Vampirism (chirwere): Zviratidzo neMarapirwo. porphyria

Chii kwakaita chirwere sezvo vampirism? The chirwere zvikuru chinoremedza vamiririri nhangemutange chena, vaigara South Africa, kubva kuzvizvarwa avo vakatama kubva Holland. zvokurwara kwakadaro kwakazara muFinland.

Types, iyo nhaka chirwere, uye chirwere unhu zvinotsanangurwa munyaya ino.

Zvipoko: chokwadi kana nhema?

Pane zvipoko muupenyu chaihwo? History inoratidza kuti, hongu. Nyanzvi dzemazuva ano vanodavira kuti kana kuvhima zvipoko akatambura vazhinji, uye, sezvo mutemo, havana vanhu vasina mhosva. Kunyange chikonzero kutambudzwa huchiripo. vanhu avo, vakanga mhosva vampirism, kwete mumwe munhu ari kunwa ropa uye havana werewolves, asi yakanga chinotyisa chitarisiko.

Masayendisiti anodavira kuti kupomerwa nenzira inotyisa mhosva aiva chisingawanzowaniki nemagene chirwere ropa, yainzi "porphyria" kubva pashoko rechiGiriki porphyros (magenta).

Zvinofungidzirwa kuti kuratidzwa chirwere vakabatsira pakaberekwa mwana nehama. Porphyria kwakapararira munzvimbo duku mumusha Transylvania (nzvimbo yechizvarirwo yaiva yakakurumbira Count Dracula), anenge makore chiuru akapfuura.

The nyaya vampirism vakanga kuwanikwa akakurumbira youmambo Dynasties. Somuenzaniso, Endryu Uilsonom mutsvakurudzi mubhuku rake rinonzi "The Victorians" rinoreva majini chimiro porphyria, iyo rinoparadzirwa pakati British choushe. Maererano naye, ichi ndicho chirwere ndiye aikonzera dementia sekuru Queen Victoria - King George Third.

chirwere zvigadziriswe

Saka zvipoko muupenyu chaihwo haako. Kana ichi zvokurwara kuputswa gadzira chiwanikwa-nonprotein chikamu Chizenga, iyo inotungamira kwazvo ropa hunokachidza porphyrin tichisunga nesimbi mumuviri, kunyanya, simbi uye magnesium. An zvakawandisa yamarudzi chepfu anongoda muviri wose.

Kune mhando inoverengeka porphyria, ivo, uye zvose asi ichangobva chimiro, rinokanganisa ganda, muri magene.

Mhando nhaka nomumwe dzemarudzi anosiyana, asi kuuya yakatsviriridza ane autosomal kureva mashandiro.

Huru vaikurudzira

Most kazhinji basa vaikurudzira chirwere vari:

• kuomerera vaitofanira kudya;
• tsinga zadzisa;
• barbiturates, tetracyclines, nemumuromo nhumbu;
• Kuvapo kuonana chepfu;
• hormone Kuchinja vakadzi;
• ari kutapuriranwa hunhu chirwere;
• kunwa doro.

Symptomatology erythropoietic uroporfirii

Ndezvipi zviratidzo vampirism? The chirwere zvakarurama ichinetsa uye inoparadzirwa autosomal, recessive mhando. Anogona kuonekwa muchizvarwa chimwe vana mashoma uye vabereki vaiona heterozygous vatakuri chirwere. Handina zvekuchipatara zvinoratidza chirwere. Chirwere chinogona kuratidza achiri mucheche. Ndezvipi nzendo vampirism?

Pakutanga Danho vachangoberekwa vakachengeta weti rakajeka yakatsvuka, kuvapo photosensitivity. In iyi, asingachaiti uroporphyrinogen erythrocyte kaviri oxidation uye inoparadzirwa mu uroporfin, nokuiswa ganda.

Kwapera nguva pashure pokuberekwa kwomwana akafukidzwa matema, izvo tinoshandurirwa nemaronda. In varwere kugamuchira mishonga maronda dzikava mavanga. Munzvimbo iyi pane chinonzi sclerosing ganda kumeso uye dzimwe nhengo dzomuviri.

Pakupedzisira padariro mwana rinowanika contracture mapfundo nokurasikirwa chiratidzo, hapana kukura bvudzi nezvipikiri.

Ndezvipi zviratidzo porphyria? Zviratidzo ayo akasiyana:

• chirwatata wevarwere kunowedzera ukuru;
• vakaratidzawo hemolytic kupererwa neropa pamwe hemolysis mukati masero;
• vakawedzera kwevari vakasununguka serum bilirubin uye reticulocyte indekisi;
• anova mazhinji utachiona eritronormoblastichesky mwongo;
• lifespan masero eropa matsvuku kuderedzwa zvinoshamisa;
• vakawedzera nhanho uroporfina uye koprporfina.

The chirwere kazhinji zvinouraya mukutanga muduku.

Sei erythropoietic protoporphyria?

Vampirism - chirwere nhaka munzvimbo autosomal kureva nenzira. Hwaro chirwere kutyora protoporphyrin kwetunhu heme, zvinhu chikamu erythrokaryocytes. Zvinogonekawo kuti kuwedzera padanho kwetunhu aminolevulinic acid.

Varwere akaratidza yakawedzerwa aikanganiswa chezuva, izvozvo rukazara ganda, kuvaviwa, kutsva pasi pane matema, vakazochinja ulcerate.

Kazhinji hazvina akacherekedza scarring. Level uroporphyrin uye coproporphyrin mune erythrocytes uye weti romuzvarirwo, uye protoporphyrin IX mumasero matsvuku - zvawanzwa. Chakarurama protoporphyrin plasma zvinogonawo kuva mukuru.

Kupererwa neropa necheseri hemolysis hariwanikwi, nokuda kuvapo nezvemarudzi maviri masero matsvuku eropa mu mumwongo. Dzimwe nguva pane panowanikwa ropa, iro chokuita hyaline deposition pamusoro mudziyo masvingo uye kwavakazoita cleavage. Akawedzera chirwatata haawanzopiwi kuchengetwa.

erythropoietic coproporphyria

Ichi chinhu apo neapo zvokurwara, nhaka munzvimbo autosomal kureva nenzira. Akaora, zviri zvakafanana erythropoietic protoporphyria.

Mukati coproporphyrin mu erythrocytes muna 30-80 nguva kupfuura dzose, pane excretion pamusoro yakawanda coproporphyrin III ne weti uye mutsvina yadzo. Anozviratidza zvakaneta akaratidza photosensitivity. The chenjera chimiro chirwere zvinogona kukonzerwa nokutora barbiturates.

Chenjera inoratidza porphyria fomu

Uyu ndiwo mumwe siyana chiropa chirwere. Heavy runoyerera chetsinga zviratidzo vampirism kunogona kuurayisa. Dzimwe nguva pakuonekwa anova zvishoma raidudzwa uye kuramba kanganwiro kunoitika. Hosha iri nhaka munzvimbo autosomal kureva nenzira. kukura kwayo chakavakirwa pakaputsika enzayemu basa uroporphyrinogen synthase uye synthetase kurera Delta aminoleaulinovoy asidhi izvo Chepfu masero etsinga.

Kwevari porphyrins mu erythrocytes kunowanikwa. Muna weti yakaratidza rwakawedzerwa zviri uroporphyrin I uye II, uyewo coproporphyrin III. Mugore chenjera danho rinowanikwa weti porphyrin aizotangira - porphobilinogen.

Zvinonzi yakataurawo yakawedzerwa hepatic chechetere synthetase uye Delta-aminolevulinic asidhi kwakawedzera porphobilinogen weti.

Unogona kusangana nemamwe matambudziko akasiyana-siyana:

• abdominal marwadzo, izvo riri siyana munzvimbo, izvo kazhinji zvinoda kuvhiyiwa;
• zvakakomba polyneuritis;
• paresthesia;
• chirwere chepfungwa;
• chaanoziva;
• cheBP;
• weti excretion ndokumuka ruvara.

Vampirism - chirwere chinogona kukonzera rufu. kazhinji murwere rufu imhaka kufema dzomuviri. Vazhinji vanorwara uchafira pakoma, kana nokuda cachexia.

Kuwedzera pamusoro chirwere zvinowanzooneka kukonzerwa pamuviri, akatora barbiturates, sulfonamides, analgin. Veukama wevarwere zviratidzo zviri chirwere chinogona kuzivikanwa pasina zviratidzo yakajeka (inoratidza subclinical chimiro chirwere).

nhaka chimiro

Man Vampire nhaka chirwere mune autosomal kureva nenzira. chirwere kazhinji anovandudza Isimba rakadini rinogona kubudiswa uye chete pachena panguva netambo fermentakoproporfirinogenoksidazy basa uye kuwedzera padanho kwetunhu Delta-aminolevulinic asidhi ari chiropa.

Maererano kukiriniki, chimiro ichi chirwere zvokurwara afanane akadai chakasimba inoratidza porphyria. The weti kuyerera mwero rikwanise Delta-aminolevulinic asidhi uye porphobilinogen rinosvika dzinonetsa pamwero zvakadai muna inoratidza chimiro. The weti uye mutsvina yadzo kwakawedzera yakati coproporphyrin.

variegated siyana

Rinewo ive autosomal kureva nhaka. Development chirwere wakatsamwisa havachanyatsogoni basa enzayemu protoporphyrinogen oxidase usage uye vakawedzera kwevari Delta-aminolevulinic acid.

On nhandare urwere hwakafanana inoratidza porphyria. Dzimwe nguva kuva itsvo kukundikana basa. Kana kugamuchira barbiturates uye dipyrone angamuka kurwadziwa mudumbu uye atadze kushanda zvakanaka pakati tsinga.

urokoproporfirii

Ndiyo mhando vachangobva cutaneous. Common vanhu vanoshungurudza doro, vakaidzwa kuzvimba chiropa kana aiva kuonana peturu Inobaya. Varwere vakawanda, pane kukundikana ari chiropa. In weti, pane mukuru zvazvingava uroporphyrin, coproporphyrin kugutsikana haisi zvikuru hwakavandudzika. Uyewo, iyo enzayemu basa iri dzakaonekwa uroporfinogen carboxylase.

Panguva iyoyowo, vazhinji hama varwere vakawana kuti yakanaka pamwero muna weti uroporphyrin uye coproporphyrin mu mutsvina yadzo. Mune dzimwe mhuri, pane vaviri kana vatatu mutakuri chirwere.

Man Vampire anotambura kubva siyana ganda pathologies:

• yakawedzera aikanganiswa UV chiedza,
• vaikuvadza zvokuimba
• thickening kana rive dete ganda,
• chimiro matema seri ruoko uye chiso pamwe nekutambira scarring.

Structural kuchinja paganda kuitika mune chikuru lesion pakati dermis. Zvakachinja Epiderme e hakukoshi.

Mukusarudza kunotoda chiratidzo chiri kuwedzera chiropa kukura. Kazhinji dambudziko functionality kwayo.

kurapa misimboti

Sei kurapa vampirism? Hapana pari haana akakurukura dzinokosha nzira yokurapa, zvichizoita nemhedziso ruyamuro zvirwere. Naizvozvo, mukuru chinangwa kurapwa nechinangwa kubvisa zvirwere zviratidzo. Kudzivirira muviri pakuona ultraviolet mwaranzi kunogona kuderedza zviratidzo chirwere.

Imwe nzira huru kuderedza kunaka zviratidzo ndiko kudzivirira muviri kubva zuva chokupfeka. Zvinofanira siya kushandisa zvinodhaka izvi, zvakadai mapiritsi, analgesics, sulfonamides.

In marwadzo manzwiro chinoratidza kushandiswa marwadzo unonyaradza marwadzo zvinhu. Somuenzaniso, zvinonzi anoratidzwa kushandiswa chlorpromazine.

Kana BP anosarudzwa zvakakodzera kurapa. "Inderal" inowanzoshandiswa.

Kuti uderedze zviri porphyrins chinoratidza kushanda kunoita gadziriro yakadaro "delagil". chinodhaka ichi anokurudzira nokusungwa porphyrin "delagilom" uye "Riboxinum".

Anoratidza randinenge nzira mavhitamini, folic uye nicotinic acid, riboflavin, retinol, nicotinic acid.

Nokuti pakurapa maronda ganda inokurudzirwa kushandisa mafuta okuzora, kusanganisira rezotsin kana corticosteroids, ebharisamu Shostakovskiy.

Zvinofanira kucherechedzwa kuti kurapa ichava maturo kana kudyiwa doro nokuda murwere.

matanho

Kuitira kudzivirira kurwara zano Kunze kugamuchira zvinodhaka zvinowedzera photosensitivity yacho muviri. Zvinonzi zvakare contraindicated kushandiswa dzakadai chokunze nzira sezvo bergamot mafuta, eosin uye rodmin.

mataridzikiro

Sei kurapa vampirism? With nenguva maitiro chirwere maitirwo ezvinhu uye zvakanaka akasarudzwa kurapa kurapwa porphyria anopa nemigumisiro yakanaka. Panguva isiri kusarudza zviyero zvose zvakakodzera kuti zvokurwara anogona yakawedzera.

Varwere chema kurwadziwa mudumbu, izvo havana imwe nzvimbo. Yakataurawo nyaya kuda kurutsa, kurutsa, uye kutyora zvomudumbu turakiti mabasa.

Kana zvisiri chirwere uye pakurapa rikwanise chimiro porphyria iri hafu nyaya kunotungamirira kurufu.

mhedziso

Pane muupenyu zvipoko? Chokwadika kwete. Manyepo kuti akazvarwa nemhaka zvokurwara, mutilating omunhu chitarisiko.

Vampirism - chirwere iyo inobva kutyora Exchange hunhu vane majini hwaro. Zvokurwara hunoita kuti kukundikana nhengo dzomukati, uye anotyora kuvimbika ganda.