ia toto mō te 5 ki te 30 meneti. Mō te hua paerewa 5 mg mō ia mL, he ōrite tēnei ki te 0.2 mL mō ia kg. Ki te noho tonu te metahimokoropini ki runga ake i te 30 ōrau, ki te mau tonu rānei ngā tohumate i muri i te kotahi hāora, ka tukuna pea tētahi horopeta tuarua o te 1 mg mō ia kg. Ki te kore ngā horopeta e rua e whakatau i te raruraru, ka tohutohu te tapanga i ngā kaimahi haumanu kia tahuri ki tētahi atu maimoatanga, kaua ki tētahi horopeta tuatoru.

He mea nui ngā taipitopito tuku. Me tuku mā tētahi ngongo ia toto e mahi tika ana, kaua rawa mā te werohanga ki raro i te kiri. Ka taea te waimeha i te wairewa ki roto i te 50 mL dextrose 5 ōrau hei whakaiti i te mamae ki taua wāhi, otirā mō ngā tamariki, ā, me whakamahi wawe. Kaua e waimeha ki ngā wairewa konutai pūhaumāota, nā te mea ka whakaiti te pūhaumāota i te āheinga o te waikano ki te memeha. Ka whiua ngā wāhanga kāore i whakamahia, ā, ka tirohia te wairewa i mua i te whakamahinga.

E toru ngā whakatūpato e ārahi ana i ia whakataunga hōhipera. Ko te whakatūpato kei roto i te pouaka e pā ana ki te mate huinga tohumate serotonin, ka mate pea te tangata ina tūtaki te kikorangi metilīni ki ngā rongoā e pā ana ki te serotonin, tae atu ki ngā SSRI, ngā SNRI, ngā aukati MAO, ētahi opioid, me te dextromethorphan. Ka tohutohutia ngā tūroro kia kaua e tango i aua rongoā mō te 72 hāora i muri i te horopeta whakamutunga. Ki te kore e taea te karo i taua whakakotahitanga, e whakahau ana te tapanga kia whakamahia te horopeta iti rawa ka taea, me te āta aroturuki. Ka kōrerohia ngā pāhekoheko serotonin i ngā pāhekoheko rongoā kikorangi metilīni me te mate huinga tohumate serotonin. Ko te whakatūpato tuarua ko te pakarutanga o ngā pūtau toto whero: ka pakaru ēnei pūtau me te putanga o ngā tinana Heinz, te pikinga o te pirirūpini autaki, te hekenga o te haptoglobin, me te anemia ka puta pea i muri i te rā kotahi, i muri atu rānei, ā, ka hiahiatia pea he whakawhitinga toto. Ko te tuatoru ko te korenga o te pānga, ko te hokinga ake rānei o te mate, e kitea ana i te pānga o te dapsone, o ngā matū momo aniline rānei, me te korenga G6PD; i ēnei āhuatanga ka tohu anō te tapanga ki tētahi atu maimoatanga. Kāore te ngoikore o ngā tākihi e rāhui i te whakamahinga, engari ki te āhua nui, ki te tino nui rānei taua ngoikore, ka whakawhāitihia te raupapa maimoatanga ki te horopeta kotahi o te 1 mg mō ia kg. I roto i te huinga raraunga pakeke o te tapanga, ko ētahi pānga kino ko te ānini, te whakapairuaki, te korere, te iti o te pāhare pāporo me te konupora, te hukihuki uaua, me ngā takahanga āhua rite ki te hukihuki roro; kotahi te takahanga taumaha āhua rite ki te hukihuki roro i waenga i ngā tūroro 31. Ka whakaheke teka hoki te waikano i ngā pānuitanga o te pūrere ine hāora ā-patumanawa i muri i te whāngai ā-ia, nō reira ka whai kē ngā kaimahi haumanu i te ine hāora maha-roangaru me ngā tohumate.

Ka haere tahi te tiaki tautoko: te hāora rere nui, te tautoko i te ara hā me te rere toto, te whakatika i te kūhuka, me te aroturuki. Ka tau pea ngā take māmā kāore he tohumate ina tangohia te pūtake. E ai ki ngā paepae e kōrerotia whānuitia ana, ka maimoatia ngā tūroro whai tohumate mai i te takiwā o te 20 ōrau, mai rānei i te 10 ki te 20 ōrau mēnā kua raru kē te tukunga hāora; ka whakaarohia hoki te maimoatanga mō ngā tūroro kāore he tohumate mai i te takiwā o te 30 ōrau. Ka maimoatia ngā tohumate tino taumaha me te kore tatari ki te tau taiwhanga. Ko te waikawa ascorbic, te whakawhitinga whakakapi toto, me te hāora i raro i te pēhanga teitei ētahi kōwhiringa whakaora ina kore e taea te whakamahi i te kikorangi metilīni, ina kore rānei e whai hua; engari he pōturi rawa ake te mahi a te waikawa ascorbic. He take hanga motuhake ngā tuhinga whakaū i te parakore o tētahi hua werohanga, ā, ka whakamāramatia i te pānui i tētahi tiwhikete tātaritanga kikorangi metilīni.

Te penetokēni me te pātai mō te rā whakaputa niho

Ko tētahi wāhanga nui o ngā rapunga kiritaki nō ngā mātua kua whakamahi i te rā, i te rehu penetokēni rānei ki te waha, kātahi ka kite i ngā ngutu kikorangi, i tētahi tau iti rānei i te pūrere ine hāora. Me noho wehe ngā wāhanga e toru.

Ko te whakapae kua pāngia te tangata te wāhanga ki te kāinga. He matū whakahāora te penetokēni e mōhiotia ana hei pūtake, ā, kua whakatūpato ngā mana whakahaere kia kaua e whakamahia ngā hua penetokēni ā-waha e hokona ana me te kore whakahaunga mō te whakaputa niho, mō ngā tamariki rānei kei raro i te 2 tau. Ko te take, ka taumaha pea te metahimokoropinemia e pā ana ki te penetokēni, ā, he iti noa tōna painga mō te mamae whakaputa niho. Whakamutua te hua, tuhia te wā me te rahinga, tuhia ngā tohumate pērā i te tino kōmā rerekē, te tae hina, kikorangi rānei o ngā ngutu me ngā papa i raro i ngā matikara, te hā poto, te hiamoe rerekē, te ngoikore rānei o te kai, ā, rapua wawe he aromatawai ohotata. Kaua e tuku i te kikorangi metilīni ki te kāinga, ahakoa te momo. Kāore he horopeta mō te kāinga, kāore hoki he whakamātautau kāinga e whakaū ana kāore tēnei mate.

Ko te tātaritanga te wāhanga ki te hōhipera. Ka whakaū ngā kaimahi haumanu mā te ine hāora maha-roangaru, ka arotake i ngā pānga matū whakahāora katoa, tae atu ki te dapsone, ngā nitrite, me ngā pūhui aniline, ā, ka aromatawai i ngā tohumate me te tukunga hāora, kaua ko tētahi tau kotahi anake.

Ko te maimoatanga i raro i te tirotiro ngaio te wāhanga ki te wāhanga tūroro kua whakaahuatia i runga ake nei. Ahakoa ko te penetokēni te pūtake, kāore te ripanga horopeta e rerekē. Kāore te tapanga werohanga o nāianei e whakaingoa motuhake ana i te penetokēni; ka taka tētahi take penetokēni ki raro i te metahimokoropinemia kua riro mai, ā, ka whakahaerehia i raro i taua tikanga maimoatanga ngaio anō, tae atu ki ngā arowhai G6PD, tākihi, me te serotonin.

Ngā wā ehara te kikorangi metilīni i te whakautu

Ehara i te whakautu ina mōhiotia te korenga G6PD. Ehara i te whakautu i muri i te korenga o ngā horopeta e rua e ai ki te tapanga, ina hoki ake rānei te metahimokoropinemia, e tohu ana ki te ngongo haere tonu o ngā rongoā pērā i te dapsone. Ehara i te whakautu hei ārai, hei ritenga tango tāpiringa, hei rongoā kāinga rānei i muri i te pānga matū. Ka whakakino pea te horopeta nui, te whakamahinga tukurua rānei, i taua tau tonu e whai ana kia whakahekea. Ka kitea pea te pakarutanga o ngā pūtau toto whero i te rā i muri mai, ina kua hoki kē te tūroro ki te kāinga. Ko taua mōrea tōmuri tētahi take ka aroturuki ngā hōhipera, kaua e tuku wawe i te tūroro kia hoki ki te kāinga.

He poto te whakarāpopototanga pono. Ko te metahimokoropinemia he rino kua whakahāoratia, kāore e taea te kawe hāora. He waka whakawhiti irahiko te kikorangi metilīni e āhei ai ngā pūtau toto whero ki te whakaiti i taua rino, mēnā ka taea e te pūtau te hanga NADPH, ā, mēnā ka noho te horopeta i roto i te raupapa whāiti kua whakaritea e te tapanga. Me whai ine hāora maha-roangaru te tātaritanga, nā te mea ka whakapohehe ngā pūrere aroturuki māmā ake. Ko ērā atu mea katoa, mai i te dextrose hei waimehatanga, ki te hītori serotonin, ki te ngongo penetokēni i te kāpata rongoā, he taipitopito hei tautoko i taua waka whakawhiti kotahi.