Aia kekahi pilikia me ka oxygen i loko o ke koko? E aʻo kākou e pili ana i ka methemoglobinemia!

Ua ʻike anei ʻoe i ka ʻili a me nā lehelehe o kekahi e lilo koke ana i polū ʻē? A i ʻole ua lohe paha ʻoe ua hānau ʻia kekahi mau pēpē me ka uliuli iki? ʻO kekahi kumu no kēia he maʻi koko kakaʻikahi loa, akā he maʻi koʻikoʻi paha. I kēia lā e kamaʻilio mākou e pili ana i kekahi ʻano maʻi , ʻo ia hoʻi ka methemoglobinemia . Ua kapa pū kekahi poʻe iā ia ʻo "blue baby syndrome."

He aha maoli ka methemoglobinemia?

I ka ʻōlelo maʻalahi, ʻo ka methemoglobinemia kahi kūlana kahi i hiki ʻole ai i nā hunaola koko ʻulaʻula i loko o ko kākou koko ke lawe i ka oxygen i nā hunaola a me nā ʻiʻo ʻē aʻe i loko o ke kino. E noʻonoʻo e pili ana i ia mea, ʻo nā hunaola koko ʻulaʻula ka mea e kōkua i ka lawe ʻana i ka oxygen ma o ko kākou kino, ʻeā? Aia i loko o kēia mau hunaola koko ʻulaʻula kahi protein kūikawā i kapa ʻia ʻo hemoglobin . Ua like ia me kahi kaʻa, a lawe kēia hemoglobin i ka oxygen ma ke kino holoʻokoʻa.

Eia naʻe, ma kēia kūlana i kapa ʻia ʻo "Methemoglobinemia", ʻo ka mea e hana ʻia, ʻo ia ka loli ʻana o ko kākou "Hemoglobin" e lawe ana i ka oxygen a lilo i ʻano mea ʻokoʻa i kapa ʻia ʻo "Methemoglobin." ʻO ka pilikia, ʻaʻole hiki i kēia "Methemoglobin" ke lawe i ka oxygen. Ua like ia me ke kaʻa e lawe ana i ka oxygen e haki ana a ʻaʻole hiki ke lawe i nā ohua.

Hiki ke hoʻoilina ʻia kēia ʻano ma ke ʻano genetic i kekahi poʻe. Akā ʻo ka hapa nui, ua hana ʻia ia e kekahi mau lāʻau lapaʻau a mākou e hoʻohana ai, nā lāʻau lapaʻau leʻaleʻa, a i ʻole ka ʻike ʻana i kekahi mau kemika . I kekahi manawa hiki ke hoʻoweliweli i ke ola, ʻoiai hoʻi i nā pēpē i hānau ʻia me kahi ʻano koʻikoʻi o ke ʻano a me ka poʻe e hoʻohana ana i nā lāʻau lapaʻau. Eia nō naʻe, i ka hapa nui o nā hihia, hiki i nā kauka ke kuhikuhi i nā lāʻau lapaʻau e hoʻohaʻahaʻa i ka pae o ka "Methemoglobin" a hoʻopau i nā hōʻailona.

Pehea kēia e pili ai i koʻu kino?

He nui ka poʻe me ka methemoglobinemia e ʻike i kahi maʻi i kapa ʻia ʻo cyanosis . ʻO ka cyanosis ka wā e lilo ai kou mau kui lima, ke alelo, nā lehelehe, a me ka ʻili i polū māmā a poni paha. Hana ʻia kēia ke ʻole lawa ka oxygen i kou koko. E like me kaʻu i ʻōlelo ai ma mua, ʻo nā hunaola koko ʻulaʻula nā mea e lawe maʻamau i ka oxygen ma ke kino holoʻokoʻa. Kōkua kahi protein i kapa ʻia ʻo hemoglobin me kēia.

I loko o ka methemoglobinemia, loaʻa ka "Methemoglobinemia" i ka wā e lilo ai ka "Hemoglobin" i "Methemoglobin", ma muli paha o kahi hoʻololi genetic a i ʻole kekahi kumu waho ʻē aʻe. ʻOiai ʻaʻole hiki i ka "Methemoglobin" ke lawe i ka oxygen, emi ka nui o ka oxygen i loko o ke koko a loaʻa iā ʻoe ka "Cyanosis".

He aha nā hōʻailona o ka Methemoglobinemia?

Hiki ke ʻokoʻa nā hōʻailona mai kēlā kanaka a kēia kanaka, a hilinaʻi nō hoʻi i ke ʻano o ka maʻi āu e loaʻa ai. E nānā kākou i ke ʻano o ka hōʻike ʻana o kēia mau hōʻailona.

Nā Hōʻailona o ka Methemoglobinemia i Loaʻa

Nui ka poʻe e loaʻa i kēia maʻi ma hope o ka lawe ʻana i kekahi mau lāʻau ʻeha, nā lāʻau lapaʻau, a i ʻole ka ʻike ʻana i nā mea ʻawahia . Ua kapa ʻia kēia he methemoglobinemia i loaʻa. ʻO nā hōʻailona e hiki ke kū mai:

• ʻIli keʻokeʻo (pallor)
• Manaʻo luhi loa (fatigue)
• Nāwaliwali
• ʻEha poʻo

I kekahi manawa, hiki i ka poʻe me ka methemoglobinemia i loaʻa ke loaʻa nā hōʻailona koʻikoʻi e pono ai ke kōkua koke ʻia e ke kauka . ʻO kēia mau mea:

• Ka luaʻi a me ka luaʻi.
• Ka hiamoe nui, ka ʻōlelo ʻole, a me ka lohi o ka pane ʻana: He mau hōʻailona paha kēia o ke kaumaha o ka ʻōnaehana nerve waena .
• Ka nalowale o ka ʻike a i ʻole nā ​​neʻe kino hiki ʻole ke kāohi ʻia (e like me ka hopu ʻana): He mau hōʻailona kēia o ka hopu ʻana i ka maʻi epileptic .
• Ka hanu wikiwiki, ka piʻi ʻana o ka puʻuwai, a me ka huikau: He mau hōʻailona kēia o kahi maʻi i kapa ʻia ʻo metabolic acidosis .

He mea nui: Inā hōʻike kekahi me ʻoe i kekahi o kēia mau hōʻailona o ka maʻi epilepsy, `(Metabolic Acidosis)` a i ʻole `(Central Nervous System Depression)`, pono ʻoe e kāhea koke i ka 1990 a kāhea i kahi ambulance .

Nā hōʻailona o ka Methemoglobinemia Congenital

He maʻi laha ʻole ka methemoglobinemia hānau. He kakaikahi wale nō nā hihia i hōʻike ʻia ma ka honua holoʻokoʻa. Ma muli o kēlā ʻike, ua hoʻokaʻawale nā ​​kauka iā ia i ʻekolu ʻano nui: ʻAno 1, ʻAno 2, a me ka maʻi Hemoglobin M (HbM) .

• ʻO ka poʻe me ka maʻi hemoglobin M (HbM) he pinepine ka cyanosis (ʻili polū), akā olakino lākou.
• Loaʻa i ka poʻe me ka Type 1 Methemoglobinemia (Type 1 MetHb) ka cyanosis, akā he mea kākaʻikahi nā pilikia olakino nui ʻē aʻe.
• Eia nō naʻe, hiki i nā pēpē i hānau ʻia me ka Type 2 methemoglobinemia (Type 2 MetHb) ke loaʻa i nā pilikia neurological koʻikoʻi i ka wā e hiki ai iā lākou ma kahi o 9 mau mahina. ʻO ka mea kaumaha, ʻaʻole ola lōʻihi kēia mau pēpē.

No ke aha e kū mai ai ka methemoglobinemia?

Hiki ke hoʻoilina ʻia kēia maʻi, a i ʻole, e like me kaʻu i ʻōlelo ai ma mua, hiki ke hana ʻia ma hope e kekahi mau lāʻau lapaʻau, nā lāʻau lapaʻau, a i ʻole nā ​​​​​​kemika ʻawahia.

Nā kumu o ka Methemoglobinemia i loaʻa

• ʻO nā lāʻau ʻeha e like me benzocaine a me lidocaine (ʻoi aku ka poʻe i loaʻa i nā gels topical a me nā sprays) a me ka antibiotic dapsone hiki ke hana i kēia.
• Nā Nitrates i hoʻohana ʻia i kekahi mau lāʻau lapaʻauʻO nā Nitrites, ka mea i loaʻa paha i loko o nā mea mālama meaʻai a me kekahi wai o ka luawai, a me ka ʻike ʻia ʻana o kekahi mau herbicides i hoʻohana ʻia ma ka mahiʻai kekahi mau kumu.
• ʻO ka poʻe e hoʻohana ana i nā lāʻau leʻaleʻa e like me ka amyl nitrite (ʻike ʻia hoʻi he poppers), nitrous oxide (kinoea ʻakaʻaka), a me kekahi mau anesthetics i hui pū ʻia me nā lāʻau e like me ka cocaine e pilikia nui ana i nā pilikia olakino e hoʻoweliweli ana i ke ola ma muli o nā pae kiʻekiʻe loa o ka methemoglobin.

Nā kumu o ka Methemoglobinemia Congenital

Hoʻokumu ʻia ka methemoglobinemia congenital (Congenital MetHb) e ʻelua mau hoʻololi genetic like ʻole.

• I nā ʻano 1 a me 2 , hoʻopilikia kahi mutation i loko o ka gene CYB5R i ke kaulike ma waena o ka hemoglobin a me ka methemoglobin i loko o nā hunaola koko ʻulaʻula.
• Hoʻokumu ʻia ka maʻi Hemoglobin M (HbM) e nā mutations i loko o kekahi mau genes Hemoglobin M.

He maʻi autosomal recessive ka methemoglobinemia congenital. I ka ʻōlelo maʻalahi, i hiki i ke keiki ke loaʻa i ka maʻi, pono nā mākua ʻelua e lawe i ka mutation gene a pono i ke keiki ke hoʻoilina i ka mutation mai iā lāua ʻelua.

He maʻi autosomal dominant ka maʻi Hemoglobin M (HbM). ʻO ia hoʻi, hiki i kahi keiki ke loaʻa i ka maʻi ʻoiai inā hoʻoilina lākou i ka mutation gene mai hoʻokahi makua wale nō.

Pehea e ʻike ai nā kauka i kēia?

Hoʻoponopono nā kauka i kēia kūlana ma ka lawe ʻana i kāu mōʻaukala olakino piha a me ka hana ʻana i kahi hoʻokolokolo kino . Inā loaʻa i kekahi nā hōʻailona o ka methemoglobinemia i loaʻa, hiki iā lākou ke nīnau e pili ana i nā lāʻau lapaʻau a lākou e hoʻohana ai a i ʻole nā ​​​​​​kemika ʻawaʻawa i hōʻike ʻia paha iā lākou.

He aha nā hoʻokolohua e hana ʻia e ʻike ai i kēia?

Hana pinepine ʻia kēia mau hoʻokolohua:

• Nā Hoʻāʻo Koko: ʻO ke koko i lawe ʻia mai nā aʻa o ka poʻe me kēia maʻi he ʻeleʻele ʻeleʻele ke kala. ʻO kēia kala ʻeleʻele ʻeleʻele ke ʻano ʻaʻole lawe pono nā aʻa i ka oxygen i loko o ke koko.
• Hoʻāʻo pae Methemoglobin (MetHb): Ke ana kēia i ka nui o ka methemoglobin i loko o kou koko.
• Hana enzyme CYB5R: Inā haʻahaʻa ka hana o kēia enzyme ma mua o ka maʻamau, he hōʻailona ia o ka methemoglobinemia hānau.
• Genotyping: Inā kānalua ʻoe he methemoglobinemia hānau kou, e nānā ʻia kāu DNA no nā loli genetic e hōʻoia i ka hōʻailona. Hiki i kēia ke kōkua i ka ʻike ʻana i ke ʻano o ka maʻi.
• ʻElekoforesis Hemoglobin:He ʻano hana kēia no ka hoʻāʻo ʻana i ka "Hemoglobin" i loko o nā hunaola koko ʻulaʻula. Hiki ke hoʻohana ʻia kēia hoʻāʻo no ka ʻike ʻana i ka maʻi hemoglobin M "(HbM).

Pehea e mālama ʻia ai kēia?

ʻOkoʻa nā koho lapaʻau ma muli o ke ʻano o ka methemoglobinemia āu e loaʻa ai. No ka laʻana, ʻokoʻa loa ka mālama ʻana no kahi keiki hānau hou i hānau ʻia me ke ʻano 2 mai ka mālama ʻana no kekahi mea i loaʻa ke kūlana ma o ka ʻike ʻana i kahi kemika ʻawaʻawa a i ʻole ka hoʻohana ʻana i nā lāʻau lapaʻau.

• ʻAʻole pono paha ka lāʻau lapaʻau i ka poʻe me ka maʻi Methemoglobinemia Type 1 a i ʻole Hemoglobin M. Inā pono, hiki i nā kauka ke hoʻohana i kēia mau lāʻau lapaʻau e hoʻohaʻahaʻa i nā pae Methemoglobin:
• Methylene Blue: ʻO kēia ka lāʻau lapaʻau nui no ka methemoglobinemia.
• ʻO ka wikamina C a me ka wikamina B2.

Lapaʻau no ka Methemoglobinemia i loaʻa

Ma muli o ke kūlana, hiki i ka methemoglobinemia i loaʻa ke lilo i pilikia olakino. I kēlā mau hihia, pono paha ka hydration intravenous a me ka oxygen . I ka manawa pinepine, hāʻawi ʻia ka methylene blue i ka poʻe me ka methemoglobinemia i loaʻa.

He aha nā pilikia o ka mālama ʻana?

Inā hāʻawi mau ʻia kekahi kanaka me ka G6PD Deficiency (he maʻi genetic nō hoʻi), hiki ke hana ʻia kahi maʻi i kapa ʻia ʻo hemolysis . ʻO ka hemolysis ka haki mua ʻana a i ʻole ka haki pono ʻole o kāu mau hunaola koko ʻulaʻula.

Hiki iaʻu ke hōʻemi i koʻu pilikia o ka loaʻa ʻana o ka methemoglobinemia?

• ʻO ka poʻe i hoʻoilina i kahi ʻano genetic o kēia maʻi e pale i nā kemika ʻawahia (e like me nā pesticides), kekahi mau lāʻau lapaʻau, a me nā anesthetics kūloko he nui e hiki ke hoʻonui i nā pae methemoglobin. Inā loaʻa iā ʻoe kēia maʻi, e nīnau i kāu kauka e pili ana i nā mea āu e maʻi ai.
• ʻO ka poʻe e hoʻohana ana i nā lāʻau lapaʻau e like me ka amyl nitrite a i ʻole ka cocaine e hoʻonui iā lākou iho i ka pilikia o ka hoʻomohala ʻana i ka methemoglobinemia. Inā ʻoe e hoʻohana i kēia mau lāʻau lapaʻau a pono ʻoe i ke kōkua e haʻalele, e kamaʻilio me ke kauka e pili ana i nā lāʻau lapaʻau i loaʻa.

He aha ka mea e hana ai inā loaʻa iaʻu kēia maʻi?

I ka hapanui o nā hihia, hiki i ka lāʻau lapaʻau ke hōʻoluʻolu i nā hōʻailona. ʻO ka hapa nui o ka poʻe me ka Type 1 Congenital Methemoglobinemia, a i ʻole ka maʻi Hemoglobin M, he kakaikahi nā hōʻailona. ʻO ka maʻamau, ola lākou e like me ka lōʻihi o kekahi me ka ʻole o ke ʻano.

Pehea wau e mālama ai iaʻu iho?

• Ma keʻano laulā, pono e makaʻala ka poʻe i hānau ʻia me ke ʻano "congenital" o ka maʻi i nā lāʻau lapaʻau a me nā kemika e hiki ke hoʻonui i ka maʻi. ʻOiai he ʻokoʻa iki ke kūlana o kēlā me kēia kanaka, ʻoi aku ka maikaʻi o ke kūkākūkā ʻana me kāu kauka.
• Pono ka poʻe me ka methemoglobinemia i loaʻa (Acquired MetHb) e pale loa i nā mea i hoʻokumu ai - e like me nā lāʻau lapaʻau, nā lāʻau ʻeha ʻeha, a i ʻole nā ​​​​​​kemika ʻawahia.

Āhea wau e ʻike ai i ke kauka?

• Inā loaʻa iā ʻoe kahi ʻano hoʻoilina o ka methemoglobinemia, e ʻike i ke kauka inā ʻike ʻoe i nā loli i kou kino, e like me ka luhi a i ʻole ka palaualelo. He mau hōʻailona paha kēia ʻaʻole hiki i kāu mau hunaola koko ʻulaʻula ke lawe i ka oxygen ma loko o kou kino.
• Inā loaʻa iā ʻoe nā hōʻailona koʻikoʻi e like me ka cyanosis (ʻili polū) me ka hopu ʻana a i ʻole ka hiamoe nui, e kāhea koke i ka 911 a i ʻole e hele i ka keʻena pilikia haukapila kokoke loa.

He aha nā nīnau e pono iaʻu e nīnau aku i ke kauka?

He maʻi koko laha ʻole ka Methemoglobinemia. Loaʻa i kekahi poʻe kēia maʻi i hoʻoilina ʻia (Congenital MetHb), akā loaʻa i ka hapa nui o ka poʻe ma hope (Acquired MetHb). Ma muli o kou kūlana, makemake paha ʻoe e nīnau i kāu kauka e like me:

E pili ana i ka Methemoglobinemia i loaʻa (MetHb i loaʻa):

• No ke aha i hiki mai ai kēia iaʻu?
• E hoʻopau anei ka lāʻau i kaʻu mau hōʻailona?
• Hiki paha i kaʻu mau hōʻailona ke hoʻi hou mai?
• He aha kaʻu e hana ai e pale aku ai i ka hana hou ʻana o kekahi mea e like me kēia?

E pili ana i ka ʻAno 1 Congenital MetHb:

• He pilikia olakino koʻikoʻi anei ka cyanosis (ʻili polū)?
• Pono anei au e lawe i ka lāʻau methemoglobinemia (MetHb) i nā manawa a pau?
• Hiki iaʻu ke loaʻa nā hōʻailona ʻē aʻe?
• Hiki i kaʻu mau keiki ke hoʻoilina i kēia maʻi?

ʻAno 2 Congenital MetHb (inā he keiki):

• Pehea e pili ai kēia maʻi i kaʻu pēpē?
• Aia kekahi mau lāʻau lapaʻau e hōʻemi ai i nā hōʻailona o kaʻu pēpē?
• He aha kaʻu e hana ai e kōkua i kaʻu pēpē?
• Inā he nui aku kaʻu mau keiki, e hana ʻia anei kēia iā lākou pū kekahi?

I ka hōʻuluʻulu manaʻo (Leka Lawe-Home)

He maʻi koko laha ʻole ka Methemoglobinemia e hoʻopilikia ai i ka hiki o kā mākou mau hunaola koko ʻulaʻula ke lawe i ka oxygen a puni ke kino. No kekahi poʻe, ua hoʻoilina ʻia, akā no ka hapa nui o ka poʻe, ua hana ʻia ia e kekahi mau lāʻau lapaʻau, ka ʻike ʻana i nā kemika ʻawahia, a i ʻole ka hoʻohana ʻana i nā lāʻau lapaʻau leʻaleʻa. Hiki i ka methemoglobinemia hānau (ʻoi aku ka ʻano 2) ke hana i nā pilikia neurological koʻikoʻi, akā ʻaʻole pinepine ia e hana i nā pilikia olakino nui. Eia nō naʻe, hiki i ka methemoglobinemia i loaʻa (methemoglobinemia i loaʻa) ke hoʻoweliweli i ke ola i kekahi manawa.

ʻO ka mea nui loa, inā manaʻo ʻoe ua pilikia ʻoe i ka loaʻa ʻana o kēia maʻi, mai makaʻu e kamaʻilio me ke kauka e pili ana i ia mea. E hauʻoli ʻo ia e kōkua iā ʻoe.

ʻO Methemoglobinemia, Methemoglobinemia, Blue baby syndrome, Cyanosis, ʻAʻohe oxygen i loko o ke koko, Hemoglobin, Nā maʻi koko, Nā maʻi genetic