I-Porphyria (I-Vampire Syndrome): Izimbangela, Izimpawu, Ukuxilongwa, Izingozi Nezokwelapha

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I-vampire syndrome yisifo esingajwayelekile sezakhi zofuzo segazi esivame ukuthinta isikhumba kanye nesistimu yezinzwa. Ngokwemigomo yezokwelapha, yaziwa ngokuthi yi-porphyria ^[1] . Lesi simo sibizwa ngokuthi 'i-vampire' ngenxa yezimpawu zayo ezifana ne-vampire yezinganekwane zekhulu le-18.

I-Porphyria yatholwa kudala, ngaphambi kokuthi kusungulwe ama-antibiotic, ukuthuthwa kwendle kanye nefriji. Ngalezo zinsuku, abantu abanalesi simo babebhekwa njenge-'vampire 'ngenxa yezimpawu zabo ezinjenge-vampire ezibandakanya amazinyo, imibuthano emnyama ezungeze amehlo, umchamo obomvu nokungazweli ekukhanyeni kwelanga. Kodwa-ke, lesi sifo safundwa kamuva ngongoti bezokwelapha nokwelashwa kwaso kwasungulwa ^[okubili] .

Imibono Yesayensi Ebangela iPorphyria

Ngokusho kukaDesiree Lyon Howe, umsunguli we-American Porphyria Foundation futhi ophethwe i-acute intermittent porphyria, lesi sifo esingajwayelekile sasandile kakhulu emiphakathini ekude eYurophu ngesikhathi seminyaka ephakathi lapho abantu babehlala kude nokuxhumana nabantu besimanje umhlaba ^[25] .

URoger Luckhurst, uprofesa ku-Modern and Contemporary Literature (London) kanye nomhleli wencwadi ethi 'Dracula' ka-Bram Stoker ubalule izinto ezimbalwa nezehlakalo ezibhekele i-porphyria ngawo-1730s. Ubalule ukuthi ngesikhathi seminyaka ephakathi, inhlekelele yahlasela izindawo ezikude zaseYurophu futhi yabangela indlala, isifo kanye nezifo eziningi ezinzima njenge-catalepsy (ukuqina komzimba nokulahlekelwa umuzwa) ^[26] .

Ngenxa yokungaxhumani nomhlaba wangaphandle nokunganele kwemithi, abantu abane-porphyria bakhubazeka ngokwengqondo ngenxa yokwesaba, ukudangala nezinye izinto base beqala ukuzidla ngokwabo ngenxa yendlala enkulu. Futhi, ngenxa yokungazi ngokugoma kwanamuhla nezidakamizwa, izifo ezibangelwe yizilwane ezilumayo njengamarabi zasakazeka kakhulu ngesikhathi esasibangela ukuthi bangathandi amanzi nokukhanya, ukubona izinto ezingekho kahle kanye nolaka.

Enye imbangela, njengoba kushiwo nguSolwazi Roger Luckhurst, ikhombisa ukuthi njengoba le miphakathi yaseYurophu yahlala yodwa isikhathi eside, yabangela ukungondleki kahle ngenxa yokungadli kahle futhi isengozini yezifo eziningi ngenxa yokuthi izakhi zofuzo zazo zazingashintsha zibe zimbi kakhulu kubangele i-vampire- njengezimpawu.

Njengoba isikhathi sihamba nemishado yenzeka, ukungajwayelekile kofuzo kwadlula kusuka kubazali kuya ezinganeni kwadala nokusabalala kwalesi simo.

Isizathu sePorphyria

Kubantu, i-oxygen evela emaphashini idluliselwa kwezinye izingxenye zomzimba ngokusebenzisa iprotheni ekhethekile kumaseli abomvu egazi abizwa nge-hemoglobin nayo ebhekele umbala obomvu wegazi. IHemoglobin iqukethe iqembu lokufakelwa elibizwa ngokuthi i-heme eliqukethe i-porphyrin ne-iron-ion enkabeni. Imvamisa yenziwa ngamaseli abomvu egazi, umnkantsha kanye nesibindi.

I-Heme yenziwa ngezinyathelo eziyisishiyagalombili ezilandelanayo ngayinye nge-enzyme ehlukile eyenziwe yi-porphyrin. Uma noma iyiphi yalezi zinyathelo eziyisishiyagalombili yehluleka ngenkathi kwakhiwa i-heme ngenxa yokuguquka kwezakhi zofuzo noma ubuthi bezemvelo, ukuhlanganiswa kwama-enzyme kuyaphazamiseka kubangele ukuntuleka kwawo futhi kuholele kwi-porphyria. Kunezinhlobo ezahlukahlukene ze-porphyria futhi isimo sixhunyaniswe nohlobo lwe-enzyme olungekho ^[3] .

Izinhlobo ZePorphyria

Kunezinhlobo ezi-4 ze-porphyria lapho ezimbili zibonakaliswa yizimpawu zayo kanti ezimbili zokugcina zihlukaniswe yi-pathophysiology.

1. I-porphyria esezimpawu

• I-acute porphyria (AP): Lesi simo esisongela impilo sivela ngokushesha futhi sithinte isimiso sezinzwa. Izimpawu ze-AP zihlala isonto elilodwa noma amabili futhi ngemuva kokuvela kwazo, izimpawu ziqala ukuba ngcono. I-AP ayivamile ukuvela ngaphambi kokuthomba nangemva kokuya esikhathini ^[4] .
• I-porphyria enqamulelayo (CP): Zihlukaniswe kakhulu ngezinhlobo ezi-6 kanti uhlobo ngalunye lukhombisa izimo ezihlobene nezifo ezinzima zesikhumba njengokuzwela ukukhanya kwelanga, amabhamuza, i-edema, ububomvu, izibazi nokumnyama kwesikhumba. Izimpawu ze-CP ziqala ngesikhathi sobuntwana ^[5] .

2. IPathophysiology-based porphyria

• I-Erythropoietic porphyria: Kubonakala ngokukhiqiza ngokweqile ama-porphyrins, ikakhulukazi kumnkantsha wethambo ^[6] .
• I-hepatic porphyria : Kubonakala ngokukhiqiza ngokweqile ama-porphyrins esibindi ^[7] .

Izimpawu ZePorphyria

Izimpawu ze-porphyria ngezinhlobo zayo zimi kanje.

I-porphyria enamandla

• Ukuvuvukala nobuhlungu obukhulu esiswini
• Ukuqunjelwa, ukuhlanza noma isifo sohudo
• Ukushaya inhliziyo
• Izimo zengqondo ezinjengokukhathazeka, ukubona izinto ezingekho emzimbeni, noma i-paranoia ^[8]
• Ukuqwasha
• Ukuquleka ^[8]
• Umchamo obomvu noma onsundu ^[9]
• Ubuhlungu bemisipha, ubuthakathaka, ukuba ndikindiki noma ukukhubazeka
• Umfutho wegazi ophezulu

I-porphyria enqamulelayo

• Ukuzwela ngokweqile ekukhanyeni kwelanga ^[10]
• Ukushisa ubuhlungu ekukhanyeni kwelanga noma ekukhanyeni okufakelwayo
• Ukuvuvukala okubuhlungu kwesikhumba
• Ukubomvu kwesikhumba
• Izibazi nokushintshwa kwesikhumba ^[10]
• Ukukhula kwezinwele okukhulayo
• Amabhamuza avela ekusakazweni okuncane
• Umchamo oboshwe ngombala oluhlaza okwesibhakabhaka
• Ukukhula kwezinwele okungavamile ebusweni ^{[ishumi nanye]}
• Ukwenza mnyama kwesikhumba esidaluliwe
• Ukushaqeka okukhulu kwesikhumba okuholela ekuvezeni amazinyo afana nezinyo nezindebe ezibomvu.

Izici Zengozi YePorphyria

Lapho kutholakala i-porphyria, ikakhulukazi ngenxa yobuthi bezemvelo obudala izimpawu ze-vampirism. Zimi kanje:

• Ukukhanya kwelanga ^[1]
• Ukudla igalikhi noma ukudla okususelwe kugalikhi ^[12]
• Imithi yeHormonal efana nama-hormone wokuya esikhathini
• Ukubhema ^[13]
• Ukucindezeleka ngokomzimba noma ngokomzwelo ^[14]
• Ukutheleleka
• Ukusebenzisa kabi izidakamizwa
• Ukudla noma ukuzila ukudla
• Izidakamizwa ezinjengamaphilisi okulawula ukubeletha noma izidakamizwa ezithinta ingqondo
• Ukuqoqwa ngokweqile kwensimbi emzimbeni ^{[ishumi nanhlanu]}
• Isifo sesibindi

Izinkinga zePorphyria

Izinkinga ze-porphyria zimi kanje:

• Ukuhluleka kwezinso ^[16]
• Ukulimala okungapheli kwesikhumba ^[5]
• Ukulimala kwesibindi
• Ukuphela kwamanzi emzimbeni ^[4]
• Hyponatremia, i-sodium ephansi emzimbeni
• Izinkinga zokuphefumula ezinzima ^[4]

Ukuxilongwa KwePorphyria

I-Porphyria kwesinye isikhathi kunzima ukuyithola njengoba izimpawu zayo zifana ne-Guillain-Barre syndrome. Kodwa-ke, ukuxilongwa kwenziwa izivivinyo ezilandelayo:

• Ukuhlolwa kwegazi, umchamo & indle: Ukuthola izinkinga zezinso nezesibindi nohlobo nezinga lama-porphyrins emzimbeni ^[17] .
• Ukuhlolwa kwe-DNA: Ukuqonda imbangela yokuguquka kwezakhi zofuzo ^[18] .

Ukwelashwa Kwe-Porphyria

Ukwelashwa kwe-porphyria kusekelwe ezinhlotsheni zayo. Zimi kanje:

• Imithi efakwa ngaphakathi: I-Hematin, i-glucose neminye imishanguzo enamanzi inikezwa ngemithambo yegazi ukugcina amazinga we-heme, ushukela noketshezi emzimbeni. Ukwelashwa kwenziwa ikakhulukazi ku-acute porphyria AP ^[4] .
• I-Phlebotomy: Ku-CP, inani elithile legazi likhishwa emithanjeni yomuntu ukunciphisa izinga lensimbi emzimbeni ^[19] .
• Izidakamizwa ze-beta carotene: Ukwenza ngcono ukubekezelelana kwesikhumba ekukhanyeni kwelanga ^{[amashumi amabili]} .
• Imithi elwa namagciwane: Izidakamizwa ezinjenge-hydroxychloroquine ne-chloroquine, ezisetshenziselwa ukwelapha izimpawu zikamalaleveva zisetshenziselwa ukumunca inani eliningi ngokweqile lama-porphyrins emzimbeni ^{[amashumi amabili nanye]} .
• Izithako ze-Vitamin D: Ukwenza ngcono izimo ezibangelwe ukushoda kukavithamini D ^[22] .
• Ukufakelwa komnkantsha wethambo: Ekhiqizweni kwamaseli egazi amasha nanempilo emzimbeni ^{[2. 3]} .
• Ukufakelwa kabusha kwe-stem cell: Lokhu kwenziwa ngokusebenzisa inkaba yegazi eliwumthombo ocebile wamaseli ezinhlayiya kunomnkantsha wethambo ^[24] .

Amathiphu Wokubhekana NePorphyria

• Gqoka amagiya okuzivikela uma ungaphandle elangeni.
• Gwema izidakamizwa noma utshwala uma une-porphyria.
• Ungayidli igalikhi ngoba ingadala izimpawu zalesi simo ^[12] .
• Yeka ukubhema ^[13]
• Ungazili isikhathi eside ngoba kungaholela ekushodeni kwezakhamzimba ezithile emzimbeni.
• Yenza ukuzindla noma i-yoga ukunciphisa ukucindezeleka.

Uma uthola ukutheleleka, kwelaphe ngokushesha okukhulu.

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