Ka maʻi palahēhau hyperkalemic

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

ʻO HyperPP kekahi o nā hui o nā maʻi genetika e pili pū me ka paralysis wā hypokalemic a me ka paralysis kau manawa thyrotoxic.

Hānau ʻia ʻo HyperPP. 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 manawa, he hopena paha ka hopena o kahi pilikia genetics i hoʻoili ʻole ʻia.

Manaʻo ʻia pili ka maʻi i nā pilikia me ke ʻano o ke kino e kaohi ai i ka sodium a me ka pae potassium i nā hunaola.

ʻO nā kumu pilikia e pili pū me ka loaʻa ʻana i nā lālā ʻohana ʻē aʻe me ka maʻi lōlō. Hoʻopili like ia i nā kāne a me nā wahine.

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 pinepine ka hoʻouka ʻana i ka wā kamaliʻi. ʻEhia ka pinepine o nā hoʻouka ʻana. Ua hōʻeha kekahi poʻe i ka lā. ʻAʻole lawa lawa lākou e pono ai ka lāʻau. Ua pili kekahi poʻe i ka myotonia, kahi hiki ʻole ke hoʻomaha koke i ko lākou mauʻiʻo ma hope o ka hoʻohana.

ʻO ka nāwaliwali a i ʻole ka maʻi lōlō:

• Kū pinepine ka mea ma nā poʻohiwi, kua, a me nā pūhaka
• Hiki pū me nā lima a me nā wāwae, akā ʻaʻole pili i nā mākala o nā maka a me nā mākala e kōkua i ka hanu ʻana a me ka ale ʻana
• Hana pinepine ʻia ʻoiai e hoʻomaha ana ma hope o ka hana a hoʻoikaika kino paha
• Kū paha i ka ala ʻana
• Kū a ma waho hoʻi
• He 15 mau minuke ka lōʻihi o ka manawa ma 1 hola, akā mau paha i hoʻokahi lā holoʻokoʻa

Hoʻokomo pū paha nā mea hoʻonāuki i:

• ʻO ka ʻai ʻana i ka ʻai ʻaʻā momona kiʻekiʻe
• Hoʻomaha ma hope o ka hoʻoikaika kino
• Hōʻike i ke anuanu
• Hōʻalo i nā meaʻai
  
• ʻO ka ʻai ʻana i nā meaʻai momona potassium a i ʻole lawe i nā lāʻau i loaʻa i ka potassium
• Kaumaha
  

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

Ma waena o nā hoʻouka kaua, hōʻike ʻia kahi hoʻokolokolo kino i kahi mea maʻamau ʻole. I ka wā a ma waena o nā hoʻouka ʻana, hiki ke kiʻekiʻe a kiʻekiʻe paha ka pae potassium.

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 ka pahuhopu o ka mālama ʻana e hōʻoluʻolu i nā ʻōuli a pale i nā hoʻouka hou aʻe.

Lawe pinepine ʻole nā ​​hoʻouka ʻana e koi aku ai i ka lāʻau lapaʻau pōpilikia. Akā hiki i nā puʻuwai kūlohelohe ʻole (arrhythmia puʻuwai) ke hana pū ʻia i ka wā o nā hoʻouka kaua, kahi e pono ai ka lāʻau ulia pōpilikia. Hiki i ka nāwaliwali o ka ʻiʻo ke lilo i mea ʻino loa me nā hoʻouka hou ʻana, no laila e mālama koke ʻia ka mālama ʻana e pale ai i nā hoʻouka.

ʻO ka glucose a i ʻole nā ​​mea ʻāpiki ʻē aʻe (sugars) i hāʻawi ʻia i ka wā o kahi hoʻouka e hōʻemi paha i ke koʻikoʻi o nā ʻōuli. Pono pono e hāʻawi ʻia nā kalipona a i ʻole nā ​​diuretics (nā pila wai) ma o ke aa e hoʻopau i nā hoʻouka hikiwawe.

I kekahi manawa, nalo nā hoʻouka ma hope o ke ola iā lākou iho. Akā hiki ke hoʻouka hou i ka nāwaliwali o nā mākala mau.

Pane maikaʻi ʻo HyperPP i ka lāʻau. Hiki ke pale i ka mālama ʻana, a i ʻole hoʻi e hoʻi i hope, nāwaliwali o nā mākala holomua.

ʻO nā pilikia olakino e pili ana i ka hyperPP:

• Nā pōhaku hakuʻala (kahi ʻaoʻao o ka lāʻau i hoʻohana ʻia e mālama i ke ʻano)
• Paʻi kuʻi ʻole
• ʻO ka nāwaliwali o ka ʻiʻo e hoʻomau mālie ana i ka ʻino

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 ʻoe e maule a paʻakikī paha i ka hanu ʻana, ʻōlelo ʻana, a me ka ale ʻana paha.

ʻO nā lāʻau acetazolamide a me thiazides e pale i ka hōʻeha i nā manawa he nui. ʻO ka potassium haʻahaʻa, kaʻai carbohydrate kiʻekiʻe, a me ka hoʻolāʻau māmā ke kōkua i ka pale ʻana i nā hōʻeha. ʻO ka pale ʻana i ka hoʻokē ʻai, hana ikaika, a i ʻole ke anuanu ke kōkua pū kekahi.

Paralysis kau - hyperkalemic; Paralysis kauā ʻohana hyperkalemic; HyperKPP; HyperPP; Maʻi Gamstorp; Ka maʻi palahē me ka pālolo pālolo

• Hoʻoiho muscular

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.

Moxley RT, Heatwole C. Channelopathies: nā maʻi myotonic a me ka maʻi palahuli wā. I: Swaiman KF, Ashwal S, Ferriero DM, et al, eds. ʻO Swaiman's Pediatric Neurology: Nā Kumuhana a me nā Hana. ʻElua huaʻōlelo Piladelapia, PA: Elsevier; 2017: chap 151.