Porphyria (Iyo Vampire Syndrome): Zvinokonzera, Zviratidzo, Kuongorora, Zviratidzo zveRisk Uye Kurapa

Mazita Akanakisa Evana

Pasi:

Zuva Rekuzvarwa:

Tumira

Nditumire Neemail Ratidza Pane Saiti

Just In

• 7 hrs apfuura Chaitra Navratri 2021: Zuva, Muhurta, Tsika Uye Kukosha Kwemutambo uyu
• 
• 8 hrs apfuura Hina Khan Anobwinya Kumusoro Nemhangura Green Girazi Shadow Uye Glossy Nude Miromo Tora Iko Kutarisa Mune Mashoma Akareruka Matanho!
• 10 hrs apfuura Ugadi Uye Baisakhi 2021: Spruce Up Yako Festive Tarisa NaCelebs-Akafuridzirwa echinyakare Masutu
• 13 hrs apfuura Mazuva ese Horoscope: 13 Kubvumbi 2021

Usapotsa

• Sravana Sameeralu Serial 4th Technology BSNL Inobvisa Kuisirwa Mitero Kubva Kure Yakareba Broadband Kubatana
• Nhau Kumbh mela vanodzoka vanogona kuwedzera denda re COVID-19: Sanjay Raut
• Mitambo IPL 2021: BalleBaazi.com inogamuchira mwaka nemushandirapamwe mutsva 'Cricket Machao'
• Mafirimu Vira Sathidar Aka Narayan Kamble Kubva Kudare Anopfuura Nekuda Kwe COVID-19
• Mota Kabira Inofamba Hermes 75 Yakakwira-Yekumusoro Kwezvekutengesa Kuendesa Magetsi Scooter Yakatangwa MuIndia
• Zvemari Goridhe Mutengo Unowira Kwete Zvakawanda Kushushikana kweNBFCs, Mabhangi Anoda Kuva Akarinda
• Dzidzo CSBC Bihar Mapurisa Constable Yekupedzisira Mhedzisiro 2021 Yakaziviswa
• Kufamba 10 Nzvimbo Dzakanakisa dzekushanyira Mu Maharashtra Muna Kubvumbi

Iyo vampire syndrome isingawanzoitika geni kusagadzikana kweropa iro rinowanzo bata ganda uye nehurongwa hwetsinga. Mukutaura kwekurapa, inozivikanwa se porphyria ^[1] . Mamiriro acho anonzi 'vampire' nekuda kwezviratidzo ayo akafanana neanovampire wengano wezana ramakore rechi18.

Porphyria yakawanikwa kare kare, yakawanda isati yagadzirwa mishonga inorwisa mabhakitiriya, hutsanana uye firiji. Mumazuva iwayo, vanhu vane chirwere ichi vaionekwa se 'vampire' nekuda kwezviratidzo zvavo zvakaita sevampire zvinosanganisira fangs, denderedzwa rerima rakatenderedza maziso, weti tsvuku uye kusanzwira chiedza chezuva. Nekudaro, mamiriro akazove akanzverwa nenyanzvi dzekurapa uye marapirwo ayo akagadzirwa ^[piri] .

Sayenzi Dzidziso Shure kwePorphyria

Sekureva kwaDesiree Lyon Howe, muvambi weAmerican Porphyria Foundation uye anorwara neacute intermittent porphyria, chirwere ichi chisingawanzozivikanwa chaive chakapararira munharaunda dziri kure muEurope mukati menguva yepakati apo vanhu vaigara kure kure nekubatana kwemazuva ano. nyika ^[25] .

Roger Luckhurst, purofesa mu Modern and Contemporary Literature (London) uye mupepeti webhuku 'Dracula' naBram Stoker akataura zvinhu zvinoverengeka uye zviitiko zvinokonzeresa porphyria muma1730s. Akataura kuti mukati menguva yepakati nepakati, njodzi yakaitika kunzvimbo dziri kure dzeEurope ndokukonzera nzara, denda nezvirwere zvakawanda zvakakomba secatalepsy (kuomarara kwemuviri uye kurasikirwa kwekunzwa) ^[26] .

Nekuda kwekushayikwa kwekubatana nenyika yekunze uye kusakwana kwemishonga, vanhu vane porphyria vakaremara mupfungwa nekuda kwekutya, kushushikana uye zvimwe zvinhu uye vakatanga kuzvidya vega nekuda kwenzara yakanyanya. Zvakare, nekuda kwekusaziva kwekudzivirira kwemazuva ano uye zvinodhaka, zvirwere zvinokonzerwa nekurumwa kwemhuka kunge chimbwamupengo chakapararira muhuwandu panguva iyo yakazovakanganisa kuvenga mvura uye chiedza, kuona zvisizvo uye hukasha.

Chimwe chikonzero, sekutaurwa kwazvakaitwa naPurofesa Roger Luckhurst, zvinoratidza kuti sezvo nharaunda dzeEurope dzakaramba dzakazvimiririra kwenguva yakareba, zvakakonzera kushomeka kwezvokudya zvinovaka muviri nekuda kwekudya zvisina tsarukano uye zvakanyanya kutapukirwa nezvirwere zvakawandisa nekuda kwekuti majini avo angangodaro akachinja zvakanyanya zvichikonzera vampire- sezviratidzo.

Sezvo nguva yakapfuura uye michato ikaitika, izvo zvisina kujairika mumagene zvakapfuura kubva kuvabereki kuenda kuvana uye zvakakonzera kupararira kwemamiriro acho.

Chikonzero chePorphyria

Muvanhu, okisijeni kubva mumapapu inoendeswa kune dzimwe nhengo dzemuviri kuburikidza neprotein yakasarudzika mumasero matsvuku eropa anonzi hemoglobin inovawo inoenderana nerudzi rutsvuku reropa. Hemoglobin ine boka rekuumbiridza rinonzi heme iro rine porphyrin uye iron-ion iri pakati. Inowanzoitwa mumasero matsvuku eropa, mwongo wemapfupa nechiropa.

Heme inogadzirwa nematanho masere akateedzana neimwe enzyme yakasarudzika inogadzirwa ne porphyrin. Kana chero eaya matanho masere akakundikana panguva yekuvakwa kwehemi nekuda kwekuchinja kwemajini kana chepfu yezvakatipoteredza, iyo enzymes inovhiringidzika ichikonzera kushomeka kwayo uye inotungamira kune porphyria. Kune akakomberedza mhando dzakasiyana dzakasiyana dze porphyria uye mamiriro acho akabatana nerudzi rwe enzyme iyo isipo ^[3] .

Mhando dzePorphyria

Kune mana marudzi e porphyria umo maviri anozivikanwa nezviratidzo zvaro uye ekupedzisira maviri akapatsanurwa nepathophysiology.

1. Chiratidzo-chakavakirwa porphyria

• Yakanaka porphyria (AP): Aya mamiriro ekutyisidzira hupenyu anoonekwa nekukurumidza uye anokanganisa system yemitsipa. Zviratidzo zveAP zvinogara kwevhiki imwechete kana maviri uye mushure mekuonekwa, zviratidzo zvinotanga kuwedzera. AP haiwanzo kuitika usati wayaruka uye mushure mekuguma kuenda kumwedzi ^[4] .
• Yakatemwa porphyria (CP): Iwo anonyanya kuve akapatsanurwa kuita matanhatu marudzi uye yega yega mhando inoratidzira mamiriro ane hukama nezvakakomba zvirwere zveganda sekunyanyisa kwezuva, blisters, edema, kutsvuka, mavanga uye kusviba kweganda. Zviratidzo zvePC zvinotanga panguva yehucheche ^[5] .

2. Pathophysiology-yakavakirwa porphyria

• Erythropoietic porphyria: Iyo inoratidzirwa nekuwedzeredza kugadzirwa kwe porphyrins, kunyanya mumabhonzo emapfupa ^[6] .
• Hepatic porphyria : Iyo inoratidzirwa nekuwedzeredza kwekuwedzera kwe porphyrins muropa ^[7] .

Zviratidzo zvePorphyria

Zviratidzo zve porphyria maererano nemhando dzayo zvinotevera.

Yakanaka porphyria

• Kuzvimba uye kurwadziwa kwakanyanya mudumbu
• Kuzvimbirwa, kurutsa kana kuita manyoka
• Mwoyo palpitation
• Mamiriro epfungwa senge kushushikana, kuona zvisizvo, kana paranoia ^[8]
• Kusarara
• Kufenda ^[8]
• Weti tsvuku kana shava ^[9]
• Kutambudzika kwemhasuru, kushaya simba, chiveve kana kuoma mitezo
• Hypertension

Yakatemwa porphyria

• Oversensitivity kune chezuva ^[10]
• Kupisa kurwadziwa muzuva kana mwenje wekugadzira
• Kunorwadza kuzvimba kweganda
• Ganda kutsvuka
• Mavanga uye kusununguka kweganda ^[10]
• Kuwedzera kwebvudzi kukura
• Blister kubva kune zvidiki zvidiki
• Weti yebhuruu-yakasungwa
• Bvudzi risina kujairika kukura kumeso ^{[gumi neimwe]}
• Kusviba kweganda rakafumurwa
• Kuora kwakanyanya kweganda kunoguma kwafumura mazino akafanana nesadza nemiromo mitsvuku.

Njodzi Zviitiko zvePorphyria

Kana porphyria ikawanikwa, inonyanya kukonzerwa nehupfu hwakatipoteredza hunokonzeresa zviratidzo zvevampirism. Izvo zvinotevera:

• Chiedza chezuva ^[1]
• Kudya gariki kana garlic-based chikafu ^[12]
• Hormonal mishonga senge mahormone ekuenda kumwedzi
• Kusvuta ^[13]
• Kushushikana mumuviri kana mupfungwa ^[14]
• Utachiona
• Kushandisa zvinodhaka
• Kudya kana kutsanya
• Zvinodhaka senge mapiritsi ekudzora kuberekwa kana mishonga yepfungwa
• Kunyanyisa kuunganidzwa kwesimbi mumuviri ^{[gumi neshanu]}
• Chiropa chirwere

Zvinetso zvePorphyria

Izvo zvinetso zve porphyria zvinotevera:

• Itsvo kutadza ^[16]
• Kukuvara kweganda zvachose ^[5]
• Kukuvara kwechiropa
• Kunyanya kupera mvura mumuviri ^[4]
• Hyponatremia, yakaderera sodium mumuviri
• Matambudziko akakura ekufema ^[4]

Kuongororwa kwePorphyria

Porphyria dzimwe nguva inonetsa kuona sezvo zviratidzo zvacho zvakafanana neGuillain-Barre syndrome. Zvisinei, kuongororwa kunoitwa nemiedzo inotevera:

• Ropa, weti & stool bvunzo: Kuti uone matambudziko eitsvo nechiropa uye mhando uye nhanho ye porphyrins mumuviri ^[17] .
• Kuedzwa kweDNA: Kuti unzwisise chinokonzera kusanduka kwemajini ^[18] .

Kurapa kwePorphyria

Kurapa kweporphyria kunoenderana nemhando dzayo. Izvo zvinotevera:

• Intravenous mishonga: Hematin, glucose uye mimwe mishonga yemvura inopihwa mukati mekubatira kuchengetedza mwero wehemi, shuga uye zvinwiwa mumuviri. Mushonga unoitwa kunyanya muacute porphyria AP ^[4] .
• Phlebotomy: MuCP, imwe huwandu hweropa hunobviswa mutsinga dzemunhu kudzikisa simbi yedare mumuviri ^[19] .
• Beta carotene zvinodhaka: Kuvandudza kushivirira kweganda kune zuva ^{[makumi maviri]} .
• Antimalarial mishonga: Zvinodhaka zvakaita sehydroxychloroquine uye chloroquine, ayo anoshandiswa kurapa zviratidzo zvemarariya anoshandiswa kutora huwandu hwakawandisa hwe porphyrins kubva mumuviri ^{[makumi maviri nerimwe]} .
• Vitamini D zvinowedzera: Kuvandudza mamiriro akakonzerwa nekuda kwekushomeka kwevhitamini D ^[22] .
• Bone marrow kuisazve: Zvekugadzirwa kwemasero eropa matsva uye ane hutano mumuviri ^{[2. 3]} .
• Stem sero kuisazve: Izvi zvinoitwa nekushandisa rukuvhute tambo yeropa inova sosi yakapfuma yemasero emadzinde pane mongo wemabhonzo ^[24] .

Matipi Ekubata nePorphyria

• Pfeka magiya ekudzivirira kana uri panze muzuva.
• Dzivisa zvinodhaka kana doro kana iwe une porphyria.
• Usadye gariki sezvo zvichikonzera zviratidzo zveichi chiitiko ^[12] .
• Regedza kusvuta ^[13]
• Usatsanye kwenguva yakareba nekuti zvinogona kutungamira mukushomeka kwehumwe hutano mumuviri.
• Ita kufungisisa kana iyo yoga kudzikisira kushushikana.

Kana iwe ukawana hutachiona, kurapa nekukurumidza sezvazvinogona.

1. [1]National Center yeBiotechnology Ruzivo (US). Magene uye Zvirwere [Internet]. Bethesda (MD): National Center yeBiotechnology Ruzivo (US) 1998-. Porphyria.
2. [piri]Cox A. M. (1995). Porphyria uye vampirism: imwe ngano mukugadzira. Pepanhau rezvekurapa rekupedza kudzidza, 71 (841), 643-644. doi: 10.1136 / pgmj.71.841.643-a
3. [3]Ramanujam, V. M., & Anderson, K. E. (2015). Porphyria Diagnostics-Chikamu 1: Pfupiso Pfupiso yePorphyrias. Zvirongwa zvazvino muhunhu hwevanhu, 86, 17.20.1-17.20.26. doi: 10.1002 / 0471142905.hg1720s86
4. [4]Gounden V, Jialal I. Acute Porphyria. [Yakagadziridzwa 2019 Jan 4]. Mune: StatPearls [Internet]. Treasure Island (FL): StatPearls Kudhinda 2019 Jan-.
5. [5]Dawe R. (2017). Mhedziso yeiyo cutaneous porphyrias. F1000Research, 6, 1906. doi: 10.12688 / f1000search.10101.1
6. [6]Lecha, M., Puy, H., & Deybach, J. C. (2009). Erythropoietic protoporphyria. Orphanet bhuku rezvirwere zvisingawanzoitika, 4, 19. doi: 10.1186 / 1750-1172-4-19
7. [7]Arora, S., Mudiki, S., Kodali, S., & Singal, A. K. (2016). Hepatic porphyria: rondedzero yekuongorora. Indian Zvinyorwa zveGastroenterology, 35 (6), 405-418.
8. [8]Whatley SD, Badminton MN. Yakasimba Intermittent Porphyria. 2005 Sep 27 [Yakagadziridzwa 2013 Feb 7]. Muna: Adam MP, Ardinger HH, Pagon RA, et al., Vapepeti. GeneReviews® [Indaneti]. Seattle (WA): Yunivhesiti yeWashington, Seattle 1993-2019.
9. [9]Bhavasar, R., Santoshkumar, G., & Prakash, B. R. (2011). Erythrodontia mukuzvarwa erythropoietic porphyria. Zvinyorwa zvemuromo uye maxillofacial pathology: JOMFP, 15 (1), 69-73. doi: 10.4103 / 0973-029X.80022
10. [10]Edel, Y., & Mamet, R. (2018). Porphyria: Chii Icho uye Ndiani Anofanira Kuongororwa?. Rambam Maimonides magazini yezvokurapa, 9 (2), e0013. doi: 10.5041 / RMMJ.10333
11. [gumi neimwe]Philip, R., Patidar, P. P., Ramachandra, P., & Gupta, K. K. (2012). Iyo ngano yeasina mahormoni bvudzi. Indian magazini ye endocrinology uye metabolism, 16 (3), 483-485. doi: 10.4103 / 2230-8210.95754
12. [12]Thunell, S., Pomp, E., & Brun, A. (2007). Kutungamira kune zvinodhaka porphyrogenicity kufanotaura uye mushonga mushonga mune yakaoma porphyrias. Bhuku reBritish remakiriniki emakemikari, 64 (5), 668-679. doi: 10.1111 / j.0306-5251.2007.02955.x
13. [13]Muromo, G. Y., McColl, K. E., Goldberg, A., & Moore, M. R. (1991). Kusvuta uye kudzokororazve kweacute intermittent porphyria. BMJ (Kliniki yekutsvagisa ed.), 302 (6775), 507. doi: 10.1136 / bmj.302.6775.507
14. [14]Naik, H., Stoecker, M., Sanderson, S. C., Balwani, M., & Desnick, R. J. (2016). Zvakaitika uye kunetsekana kwevarwere vanorwadzwazve neacute hepatic porphyria: Chidzidzo chehunhu. Molecular genetics uye metabolism, 119 (3), 278-283. doi: 10.1016 / j.ymgme.2016.08.006
15. [gumi neshanu]Willandt, B., Langendonk, J. G., Biermann, K., Meersseman, W., D'Heygere, F., George, C.,… Cassiman, D. (2016). Chiropa Fibrosis Yakabatana ne Iron Kuunganidzwa Nekuda Kwerefu-Nguva Heme-Arginate Kurapa muAcute Intermittent Porphyria: A Case Series. JIMD inoshuma, 25, 77-81. doi: 10.1007 / 8904_2015_458
16. [16]Pallet, N., Karras, A., Thervet, E., Gouya, L., Karim, Z., & Puy, H. (2018). Porphyria uye itsvo zvirwere. Clinical itsvo magazini, 11 (2), 191-1977. doi: 10.1093 / ckj / sfx146
17. [17]Woolf, J., Marsden, J. T., Degg, T., Whatley, S., Reed, P., Brazil, N., ... & Badminton, M. (2017). Yakanakisa tsika nhungamiro pane yekutanga-mutsara marabhoritari kuyedzwa kwe porphyria Zvinyorwa zvekiriniki biochemistry, 54 (2), 188-198.
18. [18]Kauppinen, R. (2004). Molecular diagnostics yeacute inopindirana porphyria. Nyanzvi kuongorora kwema molecular diagnostics, 4 (2), 243-249.
19. [19]Lundvall, O. (1982). Phlebotomy kurapwa kweporphyria cutanea tarda. Acta dermato-venereologica. Kuwedzera, 100, 107-118.
20. [makumi maviri]Mathews-Roth, M. M. (1984). Kurapa kwe erythropoietic protoporphyria ine beta-carotene. Mufananidzo-dermatology, 1 (6), 318-321.
21. [makumi maviri nerimwe]Rossmann-Ringdahl, I., & Olsson, R. (2007). Porphyria cutanea tarda: mhedzisiro uye njodzi zviitiko zvehepatotoxicity kubva kumusoro-dhiza chloroquine kurapwa. Acta dermato-venereologica, 87 (5), 401-405.
22. [22]Serrano-Mendioroz, I., Sampedro, A., Mora, M. I., Mauleón, I., Segura, V., de Salamanca, R. E., ... & Fontanellas, A. (2015). Vitamin D-inosunga mapuroteni senge biomarker yeanoshanda chirwere mune yakaoma inopindirana porphyria. Zvinyorwa zveproteomics, 127, 377-385.
23. [2. 3]Tezcan, I., Xu, W., Gurgey, A., Tuncer, M., Cetin, M., Öner, C., ... & Desnick, R. J. (1998). Congenital erythropoietic porphyria yakabudirira kurapwa ne allogeneic bone marrow kuisirwa. Ropa, 92 (11), 4053-4058.
24. [24]Zix-Kieffer, I., Langer, B., Eyer, D., Acar, G., Racadot, E., Schlaeder, G., ... & Lutz, P. (1996). Inobudirira tambo yeropa stem sero kuisirwa kweiyo congenital erythropoietic porphyria (chirwere cheGunther). Bone marora kuisazve, 18 (1), 217-220.
25. [25]Simon, A., Pompilus, F., Querbes, W., Wei, A., Strzok, S., Penz, C.,… Marquis, P. (2018). Mwoyo Murefu Pfungwa paAptute Intermittent Porphyria ine Kakawanda Kurwiswa: Chirwere chine Kuratidzika uye Kusingaperi Kuratidzwa. Murwere, 11 (5), 527-537. doi: 10.1007 / s40271-018-0319-3
26. [26]Daly, N. (2019). [Ongororo yebhuku rinonzi The Cambridge Companion to Dracula ed. naRoger Luckhurst]. Victorian Zvidzidzo 61 (3), 496-498.

Horoscope Yako Yemangwana

Kuziva

Nditumire Neemail Ratidza Pane Saiti