Porphyria chirwere

Rudzi ruvara rweropa, uyewo kukwanisa kwaro kutakura okisijeni, kunokonzerwa nekuvapo kweheme. Nekudaro, chigadzirwa ichi chemakemikari chinoumbwa nekuda kwemakemikari, izvo zvinogadzirwa zvinoda porphyrin. Kana zvinhu izvi zvisina kukwana, porphyria inokura, semurairo, hutano hunova chikonzero.

Genetics uye porphyria

Chinhu chinonyanya kukonzera kushambadzira kwechirwere ndiko kuvapo kwevatakuri ve antigen HLA B7 neHLA A3 muropa. Zvinokonzera kusakwanira kwekugadzirwa kwemakemikari anoumba heme, uyewo nekuputsa kwehutachiona hwezvinonzi lipid.

Mukuwedzera kune mazamu, zvipembenene zvisingakanganwiki hepatitis B, C uye A (muhutomu hwakaoma) nekuderedza kwechiropa parenchyma, kuora kwemarara kunokosha.

Zviratidzo zve porphyria

Iyo yakakosha enzyme mishonga inoumbwa muropa uye pfupa remafupa. Saizvozvowo, zviratidzo zve porphyria zvinowirirana zvichienderana nenhengo yakagadziriswa, pamwe nekusiyana kwezvirwere.

Rudzi rwehepatic rune zvikamu zvakatevera zvinotevera uye zvinoratidzira zviratidzo zvinoratidzika:

1. Hereditary. Chiratidzo chakanyanya kuzivikanwa chinorwadza zvikuru munharaunda yepigastric. Mukuwedzera, pane tachycardia, kushushikana kwepfungwa, unyoro hwepfungwa.
2. Acute intermittent. Dzidzo yacho yakangofanana neyechimiro, asi inokanganisa hutachiona uye hutachiona hwehutano hwehurongwa. Izvi zvinopindirana nekufungidzirwa, kuora kweputika, kuburitsa uye kuora mwoyo kwemitsipa yakashata yemabhononi, mapapu.
3. Chester. Iine zita rayo pamusana pekugara kwenzvimbo (kunyanya vanhu vanorwara veguta reKester muEngland) uye hukama hwemadzinza. Zvakafanana nepepakutanga ye porphyria, asi ine zviratidzo zvakakomba, dzimwe nguva inoputika neutsvo kusakundikana.
4. Coproprotoporphyria. Inobatanidza makiriniki ekuratidzwa kwezvinhu zvitatu zvakataurwa pamusoro apa zvechirwere, unhu hwevagari veSouth Africa. Chirwere chacho chinoperekedzwa nemashura pamusoro pepidermis.
5. Urokopproporfiriya. Inowanzoongororwa seganda porphyria - zviratidzo zvinoratidzirwa sekuputsika kwemavara uye kuumbwa kwemabhuru maduku munzvimbo yezviso uye nechiso. Inowanzokonzera vagari venyika yeRussia uye nyika dzeichimbova CIS.

Kana iyo yakafanana ye porphyrins inoshungurudzika mumupfupa, maronda aya anowanzo:

1. Erythropoietic uroporphyria. Rudzi rune ngozi zvikuru, sezvo harwanzobvumiri kuti urarame kuva munhu mukuru. Iyo inoratidzirwa nekuparara kweganda (nekusava nekuporesa ulcerous erosions), meno, kunzwisiswa kwemazuva ano, uye kufambira mberi - nehutachiona hweropa.
2. Erythropoietic coproporphyria. Chiratidzo chikuru ndechekushushikana kwakasimba kune ultraviolet, iyo inokonzera kubvunda, kuchenesa uye kutapira kwemupidermis. Mushure mekunge nguva yakareba yakasununguka, panewo kuitika kwe vesicles, kuputika kwemhepo, zvipembenene paganda uye zvinopisa.
3. Erythropoietic protoporphyria. Inozivikanwa chaizvo nekiriniki imwechete seyero yekare yehutachiona, inowanikwa nechinangwa chekuongorora ropa (iyo yakawanda yeprotoporphin inowedzera muyerythrocytes).

Kurapa kwechirwere chinonzi porphyria

Kusvika iye zvino, hapana nzira dzakanaka dzokurapa chirwere ichi. Chiratidzo chekufananidzira chinosanganisira kushandiswa kwe chinangwa chakakomba kupedza zviratidzo zvikuru:

• kuderedza ropa;
• splenectomy;
• plasmapheresis ;
• kutora anti-inflammatory and analgesic mishonga;
• kuchengetedzwa kusingagumi kumirairo ye ultraviolet;
• periodic installation of droppers ne solution glucose, kuiswa kwe riboxin, adenosine monophosphate, delagila.