Methylene blue ndi kuchepa kwa G6PD: chifukwa chake kuopsa kwake kuli kofunika

Methylene blue siyenera kugwiritsidwa ntchito mwa anthu omwe ali ndi kuchepa kwa glucose-6-phosphate dehydrogenase, komwe nthawi zambiri kumatchedwa kuchepa kwa G6PD. Malangizo operekera PROVAYBLUE amatchula kuchepa kwa magazi chifukwa cha kuwonongeka kwa maselo ofiira monga chifukwa chake. Izi zikutanthauza kuti maselo ofiira a magazi angawonongeke mofulumira kuposa momwe thupi limapangira ena m'malo mwawo. Kuchepa komweku kungalepheretsenso mankhwalawa kugwira ntchito monga momwe amayembekezeredwa pothana ndi methemoglobinemia.

Mavutowa ndi ogwirizana. Methylene blue imafuna mphamvu yopereka maelekitironi mkati mwa selo lofiira la magazi. G6PD imathandiza kuti mphamvu imeneyi ikhalepo, ndipo selo limayifunanso kuti lidziteteze ku kuwonongeka chifukwa cha oksideshoni. Kuyera kwa chinthu sikuthetsa kuchepa kwa enzyme mwa munthu amene akulandira chinthucho.

Zomwe G6PD imachita m'selo lofiira la magazi

G6PD ndi enzyme m'njira ya pentose phosphate, yomwe ndi njira yosinthira glucose. Chimodzi mwa zinthu zomwe zimapangidwa m'njirayi ndi NADPH, molekyulu yomwe imapereka maelekitironi ku zochitika zina za makemikolo. Maelekitironi amenewo amalola njira zingapo zotetezera kupitiriza kugwira ntchito.

Malangizo a CPIC okhudza mankhwala ndi mtundu wa majini a G6PD amafotokoza chifukwa chake izi zili zofunika kwambiri m'maselo ofiira a magazi: gwero la NADPH lomwe maselowa ali nalo ndi njira ya pentose phosphate. G6PD ndi enzyme ina, 6-phosphogluconate dehydrogenase, zimapanga NADPH m'njirayi. Kuchepa kwa ntchito ya G6PD kumachepetsa mphamvu ya selo yothana ndi mavuto pamene kupsinjika chifukwa cha oksideshoni kukuwonjezeka.

Ntchito imodzi yotetezera ya NADPH ndi kubwezeretsa glutathione kuti igwiritsidwenso ntchito. Glutathione reductase imagwiritsa ntchito NADPH kubwezeretsa glutathione yokhala ndi maelekitironi owonjezera, yomwe imathandiza glutathione peroxidase kuchotsa ma peroxide. Kufotokozera kwa PharmGKB kwa njira ya m'maselo ofiira a magazi kumagwirizanitsa zochitika za makemikolozi. Popanda mphamvu yokwanira yopereka maelekitironi, zinthu zoyambitsa oksideshoni zingawononge hemoglobin komanso selo lomwe ili mkati mwake.

Choncho, kuchepa kwa G6PD kumakhudza mphamvu yothana ndi zinthu zovutitsa selo. Sikutanthauza kuti munthu aliyense amene ali ndi vutoli amakhala ndi kuchepa kwa magazi kosalekeza kapena amadwala tsiku lililonse.

Chifukwa chake methylene blue imadalira mphamvu yomweyi

Nthawi zambiri hemoglobin imakhala ndi chitsulo cha mtundu wa ferrous, Fe²⁺. Oksideshoni imachisintha kukhala cha mtundu wa ferric, Fe³⁺, ndipo zimapanga methemoglobin. Chitsulo chosinthikacho sichingamangirire mpweya wa oxygen monga mwachizolowezi. Chikachuluka kwambiri, kuperekedwa kwa oxygen kumasokonekera.

Methylene blue ingathandize kubweza kusintha kumeneku, koma pali gawo lapakati lofunika. Mkati mwa selo, kulandira maelekitironi modalira NADPH kumasintha methylene blue kukhala leucomethylene blue. Kenako leucomethylene blue imapereka maelekitironi ku methemoglobin kuti ibwerere ku hemoglobin yogwira ntchito. Njira yotsatizanayi ikufotokozedwa mu kufotokozera kwa momwe mankhwalawa amagwirira ntchito pa chizindikiro chake.

Palinso njira ina yosiyana yomwe imagwira ntchito yosamalira selo mwachizolowezi: cytochrome b5 reductase yodalira NADH ndiyo imachita gawo lalikulu la kubwezeretsa methemoglobin mwachizolowezi. NADH ndi NADPH ndi mamolekyulu osiyana. Kuchepa kwa G6PD sikungotseka njira zonse zosamalira hemoglobin. Vutoli limakhala lofunika kwambiri pamene kukhudzidwa ndi chinthu choyambitsa oksideshoni kumaonjezera zofunikira ndipo methylene blue ikuperekedwa. Kalozera wathu wa methylene blue pa methemoglobinemia amafotokoza mmene izi zimakhalira pa chithandizo cha odwala.

Zotsatira ziwiri za NADPH yosakwanira

Chithunzi 1. Chithunzi chosavuta chosonyeza kudalirana kwa njira. Mivi yolumikiza mabokosi a njira ikusonyeza kuthandiza kwa zochitika; si mndandanda wathunthu wa zochitika za makemikolo. M'nthambi ya mankhwala, leucomethylene blue imapereka mphamvu yopereka maelekitironi kuti Fe³⁺ isinthe kukhala Fe²⁺. Methylene blue yokha ingaonjezere kupsinjika chifukwa cha oksideshoni. Chithunzi chofotokozerachi chikuphatikiza malangizo operekera mankhwala ndi njira ya PharmGKB yolumikizidwa.

Malangizo a EHA ndi EuroBloodNet amatchula kuchepa kwa kugwira ntchito komanso kuvulazidwa komwe kungachitike chifukwa cha kuchepa kwa G6PD:

1. Chithandizo chingalephere. NADPH yosakwanira ingachepetse kusintha kwa methylene blue kukhala mtundu wake wopereka maelekitironi, n'kusiya methemoglobin isanabwezeretsedwe mokwanira.
2. Kuvulazidwa chifukwa cha oksideshoni kungawonjezeke. Methylene blue yokha ndi chinthu choyambitsa oksideshoni. M'selo losatetezeka mokwanira, ingathandize kuwononga maselo ofiira a magazi ndipo ingakulitse methemoglobinemia.

Zotsatirazi zingachitike pamodzi: maselo ofiira a magazi osawonongeka amachepa, ndipo maselo otsalawo amanyamula oxygen mosagwira ntchito bwino. N'chifukwa chake kusayankha mokwanira kwa chithandizo si chifukwa choti munthu azingowonjezera methylene blue payekha.

Chifukwa chake kumva bwino sikutsimikizira chitetezo

Malinga ndi MedlinePlus Genetics, anthu ambiri omwe ali ndi kuchepa kwa G6PD alibe zizindikiro ndipo sadziwa kuti ali ndi vutoli. Matenda oyambitsidwa ndi tizilombo, mankhwala ena komanso zinthu zina zoyambitsa oksideshoni zingasonyeze kusatetezeka kumeneku. Ngakhale vutoli limagwirizana ndi kutengera majini kudzera mu kromosomu ya X ndipo limapezeka kwambiri mwa amuna, akazi nawonso angakhale ndi kuchepa kofunika pa chithandizo cha odwala.

Mfundo ina yothandiza ya kafukufuku ikusonyeza kusiyana pakati pa mkhalidwe wopanda kupsinjika ndi mkhalidwe wa kupsinjika. Mu kafukufuku wa 1996 wa Bozzi, Parisi ndi Strom, maselo ofiira a magazi a anthu omwe anali atangotengedwa kumene, okhala ndi kuchepa kwambiri kwa G6PD, anali ndi milingo ya glutathione yokhala ndi maelekitironi owonjezera yoyandikira ya maselo abwinobwino. Komabe, atayikidwa pa mayeso okhudzidwa ndi peroxide, maselo okhala ndi kuchepako anataya glutathione kwambiri ndipo sanathe kubwezeretsa milingo yake bwino poyerekeza ndi maselo abwinobwino. Awa anali mayeso a mu labotale pa maselo a magazi, osati mayeso otsimikizira mlingo wotetezeka wa methylene blue. Zotsatirazi zikufotokoza chifukwa chake mkhalidwe wooneka wabwinobwino poyambira sungalosere mphamvu yothana ndi kupsinjika pamene munthu wakhudzidwa ndi chinthu.

Zomwe mungachite ndi mfundozi

Ngati mwapezeka kuti muli ndi kuchepa kwa G6PD, uzani dokotala kapena katswiri wa mankhwala musanayambe kuganizira kugwiritsa ntchito methylene blue. Malangizo a CPIC amaika methylene blue m'gulu la mankhwala a kuopsa kwakukulu. Kufotokozera kwa wogulitsa kuti mlingo wake ndi wochepa, kapena kuti munakhudzidwapo kale popanda vuto, sikupereka chilolezo chonyalanyaza kuletsa kugwiritsidwa ntchito kwake.

Ngati simukudziwa mmene G6PD yanu ilili, bweretsani zotsatira zilizonse za mayeso a G6PD am'mbuyomu komanso mbiri yoyenera ya banja pokambirana ndi akatswiri a zaumoyo. Kuwonongeka kwa maselo ofiira a magazi kapena kuikidwa magazi posachedwapa kungakhudze kutanthauzira zotsatira za mayeso a enzyme. Kalozera wa labotale wa mayeso a G6PD amafotokoza mayesowo ndi malire ake; kuwerengera maselo a magazi mwachizolowezi kokha sikuyankha funso lomwelo. Mmene G6PD ilili ndi gawo limodzi lokha la kuwunika kwakukulu kwa chitetezo cha methylene blue.

Mukakhudzidwa ndi methylene blue, kufooka kwakukulu komwe kwayamba kumene, kupuma movutikira, chikasu cha khungu kapena maso, mkodzo wakuda kapena kugunda kwa mtima mofulumira zimafuna kuwunikidwa mwamsanga ndi akatswiri a zaumoyo. Kupuma movutikira kwambiri, kupweteka pachifuwa, kukomoka kapena kusokonezeka maganizo zimafuna chithandizo chadzidzidzi. Bweretsani chidebe cha chinthucho kapena mfundo zake, kuphatikizapo kuchuluka kwake pa muyeso, kuchuluka komwe munakhudzidwa nako ndi nthawi yomwe munakhudzidwa nacho. Musagwiritse ntchito mtundu wa mkodzo wokha kudziwa ngati maselo ofiira a magazi akuwonongeka.

Methemoglobinemia ikachitika mwa munthu amene ali ndi kuchepa kwa G6PD, kusankha chithandizo ndi ntchito ya gulu lachipatala lothandiza pa zadzidzidzi. Malangizo a akatswiri amakambirana njira zina, kuphatikizapo ascorbic acid komanso, pa mikhalidwe yoopsa, kuchotsa magazi n'kuika ena m'malo mwake. Izi si njira zapakhomo zomwe zingasinthanitsidwe: ascorbic acid imagwira ntchito pang'onopang'ono, ndipo momwe oxygen ikuperekedwera m'thupi la wodwala, kuwonongeka kwa maselo ofiira a magazi komanso mkhalidwe wa wodwalayo zimatsimikizira thandizo lofunika.