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