Lōkō manawa hypokalemic

ʻO ka hypalalemic periodic paralysis (hypoPP) kahi maʻi e kumu i nā manawa o ka nāwaliwali o nā mākala a i kekahi manawa ʻoi aku ka haʻahaʻa ma mua o ka pae maʻamau o ka potassium i ke koko. ʻO ka inoa olakino no ka pae potassium haʻahaʻa ʻo hypokalemia.

ʻO HypoPP kekahi o nā hui o nā maʻi genetika e pili pū me ka hyperkalemic periodic paralysis a me ka thyrotoxic period paralysis.

ʻO HypoPP ke ʻano maʻamau o ka maʻi lōlō wā. Hoʻopili pinepine ia i nā kāne.

Hānau mai ʻo HypoPP. ʻO kēia ke ʻano aia ia ma ka wā hānau. I ka hapanui o nā hihia, hoʻoili ʻia i waena o nā ʻohana (hoʻoilina) ma ke ʻano he autosomal domant maʻi. I nā huaʻōlelo ʻē aʻe, hoʻokahi wale nō mākua e pono e hāʻawi i ka ʻāpana e pili ana i kēia ʻano i kā lākou keiki i mea e pilikia ai ke keiki.

I kekahi mau hihia, he hopena paha ka hopena o kahi pilikia genetics i hoʻoili ʻole ʻia.

ʻAʻole like me nā ʻano like ʻole o ka paralysis kau, ʻo ka poʻe me ka hypoPP ka hana maʻamau o ka thyroid. Akā loaʻa iā lākou kahi pae haʻahaʻa o ka potassium haʻahaʻa i ka manawa o nā nāwaliwali. ʻO kēia nā hopena mai ka neʻe ʻana o ka potassium mai ke koko i nā hunaola i kahi ala maʻamau.

ʻO nā kumu pilikia e pili pū me ka loaʻa ʻana i nā lālā ʻohana ʻē aʻe me ka maʻi lōlō. ʻOi aku ka kiʻekiʻe o ka makaʻu i nā kāne ʻAsia i loaʻa nā maʻi thyroid.

Pākuʻi ʻia nā ʻōuli me ka hōʻeha ʻana i ka nāwaliwali o nā mākala a i ʻole ka nalo ʻana o ka neʻe ʻana o nā mākala (paralysis) i hele a hele. Aia ka ikaika o ka mākala ma waena o nā hoʻouka.

Hoʻomaka ka hoʻomaka ʻana o nā hoʻouka i nā makahiki ʻōpio, akā hiki iā lākou ke hana ma mua o ka makahiki 10. Ehia mau manawa e loli ai nā hoʻouka. Hoʻouka kekahi poʻe i kēlā me kēia lā. ʻO kekahi poʻe iā lākou i hoʻokahi manawa i ka makahiki. I ka hoʻouka kaua ʻana e kiaʻi ke kanaka.

ʻO ka nāwaliwali a i ʻole ka maʻi lōlō:

• Hana ʻia ka hapanui ma nā poʻohiwi a me nā pūhaka
• Hiki paha i ka lima, nā wāwae, nā mākala o nā maka, a me nā mākala e kōkua i ka hanu ʻana a me ka ale ʻana
• Kū a hele aku
• Hana ʻia ka hapanui ma ke ala ʻana a ma hope paha o ka hiamoe a hoʻomaha paha
• Kākaʻikahi ka wā hoʻoikaika kino, akā e hoʻomaka ʻia paha e ka hoʻomaha ma hope o ka hoʻoikaika kino
• Hiki ke hoʻoulu ʻia e ka kiʻekiʻe-carbohydrate, nā paʻakai paʻakai kiʻekiʻe, ke kaumaha, hāpai keiki, hoʻoikaika kino, a me ke anuanu
• Kū mau kahi hoʻouka no nā hola he nui a i ka lā

Hiki i kekahi ʻōuli ke komo pū me ka myotonia eyelid (kahi ʻano a ma hope o ka wehe ʻana a paʻa ʻana o nā maka, ʻaʻole hiki ke wehe ʻia no kahi manawa pōkole).

Manaʻo paha ka mea mālama ola i ka hypoPP e pili ana i ka mōʻaukala ʻohana o kēia maʻi. ʻO nā hōʻailona ʻē aʻe i ka maʻi he nāwaliwali nāwaliwali o nā mākala e hele a hele me nā hopena maʻamau a haʻahaʻa paha o kahi hoʻāʻo potassium.

Ma waena o nā hoʻouka kaua, hōʻike ʻia kahi hoʻokolokolo kino i kahi mea maʻamau ʻole. Ma mua o ka hoʻouka kaua ʻana, aia paha he ʻoʻoleʻa o ka wāwae a kaumaha paha i nā wāwae.

I ka hoʻouka kaua ʻana o ka nāwaliwali o nā mākala, haʻahaʻa ka pae o ka potassium o ke koko. Hōʻoia kēia i ka ʻike. ʻAʻole ka emi o ka nui o ka potassium o ke kino. Kūleʻa ka pae potassium o ke koko ma waena o nā hoʻouka.

I ka wā o ka hoʻouka kaua, hoʻemi ʻia a hoʻi ʻole paha nā reflexes muscle. A hele a palupalu nāʻiʻo ma mua o ka paʻakikī. ʻO nā hui Muscle kokoke i ke kino, e like me nā poʻohiwi a me nā pūhaka, pili pinepine ʻia ma mua o nā lima a me nā wāwae.

ʻO nā hoʻāʻo e hana ʻia ana paha:

• Electrocardiogram (ECG), he mea maʻamau ʻole paha i ka wā o ka hoʻouka ʻana
• ʻO Electromyography (EMG), ka mea maʻamau ma waena o nā hoʻouka a me nā mea maʻamau i ka wā o ka hoʻouka ʻana
• ʻO ka biopsy Muscle, kahi e hōʻike ai i nā mea kūpono ʻole

Kauoha ʻia nā hoʻokolohua ʻē aʻe e kāpae i nā kumu ʻē aʻe.

ʻO nā pahuhopu o ka mālama ʻana ke kōkua o nā ʻōuli a me ka pale ʻana i nā hoʻouka hou aʻe.

ʻO ka nāwaliwali o ka ʻikena e pili ana i ka hanu ʻana a me ka ale ʻana i nā mākala kahi hanana pilikia. ʻO nā puʻuwai puʻuwai weliweli ʻole (arrhythmia puʻuwai) hiki ke hana ʻia i ka wā o ka hoʻouka. Pono e mālama koke ʻia kekahi o kēia mau mea.

Hāʻawi ʻia ka potassium i ka wā hoʻouka kaua e hōʻoki paha i ka hoʻouka. Hiki ke lawe ʻia ka potassium e ka waha. Akā inā koʻikoʻi ka nāwaliwali, pono e hāʻawi ʻia ka potassium ma o kahi aa koko (IV).

Hiki ke kōkua i ka lawe ʻana i nā pāʻina potassium e pale i ka nāwaliwali o nā mākala.

ʻO ka ʻai ʻana i kahi papaʻai low-carbohydrate hiki ke kōkua i ka hōʻemi ʻana i nā ʻōuli.

Kākau ʻia kahi lāʻau i kapa ʻia acetazolamide e pale ai i ka hōʻeha. E haʻi paha kāu mea lawelawe iā ʻoe e lawe pū i nā mea pāheona potassium no ka mea hiki i ka acetazolamide ke lilo i kou kino e nalowale i ka potassium.

Inā ʻaʻole holo pono ka acetazolamide iā ʻoe, e kuhikuhi ʻia nā lāʻau ʻē aʻe.

Pane maikaʻi ka HypoPP i ka lāʻau. Hiki i ka mālama ʻana ke pale, a hoʻi i hope, nāwaliwali o nā mākala holomua. ʻOiai ʻo ka ikaika o ka mākala e hoʻomaka maʻamau ma waena o nā hoʻouka ʻana, hiki i nā hoʻouka hou ʻana ke kumu i ka ʻino a me ka nāwaliwali o nā mākala mau ma waena o nā hoʻouka.

ʻO nā pilikia olakino e pili ana i kēia ʻano nā:

• Nā pōhaku hakuʻala (kahi ʻaoʻao o acetazolamide)
• Kuʻi puʻuwai ʻole i ka wā hoʻouka
• Ka hanu paʻakikī ʻana, ʻōlelo ʻana, a me ka ale ʻana i ka wā hoʻouka (kakaʻikahi)
• Ka nāwaliwali o ka ʻiʻī e ʻoi aku ai i ka manawa

Kāhea i kāu mea hāʻawi inā loaʻa ʻoe a i ʻole paha paha i kāu keiki i ka nāwaliwali o ka ʻiʻo e hele mai a hele, keu hoʻi inā he ʻohana kou i loaʻa i ka maʻi lōlō wā.

E hele i ka lumi pilikia a kāhea paha i ka helu kūloko kūloko (e like me 911) inā pilikia ʻoe i ka hanu ʻana, ʻōlelo ʻana, a ale paha i kāu keiki.

ʻAʻole hiki ke pale ʻia ka hypoPP. Ma muli o ka hoʻoilina e hoʻoili ʻia ai, e aʻoaʻo ʻia paha ka ʻōlelo aʻoaʻo kūpuna no nā kāne a me ka pilikia o ka maʻi.

Pale ka mālama ʻana i nā hōʻeha o ka nāwaliwali. Ma mua o ka hoʻouka kaua ʻana, aia paha he ʻoʻoleʻa o ka wāwae a kaumaha paha i nā wāwae. Ke hana nei i ka hoʻoikaika kino i ka hoʻomaka ʻana o kēia mau ʻōuli ke kōkua i ka pale ʻana i ka hoʻouka kaua piha.

Paralysis kau - hypokalemic; Paralysis kau manawa hypokalemic Familial; HOKPP; HypoKPP; HypoPP

Amato AA. Nā maʻi o ka mākala iwi. I: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. ʻO Bradley's Neurology i ka hana lapaʻau. ʻEpekema 7 Piladelapia, PA: Elsevier; 2016: chap 110.

Kerchner GA, Ptácek LJ. Channelopathies: nā maʻi episodic a me nā uila o ka ʻōnaehana hopohopo. I: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SK, eds. ʻO Bradley's Neurology i ka hana lapaʻau. ʻEpekema 7 Piladelapia, PA: Elsevier; 2016: chap 99.

Tilton AH. ʻO nā maʻi neuromuscular ākea a me nā maʻi. I: Fuhrman BP, Zimmerman JJ, eds. Mālama Nui ʻo Pediatric. 5th lā. Piladelapia, PA: Elsevier; 2017: chap 71.