Tuhinga
Te puru metilīni i ngā whakamātautau taiwhanga G6PD: he aha tonu te mahi a te waikano
Ka mātakitaki tētahi ākonga i ngā ipuipu e rua i muri i te whakamahana. Kua huri tētahi ki te tae i tūmanakohia. Kāore tērā atu i huri. Ka whakatau te ākonga he hapa glucose-6-phosphate dehydrogenase tō te tūroro tuarua. Tērā pea he tika taua whakatau, engari kua mahue i tana whakaaro te ariā e taea ai te whakamātautau. Kāore tētahi mea i roto i te ipuipu e ine hāngai ana i te whākōkī. Ka ine te ipuipu i tā te whākōkī e whakaputa ai, mā te puru metilīni hei takawaenga.
Kia mārama taua ara autaki, ka kore te tukanga, ngā tauira whakahaere, me te rārangi roa o ngā hua hē e āhua matapōkere.
Te whakautu poto
He tohu whakahāora-whakaheke te puru metilīni i te whakamātautau G6PD. Ka whakaputa te whākōkī G6PD i te NADPH, ā, ka whakaheke ngā pūnaha e whakawhirinaki ana ki te NADPH i te puru metilīni kikorangi ki tōna āhua leuco kore tae (ka whakamahi rānei i taua waikano hei kawe irahiko). Ina noa te hohe whākōkī, ka pai te whakaheke. Ina hapa te hohe, ka pōturi, ka kore rānei e oti te whakaheke. Koinei te tohu katoa.
E rua ngā tikanga o mua e whakamahi ana i taua waikano, engari he rerekē ngā pito whakamutunga. Me wehewehe ēnei, ā, ka ngaro te nuinga o te rangirua.
E rua ngā whakamātautau, kotahi te waikano, he rerekē ngā pito whakamutunga
Ko te tuatahi ko te whakamātautau whakaheke hāngai i te puru metilīni, nā Sass me ōna hoa i whakaahua, ā, nō muri ka aromatawaitia e Gibbs. I konei, ko te waikano tonu te pito whakamutunga: ka whakahekea te puru metilīni kikorangi kua whakahāoratia ki te leucomethylene blue kore tae, ā, ka whakaatu te tere o taua huringa i te kaha ki te whakaputa NADPH. Kua whakarārangitia te pepa matua mō te tātari tuatahi ki PubMed i raro i a Sass me ōna hoa (1966).
Ko te tuarua ko te whakamātautau whakaheke methemoglobin a Brewer. Ka whakahāoratia tuatahitia te hemoglobin e te sodium nitrite kia puta ko te methemoglobin parauri. Kātahi ka mahi te puru metilīni hei takawaenga whakawhiti irahiko: ka whakawhiti te puru metilīni kua whakahekea i ngā ōrite whakaheke ki te methemoglobin, kia hoki ai ki te hemoglobin whero. Nō reira, ko te pito whakamutunga e kitea ana ko te parauri ki te whero, ehara i te kikorangi ki te kore tae. I whakaputaina te tikanga taketake ki te Pukapuka Pūrongo a te Whakahaere Hauora o te Ao (Brewer me ōna hoa, 1960).
Kāore e tino tika ā-matū te kī he whakamātautau tango tae i te waikano te whakamātautau Brewer. Ka huri haere te waikano i tētahi huringa tauhohenga; ko te hemoglobin kē ka huri te tae. Ka whakaatu te hoahoa i raro nei i ngā ara e rua.
Te tauhohenga taketake
Ka whakakōkī te G6PD i te hipanga whakahāora tuatahi o te ara pentose phosphate:
Glucose-6-phosphate + NADP+ ka huri hei 6-phosphogluconolactone + NADPH + H+
Ka tino whakawhirinaki ngā pūtau toto whero pakeke ki tēnei ara mō te NADPH, e ai ki te arotake i Blood nā Luzzatto me ōna hoa (2020). Ka pupuri te NADPH i te glutathione ki tōna āhua whakaheke, ā, ka tiaki i te pūtau i te tūkino whakahāora. Ka uru te puru metilīni ki tēnei pūnaha hei kaiwhiwhi irahiko. Ina rawaka te G6PD, ka rawaka te NADPH, ā, ka pai te whakaheke. Ina iti te G6PD, ka motu te mekameka i tōna hononga tuatahi.
Whakaarohia te waikano hei ine wahie, ehara i te pūkaha. Ka whakaatu i te nui o te NADPH e wātea ana. Ko ētahi atu āhuatanga e whakarerekē ana i taua nui, i te pānuitanga rānei, ka taea te neke i te ngira ahakoa kāore he huringa o te ira.
Te whakahaere i te tukanga momo Brewer
Kua rerekē ngā tohutaka tauhohe matū i roto i ngā tekau tau, nō reira kaua e pōhēhētia he kawa haumanu te whakamārama mō te whakaako. Ka whai ia taiwhanga i tāna ake tukanga whakahaere paerewa kua whakamanatia. I runga i taua whakatūpato, koinei te whakahaere o te whakaritenga ipuipu tuku iho, e ai ki te whakaritenga e toru ngā ipuipu me te whakamahana mō te 3 hāora i tuhia ki tēnei whakatinanatanga kua whakaputaina:
- Te ipuipu tūroro: he toto katoa me te glucose, te sodium nitrite, me te puru metilīni. Ka hanga te nitrite i te methemoglobin, ka whāngai te glucose i te pākia o ngā pūtau toto whero, ā, ka whakarato te waikano i te kawe irahiko e whakawhirinaki ana ki te NADPH.
- Te tauira whakahaere kua whakahāoratia: he toto me te nitrite me te glucose, engari kāore he puru metilīni. Ka whakaatu i te pito whakamutunga methemoglobin parauri, arā, te tauira o te hapa.
- Te tauira whakahaere hemoglobin: he toto me te wai. Ka whakaatu i te pito whakamutunga hemoglobin whero, arā, te tauira noa.
- Whakamahanatia ngā ipuipu katoa kia tata ki te 37 C, mō te 3 hāora i te nuinga o te wā, me te whakaranu āta.
- Waimehatia tētahi wāhanga iti o ia ipuipu ki te wai māturu kia pakaru ai ngā pūtau, kia mārama ai te whakataurite i ngā tae.
- Whakatauritea te ipuipu tūroro ki ngā tauira whakahaere e rua. Ko te hoki ki te whero he tohu i te kaha whakaheke nui tonu. Ko te mau tonu ki te parauri he tohu pea i te hapa nui.
I whakamahia e tētahi whakaritenga o mua te toto tata ki te 1 mL me ngā tauhohe matū tata ki te 180 mM sodium nitrite, te 280 mM dextrose, me te 0.4 mM puru metilīni. Me whakaaro ki aua tau hei tauira nō ngā tuhinga rangahau, kaua hei whakaritenga mō ngā wāhi katoa.
Te pānui i te hua
| Tauira | Āhua i te whakamātautau Brewer | Tikanga |
|---|---|---|
| Noa | Ka tata te ipuipu tūroro ki te tauira whakahaere hemoglobin whero | Kei runga ake te kaha whakaheke i te paepae tātari tuatahi |
| Hapa | Ka noho parauri te ipuipu tūroro, e tata ana ki te tauira whakahaere methemoglobin | E tohu ana te tātari tuatahi i te hapa G6PD nui |
| Waenga | Kei waenganui te tae i ngā tauira whakahaere | Kaua e whakatau i tētahi momo āhuatanga; inea te hohe ā-rahinga |
He pai rawa te wehewehe a te tātari tuatahi ā-kounga i te takiwā o te 30 ki te 40 ōrau o te hohe noa, e ai ki tēnei arotake i Malaria Journal mō ngā āputa i te tautuhi G6PD. Nō reira, ko te hua noa o te tātari tuatahi e tohu ana kāore te whakamātautau i kite i te hapa tino nui. Kāore e whakaū ana he tino noa katoa te hohe.
He aha te tātari tuatahi e whakapohehe ai
Ko te pikinga o ngā reticulocyte i muri i te pakarutanga o ngā pūtau toto whero te take rongonui o te hua noa hē. He nui ake te G6PD kei ngā pūtau toto whero taitamariki me ngā reticulocyte i tō ngā pūtau tawhito. I tētahi wā pakarutanga ohorere o ngā pūtau toto whero, kua ngaro kē pea ngā pūtau tawhito rawa, hapa rawa hoki, ka mahue he huinga pūtau taitamariki e puta noa ai tō rātou hohe i te whakamātautau. Ka whakapohehetia hoki ngā whakamātautau ā-rahinga e tēnei āhuatanga koiora, ehara i te mea ko ngā tātari waikano anake.
Ka huna te whakawhitinga toto nō nā tata nei i te hapa, nā te mea ka tāpiri ngā pūtau toto whero o te kaituku i tō rātou ake whākōkī ki te tauira. Ka whakarārangi a Mayo Clinic Laboratories i te whakawhitinga toto hei take e kore ai e pono ngā hua whākōkī, me te pikinga o ngā reticulocyte me te whakararuraru a ngā pūtau toto mā.
Ko ngā wāhine heterozygous te rōpū uaua rawa. Nā te whakakorenga hohe matapōkere o te pūira X, ka puta he ranunga o ngā pūtau toto whero noa me ngā mea hapa, ā, ka noho te hohe whānui ki hea noa i waenga i te tino hapa me te āhua noa. He rite tonu te kore kitea o ngā momo āhuatanga waenga e ngā tātari tuatahi ā-kounga. Ka āta kōrero ngā tuhinga a WHO mō te whakamātautau G6PD mō tēnei herenga.
Ka tāpiri te pakeke o te tauira, te pāmahana rokiroki, te whakawhirinaki o te whakatau pito whakamutunga ki tā te kaititiro e kite ai, te hematocrit, me te kounga o ngā tauhohe matū i te rangirua tātari ki runga ake i ēnei pānga koiora.
Ehara te tātari tuatahi i te whakatau mate
Ina puta he hua hapa, he hua waenga, he hua rānei kāore e hāngai ki ngā kitenga haumanu, me whai he whakamātautau ā-rahinga mō te whākōkī G6PD. Ki tā WHO, ko te ine ā-rahinga mā te spectrophotometry, me te pāmahana e whakahaeretia ana, te tikanga tohutoro. I taua whakamātautau, ka tāpirihia te glucose-6-phosphate me te NADP+ ki tētahi tauira o ngā pūtau toto whero kua pakaru, ā, ka aroturukihia te NADPH ka puta mā te pikinga o te ngongo māramatanga i te 340 nm. Ka hāngai te tere o te huringa ki te hohe whākōkī, ā, i te nuinga o te wā ka whakatauritea ki te hemoglobin hei waeine mō ia karamu. Kua whakarāpopototia ngā mahi haumanu-taiwhanga o nāianei ki te urunga rārangi whakamātautau a Mayo mō te hohe G6PD.
Ka whakamahia te awhe tohutoro kua whakamanatia e te taiwhanga hei whakamārama i te hua. I te nuinga o te wā ka whakatauritea ki te hohe noa o te taupori, ā, ka kōrerotia te hapa i te takiwā o te paepae 30 ōrau, me te hohe waenga kei runga ake i taua paepae. Ka whakatakoto ngā kōtaha hua whāinga a WHO mō ngā whakamātautau G6PD (2022) i taua anga whakatau mate. Ki te kore e pono te tautuhi momo āhuatanga nā te pakarutanga o ngā pūtau toto whero, nā te whakawhitinga toto rānei nō nā tata nei, me whakamātautau anō i muri i te hokinga o te huinga pūtau toto whero ki te taurite. Ka taea hoki e te whakamātautau rāpoi te tāpiri mōhiohio, engari kāore e whakakapi i te inenga whākōkī.
He rohe e tika ana kia mau
I ēnei whakamātautau, he tauhohe matū taiwhanga i waho i te tinana te puru metilīni. He take motuhake tēnei i te kōrero mō te haumaru o te hoatu puru metilīni ki tētahi tūroro, e kōrerotia ana i ngā aratohu mō ngā āhuatanga e kore ai e tika te whakamahi puru metilīni. Ka tūhono te matū koiora i ēnei take, nā te mea ka whakawhirinaki hoki te rongoā ki te whakaheke e whakawhirinaki ana ki te NADPH, engari ehara te waikano i roto i te ipuipu whakamātautau i te horopeta i roto i te rerenga toto. Mō te horopaki o te mahi a te waikano hei whakapoke i tētahi atu whakamahinga, tirohia ngā mātāpono whakapoke o te puru metilīni, ā, mō tōna whānau matū, tirohia ngā waikano whakahāora-whakaheke i te taiwhanga.
Ka pātai te tātari tuatahi mēnā e āhua mau tonu ana te kaha whakaheke. Ka pātai te whakamātautau whakaū e hia tonu te whākōkī kei reira. He pai te mahi a te waikano mō te pātai tuatahi, mehemea kāore tētahi e tono kia whakautua e ia te pātai tuarua.