Porphyria (The Vampire Syndrome): Zomwe Zimayambitsa, Zizindikiro, Kuzindikira, Zowopsa Ndi Chithandizo

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Vampire syndrome ndimatenda achilengedwe amwazi omwe amakhudza khungu komanso dongosolo lamanjenje. Pazachipatala, amadziwika kuti porphyria ^[1] . Vutoli limatchedwa 'vampire' chifukwa cha zizindikilo zake zomwe zikufanana ndi mzukwa wakale wazaka za m'ma 1700.

Porphyria idadziwika kalekale, kutatsala pang'ono kupangidwa kwa maantibayotiki, ukhondo ndi firiji. M'masiku amenewo, anthu omwe ali ndi vutoli amawerengedwa kuti ndi 'vampire' chifukwa cha zizindikilo zawo za vampire zomwe zimakhudza mano, mdima wakuda mozungulira maso, mkodzo wofiira komanso kusazindikira dzuwa. Komabe, matendawa pambuyo pake adaphunziridwa ndi akatswiri azachipatala ndipo mankhwala ake adapangidwa ^[ziwiri] .

Mfundo Za Sayansi Zoyambitsa Porphyria

Malinga ndi a Desiree Lyon Howe, omwe anayambitsa American Porphyria Foundation komanso odwala matenda a porphyria, matenda osowawa anali ofala pakati pa madera akutali ku Europe munthawi yazaka zapakati pomwe anthu amakhala kutali kwambiri ndi anthu amakono dziko ^[25] .

Roger Luckhurst, pulofesa wa Modern and Contemporary Literature (London) komanso mkonzi wa buku la 'Dracula' lolembedwa ndi Bram Stoker adatchulapo zinthu zingapo ndi zochitika za porphyria m'ma 1730. Anatinso m'zaka zamakedzana, tsoka linagwera madera akutali ku Europe ndipo linayambitsa njala, miliri ndi matenda ambiri oopsa monga catalepsy (kuuma kwa thupi ndi kutaya chidwi) ^[26] .

Chifukwa chosalumikizana ndi akunja komanso kusakwanira kwa mankhwala, anthu omwe ali ndi porphyria adalemala m'malingaliro chifukwa cha mantha, kukhumudwa ndi zina ndikuyamba kudya okha chifukwa cha njala yayikulu. Komanso, chifukwa chosazindikira katemera wamakono ndi mankhwala osokoneza bongo, matenda omwe amabwera chifukwa cha nyama kuluma ngati matenda a chiwewe anafalikira kwambiri munthawiyo zomwe zidawapangitsa kuti asamamwe madzi ndi kuwala, kuyerekezera zinthu kwanyengo komanso kuchita ndewu.

Chifukwa china, monga adanenera Pulofesa Roger Luckhurst, chikuwonetsa kuti momwe madera aku Europe adakhalabe kwayokha kwa nthawi yayitali, zidadzetsa kusowa zakudya m'thupi chifukwa chodya moperewera komanso kumayambukiridwa ndimatenda angapo chifukwa cha zomwe majini awo akadatha kusintha poyambitsa vampire- monga zizindikiro.

Nthawi ikamadutsa ndikukwatira kumachitika, zovuta zamtundu zimadutsa kuchokera kwa makolo kupita kwa ana ndikupangitsa kufalikira kwa vutoli.

Chifukwa cha Porphyria

Mwa anthu, mpweya wochokera m'mapapu umasamutsidwa mbali zina za thupi kudzera mu protein yapadera m'maselo ofiira ofiira otchedwa hemoglobin yemwenso amathandizira mtundu wofiira wamagazi. Hemoglobin ili ndi gulu lopangira lotchedwa heme lomwe lili ndi porphyrin ndi ayoni wachitsulo pakatikati. Nthawi zambiri amapangidwa m'maselo ofiira ofiira, m'mafupa ndi chiwindi.

Heme amapangidwa munjira zisanu ndi zitatu zotsatizana ndi enzyme yopangidwa ndi porphyrin. Ngati imodzi mwanjira izi zisanu ndi zitatu zikulephera pomanga heme chifukwa cha kusintha kwa majini kapena poizoni wachilengedwe, kaphatikizidwe ka michere kamasokonezeka komwe kumapangitsa kusowa kwake ndikupangitsa porphyria. Pali mitundu yosiyanasiyana ya porphyria ndipo vutoli limalumikizidwa ndi mtundu wa enzyme womwe kulibe ^[3] .

Mitundu Ya Porphyria

Pali mitundu 4 ya porphyria momwe mitundu iwiri imadziwika ndi zizindikilo zake ndipo ziwiri zomalizirazo zimagawidwa ndi matenda am'mimba.

1. Chizindikiro cha porphyria

• Pachimake porphyria (AP): Mkhalidwe wowopsawu umawonekera mwachangu ndipo umakhudza dongosolo lamanjenje. Zizindikiro za AP zimakhala kwa sabata limodzi kapena awiri ndipo atawonekera, zizindikirazo zimayamba kusintha. AP sichimachitika msinkhu wosatha msinkhu komanso pambuyo pa kusamba ^[4] .
• Kudula porphyria (CP): Amagawidwa makamaka mitundu isanu ndi umodzi ndipo mtundu uliwonse umatanthauza mikhalidwe yokhudzana ndi matenda akulu akhungu monga kutentha kwambiri dzuwa, matuza, edema, kufiira, zipsera ndi kuda kwa khungu. Zizindikiro za CP zimayamba ali mwana ^[5] .

2. Pathophysiology-based porphyria

• Erythropoietic porphyria: Amadziwika ndi kuchuluka kwa ma porphyrins, makamaka m'mafupa ^[6] .
• Chiwindi porphyria : Amadziwika ndi kuchuluka kwa porphyrins m'chiwindi ^[7] .

Zizindikiro Za Porphyria

Zizindikiro za porphyria malinga ndi mitundu yake ndi izi.

Porphyria yovuta

• Kutupa ndi kupweteka kwambiri m'mimba
• Kudzimbidwa, kusanza kapena kutsegula m'mimba
• Kugunda kwamtima
• Maganizo monga nkhawa, kuyerekezera zinthu m'maganizo, kapena paranoia ^[8]
• Kusowa tulo
• Kugwidwa ^[8]
• Mkodzo wofiira kapena wofiirira ^[9]
• Kupweteka kwa minofu, kufooka, dzanzi kapena ziwalo
• Matenda oopsa

Kudula porphyria

• Kutengeka kwambiri ndi dzuwa ^[10]
• Kuwotcha kupweteka kwa dzuwa kapena kuwala kochita kupanga
• Kutupa kowawa kwa khungu
• Kufiira kwa khungu
• Zipsera ndi khungu ^[10]
• Kuchuluka kwa kukula kwa tsitsi
• Matuza ochokera pakapangidwe kakang'ono
• Mkodzo wokhala ndi buluu
• Kukula kopanda tsitsi kumaso ^{[khumi ndi chimodzi]}
• Mdima wa khungu lowonekera
• Zipsera zazikulu pakhungu zimabweretsa mano owoneka ngati mano ndi milomo yofiira.

Zowopsa Za Porphyria

Porphyria ikapezeka, imachitika makamaka chifukwa cha poizoni wazachilengedwe omwe amayambitsa zizindikiritso za vampirism. Ndi awa:

• Kuwala kwa dzuwa ^[1]
• Kudya adyo kapena zakudya zopangidwa ndi adyo ^[12]
• Mankhwala a mahormonal monga mahomoni akusamba
• Kusuta ^[13]
• Kupsinjika kwakuthupi kapena kwamaganizidwe ^[14]
• Matenda
• Kugwiritsa ntchito mankhwala osokoneza bongo
• Kudya kapena kusala kudya
• Mankhwala monga mapiritsi oletsa kubereka kapena mankhwala osokoneza bongo
• Kuchulukirachulukira kwachitsulo mthupi ^{[khumi ndi zisanu]}
• Matenda a chiwindi

Zovuta za Porphyria

Zovuta za porphyria ndi izi:

• Impso kulephera ^[16]
• Kuwonongeka kwamuyaya khungu ^[5]
• Kuwonongeka kwa chiwindi
• Kutaya madzi m'thupi kwambiri ^[4]
• Hyponatremia, otsika ndi sodium mu thupi
• Mavuto akulu kupuma ^[4]

Kuzindikira Kwa Porphyria

Porphyria nthawi zina zimakhala zovuta kuzizindikira chifukwa zizindikiro zake zimakhala zofanana ndi matenda a Guillain-Barre. Komabe, matendawa amachitika ndi mayesero otsatirawa:

• Kuyesa magazi, mkodzo & chopondapo: Kuti mupeze mavuto a impso ndi chiwindi komanso mtundu ndi mulingo wa porphyrins mthupi ^[17] .
• Kuyesa kwa DNA: Kuti mumvetsetse chomwe chimayambitsa kusintha kwa majini ^[18] .

Chithandizo cha Porphyria

Chithandizo cha porphyria chimachokera pamitundu yake. Ndi awa:

• Mankhwala osokoneza bongo: Hematin, shuga ndi mankhwala ena amadzimadzi amaperekedwa kudzera m'mitsempha kuti mukhale ndi heme, shuga ndi madzi amthupi. Mankhwalawa amachitika makamaka pachimake porphyria AP ^[4] .
• Phlebotomy: Mu CP, magazi ena amachotsedwa m'mitsempha ya munthu kuti achepetse chitsulo m'thupi ^[19] .
• Beta carotene mankhwala: Kupititsa patsogolo kulolerana kwa khungu ku dzuwa ^{[makumi awiri]} .
• Mankhwala opatsirana pogonana: Mankhwala monga hydroxychloroquine ndi chloroquine, omwe amagwiritsidwa ntchito pochiza matenda a malungo amagwiritsidwa ntchito kuyamwa porphyrins wochuluka mthupi ^{[makumi awiri ndi mphambu imodzi]} .
• Mavitamini D owonjezera: Kupititsa patsogolo zomwe zimachitika chifukwa chakuchepa kwa vitamini D ^[22] .
• Kuika mafuta m'mafupa: Kupanga maselo atsopano komanso athanzi mthupi ^{[2. 3]} .
• Kuika khungu la tsinde: Izi zimachitika pogwiritsa ntchito umbilical cord magazi omwe ndi magwero olemera a maselo kuposa mafupa ^[24] .

Malangizo Okuthandizani Porphyria

• Valani zida zoteteza mukakhala kunja padzuwa.
• Pewani mankhwala osokoneza bongo kapena mowa ngati muli ndi porphyria.
• Osadya adyo chifukwa zimatha kuyambitsa matendawa ^[12] .
• Siyani kusuta ^[13]
• Osasala kudya kwakanthawi chifukwa zitha kupangitsa kusowa kwa michere m'thupi.
• Chitani kusinkhasinkha kapena yoga kuti muchepetse kupsinjika.

Mukalandira matenda, amuthandizeni posachedwa.

1. [1]National Center for Biotechnology Information (US). Chibadwa ndi Matenda [Intaneti]. Bethesda (MD): National Center for Biotechnology Information (US) 1998-. Zovuta.
2. [ziwiri]Cox A. M. (1995). Porphyria ndi vampirism: nthano ina pakupanga. Magazini azachipatala omaliza maphunziro awo, 71 (841), 643-644. onetsani: 10.1136 / pgmj.71.841.643-a
3. [3]Ramanujam, V. M., & Anderson, K. E. (2015). Porphyria Diagnostics-Gawo 1: Mwachidule Porphyrias. Zotsatira zamakono zamtundu wamunthu, 86, 17.20.1-17.20.26. onetsani: 10.1002 / 0471142905.hg1720s86
4. [4]Gounden V, Jialal I. Porphyria Yovuta. [Idasinthidwa 2019 Jan 4]. Mu: StatPearls [Intaneti]. Treasure Island (FL): StatPearls Yofalitsa 2019 Jan-.
5. [5]Dawe R. (2017). Chidule cha porphyrias yodula. F1000Kufufuza, 6, 1906. doi: 10.12688 / f1000search.10101.1
6. [6]Lecha, M., Puy, H., & Deybach, J. C. (2009). Erythropoietic protoporphyria. Buku la Orphanet la matenda osowa, 4, 19. doi: 10.1186 / 1750-1172-4-19
7. [7]Arora, S., Young, S., Kodali, S., & Singal, A. K. (2016). Hepatic porphyria: ndemanga yofotokozera. Indian Journal ya Gastroenterology, 35 (6), 405-418.
8. [8]Whatley SD, Badminton MN (Adasankhidwa) Porphyria Yokhazikika. 2005 Sep 27 [Idasinthidwa 2013 Feb 7]. Mu: Adam MP, Ardinger HH, Pagon RA, et al., Akonzi. GeneReviews® [Intaneti]. Seattle (WA): Yunivesite ya Washington, Seattle 1993-2019.
9. [9]Bhavasar, R., Santoshkumar, G., & Prakash, B. R. (2011). Erythrodontia mu kobadwa nako erythropoietic porphyria. Zolemba zamatenda am'kamwa ndi maxillofacial: JOMFP, 15 (1), 69-73. onetsani: 10.4103 / 0973-029X.80022
10. [10]Edel, Y., & Mamet, R. (2018). Porphyria: Ndi Chiyani Ndipo Ndani Ayenera Kuunikidwa? Magazini azachipatala a Rambam Maimonides, 9 (2), e0013. onetsani: 10.5041 / RMMJ.10333
11. [khumi ndi chimodzi]Philip, R., Patidar, P. P., Ramachandra, P., & Gupta, K. K. (2012). Nkhani ya tsitsi lopanda mahomoni. Nyuzipepala yaku India ya endocrinology ndi metabolism, 16 (3), 483-485. onetsani: 10.4103 / 2230-8210.95754
12. [12]Thunell, S., Pomp, E., & Brun, A. (2007). Kuwongolera kunenedweratu ka porphyrogenicity ndikulamula kwa mankhwala mu porphyrias yovuta. Magazini aku Britain azamankhwala azachipatala, 64 (5), 668-699. onetsani: 10.1111 / j.0306-5251.2007.02955.x
13. [13]Lip, G. Y., McColl, K. E., Goldberg, A., & Moore, M. R. (1991). Kusuta ndi kuukira mobwerezabwereza kwa porphyria yapakatikati. BMJ (Kafukufuku wamankhwala ed.), 302 (6775), 507. onetsani: 10.1136 / bmj.302.6775.507
14. [14]Naik, H., Stoecker, M., Sanderson, S. C., Balwani, M., & Desnick, R. J. (2016). Zochitika ndi kuda nkhawa kwa odwala omwe amabwerezedwanso ndi hepatic porphyria: Kafukufuku woyenera. Ma genetics am'thupi ndi kagayidwe kake, 119 (3), 278-283. onetsani: 10.1016 / j.ymgme.2016.08.006
15. [khumi ndi zisanu]Willandt, B., Langendonk, J. G., Biermann, K., Meersseman, W., D'Heygere, F., George, C.,… Cassiman, D. (2016). Chiwindi cha Fibrosis Chophatikiza ndi Kukundikira Kwachitsulo Chifukwa Chachithandizo Cha Heme-Arginate Chachikulu Porphyria Yovuta Kwambiri: Nkhani Yoyeserera. Malipoti a JIMD, 25, 77-81. onetsani: 10.1007 / 8904_2015_458
16. [16]Pallet, N., Karras, A., Thervet, E., Gouya, L., Karim, Z., & Puy, H. (2018). Matenda a Porphyria ndi impso. Nkhani yachipatala ya impso, 11 (2), 191-197. onetsani: 10.1093 / ckj / sfx146
17. [17]Woolf, J., Marsden, J. T., Degg, T., Whatley, S., Reed, P., Brazil, N., ... & Badminton, M. (2017). Maupangiri abwino kwambiri pakuyesa kwa labu yoyamba kwa porphyria. Zolengeza zamagetsi zamankhwala, 54 (2), 188-198.
18. [18]Kauppinen, R. (2004). Matenda a maselo a porphyria ovuta kwambiri. Kuwunika kwa akatswiri pama cell diagnostics, 4 (2), 243-249.
19. [19]Lundvall, O. (1982). Chithandizo cha Phlebotomy cha porphyria cutanea tarda. Acta dermato-venereologica. Zowonjezera, 100, 107-118.
20. [makumi awiri]Mathews-Roth, M. M. (1984). Chithandizo cha erythropoietic protoporphyria ndi beta-carotene. Chithunzi-dermatology, 1 (6), 318-321.
21. [makumi awiri ndi mphambu imodzi]Rossmann-Ringdahl, I., & Olsson, R. (2007). Porphyria cutanea tarda: zomwe zimayambitsa chiwopsezo cha hepatotoxicity kuchokera kuchipatala cha chloroquine. 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). Mapuloteni omanga Vitamini D ngati biomarker a matenda opatsirana mu pormyent porphyria. Zolemba za proteomics, 127, 377-385.
23. [2. 3]Tezcan, I., Xu, W., Gurgey, A., Tuncer, M., Cetin, M., Öner, C., ... & Desnick, R. J. (1998). Kobadwa nako erythropoietic porphyria bwino mankhwala ndi allogeneic m`mafupa kupatsidwa zina. Magazi, 92 (11), 4053-4058.
24. [24]Zix-Kieffer, I., Langer, B., Eyer, D., Acar, G., Racadot, E., Schlaeder, G., ... & Lutz, P. (1996). Chingwe chopambana chamagazi chothandizira kupatsirana kwa kobadwa nako erythropoietic porphyria (Matenda a Gunther). Kuika mafuta m'mafupa, 18 (1), 217-220.
25. [25]Simoni A., Pompilus F., Querbes W., Wei A., Strzok S., Penz C., Marquis P. (2018). Maganizo a Wodwala Porphyria Wowopsa Wamkati Womwe Amakumana Ndi pafupipafupi: Matenda Omwe Amawonetseredwa Mosasintha Komanso Kwanthawi Yonse. Wodwala, 11 (5), 527-537. onetsani: 10.1007 / s40271-018-0319-3
26. [26]Zamgululi [Ndemanga ya The Cambridge Companion to Dracula ed. Wolemba Roger Luckhurst]. Maphunziro a Victorian 61 (3), 496-498.

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