Cudurka 'Porphyria' (Cudurka 'Vampire Syndrome'): Sababaha, Astaamaha, Cilad-baarista, Cunsurrooyinka Halista Iyo Daaweynta

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Cudurka 'vampire syndrome' waa cillad dhif ah oo ku dhacda hiddo-wadaha dhiiga kaasoo inta badan ku dhaca maqaarka iyo nidaamka neerfaha. Marka laga eego xagga caafimaadka, waxaa loo yaqaannaa porphyria ^[1] . Xaaladda waxaa loogu yeeraa 'vampire' sababtoo ah astaamaheeda oo la mid ah khuraafaadkii khuraafaadka ahaa ee qarnigii 18aad.

Porphyria waxaa la helay waqti fog ka hor, wax badan ka hor soo saarista antibiyootigyada, fayadhowrka iyo qaboojiyaha. Waagaas, dadka xaaladdan qaba waxaa loo tixgeliyey inay yihiin 'vampire' astaamahooda astaamaha vampire-ka oo ay ka mid yihiin fangaska, wareegyada mugdiga ah ee indhaha, kaadida guduudan iyo dareen la’aanta iftiinka qorraxda. Si kastaba ha noqotee, xaaladda ayaa markii dambe ay darseen khubaro caafimaad oo daaweynteeda la ikhtiraacay ^[laba] .

Aragtida cilmiga ah ee ka dambeysa Porphyria

Sida laga soo xigtay Desiree Lyon Howe, oo ah aasaasaha American Porphyria Foundation isla markaana la ildaran xanuunka loo yaqaan 'porphyria' oo aan kala go 'lahayn, cudurkan dhifka ah wuxuu ku faafay bulshooyinka fogfog ee Yurub intii lagu jiray xilligii da'da dhexe ee dadku ku noolaan jireen meel ka fog xiriirka casriga ah. adduunka ^[25] .

Roger Luckhurst, oo ah borofisar ku takhasusay Suugaanta Casriga iyo Casriga (London) iyo tifaftiraha buugga 'Dracula' ee Bram Stoker ayaa xusay dhawr arrimood iyo dhacdooyin mas'uul ka ah porphyria 1730-meeyadii. Wuxuu sheegay in muddadii dhexe, masiibo ku habsatay meelaha fogfog ee Yurub ayna sababtay gaajo, aafo iyo cuduro badan oo daran sida catalepsy (jirka oo adkaada iyo dareenka oo lumiya) ^[26] .

Xiriir la'aanta ka jirta dunida dibeddeeda iyo ku filnaan la'aanta daawooyinka, dadka qaba xanuunka loo yaqaan 'porphyria' ayaa maskax ahaan naafo u ah cabsi, niyad jab iyo arrimo kale waxayna bilaabeen inay is cunaan gaajo aad u daran. Sidoo kale, ogaal la'aanta talaalka casriga ah iyo daroogada, cudurada ka dhasha qaniinyada xoolaha sida cudurka qaniinyada ayaa ku faafay tiro aad u badan mudadaas taas oo u horseeday inay neceb yihiin biyaha iyo iftiinka, dhalanteedka iyo gardarada.

Sabab kale, sida uu xusay Professor Roger Luckhurst, waxay muujineysaa in maadaama bulshooyinkan reer Yurub ay go'doomin ku jireen muddo dheer, ay sababtay nafaqo-xumo cunno xumo darteed iyo u nuglaanta cuduro badan oo ay sababi karto in hiddo-wadayaashoodu isu beddeleen kuwo sii xumaanaya oo keenaya vampire- sida astaamaha.

Waqti ka dib iyo guurkuba wuu dhacay, cilladaha hidde-wadaha ee aan caadiga ahayn ayaa waalidiinta u gudbiyay carruurta waxayna sababeen faafitaanka xaaladda.

Sababta Porphyria

Bini'aadamka, oksijiinta sambabaha waxaa loo wareejiyaa qaybaha kale ee jirka iyadoo loo marayo borotiin gaar ah oo ku jira unugyada dhiigga cas ee loo yaqaan hemoglobin kaas oo sidoo kale mas'uul ka ah midabka casaanka dhiigga. Hemoglobin wuxuu ka kooban yahay koox suuxdin ah oo loo yaqaan heme oo ka kooban porphyrin iyo bir-ion xarunta. Badanaa waxaa lagu sameeyaa unugyada dhiigga cas, dhuuxa lafta iyo beerka.

Heme waxaa lagu sameeyaa sideed tallaabo oo isdaba-joog ah oo midkiiba lagu sameeyo enzyme gooni ah oo ay soo saarto porphyrin. Haddii mid ka mid ah siddeeddan tallaabo uu ku guuldareysto inta lagu guda jiro dhismaha heme sababtoo ah isbeddelka hidde-wadaha ama sunta bey'adda, isku-darka enzymes-ka ayaa khalkhal ku keenaya yaraanshaha oo u horseedaya porphyria. Waxaa jira ku dhowaad noocyo badan oo kala duwan oo loo yaqaan 'porphyria' oo xaaladdu waxay ku xiran tahay nooca enzyme-ka ee maqan ^[3] .

Noocyada Porphyria

Waxaa jira 4 nooc oo loo yaqaan 'porphyria' oo labo ka mid ah lagu garto astaamaheeda halka labada dambe ay u qaybsan yihiin cudurada 'pathophysiology'.

1. Cudurka faafa ee ku saleysan calaamadaha

• Xanuun daran (AP): Xaaladdan nolosha halis gelineysa ayaa si dhakhso leh u muuqata waxayna saameyn ku yeelaneysaa nidaamka neerfaha. Astaamaha AP waxay soconayaan hal ama laba toddobaad ka dib marka ay soo if baxaan, astaamuhu waxay billaabaan inay soo fiicnaadaan. AP dhif ayey dhacdaa ka hor qaan-gaarnimada iyo caadada ka dib ^[4] .
• Cudurka 'porphyria' (CP): Waxay badanaa u kala baxaan 6 nooc nooc walbana wuxuu muujinayaa xaaladaha la xiriira cudurada maqaarka ee daran sida u nuglaanta iftiinka qoraxda, nabarro, barar, casaan, nabarro iyo maqaarka oo madoobaada. Calaamadaha CP waxay bilaabmaan inta lagu jiro carruurnimada ^[5] .

2. Cudurka faafa ee ku saleysan cudurada nafsaaniga ah

• Erythropoietic porphyria: Waxaa lagu gartaa soosaarida xad dhaafka ah ee porphyrins, gaar ahaan dhuuxa lafta ^[6] .
• Cagaarshowga : Waxaa lagu gartaa soo saaridda xad-dhaafka ah ee porphyrins ee beerka ^[7] .

Astaamaha Cudurka 'Porphyria'

Astaamaha loo yaqaan 'porphyria' noocyadeeda waa sida soo socota.

Cudurka faafa ee daran

• Barar iyo xanuun daran oo caloosha ah
• Calool istaag, matag ama shuban
• Wadna garaacid
• Xaaladaha maskaxda sida welwelka, dhalanteedka, ama paranoia ^[8]
• Hurdo la'aan
• Suuxdin ^[8]
• Kaadi cas ama bunni ah ^[9]
• Murqo xanuun, daciifnimo, kabuubyo ama curyaannimo
• Dhiig kar

Cudurka 'porphyria'

• U nuglaanta iftiinka qoraxda ^[10]
• Xanuun ku gubanaya iftiinka qoraxda ama iftiinka macmalka ah
• Barar xanuun leh oo maqaarka ah
• Guduudasho maqaarka
• Nabarro iyo maqaarka oo midab doorsooma ^[10]
• Kordhinta timaha
• Nabaro ka soo baxa nabarrada yaryar
• Kaadi buluug leh
• Kobaca timaha aan caadiga ahayn ee wajiga ^{kow iyo toban}
• Madowga maqaarka soo ifbaxay
• Nabar daran oo maqaarka ka soo gaadha ayaa keena ilkaha u eg fangaska iyo bushimaha guduudan.

Cunsurrada Halista ah ee loo yaqaan 'Porphyria'

Marka porphyria la helo, badanaa waxay sabab u tahay sunta deegaanka ee kicisa astaamaha vampirism. Waxay kala yihiin sidan soo socota:

• Soo-if-baxa qoraxda ^[1]
• Cunista toonta ama cuntooyinka toonta ku saleysan ^[12]
• Daawooyinka hormoonnada sida hormoonnada caadada
• Sigaar cabid ^[13]
• Culeyska jirka ama shucuurta ^[14]
• Caabuq
• Ku xadgudubka maandooriyaha
• Cunnida ama soonka
• Daawooyinka sida kaniiniyada ka hortagga uurka ama daroogada nafsiga ah
• Xadi badan oo bir ah jirka ^{[shan iyo toban]}
• Cudurka beerka

Dhibaatooyinka Porphyria

Dhibaatooyinka 'porphyria' waa sida soo socota:

• Kelyaha oo hawl gab ^[16]
• Dhaawaca maqaarka ee joogtada ah ^[5]
• Dhaawaca beerka
• Fuuqbax daran ^[4]
• Hyponatremia, sodium ku yar jirka
• Dhibaatooyin xagga neefsashada ah oo daran ^[4]

Baadhista Cudurka 'Porphyria'

Cudurka 'Porphyria' mararka qaar way adag tahay in la ogaado maadaama calaamadihiisu ay la mid yihiin cudurka Guillain-Barre syndrome. Si kastaba ha noqotee, ogaanshaha waxaa lagu sameeyaa baaritaanada soo socda:

• Tijaabada dhiigga, kaadida & saxarada: Si loo ogaado dhibaatooyinka kelyaha iyo beerka iyo nooca iyo heerka boorfiinka jirka ^[17] .
• Tijaabada DNA: Si loo fahmo sababta ka dambeysa isbedelka hiddo-wadaha ^[18] .

Daaweynta Cudurka 'Porphyria'

Daaweynta porphyria waxay ku saleysan tahay noocyadeeda. Waxay kala yihiin sidan soo socota:

• Daawada xididada: Hematin, gulukooska iyo dawooyinka kale ee dareeraha ah ayaa la siiyaa xididada si loo ilaaliyo heerarka jirka, sonkorta iyo dareerayaasha jirka. Daaweynta waxaa lagu sameeyaa badanaa 'APphy' oo ba'an ^[4] .
• Flebotomi: CP, qadar cayiman oo dhiig ah ayaa lagala baxayaa xididdada qofka si loo yareeyo heerka birta ee jirka ku jirta ^[19] .
• Daawooyinka beta beta: Si kor loogu qaado dulqaadka maqaarku u leeyahay ileyska qorraxda ^[labaatan] .
• Daawooyinka cudurka duumada: Daawooyinka sida hydroxychloroquine iyo chloroquine, oo loo isticmaalo in lagu daaweeyo astaamaha cudurka malaariyada ayaa loo isticmaalaa in jirka looga dhuuqo qadar xad dhaaf ah ^{[kow iyo labaatan]} .
• Faytamiin D dheeri ah: Si loo hagaajiyo xaaladaha ka dhashay yaraanta fiitamiin D-ga ^[22] .
• Beerista dhuuxa lafta: Soo saarida unugyo dhiig oo cusub oo caafimaad qaba jirka ^{[2. 3]} .
• Beerista unugyada jirridda: Tan waxaa lagu fuliyaa iyadoo la adeegsanayo dhiigga xudunta xudunta oo ah isha hodanka ah ee unugyada asliga ah marka loo eego dhuuxa lafta ^[24] .

Tilmaamaha lagula macaamilayo Porphyria

• Xidho qalabka wax difaaca marka aad bannaanka ka joogto qorraxda.
• Ka fogow daroogada ama aalkolada haddii aad qabtid wax cuncun leh.
• Ha cunin toon maxaa yeelay waxay kicin kartaa calaamadaha xaaladdan ^[12] .
• Sigaar joojinta ^[13]
• Ha soomin muddo dheer maxaa yeelay waxay u horseedi kartaa yaraanta nafaqooyinka qaarkood ee jirka ku jira.
• Samee feker ama yoga si loo yareeyo buuqa.

Haddii aad qaadsiiso infekshan, u daawee sida ugu dhakhsaha badan ee suurtogalka ah.

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