{"id":13483,"date":"2023-06-21T15:46:09","date_gmt":"2023-06-21T12:46:09","guid":{"rendered":"http:\/\/blog.ulubat.org\/?p=13483"},"modified":"2023-06-21T15:46:13","modified_gmt":"2023-06-21T12:46:13","slug":"porphyria-vampirler-ve-tarim-ilaclari","status":"publish","type":"post","link":"https:\/\/blog.ulubat.org\/index.php\/genel\/porphyria-vampirler-ve-tarim-ilaclari\/","title":{"rendered":"PORPHYRIA: VAMP\u0130RLER VE TARIM \u0130LA\u00c7LARI"},"content":{"rendered":"\n<p>Bu yaz\u0131da vampir ve kurt adam hikayelerine ilham oldu\u011fu d\u00fc\u015f\u00fcn\u00fclen porphyria (porfiri-porfiria) ve bu hikayeler ile ilgili genel bilgiler edinece\u011fiz.<\/p>\n\n\n\n<blockquote class=\"wp-block-quote\"><p>&nbsp; Hemoglobindeki Hem&nbsp;molek\u00fcl\u00fc i\u00e7in gereken sekiz basamakl\u0131 \u00fcretim a\u015famalar\u0131n\u0131n herhangi bir noktas\u0131nda, bir nedenle&nbsp;kesilme olursa bu duruma Porfiri Hastal\u0131\u011f\u0131 denir. Hangi basama\u011f\u0131n durdu\u011funa g\u00f6re porfiri hastal\u0131\u011f\u0131n\u0131n alt tiplerinden birisi belirlenir. Bu alt tipler akut (hepatik) porfiri ya da kutan\u00f6z (eritropoetik) porfiri olmak \u00fczere iki alt ba\u015fl\u0131kta toplanabilir.<\/p><cite>T\u00dcRK\u0130YE PORF\u0130R\u0130 DERNE\u011e\u0130<\/cite><\/blockquote>\n\n\n\n<p><\/p>\n\n\n\n<figure class=\"wp-duotone-rgb255255255-rgb187114114-1 wp-block-image size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/a\/a2\/Heme_synthesis.svg\" alt=\"\" width=\"803\" height=\"405\" \/><figcaption>Hem Sentezi<br>https:\/\/commons.wikimedia.org\/wiki\/File:Heme_synthesis.svg<\/figcaption><\/figure>\n\n\n\n<h2>PORF\u0130R\u0130LER<\/h2>\n\n\n\n<p>Porfiriler, hem biyosentezi yolu boyunca kusurlu enzim metabolizmas\u0131na ba\u011fl\u0131 olarak n\u00f6rovisseral (akut) ve\/veya kutan\u00f6z semptomlarla kendini g\u00f6sterebilen kal\u0131tsal veya edinilmi\u015f bir grup nadir metabolik bozukluktur. Porfirileri klinik olarak akut ve akut olmayan (kutan\u00f6z) olarak s\u0131n\u0131fland\u0131rabiliriz.<\/p>\n\n\n\n<p><strong>Akut Porfiri:<\/strong><\/p>\n\n\n\n<p>N\u00f6rovisseral (akut) porfiriler, bir tetikleyiciye maruz kalma dolay\u0131s\u0131yla a\u015f\u0131r\u0131 hem \u00fcretiminin ind\u00fcklendi\u011fi akut ataklar ile karakterize edilir.&nbsp;Akut atak genellikle \u015fiddetli kar\u0131n a\u011fr\u0131s\u0131, kusma ve ta\u015fikardi semptomlar\u0131na neden olur. Bunun yan\u0131 s\u0131ra hipertansiyon, hiponatremi, periferik n\u00f6ropati ve hafif zihinsel semptomlar da g\u00f6zleyebiliriz. \u015eiddetli ataklarda, n\u00f6betler ve psikoz gibi a\u011f\u0131r durumlarla kar\u015f\u0131la\u015fabiliriz. Hastal\u0131k a\u00e7\u0131s\u0131ndan etkili bir tedavi ba\u015flat\u0131l\u0131p kontrol alt\u0131na al\u0131nmal\u0131d\u0131r; aksi takdirde ataklar dolay\u0131s\u0131yla periferik, merkezi ve otonom sinir sistemi etkilenerek fel\u00e7, solunum yetmezli\u011fi, koma ve \u00f6l\u00fcme kadar gidebilen ciddi harabiyetlere sebep olabilir.&nbsp;<\/p>\n\n\n\n<p><strong>Kutan\u00f6z Porfiri:<\/strong><\/p>\n\n\n\n<p>Kutan\u00f6z semptomlar, kan dola\u015f\u0131m\u0131ndaki y\u00fcksek porfirinlerin bir sonucudur. I\u015f\u0131\u011fa duyarl\u0131l\u0131k, cilt k\u0131r\u0131lganl\u0131\u011f\u0131 ve kabarc\u0131klar ile kendini g\u00f6sterir. G\u00fcne\u015f \u0131\u015f\u0131\u011f\u0131na en k\u00fc\u00e7\u00fck bir maruz kalma bile v\u00fccutlar\u0131nda ciddi \u015fekil bozukluklar\u0131na yol a\u00e7abilir.<\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/0\/0d\/Blister_in_porphyria_cutanea_tarda.jpg\" alt=\"\" width=\"325\" height=\"432\" \/><figcaption>Porphyria Cutanea Tarda&#8217;da Ciltte Olu\u015fan Kabarc\u0131klar<br>https:\/\/commons.wikimedia.org\/wiki\/File:Blister_in_porphyria_cutanea_tarda.jpg<\/figcaption><\/figure><\/div>\n\n\n<h2>PORF\u0130R\u0130LERDE ALT T\u0130P SINIFLANDIRMASI<\/h2>\n\n\n\n<p>Porfirilerde  alt tip s\u0131n\u0131fland\u0131rmas\u0131 hangi enzimin eksik oldu\u011funa g\u00f6re yap\u0131l\u0131r.<\/p>\n\n\n\n<div class=\"wp-container-6 wp-block-columns\">\n<div class=\"wp-container-5 wp-block-column\" style=\"flex-basis:100%\">\n<div class=\"wp-container-4 wp-block-columns\">\n<div class=\"wp-container-3 wp-block-column\" style=\"flex-basis:100%\">\n<div class=\"wp-container-2 wp-block-group\"><div class=\"wp-block-group__inner-container\">\n<figure class=\"wp-block-table\"><table><tbody><tr><td><strong>Porfiri Alt Tipi<\/strong><\/td><td><strong>Eksik Enzim<\/strong><\/td><td><strong>Tipi<\/strong><\/td><td><strong>Kal\u0131t\u0131m T\u00fcr\u00fc<\/strong><\/td><\/tr><tr><td>Aminolevulinat Dehidrataz Eksik Porfiri (ALADP)<\/td><td>5-aminolevulinat Dehidrataz (ALAD)<\/td><td>Hepatik (Akut)<\/td><td>Otozomal Resesif<\/td><\/tr><tr><td>Akut \u0130ntermittan Porfiri (AIP)<\/td><td>Hidroksimetilbilan Sentaz (HMBS)<\/td><td>Hepatik (Akut)<\/td><td>Otozomal Dominant<\/td><\/tr><tr><td>Konjenital Eritropoietik Porfiri (CEP)<\/td><td>\u00dcroporfirinojen Sentaz (UROS)<\/td><td>Eritropoietik (Kutan\u00f6z)<\/td><td>Otozomal Resesif<\/td><\/tr><tr><td>Porphyria Cutanea Tarda (PCT)<\/td><td>\u00dcroporfirinojen Dekarboksilaz (UROD)<\/td><td>Hepatik (Akut)<\/td><td>%80 Sporadik %20 Otozomal Dominant<\/td><\/tr><tr><td>Herediter Koproporfiri (HCP)<\/td><td>Koproporfirinojen Oksidaz (CPOX)<\/td><td>Hepatik (Akut)<\/td><td>Otozomal Dominant<\/td><\/tr><tr><td>Harderoporfiri (\u00c7ok Nadirdir)<\/td><td>Koproporfirinojen Oksidaz (CPOX)<\/td><td>Eritropoietik (Kutan\u00f6z)<\/td><td>Otozomal Resesif<\/td><\/tr><tr><td>Variegate Porfiri (VP)<\/td><td>Protoporfirinojen Oksidaz (PPOX)<\/td><td>Hepatik (Akut)<\/td><td>Otozomal Dominant<\/td><\/tr><tr><td>Eritropoietik Protoporfiri (EPP)<\/td><td>Ferro\u015felataz (FECH)<\/td><td>Eritropoietik (Kutan\u00f6z)<\/td><td>Otozomal Resesif<\/td><\/tr><\/tbody><\/table><\/figure>\n<\/div><\/div>\n<\/div>\n<\/div>\n<\/div>\n<\/div>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Prfr1.jpg\" alt=\"\" width=\"603\" height=\"300\" \/><figcaption>https:\/\/commons.wikimedia.org\/wiki\/File:Prfr1.jpg<\/figcaption><\/figure><\/div>\n\n\n<h2>LABORATUVAR TE\u015eH\u0130S\u0130<\/h2>\n\n\n\n<p>Akut porfiri ataklar\u0131, aminolaevulinik asit ve porfobilinojenin artan idrar at\u0131l\u0131m\u0131 ile ili\u015fkilidir. &nbsp;Porfobilinojenin porfirinlere ve di\u011fer pigmentlere polimerizasyonu nedeniyle idrar renginin koyu oldu\u011fu g\u00f6zlenir. Aminolaevulinik asit ve porfobilinojen konsantrasyonlar\u0131n\u0131n do\u011fru \u00f6l\u00e7\u00fcm\u00fc i\u00e7in \u0131\u015f\u0131ktan korunan taze bir idrar \u00f6rne\u011fi uzman bir laboratuvara g\u00f6nderilmelidir.<\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g002-1024x1024.jpg\" alt=\"\" class=\"wp-image-13823\" width=\"669\" height=\"669\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g002-1024x1024.jpg 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g002-150x150.jpg 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g002-1536x1536.jpg 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g002-2048x2048.jpg 2048w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g002-768x768.jpg 768w\" sizes=\"(max-width: 669px) 100vw, 669px\" \/><figcaption>Akut porfiride enzim ve enzim \u00fcr\u00fcnleri miktar\u0131ndan yararlanarak tan\u0131ya ula\u015fma.<br>https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC5916231\/figure\/f2-rmmj-9-2-e0013\/<\/figcaption><\/figure><\/div>\n\n\n<p>Eritropoietik protoporfiri, eritrosit i\u00e7ermeyen protoporfirin \u00f6l\u00e7\u00fclerek te\u015fhis edilir veya d\u0131\u015flan\u0131r.&nbsp;\u0130drar ve d\u0131\u015fk\u0131 analizi faydal\u0131d\u0131r. Kalan kutan\u00f6z porfirilerden birine sahip oldu\u011fundan \u015f\u00fcphelenilen hastalarda; bireysel porfirinlerin suda \u00e7\u00f6z\u00fcn\u00fcrl\u00fc\u011f\u00fc farkl\u0131 oldu\u011fundan, hem idrarda hem de d\u0131\u015fk\u0131da a\u015f\u0131r\u0131 porfirin olup olmad\u0131\u011f\u0131n\u0131n incelenmesi esast\u0131r. Pozitif bulgular, ayr\u0131 ayr\u0131 porfirinleri (izomerler dahil olmak \u00fczere) tan\u0131mlamak ve \u00f6l\u00e7mek i\u00e7in fraksiyonlama ile takip edilmelidir.&nbsp;Bu durumlarda hasta genellikle porfiri konusunda deneyimli uzman bir laboratuvara sevk edilir.<\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g003-1024x1024.jpg\" alt=\"\" class=\"wp-image-13822\" width=\"581\" height=\"581\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g003-1024x1024.jpg 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g003-150x150.jpg 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g003-1536x1536.jpg 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g003-2048x2048.jpg 2048w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/rmmj-9-2-e0013-g003-768x768.jpg 768w\" sizes=\"(max-width: 581px) 100vw, 581px\" \/><figcaption>Kutan\u00f6z porfiride semptomlar ve metabolik \u00fcr\u00fcn d\u00fczeyleri ile tan\u0131ya ula\u015fma.<br>https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC5916231\/figure\/f3-rmmj-9-2-e0013\/<\/figcaption><\/figure><\/div>\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large\"><img src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Urine_of_patient_with_porphyria.png\" alt=\"\" \/><figcaption>(Sa\u011fda) Porfiri Hastas\u0131 \u0130drar\u0131<br>https:\/\/commons.wikimedia.org\/wiki\/File:Urine_of_patient_with_porphyria.png<\/figcaption><\/figure><\/div>\n\n\n<h2>TEDAV\u0130<\/h2>\n\n\n\n<p>\u00d6ncelikle porfiri hastalar\u0131na re\u00e7ete edilecek ila\u00e7lar\u0131n hastal\u0131\u011f\u0131 tetiklemeyecek t\u00fcrden olmas\u0131na (porfirinojenik olmayan) dikkat edilmelidir. Hastan\u0131n hangi porfiri alt tipine sahip oldu\u011funa karar verdikten sonra tedaviye h\u0131zl\u0131ca ba\u015flanmal\u0131d\u0131r.<\/p>\n\n\n\n<p>Akut porfirilerde en k\u0131sa s\u00fcrede ba\u015flanan intraven\u00f6z hemin tedavisi genellikle en etkili tedavidir.&nbsp;Tek ba\u015f\u0131na intraven\u00f6z glukoz, genellikle hafif ataklar i\u00e7in veya hemin tedavisi uygun olana kadar kullan\u0131l\u0131r. Akut porfiri ataklar\u0131n\u0131 tetikleyen baz\u0131 unsurlar vard\u0131r ( barbit\u00fcratlar ve \u00f6strojenler, progesteronlar, s\u00fclfonamidler, metildopa, danazol, diazepam, fenitoin, karbamazepin, s\u00fclfonil\u00fcreler, kloramfenikol, tetrasiklinler, baz\u0131 antihistaminikler, oru\u00e7, sigara i\u00e7mek, alkol, uyu\u015fturucu madde kullan\u0131m\u0131, enfeksiyon, duygusal ve fiziksel stres, regl). Bu tetikleyici fakt\u00f6rler ortadan kald\u0131r\u0131lmal\u0131 ve uygun destekleyici ve semptomatik tedavi ba\u015flat\u0131lmal\u0131d\u0131r. &nbsp;H\u0131zl\u0131 tan\u0131 ve tedavi, prognozu b\u00fcy\u00fck \u00f6l\u00e7\u00fcde iyile\u015ftirme ve ciddi veya kronik n\u00f6ropatik semptomlar\u0131n geli\u015fimini \u00f6nleme potansiyeline sahiptir. Hastan\u0131n risk alt\u0131nda olacak akrabalar\u0131n\u0131n da taranmas\u0131 ve dan\u0131\u015fmanl\u0131k hizmeti almalar\u0131 \u015fiddetle \u00f6nerilir.<\/p>\n\n\n\n<p>Kutan\u00f6z porfirilerde g\u00fcne\u015f \u0131\u015f\u0131\u011f\u0131ndan ka\u00e7\u0131nmak ve g\u00fcne\u015f \u0131\u015f\u0131\u011f\u0131na kar\u015f\u0131 tolerans\u0131 art\u0131rabilecek y\u00fcksek dozlarda beta-karoten verilmesi hastal\u0131\u011f\u0131n y\u00f6netiminde en \u00f6nemli unsurlardand\u0131r. Porphyria cutanea tarda, flebotomi veya d\u00fc\u015f\u00fck doz klorokin ile etkili bir \u015fekilde tedavi edilebilir. Konjenital eritropoietik porfiri tedavisinde, kemik ili\u011fi transplantasyonunun ba\u015far\u0131l\u0131 oldu\u011funu g\u00f6steren birka\u00e7 vaka vard\u0131r.<\/p>\n\n\n<div class=\"wp-block-image is-style-default\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/c\/c0\/Congenital-erythropoietic-porphyria-2.jpg\" alt=\"\" width=\"480\" height=\"347\" \/><figcaption>Konjenital Eritropoietik Porfiri Hastas\u0131<br>https:\/\/commons.wikimedia.org\/wiki\/File:Congenital-erythropoietic-porphyria-2.jpg<\/figcaption><\/figure><\/div>\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/6\/6c\/Congenital-erythropoietic-porphyria-5.jpg\" alt=\"\" width=\"505\" height=\"366\" \/><figcaption>Konjenital Eritropoietik Porfiri Hastas\u0131n\u0131n Di\u015flerinin G\u00f6r\u00fcn\u00fcm\u00fc<br>https:\/\/commons.wikimedia.org\/wiki\/File:Congenital-erythropoietic-porphyria-5.jpg<\/figcaption><\/figure><\/div>\n\n\n<h2>PORF\u0130R\u0130LER VE VAMP\u0130R-KURT ADAM H\u0130KAYELER\u0130<\/h2>\n\n\n\n<p>Vampirler ve kurt adamlar daha \u00e7ok Avrupa masallar\u0131nda kar\u015f\u0131m\u0131za \u00e7\u0131kan mitler olmalar\u0131na kar\u015f\u0131n bir\u00e7ok k\u00fclt\u00fcrde yer bulmu\u015ftur. Sanat d\u00fcnyas\u0131nda da gerek filmler gerek resimler gerek edebi eserler olarak \u00e7ok\u00e7a yer alm\u0131\u015flard\u0131r. Daha yak\u0131n tarihe bakt\u0131\u011f\u0131m\u0131zda ise bilgisayar oyunlar\u0131nda da s\u0131k\u00e7a g\u00f6rd\u00fc\u011f\u00fcm\u00fcz ciddi bir pop\u00fcler k\u00fclt\u00fcr \u00f6gesi haline geldi\u011fini g\u00f6r\u00fcyoruz. Daha \u00e7ok \u00fcrpertici giyimleri, sivri ve uzun di\u015fleri, soluk y\u00fczleri ile karakterize edilen vampirlerin kan emdikleri ve \u00e7e\u015fitli paranormal rit\u00fceller ger\u00e7ekle\u015ftirdiklerine inan\u0131l\u0131r. G\u00fcne\u015f \u0131\u015f\u0131\u011f\u0131ndan ve sar\u0131msaktan son derece rahats\u0131z olduklar\u0131 \u015feklindeki tasvirler \u00e7o\u011funluktad\u0131r.  Fiziksel olarak ise kimi zaman yarasaya d\u00f6n\u00fc\u015febilen, u\u00e7abilen, \u00e7ok h\u0131zl\u0131 hareket edebilen, \u00e7ok uzun pen\u00e7eleri olan, \u00e7ok g\u00fc\u00e7l\u00fc olan ve aynada g\u00f6r\u00fcnmeyen gibi \u015fekillerde de i\u015flenmi\u015ftirler. <\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/upload.wikimedia.org\/wikipedia\/commons\/6\/60\/Sharon_Tate_in_The_Fearless_Vampire_Killers_%281969%29_%28cropped%29.jpg\" alt=\"\" width=\"280\" height=\"430\" \/><figcaption>The Fearless Vampire Killers Filminde Sharon Tate<br>https:\/\/commons.wikimedia.org\/wiki\/File:Sharon_Tate_in_The_Fearless_Vampire_Killers_(1969)_(cropped).jpg<\/figcaption><\/figure><\/div>\n\n\n<p>En \u00fcnl\u00fc vampir hikayesi olan &#8220;Dracula&#8221;, Bram Stoker taraf\u0131ndan 1897 y\u0131l\u0131nda yaz\u0131lm\u0131\u015ft\u0131r. Bu romanda, Do\u011fu Avrupa&#8217;n\u0131n Transilvanya b\u00f6lgesinde ya\u015fayan Kont Dracula&#8217;n\u0131n hikayesi anlat\u0131l\u0131r. Kont Dracula&#8217;n\u0131n karakteri, g\u00fc\u00e7l\u00fc, karizmatik ve insanlar\u0131n kan\u0131n\u0131 i\u00e7en bir vampir olarak tasvir edilir. Bu roman, pop\u00fcler k\u00fclt\u00fcrdeki vampir imaj\u0131n\u0131n temelini atm\u0131\u015f ve bir\u00e7ok di\u011fer vampir hikayesine ilham kayna\u011f\u0131 olmu\u015ftur.<\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/VampireE3.jpg\" alt=\"\" width=\"250\" height=\"334\" \/><figcaption>Bir Vampir Cosplayi<br>https:\/\/commons.wikimedia.org\/wiki\/File:VampireE3.jpg<\/figcaption><\/figure><\/div>\n\n\n<p>G\u00f6r\u00fcn\u00fc\u015f itibariyle di\u015fleri sivrile\u015fen ve zaman zaman burunlar\u0131n\u0131n bir k\u0131sm\u0131n\u0131 kaybeden porfiri hastalar\u0131n\u0131n vampir ve kurt adam hikayelerinin kayna\u011f\u0131 oldu\u011fu d\u00fc\u015f\u00fcn\u00fclmektedir. G\u00fcne\u015f \u0131\u015f\u0131\u011f\u0131ndan ciddi derecede rahats\u0131z olmalar\u0131 bu iddiay\u0131 g\u00fc\u00e7lendiren en g\u00fc\u00e7l\u00fc arg\u00fcmanlardan biridir. Ayr\u0131ca baz\u0131 hastalarda sar\u0131msak yemenin porfiri ataklar\u0131n\u0131 tetikledi\u011fi g\u00f6r\u00fclm\u00fc\u015ft\u00fcr. Bu y\u00f6nden de vampirlerin sar\u0131msaktan zarar g\u00f6rmesi ve uzak durmas\u0131 ile benzerlik kurulabiliyor. <\/p>\n\n\n<div class=\"wp-block-image\">\n<figure class=\"aligncenter size-large is-resized\"><img loading=\"lazy\" src=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Edvard_Munch_-_Vampire_1895_-_Google_Art_Project-1024x1024.jpg\" alt=\"\" class=\"wp-image-13778\" width=\"589\" height=\"589\" srcset=\"https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Edvard_Munch_-_Vampire_1895_-_Google_Art_Project-1024x1024.jpg 1024w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Edvard_Munch_-_Vampire_1895_-_Google_Art_Project-150x150.jpg 150w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Edvard_Munch_-_Vampire_1895_-_Google_Art_Project-1536x1536.jpg 1536w, https:\/\/blog.ulubat.org\/wp-content\/uploads\/2023\/05\/Edvard_Munch_-_Vampire_1895_-_Google_Art_Project-2048x2048.jpg 2048w\" sizes=\"(max-width: 589px) 100vw, 589px\" \/><figcaption>Edvard Munch&#8217;un Vampire Adl\u0131 Eseri<br>https:\/\/commons.wikimedia.org\/wiki\/File:Edvard_Munch_-<em>Vampire<\/em>(1895)_-_Google_Art_Project.jpg<\/figcaption><\/figure><\/div>\n\n\n<h2>T\u00dcRK\u0130YE&#8217;DE HEKZAKLOROBENZEN KULLANIMI VE PORF\u0130R\u0130<\/h2>\n\n\n\n<p>Hekzaklorobenzen &nbsp;form\u00fcl\u00fc C6Cl6 olan bir organoklor\u00fcrd\u00fcr. Eskiden ba\u015fta bu\u011fday olmak \u00fczere tohum \u00fczerindeki mantar enfeksiyonlar\u0131n\u0131n \u00f6nlenmesi amac\u0131yla tar\u0131m ilac\u0131 olarak kullan\u0131lm\u0131\u015ft\u0131r.<\/p>\n\n\n\n<p>\u00dckemizde 1955-1959 y\u0131llar\u0131 aras\u0131nda hekzaklorobenzen maruziyeti nedeniyle G\u00fcneydo\u011fu Anadolu b\u00f6lgesinde yakla\u015f\u0131k 4000 ki\u015finin porfiri geli\u015ftirdi\u011fi saptanm\u0131\u015ft\u0131r.<\/p>\n\n\n\n<p>Bu maruziyet daha sonra g\u00fcne\u015fe maruz kalan alanlarda b\u00fcl geli\u015fimine, hiperpigmentasyona, hipertrikoza ve porfirin\u00fcriye yol a\u00e7m\u0131\u015ft\u0131r. Bu durum kara yara olarak adland\u0131r\u0131lm\u0131\u015ft\u0131r. Anneleri hekzaklorobenzene maruz kalm\u0131\u015f, anne s\u00fct\u00fcyle beslenen 2 ya\u015f\u0131n alt\u0131ndaki \u00e7ocuklar\u0131n \u00e7o\u011fu hayat\u0131n\u0131 kaybetmi\u015ftir. Mortalitenin %10 oldu\u011fu d\u00fc\u015f\u00fcn\u00fclmektedir. <\/p>\n\n\n\n<p>Maruziyetten 25 y\u0131l sonra yap\u0131lan bir <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/7138315\/\">\u00e7al\u0131\u015fmada<\/a> \u00e7ocuk veya gen\u00e7 eri\u015fkinlik d\u00f6neminde hekzaklorobenzen maruziyeti bulunan 63&#8217;\u00fc kad\u0131n ve 98&#8217;i erkek olmak \u00fczere 161 hasta incelenmi\u015ftir. Bir\u00e7ok hastada kal\u0131c\u0131 anormal porfirin metabolizmas\u0131 ve belirgin n\u00f6rolojik, dermatolojik ve ortopedik semptomlar saptanm\u0131\u015ft\u0131r. Kad\u0131nlar\u0131n %60&#8217;\u0131nda, erkeklerin ise %27&#8217;sinde tiromegali saptanm\u0131\u015ft\u0131r.<\/p>\n\n\n\n<p>Ayn\u0131 \u015fekilde maruziyetten itibaren 20-30 y\u0131l aras\u0131 zaman ge\u00e7mi\u015f hastalar \u00fczerinde yap\u0131lan bir <a href=\"https:\/\/pubmed.ncbi.nlm.nih.gov\/2590490\/\">takip \u00e7al\u0131\u015fmas\u0131nda<\/a> 162 erkek ve 90 kad\u0131ndan olu\u015fan toplam 252 hasta incelenmi\u015ftir. G\u00f6zlenen klinik bulgular aras\u0131nda y\u00fcz ve ellerde yara (%83,7), hiperpigmentasyon (%65), hipertrikoz (%44,8), k\u0131s\u0131lm\u0131\u015f y\u00fcz (%40,1), a\u011fr\u0131s\u0131z artrit (%70,2), k\u00fc\u00e7\u00fck eller (%66,6), duyusal g\u00f6lgeleme (%60,6), miyotoni (%37,9), cogwheeling (%41,9), b\u00fcy\u00fcm\u00fc\u015f tiroid (%34,9) ve b\u00fcy\u00fcm\u00fc\u015f karaci\u011fer (%4,8). T\u00fcm hastalar\u0131n 17&#8217;sinde d\u0131\u015fk\u0131 ve idrar tetkiklerinde porfirinlerden en az birinin seviyesi y\u00fcksek bulunmu\u015ftur.<\/p>\n\n\n\n<p><\/p>\n\n\n\n<p><\/p>\n\n\n\n<p><\/p>\n\n\n\n<p><\/p>\n\n\n\n<h2>Kaynak\u00e7a:<\/h2>\n\n\n\n<p>Peters HA, Gocmen A, Cripps DJ, Bryan GT, Dogramaci I. Epidemiology of hexachlorobenzene-induced porphyria in Turkey: clinical and laboratory follow-up after 25 years. Arch Neurol. 1982 Dec;39(12):744-9. doi: 10.1001\/archneur.1982.00510240006002. PMID: 7138315.<\/p>\n\n\n\n<p>Gocmen A, Peters HA, Cripps DJ, Bryan GT, Morris CR. Hexachlorobenzene episode in Turkey. Biomed Environ Sci. 1989 Mar;2(1):36-43. PMID: 2590490.<\/p>\n\n\n\n<p>St\u00f6lzel U, Stauch T, Doss MO. Porphyrien [Porphyrias]. Internist (Berl). 2010 Dec;51(12):1525-33; quiz 1534. German. doi: 10.1007\/s00108-010-2751-x. PMID: 21104216.<\/p>\n\n\n\n<p>Puy H, Gouya L, Deybach JC. Porphyrias. Lancet. 2010 Mar 13;375(9718):924-37. doi: 10.1016\/S0140-6736(09)61925-5. PMID: 20226990.<\/p>\n\n\n\n<p>Anderson KE, Bloomer JR, Bonkovsky HL, Kushner JP, Pierach CA, Pimstone NR, Desnick RJ. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. 2005 Mar 15;142(6):439-50. doi: 10.7326\/0003-4819-142-6-200503150-00010. Erratum in: Ann Intern Med. 2005 Aug 16;143(4):316. PMID: 15767622.<\/p>\n\n\n\n<p>Badminton MN, Elder GH. Management of acute and cutaneous porphyrias. Int J Clin Pract. 2002 May;56(4):272-8. PMID: 12074210.<\/p>\n\n\n\n<p><a href=\"https:\/\/en.wikipedia.org\/wiki\/Porphyria\">https:\/\/en.wikipedia.org\/wiki\/Porphyria<\/a><\/p>\n\n\n\n<p><a href=\"https:\/\/www.porfiriturkiye.org\/porfiri-nedir\">https:\/\/www.porfiriturkiye.org\/porfiri-nedir<\/a><\/p>\n\n\n\n<p><a href=\"https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC1127427\/\">https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC1127427\/<\/a><\/p>\n\n\n\n<p><a href=\"https:\/\/tr.wikipedia.org\/wiki\/Porfiria\">https:\/\/tr.wikipedia.org\/wiki\/Porfiria<\/a><\/p>\n\n\n\n<p><a href=\"https:\/\/archive.org\/details\/biochemistrymole0000swan_f9p0\/page\/n499\/mode\/2up\">https:\/\/archive.org\/details\/biochemistrymole0000swan_f9p0\/page\/n499\/mode\/2up<\/a><\/p>\n\n\n\n<p><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Bu yaz\u0131da vampir ve kurt adam hikayelerine ilham oldu\u011fu d\u00fc\u015f\u00fcn\u00fclen porphyria (porfiri-porfiria) ve bu hikayeler ile ilgili genel bilgiler edinece\u011fiz.<\/p>\n","protected":false},"author":1139,"featured_media":13880,"comment_status":"open","ping_status":"open","sticky":false,"template":"","format":"standard","meta":[],"categories":[1,2173],"tags":[2432,73,115,2429,2430,2433,2428,2431,2427,265,66,34,2426],"acf":[],"views":305,"_links":{"self":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/13483"}],"collection":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/users\/1139"}],"replies":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/comments?post=13483"}],"version-history":[{"count":16,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/13483\/revisions"}],"predecessor-version":[{"id":13881,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/posts\/13483\/revisions\/13881"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media\/13880"}],"wp:attachment":[{"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/media?parent=13483"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/categories?post=13483"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/blog.ulubat.org\/index.php\/wp-json\/wp\/v2\/tags?post=13483"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}