I-methylene blue nokunqongophala kwe-G6PD: kutheni umngcipheko ubalulekile

I-methylene blue ayifanele isetyenziswe kubantu abanokunqongophala kwe-glucose-6-phosphate dehydrogenase, okudla ngokufinyezwa kuthiwe kukunqongophala kwe-G6PD. Inkcazelo yokumiselwa kwe-PROVAYBLUE ichaza i-hemolytic anemia njengesizathu. Oku kuthetha ukuba iiseli ezibomvu zegazi zinokuqhekeka ngokukhawuleza kunokuba umzimba uzibuyisela. Oku kunqongophala kunokuthintela iyeza ekusebenzeni ngendlela elindelekileyo ekunyangeni i-methemoglobinemia.

Ezi ngxaki zinxibelelene. I-methylene blue ifuna amandla okunciphisa ngaphakathi kwiseli ebomvu yegazi. I-G6PD inceda ukugcina la mandla efumaneka, kwaye iseli ikwawadinga ukuze ikhuseleke kumonakalo obangelwa yi-oxidation. Ukucoceka kwemveliso akulungisi ukunqongophala kwe-enzyme kumntu oyifumanayo.

Yintoni eyenziwa yi-G6PD kwiseli ebomvu yegazi

I-G6PD yi-enzyme ekwindlela ye-pentose phosphate, indlela yokulungisa i-glucose. Enye yezinto eziveliswa yile ndlela yi-NADPH, imolekyuli enikela ngee-electron kwezinye iintshukumo zekhemikhali. Ezo electron zivumela iinkqubo ezininzi zokukhusela ukuba ziqhubeke zisebenza.

Isikhokelo se-CPIC esimalunga namayeza kunye nohlobo lwemfuza ye-G6PD sicacisa ukuba kutheni oku kubalulekile ngokukhethekileyo kwiiseli ezibomvu zegazi: umthombo we-NADPH ezifumaneka kuwo yindlela ye-pentose phosphate. I-G6PD kunye nenye i-enzyme, i-6-phosphogluconate dehydrogenase, zivelisa i-NADPH kuloo ndlela. Ukuncipha kokusebenza kwe-G6PD kunciphisa amandla eseli okuphendula xa uxinzelelo lwe-oxidation lusanda.

Enye indlela yokukhusela esebenzisa i-NADPH kukubuyisela i-glutathione ukuze iphinde isetyenziswe. I-glutathione reductase isebenzisa i-NADPH ukubuyisela i-glutathione kwimo encitshisiweyo, nto leyo enceda i-glutathione peroxidase ukususa ii-peroxide. Inkcazelo ye-PharmGKB yendlela ekwiiseli ezibomvu zegazi idibanisa ezi ntshukumo zekhemikhali. Ngaphandle kwamandla aneleyo okunciphisa, ii-oxidant zinokonakalisa i-hemoglobin kunye neseli eyijikelezileyo.

Ngoko ke, ukunqongophala kwe-G6PD kuchaphazela amandla okumelana nomceli mngeni. Akuthethi ukuba wonke umntu onako uhlala ene-anemia okanye eziva egula yonke imihla.

Kutheni i-methylene blue ixhomekeke kwakula mandla

I-hemoglobin idla ngokuba nentsimbi ekwimo ye-ferrous, Fe²⁺. I-oxidation iyitshintsha ibe kwimo ye-ferric, Fe³⁺, ivelise i-methemoglobin. Loo ntsimbi itshintshileyo ayikwazi ukubophelela ioksijini ngendlela eqhelekileyo. Xa kuqokelelana eninzi kakhulu, ukuhanjiswa kweoksijini kuyaphazamiseka.

I-methylene blue inokunceda ukubuyisela umva olu tshintsho, kodwa inyathelo eliphakathi libalulekile. Ngaphakathi kwiseli, ukuncitshiswa okuxhomekeke kwi-NADPH kuguqula i-methylene blue ibe yi-leucomethylene blue. I-leucomethylene blue ke inciphisa i-methemoglobin ukuze ibuyele kwi-hemoglobin esebenzayo. Olu landelelwano luchazwa kwinkcazelo yendlela elisebenza ngayo iyeza ekwileyibhile yalo.

Kukwakho nenye indlela eyahlukileyo, eqhelekileyo yokugcina ukusebenza kweseli: i-cytochrome b5 reductase exhomekeke kwi-NADH yenza uninzi lokuncitshiswa kwe-methemoglobin okuqhubekayo ngokuqhelekileyo. I-NADH ne-NADPH ziimolekyuli ezahlukileyo. Ukunqongophala kwe-G6PD akuvele kuvale zonke iindlela zokugcina i-hemoglobin ikwimo efanelekileyo. Ingxaki ibaluleka ngokukhethekileyo xa ukuchanabeka kwi-oxidant kwandisa imfuno kwaye kusetyenziswa i-methylene blue. Isikhokelo sethu se-methylene blue ekunyangeni i-methemoglobinemia sicacisa imeko yonyango.

Iziphumo ezimbini zokungoneli kwe-NADPH

Umzobo 1. Imephu elula yokuxhomekeka kweenkqubo. Iintolo ezidibanisa iibhokisi zeendlela zibonisa ukuxhaswa kwenkqubo; azibonisi zonke iintshukumo zekhemikhali. Kwisebe leyeza, i-leucomethylene blue inika amandla okunciphisa ukuze i-Fe³⁺ iguqulwe ibe yi-Fe²⁺. I-methylene blue ngokwayo inokongeza uxinzelelo lwe-oxidation. Lo mzobo wengcamango udibanisa inkcazelo yokumiselwa kweyeza kunye nendlela ye-PharmGKB edityaniswe ngekhonkco.

Iingcebiso ze-EHA ne-EuroBloodNet zichaza kokubini ukuncipha kokusebenza kunye nomonakalo onokubakho xa kukho ukunqongophala kwe-G6PD:

1. Unyango lunokusilela. Ukungoneli kwe-NADPH kunokuthintela ukuguqulwa kwe-methylene blue ibe kwimo yayo enciphisayo, kushiye i-methemoglobin ingalungiswanga ngokwaneleyo.
2. Umonakalo obangelwa yi-oxidation unokwanda. I-methylene blue ngokwayo yi-oxidant. Kwiseli esesichengeni, inokuba negalelo kwi-hemolysis kwaye inokuyenza mandundu i-methemoglobinemia.

Ezi ziphumo zinokwenzeka ngaxeshanye: iiseli ezibomvu zegazi ezingonakalanga zibe mbalwa, kwaye ukuhanjiswa kweoksijini kungasebenzi kakuhle kwiiseli eziseleyo. Yiyo loo nto ukungaphenduli ngokwaneleyo kunyango kungesosizathu sokuba umntu aqhubeke ezongezela i-methylene blue ngokwakhe.

Kutheni ukuziva uphilile kungabonisi ukhuseleko

Ngokutsho kwe-MedlinePlus Genetics, abantu abaninzi abanokunqongophala kwe-G6PD abanazo iimpawu kwaye abazi ukuba banako. Usulelo, amayeza athile nezinye izinto ezibangela uxinzelelo lwe-oxidation zinokubonakalisa obu buthathaka. Nangona le meko inxulunyaniswa nelifa lemfuza elinxulumene nekhromozom X kwaye ixhaphake ngakumbi emadodeni, nabasetyhini banokuba nokunqongophala okubalulekileyo ngokwezonyango.

Inkcukacha eluncedo evela kuvavanyo ibonisa umahluko phakathi kweemeko zokuphumla kunye noxinzelelo. Kuphononongo luka-1996 olwenziwa nguBozzi, uParisi noStrom, iiseli ezibomvu zegazi zabantu ezaziqokelelwe kutshanje, ezinokunqongophala okukhulu kwe-G6PD, zazinamanqanaba e-glutathione ekwimo encitshisiweyo asondeleyo kulawo eeseli eziqhelekileyo. Noko ke, emva kokuchanatywa kwi-peroxide kuvavanyo, iiseli ezinokunqongophala zabonisa ukulahleka okukhulu kwe-glutathione kunye nokungakwazi ukubuyela kwimeko yazo ngokufanelekileyo xa zithelekiswa neeseli eziqhelekileyo. Olu yayiluvavanyo lwaselebhu kwiiseli zegazi, hayi ulingo olumisela idosi ekhuselekileyo ye-methylene blue. Esi siphumo sicacisa ukuba kutheni imeko yokuqala ebonakala iqhelekile ingenakuqinisekisa amandla okumelana noxinzelelo ngexesha lokuchanabeka.

Into omawuyenze ngale nkcazelo

Ukuba kufunyaniswe ukuba unokunqongophala kwe-G6PD, xelela ugqirha okanye usokhemesti ngaphambi kokuba kuqwalaselwe i-methylene blue. Isikhokelo se-CPIC sibeka i-methylene blue kudidi lwamayeza anomngcipheko ophezulu. Inkcazelo yomthengisi yokuba idosi iphantsi, okanye ukuchanabeka kwangaphambili okungakhange kubangele ngxaki, ayisosizathu sokungawuthobeli umqathango wokungayisebenzisi.

Ukuba awuqinisekanga ngemeko yakho, yiza nazo naziphi na iziphumo zangaphambili zovavanyo lwe-G6PD kunye nembali efanelekileyo yosapho kwingxoxo. I-hemolysis yakutshanje okanye utofelo-gazi lunokuchaphazela ukutolikwa kwesiphumo sovavanyo lwe-enzyme. Isikhokelo saselebhu sovavanyo lwe-G6PD sicacisa uvavanyo kunye nemida yalo; ukubalwa kweeseli zegazi okuqhelekileyo kukodwa akuwuphenduli lo mbuzo. Imeko ye-G6PD ikwayinxalenye enye kuphela yovavanyo olubanzi lokhuseleko lwe-methylene blue.

Emva kokuchanabeka, ubuthathaka obukhulu obutsha, ukuphelelwa ngumoya, ijondisi, umchamo omnyama okanye ukubetha kwentliziyo ngokukhawuleza kufuna uvavanyo lwezonyango olukhawulezileyo. Ubunzima obukhulu bokuphefumla, iintlungu zesifuba, ukuwa okanye ukudideka kufuna unyango olungxamisekileyo. Yiza nesikhongozeli semveliso okanye iinkcukacha zayo, kuquka ukuxinana kwayo, isixa kunye nexesha lokuchanabeka. Musa ukusebenzisa umbala womchamo kuphela ukugqiba ukuba iiseli ezibomvu zegazi ziyaqhekeka na.

Xa i-methemoglobinemia isenzeka kumntu onokunqongophala kwe-G6PD, ukukhetha unyango luxanduva lweqela lezonyango olungxamisekileyo. Iingcebiso zeengcali zixoxa ngezinye iindlela eziquka i-ascorbic acid kwaye, kwiimeko ezinzima, utofelo-gazi lokutshintsha igazi. Ezi ayizozisombululo zasekhaya ezinokutshintshana: i-ascorbic acid isebenza kancinci ngakumbi, kwaye ukuhanjiswa kweoksijini kwisigulana, i-hemolysis kunye nemeko yaso yezonyango zimisela ukuba yeyiphi inkxaso efunekayo.