PORPHYRIA (VAMPIRE SYNDROME): UA RAU, TSOS MOB, KUAJ POM, QHOV TSEEB YUAV RAUG MOB THIAB KHO - PAMPEREDPEOPLENY.COM

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Tsev Kev Noj Qab Haus Huv Kev kho tsis meej Tshuaj Tiv Thaiv Tsis Zoo Kho los-Shivangi Karn Los Ntawm Shivangi Karn hnub tim 18 lub 10 hli xyoo 2019

Lub vampire syndrome yog qhov tsis tshua muaj caj ces tsis yooj yim ntawm cov ntshav uas feem ntau cuam tshuam rau daim tawv nqaij thiab cov leeg ntshav. Hauv cov ntawv kho mob, nws yog lub npe hu ua porphyria ^[1] Cov. Tus mob no yog hu ua 'vampire' vim tias nws cov tsos mob uas zoo sib xws nrog mythological vampire ntawm 18th caug xyoo.

Porphyria tau nrhiav pom ntev dhau los, ntau ntau ua ntej kev tsim tshuaj tua kab mob, kev ntxuav tu thiab tub yees. Hauv cov hnub ntawd, cov neeg muaj tus mob no tau suav hais tias yog 'vampire' vim lawv cov vampire zoo li cov tsos mob uas cuam tshuam nrog cov fangs, tsaus voj ncig ncig lub qhov muag, tso zis liab thiab tsis raug rau lub hnub ci. Txawm li cas los xij, qhov xwm txheej tom qab tau kawm los ntawm cov kws paub kho mob thiab nws cov kev kho tau tsim ^[ob] Cov.

Txuj Ci Kev Tshawb Fawb Tom Qab Porphyria

Raws li Desiree Lyon Howe, tus tsim lub koom haum American Porphyria Foundation thiab tus neeg raug mob siab, tau muaj tus kab mob no tsis muaj kis txaus rau cov zej zog nyob deb nyob teb chaws Europe thaum lub caij nyoog nruab nrab thaum tib neeg siv los nyob deb ntawm kev sib cuag ntawm lub niaj hnub. ntiaj teb no ^[25] Cov.

Roger Luckhurst, tus xibfwb ntawm Kev Sau Ntawv Niaj Hnub nimno thiab Txheej Txheem (London) thiab tus kws sau ntawv ntawm phau ntawv 'Dracula' los ntawm Bram Stoker tau hais txog ntau yam thiab xwm txheej ua lub luag haujlwm rau porphyria hauv xyoo 1730. Nws tau hais tias thaum lub caij nyoog nruab nrab, muaj kev puas tsuaj loj heev tau ua rau thaj chaw deb ntawm Tebchaws Europe thiab ua rau muaj kev tshaib nqhis, kab mob plaw thiab ntau yam kabmob phem xws li catalepsy (kev nruj ntawm lub cev thiab kev hnov ploj) ^[26] Cov.

Vim tias tsis muaj kev sib cuag nrog lub ntiaj teb sab nraud thiab tsis txaus tshuaj, cov neeg muaj kab mob porphyria tau mob hlwb vim kev ntshai, kev nyuaj siab thiab lwm yam thiab tau pib noj lawv tus kheej tawm ntawm kev tshaib nqhis heev. Tsis tas li, vim tsis muaj qhov tsis paub txog kev txhaj tshuaj tiv thaiv niaj hnub no thiab cov tshuaj, kab mob tshwm sim los ntawm cov tsiaj tom xws li tus mob rabies tau kis tus kab mob ntau thaum lub sijhawm uas ua rau lawv tsis tuaj yeem cuam tshuam rau dej thiab lub teeb, ua kom pom tseeb thiab tsim kev kub ntxhov.

Lwm qhov laj thawj, raws li tau hais los ntawm xibfwb Roger Luckhurst, tau qhia tias vim cov zej zog nyob sab Europe no tseem nyob rau hauv kev nyob ib leeg ntev heev, nws ua rau kev noj zaub mov tsis txaus vim yog kev noj zaub mov tsis zoo thiab ua rau muaj ntau cov kab mob vim tias lawv cov noob yuav tau hloov mus rau qhov ua rau vampire- zoo li cov tsos mob.

Raws li lub sijhawm dhau los thiab kev muaj txij nkawm tau tshwm sim, qhov txawv txav ntawm cov gene dhau los ntawm niam txiv mus rau menyuam yaus thiab ua rau kis tus mob.

Ciaj Ua Si Ntawm Porphyria

Hauv tib neeg, cov pa oxygen los ntawm lub ntsws tau hloov mus rau lwm qhov ntawm lub cev los ntawm cov protein tshwj xeeb hauv cov ntshav liab hu ua hemoglobin uas kuj yog lub luag haujlwm rau cov xim liab ntawm cov ntshav. Hemoglobin muaj cov pab pawg ntxim nyiam hu ua heme uas muaj porphyrin thiab hlau-ion ntawm qhov chaw. Feem ntau nws ua hauv cov ntshav liab, mob pob txha thiab mob siab.

Heme yog tsim nyob rau hauv yim ntu ua ntu zus ib qho los ntawm nyias muaj nyias enzyme tsim los ntawm porphyrin. Yog tias ib qho ntawm yim kauj ruam no ua tsis tiav thaum lub tsev ntawm heme vim kev hloov caj ces lossis ib puag ncig cov co toxin, qhov synthesis ntawm cov enzymes tau txais kev puas tsuaj ua rau nws qhov tsis muaj peev xwm thiab ua rau muaj kab mob hlwb. Muaj ntau nyob ib ncig ntawm ntau ntau hom ntawm plooj (porphyria) thiab tus mob no tau txuas nrog rau hom enzyme uas tsis tuaj ^[3] Cov.

Hom Ntawm Porphyria

Muaj 4 hom porphyria nyob rau hauv uas ob tug tau cim los ntawm nws cov tsos mob thiab tom kawg ob tau faib los ntawm pathophysiology.

1. Ua raws li cov cim porphyria

Acphy porphyria (AP): Qhov uas muaj txoj sia nyob no tshwm sim sai thiab cuam tshuam rau cov leeg hlwb. Cov tsos mob ntawm AP kav rau ib lossis ob lub lim tiam thiab tom qab lawv tshwm sim, cov tsos mob pib txhim kho. AP tsis tshua muaj tshwm sim ua ntej tiav nkauj tiav nraug thiab tom qab lawm ^[4] Cov.
Kev Txiav Txim Siab (CP): Lawv feem ntau muab faib ua 6 hom thiab txhua hom kis tau tus mob ntsig txog kab mob ntawm daim tawv nqaij zoo li kev tswj hwm lub hnub ci, blisters, edema, liab, nti thiab nti ntawm daim tawv. Cov tsos mob ntawm CP pib thaum menyuam yaus ^[5] Cov.

2. Pathophysiology-based porphyria

Erythropoietic porphyria: Nws yog tus cwj pwm los ntawm kev ua haujlwm ntau dhau ntawm porphyrins, tshwj xeeb tshaj yog hauv hlwb pob txha ^[6] Cov.
Hepatic porphyria : Nws yog tus cwj pwm los ntawm kev ua haujlwm ntau dhau ntawm porphyrins hauv lub siab ^[7] Cov.

Cov tsos mob ntawm Porphyria

Cov tsos mob ntawm porphyria raws li nws cov hom muaj raws li nram no.

Acphy porphyria

O o thiab mob hnyav hauv plab
Cem quav, ntuav lossis raws plab
Lub plawv palpitation
Cov kev mob hlwb zoo li kev ntxhov siab, xav tob tob lossis tsis paub qab hau ^[8]
Insomnia
Qaug dab peg ^[8]
Liab lossis ntshav zis ^[9]
Mob leeg, tsis muaj zog, loog lossis ib ce txhav
Tawg siab

Hlais nplooj qeej

Oversensitivity rau tshav ntuj ^[10]
Hlawv mob hauv tshav ntuj lossis cov teeb ci cuav
Kev mob ntawm daim tawv nqaij o
Daim tawv nqaij liab
Caws pliav thiab tawv nqaij tsis zoo ^[10]
Nce cov plaub hau kev loj hlob tuaj
Blisters los ntawm ib tus me scrapes
Tiav qhov muag xiav
Txawv plaub hau tsis zoo rau ntawm lub ntsej muag ^{[kaum ib]}
Qhov tsaus ntuj ntawm daim tawv nqaij
Qhov hnyav ntawm daim tawv nqaij ua rau muaj qhov ncauj zoo li hniav thiab daim di ncauj liab.

Kev Pheej hmoo Txoj Cai Ntawm Porphyria

Thaum porphyria nrhiav, nws yog feem ntau vim yog ib puag ncig co toxins uas ua rau tus tsos mob ntawm vampirism. Lawv muaj raws li hauv qab no:

Kev tiv tshav ntuj ^[1]
Noj qej lossis qej ua khoom noj ^[12]
Cov tshuaj hormones zoo li cov poj niam coj khaub ncaws
Haus Yeeb ^[13]
Lub cev lossis kev ntxhov siab ^[14]
Kab mob
Raug yeeb tshuaj
Kev noj haus lossis yoo mov
Cov tshuaj nyiam cov tshuaj tswj kom txhob muaj menyuam lossis cov tshuaj khomob psychoactive
Kev siv hlau ntau dhau nyob rau hauv lub cev ^{[kaum tsib]}
Mob siab

Teeb meem Ntawm Porphyria

Cov teeb meem ntawm cov kab porphyria muaj raws li hauv qab no:

Raum tsis ua haujlwm ^[16]
Daim tawv puas tas li ^[5]
Nplooj siab puas tsuaj
Lub cev qhuav dej kom hnyav ^[4]
Hyponatremia, muaj ntsev tsawg nyob rau hauv lub cev
Cov teeb meem ua pa hnyav ^[4]

Kev kuaj pom ntawm Porphyria

Porphyria qee zaus nyuaj rau kuaj tau raws li nws cov tsos mob zoo li Guillain-Barre syndrome. Txawm li cas los xij, kev kuaj mob yog nqa tawm los ntawm cov kev sim hauv qab no:

Ntshav, zis thiab kuaj quav: Txhawm rau tshawb xyuas cov teeb meem lub raum thiab mob siab thiab hom thiab qib ntawm porphyrins hauv lub cev ^[17] Cov.
Kev kuaj DNA: Txhawm rau kom nkag siab qhov ua rau muaj qab hloov noob ^[18] Cov.

Kev Kho Mob Ntawm Porphyria

Kev kho ntawm porphyria yog raws li nws cov hom. Lawv muaj raws li hauv qab no:

Tso cov tshuaj: Hematin, ntshav qabzib thiab lwm yam tshuaj kua tau muab zuaj kom tswj tau li ntawm heme, piam thaj thiab dej hauv lub cev. Cov kev kho mob yog ua tsuas yog nyob rau hauv siab ntev porphyria AP ^[4] Cov.
Phlebotomy: Hauv CP, qee qhov ntshav ntawm cov ntshav tawm ntawm cov leeg ntshav ntawm tus neeg txhawm rau txhawm rau txo qib hlau hauv lub cev ^[19] Cov.
Cov tshuaj carotene Beta: Txhawm rau txhim kho daim tawv nqaij kom tiv thaiv tshav ntuj ^{[nees nkaum]} Cov.
Tshuaj noj tiv thaiv kabmob: Cov tshuaj zoo li hydroxychloroquine thiab chloroquine, uas yog siv los kho cov tsos mob malarial yog siv los nqus cov ntau ntawm porphyrins los ntawm lub cev ^{[nees nkaum-ib]} Cov.
Tshuaj Vitamin D: Txhawm rau txhawm rau txhim kho cov mob uas tshwm sim vim muaj vitamin D tsis txaus ^[22] Cov.
Hloov hlwb pob txha: Rau kev tsim cov qe ntshav tshiab thiab noj qab nyob zoo hauv lub cev ^{[2. 3]} Cov.
Kev hloov ntawm qia: Qhov no tau ua los ntawm kev siv txoj hlab ntaws ntshav uas yog qhov muaj txiaj ntsig ntawm qia hlwb ntau dua li cov hlwb pob txha ^[24] Cov.

Cov Lus Qhia Yuav Tsum Nrog Porphyria

Hnav cov iav tiv thaiv thaum sab nraum lub hnub.
Tsis txhob siv tshuaj yeeb los yog dej caw yog tias koj muaj kab mob porphyria.
Tsis txhob noj qej vim tias nws yuav ua rau muaj tus tsos mob ntawm qhov mob no ^[12] Cov.
Txiav Luam Yeeb ^[13]
Tsis txhob yoo ncua ntev yog tias nws yuav ua rau lub cev tsis muaj qee yam as-ham hauv lub cev.
Ua kev ua si los yog yoga ua kom txo kev ntxhov siab.

Yog tias koj kis mob, kho nws kom sai li sai tau.

Xav txog tus kws kho mob ua ntej pib qee yam tshuaj vim tias nws tuaj yeem ua rau muaj tsos mob.

Yog tias koj muaj tus mob, tsis txhob hnov qab mus rau kev kuaj keeb kwm kom nkag siab txog qhov ua rau muaj kev sib hloov.

Saib Tshooj Ntawv Rov Ntxiv

[1]National Center rau Biotechnology Cov Ntaub Ntawv (Asmeskas). Noob thiab Kab Mob [Is Taws Nem]. Bethesda (MD): Lub Chaw National rau Biotechnology Cov Ntaub Ntawv (Asmeskas) 1998-. Porphyria.
[ob]Cox A. M. (1995). Porphyria thiab vampirism: lwm qhov tswvyim hais ua dabneeg. Cov phau ntawv kho mob postgraduate, 71 (841), 643–644. doi: 10.1136 / pgmj.71.841.643-a
[3]Ramanujam, V. M., & Anderson, K. E. (2015). Porphyria Kev Qhia Txog Tus Kheej-Ntu 1: Cov Lus Qhia luv luv ntawm cov Porphyrias. Cov tam sim no ntawm tib neeg cov noob caj noob ces, 86, 17.20.1–17.20.26. doi: 10.1002 / 0471142905.hg1720s86
[4]Gounden V, Jialal I. Acute Porphyria. [Hloov kho 2019 Jan 4]. Hauv: StatPearls [Is Taws Nem]. Khoom Plig Nyiaj Pov (FL): StatPearls Tshaj Tawm 2019 Jan-.
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[14]Naik, H., Stoecker, M., Sanderson, S. C., Balwani, M., & Desnick, R. J. (2016). Cov kev paub dhau los thiab kev txhawj xeeb ntawm cov neeg mob uas muaj kev mob rov qab los ntawm kev muaj mob rau cov ntshav qab zib siab: muaj kev kawm paub. Cov noob caj noob ces txog cev thiab cov metabolism, 119 (3), 278–283. doi: 10.1016 / j.ymgme.2016.08.006
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[18]Kauppinen, R. (2004). Cov kev kuaj mob ntawm cov nqaij mos thaum sib nqus. Kev tshuaj xyuas kev txawj ntse ntawm cov ntsuas kev ntsuas molecular, 4 (2), 243-249.
[19]Lundvall, O. (1982). Kev kho phlebotomy ntawm porphyria cutanea tarda. Acta dermato-ntsigologica. Ntxiv Ntxiv, 100, 107-118.
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