Ðe oxygen ƒe kuxi aɖe le ʋua mea? Mina míasrɔ̃ nu tso methemoglobinemia ŋu!

Èkpɔ ame aɖe ƒe ŋutigbalẽ kple nuyiwo ƒe amadede trɔ zi ɖeka le blɔ wɔnuku aɖe kpɔa? Alo èse be wodzia vidzĩ aɖewo blɔ viea? Susu ɖeka si tae wòle alea ɖo ate ŋu anye ʋumenugbagbeviɣidɔ si mebɔ kura o, gake ate ŋu agblẽ nu le ame ŋu vevie. Egbea míele nu ƒom tso nɔnɔme mawo dometɔ ɖeka ŋu , si nye methemoglobinemia . Ame aɖewo hã yɔnɛ be "blue baby syndrome."

Nuka tututue nye methemoglobinemia?

Kpuie ko la, methemoglobinemia nye nɔnɔme si me ʋumenugbagbevi dzĩ siwo le míaƒe ʋu me mete ŋu tsɔa ɔksidzin yia lãmenugbagbevi kple lãmenugbagbevi bubuwo le ŋutilãa me o. Bu eŋu kpɔ, ʋumenugbagbevi dzĩwoe kpena ɖe ame ŋu wòtsɔa oxygen toa míaƒe ŋutilã katã me, alo? Protein tɔxɛ aɖe si woyɔna be hemoglobin le ʋumenugbagbevi dzĩ siawo me . Ele abe bɔs ene, eye ʋumenugbagbevi dzĩ sia tsɔa ɔksidzin yia ŋutilã bliboa me.

Ke hã, le nɔnɔme sia si woyɔna be ``Methemoglobinemia`` me la, nusi dzɔna enye be míaƒe ``Hemoglobin`` si tsɔa oxygen la trɔna eye wòzua nu bubu si woyɔna be ``Methemoglobin.`` Kuxia enye be, ``Methemoglobin`` sia mateŋu atsɔ oxygen o. Ele abe bɔs si tsɔa oxygen gblẽna eye mete ŋu tsɔa mɔzɔlawo o ene.

Woate ŋu anyi nɔnɔme sia ƒe dome (le domenyiŋusẽfianu me) le ame aɖewo me. Gake zi geɖe la , atike aɖewo siwo míezãna, modzakaɖetikewo, alo atike aɖewo zazã gbɔe wòtsona . Ate ŋu ade ame ƒe agbe afɔku me ɣeaɖewoɣi, vevietɔ le vidzĩ siwo wodzi kple dɔlélea ƒe nɔnɔme sesẽ aɖe kple amesiwo zãa atike vɔ̃ɖiwo gome. Ke hã, zi geɖe la, ɖɔktawo ateŋu aŋlɔ atike na amewo be woatsɔ aɖe ``Methemoglobin`` ƒe agbɔsɔsɔ dzi akpɔtɔ eye woaɖe dzesiawo ɖa.

Aleke esia kpɔa ŋusẽ ɖe nye ŋutilã dzii?

Ame geɖe siwo ŋu methemoglobinemia le la doa go dɔléle aɖe si woyɔna be cyanosis . Cyanosis nye ne wò asibidɛwo, aɖe, nuyiwo, kple ŋutigbalẽ trɔ zu blɔ alo aŋutiɖiɖi. Esia dzɔna ne oxygen mesɔ gbɔ na wò ʋu o. Abe alesi megblɔe va yi ene la, ʋumenugbagbevi dzĩwoe tsɔa ɔksidzin yia ŋutilã bliboa me le dzɔdzɔme nu. Protein aɖe si woyɔna be hemoglobin kpena ɖe esia ŋu.

Le methemoglobinemia me la, ``Methemoglobinemia`` dzɔna ne ``Hemoglobin`` va zu ``Methemoglobin``, le domenyiŋusẽfianu ƒe tɔtrɔ alo gotagome nu bubu aɖe ta. Esi ``Methemoglobin`` mateŋu atsɔ oxygen o ta la, oxygen ƒe agbɔsɔsɔ si le ʋu me dzi ɖena kpɔtɔna eye nèxɔa ``Cyanosis``.

Nukae nye Methemoglobinemia ƒe dzesiwo?

Dzesiawo ate ŋu ato vovo tso ame ɖeka gbɔ yi bubu gbɔ, eye wòanɔ te ɖe dɔléle si ƒomevi le ŋuwò hã dzi. Mina míalé ŋku ɖe alesi dzesi siawo dzena zi geɖee ŋu.

Methemoglobinemia si Woxɔ ƒe Dzesiwo

Ame geɖe va xɔa nɔnɔme sia ne wozã vevesesenutsitike aɖewo, atikewo, alo aɖinuwo zazã vɔ . Woyɔa esia be methemoglobinemia si woxɔ. Dzesi siwo ate ŋu ado mo ɖa dometɔ aɖewoe nye:

• Ŋutilã si le ɣie (si ƒe amadede nye blɔ) .
• Sese le eɖokui me be ɖeɖi te ye ŋu ŋutɔ (ɖeɖiteameŋu) .
• Gbɔdzɔgbɔdzɔ
• Taɖuame

Ɣeaɖewoɣi la, amesiwo ŋu methemoglobinemia si woxɔ la ate ŋu adze dzesi sesẽwo gɔme si ahiã be woakpɔ wo gbɔ enumake . Esiawo dometɔ aɖewoe nye:

• Aɖuɖɔtoe kple ʋuʋudedi.
• Alɔ̃dɔdɔ fũ akpa, nuƒoƒo ƒuƒlu, kple nuwɔwɔ blewu: Esiawo ate ŋu anye lãmekawo ƒe blanuiléle ƒe dzesiwo .
• Alɔ̃madɔmadɔ alo ŋutilã ƒe ʋuʋu si dzi womate ŋu aɖu o (abe alɔ̃madɔmadɔ ene): Esiawo nye alɔ̃madɔmadɔ ƒe dzesiwo .
• Gbɔgbɔ kabakaba, dzi ƒe tsotso ƒe dzidziɖedzi, kple tɔtɔ: Esiawo nye nɔnɔme aɖe si woyɔna be metabolic acidosis ƒe dzesiwo .

Vevietɔ: Ne ame aɖe si le gbɔwò ɖe asrãdɔ ƒe dzesi siawo dometɔ aɖe fia, `(Metabolic Acidosis)` alo `(Central Nervous System Depression)` la, ele be nàƒo ka na 1990 enumake eye nàyɔ dɔnɔdzikpɔʋu .

Methemoglobinemia si Wodzina ƒe Dzesiwo

Methemoglobinemia si wodzina nye dɔléle si mebɔ kura o. Ame ʋɛ aɖewo koe wokpɔ dɔlélea le le xexeame katã. Ðɔktawo nɔ te ɖe nyatakaka ma dzi mae ɖe hatsotso vevi etɔ̃ me: Ƒomevi 1, Ƒomevi 2, kple Hemoglobin M dɔléle (HbM) .

• Zi geɖe la, cyanosis (ŋutilã blɔ) nɔa amesiwo ŋu hemoglobin M dɔléle (HbM) le ŋu, gake zi geɖe la, woƒe lãme sẽna.
• Cyanosis le amesiwo ŋu Methemoglobinemia Ƒomevi 1 (MetHb Ƒomevi 1) le hã ŋu, gake lãmesẽkuxi gã bubuwo mebɔ o.
• Gake vidzĩ siwo wodzi kple Type 2 methemoglobinemia (Type 2 MetHb) ate ŋu axɔ ahɔhɔ̃mekawo ƒe kuxi sesẽwo hafi woaxɔ abe ɣleti 9 ene. Nublanuitɔe la, vidzĩ siawo menɔa agbe didi o.

Nukatae methemoglobinemia dzɔna?

Nɔnɔme sia ate ŋu atso dome, alo abe alesi megblɔe va yi ene la, ate ŋu atso atike aɖewo, atikewo, alo atike siwo me aɖi le hã gbɔ emegbe.

Nusiwo Hea Methemoglobinemia si Xɔa Gbɔ vɛ

• Vevesesenutsitikewo abe benzocaine kple lidocaine (vevietɔ esiwo le gel kple atike siwo wotsɔna ƒua gbe me) kple dɔlékuiwutike si nye dapsone ate ŋu ahe esia vɛ.
• Nitrates si wozãna le atike aɖewo me, Nitrites, si ate ŋu anɔ nuɖuɖutakpɔnu kple vudo me tsi aɖewo me, kple gbewutike aɖewo siwo wozãna le agbledede me ƒe amedzidzedze hã nye nusiwo gbɔ wòtsona.
• Ame siwo zãa modzakaɖetikewo abe amyl nitrite (si wogayɔna be poppers), nitrous oxide (nukoko ya), kple atike aɖewo siwo naa alɔ̃dɔdɔ siwo wotsaka kple atikewo abe cocaine ene la le afɔku me wu be woakpɔ atikewɔwɔ me kuxi siwo ate ŋu ade woƒe agbe afɔku me le methemoglobin ƒe agbɔsɔsɔ si sɔ gbɔ ŋutɔ ta.

Nusiwo Hea Methemoglobinemia si Wodzina vɛ

Domenyiŋusẽfianu ƒe tɔtrɔ vovovo eve gbɔe methemoglobinemia si wodzina (Congenital MetHb) tsona.

• Le ƒomevi 1 kple 2 me la, domenyiŋusẽfianu si nye CYB5R ƒe tɔtrɔ gblẽa ʋumenugbagbevi dzĩwo ƒe dadasɔ si le hemoglobin kple methemoglobin dome.
• Hemoglobin M dɔléle (HbM) tsoa Hemoglobin M domenyiŋusẽfianu geɖe ƒe tɔtrɔ gbɔ.

Methemoglobinemia si wodzina nye autosomal recessive dɔléle . Kpuie ko la, hafi ɖevi nate ŋu axɔ dɔlélea la, ele be dzila evea siaa nanye domenyiŋusẽfianu ƒe tɔtrɔ la tsɔlawo eye ɖevia nanyi tɔtrɔa ƒe dome tso wo ame evea siaa gbɔ.

Hemoglobin M dɔléle (HbM) nye dɔléle si xɔa ame ɖokui dzi . Esia fia be ɖevi ate ŋu axɔ dɔlélea ne wonyi domenyiŋusẽfianu ƒe tɔtrɔ tso dzila ɖeka ko gbɔ gɔ̃ hã.

Aleke ɖɔktawo dea dzesi esiae?

Ðɔktawo dea dzesi nɔnɔme sia to wò atikewɔwɔ ŋutinya bliboa xɔxɔ kple ŋutilãmedodokpɔ me . Ne methemoglobinemia si woxɔ ƒe dzesiwo le ame aɖe ŋu la, ate ŋu abia nya tso atike ɖesiaɖe si wòzãna alo atike siwo me aɖi le siwo ate ŋu ado goe ŋu.

Dodokpɔ kawoe wowɔna tsɔ dea dzesi esia?

Zi geɖe la, wowɔa dodokpɔ siawo:

• Ʋudodokpɔ: Zi geɖe la, ʋu si woɖena tso amesiwo ŋu dɔléle sia le ƒe ʋukawo me ƒe amadede nyea aŋutiɖiɖi. Amadede sia si ƒe amadede nye aŋutiɖiɖi la fia be ʋukawo metsɔa ɔksidzin le ʋua me nyuie o.
• Methemoglobin (MetHb) ƒe agbɔsɔsɔ dodokpɔ: Esia dzidzea methemoglobin agbɔsɔsɔme si le wò ʋu me.
• CYB5R enzyme ƒe dɔwɔwɔ: Ne enzyme sia ƒe dɔwɔwɔ le sue wu alesi wòle dzɔdzɔmee la, ke enye methemoglobinemia si wodzina ƒe dzesi.
• Domenyiŋusẽfianu: Ne èsusu be methemoglobinemia si wodzi na ye le ye ŋu la, woalé ŋku ɖe wò DNA ŋu be domenyiŋusẽfianu ƒe tɔtrɔwo le ye ŋu hã atsɔ aɖo kpe dɔlélea dzi. Esia ate ŋu akpe ɖe ame ŋu hã be woanya dɔlélea ƒomevi.
• Hemoglobin ƒe Elektrikŋusẽ:Esia nye mɔnu si wozãna tsɔ doa ``Hemoglobin`` kpɔ le ʋumenugbagbevi dzĩwo me. Woateŋu azã dodokpɔ sia atsɔ anya hemoglobin M dɔléle ``(HbM).``

Aleke wowɔa nu ɖe ​​esia ŋui?

Atikewɔwɔ ƒe tiatia vovovowo le alesi methemoglobinemia ƒomevi si le ŋuwò nu. Le kpɔɖeŋu me, atikewɔwɔ na vidzĩ yeye si wodzi kple ƒomevi 2 la to vovo kura na atikewɔwɔ na amesi xɔ dɔlélea to atike alo atike vɔ̃ɖi aɖe zazã me.

• Ðewohĩ mahiã be woawɔ atike na amesiwo ŋu Methemoglobinemia Ƒomevi 1 alo Hemoglobin M dɔléle le o. Ne ehiã la, ɖɔktawo ate ŋu azã atike siawo atsɔ aɖe Methemoglobin ƒe agbɔsɔsɔ dzi akpɔtɔ:
• Methylene Blue: Esiae nye atike vevitɔ si wozãna na methemoglobinemia.
• Vitamin C kple vitamin B2.

Atikewɔwɔ na Methemoglobinemia si Woxɔ

Le nɔnɔmea nu la, methemoglobinemia si woxɔ ate ŋu anye atikewɔwɔ kpata. Le nɔnɔme mawo me la, ate ŋu ahiã be woade tsi kple oxygen si wodona ɖe ʋuka me . Zi geɖe la, wonaa methylene blue na amesiwo ŋu methemoglobinemia si woxɔ.

Nukawoe nye kuxi siwo dona tso atikewɔwɔ me?

Ne wona methylene blue atike na amesi ŋu G6PD ƒe Dɔmawɔmawɔ nyuie (si hã nye domenyiŋusẽfianu ƒe dɔléle) le atraɖii la, nɔnɔme aɖe si woyɔna be hemolysis ate ŋu adzɔ. Ʋumenugbagbeviɣidɔ nye wò ʋumenugbagbevi dzĩwo ƒe gbagbã kaba alo esi mehiã o.

Ðe mate ŋu aɖe afɔku si le methemoglobinemia xɔxɔ me dzi akpɔtɔa?

• Ele be amesiwo nyi nɔnɔme sia ƒe domenyiŋusẽfianu ƒe tɔtrɔ ƒe dome naƒo asa na atike siwo me aɖi le (e.g. nudzodzoeviwutikewo), atike aɖewo, kple atike geɖe siwo naa alɔ̃dɔdɔ le nutoa me siwo ate ŋu ana methemoglobin ƒe agbɔsɔsɔ nadzi ɖe edzi. Ne dɔléle sia le ŋuwò la, bia wò ɖɔkta tso nusiwo ŋu wò nulɔdɔ ate ŋu anɔ ŋu.
• Ame siwo zãa atikewo abe amyl nitrite alo cocaine ene tsɔa wo ɖokui dea afɔku me be woaxɔ methemoglobinemia. Ne èzãa atike siawo eye nèhiã kpekpeɖeŋu be nàdzudzɔ la, ƒo nu kple ɖɔkta tso atikewɔmɔnu siwo li ŋu.

Nukae adzɔ ne nɔnɔme sia le ŋunye?

Zi geɖe la, atikewɔwɔ ate ŋu aɖe dzesiawo dzi akpɔtɔ. Ame akpa gãtɔ siwo ŋu Congenital Methemoglobinemia Ƒomevi 1, alo Hemoglobin M dɔléle le, ƒe dzesi ʋɛ aɖewo koe dzena. Zi geɖe la, wonɔa agbe didi abe amesi si nɔnɔmea mele o ene.

Aleke malé be na ɖokuinye?

• Le goawo katã me la, ele be amesiwo wodzi kple dɔlélea ƒe nɔnɔme si nye ``dzidzime`` nakpɔ nyuie le atikewo kple atike siwo ateŋu ana dɔlélea nagado gã ɖe edzi ŋu. Esi wònye be amesiame ƒe nɔnɔme to vovo vie ta la, anyo wu be nàbia wò ɖɔkta.
• Ele be amesiwo ŋu methemoglobinemia si woxɔ (Acquired MetHb) le naƒo asa na nusiwo gbɔ wòtso keŋkeŋ – abe atikewo, vevesesenutsitike siwo wotsɔna ƒoa ʋu, alo atike siwo me aɖi le ene.

Ɣekaɣie wòle be makpɔ ɖɔkta?

• Ne methemoglobinemia ƒe domenyiŋusẽfianu le ŋuwò la, yi ɖɔkta gbɔ ne ède dzesi tɔtrɔwo le wò ŋutilã me, abe ɖeɖiteameŋu alo kuviawɔwɔ ene. Esiawo ate ŋu anye dzesi siwo fia be wò ʋumenugbagbevi dzĩwo megate ŋu tsɔa oxygen yia wò ŋutilã katã me o.
• Ne dzesi sesẽwo abe cyanosis (ŋutilã blɔ) ene kpe ɖe alɔ̃madɔmadɔ alo alɔ̃dɔdɔ fũu akpa ŋu la, ƒo ka na 911 enumake alo yi kɔdzi ƒe dɔwɔƒe si te ɖe ŋuwò si kpɔa nudzɔdzɔ kpatawo gbɔ.

Nya kawoe wòle be mabia ɖɔktaa?

Methemoglobinemia nye ʋumenugbagbeviɣidɔ si mebɔ kura o. Ame aɖewo ƒe domee wòxɔna (Congenital MetHb), gake ame akpa gãtɔ va xɔae emegbe (Acquired MetHb). Le wò nɔnɔmea nu la, àte ŋu abia nyawo abe:

Ku ɖe Methemoglobinemia si Woxɔ (MetHb si Woxɔ) ŋu:

• Nukatae esia dzɔ ɖe dzinye?
• Ðe atikewɔwɔa ana nye dzesiawo nu ayia?
• Ðe nye dzesiwo ate ŋu atrɔ ava?
• Nukae wòle be mawɔ be nu sia tɔgbi nagadzɔ ake o?

Ku ɖe Vidzidzi MetHb Ƒomevi 1 ŋu:

• Ðe cyanosis (ŋutilã blɔ) nye atikewɔwɔ me kuxi sesẽ aɖea?
• Ðe wòahiã be mano methemoglobinemia (MetHb) ƒe atike ɣesiaɣia?
• Ðe dzesi bubuwo ate ŋu anɔ ŋunyea?
• Ðe vinyewo ate ŋu anyi nɔnɔme sia ƒe domea?

MetHb si Wodzina Ƒomevi 2 (ne ɖevi mee wòle):

• Aleke nɔnɔme sia akpɔ ŋusẽ ɖe vinyea dzii?
• Ðe atikewɔmɔnu aɖewo li siwo ana vinyea ƒe dzesiwo dzi naɖe akpɔtɔa?
• Nukae mate ŋu awɔ atsɔ akpe ɖe vinyea ŋu?
• Ne vi geɖe le asinye la, ɖe esia adzɔ ɖe woawo hã dzia?

Kpuie ko la (Take-Home Message) .

Methemoglobinemia nye ʋumenugbagbeviɣidɔ si mebɔ o si gblẽa nu le míaƒe ʋumenugbagbevi dzĩwo ƒe ŋutete be woatsɔ oxygen atsa le ŋutilãa me ŋu. Le ame aɖewo gome la, domee wònye, gake le ame akpa gãtɔ gome la, atike aɖewo, atike siwo me aɖi le zazã, alo atike muamewo zazã tsɔ ɖea modzakae gbɔe wòtsona. Methemoglobinemia si wodzina (vevietɔ ƒomevi 2) ate ŋu ahe ahɔhɔ̃mekawo ƒe kuxi sesẽwo vɛ ɣeaɖewoɣi, gake zi geɖe la, mehea lãmesẽkuxi gãwo vɛ o. Gake methemoglobinemia si woxɔ (methemoglobinemia si woxɔ) ate ŋu ade ame ƒe agbe afɔku me ɣeaɖewoɣi.

Nu vevitɔ kekeakee nye be ne èsusu be yeate ŋu axɔ dɔléle sia la, mègavɔ̃ be yeaƒo nu tso eŋu na ɖɔkta o. Adzɔ dzi nɛ be yeakpe ɖe ŋuwò.

` Methemoglobinemia, Methemoglobinemia, Blue baby syndrome, Cyanosis, Oxygen ƒe anyimanɔmanɔ le ʋu me, Hemoglobin, Ʋumedɔlélewo, Domenyiŋusẽfianu ƒe dɔlélewo