ʻO ka maʻi Gamstorp (Hyperkalemic Periodic Paralysis)

Anter

• He aha ka maʻi Gamstorp?
• He aha nā hōʻailona o ka maʻi Gamstorp?
• Pālolo
• Myotonia
• He aha nā kumu o ka maʻi Gamstorp?
• ʻO wai ka mea e makaʻu i ka maʻi Gamstorp?
• Pehea ʻike ʻia ka maʻi Gamstorp?
• Ke hoʻomākaukau nei e ʻike i kāu kauka
• He aha nā hōʻailona no ka maʻi Gamstorp?
• Nā Lapaʻau
• Nā hoʻoponopono home
• Ke kāohi nei i ka maʻi Gamstorp
• He aha ka manaʻo lōʻihi?

He aha ka maʻi Gamstorp?

ʻO ka maʻi Gamstorp kahi ʻano laha ʻole loa e hiki ai iā ʻoe ke loaʻa i nā ʻāpana o ka nāwaliwali o nā mākala a i ʻole ka palu manawa. ʻIke ʻia ka maʻi e nā inoa he nui, e like me ka maʻi palapū hyperkalemic.

He maʻi hoʻoilina ia, a he hiki i ka poʻe ke hāpai a hele i ka ʻāpana me ka ʻike ʻole ʻia o nā ʻōuli. Hoʻokahi ma 250,000 poʻe i loaʻa i kēia ʻano.

ʻOiai ʻaʻohe lāʻau lapaʻau no ka maʻi Gamstorp, ʻo ka hapanui o ka poʻe i loaʻa iā ia e hiki ke ola maʻamau, nā ola hana.

ʻIke nā kauka i nā kumu he nui no nā māhele paralytic a hiki ke kōkua maʻamau i ka palena o nā hopena o ka maʻi e ke alakaʻi ʻana i ka poʻe me kēia maʻi e hōʻalo i kekahi mau mea i hōʻike ʻia.

He aha nā hōʻailona o ka maʻi Gamstorp?

Hoʻokumu ka maʻi Gamstorp i nā hōʻailona kūikawā, e like me:

• nāwaliwali loa o kahi lālā
• paralysis hapa
• puʻuwai kūlohelohe ʻole
• hōʻalo i nā kuʻi puʻuwai
• ʻoʻoleʻa o ka mākala
• nāwaliwali paʻa mau
• immobility

Pālolo

Pōkole nā ​​māhele paralytic a e pau paha ma hope o kekahi mau minuke. ʻOiai he kau lōʻihi kāu, pono ʻoe e ola holoʻokoʻa i loko o 2 mau hola o nā ʻōuli e hoʻomaka nei.

Eia nō naʻe, kū koke nā hanana. E ʻike paha ʻoe ʻaʻole lawa kāu kau ʻōlelo aʻoaʻo e ʻike i kahi wahi palekana e kali ai i kahi hanana. No kēia kumu, maʻamau nā ʻeha mai ka hāʻule.

Hoʻomaka ka hoʻomaka ʻana o nā ʻāpana i ka wā kamaliʻi a i ʻole ke kamaliʻi. No ka hapanui o ka poʻe, hoʻonui ka pinepine o nā ʻāpana i nā makahiki ʻōpio a i ke kau waena o 20.

Ke hoʻokokoke aku nei ʻoe i kāu mau makahiki 30, e emi pinepine ka hoʻouka ʻana. No kekahi poʻe, nalo loa lākou.

Myotonia

ʻO kekahi o nā ʻōuli o ka maʻi Gamstorp ʻo myotonia.

Inā loaʻa iā ʻoe kēia ʻōuli, hiki i kekahi o kou mau pūʻulu muscle ke paʻakikī i ka manawa pōkole a paʻakikī e neʻe. He mea ʻeha loa kēia. Eia nō naʻe, ʻaʻole manaʻo kekahi poʻe i kekahi ʻano ʻino i ka wā o kahi hanana.

Ma muli o nā kuʻikahi mau, nāʻiʻo i hoʻopili ʻia e myotonia e nānā pinepine a wehewehe ʻia me ka ikaika, akā ʻike paha ʻoe hiki iā ʻoe ke hoʻoikaika iki a i ʻole ka hoʻohana ʻana i kēia mauʻiʻo.

ʻO Myotonia ke kumu o ka pohō mau i nā manawa he nui. Hoʻohana kekahi poʻe me ka maʻi Gamstorp i nā noho huila ma muli o ka hōʻino ʻana o ko lākou mau mākala wāwae.

Hiki i ka mālama ʻana ke pale a pale hou paha i ka nāwaliwali o nā mākala holomua.

He aha nā kumu o ka maʻi Gamstorp?

ʻO ka maʻi Gamstorp ka hopena o kahi hoʻololi, a hoʻololi paha, i kahi moʻokūʻauhau i kapa ʻia ʻo SCN4A. Kōkua kēia gen i ka hoʻohua ʻana i nā kaha sodium, a i ʻole nā ​​puka microscopic e neʻe ai ka sodium ma loko o kāu mau hunaola.

ʻO nā kahe uila i hana ʻia e nā sodium a me nā mole potassium ʻokoʻa e hele ana ma waena o nā membrane cell e kaohi i ka neʻe ʻana o nā mākala

I ka maʻi Gamstorp, loaʻa kēia mau kahawai i nā kino kīnā ʻole e hoʻākoakoa ai ka potassium ma kekahi ʻaoʻao o ka membrane pūnaewele a kūkulu i loko o ke koko.

Pale kēia i ka uila uila e pono ai mai ka hoʻokumu ʻana a hiki ʻole iā ʻoe ke neʻe i ka mākala i hoʻopili ʻia.

ʻO wai ka mea e makaʻu i ka maʻi Gamstorp?

ʻO ka maʻi Gamstorp kahi maʻi hoʻoilina, a he autosomal mana ia. ʻO kēia ke kumu he hoʻokahi wale nō kope o ka gen mutated e hoʻomohala i ka maʻi.

Aia he 50 pākēneka loaʻa iā ʻoe ka ʻāpana inā he lawe kekahi o kou mau mākua. Eia nō naʻe, ʻaʻole hiki i kekahi poʻe i loaʻa ka ʻāpana ke hoʻomohala i nā ʻōuli.

Pehea ʻike ʻia ka maʻi Gamstorp?

No ke kuhikuhi ʻana i ka maʻi Gamstorp, e kāpae mua kāu kauka i nā maʻi adrenal e like me ka maʻi ʻo Addison, kahi e hana ai ke hana ʻole kāu mau adrenal glands i ka nui o nā homone cortisol a me aldosterone.

E hoʻāʻo nō hoʻi lākou e kāpae i nā maʻi maʻi ʻōiwi e hiki ai ke hōʻemi i nā pae potassium.

I ka manawa e kāpae i kēia mau maʻi adrenal a me nā maʻi maʻi ʻōiwi i hoʻoili ʻia, hiki i kāu kauka ke hōʻoia inā ʻo Gamstorp ka maʻi ma o nā hoʻokolohua koko, DNA analysis, a i ʻole ma ka loiloi ʻana i kāu mau electrolyte serum a me nā pae potassium.

I mea e loiloi ai i kēia mau pae, e hoʻokau paha kāu kauka iā ʻoe e hana i nā hoʻokolohua e pili ana i ka hoʻoikaika kino i ukali ʻia e ka hoʻomaha e ʻike ai i ka loli ʻana o kāu pae potassium.

Ke hoʻomākaukau nei e ʻike i kāu kauka

Inā manaʻo ʻoe he maʻi Gamstorp paha kāu, kōkua paha ia i ka mālama ʻana i kahi diary i kāu mau pae ikaika i kēlā me kēia lā. E mālama ʻoe i nā memo e pili ana i kāu mau hana a me kāu papaʻai i kēlā mau lā e kōkua ai i ka hoʻoholo ʻana i kāu mau ʻōpala.

Lawe pū ʻoe i kekahi ʻike i hiki iā ʻoe ke hōʻiliʻili e pili ana inā ʻaʻole ʻoe i ka mōʻaukala ʻohana o ka maʻi.

He aha nā hōʻailona no ka maʻi Gamstorp?

Hoʻokumu ʻia ka mālama ʻana i ka paʻakikī a me ke alapine o kāu mau māhele. Hana maikaʻi nā lāʻau a me nā mea hoʻopihapiha no nā poʻe he nui i loaʻa i kēia maʻi. Kūpono ka pale ʻana i kekahi mau mea hoʻonāukiuki no haʻi.

Nā Lapaʻau

Pono ka hapa nui o ka poʻe e hilinaʻi i ka lāʻau e kāohi ai i nā hōʻeha palalā. ʻO kekahi o nā lāʻau i kuhikuhi pinepine ʻia ʻo acetazolamide (Diamox), kahi i hoʻohana mau ʻia e kaohi i ka hopu ʻana.

Kauoha paha kāu kauka i nā diuretics e kaupalena i nā pae potassium i ke koko.

Hiki ke mālama ʻia ka poʻe me myotonia ka hopena o ka maʻi me ka hoʻohana ʻana i nā lāʻau liʻiliʻi e like me mexiletine (Mexitil) a i ʻole paroxetine (Paxil), kahi e kōkua ai i ka hoʻopaʻa ʻana i nā spasms ʻehaʻeha loa.

Nā hoʻoponopono home

ʻO ka poʻe i ʻike i nā ʻāpana ʻoluʻolu a pinepine ʻole paha hiki i kekahi manawa ke kāohi i kahi maʻi lōlō me ka hoʻohana ʻole o ka lāʻau.

Hiki iā ʻoe ke hoʻohui i nā mea hoʻopili mineral, e like me ka calcium gluconate, i kahi mea inu momona e hoʻōki i kahi ʻāpana māmā.

ʻO ka inu ʻana i ke kīʻaha o ka wai tonic a i ʻole ka omo ʻana i kahi ʻāpana o nā kēpau paʻakikī i nā hōʻailona mua o kahi paralytic episode e kōkua pū kekahi.

Ke kāohi nei i ka maʻi Gamstorp

Hiki i nā meaʻai waiwai potassium a i ʻole kekahi mau ʻano ke hoʻomaka i nā hanana. ʻO ka nui o ka potassium i ke kahe o ke koko e hoʻonāwaliwali i nā mākala a ʻo ka poʻe i loaʻa ʻole ka maʻi Gamstorp.

Eia nō naʻe, hiki i ka poʻe me ka maʻi ke hana i nā loli liʻiliʻi loa i nā pae potassium e pili ʻole i ka mea i loaʻa ʻole i ka maʻi Gamstorp.

ʻO nā mea hoʻouluulu maʻamau e:

• nā hua kiʻekiʻe i loko o ka potasiuma, e like me ka maiʻa, nā apricot, a me nā hua hua waina
• ʻO nā mea kanu potassium, e like me ka milo, kaʻuala, broccoli, a me ka pua kō
• nā lihi, nā pi, a me nā nati
• ʻalekohola
• wā lōʻihi o ka hoʻomaha a hana ʻole paha
• hele lōʻihi me ka ʻai ʻole
• anu loa
• wela loa

ʻAʻole like nā mea like me nā poʻe āpau me ka maʻi Gamstorp. E kamaʻilio me kāu kauka, a hoʻāʻo e hoʻopaʻa i kāu mau hana a me kāu papaʻai i kahi diary e kōkua ai i ka hoʻoholo ʻana i kāu mau kikoʻī kikoʻī.

He aha ka manaʻo lōʻihi?

Ma muli o ka hoʻoilina o ka maʻi Gamstorp, ʻaʻole hiki iā ʻoe ke pale aku. Eia nō naʻe, hiki iā ʻoe ke kaulike i nā hopena o ke ʻano me ka mālama pono ʻana i kāu mau pilikia. Hoʻemi ka ʻelemakule i ke alapine o nā māhele.

He mea nui e kamaʻilio me kāu kauka e pili ana i nā meaʻai a me nā hana i hoʻokumu ʻia i kāu mau hanana. Ke pale nei i nā mea hoʻonāukiuki i kumu i nā maʻi paralytic hiki ke kaupalena i nā hopena o ka maʻi.