Isifo se-Porphyria

Umbala obomvu wegazi, kanye nekhono layo lokuthutha i-oksijini, kungenxa yokutholakala kwe-heme. Kanti, le nhlanganisela ye-biochemical ikhiwa ngenxa yezinyomi, okukhiqizwa okudinga i-porphyrin. Uma lezi zinto zingenalutho, i-porphyria iqala, njengomthetho, ubuhlobo buba yisisusa.

I-Genetics ne-porphyria

Isici esiyinhloko esilungiselela ukuqhubeka kwesifo yileso sokuba khona abathwali be-antigen HLA B7 ne-HLA A3 egazini. Bavusa ukukhiqiza okungenele kwama-enzyme abumba i-heme, kanye nokwephulwa kwe-oxidation yamakhemikhali e-lipid.

Ngaphandle kwezakhi zofuzo, i-hepatitis B, C no-A engafinyeleleki (ngesimo esibi) ngokuchichima kwesibindi se-parenchyma, ukulimala komnkantsha wamathambo kubalulekile.

Izimpawu ze-porphyria

Amakhemikhali e-enzyme adingekayo akhiwa esibindi nesomnyo. Ngakho-ke, izimpawu ze-porphyria ziyahlukahluka kuye ngokuthi isakhi esithintekile, kanye nezinhlobo ezihlukahlukene ze-pathology.

Uhlobo lwe-hepatic lunezigaba ezincane ezilandelayo nezibonakaliso zemitholampilo:

1. Hereditary. Isibonakaliso esivela kunazo zonke ubuhlungu obukhulu esifundeni se-epigastric. Ngaphezu kwalokho, kune-tachycardia, izifo zengqondo ezincane, ukucindezeleka komfutho wegazi .
2. I-intermittent enembile. Inkambo ifana nefana nefa, kodwana ithinta isistimu ye-central neyomunye wesistimu. Lokhu kuhambisane nokucabangela, ukugwinya kwesisu, ukudilizwa nokukhubazeka kwemisipha ebushelelezi ye-bronchi, amaphaphu.
3. Chester. Uthole igama lakhe ngenxa yendawo yokuhlala (ikakhulukazi abagulayo bomuzi waseCherster eNgilandi) nobuhlobo bozalo. Ngokufana nefomu langaphambilini le-porphyria, kodwa linempawu ezinzima kakhulu, ngezinye izikhathi ligcwele ukwehluleka kwezinso.
4. I-Coproprotoporphyria. Ihlanganisa ukubonakaliswa kwemitholampilo yezinhlobo ezintathu ezibalulwe ngenhla zesifo, isici sabakhileyo eNingizimu Afrika. Lesi sifo sihambisana nokuqhuma ebusweni be-epidermis.
5. Urokopproporfiriya. Kuvame ukutholakala ukuthi isikhumba se-porphyria - izimpawu zikhonjiswe njengokuphulwa kwezingulube nokubunjwa kwamabhulebhu amancane endaweni yezandla nobuso. Ngokuvamile kuthinta izakhamizi zaseRussia namazwe e-CIS yangaphambili.

Uma ukuhlanganiswa kwe-porphyrins kuphazamiseka emnothweni wamathambo, lezi zinhlobo zezifo zikhula:

1. Erythropoietic uroporphyria. Uhlobo oluyingozi kakhulu lokugula, ngoba akuvami ukuvumela ukuba uphile kuze kube umuntu omdala. Ibonakala ngokulimala esikhunjeni (ngezinkinga ezingezona ukuphulukisa izilonda), amazinyo, ukuzwela emisebeni yelanga, nokuqhubekela phambili - ngokutheleleka kwegazi.
2. I-coproporphyria ye-Erythropoietic. Uphawu oluyinhloko ukuzwela okuqinile ku-ultraviolet, okubangela ukuvuvukala, ukuchithwa nama-hyperemia we-epidermis. Ngemuva kokushisa kwelanga isikhathi eside, kukhona nokutholakala kwama-vesicles, ukuqubuka kwamanzi, izilonda esikhumbeni kanye nezibazi.
3. I-protoporphyria e-Erythropoietic. Eyaziwa kahle ngemtholampilo efanayo njengokwedlule uhlobo lwe-pathology, itholakala ngokuhlolwa kwegazi (ukuxilongwa kweprotoporphin kwanda kuma-erythrocytes).

Ukwelashwa kwesifo se-porphyria

Kuze kube manje, azikho izindlela eziphumelelayo zokwelapha lesi sifo. Ukwelapha okuphawulekayo kuhilela ukusebenzisa uhlelo oluyinkimbinkimbi ukuqeda izimpawu eziyinhloko:

• ukunciphisa ukucindezelwa kwegazi;
• i-splenectomy;
• i-plasmapheresis ;
• ukuthatha imithi elwa nokuvuvukala kanye ne-analgesic;
• ukuvikelwa okuphakade emisebeni ye-ultraviolet;
• ukufakwa ngezikhathi ezithile ze-droppers ngesisombululo se-glucose, ukwethulwa kwe-riboxin, i-adenosine monophosphate, i-delagila.