Porphyria (The Vampire Syndrome): Lisosa, Matšoao, Tlhahlobo, Kotsi le Phekolo

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Vampire syndrome ke lefu le sa tloaelehang la lefutso la mali leo hangata le amang letlalo le sistimi ea methapo. Mabapi le bongaka, e tsejoa e le porphyria ^[1] . Boemo bona bo bitsoa 'vampire' ka lebaka la matšoao a eona a ts'oanang le vampire ea nalane ea lekholo la bo18 la lilemo.

Porphyria e ile ea sibolloa khale haholo, pele ho qaptjoa lithibela-mafu, tsamaiso ea likhoerekhoere le sehatsetsi. Mehleng eo, batho ba nang le boemo bona ba ne ba nkuoa e le 'vampire' ka lebaka la matšoao a bona a kang a vampire a amang meno, masapo a lefifi a pota-potileng mahlo, moroto o mofubelu le ho se tsotelle khanya ea letsatsi. Leha ho le joalo, boemo bona bo ile ba ithutoa hamorao ke litsebi tsa bongaka mme kalafo ea eona ea qaptjoa ^[pedi] .

Likhopolo tsa Saense Ka Mor'a Porphyria

Ho ea ka Desiree Lyon Howe, mothehi oa American Porphyria Foundation le ea nang le lefu la porphyria e kenang khafetsa, lefu lena le sa tloaelehang le ne le atile har'a libaka tse hole tsa Europe nakong ea lilemo tse bohareng ha batho ba ne ba lula hole le moo batho ba mehleng ena ba sa kopaneng lefats'e ^[25] .

Roger Luckhurst, moprofesa oa Modern and Contemporary Literature (London) le mohlophisi oa buka 'Dracula' ea Bram Stoker o boletse lintlha le liketsahalo tse ikarabellang bakeng sa porphyria lilemong tsa bo-1730. O boletse hore nakong ea mehla ea mahareng, koluoa ​​e ile ea hlasela libaka tse hole tsa Europe mme ea baka tlala, lefu la seoa le mafu a mangata a matla joalo ka catalepsy (ho satalla ha 'mele le ho felloa ke maikutlo) ^[26] .

Ka lebaka la ho haelloa ke puisano le lefats'e le kantle le ho haella ha meriana, batho ba nang le porphyria ba ile ba holofala kelellong ka lebaka la tšabo, khatello ea maikutlo le lintlha tse ling mme ba qala ho itjella ka lebaka la tlala e feteletseng. Hape, ka lebaka la ho se hlokomele ha ente ea sejoale-joale le lithethefatsi, mafu a bakoang ke ho loma ha liphoofolo joalo ka rabies a ile a hasana ka bongata ka nako e ileng ea ba bakela lehloeo la metsi le khanya, hallucination le mabifi.

Lebaka le leng, joalo ka ha ho boletsoe ke Moprofesa Roger Luckhurst, le bonts'a hore ha libaka tsena tsa Europe li lula ka thoko nako e telele, li bakile khaello ea phepo e nepahetseng ka lebaka la phepo e nepahetseng le ho hlaseloa habonolo ke mafu a mangata ka lebaka leo liphatsa tsa bona tsa lefutso li ka beng li fetohile hampe ho baka vampire- joalo ka matšoao.

Ha nako e ntse e feta mme manyalo a etsahala, ho se ts'oanehe hoa liphatsa tsa lefutso ho tloha ho batsoali ho ea ho bana mme ho baka ho ata ha boemo boo.

Sesosa sa Porphyria

Ho batho, oksijene e tsoang matšoafong e fetisetsoa likarolong tse ling tsa 'mele ka protheine e khethehileng liseleng tse khubelu tsa mali tse bitsoang hemoglobin eo hape e ikarabellang bakeng sa' mala o mofubelu oa mali. Hemoglobin e na le sehlopha sa maiketsetso se bitsoang heme se nang le porphyrin le ion-iron bohareng. Hangata e entsoe ka lisele tse khubelu tsa mali, moko oa masapo le sebete.

Heme e entsoe ka mehato e robeli e latellanang e 'ngoe le e' ngoe ka enzyme e arohaneng e hlahisoang ke porphyrin. Haeba efe kapa efe ea mehato ena e robeli e hloleha nakong ea kaho ea heme ka lebaka la phetoho ea liphatsa tsa lefutso kapa chefo ea tikoloho, tšebetso ea li-enzyme e ea khathatseha e baka khaello ea eona mme e lebise porphyria. Ho na le mefuta e fapaneng ea porphyria mme boemo bo hokahane le mofuta oa enzyme e seng teng ^[3] .

Mefuta Of Porphyria

Hona le mefuta e mene ea porphyria eo ho eona e 'meli e khetholloang ke matšoao a eona mme ea bobeli e arotsoe ke pathophysiology.

1. Porphyria e nang le matšoao

• Acute porphyria (AP): Boemo bona bo sokelang bophelo bo hlaha kapele mme bo ama tsamaiso ea methapo. Matšoao a AP a nka beke e le 'ngoe kapa tse peli mme kamora hore a bonahale, matšoao a qala ho ntlafala. AP ha e etsahale hangata pele ho bocha le kamora ho khaotsa ho ilela khoeli ^[4] .
• Li-porphyria tse arohaneng (CP): Li arotsoe haholo ka mefuta e 6 mme mofuta o mong le o mong o supa maemo a amanang le maloetse a mabe a letlalo joalo ka khanya ea letsatsi, machachetsi, edema, bofubelu, mabali le ho fifala ha letlalo. Matšoao a CP a qala bongoaneng ^[5] .

2. Pathophysiology e thehiloeng porphyria

• Erythropoietic porphyria: E tšoauoa ka tlhahiso e fetelletseng ea li-porphyrin, haholoholo mokong oa masapo ^[6] .
• Hepatic porphyria : E khetholloa ka ho hlahisa li-porphyrin tse ngata sebeteng ^[7] .

Matšoao a Porphyria

Matšoao a porphyria ho latela mefuta ea ona ke a latelang.

Porphyria e matla

• Ho ruruha le bohloko bo boholo mpeng
• Ho sokela, ho hlatsa kapa letshollo
• Ho otla ha pelo
• Maemo a kelello a kang ho tšoenyeha, ho bona lintho ka leihlo le leng kapa ho hlanya ^[8]
• Ho hlobaela
• Ho oa ^[8]
• Moroto o mokgubedu kapa o sootho ^[9]
• Bohloko ba mesifa, bofokoli, ho shoeloa ke matla kapa ho shoa litho
• Hypertension

Porphyria e fokolang

• Ho ameha haholo ka khanya ea letsatsi ^[10]
• Ho chesa bohloko ba letsatsi kapa leseli la maiketsetso
• Ho ruruha ho bohloko ha letlalo
• Bofubelu ba letlalo
• Mabali le ho phatloha ha letlalo ^[10]
• Keketseho ea moriri
• Blisters e tsoang linthong tse nyane
• Moroto o nang le masela a maputsoa
• Khōlo e sa tloaelehang ea moriri sefahlehong ^{[leshome le motso o mong]}
• Ho fifala ha letlalo le pepesitsoeng
• Letšoele le leholo la letlalo le hlahisang meno a kang meno le menoana e khubelu.

Lisosa tsa Likotsi Tsa Porphyria

Ha porphyria e fumanoa, haholo-holo e bakoa ke chefo ea tikoloho e bakang matšoao a vampirism. Ke tse latelang:

• Ho pepeseha ha letsatsi ^[1]
• Ho ja konofolo kapa lijo tse entsoeng ka konofolo ^[12]
• Meriana ea Hormonal joaloka li-hormone tsa ho ilela khoeli
• Ho tsuba ^[13]
• Ho imeloa kelellong kapa maikutlong ^[14]
• Tšoaetso
• Tšebeliso e mpe ea lithethefatsi
• Ho ja kapa ho itima lijo
• Lithethefatsi tse kang lipilisi tsa thibelo ea bokhachane kapa lithethefatsi tse sebelisang kelello
• Ho bokellana haholo hoa tshepe mmeleng ^{[leshome le metso e mehlano]}
• Lefu la sebete

Mathata a Porphyria

Mathata a porphyria ke a latelang:

• Ho hloleha ha liphio ^[16]
• Tšenyo e sa feleng ea letlalo ^[5]
• Tšenyo ea sebete
• Ho felloa ke metsi 'meleng haholo ^[4]
• Hyponatremia, sodium e tlase 'meleng
• Mathata a matla a ho hema ^[4]

Tlhahlobo ea Porphyria

Porphyria ka linako tse ling ho thata ho e fumana kaha matšoao a eona a ts'oana le lefu la Guillain-Barre. Leha ho le joalo, tlhahlobo e etsoa ke liteko tse latelang:

• Teko ea mali, moroto le setuloana: Ho fumana mathata a liphio le sebete le mofuta le boemo ba li-porphyrin 'meleng ^[17] .
• Teko ea DNA: Ho utloisisa sesosa sa phetoho ea lefutso ^[18] .

Phekolo ea Porphyria

Kalafo ea porphyria e ipapisitse le mefuta ea eona. Ke tse latelang:

• Meriana e kenang ka hare Hematin, tsoekere le meriana e meng ea mokelikeli e fuoa methapo ho boloka maemo a heme, tsoekere le maro 'meleng. Kalafo e etsoa haholoholo ka porphyria AP e matla ^[4] .
• Phlebotomy: Ho CP, mali a mang a ntšoa methapong ea motho ho fokotsa boemo ba tšepe 'meleng ^[19] .
• Lithethefatsi tsa Beta carotene: Ho ntlafatsa mamello ea letlalo ho khanya ea letsatsi ^{[mashome a mabeli]} .
• Meriana ea likokoana-hloko: Lithethefatsi tse kang hydroxychloroquine le chloroquine, tse sebelisetsoang ho alafa matšoao a malaria li sebelisetsoa ho monya li-porphyrin tse ngata haholo 'meleng ^{[mashome a mabeli a motso o le mong]} .
• Lisebelisoa tsa vithamine D: Ho ntlafatsa maemo a bakiloeng ke khaello ea vithamine D. ^[22] .
• Ho kenyelletsa 'moko oa masapo: Bakeng sa tlhahiso ea lisele tse ncha le tse phetseng hantle tsa mali 'meleng ^{[2. 3]} .
• Ho kenyelletsa sethala sa stem: Sena se etsoa ka ho sebelisa lesela la mokhubu leo ​​e leng mohloli o enneng oa lisele tsa kutu ho feta mongo ^[24] .

Malebela a ho sebetsana le Porphyria

• Apara likere tse sireletsang ha u le kantle letsatsing.
• Qoba lithethefatsi kapa joala haeba u na le porphyria.
• Se ke oa ja konofolo kaha e ka baka matšoao a boemo bona ^[12] .
• Khaotsa ho tsuba ^[13]
• Se ke oa itima lijo nako e telele hobane e ka baka khaello ea limatlafatsi tse itseng 'meleng.
• Etsa ho thuisa kapa yoga ho fokotsa khatello ea maikutlo.

Haeba u fumana tšoaetso, e phekole kapele kamoo ho ka khonehang.

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