Porphyria (Ciwon Vampire): Sanadin, Ciwon Cutar, Ciwon Gano, Abubuwan Haɗari da Jiyya

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Ciwon vampire cuta ce mai saurin yaduwa ta jini wanda yawanci yakan shafi fata da tsarin juyayi. A cikin likitocin kiwon lafiya, an san shi da porphyria ^[1] . An kira yanayin a matsayin 'vampire' saboda alamun ta wanda yayi kama da tatsuniya na ƙarni na 18.

An gano Porphyria lokaci mai tsawo, da yawa kafin ƙirƙirar maganin rigakafi, tsabtace jiki da sanyaya jiki. A waccan zamanin, ana daukar mutanen da ke wannan yanayin a matsayin 'vampire' saboda alamun cutar vampire wanda ya shafi fuka-fuka, duhun dare a kusa da idanuwa, fitsari jajaye da rashin hasken rana. Koyaya, daga baya masana likitanci sunyi nazarin yanayin kuma an ƙirƙira hanyoyin magance ta ^[biyu] .

Ka'idojin kimiyya Bayan Porphyria

A cewar Desiree Lyon Howe, wanda ya kafa Gidauniyar Porphyria ta Amurka kuma mai fama da mummunan cutar porphyria, wannan cuta wacce ba a cika samunta ba ta yadu a tsakanin al'ummomin da ke nesa da Turai a lokacin tsakiyar shekaru lokacin da mutane suke rayuwa nesa da mutanen zamani. duniya ^[25] .

Roger Luckhurst, farfesa a cikin Littattafan Zamani da Zamani (London) kuma editan littafin 'Dracula' na Bram Stoker ya ambaci abubuwa da yawa da abubuwan da suka haifar da cutar sankara a cikin 1730s. Ya ambaci cewa a zamanin da, wani bala'i ya aukawa yankuna masu nisa na Turai kuma ya haifar da yunwa, annoba da yawancin cututtuka masu tsanani kamar catalepsy (taurin jiki da rashin jin daɗi) ^[26] .

Sakamakon rashin mu'amala da kasashen waje da karancin magunguna, mutanen da ke fama da cutar sanyin jiki sun sami tabin hankali saboda tsoro, damuwa da wasu abubuwan kuma sun fara cin kansu saboda tsananin yunwa. Hakanan, saboda rashin sanin allurar rigakafi ta zamani da magunguna, cututtukan da ake haifarwa sakamakon cizon dabbobi kamar cutar kumburi sun bazu cikin lokaci mai yawa wanda hakan ya haifar musu da ƙyamar ruwa da haske, kallon mafarki da tashin hankali.

Wani dalili kuma, kamar yadda Farfesa Roger Luckhurst ya ambata, yana nuna cewa yayin da waɗannan al'ummomin Turai suka kasance cikin keɓewa na dogon lokaci, hakan ya haifar da rashin abinci mai gina jiki saboda rashin abinci mai gina jiki kuma mai saukin kamuwa da cututtuka da yawa saboda abin da ƙwayoyinsu na iya canzawa don mafi munin abin da ya haifar da vampire- kamar alamomi.

Yayin da lokaci ya wuce kuma aka yi aure, rashin daidaito a cikin kwayar halitta ya wuce ne daga iyaye zuwa yara kuma ya haifar da yaduwar yanayin.

Dalilin Porphyria

A cikin mutane, oxygen daga huhu ana canza shi zuwa wasu sassan jiki ta hanyar furotin na musamman a cikin jajayen jinin da ake kira hemoglobin wanda shi ma ke da alhakin jan launi na jini. Hemoglobin yana dauke da kungiyar roba wacce ake kira heme wacce ta hada da sinadarin porphyrin da kuma iron-ion a tsakiya. Yawanci ana yin sa ne cikin jajayen ƙwayoyin jini, ƙashi da hanta.

Ana yin Heme a matakai takwas na jere kowane ɗayan enzyme daban wanda porphyrin ya samar. Idan kowane ɗayan waɗannan matakai guda takwas ya faɗi a lokacin ginin heme saboda maye gurbi ko gurɓataccen muhalli, haɗarin enzymes zai shiga damuwa wanda zai haifar da rashi kuma yana haifar da porphyria. Akwai kusan nau'ikan nau'ikan porphyria da yawa kuma yanayin yana da alaƙa da nau'in enzyme wanda ba ya nan ^[3] .

Iri Na Porphyria

Akwai nau'ikan nau'ikan porphyria guda 4 wanda biyu ke bayyana ta alamomin ta sannan na biyun sun kasu kashi biyu ta hanyar ilimin mahaifa.

1. Ciwon kamuwa da cututtukan cututtuka

• Babban porphyria (AP): Wannan yanayin barazanar rai ya bayyana da sauri kuma yana shafar tsarin juyayi. Kwayar cututtukan AP na tsawan sati daya ko biyu kuma bayan sun bayyana, alamun sun fara inganta. AP ba safai yake faruwa ba kafin ya balaga da kuma bayan gama al'ada ^[4] .
• Cututtukan porphyria (CP): An rarraba su galibi zuwa nau'ikan 6 kuma kowane nau'in yana nuna yanayin da ke da alaƙa da cututtukan fata masu tsanani kamar ƙarancin hasken rana, kumbura, kumburi, kumburi, jan tabo, tabon fata da duhunta. Alamomin cutar CP suna farawa yayin yarinta ^[5] .

2. Patphyphysiology-based porphyria

• Erythropoietic porfyria: An bayyana shi ta hanyar yawan fito da sinadarin porphyrins, musamman a cikin kashin ƙashi ^[6] .
• Hanyoyin hanta mai zafi : Ana halayyar shi ta hanyar yawan yin porphyrins a cikin hanta ^[7] .

Kwayar cutar Porphyria

Kwayar cutar porphyria bisa ga nau'ikan ta sune kamar haka.

M porphyria

• Kumburi da ciwo mai zafi a cikin ciki
• Maƙarƙashiya, amai ko gudawa
• Bugun zuciya
• Yanayin tunani kamar damuwa, mafarki, ko damuwa ^[8]
• Rashin bacci
• Kamawa ^[8]
• Fitsarin ja ko ruwan kasa ^[9]
• Ciwon tsoka, rauni, rauni ko nakasawa
• Hawan jini

Cututtukan porphyria

• Yada hankali ga hasken rana ^[10]
• Jin zafi a cikin hasken rana ko hasken wucin gadi
• Fata mai zafi na fata
• Jan fata
• Scars da canza launin fata ^[10]
• Growthara haɓakar gashi
• Fusoshi daga ƙananan ƙananan abubuwa
• Fitsarin mai shuɗi
• Ciwan gashi mara kyau a fuska ^{[goma sha]}
• Duhun fatar da ta fallasa
• Tsananin tabo na fata wanda ke haifar da hakora masu kama da fatar baki da jan lebe.

Dalilan Hadarin Na Porphyria

Lokacin da aka samo porphyria, yawanci saboda gubobi masu guba wanda ke haifar da alamun cutar vampirism. Su ne kamar haka:

• Hasken rana ^[1]
• Cin tafarnuwa ko kayan abinci na tafarnuwa ^[12]
• Magungunan Hormonal kamar homonin haila
• Shan taba ^[13]
• Jiki ko halin damuwa ^[14]
• Kamuwa da cuta
• Zaman abubuwa
• Cin abinci ko azumi
• Magunguna kamar ƙwayoyin hana haihuwa ko ƙwayoyin cuta
• Yawan iron a jiki ^{[goma sha biyar]}
• Ciwon Hanta

Rarraba na Porphyria

Rikitarwa na porphyria sune kamar haka:

• Rashin koda ^[16]
• Lalacewar fata na dindindin ^[5]
• Lalacewar hanta
• Rashin ruwa mai tsanani ^[4]
• Hyponatremia, ƙananan sodium a jiki
• Matsaloli masu tsananin numfashi ^[4]

Ganewar asali na Porphyria

Porphyria wani lokacin yana da wahalar ganowa saboda alamun ta sunyi kama da cutar Guillain-Barre. Koyaya, ganewar asali ana aiwatar dashi ta hanyar gwaje-gwaje masu zuwa:

• Jini, fitsari & gwajin kwalliya: Don gano matsalolin koda da na hanta da nau'in da matakin sinadarin porphyrins a jiki ^[17] .
• Gwajin DNA: Don fahimtar dalilin bayan maye gurbi ^[18] .

Jiyya Na Porphyria

Maganin porphyria ya dogara da nau'ikansa. Su ne kamar haka:

• Magungunan jijiyoyin jini: Ana ba da sinadarin Hematin, glucose da sauran magunguna ta jijiyoyi don kiyaye matakan heme, sukari da ruwa a jiki. Ana yin maganin musamman a cikin AP mai tsanani ^[4] .
• Tsarin halitta: A cikin CP, ana cire wani adadi na jini daga jijiyoyin mutum don rage matakin ƙarfe a jiki ^[19] .
• Beta carotene magunguna: Don inganta haƙurin fata zuwa hasken rana ^[ashirin] .
• Magungunan antimalaria: Ana amfani da magunguna kamar hydroxychloroquine da chloroquine, waɗanda ake amfani da su don magance cututtukan zazzabin cizon sauro don shanye yawan iska mai yawa daga jiki ^{[ashirin da daya]} .
• Vitamin D kari: Don inganta yanayin da ya haifar saboda ƙarancin bitamin D ^[22] .
• Dasawar kasusuwa: Don samar da sabbin lafiyayyan kwayoyin jini a jiki ^{[2. 3]} .
• Dasawar kwayar kara: Ana gudanar da wannan ta amfani da jinin igiyar cibiya wanda shine asalin tushen ƙwayoyin sel fiye da bargon ƙashi ^[24] .

Nasihu Don Magance tare da Porphyria

• Sanya kayan aiki masu kariya yayin waje a rana.
• Guji ƙwayoyi ko barasa idan kuna da porphyria.
• Kada ku ci tafarnuwa saboda tana iya haifar da alamun wannan yanayin ^[12] .
• Dakatar da shan taba ^[13]
• Kada a dade ana azumi domin hakan na iya haifar da karancin wasu sinadarai a jiki.
• Yi tunani ko yoga don rage damuwa.

Idan ka kamu da cuta, yi magani da wuri-wuri.

1. [1]Cibiyar Kula da Bayanan Halitta ta Duniya (US). Kwayar Halitta da Cuta [Intanet]. Bethesda (MD): Cibiyar Nazarin Harkokin Kimiyyar Kimiyyar Kimiyya ta Duniya (US) 1998-. Porphyria.
2. [biyu]Cox A. M. (1995). Porphyria da vampirism: wani labari ne a cikin yin. Postgraduate likita jarida, 71 (841), 643-644. Doi: 10.1136 / pgmj.71.841.643-a
3. [3]Ramanujam, V. M., & Anderson, K. E. (2015). Ciwon Cutar Porphyria-Sashe na 1: Takaitaccen Bayani game da Porphyrias. Ka'idoji na yau da kullun a cikin kwayar halittar mutum, 86, 17.20.1-17.20.26. Doi: 10.1002 / 0471142905.hg1720s86
4. [4]Gounden V, Jialal I. Porananan Porphyria. [An sabunta 2019 Jan 4]. A cikin: StatPearls [Intanit]. Tsibirin Taskar (FL): StatPearls Publishing 2019 Jan-.
5. [5]Dawe R. (2017). Bayani game da cututtukan fata. F1000Research, 6, 1906. doi: 10.12688 / f1000research.10101.1
6. [6]Lecha, M., Puy, H., & Deybach, JC (2009). Maganin kwayar cutar Erythropoietic. Littafin marayu na cututtukan da ba safai ba, 4, 19. doi: 10.1186 / 1750-1172-4-19
7. [7]Arora, S., Young, S., Kodali, S., & Singal, A. K. (2016). Hepatic porphyria: nazari mai mahimmanci. Jaridar Indiya ta Gastroenterology, 35 (6), 405-418.
8. [8]Whatley SD, Badminton MN. Mutuwar Cutar Porphyria. 2005 Sep 27 [An sabunta 2013 Feb 7]. A cikin: Adam MP, Ardinger HH, Pagon RA, et al., Editoci. GeneReviews® [Intanet]. Seattle (WA): Jami'ar Washington, Seattle 1993-2019.
9. [9]Bhavasar, R., Santoshkumar, G., & Prakash, B. R. (2011). Erythrodontia a cikin ƙwararriyar erythropoietic porphyria. Littafin jarida na maganin cututtukan baka da maxillofacial: JOMFP, 15 (1), 69-73. Doi: 10.4103 / 0973-029X.80022
10. [10]Edel, Y., & Mamet, R. (2018). Porphyria: Mecece kuma Wanene Yakamata a ?.ididdige?. Rambam Maimonides mujallar likita, 9 (2), e0013. Doi: 10.5041 / RMMJ.10333
11. [goma sha]Philip, R., Patidar, P. P., Ramachandra, P., & Gupta, K. K. (2012). Labari na gashin mara izini. Jaridar Indiya ta endocrinology da metabolism, 16 (3), 483-485. Doi: 10.4103 / 2230-8210.95754
12. [12]Thunell, S., Pomp, E., & Brun, A. (2007). Jagora ga yin amfani da kwayar cutar ta shan magani da kuma takardar magani a cikin mummunan cutar. Jaridar Burtaniya ta likitancin magunguna, 64 (5), 668-679. Doi: 10.1111 / j.0306-5251.2007.02955.x
13. [13]Lip, G. Y., McColl, K. E., Goldberg, A., & Moore, M. R. (1991). Shan sigari da kuma sake kai hare-hare na mummunan porphyria. BMJ (Binciken na asibiti), 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). Experiwarewa da damuwa na marasa lafiya tare da hare-hare na yau da kullun na cututtukan hanta mai haɗari: Nazarin ƙwarewa. Kwayoyin halittar jini da canzawa, 119 (3), 278-283. Doi: 10.1016 / j.ymgme.2016.08.006
15. [goma sha biyar]Willandt, B., Langendonk, J. G., Biermann, K., Meersseman, W., D'Heygere, F., George, C.,… Cassiman, D. (2016). Fibrosis na Hanta da ke haɗuwa da Haɗar ƙarfe Saboda Kulawar Heme-Arginate na Tsawon Lokaci a cikin Babban Tsakanin Porphyria: Tsarin Layi. Rahoton JIMD, 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 da cututtukan koda. Jaridar koda ta asibiti, 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). Mafi kyawun jagororin aiki akan gwajin dakin gwaje-gwaje na farko don porphyria. Litattafan ilimin kimiyyar biochemistry, 54 (2), 188-198.
18. [18]Kauppinen, R. (2004). Hanyoyin binciken kwayar halitta na saurin rikicewar cutar porphyria. Binciken masana game da binciken kwayoyin, 4 (2), 243-249.
19. [19]Lundvall, O. (1982). Maganin Phlebotomy na porphyria cutanea tarda. Dokar dermato-venereologica. Arin kari, 100, 107-118.
20. [ashirin]Mathews-Roth, M. M. (1984). Jiyya na erythropoietic protoporphyria tare da beta-carotene. Photo-dermatology, 1 (6), 318-321.
21. [ashirin da daya]Rossmann-Ringdahl, I., & Olsson, R. (2007). Porphyria cutanea tarda: sakamako da halayen haɗari don rashin jinƙai daga maganin chloroquine mai ƙarfi. Dokar 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 furotin mai ɗaurewa azaman biomarker na cuta mai aiki a cikin mummunan tsaka-tsakin porphyria. Jaridar proteomics, 127, 377-385.
23. [2. 3]Tezcan, I., Xu, W., Gurgey, A., Tuncer, M., Cetin, M., Öner, C., ... & Desnick, R.J (1998). Enanƙasar erythropoietic porphyria da aka samu nasarar magance shi ta hanyar dashen ƙwayar ƙashi na allogeneic. Jini, 92 (11), 4053-4058.
24. [24]Zix-Kieffer, I., Langer, B., Eyer, D., Acar, G., Racadot, E., Schlaeder, G., ... & Lutz, P. (1996). Nasarar da tayi mai nasara ta haifar da dashen kwayar halitta don haifarda cututtukan erythropoietic porphyria (cutar Gunther). Dashen dusar ƙashi, 18 (1), 217-220.
25. [25]Simon, A., Pompilus, F., Querbes, W., Wei, A., Strzok, S., Penz, C.,… Marquis, P. (2018). Hankalin Masu haƙuri a kan Mutuwar Cutar Porphyria tare da Kai Hare-hare akai-akai: Cuta tare da Bayyanarwar kai tsaye da Bayyanar Bayani. Mai haƙuri, 11 (5), 527-537. Doi: 10.1007 / s40271-018-0319-3
26. [26]Daly, N. (2019). [Bita na littafin Abokin Cambridge zuwa Dracula ed. by Roger Luckhurst]. Nazarin Victoria na 61 (3), 496-498.

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