Methemoglobinemia

ʻO Methemoglobinemia (MetHb) kahi maʻi maʻi koko kahi e hana ʻia ai kahi nui maʻamau o ka methemoglobin. ʻO Hemoglobin ka protein i loko o nā hunaola ʻulaʻula (RBC) e halihali a hāʻawi i ka oxygen i ke kino. ʻO Methemoglobin kahi ʻano o hemoglobin.

Me ka methemoglobinemia, hiki i ka hemoglobin ke hāpai i ka oxygen, akā ʻaʻole hiki ke hoʻokuʻu maikaʻi iā ia i nā kino o ke kino.

Hiki i ke ʻano MetHb ke:

• Hoʻoholo ʻia i waena o nā ʻohana (hoʻoilina a hānau hānau paha)
• Hana ʻia e ka hōʻike ʻana i kekahi mau lāʻau, kemikō, a i ʻole nā ​​meaʻai (loaʻa)

ʻElua ʻano o ka MetHb hoʻoilina. Hoʻoholo ʻia ka palapala mua e nā mākua ʻelua. ʻAʻohe o nā mākua i ke ʻano ponoʻī. Hāpai lākou i ka ʻāpana i kumu i ke ʻano. Kū ʻia ia inā loaʻa kahi pilikia me kahi enzyme i kapa ʻia ʻo cytochrome b5 reductase.

ʻElua ʻano o ka hoʻoilina MetHb:

• ʻAno 1 (i kapa ʻia hoʻi e defry erythrocyte reductase deficience) ke loaʻa ʻole nā ​​RBC i ka ʻekema.
• ʻAno 2 (i kapa ʻia hoʻi deficit reductase ākea) hiki ke hana ʻole ka enzyme i ke kino.

ʻO ka lua o nā hana o MetHb hoʻoilina i kapa ʻia ʻo hemoglobin M maʻi. Hoʻokumu ʻia ia e nā kīnā i ka protein hemoglobin ponoʻī. Hoʻokahi wale nō mākua e pono e hoʻoili i ka gen abnormal no ke keiki e hoʻoilina i ka maʻi.

ʻOi aku ka maʻamau o MetHb i loaʻa ma mua o nā ʻano hoʻoilina. Kū ʻia ia i kekahi poʻe ma hope o ka hōʻike ʻia ʻana i kekahi mau kemikal a me nā lāʻau, e like me:

• Nā Anesthetics e like me benzocaine
• Nitrobenzene
• Kekahi antibiotics (me dapsone a me chloroquine)
• Nā Nitrites (hoʻohana ʻia e like me nā mea hoʻohui i mea e pale aku ai i kaʻiʻo mai ka hōʻino ʻana)

Nā ʻōuli o ke ʻano 1 MetHb me:

• ʻO ka waihoʻoluʻu ʻōniʻoniʻo o ka ʻili

Nā ʻōuli o ke ʻano 2 MetHb e komo pū me:

• Hoʻolālā hoʻomohala
• Ka holomua ʻole ʻana
• Kīnā ʻole o ka noʻonoʻo
• Ka hopu ʻana i nā maʻi

Nā ʻōuli o ka maʻi hemoglobin M:

• ʻO ka waihoʻoluʻu ʻōniʻoniʻo o ka ʻili

Nā ʻōuli o MetHb i loaʻa:

• Waihoʻoluʻu polū o ka ʻili
• ʻEha poʻo
• ʻO Giddiness
• Ke kūlana noʻonoʻo i hoʻololi ʻia
• Ka luhi
• Pokole o ka hanu
• Ka nele o ka ikehu

ʻO ka pēpē me kēia ʻano e loaʻa i ka ʻili polū (cyanosis) i ka wā hānau a i ʻole ma hope koke iho. E hana ka mea mālama ola i nā hoʻokolohua koko e hōʻoia i ke ʻano. E komo pū paha nā hoʻāʻo:

• Ke nānā nei i ka pae o ka oxygen i ke koko (oximetry pulse)
• Kākoʻo koko e nānā i nā pae o nā kinoea i loko o ke koko (ka nānā ʻana o ke kinoea koko)

ʻAʻole loaʻa nā ʻōuli i ka poʻe me ka maʻi hemoglobin M. No laila, ʻaʻole pono lākou i ka lāʻau.

Hoʻohana ʻia kahi lāʻau i kapa ʻia he methylene blue e mālama i ka MetHb koʻikoʻi. ʻAʻole palekana ʻo Methylene polū i ka poʻe i loaʻa a makaʻu paha i kahi maʻi koko i kapa ʻia ʻo G6PD deficience. ʻAʻole pono lākou e lawe i kēia lāʻau. Inā loaʻa ʻole kāu a ʻo kāu keiki i ka hemahema G6PD, e haʻi mau i kāu mea hoʻolako ma mua o ka loaʻa ʻana o ka lāʻau.

Hiki ke hoʻohana ʻia ka waikawa Ascorbic e hōʻemi i ka pae o ka methemoglobin.

ʻO nā kuʻina ʻē aʻe e pili ana i ka hyperbaric oxygen therapy, ka hoʻokaʻa koko ʻulaʻula a me nā transfusions exchange.

I ka hapanui o nā hihia o ka loaʻa MetHb akahai, ʻaʻohe pono e mālama ʻia. Akā pono ʻoe e hōʻalo i ka lāʻau a i ʻole ka kemikina i kumu i ka pilikia. Pono paha nā hihia koʻikoʻi i kahi kōpili.

Hana maikaʻi nā poʻe me ka ʻano 1 MetHb a me ka hemoglobin M maʻi maikaʻi. ʻAno nui ʻo MetHb 2. Hoʻokumu pinepine ia i ka make i loko o nā makahiki mua o ke ola.

ʻO ka poʻe me ka MetHb i loaʻa pinepine i ka maikaʻi ke hana maikaʻi loa i ka lāʻau, nā meaʻai, a i ʻole nā ​​kemika i hoʻokumu i ka pilikia a ʻike ʻia hoʻi.

Nā pilikia o MetHb me:

• Pāhaʻi
• Ka hopu ʻana i nā maʻi
• Make

Kāhea i kāu mea hāʻawi inā ʻoe:

• Loaʻa i kahi moʻolelo ʻohana o MetHb
• E hoʻomohala i nā ʻōuli o kēia maʻi

Kāhea koke i kāu mea lawelawe a lawelawe lawelawe kūikawā paha (911) inā loaʻa ka hanu nui.

Kuhi ʻia ka ʻōlelo aʻoaʻo genetic no nā kāne me ka moʻolelo ʻohana o MetHb a ke noʻonoʻo nei i ka hānau keiki.

ʻO nā pēpē he 6 mau mahina a ʻoi paha ka liʻiliʻi e hoʻomohala i ka methemoglobinemia. No laila, ʻo nā mea hoʻomaʻemaʻe pēpē homemade hana ʻia mai nā mea kanu i loaʻa nā pae kiʻekiʻe o nā nitrates maoli, e like me nā kāloti, beetroots, a i ʻole ka milo.

Maʻi Hemoglobin M; Ka nele o Erythrocyte reductase; Ka nui o ka hemahema reductase; MetHb

• Nā pūnaewele koko

ʻO Benz EJ, ʻo Ebert BL. ʻO nā ʻano hemoglobin e pili ana me ka hemolytic anemia, ka loli ʻana o ka oxygen, a me ka methemoglobinemias. I: Hoffman R, Benz EJ, Silberstein LE, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 43.

Letterio J, Pateva I, Petrosiute A, Ahuja S. Hematologic a me nā pilikia oncologic i ka fetus a neonate. I: Martin RJ, Fanaroff AA, Walsh MC, hua. ʻO Fanaroff lāua ʻo Martin's Neonatal-Perinatal Medicine. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 79.

ʻO RT. Hele i nā anemias. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 149.