He maʻi ʻoe i ka anesthesia? E aʻo kākou e pili ana i ka (Pseudocholinesterase Deficiency)?

Ua hopohopo iki paha ʻoe e pili ana i ka anesthesia i hāʻawi ʻia iā ʻoe no kahi hana ʻoki? He mea maʻamau ia no kekahi poʻe ke manaʻo pēlā. Akā e noʻonoʻo i ka mea e hiki mai ana inā ʻaʻole kūpono kekahi o nā lāʻau anesthesia i hāʻawi ʻia iā ʻoe no kou kino. I kēia lā e kamaʻilio mākou e pili ana i kekahi ʻano kūlana kakaʻikahi, akā he mea nui loa e makaʻala. ʻO ia ke ʻano i kapa ʻia ʻo `(Pseudocholinesterase Deficiency)`.

He aha ka `(Pseudocholinesterase Deficiency)`? E hoʻomaopopo maʻalahi kākou, ʻeā?

ʻAe, he lōʻihi iki ke kani ʻana o kēia inoa, ʻeā? E mālama maʻalahi kākou. ʻO ka `(Pseudocholinesterase)` kahi enzyme kūikawā `(Enzyme)` i loko o ko kākou kino. ʻO kekahi o nā hana nui o kēia `(Enzyme)` ʻo ia ka wāwahi ʻana (ʻo ia hoʻi, `(metabolize)`) i loko o ke kino i kekahi o nā lāʻau i hāʻawi ʻia e hoʻomaha i nā ʻiʻo , ʻoiai ke hoʻomaha ʻia mākou (kapa mākou i kēia mau `(Choline esters)`). E noʻonoʻo, i ka wā o ke ʻoki ʻana, pono e hoʻopalupalu ʻia kou mau ʻiʻo no kekahi manawa. ʻO kēia nā lāʻau i hāʻawi ʻia no kēlā.

I kēia manawa, ma ke ʻano i kapa ʻia ʻo `(Pseudocholinesterase Deficiency)`, ʻo ka mea e hana ʻia nei, ʻo kēia `(Enzyme)` i kapa ʻia ʻo `(Pseudocholinesterase)` i loko o kou kino ʻaʻole i hana lawa ʻia , a i ʻole ʻaʻole ia e hana pono ana . He aha ka mea e hana ʻia a laila? ʻO nā lāʻau lapaʻau i hāʻawi ʻia e hoʻomaha i nā ʻiʻo (`(Succinylcholine)` a me `(Mivacurium)` ʻelua mau laʻana o kēia) ʻaʻole i wehe koke ʻia mai ke kino. Noho lākou i loko o ke kino no ka manawa lōʻihi.

ʻO ka hopena, hiki i kou mau ʻiʻo ke noho palupalu no ka manawa lōʻihi ma hope o ka anesthesia, a hiki paha iā ʻoe ke paʻakikī i ka hanu ʻana. ʻO ke ʻano kēia, ʻaʻole paha ʻoe e hiki ke neʻe a hanu paha iā ʻoe iho a hiki i ka pau ʻana o nā hopena o ka lāʻau.

Pehea ka maʻamau o kēia ʻano?

ʻOiaʻiʻo, ʻaʻole maʻamau kēia kūlana i kapa ʻia ʻo `(Pseudocholinesterase Deficiency)`. Ma ka awelika, hoʻokahi wale nō i loko o 3,200 a i ʻole 5,000 mau kānaka o ka heluna kanaka e ʻike nei i kēia kūlana. ʻO ia hoʻi, he helu liʻiliʻi loa.

He aha nā hōʻailona? Pehea ʻoe e ʻike ai i kēia?

ʻAʻole ʻike ka hapa nui o ka poʻe me kēia maʻi a hiki i ka loaʻa ʻana o ke ʻano o ka lāʻau hoʻomāmā aʻu i ʻōlelo ai ma mua, i kapa ʻia ʻo "Choline esters." ʻO ia ka manawa e ʻike ʻia ai nā hōʻailona nui:

• Nā ʻiʻo i hoʻomaha nui ʻia (ʻo ia hoʻi, make) no ka manawa lōʻihi ma mua o ka mea i manaʻo ʻia.
• ʻOiai nā ʻiʻo e kōkua iā ʻoe e hanu e lilo i mea hana ʻole no kekahi manawa .

He mau hola paha e emi ai kēia mau hōʻailona. ʻOkoʻa kēia manawa mai kēlā kanaka a kēia kanaka. I loko o kēia manawa, e mālama kāu hui lapaʻau iā ʻoe ma lalo o ka anesthesia a kōkua iā ʻoe e hanu ma o kahi "ventilator", kahi mīkini hanu hana, a hiki i kou hiki ke hanu iā ʻoe iho.

He aha nā kumu? Pehea e hana ai kēia?

Hiki ke hoʻoilina ʻia kēia kūlana (Pseudocholinesterase Deficiency), a i ʻole hiki ke ulu aʻe ma hope o ke ola..

Nā kumu hoʻoilina

ʻO ka poʻe i hānau ʻia me kēia maʻi, he loli a he mutation paha ko lākou i loko o kahi gene i kapa ʻia ʻo `(BCHE)`. ʻO kēia gene `(BCHE)` ka mea e haʻi aku i ko mākou kino e hana i ka enzyme `(Pseudocholinesterase)`. No laila, i ka wā e loli ai kēia gene, ʻaʻole paha e hana iki kou kino i kēia enzyme, a i ʻole ʻaʻole paha e hana iki.

Nā kumu e ulu ana i ke ola (Loaʻa)

I kekahi manawa, hiki ke loaʻa kēia ʻano (Pseudocholinesterase Deficiency) i ke ola holoʻokoʻa. Hiki ke hana ʻia e nā maʻi like ʻole a me kekahi mau lāʻau lapaʻau.

ʻO kekahi mau maʻi e hiki ke hoʻoulu i kēia ʻano:

• Ka ʻai ʻole ʻana
• Nā ʻeha kuni koʻikoʻi
• Maʻi puʻupaʻa
• Maʻi akepaʻa
• Hypothyroidism (thyroid hana ʻole)
• Ka maʻi ʻaʻai
• Hāpai keiki

ʻO kekahi mau lāʻau lapaʻau e hiki ke hoʻonui i ka pilikia o kēia ʻano:

• Nā mea hoʻopaneʻe MAO (e.g. Phenelzine (Nardil®))
• Nā lāʻau hoʻomalu hānau (Nā lāʻau pale waha)
• Nā kulu maka Echothiophate
• ʻO Metoclopramide
• ʻO Cyclophosphamide
• Pyridostigmine

Pehea e ʻike ai nā kauka i kēia?

Inā pilikia ʻoe i ka kaohi ʻana i kou mau ʻiʻo a i ʻole ka hanu ʻana ma hope o ka anesthesia laulā, hiki i kāu kauka ke kānalua i ka hemahema o ka pseudocholinesterase. No ka hōʻoia ʻana i kēia, hiki iā lākou ke hana i kahi hoʻāʻo koko e nānā i kou pae o ka enzyme pseudocholinesterase.

Inā loaʻa i kekahi o kou ʻohana kēia maʻi, hiki i ke kauka ke hana i nā hoʻāʻo genetic e ʻike ai inā loaʻa iā ʻoe pū kekahi. Pili kēia i ka lawe ʻana i kahi hāpana koko a me ka nānā ʻana e ʻike inā loaʻa iā ʻoe ka mutation gene (BCHE).

Inā kānalua kāu kauka he loaʻa iā ʻoe kēia ʻano maʻi, e hoʻohana lākou i nā ʻano hoʻomaha ʻiʻo ʻē aʻe i hilinaʻi ʻole i ka enzyme "Pseudocholinesterase".

He aha ka lāʻau lapaʻau?

Inā loaʻa iā ʻoe nā hōʻailona o ka `(Pseudocholinesterase Deficiency)` ma hope o kou hāʻawi ʻia ʻana i ka anesthesia laulā, pono paha ʻoe i ke kōkua lapaʻau wikiwiki. E nānā pono ke kime lapaʻau iā ʻoe a hiki i kou hoʻōla ʻana. Inā pono, e mālama lākou iā ʻoe ma lalo o ka anesthesia a hoʻohana i kahi mīkini ``mechanical ventilation`` e kōkua iā ʻoe e hanu a hiki i kou hiki ke hanu iā ʻoe iho.

He aha kāu e manaʻo ai inā loaʻa iā ʻoe kēia maʻi?

Inā ua ʻike ʻia ʻoe me ka `(Pseudocholinesterase Deficiency)`, ʻo ka mea maikaʻi loa hiki iā ʻoe ke hana, ʻo ia ke hoʻomaopopo i kāu mau kauka e pili ana i ia mea - ʻoiai hoʻi nā kauka e ʻike iā ʻoe no ke ʻoki kino .

Eia kekahi, pono ʻoe e haʻi i kou ʻohana e pili ana i kēia., a laila hiki ke hoʻāʻo ʻia lākou e ʻike inā loaʻa iā lākou ka mutation gene `(BCHE)`. Inā pēlā, pono lākou e hoʻomaopopo i kā lākou mau kauka ma mua o ke ʻoki ʻana.

He manaʻo maikaʻi nō hoʻi e komo i kahi kūpeʻe lima ID lapaʻau i hiki i nā kauka ke ʻike inā pono ʻoe e hana i ke ʻoki kino ulia pōpilikia.

Hiki ke pale ʻia kēia?

ʻO ka hemahema o ka pseudocholinesterase kahi maʻi hānau ʻole ʻaʻole hiki ke pale ʻia. Eia nō naʻe, inā loaʻa i kekahi o kou ʻohana, hiki ke hoʻāʻo ʻia ʻoe no ka hoʻololi ʻana o ka gene BCHE ma mua o ka hana ʻana i ka anesthesia laulā. Hiki i kēia ke kōkua i ka pale ʻana i nā pilikia i ka wā o ke ʻoki ʻana.

Inā loaʻa iā ʻoe ka hoʻololi ʻana o ka genetic, e kāpae paha kāu kauka i nā Choline esters a koho i kahi anesthetic ʻokoʻa. ʻO Rocuronium ka anesthetic i hoʻohana pinepine ʻia no ka poʻe me ka Pseudocholinesterase Deficiency.

Hiki paha iā ʻoe ke hōʻemi i kou pilikia o ka loaʻa ʻana o ka Acquired Pseudocholinesterase Deficiency ma hope o ke ola ma o ka mālama ʻana i nā kūlana kumu, e like me ka malnutrition a i ʻole ka maʻi puʻupaʻa mau (CKD). Eia kekahi, inā ʻoe e lawe ana i kekahi mau lāʻau lapaʻau e hōʻemi ana i ka hana ʻana o ka enzyme Pseudocholinesterase, makemake paha ʻoe e kamaʻilio me kāu kauka e pili ana i ka hoʻololi ʻana i kāu lāʻau lapaʻau.

He aha nā contraindications, ʻo ia hoʻi, he aha nā lāʻau lapaʻau maikaʻi ʻole e hoʻohana no ka poʻe me kēia maʻi?

Inā ua ʻike ʻia ʻoe me ka `(Pseudocholinesterase Deficiency)`, pono ʻoe e pale i nā ʻano lāʻau hoʻomāmā :

• ʻO Succinylcholine `(Succinylcholine)`
• ʻO Mivacurium
• ʻO Procaine
• Chloroprocaine
• Benzocaine
• ʻO Tetracaine

Nā nīnau e nīnau aku ai i kāu kauka

Inā loaʻa iā ʻoe kahi maʻi i kapa ʻia ʻo "Pseudocholinesterase Deficiency", inā paha ua hānau ʻia ʻoe me ia a i ʻole ua loaʻa iā ʻoe ma hope, makemake paha ʻoe e nīnau i kāu kauka i kekahi mau nīnau e like me:

• He aha nā contraindications pololei noʻu, ʻo ia hoʻi, he aha nā lāʻau lapaʻau e pono iaʻu e pale aku?
• He maʻi anei kēia i hānau ʻia mai iaʻu a i ʻole ua hoʻokumu ʻia e kekahi maʻi ʻē aʻe?
• He manaʻo maikaʻi anei ka hana ʻana i nā hoʻāʻo genetic ma koʻu ʻohana?

Hiki ke lilo i mea kaumaha iki ke komo ʻana i ke ʻoki kino. Nui ka poʻe o mākou e kau nui ana ma ke ʻoki kino a ʻaʻole mākou e noʻonoʻo i ka hiki ke loaʻa kahi hopena maikaʻi ʻole i ka anesthesia. ʻAʻohe lāʻau lapaʻau no ka Pseudocholinesterase Deficiency. Eia nō naʻe, ʻo ka ʻike inā loaʻa iā ʻoe hiki ke kōkua iā ʻoe e pale aku i ka nāwaliwali o nā ʻiʻo manawa pōkole a me nā pilikia hanu e hiki ke hana ma hope o ke ʻoki kino. Inā loaʻa iā ʻoe ka Pseudocholinesterase Deficiency, e haʻi i kou ʻohana e pili ana i ia mea. Hiki iā lākou ke hoʻāʻo ʻia no ka gene a pale i nā pilikia pono ʻole e kū mai ana i kā lākou huakaʻi olakino.

ʻO ka mea hope loa, nā mea e hoʻomanaʻo ai

No laila, he inoa weliweli iki ka `(Pseudocholinesterase Deficiency)`, akā he mea nui loa ia e makaʻala i ia mea. ʻOi loa aku inā e hana ʻia ana ʻoe i ke ʻoki kino, a inā paha ua loaʻa i kekahi o kou ʻohana kekahi mea e like me kēia , ʻoi aku ka maikaʻi o ke kamaʻilio ʻana me kāu mau kauka e pili ana i kēia. E hoʻomanaʻo, ʻo ka ʻike pinepine ka pale maikaʻi loa e hiki ai ke hoʻopakele iā mākou mai nā pilikia pono ʻole!

ʻAnesthesia , pseudocholinesterase, hemahema enzyme, nā pilikia ʻoki kino, ke kūlana genetic, ka hana ʻino o nā ʻiʻo, nā lāʻau hoʻomaha