Vampirism (cudur): Calaamadaha iyo Daaweynta. porfyri

Waa maxay baahsanaanta cudurka sida vampirism? Cudurku waa mid ka mid ah wakiilo ka badan tartanka cad, oo ku nool Koonfur Afrika, ka Faracoodii kuwaas oo ka soo haajiray Holland. cudurada noocan oo kale ah waa wax caadi ah in Finland.

Noocyada, kaas oo dhaxlay cudurka, iyo sifooyinka cudurka waxa lagu sharaxay qoraalkan.

Vampires: xaqiiqada ama sheekooyin?

Ma jiraan vampires nolosha dhabta ah? Taariikhda waxay muujinaysaa in, haa. culimada casriga ah waxay aaminsan yihiin in marka vampires ugaarsadaan soo gaaray dad badan oo, iyo, sida caadiga ah, ma ay ahaayeen dad aan waxba galabsan. Inkasta oo sabab u ah cadaadis weli jirey. dadka, kuwaas oo lagu eedeeyay vampirism, ma qof kale uu cabbo dhiigga, mana ayan Supatra, laakiin waxay lahayd muuqaal argagax.

Qubarada badankooda ayaa aaminsan in la soo ashkateeyey denbiga laga cabsado noocan oo kale ah lahaa cudur dhif ah hidde dhiig, kaas oo la odhan jiray "porfyri" ka porphyros erayga Giriigga ah (magenta).

Waxaa la rumeysan yahay in muujinta cudurka qayb qaatay dhalashada ilmaha by qaraabada. Porfyri baahsan tuulada yar ee Transylvania (ku dhashay oo ka mid ah caan ku Tirada Dracula), oo ku saabsan kun sano ka hor.

Arimaha vampirism ayaa sidoo kale laga helay dhaxaysay qarniyadii boqornimada caan ah. Tusaale ahaan, Endryu Uilsonom cilmi baare in uu buugga "Victoria The" waxa loola jeedaa qaab hidaha ee porfyri, oo carshiga British mid la siidaayey. Sida laga soo xigtay isaga si, tani waa cudurka sabab u ahayd awoowe dementia ee Queen Victoria - King George Saddexaad.

hababka cudurka

Sidaas vampires nolosha dhabta ah kajirin. Marka ay tani cudurada jebiyey saaridda jajab jawharad-nonprotein haemoglobin, taas oo horseedaysa porphyrin qabanaya Biraha sun dhiig xad-dhaaf ah ee jirka, gaar ahaan, birta iyo magnesium. xad-dhaaf ah oo sunta mukhaadaraadka shiidaa jirka oo dhan.

Waxaa jira dhawr nooc oo ah porfyri, waxay, oo dhan laakiin qaab ugu dambeeyay, waxay saamaysaa maqaarka, waa hidde.

Noocyada dhaxal ee noocyada kasta oo ku kala duwan yihiin, laakiin bannaanka u soo autosomal qaab ku xukuma.

The dilaaliinta ugu weyn

Inta badan in doorka dilaaliinta cudurka waxay yihiin:

• u hogaansanaanta cunto adag;
• dollar dareemayaasha,
• barbiturates, tetracycline'nada, piller,
• joogitaanka of xiriir la kiimikooyin sun ah;
• hormoon badala haweenka;
• nooca faafa cudurka,
• isticmaalka cabitaanka khamriga.

Symptomatology uroporfirii erythropoietic

Waa maxay calaamadaha of vampirism? Cudurka ayaa ah mid si cadaalad ah u dhif ah, waxaana la autosomal kala qaado, nooca recessive. waxaa laga arki karaa in mid ka mid ah jiilka leedahay caruur yar iyo waalidka loo arkaa sidayaal heterozygous cudurka. Anigu ma daliilka kiliinikada ee cudurka. cudurka muujin kara in yaraantaadii. Maxay yihiin marxaladaha kala duwan ee vampirism?

Bilowga hore ee dhallaanka lagu arkay kaadi midabka cas oo dhalaalaya, joogitaanka photosensitivity. In habkan, ka reeban erythrocyte uroporphyrinogen Oo Qaliin lagu qayilo iyo waxaa lagu kala qaadaa in uroporfin, oo yeynan on maqaarka.

markii qaar ka mid ah ka dib dhalashada ilmo daboolay goobooyin, kuwaas oo loo beddelaa boogaha ka dib markii. In bukaanlayda qaadato nabaro antibiotics leexdo xagga nabarradii. In deegaanka this waxaa jira maqaarka sclerosing on wajiga iyo qaybaha kale ee jidhka.

Marka marxaladdan ugu dambeeyey ee ilmaha u muuqataa contracture xubnaha iyo lumista aragga, waxaa jira koritaan ma timaha iyo ciddiyaha.

Waa maxay calaamadaha of porfyri? calaamadaha ay yihiin kala duwan:

• beeryarada ee bukaanka waxay kordhisaa in size,
• anemia hemolytic muujiyey iyadoo hemolysis unugyada gudahood;
• heerka sii kordhaya ee bilirubin serum xor ah oo index reticulocyte;
• noqdo eritronormoblastichesky jeermiga sare dhuuxa lafta,
• dari ee unugyada dhiigga cas weyn ayay hoos;
• kor u kacay heerka uroporfina iyo koprporfina.

Cudurku wuxuu inta badan waa dilaa ah ee carruurnimada hore.

Sidee protoporphyria erythropoietic?

Vampirism - cudur dhaxlay in autosomal hab adag. The salaysan cudurka waa xadgudub ku ah Avv protoporphyrin of heme, taas oo ku saabsan erythrokaryocytes qayb. Waxa kale oo suuragal ah in la kordhiyo heerka Avv aminolevulinic acid.

Bukaan-socodka ayaa muujiyay dareen dheeraad ah iftiinka qoraxda, Biyana way maqaarka, cuncun, gubashadu waxay dhulka ku jira goobooyin, taas oo markii dambe-dilaaco.

Sida caadiga ah ma lagu calaamadeeyay nabarro. Heerka uroporphyrin iyo coproporphyrin in erythrocytes iyo kaadida waa caadi, iyo IX protoporphyrin ee unugyada dhiigga cas - kordhay. Tusiyaha plasma dhiig protoporphyrin sidoo kale noqon kartaa sare.

Dhiig in hemolysis asalka waa dhif, ay sabab u tahay jiritaanka laba nooc oo ah unugyada dhiigga cas ee dhuuxa lafta. Mararka qaarkood waxaa jira dhacdo of hemorrhage, kaas oo la xidhiidha dhigaalka hyaline on darbiyada markabka iyo cholesterol xiga. Balaadha ee beeryarada waa dhif arkay.

coproporphyria erythropoietic

Tani waa cudurada aad u dhif ah, in dhaxlay autosomal hab adag. Caafimaad ahaan, waxa ay la mid tahay protoporphyria erythropoietic.

Tusmada coproporphyrin in erythrocytes in 30-80 jeer ka badan sida caadiga ah, waxaa jira Digo of xaddi badan oo coproporphyrin III la kaadida iyo saxarada. U muuqataa photosensitivity imaanshada muujiyay. Foomka ba'an cudurka sababi kara qaadashada barbiturates.

foomka porfyri go'aysa Ba'an

Tani waa mid ka mid ah noocyada cudurka beerka. Culus socodka calaamado dareemayaasha ee vampirism noqon karaan kuwo raga. Mararka qaarkood ay muuqaalka noqdo yar fahmayaan iyo dembidhaafka joogto ah dhacdaa. Cudurku waa dhaxalkii ay reer autosomal hab adag. Its horumarinta ku salaysan yahay jebinta synthase uroporphyrinogen hawl enzyme iyo synthetase kor Delta acid aminoleaulinovoy oo la sumeeyay unugyada dareenka.

Heerarka porphyrins in erythrocytes waa caadi. In kaadida tilmaamay content ah sii kordhaya ee uroporphyrin aniga iyo II, iyo sidoo kale coproporphyrin III. Inta lagu jiro marxaladda ba'an ka dhex helay maamaan porphyrin kaadida - porphobilinogen.

Waxaa sidoo kale xusay kordhaysa synthetase heerka cagaarshowga iyo Delta-aminolevulinic acid kordhay kaadida porphobilinogen.

Waxaad la kulmi karaan dhibaatooyin kala duwan:

• calool-xanuun, kuwaas oo ku yaalla meelo kala duwan, taas oo inta badan u baahan tahay qaliin,
• polyneuritis daran;
• paresthesia;
• xanuunada maskaxda;
• kooma,
• cadaadiska dhiigga ee sareeya,
• Digo kaadida kacay midab.

Vampirism - cudur keeni kara dhimasho. dhimashada bukaanka sida caadiga ah waxaa sabab u ah curyaannimo neefsashada. Qaar badan oo ka dhiman buka ee kooma ah, ama ay sabab u tahay cachexia.

Sii xumaanaysa ee cudurka waxaa badanaa keena uurka, qaadashada barbiturates, sulfonamides, analgin. Qaraabada bukaanka calaamadaha cudurka loo aqoonsado laga yaabaa in ay ka maqan yihiin calaamadaha cad (foomka subclinical go'aysa cudurka).

foomka la iska dhaxlo

Man Dadqalato dhaxli cudurka in autosomal hab adag. cudurka inta badan horumarisa qarsoon oo cad oo kaliya wax looga qabto hawlaha fermentakoproporfirinogenoksidazy iyo kordhinta heerka Avv of acid Delta-aminolevulinic beerka.

Sida laga soo xigtay rugta, foomkan of cudurada cudurka eg sida porfyri go'aysa ba'an. Heerka kaadida socodka ee acid Delta-aminolevulinic ba'an iyo porphobilinogen gaadho heerarka muhiimka ah sida qaab go'aysa. The kaadida iyo saxarada kordhay coproporphyrin heer.

foomamka qaxay halkaas

Waxa kale oo uu leeyahay autosomal dhaxal xukuma. Horumarinta cudurka xanaajiyeen dhaqdhaqaaqa oo yaraada ee Avv protoporphyrinogen enzyme oxidase iyo heerka sii kordhaya ee Delta-aminolevulinic acid.

Ku salaysan jirro ay la mid tahay porfyri oo ka go'aysa. Mararka qaarkood horumariyo shaqada failure kelyaha. Marka la helo barbiturates iyo dipyrone bixi kara xanuun ba'an ee caloosha iyo cillad ka mid ah habka dareemayaasha jirka.

urokoproporfirii

Tani waa nooca cutaneous daahay. Caadiga ah ee dadka dhiba khamriga, maray hepatitis ama lahaa xiriir la baatrool iyo sunta. In bukaanka ugu badan, waxaa jira fashilaad beerka. In kaadida, waxaa jira uroporphyrin ah heerka sare, coproporphyrin content wax badan ma soo hagaagay. Sidoo kale, hawlaha enzyme la ogaado carboxylase uroporfinogen.

Isla mar ahaantaana, tirada ugu badan oo ka mid ah qaraabada bukaanka ogaaday in ay leeyihiin heerar sare oo ku uroporphyrin kaadida iyo saxarada coproporphyrin in. In qoysaska qaar ka mid ah, waxaa jira laba ama saddex side cudurka.

Man Dadqalato xanuunka noocyo kala duwan oo heley maqaarka:

• dareenka kor u kacay ay iftiinka UV,
• dhaawac farsamo ah
• adag ama khafiifinta ee maqaarka,
• muuqaalka kore ee xumbo on dambe ee gacanta iyo wejiga la nabarro ku xiga.

isbadal leh qaabka ay dubka sare ku dhaco dhaawaca hoose ee maqaarkaba ah. Isbeddelka ku epidermis waa sare.

Taasoo keentay calaamad caafimaad oo waa in la kordhiyo size beerka. Inta u xanuunsadaa ay ka shaqeynayaan.

mabaadi'da therapy

Sida loo daaweeyo vampirism? Waxaa hadda aan horumariyo hababka aasaasiga ah ee therapy, taasoo keentay in gargaarka ugu wayn ee cudurka. Sidaa darteed, goolkiisii ugu weyn ee daaweynta waxaa loogu talagalay baabi'inta calaamadaha cudurka. jidhka Ilaalinta ka-gaadhista Ultraviolet rays yarayn kartaa calaamadaha cudurka.

Mid ka mid ah hababka ugu muhiimsan ee ah in la yareeyo astaamaha xun waa in ay jirka ka difaaco qoraxda. Waa in laga saaro isticmaalka daroogada, kuwaas oo, sida dajiye, analgesics, sulfonamides.

In xanuunka dareen fiiqan muujinaysaa isticmaalka walxaha xanuunka maandooriyaha. Tusaale ahaan, waxaa lagu muujiyay isticmaalka chlorpromazine.

Marka cadaadiska dhiigga ee sareeya waxaa loo doortay therapy ku haboon. "Inderal" waxaa caadi ahaan loo isticmaalo.

In si loo yareeyo content ee porphyrins muujinaysaa codsi of diyaarinta ah sida "delagil" sida. daroogada Tani waxay kor u qabanaya of porphyrin "delagilom" iyo "Riboxinum".

Waxay tusineysaa koorso caadiga ah ee vitamins, folic acid iyo nicotinic, riboflavin, retinol, nicotinic acid.

Wixii lagu daaweyn jiray lesions maqaarka waxaa lagu talinayaa in la isticmaalo saliidda, oo ay ku jiraan rezotsin ama corticosteroids, Shostakovskiy bukaa.

Waa in la ogaadaa in daaweyn waxay noqon doontaa falayaal haddii isticmaalka cabitaanka khamriga by bukaanka.

tallaabooyinka ka hortagga

Si looga hortago marka laga reebo helo xanuunsan talinayaa daroogada in la kordhiyo photosensitivity ee u shaqeeyo. Waxaa sidoo kale ahaan uu u isticmaalka hab dibadda sida saliid bergamot, eosin iyo rodmin.

Odoroska

Sida loo daaweeyo vampirism? Iyadoo dhaqanka waqtigii nidaamka baaritaanka iyo si fiican u doortay porfyri daaweynta therapy siinaysaa natiijooyin fiican. At doorasho khalad ah ee dhammaan tallaabooyinka ku haboon cudurada la sii dartay laga yaabaa.

Bukaan-socodka ka cabanayay in xanuun ba'an ee caloosha, kuwaas oo aan lahayn meel gaar ah. Sidoo kale xusay xaaladaha lallabo, matag, iyo xadgudub ka dhan ah shuqullada dheefshiidka mareenka.

Marka cudurka aan sax ahayn iyo daaweynta foomka ba'an ee porfyri in kala badh kiisaska waddooyinka dhimashada.

gunaanad

Ma jiraan wax vampires nolosha dhabta ah? Dabcan ma. Waa kutidhikuteen, in uu ku dhashay mahad cudurada ee, gudniin jirdilyo muuqaalka qofka ee.

Vampirism - cudur oo ku salaysan xadgudub ku ah qof exchange of isagoo saldhig hidde ah. Pathology kordhinaya failure of xubnaha gudaha, iyo ku xad daacadnimada maqaarka.