So ɔhaw bi wɔ oxygen a ɛwɔ mogya no mu ho? Momma yensua methemoglobinemia ho ade!

So woahu sɛ obi honam ani ne n’ano dan kɔla bruu a ɛyɛ nwonwa mpofirim pɛn? Anaasɛ woate sɛ wɔwo nkokoaa binom bruu kakra? Ade biako nti a eyi ba no betumi ayɛ mogya mu yare a ɛntaa nsi koraa, nanso ebetumi ayɛ nea emu yɛ den. Ɛnnɛ yɛrebɛka tebea a ɛte saa biako ho asɛm , methemoglobinemia . Nnipa binom nso frɛ no "blue baby syndrome."

Dɛn ankasa ne methemoglobinemia?

Sɛ yɛbɛka no tiawa a, methemoglobinemia yɛ tebea a ɛma mogya mu nkwammoaa kɔkɔɔ a ɛwɔ yɛn mogya mu no ntumi mfa mframa pa nkɔ nipadua no mu nkwammoaa ne ntini afoforo mu. Susuw ho hwɛ, mogya mu nkwammoaa kɔkɔɔ ne nea ɛboa ma oxygen kɔ yɛn nipadua nyinaa mu, ɛnte saa? Protein soronko bi a wɔfrɛ no hemoglobin wɔ mogya mu nkwammoaa kɔkɔɔ yi mu . Ɛte sɛ bɔs, na saa hemoglobin yi de mframa pa kɔ nipadua no mu nyinaa.

Nanso, wɔ saa tebea yi a wɔfrɛ no ``Methemoglobinemia`` mu no, nea ɛba ne sɛ yɛn ``Hemoglobin`` a ɛde oxygen kɔ no sesa na ɛbɛyɛ ade foforo a wɔfrɛ no ``Methemoglobin.`` Ɔhaw no ne sɛ, saa ``Methemoglobin`` yi ntumi mfa oxygen nkɔ. Ɛte sɛ bɔs a ɛde oxygen kɔ no sɛe na entumi mfa akwantufo.

Saa tebea yi betumi afi awo mu (wɔ awosu mu) wɔ nnipa binom mu. Nanso mpɛn pii no , nnuru bi a yɛde di dwuma, nnuru a yɛde gye yɛn ani, anaa nnuru bi a yɛde di dwuma na ɛde ba . Ɛtɔ mmere bi a ebetumi de nkwa ato asiane mu, titiriw wɔ nkokoaa a wɔwo wɔn a yare no mu yɛ den ne nnipa a wɔde nnubɔne di dwuma no mu. Nanso, mpɛn pii no, nnuruyɛfo betumi akyerɛw nnuru a ɛbɛma ``Methemoglobin`` dodow akɔ fam na wɔayi sɛnkyerɛnne ahorow no afi hɔ.

Eyi ka me nipadua dɛn?

Nnipa pii a wɔwɔ methemoglobinemia no nya tebea bi a wɔfrɛ no cyanosis . Cyanosis yɛ bere a wo nsateaa nnadewa, wo tɛkrɛma, w’ano, ne wo honam ani dan kɔla bruu anaa kɔkɔɔ a ɛyɛ hann. Eyi ba bere a wo mogya nni oxygen a ɛdɔɔso no. Sɛnea madi kan aka no, mogya mu nkwammoaa kɔkɔɔ ne nea ɛtaa de mframa pa kɔ nipadua no mu nyinaa. Protein bi a wɔfrɛ no hemoglobin boa wɔ eyi mu.

Wɔ methemoglobinemia mu no, ``Methemoglobinemia`` ba bere a ``Hemoglobin`` dan ``Methemoglobin``, esiane awosu mu nsakrae anaa biribi foforo bi a efi akyi nti. Esiane sɛ ``Methemoglobin`` ntumi mfa oxygen nkɔ nti, oxygen dodow a ɛwɔ mogya no mu no so tew na wunya ``Cyanosis``.

Dɛn ne Methemoglobinemia ho sɛnkyerɛnne ahorow?

Nsɛnkyerɛnne no betumi ayɛ soronko wɔ onipa biara ho, na egyina yare ko a wowɔ nso so. Momma yɛnhwɛ sɛnea saa sɛnkyerɛnne ahorow yi taa da adi.

Methemoglobinemia a Wonya Ho Nsɛnkyerɛnne

Nnipa pii nya saa tebea yi bere a wɔanom nnuru bi a wɔde kum ɛyaw, nnuru, anaa nneɛma a awuduru wom akyi . Wɔfrɛ eyi sɛ methemoglobinemia a wonya. Nsɛnkyerɛnne a ebetumi aba no bi ne:

• Wora a ɛyɛ mmerɛw (pale) .
• Ɔbrɛ kɛse a wɔte nka (ɔbrɛ) .
• Mrɛyɛ
• Tipaeɛ

Ɛtɔ mmere bi a, nnipa a wɔwɔ methemoglobinemia a wɔanya no betumi anya sɛnkyerɛnne a emu yɛ den a ɛsɛ sɛ wɔde wɔn kɔ ayaresabea ntɛm ara . Eyinom bi ne:

• Akisikuru ne ɔfe.
• Dae a ɔda dodo, kasa a ɛyɛ mmerɛw, ne nea ɔyɛ brɛoo: Eyinom betumi ayɛ ntini a ɛwɔ nipadua no mfinimfini no adwenemhaw ho sɛnkyerɛnne .
• Nhumu a ɛyera anaa nipadua a ɛkeka ne ho a wontumi nni so (te sɛ akisikuru): Eyinom yɛ akisikuru ho sɛnkyerɛnne .
• Ɔhome ntɛmntɛm, koma a ɛbɔ kɛse, ne adwene a ɛyɛ basaa: Eyinom yɛ tebea bi a wɔfrɛ no metabolic acidosis ho sɛnkyerɛnne .

Nea ɛho hia: Sɛ obi a ɔne wo da akisikuru ho sɛnkyerɛnne yi bi adi, `(Metabolic Acidosis)` anaa `(Central Nervous System Depression)` a, ɛsɛ sɛ wofrɛ 1990 ntɛm ara na wofrɛ ambulance .

Nsɛnkyerɛnne a Ɛkyerɛ sɛ Methemoglobinemia a Wɔde Awo Wɔn

Methemoglobinemia a wɔde awo obi yɛ tebea a ɛntaa nsi koraa. Nnipa kakraa bi pɛ na wɔabɔ amanneɛ wɔ wiase nyinaa. Nnuruyɛfo gyina saa nsɛm no so akyekyɛ mu ayɛ no ahorow abiɛsa: Ɔkwan 1, Ɔkwan 2, ne Hemoglobin M yare (HbM) .

• Nnipa a wɔwɔ hemoglobin M yare (HbM) taa nya cyanosis (honam ani bruu), nanso mpɛn pii no wɔwɔ apɔwmuden.
• Ɛwom sɛ nnipa a wɔwɔ Type 1 methemoglobinemia (Type 1 MetHb) nso wɔ cyanosis de, nanso akwahosan ho haw atitiriw afoforo ntaa mma.
• Nanso, nkokoaa a wɔwo wɔn a wɔwɔ Type 2 methemoglobinemia (Type 2 MetHb) no betumi anya ntini mu haw ahorow a emu yɛ den bere a wɔadi bɛyɛ asram 9 no. Awerɛhosɛm ne sɛ, saa nkokoaa yi ntra ase nkyɛ.

Dɛn nti na methemoglobinemia ba?

Saa tebea yi betumi afi awo mu, anaasɛ sɛnea madi kan aka no, nnuru, nnuru, anaa nnuru bi a awuduru wom nso betumi de aba akyiri yi.

Nneɛma a Ɛde Methemoglobinemia a Wonya Ba

• Nnuru a wɔde kum ɛyaw te sɛ benzocaine ne lidocaine (titiriw nea wohu wɔ gel ne nnuru a wɔde petepete so mu) ne nnuru a ekum ɔyare mmoawa a wɔfrɛ no dapsone betumi de eyi aba.
• Nitrates a wɔde di dwuma wɔ nnuru bi mu, Nitrites, a ebia ɛwɔ nnuru a wɔde kora nnuan ne abura mu nsu bi mu, ne nnuru bi a wɔde kum wura a wɔde di dwuma wɔ kuayɛ mu a wɔde wɔn ho hyɛ mu nso yɛ nea ɛde ba.
• Nnipa a wɔde nnuru a wɔde gye wɔn ani te sɛ amyl nitrite (a wɔsan frɛ no poppers), nitrous oxide (mframa a wɔde serew), ne nnuru bi a wɔde kum nipadua no a wɔde afra nnubɔne te sɛ cocaine di dwuma no wɔ asiane kɛse mu sɛ wobenya aduruyɛ mu haw ahorow a ɛde wɔn nkwa to asiane mu esiane methemoglobin dodow a ɛdɔɔso yiye nti.

Nea Ɛde Methemoglobinemia a Wɔde Awo Wɔn Ba

Awosu mu nkwaadɔm mu nsakrae ahorow abien na ɛde methemoglobinemia (Congenital MetHb) ba.

• Wɔ ahorow 1 ne 2 mu no, nkwaadɔm mu nsakrae a ɛba awosu mu nkwaadɔm CYB5R mu no sɛe hemoglobin ne methemoglobin a ɛkari pɛ wɔ mogya mu nkwammoaa kɔkɔɔ mu no.
• Hemoglobin M yare (HbM) fi nsakrae a ɛba Hemoglobin M awosu mu nkwaadɔm pii mu.

Methemoglobinemia a wɔde awo obi yɛ yare a ɛma nipadua no tumi kɔ nipadua no mu . Sɛ yɛbɛka no tiawa a, sɛ abofra betumi anya yare no a, ɛsɛ sɛ awofo baanu no nyinaa yɛ awosu mu nkwaadɔm mu nsakrae no kurafo na ɛsɛ sɛ abofra no nya nkwaadɔm mu nsakrae no fi wɔn baanu nyinaa hɔ.

Hemoglobin M yare (HbM) yɛ ɔyare a ɛma nipadua no tumi yɛ adwuma . Eyi kyerɛ sɛ abofra betumi anya yare no sɛ wonya awosu mu nkwaadɔm mu nsakrae no fi ɔwofo biako pɛ hɔ mpo a.

Ɔkwan bɛn so na nnuruyɛfo hu eyi?

Nnuruyɛfo hu saa tebea yi denam w'aduruyɛ ho abakɔsɛm a edi mũ a wogye na wɔyɛ nipadua mu nhwehwɛmu so . Sɛ obi wɔ methemoglobinemia a wanya ho sɛnkyerɛnne a, obetumi abisa nnuru biara a ɔde di dwuma anaa nnuru a awuduru wom a ebia ɔde dii dwuma ho asɛm.

Nhwehwɛmu bɛn na wɔyɛ de hu eyi?

Wɔtaa yɛ sɔhwɛ ahorow yi:

• Mogya mu Nhwehwɛmu: Mogya a woyi fi nnipa a wɔwɔ saa yare yi ntini mu no taa yɛ bruu tuntum. Saa kɔla a ɛyɛ bruu tuntum yi kyerɛ sɛ ntini no mfa mframa pa nkɔ mogya no mu yiye.
• Methemoglobin (MetHb) dodow nhwehwɛmu: Eyi kyerɛ methemoglobin dodow a ɛwɔ wo mogya mu.
• CYB5R enzyme dwumadi: Sɛ saa enzyme yi dwumadi sua sen sɛnea ɛte daa a, ɛkyerɛ sɛ obi awo methemoglobinemia.
• Awosu mu nkwaadɔm: Sɛ wususuw sɛ wowɔ methemoglobinemia a wofi awo wo mu a, wɔbɛhwɛ wo DNA mu ahwɛ sɛ awosu mu nsakrae bi wɔ hɔ de si so dua sɛ woahu sɛ wowɔ yare no. Eyi nso betumi aboa ma wɔahu yare no ko.
• Hemoglobin Electrophoresis a Wɔde Yɛ Adwuma:Eyi yɛ ɔkwan a wɔfa so sɔ ``Hemoglobin`` a ɛwɔ mogya mu nkwammoaa kɔkɔɔ mu hwɛ. Wobetumi de saa sɔhwɛ yi adi dwuma de ahu hemoglobin M yare ``(HbM).``

Ɔkwan bɛn so na wɔsa eyi?

Ɛsono ayaresa a wubetumi apaw a egyina methemoglobinemia ko a wowɔ so. Sɛ nhwɛso no, ayaresa a wɔde ma abofra a wɔawo no foforo a ɔwɔ type 2 no yɛ soronko koraa wɔ ayaresa a wɔde ma obi a onyaa tebea no denam nnuru a awuduru wom anaa nnubɔne a ɔde dii dwuma so.

• Ebia nnipa a wɔwɔ Type 1 Methemoglobinemia anaa Hemoglobin M yare no renhia ayaresa. Sɛ ɛho hia a, nnuruyɛfo betumi de nnuru yi adi dwuma de atew Methemoglobin dodow so:
• Methylene Blue: Eyi ne aduru titiriw a wɔde ko tia methemoglobinemia.
• Vitamin C ne vitamin B2.

Ayaresa a Wɔde Ma Methemoglobinemia a Wonya

Ɛdenam tebea no so no, methemoglobinemia a wonya no betumi ayɛ aduruyɛ mu asɛm a egye ntɛmpɛ. Wɔ tebea horow a ɛtete saa mu no, ebia ɛho behia sɛ wɔde nsu ne mframa pa a wɔde fa ntini mu ma . Mpɛn pii no, wɔma nnipa a wɔanya methemoglobinemia a wɔanya methylene blue .

Dɛn ne nsɛnnennen a ɛwɔ ayaresa mu?

Sɛ wɔma obi a ɔwɔ G6PD Deficiency (a ɛno nso yɛ awosu mu tebea) ayaresa methylene blue bere nyinaa a, tebea bi a wɔfrɛ no hemolysis betumi aba. Hemolysis yɛ wo mogya mu nkwammoaa kɔkɔɔ a ɛpaapae ntɛm anaasɛ ɛho nhia.

So metumi atew asiane a ɛwɔ hɔ sɛ menya methemoglobinemia no so?

• Ɛsɛ sɛ nnipa a wonya awosu mu nsakrae bi a ɛwɔ tebea yi mu no kwati nnuru a awuduru wom (e.g. nnuru a ekum mmoawa), nnuru bi, ne nnuru pii a wɔde kum nipadua no wɔ mpɔtam hɔ a ebetumi ama methemoglobin dodow akɔ soro. Sɛ wowɔ saa tebea yi a, bisa wo duruyɛfo mfa nea ebia wo ho nyɛ wo hyew ho asɛm.
• Nnipa a wɔde nnubɔne te sɛ amyl nitrite anaa cocaine di dwuma no de wɔn ho to asiane mu kɛse sɛ wobenya methemoglobinemia. Sɛ wode saa nnuru yi di dwuma na wuhia mmoa na woagyae a, wo ne oduruyɛfo nkasa mfa ayaresa ahorow a ɛwɔ hɔ ho.

Sɛ mewɔ saa tebea yi a, dɛn na ɛba?

Mpɛn pii no, ayaresa betumi ama yare no ho sɛnkyerɛnne no afi hɔ. Nnipa dodow no ara a wɔwɔ Type 1 Congenital Methemoglobinemia, anaa Hemoglobin M yare no, nnya yare no ho sɛnkyerɛnne kakraa bi. Wɔtaa tra ase kyɛ te sɛ obi a onni tebea no.

Mɛyɛ dɛn ahwɛ me ho?

• Mpɛn pii no, ɛsɛ sɛ nnipa a wɔwo wɔn a wɔwɔ yare no ``congenital`` no hwɛ yiye wɔ nnuru ne nnuru a ebetumi ama yare no ayɛ kɛse ho. Esiane sɛ ɛsono obiara tebea kakra nti, ɛbɛyɛ papa sɛ wobɛkɔ wo duruyɛfo nkyɛn.
• Ɛsɛ sɛ nnipa a wɔwɔ methemoglobinemia a wɔanya (Acquired MetHb) kwati nneɛma a ɛde bae no koraa – te sɛ nnuru, nnuru a wɔde kum ɛyaw a wɔde gu nipadua no so, anaa nnuru a awuduru wom.

Bere bɛn na ɛsɛ sɛ mekɔ oduruyɛfo nkyɛn?

• Sɛ wowɔ methemoglobinemia a wonya fi awo mu a, kɔ oduruyɛfo nkyɛn sɛ wuhu nsakrae bi wɔ wo nipadua mu, te sɛ ɔbrɛ anaa ɔbrɛ a. Ebia eyinom yɛ nsɛnkyerɛnne a ɛkyerɛ sɛ wo mogya mu nkwammoaa kɔkɔɔ no ntumi mfa mframa pa nkɔ wo nipadua nyinaa mu bio.
• Sɛ wowɔ sɛnkyerɛnne a emu yɛ den te sɛ cyanosis (honam ani bruu) a ɛka ho ne akisikuru anaa nna a ɛtra so a, frɛ 911 ntɛm ara anaa kɔ ayaresabea dwumadibea a ɛbɛn wo a wɔhwɛ ntɛmpɛ so.

Nsɛm bɛn na ɛsɛ sɛ mibisa oduruyɛfo no?

Methemoglobinemia yɛ mogya mu yare a ɛntaa nsi koraa. Nnipa binom nya bi fi awo mu (Congenital MetHb), nanso nnipa dodow no ara nya bi akyiri yi (Acquired MetHb). Ɛdenam wo tebea so no, ebia wobɛpɛ sɛ wubisa wo duruyɛfo nsɛm te sɛ:

Ɛfa Methemoglobinemia a Wonya (MetHb a Wonya) ho:

• Adɛn nti na eyi too me?
• So ayaresa no bɛma me yare no ho sɛnkyerɛnne no afi hɔ?
• So me yare no ho sɛnkyerɛnne no betumi asan aba bio?
• Dɛn na ɛsɛ sɛ meyɛ na amma biribi a ɛte sɛɛ ansi bio?

Ɛfa Type 1 Congenital MetHb ho:

• So cyanosis (honam ani bruu) yɛ aduruyɛ mu ɔhaw a emu yɛ den?
• So ɛsɛ sɛ mefa methemoglobinemia (MetHb) aduru bere nyinaa?
• So metumi anya yare no ho sɛnkyerɛnne afoforo?
• So me mma betumi anya tebea yi fi awo mu?

Type 2 Congenital MetHb (sɛ ɛwɔ abofra mu a):

• Ɔkwan bɛn so na saa tebea yi bɛka me ba no?
• So ayaresa ahorow bi wɔ hɔ a wɔde bɛtew me ba no yare no ho sɛnkyerɛnne so?
• Dɛn na metumi ayɛ de aboa me ba no?
• Sɛ mewo mma pii a, so eyi bɛto wɔn nso?

Sɛ yɛbɛbɔ no mua a (Take-Home Message) .

Methemoglobinemia yɛ mogya mu yare a ɛntaa nsi a ɛka tumi a yɛn mogya mu nkwammoaa kɔkɔɔ no tumi de oxygen di akɔneaba wɔ nipadua no mu. Wɔ ebinom fam no, wonya fi awo mu, nanso wɔ nnipa dodow no ara fam no, efi nnuru bi, nnuru a awuduru wom a wɔde wɔn ho hyɛ mu, anaa nnubɔne a wɔde di dwuma de gye wɔn ani. Ɛtɔ mmere bi a, methemoglobinemia a wɔde awo obi (titiriw nea ɛto so abien) betumi de ntini mu haw ahorow a emu yɛ den aba, nanso mpɛn pii no, ɛmma akwahosan ho haw akɛse mma. Nanso, ɛtɔ mmere bi a methemoglobinemia a wonya (methemoglobinemia a wonya) betumi de nkwa ato asiane mu.

Nea ɛho hia paa ne sɛ, sɛ wususuw sɛ asiane wɔ hɔ sɛ wubenya saa yare yi a, nsuro sɛ wo ne oduruyɛfo bɛkasa afa ho. N’ani begye sɛ ɔbɛboa wo.

` Methemoglobinemia, Methemoglobinemia, Blue baby syndrome, Cyanosis, Oxygen a enni mogya mu, Hemoglobin, Mogya mu nyarewa, Awosu mu nyarewa