Urbach-Vite chirwere: zviratidzo, kurapwa, kufungidzira

Urbach-Vite chirwere ndechimwe chinokanganisa maitiro ezve genetic, chinoparidzirwa ne-autosomal recessive type. Chikonzero chechirwere ndechekuchinjwa kwekromosome yekutanga muEGM geni. Mumabhuku ekurapa mune rimwe zita rechirwere - proteinosis lipoid.

Nhoroondo yekuwanikwa

Chiitiko chekutanga chechirwere, zviratidzo zvayo zvakaenderana neUrbach-Vite, yakanyoreswa muna 1908. Akatsanangurwa neSwitzerland purofesa-otolaryngologist Friedrich Siebenmann. Uye muna 1925 mutapi wenhau dzeSwitzerland uye dermatologist Johann Mischer akarondedzera zvitatu zvakadaro.

Zvisinei, zvakasununguka ichi chirwere chisingawanzorese rezve genetic chakanyorerwa muna 1929 chete nedermatologist Urbach uye otolaginologist Vite. Yaiva Erich Urbach uyo akadana chirwere chepiddiproteinosis, sezvo aifunga kuti yakabatanidzwa nekusawirirana kwepachidimbu uye mapuroteni anoiswa mumatumbu emuviri.

Kubva panguva yekuwanikwa, kubva pamatatu mazana matatu kusvika mazana mana ehutachiona ichi chakanyorwa kubva kune zvakasiyana-siyana pasi pose.

Zviratidzo

Pakati pezviratidzo zvakajairika zvechirwere ndechokukomberedza maziso epapuru (ivo varipowo pa lyub nemutsipa), inzwi rinowedzera, kune kuderera kwekugadziriswa kweganda, kuwedzerwa kwemucosa uye kuwedzerwa kwehutano hwehuputi munzvimbo yemazuva. Chiratidzo chekupedzisira ndicho chakanyanya kuipa, sezvo chinogona kutungamirira kune kuora kwekufa kwepanguva.

Chirwere ichi chinotanga kuratidza muhuduku (makore manomwe kusvika kune manomwe). Ichocho ipapo apo pane mashesi paganda uye kutinhira kwezwi.

Zvakakosha kuziva kuti zviratidzo izvi zvinoshamisa uye zvinogona kunge zvakasiyana chaizvo nevarwere vakasiyana. Sezvo chirwere cheUrbach-Wite chinoparidzirwa kuzviita zvekuzvidzivirira, hazvizviratidzi pachedu kana pane chimiro chinowanzogara chiri mujenotype. Kazhinji vanhu havatombofungi kuti ivo vanotakura mutendi wegandanyama. Mune zvimwe zvinotakura, chirwere ichi chisingawanzo chine zviratidzo zvachose. Uye chete kana vabereki vose vane geni iro rinokonzera lipoid proteinosis, chirwere chinogona kuzviratidza mumwana.

Diagnostics

Urbach-Vite's disease inonetsa kuongorora, sezvo zviratidzo zvayo hazvisi zvepasi rose. Kana paine kuvhara (papules) pamakumbo emawere, kuputika kwepachivara paganda remutsipa nehuma, kuongorora kunosanganisira kushanya kune dermatologist.

Kana munhu akambofungidzirwa kuti Urbach-Vita chirwere chinorayirwa, MRI isina kupikisa inoshandiswa kuona kuunganidza kalmicum munyama. Zvisinei, ichi chiratidzo hachirevi kuti chinonzi proteinase. Calcification inosanganisirawo herpes simplex uye encephalitis.

Chimwe chinhu chinokosha mukuongororwa kwacho kunowedzerwa nekutsvakurudza kwemashoko. Izvozvi zvinoita kuti zviboneke kutsvaga kunyatsoziva kana kune kuchinja mutemo we ECM1, uye kana zvakadaro, kuchinja uku kwakakonzera kuUrbach-Vita chirwere.

Kurapa

Pasinei nokuti ipoid proteinosis yakawanikwa kwenguva yakareba, chirwere ichi chisingawanzoitiki, kufanana nehutachiona hwemagetsi, hachikwanisi kurapwa. Zvisinei, zviitiko zvenguva pfupi mumunda we biochemistry zvinoita kuti zvibvirire kuderedza zviratidzo zvakawanda zvechirwere.

Saka, iyo miitiro yakasimba yakaonekwa ne "Heparin" uye "Dimethylsulfoxide", zvisinei kwete mumatambudziko ose. Kurairwa kwemuviri kwemaduku maduku e "Dimethyl Sulfoxide" (dimexide), pamusana pezvirwere zvinopisa simba zvechirwere, zvinobvumira kuderedza dermatotropic zviratidzo zvechirwere.

Kushandiswa kwe "D-penicilamine" kwakabudirirawo, asi yakanga isiri kushandiswa zvikuru. Mhosva dzechirwere chevarwere vane "Tygason", iyo inowanzotarisirwa ye psoriasis, yakanyoreswa zvakare.

Sezvo kuiswa kwe calcium muhutachi tissue kunogona kutungamirira kuchizvarwa chekuwedzerwa kwemagetsi uye kuurayiwa kweputi, varwere vanorayirwa anticonvulsants ("Methindione", "Ethosuximide").

Forecast

Urbach-Vite chirwere ine chirwere chakakodzera, sekutonga, haisi ngozi. Vanhu vanotambura nechirwere ichi vanogona kurarama upenyu hurefu uye hunogutsa. Zvisinei, usakanganwa kugara uchishanyira chiremba. Pamusana pekuwedzera kwemucosa, mune dzimwe nguva, kuparara kwemhepo kunowedzera. Varwere vakadaro vanoda chirwere chetracheotomy kana carbon dioxide laser kuti vadzorere gwara rekufamba.

Muzviitiko apo calcium deposits inotungamirira mukurwiswa kweputika, zvakakosha kubvunza munhu anouraya chirwere chepfungwa uyo achasarudza kurapa kwakakodzera, zvichienderana nekuoma kwezviratidzo zvechirwere.