Tuhinga
Te kikorangi methylene me te ngoikoretanga G6PD: he aha i whai tikanga ai te mōrea
Ka aukatia te whakamahi i te kikorangi methylene ki ngā tāngata he ngoikoretanga tō rātou o te glucose-6-phosphate dehydrogenase, e whakapotohia ana i te nuinga o te wā hei ngoikoretanga G6PD. E tautuhi ana ngā mōhiohio tuku rongoā mō PROVAYBLUE i te anemia nā te pakarutanga o ngā pūtau toto whero hei take mō tēnei aukatinga. Ko te tikanga, ka tere ake pea te pakaru o ngā pūtau toto whero i te hanga a te tinana i ngā pūtau hei whakakapi. Mā taua ngoikoretanga anō e kore ai pea te rongoā e mahi i tōna mahi e tūmanakohia ana ki te methemoglobinemia.
He raruraru ēnei e hono ana. Me whai puna kaha whakaheke te kikorangi methylene i roto i te pūtau toto whero. Ka āwhina te G6PD ki te pupuri i taua puna, e hiahiatia ana hoki e te pūtau hei tiaki i a ia i te tūkino hāora. Kāore te parakore o tētahi hua e whakatika i te ngoikoretanga whākōkī o te tangata e whiwhi ana i taua hua.
Te mahi a G6PD i roto i te pūtau toto whero
He whākōkī te G6PD i roto i te ara pentose phosphate, arā, he ara tukatuka kūhuka. Ko tētahi hua o tēnei ara ko te NADPH, he rāpoi ngota e tuku ana i ngā irahiko ki ētahi atu tauhohenga. Mā aua irahiko e haere tonu ai ētahi tukanga tiaki.
E whakamārama ana te aratohu CPIC mō ngā rongoā me te momo ira G6PD i te take e tino whai tikanga ai tēnei ki ngā pūtau toto whero: ko tō rātou puna NADPH e wātea ana ko te ara pentose phosphate. Ka whakaputa te G6PD me tētahi atu whākōkī, te 6-phosphogluconate dehydrogenase, i te NADPH i roto i taua ara. Ina heke te mahi a G6PD, ka iti ake te kaha o te pūtau ki te urupare ina piki te ahotea hāora.
Ko tētahi whakamahinga tiaki o te NADPH ko te hangarua i te glutathione. Ka whakamahia e te glutathione reductase te NADPH hei whakahoki i te glutathione ki tōna āhua whakaheke, e āwhina ana i te glutathione peroxidase ki te tango i ngā peroxide. E hono ana te whakamārama a PharmGKB mō te ara i roto i te pūtau toto whero i ēnei tauhohenga. Ki te kore e rawaka te kaha whakaheke, ka tūkino pea ngā matū whakahāora i te pūtoto me te pūtau e karapoti ana i a ia.
Nō reira, e pā ana te ngoikoretanga G6PD ki te kaha ki te tū atu ki tētahi wero. Ehara i te mea he anemia pūmau tō ia tangata e pāngia ana, he māuiui rānei ia i ia rā.
He aha te kikorangi methylene e whakawhirinaki ai ki taua puna anō
I te nuinga o te wā, kei roto i te pūtoto te rino i te āhua ferrous, Fe²⁺. Mā te whakahāoratanga e huri ai ki te āhua ferric, Fe³⁺, ka puta ai te methemoglobin. Kāore taua rino kua panoni e here tika i te hāora. Ina nui rawa te whakaeminga, ka raruraru te tuku hāora.
Ka āwhina pea te kikorangi methylene ki te whakahoki i tēnei panonitanga, engari he mea nui tētahi takahanga i waenganui. I roto i te pūtau, ka hurihia te kikorangi methylene hei kikorangi leucomethylene mā te whakahekenga e whakawhirinaki ana ki te NADPH. Kātahi ka whakaheke te kikorangi leucomethylene i te methemoglobin kia hoki atu ki te pūtoto e mahi tika ana. Ka whakaahuatia tēnei raupapa i roto i te whakamārama o te tapanga rongoā mō te tikanga mahi.
He ara motuhake anō mō te tiaki noa: ka whakahaere te cytochrome b5 reductase e whakawhirinaki ana ki te NADH i te nuinga o te whakahekenga methemoglobin e haere noa ana. He rāpoi ngota rerekē te NADH me te NADPH. Ehara te ngoikoretanga G6PD i te mea ka whakaweto noa i ia ara pupuri pūtoto. Ka tino whai tikanga te raruraru ina whakapiki te pānga o tētahi matū whakahāora i te hiahia, ā, ka whakaurua te kikorangi methylene. E whakamārama ana tā mātou aratohu mō te kikorangi methylene mō te methemoglobinemia i te horopaki haumanu.
E rua ngā hua o te kore rawaka o te NADPH
Whakaahua 1. He mahere ngāwari o ngā hononga whakawhirinaki. E whakaatu ana ngā pere e hono ana i ngā pouaka ara i te tautoko mō tētahi tukanga; ehara i te huinga katoa o ngā tauhohenga matū. I te peka rongoā, ka tuku te kikorangi leucomethylene i te kaha whakaheke mō te hurihanga mai i Fe³⁺ ki Fe²⁺. Ka whakapiki pea te kikorangi methylene tonu i te ahotea hāora. Ka whakakotahi tēnei whakaahua ariā i ngā mōhiohio tuku rongoā me te ara PharmGKB kua honoa.
E tautuhi ana ngā tūtohutanga a EHA me EuroBloodNet i te hekenga o te whaihua me te tūponotanga o te kino i te ngoikoretanga G6PD:
- Ka kore pea te maimoatanga e whaihua. Mā te kore rawaka o te NADPH e iti ai pea te hurihanga o te kikorangi methylene ki tōna āhua whakaheke, ka waiho te methemoglobin kāore anō kia tika te whakatikaina.
- Ka nui ake pea te wharanga nā te whakahāoratanga. He matū whakahāora te kikorangi methylene tonu. I roto i tētahi pūtau whakaraerae, ka whai wāhi pea ki te pakarutanga o ngā pūtau toto whero, ā, ka whakakino ake pea i te methemoglobinemia.
Ka puta ngātahi pea ēnei hua: ka iti ake ngā pūtau toto whero e mau tonu ana, ā, ka iti ake te whaihua o te kawe hāora i ngā pūtau e toe ana. Koia te take kāore te urupare takarepa e tika hei take kia tāpiri tonu te tangata i te kikorangi methylene i runga i tāna ake whakatau.
He aha te noho ora e kore ai e whakaū i te haumaru
E ai ki MedlinePlus Genetics, he tokomaha ngā tāngata he ngoikoretanga G6PD tō rātou kāore he tohumate, ā, kāore rātou e mōhio kei a rātou tēnei āhuatanga. Mā ngā mate whakapokenga, ētahi rongoā me ētahi atu whakaoho ahotea hāora e kitea ai te whakaraeraetanga. Ahakoa e hono ana tēnei āhuatanga ki te tuku iho mā te pūira X, ā, he nui ake te kitea i ngā tāne, ka pā hoki ki ngā wāhine tētahi ngoikoretanga e whai tikanga ana ki te tiaki haumanu.
Ka whakaatu tētahi taipitopito whaihua o te whakamātau i te rerekētanga i waenga i ngā āhuatanga whakatā me te ahotea. I roto i tētahi rangahau nō 1996 nā Bozzi, Parisi rāua ko Strom, he tata ngā taumata glutathione i te āhua whakaheke i ngā pūtau toto whero tangata kātahi anō ka kohia, he tino ngoikoretanga G6PD tō rātou, ki ērā o ngā pūtau noa. Heoi, i muri i tētahi wero peroxide whakamātau, he nui te ngaronga glutathione i ngā pūtau ngoikore, ā, he iti ake tō rātou whakaora i tō ngā pūtau noa. He whakamātau taiwhanga ēnei ki ngā pūtau toto, ehara i te whakamātautau hei whakaū i tētahi rahinga haumaru o te kikorangi methylene. E whakamārama ana tēnei kitenga i te take kāore tētahi āhua tīmatanga e kitea ana he noa e matapae tika ai i te kaha ki te tū atu i te wā e pāngia ana.
Me aha ki ēnei mōhiohio
Mēnā kua whakatauria he ngoikoretanga G6PD tōu, whakamōhiotia te kaiwhakaora haumanu, te kaitaka rongoā rānei i mua i te whakaaro ki te whakamahi i te kikorangi methylene. E whakarōpū ana te aratohu CPIC i te kikorangi methylene hei rongoā mōrea nui. Ehara te whakaahuatanga a te kaihoko he iti te rahinga rongoā, tētahi whakamahinga o mua rānei kāore i puta he raruraru, i te whakaaetanga ki te takahi i te aukatinga whakamahinga.
Mēnā kāore tō tūnga i te tino mōhiotia, kawea mai ngā hua whakamātau G6PD o mua me ngā hītori whānau e hāngai ana ki te kōrerorero. Ka pā pea te pakarutanga o ngā pūtau toto whero i tata nei, tētahi tuku toto rānei, ki te whakamāoritanga o tētahi hua whakamātau whākōkī. E whakamārama ana te aratohu taiwhanga mō te whakamātau G6PD i te whakamātau me ōna herenga; kāore te tatau pūtau toto noa anake e whakautu i taua pātai anō. Ko te tūnga G6PD hoki tētahi wāhanga noa iho o te aromatawai haumaru whānui ake mō te kikorangi methylene.
Whai muri i te pānga, mēnā ka puta mai he tino ngoikore hou, he hēmanawa, he kōwhai o te kiri me ngā karu, he mimi pōuri, he manawa tere rānei, me tere te whiwhi aromatawai hauora. Ki te tino uaua te hā, ki te mamae te uma, ki te hinga, ki te rangirua rānei, me whiwhi tiaki ohotata. Kawea mai te ipu o te hua, ōna taipitopito rānei, tae atu ki te kukūtanga, te rahinga me te wā o te pānga. Kaua e whakamahi i te tae o te mimi anake hei whakatau mēnā kei te pakaru ngā pūtau toto whero.
Ina puta te methemoglobinemia ki tētahi tangata he ngoikoretanga G6PD tōna, mā te rōpū ohotata e kōwhiri te maimoatanga. E matapaki ana ngā tūtohutanga mātanga i ētahi atu kōwhiringa, tae atu ki te waikawa ascorbic me te whakawhitinga toto whakakapi i ngā āhuatanga tino taumaha. Ehara ēnei i ngā rongoā kāinga ka taea te whakamahi hei whakakapi i tētahi ki tētahi: he pōturi ake te mahi a te waikawa ascorbic, ā, mā te tuku hāora o te tūroro, te pakarutanga o ōna pūtau toto whero me tōna āhua haumanu e whakatau he aha te tautoko e hiahiatia ana.