I-methylene blue nokuntuleka kwe-G6PD: kungani ubungozi bubalulekile

I-methylene blue akufanele isetshenziswe kubantu abantula i-glucose-6-phosphate dehydrogenase, okuvame ukubizwa ngokufushane ngokuthi ukuntuleka kwe-G6PD. Imininingwane yokunquma ukusetshenziswa kwe-PROVAYBLUE ibalula i-anemia ebangelwa ukubhidlika kwamangqamuzana abomvu egazi njengesizathu. Lokhu kusho ukuthi amangqamuzana abomvu egazi angabhidlika ngokushesha kunendlela umzimba owakha ngayo amasha ukuze uwashintshe. Ukuntuleka okufanayo kungavimbela nomuthi ukuthi usebenze ngendlela okuhloswe ngayo ekwelapheni i-methemoglobinemia.

Lezi zinkinga zixhumene. I-methylene blue idinga amandla okunciphisa ngokwamakhemikhali ngaphakathi kwengqamuzana elibomvu legazi. I-G6PD isiza ekugcineni lawo mandla etholakala, futhi ingqamuzana liwadinga ukuze lizivikele emonakalweni obangelwa i-oxidation. Ubumsulwa bomkhiqizo abulungisi ukuntuleka kwe-enzyme kumuntu owutholayo.

Lokho okwenziwa yi-G6PD engqamuzaneni elibomvu legazi

I-G6PD iyi-enzyme esemzileni we-pentose phosphate, okuyindlela yokucubungula i-glucose. Enye yezinto ezikhiqizwa yilo mzila yi-NADPH, okuyi-molecule enikela ngama-electron kokunye ukusabela kwamakhemikhali. Lawo ma-electron avumela izinqubo eziningana zokuvikela ukuthi ziqhubeke nokusebenza.

Umhlahlandlela we-CPIC ngemithi nohlobo lwezakhi zofuzo ze-G6PD uchaza ukuthi kungani lokhu kubaluleke kakhulu emangqamuzaneni abomvu egazi: umthombo we-NADPH otholakala kuwo umzila we-pentose phosphate. I-G6PD nenye i-enzyme, i-6-phosphogluconate dehydrogenase, zikhiqiza i-NADPH kulowo mzila. Ukuncipha kokusebenza kwe-G6PD kunciphisa ikhono lengqamuzana lokubhekana nokwanda kokucindezeleka okubangelwa i-oxidation.

Enye indlela i-NADPH esetshenziselwa ngayo ukuvikela ukubuyisela i-glutathione esimweni sokuba iphinde isetshenziswe. I-glutathione reductase isebenzisa i-NADPH ukubuyisela i-glutathione esimweni esincishisiwe, okusiza i-glutathione peroxidase ukuthi isuse ama-peroxide. Incazelo ye-PharmGKB yomzila wamangqamuzana abomvu egazi ixhumanisa lokhu kusabela kwamakhemikhali. Uma amandla okunciphisa ngokwamakhemikhali enganele, izinto ezibangela i-oxidation zingalimaza i-hemoglobin nengqamuzana eliyizungezile.

Ngakho ukuntuleka kwe-G6PD kuphathelene nekhono lokumelana nenselele. Akusho ukuthi wonke umuntu onakho uhlale ene-anemia noma ezizwa egula nsuku zonke.

Kungani i-methylene blue incike emandleni afanayo

Ngokuvamile i-hemoglobin iqukethe i-iron esesimweni se-ferrous, Fe²⁺. I-oxidation iyiguqulela esimweni se-ferric, Fe³⁺, ikhiqize i-methemoglobin. Leyo iron eguqukile ayikwazi ukubopha umoya-mpilo ngendlela evamile. Lapho kunqwabelana i-methemoglobin eningi kakhulu, ukuthuthwa komoya-mpilo kuyaphazamiseka.

I-methylene blue ingasiza ekuhlehliseni lolu shintsho, kodwa kunesinyathelo esiphakathi esibalulekile. Ngaphakathi kwengqamuzana, ukuncishiswa ngokwamakhemikhali okuncike ku-NADPH kuguqula i-methylene blue ibe yi-leucomethylene blue. I-leucomethylene blue ibe isinciphisa i-methemoglobin ngokwamakhemikhali, iyibuyisele ekubeni yi-hemoglobin esebenzayo. Lokhu kulandelana kuchazwe engxenyeni yelebula yomuthi echaza indlela osebenza ngayo.

Kukhona nomzila ohlukile, ovamile wokugcina isimo silungile: i-cytochrome b5 reductase encike ku-NADH yenza ingxenye enkulu yokuncishiswa kwe-methemoglobin okuqhubeka ngokujwayelekile. I-NADH ne-NADPH kungama-molecule ahlukene. Ukuntuleka kwe-G6PD akumane kuvale yonke imizila yokugcina i-hemoglobin isesimweni esifanele. Inkinga iba ngeyokubaluleka okukhethekile lapho ukuchayeka entweni ebangela i-oxidation kwandisa isidingo bese kusetshenziswa i-methylene blue. Umhlahlandlela wethu we-methylene blue yokwelapha i-methemoglobinemia uchaza isimo sokwelashwa.

Imiphumela emibili ye-NADPH enganele

Umfanekiso 1. Imephu eyenziwe lula yokuncika kwezinqubo kwezinye. Imicibisholo exhumanisa amabhokisi emizila ibonisa ukusekelwa kwenqubo; ayilona iqoqo eliphelele lokusabela kwamakhemikhali. Egatsheni lomuthi, i-leucomethylene blue inikeza amandla okunciphisa ngokwamakhemikhali ukuze i-Fe³⁺ iguqulwe ibe yi-Fe²⁺. I-methylene blue ngokwayo ingangeza ukucindezeleka okubangelwa i-oxidation. Lo mfanekiso womqondo uhlanganisa imininingwane yokunquma ukusetshenziswa komuthi nomzila we-PharmGKB oxhunywe lapha.

Izincomo ze-EHA ne-EuroBloodNet ziveza kokubili ukuncipha kokusebenza nomonakalo ongaba khona lapho kunokuntuleka kwe-G6PD:

1. Ukwelashwa kungase kwehluleke. I-NADPH enganele inganciphisa ukuguqulwa kwe-methylene blue ibe sesimweni sayo esinciphisa ngokwamakhemikhali, kushiye i-methemoglobin ingalungisiwe ngokwanele.
2. Ukulimala okubangelwa i-oxidation kungase kwande. I-methylene blue ngokwayo iyinto ebangela i-oxidation. Engqamuzaneni elisengozini, ingaba nesandla ekubhidlizeni amangqamuzana abomvu egazi futhi ingabhebhethekisa i-methemoglobinemia.

Le miphumela ingenzeka ngesikhathi esisodwa: kube namangqamuzana abomvu egazi ambalwa angakalimalanga, nokuthuthwa komoya-mpilo kungasebenzi kahle emangqamuzaneni asele. Yingakho impendulo enganele ekwelashweni ingesona isizathu sokuthi umuntu aqhubeke engeza i-methylene blue ngokwakhe.

Kungani ukuzizwa uphilile kungaqinisekisi ukuphepha

Ngokusho kwe-MedlinePlus Genetics, abantu abaningi abantula i-G6PD abanazo izimpawu futhi abazi ukuthi banalesi simo. Izifo ezithathelwanayo, imithi ethile nezinye izinto ezibangela i-oxidation zingaveza lobu buthakathaka. Nakuba lesi simo sihlotshaniswa nokudluliselwa ngofuzo oluxhumene ne-chromosome engu-X futhi sivame kakhulu kwabesilisa, nabesifazane bangaba nokuntuleka okubalulekile ekwelashweni.

Imininingwane ewusizo yocwaningo ikhombisa umehluko phakathi kwezimo ezingenakho ukucindezeleka nezimo zokucindezeleka. Ocwaningweni lwango-1996 olwenziwa uBozzi, uParisi noStrom, amangqamuzana abomvu egazi labantu ayesanda kuqoqwa futhi enokuntuleka okukhulu kwe-G6PD ayenamazinga e-glutathione esincishisiwe asondele kulawo amangqamuzana avamile. Kodwa ngemva kokuchayeka ku-peroxide ocwaningweni, amangqamuzana antula i-G6PD abonisa ukulahleka okukhulu kwe-glutathione nokuncipha kwekhono lokululama uma kuqhathaniswa namangqamuzana avamile. Lokhu kwakuyizivivinyo zaselabhorethri kumangqamuzana egazi, hhayi ucwaningo olwalusungula umthamo ophephile we-methylene blue. Lokhu okutholakele kuchaza ukuthi kungani isimo sokuqala esibonakala sivamile singaqinisekisi amandla okumelana nokuchayeka.

Okufanele ukwenze ngalolu lwazi

Uma kutholakele ukuthi untula i-G6PD, tshela uchwepheshe wezokwelapha noma usokhemisi ngaphambi kokuba kucatshangelwe ukusetshenziswa kwe-methylene blue. Umhlahlandlela we-CPIC ubeka i-methylene blue esigabeni semithi enobungozi obukhulu. Incazelo yomthengisi ethi umthamo uphansi, noma ukuchayeka kwangaphambili okungazange kube nenkinga, akuyona imvume yokuziba isizathu sokungawusebenzisi lo muthi.

Uma ungaqiniseki ngesimo sakho, letha noma yimiphi imiphumela yangaphambili ye-G6PD kanye nomlando womndeni ohlobene nalokhu engxoxweni. Ukubhidlika kwamangqamuzana abomvu egazi okusanda kwenzeka noma ukumpontshelwa igazi kungathinta indlela umphumela wokuhlolwa kwe-enzyme ochazwa ngayo. Umhlahlandlela waselabhorethri wokuhlolwa kwe-G6PD uchaza ukuhlolwa nemikhawulo yakho; ukubalwa kwamangqamuzana egazi okuvamile kukodwa akuwuphenduli lo mbuzo. Isimo se-G6PD naso siyingxenye eyodwa kuphela yokuhlolwa okubanzi kokuphepha kwe-methylene blue.

Ngemva kokuchayeka, ubuthakathaka obukhulu obuqala ukuvela, ukuphelelwa umoya, ukuphuzi kwesikhumba noma kwamehlo, umchamo omnyama noma inhliziyo eshaya ngokushesha kudinga ukuhlolwa ngokushesha kwezokwelapha. Ukuphefumula kanzima kakhulu, ubuhlungu besifuba, ukuwa noma ukudideka kudinga ukunakekelwa okuphuthumayo. Letha isitsha somkhiqizo noma imininingwane yawo, okuhlanganisa ukugxila kwawo, ubungako kanye nesikhathi sokuchayeka. Ungasebenzisi umbala womchamo kuphela ukunquma ukuthi amangqamuzana abomvu egazi ayabhidlika yini.

Lapho i-methemoglobinemia ivela kumuntu ontula i-G6PD, ukukhetha ukwelashwa kuwumsebenzi wethimba lezimo eziphuthumayo. Izincomo zochwepheshe zixoxa ngezinye izindlela, okuhlanganisa i-ascorbic acid futhi, ezimweni ezinzima, ukumpontshelwa igazi ngokushintshanisa elikhona. Lezi akuzona izindlela zokwelapha ekhaya ezingasetshenziswa ngokushintshana: i-ascorbic acid isebenza kancane, futhi ukuthuthwa komoya-mpilo wesiguli, ukubhidlika kwamangqamuzana abomvu egazi kanye nesimo saso sempilo kunquma ukuthi yiluphi usizo oludingekayo.