ʻO Porphyria (The Vampire Syndrome): Nā kumu, nā ʻōuli, nā hōʻailona, ​​nā mea ʻino a me ka mālama ʻana.

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ʻO ka maʻi vampire kahi maʻi laha ʻole o ke koko e pili mau i ka ʻili a me nā ʻōnaehana. I nā huaʻōlelo lapaʻau, ʻike ʻia ʻo porphyria ^[1] . Kāhea ʻia ke ʻano he 'vampire' ma muli o kāna mau ʻōuli i like me ka vampire mythological o ke kenekulia 18.

Ua ʻike ʻia ʻo Porphyria i ka wā ma mua loa, ma mua o ka hoʻokumu ʻia ʻana o antibiotic, hoʻomaʻemaʻe a me ka hau. I kēlā mau lā, ua manaʻo ʻia ka poʻe me kēia ʻano he 'vampire' ma muli o kā lākou mau ʻōuli e like me nā vampire e pili ana i nā ʻāʻī, nā pōʻaiʻeleʻele a puni nā maka, ka mimi ʻulaʻula a me ka noʻonoʻo ʻole i ka lā. Eia nō naʻe, ua aʻo ʻia ke ʻano e nā loea lapaʻau a ua hoʻokumu ʻia kāna mau lapaʻau ^[elua] .

Nā Manaʻo ʻepekema ma hope o Porphyria

Wahi a Desiree Lyon Howe, ka mea nāna i hoʻokumu i ka American Porphyria Foundation a me ka mea i loaʻa i ka porphyria intermittent koʻikoʻi, ua laha kēia maʻi laha ʻole i waena o nā kaiāulu mamao ma ʻEulopa i ka wā waena i ka wā i noho ka poʻe ma kahi mamao loa mai ka pilina o kēia manawa honua ^[25] .

ʻO Roger Luckhurst, he polofesa ma Modern and Contemporary Literature (Ladana) a me ka hoʻoponopono o ka puke 'Dracula' na Bram Stoker i haʻi ʻia i kekahi mau kumu a me nā hanana i kuleana no porphyria i nā makahiki 1730. Ua ʻōlelo ʻo ia i ka wā o ka wā medieval, ua luku ʻia kahi wahi mamao loa o ʻEulopa a ua lilo ka pōloli, ka maʻi ahulau a me nā maʻi ʻino he nui e like me catalepsy (ʻoʻoleʻa o ke kino a nalowale o ka naʻau) ^[26] .

Ma muli o ka loaʻa ʻole o ka launa ʻana me ka honua waho a me ka lawa ʻole o nā lāʻau, ua kīnā ka poʻe me ka porphyria ma muli o ka makaʻu, kaumaha a me nā kumu ʻē aʻe a hoʻomaka e ʻai iā lākou iho no ka pōloli loa. Eia kekahi, ma muli o ka ʻike ʻole ʻia o ka hana hoʻōla hou ʻana a me nā lāʻau, ua pāhola ʻia nā maʻi ma muli o nā nahu o nā holoholona e like me ka rabies i ka manawa i hoʻonāukiuki ai lākou i ka wai a me nā mālamalama, nā hallucination a me ka huhū.

ʻO kekahi kumu, e like me ka mea i ʻōlelo ʻia e Professor Roger Luckhurst, e hōʻike ana i ka noho kaʻawale ʻana o kēia mau kaiāulu o ʻEulopa no ka lōʻihi, ua kumu ia ka malnutrice ma muli o ka maikaʻi ʻole o ka papaʻai a ʻoi aku ka maʻalahi o nā maʻi he nui ma muli o ka hopena o kā lākou genes no ka mea ʻoi aku ka maikaʻi o ka vampire- e like me nā ʻōuli.

I ka hala ʻana o ka manawa a me ka male ʻana, ua hala nā mea ʻē aʻe ma ka moʻokūʻauhau mai nā mākua a hiki i nā keiki a hoʻokumu i ka maʻi.

Ke kumu o Porphyria

I nā kānaka, hoʻoneʻe ʻia ka oxygen mai nā akemāmā i nā ʻāpana kino ʻē aʻe ma o kahi protein kūikawā i loko o nā hunaola ʻulaʻula i kapa ʻia ʻo hemoglobin ʻo ia ke kuleana no ka ʻulaʻula o ke koko. Loaʻa iā Hemoglobin kahi hui prosthetic i kapa ʻia ʻo heme i loko o ka porphyrin a me ka hao-ion ma waenakonu. Hana mau ʻia ia i nā hunaola ʻulaʻula, ka iwi iwi a me ke ake.

Hana ʻia ʻo Heme i ʻewalu mau hanana i kēlā me kēia e kahi enzyme ʻokoʻa i hana ʻia e porphyrin. Inā kū ʻole kekahi o kēia mau ʻanuʻu he ʻewalu i ka wā o ke kūkulu ʻana o ka heme ma muli o ka mutation a i ʻole ka toxin puni honua, hoʻopilikia ʻia ka synthesite o nā enzyme e hana ana i kona hemahema a alakaʻi i ka porphyria. Aia a puni nā ʻano like ʻole o ka porphyria a pili ke ʻano i ke ʻano o ka enzyme e noho ʻole ana ^[3] .

Nā ʻano o Porphyria

Aia he 4 ʻano o ka porphyria kahi i ʻike ʻia ai ʻelua e kāna mau hōʻailona a hoʻokaʻawale ʻia nā hope ʻelua e ka pathophysiology.

1. ʻO ka porphyria ma muli o ka hōʻailona

• Kaʻi porphyria (AP): Hōʻike koke kēia kūlana hoʻoweliweli ola a hoʻopili i ka ʻōnaehana hopohopo. ʻO nā ʻōuli o ka AP mau no hoʻokahi a ʻelua paha mau pule a ma hope o ka puka ʻana, hoʻomaka ka hoʻomaikaʻi ʻana o nā ʻōuli. ʻAno ʻole ʻo AP ma mua o ka wā ʻōpio a ma hope o ka menopause ^[4] .
• Kaʻi porphyria (CP): Hoʻokaʻawale nui ʻia lākou i nā ʻano 6 a me kēlā me kēia ʻano e hōʻike i nā kūlana e pili ana i nā maʻi ʻili koʻikoʻi e like me ka nui o ka lā, blister, edema, redness, scars a me ka ʻeleʻele o ka ʻili. Hoʻomaka nā ʻōuli o CP i ka wā kamaliʻi ^[5] .

2. ʻO ka porphyria ma muli o Pathophysiology

• ʻO porteryria Erythropoietic: Hoʻohālikelike ʻia ia e ka overproduction o porphyrins, keu hoʻi i ka iwi iwi ^[6] .
• Hepatic porphyria : Kuhi ʻia ia e ka overproduction o porphyrins i ke ake ^[7] .

Nā ʻōuli o Porphyria

ʻO nā ʻōuli o ka porphyria e like me kona ʻano penei.

Porphyria huehue

• ʻO ka pehu a me ka ʻeha nui i loko o ka ʻōpū
• ʻO ka paʻa ʻana, ka luaʻi a i ʻole ka diarrhea
• Palpitation puʻuwai
• Nā kūlana noʻonoʻo e like me ka hopohopo, hallucination, a paranoia paha ^[8]
• ʻO Insomnia
• Ka hopu ʻana i nā maʻi ^[8]
• Mimi ʻulaʻula a ʻeleʻele paha ^[9]
• ʻEha ʻeha, nāwaliwali, numbness a palalysis paha
• Hypertension

Porphyria Cutaneus

• Oversensitivity i ka lā ^[10]
• ʻO ka ʻā ʻana i ka ʻeha ma ka lā a i ʻole ke kukui ʻino
• ʻEha ʻeha o ka ʻili
• ʻUlaʻula ʻili
• Pākuʻi a me ka hoʻoliʻiliʻi o ka ʻili ^[10]
• Hoʻonui ka ulu o ka lauoho
• ʻO nā pupuhi mai kahi ʻōpala liʻiliʻi
• Mimi ʻalani polū
• ʻO ka ulu lauoho maʻamau i ka maka ^{[umikumākahi]}
• ʻEleʻele o ka ʻili ku waho
• ʻO ka scarring nui o ka ʻili e hopena i nā niho like fang a me nā lehelehe ʻulaʻula.

Nā Kūlana Pilikia o Porphyria

Ke loaʻa ka porphyria, pili ia i nā toxins kaiapuni e hoʻonāuki i nā ʻōuli o vampirism. Penei lākou:

• Hōʻike lā lā ^[1]
• ʻO ka ʻai ʻana i ke kālaki a i ʻole nā ​​mea ʻai i kālika ʻia ^[12]
• ʻO nā lāʻau lapaʻau Hormonal e like me nā hormones menstrual
• Puhipaka ^[13]
• Kaumaha a me ke koʻikoʻi koʻikoʻi ^[14]
• Maʻi kīnā
• Hana hoʻomāinoino ʻino
• Kaʻai ʻana a i ʻole ka hoʻokē ʻai ʻana
• Nā lāʻau e like me nā lāʻau hoʻomalu hānau a i ʻole nā ​​lāʻau psychoactive
• ʻO ka nui o ka hōʻiliʻili o ka hao i ke kino ^{[umikumālima]}
• Maʻi maʻi ate

Nā pilikia o Porphyria

ʻO nā pilikia o ka porphyria penei:

• Hewa ʻole nā ​​hakuʻala ^[16]
• Poino paʻa paʻa ^[5]
• Poino ke ake
• Ka make nui loa ʻana ^[4]
• Hyponatremia, haʻahaʻa sodium i loko o ke kino
• Nā pilikia hanu nui ^[4]

ʻImi o Porphyria

ʻAʻole paʻakikī ʻo Porphyria i kekahi manawa e ʻike ʻoiai ua like nā ʻōuli me Guillain-Barre Syndrome. Eia naʻe, hoʻokō ʻia ke kuhi e nā hōʻike aʻe:

• Hōʻike koko, mimi a me ka noho: E ʻike ai i nā pilikia o ke ake a me ke ake a me ke ʻano a me ka pae o nā porphyrins i loko o ke kino ^[17] .
• ʻIke DNA: E hoʻomaopopo i ke kumu ma hope o ka hoʻololi ʻana o ka moʻokūʻauhau ^[18] .

Ka mālama ʻana iā Porphyria

Hoʻokumu ʻia ka mālama ʻana i ka porphyria i kona ʻano. Penei lākou:

• Nā lāʻau hoʻoweliweli Hāʻawi pinepine ʻia ʻo Hematin, glucose a me nā lāʻau wai e mālama i nā kiʻekiʻe o ka heme, ke kō a me nā wai i ke kino. Hana ʻia ka lapaʻau ma ka nui porphyria AP ^[4] .
• Phlebotomy: I ka CP, hemo aku kekahi ʻano o ke koko mai nā veins o ke kanaka e hoʻemi i ke kiʻekiʻe o ka hao i ke kino ^[19] .
• Nā lāʻau carotene Beta: I mea e hoʻomaikaʻi ai i ka ʻili i ka ʻae ʻana i ka lā ^[iwakālua] .
• Nā lāʻau Antimalarial: ʻO nā lāʻau e like me hydroxychloroquine a me chloroquine, i hoʻohana ʻia e mālama i nā hōʻailona malarial e hoʻohana ʻia e komo i ka nui o nā porphyrins mai ke kino. ^{[iwakāluakumakahi]} .
• Nā mea hoʻohui Vitamin D: I mea e hoʻomaikaʻi ai i nā kumu i hopena ʻia ma muli o ka lawa ʻole o ka wikamina D ^[22] .
• Ka hoʻoneʻe iwi iwi: No ka hana ʻana i nā hunaola hou a olakino i ke kino ^{[2. 3]} .
• Ka hoʻoliʻi ʻana o ka paʻa ʻōpala: Lawe ʻia kēia ma ka hoʻohana ʻana i ke koko umbilical cord kahi kumu waiwai o nā hunaola koʻokoʻo ma mua o ka iwi iwi ^[24] .

Nā ʻōlelo aʻoaʻo e hana ai me Porphyria

• E komo i nā kapa pale i waho o ka lā.
• Hōʻalo i nā lāʻau a i ʻole ka waiʻona inā loaʻa ʻoe i ka porphyria.
• Mai ʻai i ke kālaka, no ka mea e hoʻomaka ia i nā ʻōuli o kēia ʻano ^[12] .
• E haʻalele i ka puhi pua ʻana ^[13]
• Mai hoʻokē ʻai no ka mea hiki ke alakaʻi i ka hemahema o kekahi mau meaola i ke kino.
• Hana i ka noʻonoʻo a i ʻole yoga e hoʻemi i ke koʻikoʻi.

Inā loaʻa ʻoe i kahi maʻi, mālama iā ia i ka wā hiki.

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15. [umikumālima]Willandt, B., Langendonk, J. G., Biermann, K., Meersseman, W., D'Heygere, F., George, C.,… Cassiman, D. (2016). Hoʻopili ka Liver Fibrosis me ka hōʻiliʻili hao ma muli o ka mālama lōʻihi Heme-Arginate ma Porphyria ākea ākea: kahi hihia hihia. Hōʻike ʻo JIMD, 25, 77–81. hana: 10.1007 / 8904_2015_458
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