Methylene blue nekushomeka kweG6PD: nei njodzi ichikosha

Methylene blue haifaniri kushandiswa muvanhu vane kushomeka kweglucose-6-phosphate dehydrogenase, kunowanzopfupikiswa kuchinzi kushomeka kweG6PD. Mashoko ePROVAYBLUE ekunyorerwa mushonga anoti chikonzero chacho ihemolytic anemia. Izvi zvinoreva kuti masero matsvuku eropa anogona kuparara nokukurumidza kupfuura kutsiviwa kwaanoitwa nemuviri. Kushomeka kumwe chete uku kunogonawo kudzivirira mushonga kuti usashande sezvakarongwa pakurapa methemoglobinemia.

Matambudziko aya akabatana. Methylene blue inoda simba rinopa maerekitironi mukati mesero dzvuku reropa. G6PD inobatsira kuchengetedza simba iroro, iro sero rinodawo kuti rizvidzivirire pakukuvadzwa neoxidation. Kuchena kwechigadzirwa hakugadzirisi kushomeka kweenzayimu mumunhu ari kuchigamuchira.

Zvinoitwa neG6PD musero dzvuku reropa

G6PD ienzayimu iri munzira yepentose phosphate, nzira yekugadzirisa glucose. Chimwe chezvinogadzirwa nenzira iyi iNADPH, morekuru rinopa maerekitironi kune mamwe maitiro emakemikari. Maerekitironi iwayo anobvumira maitiro akawanda ekudzivirira kuti arambe achishanda.

Gwaro reCPIC pamusoro pemishonga nemamiriro emajini eG6PD rinotsanangura kuti nei izvi zvichinyanya kukosha mumasero matsvuku eropa: nzira yepentose phosphate ndiyo tsime ravo reNADPH. G6PD neimwe enzayimu, 6-phosphogluconate dehydrogenase, zvinogadzira NADPH munzira iyoyo. Kudzikira kwekushanda kweG6PD kunoderedza kukwanisa kwesero kupindura kana kuremerwa neoxidation kuchiwedzera.

Imwe nzira iyo NADPH inoshandiswa pakudzivirira nayo ndeyekugadziridza glutathione kuti ishandiswe zvakare. Glutathione reductase inoshandisa NADPH kudzoreredza glutathione iri muchimiro chakagamuchira maerekitironi, iyo inobatsira glutathione peroxidase kubvisa maperoxide. Tsananguro yePharmGKB yenzira iri mumasero matsvuku eropa inobatanidza maitiro aya. Kana simba rekupa maerekitironi risina kukwana, zvinhu zvinokonzera oxidation zvinogona kukuvadza hemoglobin nesero rairi.

Naizvozvo, kushomeka kweG6PD kune chekuita nekukwanisa kutsungirira zvinoremedza sero. Hazvirevi kuti munhu wese ane kushomeka uku ane anemia inoramba iripo kana kuti anonzwa achirwara zuva nezuva.

Nei methylene blue ichitsamira pasimba rimwe chete iri

Hemoglobin inowanzova neiron iri muchimiro cheferrous, Fe²⁺. Oxidation inoichinja kuva muchimiro cheferric, Fe³⁺, ichigadzira methemoglobin. Iron iyoyo yashanduka haichakwanisi kusunga oxygen sezvainofanira. Kana methemoglobin yakawandisa ikaungana, kuendeswa kweoxygen kunokanganisika.

Methylene blue inogona kubatsira kudzosa shanduko iyi, asi danho riri pakati rinokosha. Mukati mesero, kugamuchira maerekitironi kunotsamira paNADPH kunochinja methylene blue kuva leucomethylene blue. Leucomethylene blue inobva yapa maerekitironi kumethemoglobin, ichiidzorera kuva hemoglobin inokwanisa kushanda. Kutevedzana uku kunotsanangurwa muchikamu chemashoko emushonga chinotsanangura mashandiro awo.

Panewo imwe nzira yakasiyana inowanzochengetedza zvinhu zviri mugwara: cytochrome b5 reductase inotsamira paNADH ndiyo inoita chikamu chikuru chekudzoreredza methemoglobin kunowanzoitika. NADH neNADPH mamorekuru akasiyana. Kushomeka kweG6PD hakungodzimi nzira dzese dzekuchengetedza hemoglobin. Dambudziko rinonyanya kukosha kana kusangana nechinhu chinokonzera oxidation kuchiwedzera zvinodiwa nesero uye methylene blue yoshandiswa. Gwaro redu remethylene blue pakurapa methemoglobinemia rinotsanangura mamiriro ekurapa aya.

Migumisiro miviri yeNADPH isina kukwana

Mufananidzo 1. Mepu yakarerutswa yezvinotsamira pane zvimwe. Miseve inobatanidza mabhokisi enzira inoratidza kutsigirwa kwemaitiro; haisi seti yakakwana yemaitiro emakemikari. Mubazi remushonga, leucomethylene blue inopa simba rekuwedzera maerekitironi pakuchinja Fe³⁺ kuva Fe²⁺. Methylene blue pachayo inogona kuwedzera kuremerwa neoxidation. Mufananidzo uyu unotsanangura pfungwa uchibatanidza mashoko ekunyorerwa mushonga nenzira yePharmGKB iri palink.

Kurudziro dzeEHA neEuroBloodNet dzinoratidza zvose kudzikira kwekushanda kwemushonga nekugona kukuvadza kana G6PD ichishomeka:

1. Kurapa kunogona kukundikana. NADPH isina kukwana inogona kuderedza kuchinjwa kwemethylene blue kuva muchimiro chayo chinopa maerekitironi, zvichisiya methemoglobin isina kudzoreredzwa zvakakwana.
2. Kukuvadzwa neoxidation kunogona kuwedzera. Methylene blue pachayo chinhu chinokonzera oxidation. Musero riri nyore kukuvadzwa, inogona kubatsira kukonzera kuparara kwemasero matsvuku eropa uye inogona kuwedzera methemoglobinemia.

Migumisiro iyi inogona kuitika pamwe chete: masero matsvuku eropa asina kukuvara anova mashoma, uye kutakurwa kweoxygen kunoderera mumasero anenge asara. Ndokusaka kusapindura zvakakwana pakurapwa kusiri chikonzero chekuti munhu arambe achiwedzera methylene blue ari oga.

Nei kunzwa uine utano kusingaratidzi kuti zvakachengeteka

Maererano neMedlinePlus Genetics, vanhu vakawanda vane kushomeka kweG6PD havana zviratidzo uye havazivi kuti vanako. Zvirwere zvinokonzerwa neutachiona, mimwe mishonga nezvimwe zvinokonzera oxidation zvinogona kuratidza kusasimba uku. Kunyange zvazvo mamiriro aya achibatana nekugarwa nhaka kuburikidza neX chromosome uye achinyanya kuwanikwa muvarume, vakadzi vanogonawo kuva nekushomeka kwakakosha pakurapwa.

Chimwe chinhu chinobatsira kubva mukuedza chinoratidza musiyano pakati pemamiriro asina kuremerwa neane kuremerwa. Muongororo ya1996 yakaitwa naBozzi, Parisi naStrom, masero matsvuku eropa revanhu achangobva kutorwa, ane kushomeka kwakanyanya kweG6PD, aiva nemwero weglutathione iri muchimiro chakagamuchira maerekitironi waiva pedyo newemasero akajairika. Asi pashure pekuiswa pasi pekuremerwa neperoxide mukuedza, masero ane kushomeka akaratidza kurasikirwa kwakakura neglutathione nekutadza kudzokera pamamiriro awo zvakanaka kana achienzaniswa nemasero akajairika. Uku kwaiva kuedza kwemasero eropa murabhoritari, kwete kuedza kwaisimbisa mwero wakachengeteka wemethylene blue. Zvakawanikwa zvinotsanangura kuti nei mamiriro ekutanga anoita seakajairika asingafaniri kufanotaura kukwanisa kutsungirira panguva yekusangana nechinhu chinoremedza masero.

Zvaungaita nemashoko aya

Kana wakaonekwa kuti une kushomeka kweG6PD, udza nyanzvi yezvekurapa kana wemishonga methylene blue isati yafungwa kushandiswa. Gwaro reCPIC rinoisa methylene blue muboka remishonga ine njodzi yakakura. Kutsanangura kwemutengesi kuti mwero wacho mudiki, kana kuti wakambosangana nayo pasina dambudziko, hakupi mvumo yekuregeredza kurambidzwa kwekuishandisa.

Kana usingazivi mamiriro ako, uya nemigumisiro ipi neipi yawakambowana yekuongororwa kweG6PD uye nhoroondo yemhuri yakakodzera pakukurukurirana. Kuparara kwemasero matsvuku eropa kuchangobva kuitika kana kuwedzerwa ropa kunogona kukanganisa kududzirwa kwemigumisiro yeenzayimu. Gwaro rerabhoritari rekuongororwa kweG6PD rinotsanangura kuongororwa uku nezvakunogumira pakuratidza; kungoverengwa kwemasero eropa kunowanzoitwa hakupinduri mubvunzo mumwe chete. Mamiriro eG6PD anongovawo chikamu chimwe chete chekuongororwa kwakazara kwekuchengeteka kwemethylene blue.

Pashure pekusangana nayo, kushaya simba kwakanyanya kunotanga patsva, kufemereka, kutsvukirira kweyero paganda kana mumaziso, weti yakasviba kana kurova kwemwoyo nokukurumidza zvinoda kuongororwa nachiremba pasina kunonoka. Kutadza kufema kwakanyanya, kurwadziwa muchipfuva, kudonha kana kuvhiringidzika mupfungwa zvinoda rubatsiro rwechimbichimbi. Uya nemudziyo wechigadzirwa kana mashoko acho, kusanganisira kusimba kwemushonga, uwandu nenguva yawakasangana nawo. Usashandisa ruvara rweweti chete kusarudza kana masero matsvuku eropa ari kuparara.

Kana methemoglobinemia ikaitika mumunhu ane kushomeka kweG6PD, kusarudza kurapa ibasa reboka rinopa rubatsiro rwechimbichimbi. Kurudziro dzenyanzvi dzinokurukura dzimwe nzira dzinosanganisira ascorbic acid uye, kana mamiriro acho akanyanya, kubvisa ropa richitsiviwa nerimwe. Idzi hadzisi nzira dzekurapa kumba dzinogona kungotsiviwa neimwe: ascorbic acid inoshanda zvishoma nezvishoma, uye kuendeswa kweoxygen mumurwere, kuparara kwemasero matsvuku eropa nemamiriro ake ekurwara ndizvo zvinoratidza rubatsiro runodiwa.