Vampirism (isifo): Izimpawu kanye Ukwelashwa. porphyria

Uyini ukwanda kwalesi sifo njengoba vampirism? Lesi sifo okungukuthi ngaphezulu owawuvamile abameleli esimhlophe uhlanga, ephilayo eNingizimu Afrika, kusukela inzalo abathuthela waseHolland. zokugula onjalo uvamile Finland.

Izinhlobo, okuyinto njengefa lesi sifo, futhi isifo izici ezichazwe kulesi sihloko.

Vampire: ngokoqobo noma ziyindaba eqanjiwe?

Zikhona eyayisekhanda lami empilweni yangempela? Umlando ubonisa ukuthi, yebo. Izazi zanamuhla bakholelwa ukuthi lapho eba kule nhlobo abazingela ngabhekana abantu, futhi, njengoba umthetho, babengebona abantu abangenacala. Nakuba isizathu ushushiso isekhona. Labo abantu, ubani babesolwa ngokuchema vampirism, hhayi omunye umuntu ephuza igazi nokuthi kwakungafanele werewolves, kodwa waba ukubukeka elesabekayo.

Ososayensi bakholelwa ukuthi abasolwa ubugebengu esibi kangaka kwadingeka onobulwelwe yegazi, okwakubizwa ngokuthi i "porphyria" kusukela lesiGreki elithi porphyros (imajenta).

Kukholakala ukuthi kube yikwembulwa isifo nomthelela ukuzalwa kwengane yizihlobo. Porphyria isakazeke edolobhaneni elincane eTransylvania (kwazalelwa khona abadumile Ukubala Dracula), eminyakeni engaba yinkulungwane edlule.

I amacala vampirism nazo ezitholakala amakhosi adumile wasebukhosini. Ngokwesibonelo, Endryu Uilsonom umcwaningi encwadini yakhe "The Victorians" libhekisela ifomu kwezakhi porphyria, okuyinto elasakazwa phakathi kwesihlalo sobukhosi British. Ngokusho kwakhe, lena isifo ombangela komqondo Umkhulu zeNdlovukazi uVictoria - King George Third.

izindlela isifo

Ngakho eyayisekhanda lami empilweni yangempela ayikho. Uma lokhu zokugula ngobugebengu womshini ingxenyenamba gem-nonprotein ka-hemoglobin, okuholela igazi ngokweqile anobuthi porphyrin ngibopha izinsimbi emzimbeni, ikakhulukazi, yensimbi kanye magnesium. Ngokweqile lomcebo ubuthi cishe wonke umzimba.

Kunezinhlobo eziningana izinhlobo porphyria, futhi bonke ngothi lwabo, ngaphandle ngasekupheleni ifomu, zithinta isikhumba, ingabe zofuzo.

Izinhlobo ifa zohlobo ngalunye oluphilayo zakwazi ziyahlukahluka, kodwa ziqokonyiswe i autosomal evelele indlela.

Abangobhongoza main

Ngokuvamile e indima abangobhongoza lesi sifo yilezi:

• ukunamathela ukudla okuqinile;
• sindisa ezinzwa;
• barbiturates, tetracyclines inzalo ngomlomo;
• khona ukuxhumana anobuthi;
• hormone eshintsha abesifazane;
• ubunjalo ezithathelwanayo lesi sifo;
• ukusetshenziswa uphuzo oludakayo.

Symptomatology erythropoietic uroporfirii

Yiziphi izimpawu vampirism? Lesi sifo oluphakeme ezingavamile futhi idluliselwa autosomal, uhlobo esikhubazayo. Kungabonwa olungcono phakathi nesizukulwane esisodwa unezingane ambalwa nabazali bebheka abathwali heterozygous lesi sifo. Anginayo ukubonakaliswa emitholampilo ye isifo. Ukugula engaveza isakhasa. Yiziphi izigaba vampirism?

Ngesikhathi sigaba sokuqala izinsana waphawula umchamo umbala obomvu, khona photosensitivity. Uma le nqubo ebugqilini erythrocyte uroporphyrinogen oxidized futhi idluliselwe uroporfin, ebeka esikhumbeni.

Ngemva kwesikhathi esithile ngemva kokuzalwa kwengane embozwe Bubbles, okuyinto siguqulelwa izilonda. Ngo seziguli ezithola imithi elwa namagciwane Izilonda jikisa izibazi. Kule ndawo kukhona sclerosing isikhumba ebusweni nezinye izingxenye zomzimba.

Ngesikhathi zokugcina isigaba ingane kuvela contracture emalunga ukulahlekelwa umbono, akukho ukukhula izinwele nezinzipho.

Yiziphi izimpawu porphyria? Izimpawu zayo zihlukene:

• ubende iziguli inyuka ngobukhulu;
• kubonakala anaemia hemolytic nge hemolysis ngaphakathi kwamaseli womuntu;
• okhushuliwe amazinga khulula serum bilirubin futhi reticulocyte Inkomba;
• kuba eziphambili eritronormoblastichesky ihlumela umnkantsha;
• Isikhathi se-amangqamuzana abomvu egazi encishisiwe kakhulu;
• anda amazinga uroporfina futhi koprporfina.

Lesi sifo ngokuvamile ziyabulala ekuqaleni kuka ebuntwaneni.

Kanjani protoporphyria erythropoietic?

Vampirism - isifo ngofuzo ku-autosomal evelele ngendlela. Ngesisekelo isifo kungukuphula zamagama protoporphyrin ka-heme, okuphathelene ingxenye erythrokaryocytes. Kungenzeka futhi ukuba ukwandisa ezingeni synthesis aminolevulinic acid.

Iziguli eveza uzwela anda elangeni, ke siyakhukhumala isikhumba, ukulunywa, ukusha phansi kukhona Bubbles, okuyinto kamuva ulcerate.

Ngokuvamile ayifani nezibazi. Level uroporphyrin futhi coproporphyrin e erythrocyte futhi umchamo kuyinto evamile, futhi protoporphyrin IX ngo amangqamuzana abomvu egazi - anda. Inkomba protoporphyrin plasma yegazi ingaba futhi iphakeme.

Isifo sokulahlekelwa yigazi ku hemolysis isizinda iyindlala, ngenxa yokubakhona ezimbili zama-amangqamuzana abomvu egazi umnkantsha. Ngezinye izikhathi kukhona okwenziwe ukopha, okuyinto lihlotshaniswa hyaline sebethule ku isitsha izindonga cleavage yabo okwalandela. KweNdikimba ubende kuyaqabukela uyalandelwa.

coproporphyria erythropoietic

Lena zokugula ezingavamile kakhulu, ngofuzo ku-autosomal ngendlela evelele. Emtholampilo, ithi ayifane protoporphyria erythropoietic.

Okuqukethwe coproporphyrin e erythrocyte e 30-80 izikhathi ephakeme kunaleyo evamile, kukhona excretion esiningi coproporphyrin III nge umchamo futhi indle. Ivela photosensitivity wavuma wazwakalisa. Ifomu acute lesi sifo kungadalwa ngokuthatha barbiturates.

Acute ngakanye porphyria ifomu

Lona omunye izinhlobo isifo sesibindi. Heavy notshwala zezinzwa izimpawu vampirism kungaba yingozi. Ngezinye izikhathi ukubukeka kwabo kuba ngaphansi evelele kanye ukuthethelelwa okuqhubekayo kwenzeka. Lesi sifo sitholwa njengefa ku-autosomal ngendlela evelele. Ukuthuthukiswa Its kusekelwe ukuhlukumeza womsebenzi enzyme uroporphyrinogen synthetase futhi sithuthukisa synthase delta aminoleaulinovoy acid okuyinto ushevu amangqamuzana ezinzwa.

Amazinga porphyrins e erythrocyte kuyinto evamile. Esikhathini umchamo kubonakala i kakhudlwana okuqukethwe uroporphyrin I neyesi-II, kanye coproporphyrin III. Esigabeni acute ezitholakala eyandulela umchamo porphyrin - porphobilinogen.

Kuyaqapheleka futhi i anda ezingeni hepatic synthetase futhi delta-aminolevulinic acid anda porphobilinogen umchamo.

Ungezwa ahlukahlukene yezinkinga:

• Ubuhlungu obukhulu besisu, okuyinto zitholakala ezindaweni ezahlukene, okuyinto ngokuvamile kudingeka uhlinzwe;
• polyneuritis ezinzima;
• paresthesia;
• yezifo zengqondo;
• ikhoma;
• umfutho wegazi ophakeme;
• umchamo excretion rose umbala.

Vampirism - isifo engaholela ekufeni. ukufa nesineke ngokuvamile ngenxa ukukhubazeka zokuphefumula. Abaningi abagulayo die equlekile, noma ngenxa cachexia.

Eziya ziba zimbi lesi sifo okungukuthi ngokuvamile esibangelwa sokukhulelwa, ukungenisa barbiturates, sulfonamides, analgin. Nezihlobo zeziguli kungenziwa izimpawu zesifo i-ezikhonjwe ngokungabi bikho izimpawu elikhanyayo (ifomu subclinical yesifo ngakanye).

lezinceku ifomu

Man vampire ifa isifo i autosomal ngendlela evelele. isifo ngokuvamile eba esicashile nokuzivelelayo kuphela wokuqalisa umsebenzi fermentakoproporfirinogenoksidazy nokukhuphula ezingeni synthesis delta-aminolevulinic acid esibindini.

Ngokwe-umtholampilo, lolu hlobo zokugula isifo sifana ezifana acute porphyria ngakanye. I umchamo ukugeleza rate e-acute acid delta-aminolevulinic futhi porphobilinogen ifika emazingeni ezibucayi ezifana ngefomu ngakanye. I umchamo kanye endleni okhushuliwe ezingeni coproporphyrin.

amafomu variegated

Iphinde ine autosomal evelele ifa. Ukuthuthukiswa lesi sifo sokucasuka umsebenzi kahle ka-enzayimu protoporphyrinogen oxidase zamagama kanye namazinga anda of delta-aminolevulinic acid.

On the ngezizathu lokugula kufana porphyria ngakanye. Ngezinye izikhathi ukuthuthukisa izinso umsebenzi ukwehluleka. Lapho ethola barbiturates futhi dipyrone kungase kudingeke ubuhlungu obuhlabayo esiswini futhi ukungasebenzi isimiso sezinzwa.

urokoproporfirii

Lona uhlobo cutaneous ngasekupheleni. Common kubantu ababusebenzisa kabi utshwala, kwavele sokusha kwesibindi noma ukuxhumana uphethiloli kanye ubuthi. In iziguli eziningi, kukhona ukwehluleka esibindini. Ngo umchamo, kunezinga eliphakeme izinga uroporphyrin, coproporphyrin okuqukethwe okuningi ngcono. Futhi, enzyme umsebenzi itholwa uroporfinogen carboxylase.

Ngesikhathi esifanayo, iningi le nezihlobo zeziguli etholakala inakho isigqugquzeli amazinga uroporphyrin umchamo kanye coproporphyrin endleni. Kweminye imindeni, kukhona khona ababili noma abathathu othwala isifo.

Man vampire uhlushwa ezihlukahlukene pathologies isikhumba:

• ukuzwela anda UV ukukhanya,
• ukulimala a mechanical
• ukuqina noma thinning kwesikhumba,
• ukubukeka Bubbles ngemuva esandleni Ubuso okwalandela nezibazi.

izinguquko ezihlelekile esikhumbeni zenzeka lesion eyinhloko dermis. Ushintsho E-epidermis kuyinto yesibili.

Oholela uphawu emitholampilo kuwukwanda ngobukhulu isibindi. Ngokuvamile ehlupheka ukusebenza yayo.

izimiso yokwelapha

Indlela ukwelapha vampirism? Kukhona okwamanje athuthukile eyisisekelo izindlela okwelashwa, kwaphumela i ngokuphelele esiqoshiwe isifo. Ngakho-ke, umgomo oyinhloko ukwelashwa okuhloswe ngalo ukuqeda izimpawu isifo. Ukuvikela umzimba nokusondelana imisebe ultraviolet kunganciphisa izimpawu zesifo.

Enye yezindlela esemqoka ukunciphisa izimpawu ezingezinhle ukuvikela umzimba kusuka elangeni. Kufanele ngakususi ukusetshenziswa kwalezi izidakamizwa, ezifana zinhlungu, analgesics, sulfonamides.

Ngo abukhali ubuhlungu ukuzwa imibukiso ukusetshenziswa kwezidakamizwa analgesic izinto. Ngokwesibonelo, sikhonjiswa ukusetshenziswa chlorpromazine.

Lapho-high blood pressure ikhethwa yokwelapha ezifanele. "Inderal" ngokuvamile lisetshenziselwa.

Ukuze kuncishiswe okuqukethwe porphyrins ubonisa isicelo of a ukulungiselela ezifana "delagil". Lesi sidakamizwa usuke ethuthukisa esibophezelayo ka porphyrin "delagilom" futhi "Riboxinum".

Ibonisa inkambo ejwayelekile amavithamini, i-folic futhi nicotinic acid, riboflavin, retinol, nicotinic acid.

Ukuze ekwelapheni izilonda zesikhumba Kunconywa ukuba asebenzise yokugcoba, kuhlanganise rezotsin noma corticosteroids, ebhalsamu Shostakovskiy.

Kufanele kuqashelwe ukuthi ukwelashwa kuyoba yize uma ukusetshenziswa utshwala yisiguli.

izinyathelo

Ukuze uvimbele isiguli ethola ukukhishwa Kunconywa imithi ukuthi ukwandisa photosensitivity zomzimba. Liphinde contraindicated ukusetshenziswa okunjalo izindlela zangaphandle njengoba uwoyela bergamot, eosin futhi rodmin.

ukuba nombono ongafani ngeke

Indlela ukwelapha vampirism? Nge ukuziphatha esifike izindlela zokuxilonga abakhethiwe kahle ukwelashwa ukwelashwa porphyria linikeza imiphumela emihle. Ngesikhathi okungalungile kukhethwa zonke ezifanele izinyathelo ukuze zokugula kungenzeka zaba zimbi.

Iziguli bakhalaza acute ubuhlungu esiswini, lapho ungenayo i-ethize indawo. Futhi waphawula izimo isicanucanu, ukuhlanza, futhi kwephula kwe wokugaya ukudla pheshana imisebenzi.

Lapho ayilungile ukuxilongwa kanye nokwelashwa kwe-acute uhlobo porphyria e kwesigamu amacala kuholela ekufeni.

isiphetho

Ingabe ikhona eyayisekhanda lami zangempela? Lutho neze. Kuyinto inganekwane ukuthi wazalwa sibonga zokugula, mutilating ukubukeka komuntu.

Vampirism - isifo ogxile ukuhlukumeza yohlamvu exchange kokuba isisekelo zofuzo. Pathology kunomthelela ukwehluleka izitho zangaphakathi kwephula ubuqotho isikhumba.