Maʻi ʻo Creutzfeldt-Jakob

ʻO ka maʻi ʻo Creutzfeldt-Jakob (CJD) kahi ʻano o ka poino o ka lolo e hiki ai i ka hōʻemi wikiwiki ʻana o ka neʻe a me ka nalo ʻana o ka hana noʻonoʻo.

Hoʻokumu ʻia ʻo CJD e kahi protein i kapa ʻia he prion. Hoʻokumu kahi prion i nā protein maʻamau e pelu maʻamau. Hoʻopili kēia i nā protein ʻē aʻe i ka hiki ke hana.

Kākaʻikahi loa ʻo CJD. Nui a hewahewa nā ʻano. ʻO nā ʻano maʻamau o ka CJD:

• Hana ʻo Sporadic CJD i ka hapa nui o nā hihia. Kū ʻia ia no ke kumu ʻole i ʻike ʻia. ʻO ka makahiki maʻamau e hoʻomaka ai ia he 65.
• Kū ʻo Familial CJD ke hoʻoilina ke kanaka i ka prion abnormal mai kahi makua (kākaʻikahi kēia ʻano CJD).
• Loaʻa iā CJD i loaʻa iā CJD ʻokoʻa (vCJD), ke ʻano e pili ana i ka maʻi bipi wahine. ʻO Iatrogenic CJD kahi ʻano i loaʻa o ka maʻi. Hoʻoholo ʻia ʻo Iatrogenic CJD i kekahi manawa ma o kahi transfusion huahana koko, transplant, a i ʻole nā ​​mea ʻoki lāʻau hoʻohaumia.

Hoʻokumu ʻia ka Variant CJD e ka ʻai ʻana i ka ʻiʻo i loaʻa i ka maʻi. ʻO ka maʻi i hōʻeha i ka maʻi i nā bipi wahine i manaʻo ʻia he like ia e kumu ai ka vCJD i loko o nā kānaka.

Hoʻohuli ka Variant CJD ma lalo o ka 1 pakeneka o nā hihia CJD āpau. Hoʻopili ia i ka poʻe ʻōpio. Ma mua o 200 mau kānaka ma ka honua holoʻokoʻa i loaʻa i kēia maʻi. ʻAneʻane pili nā hihia āpau ma ʻEnelani a me Palani.

Pili paha ʻo CJD i kekahi mau maʻi ʻē aʻe i hoʻokumu ʻia e prion, me:

• ʻO ka maʻi hōʻino mau (loaʻa i nā dia)
• ʻO Kuru (pili i ka hapanui o nā wahine ma New Guinea i ʻai i ka lolo o nā ʻohana i make ma ke ʻano he ʻāpana o kahi hoʻolewa)
• Scrapie (loaʻa i nā hipa)
• ʻO nā maʻi kanaka like ʻole i hoʻoili ʻia, e like me ka maʻi ʻo Gerstmann-Straussler-Scheinker a me ka hiamoe ʻohana fatal

Hiki i nā ʻōuli o ka CJD ke hoʻopili i kekahi o kēia mau mea:

• ʻO Dementia e hele a ʻoi aku ka maikaʻi ma mua o kekahi mau pule a i ʻole mau mahina
• ʻIke makapō (kekahi manawa)
• Nā hoʻololi i ka hele (hele wāwae)
• Huikau, disorientation
• Hallucination (ʻike a hoʻolohe ʻana i nā mea ʻaʻole ma laila)
• Ka nele o ka hoʻonohonoho ʻana (e laʻa me ke kuʻi a hāʻule ʻana)
• ʻĀʻī o ka ʻili, wili
• Manaʻo hopohopo, lele
• Hoʻololi ka pilikino
• Hiamoe
• ʻO ka neʻe koke ʻana a i ʻole ka hopu ʻana
• Pilikia i ka ʻōlelo ʻana

I ka wā mua o ka maʻi, kahi ʻōnaehana a me ka noʻonoʻo noʻonoʻo e hōʻike i nā pilikia hoʻomanaʻo a me ka noʻonoʻo. Ma hope i ka maʻi, hōʻike paha kahi ʻōnaehana moto (kahi hōʻike e hoʻāʻo i nā reflexes i nā mākala, ka ikaika, ka hoʻohui ʻana, a me nā hana kino ʻē aʻe).

• Nā reflexes maʻamau a hoʻonui paha i nā pane reflex maʻamau
• Hoʻonui i ke kani o ka mākala
• Pākuʻi i ka mākala a me nā spasms
• Pane haʻalulu ikaika
• Ka nāwaliwali a me ka nalo ʻana o nāʻiʻo puʻupuʻu.

Loaʻa ka lilo o ka hoʻohui a me nā loli i ka cerebellum. ʻO kēia kahi o ka lolo e kaohi ana i ka hoʻohui ʻana.

Hōʻike kahi hoʻokolohua maka i nā wahi o ka makapō a ka kanaka e ʻike ʻole ai.

ʻO nā hoʻokolohua i hoʻohana ʻia e kuhikuhi i kēia ʻano hiki ke hoʻopili ʻia:

• Nā hoʻokolohua koko e kāpae i nā ʻano dementia ʻē aʻe a e nānā i nā māka i kekahi manawa me ka maʻi
• CT scan o ka lolo
• Electroencephalogram (EEG)
• MRI o ka lolo
• Spinal tap e hoʻāʻo no kahi protein i kapa ʻia 14-3-3

Hiki ke hōʻoia ʻia ka maʻi me ka biopsy lolo a me ka autopsy. I kēia lā, kākaʻikahi loa ka hana ʻia ʻana o kahi biopsy lolo e nānā no kēia maʻi.

ʻAʻohe lāʻau i ʻike ʻia no kēia maʻi. Ua hoʻāʻo ʻia nā lāʻau ʻokoʻa e hoʻolohi i ka maʻi. Hoʻopili kēia i nā lāʻau antibiotic, nā lāʻau no ka maʻi epilepsy, nā mea lahilahi o ke koko, nā antidepressants, a me nā interferon. Akā ʻaʻohe mea e holo pono.

ʻO ka pahuhopu o ka mālama ʻana e hoʻolako i kahi nohona palekana, kaohi ʻana i ka lawena hōʻeuʻeu a hoʻonāukiuki paha, a hoʻokō i nā makemake o ke kanaka. Pono paha kēia i ka nānā ʻana a me ke kōkua ma ka home a i ʻole kahi wahi mālama. Hiki paha i ka ʻōlelo aʻoaʻo ʻohana ke kōkua i ka ʻohana e kū i nā hoʻololi e pono ai no ka mālama home.

Pono paha ka poʻe me kēia ʻano i ke kōkua i ke kaohi ʻana i nā ʻano hana ʻae ʻole a weliweli paha. Hoʻopili kēia i ka uku ʻana i nā lawena maikaʻi a me ka nānā ʻana i nā lawena maikaʻi ʻole (ke palekana ia). Pono paha lākou i kōkua e kōkua i ke ʻano i ko lākou wahi. I kekahi manawa, pono nā lāʻau e kōkua i ke kāohi i ka huhū.

Pono paha nā poʻe me CJD a me ko lākou ʻohana e ʻimi i nā ʻōlelo loio i ka wā mua o ka maʻi. ʻO ka papa kuhikuhi mua, ka mana o ka loio, a me nā hana kū kānāwai e hiki ke maʻalahi i ka hoʻoholo ʻana e pili ana i ka mālama o ke kanaka me CJD.

ʻO ka hopena o ka CJD ʻilihune loa. ʻAʻole hiki i ka poʻe me ka CJD sporadic ke mālama iā lākou iho ma waena o 6 mau mahina a ʻoi iki paha ma hope o ka hoʻomaka ʻana o nā ʻōuli.

Make ka maʻi i kahi manawa pōkole, ma waena o 8 mau mahina. ʻO ka poʻe i loaʻa ka CJD ʻano ʻē e hele lohi a hewa iki, akā make mau ke ʻano. Ola kekahi mau kānaka no ka lōʻihi o 1 a 2 mau makahiki paha. ʻO ke kumu o ka make ka maʻi maʻamau, ka puʻuwai puʻuwai, a me ka hanu ʻole.

ʻO ka papa o CJD:

• ʻO ka maʻi me ka maʻi
• Kaʻai maikaʻi ʻole
• Dementia i kekahi mau hihia
• Loaʻa ka hiki ke launa pū me nā poʻe ʻē aʻe
• Ka nalowale o ka hiki ke hana a mālama paha iā ʻoe iho
• Make

ʻAʻole ʻo CJD kahi pilikia olakino. Eia nō naʻe, e loaʻa ana i ka diagnose a mālama ʻia i ka wanaʻao e hoʻomaʻalahi paha i nā ʻōuli, e hāʻawi i nā manawa i nā mea maʻi e hana i nā kuhikuhi mua a hoʻomākaukau no ka hopena o ke ola, a hāʻawi i nā manawa keu i nā ʻohana e hiki i nā ʻōlelo me ke ʻano.

Pono e hemo i nā pono lapaʻau e hoʻohaumia ʻia mai ka lawelawe a hoʻolei ʻia. ʻAʻole pono ka poʻe i ʻike he CJD e hāʻawi manawaleʻa i ke kornea a i ʻole nā ​​kino ʻē aʻe.

ʻO ka hapa nui o nā ʻāina i kēia manawa nā kulekele koʻikoʻi no ka mālama ʻana i nā bipi maʻi e pale i ka lawe ʻana i ka CJD i nā kānaka.

Transmissible spongiform encephalopathy; vCJD; CJD; Maʻi ʻo Jacob-Creutzfeldt

• Maʻi ʻo Creutzfeldt-Jakob
• ʻŌnaehana ʻōnaehana waena a me ka ʻōnaehana ʻōnaehana peripheral

ʻO Bosque PJ, ʻo Tyler KL. ʻO Prions a me ka maʻi prion o ka ʻōnaehana waena (transmissible neurodegenerative maʻi). I: Bennett JE, Dolin R, Blaser MJ, hua pā. ʻO Mandell, Douglas, a me Bennett's Principle a me ka hoʻomaʻamaʻa ʻana i nā maʻi kūpilikiʻi. 9th ed. Piladelapia, PA: Elsevier; 2020: chap 179.

ʻO Geschwind MD. Nā maʻi Prion. 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 94.