Porphyria - chirwere vanhu ano

porphyria chirwere ndiye chiropa pathologies panguva nemagene vanochiva umo zvachose zvisiri Chizenga usage chinokosha. Zvinofungidzirwa kuti pane sere anotevedzana inonzi matanho enzymatic ari biosynthesis pamusoro hemoglobin. Chero pakaputsika panguva padariro mumwe wavo anogona nechekare kutungamirira dambudziko yakadai porphyria. Kazhinji The chirwere hwapedyo nokukurumidza. Munyaya ino tichakurukura zvikuru akadzama kuti chirwere ichi uye ndedzipi yazvino nzira kurapwa.

Porphyria - chirwere vanhu ano. Chii mashandiro chirwere?

Pakutanga, asiri mapuroteni chikamu Chizenga (zvimwe - heme) ari kutendeuka kuva chepfu venhumbi, akavapindurawo, nguva kudya kure zvachose vose subcutaneous tishu. Saka, ganda zvishoma nezvishoma anowana brownish tint, achiva wakawondereiko kudai mazuva ose pamwe nguva, uye kubva zvakananga chezuva watsemuka. Ndokusaka varwere nechirwere ichi, unogona kuwana dzakawanda maronda uye mavanga. Saka zvishoma nezvishoma kuva porphyria. Zvirwere, tinoona pasina nenguva kurapwa, kazhinji inobudisa munzvimbo itsva vose ganda.

zvikonzero

Sequential zvaipesana heme biosynthesis, iyo yakakurukura zvishoma kumusoro, inotungamirira kwazvo kuunganidza porphyrins vari nhengo, uyewo rinonzi saka Precursors (semuenzaniso, ala uye porphobilinogen). Rakanga yokupedzisira yemuraudzo ane simba hunokachidza zvainoita muviri, somugumisiro zvinokonzera kare dzichiri zvekuchipatara zviratidzo. Chikonzero kutyorwa yakadaro mutation pakati majini anokonzera webasa zvimwe makemikari vanobatanidzwa mune multi-danho kwetunhu heme. Panguva iyoyo, masayendisiti vari kuita uwane kudzidza azvaiitwa uye chirwere ichi sezvo porphyria.

Disease zviratidzo

• Kuvadza nechepakati tsinga.
• Weti ine pinkish Hue.
• Pane zvakawanda maronda uye mavanga muviri.
• Kurwadziwa uye kusagadzikana mune abdominal munharaunda.
• Inosimudzira BP.

Chii chinofanira kubatwa?

Zvinosuruvarisa, panguva, nyanzvi havagoni kupa dzinobudirira nzira pathogenetic kurapa chirwere ichi. Mune zvimwe zviitiko (semuenzaniso, erythropoietic uroporfiriya) kunokwanisa kunobudirira. Intermittent porphyria (chirwere iri rikwanise Danho) zvachose hatibvumiri kushandisa dipyrone uye mapiritsi, sezvo ivo kuti exacerbation zviratidzo. In pamberi marwadzo ari zvirwere zvakadai porphyria, sezvinoita mutemo, anoperetera kunze yakasimba zvinodhaka.

mhedziso

Porphyria chirwere (mufananidzo nhamba 1 inoratidza imwe kuonekwa), zvinosuwisa ari akaonekwa mazuva aya kakawanda. Mukugumisa, zvinofanira kucherechedzwa kuti nhasi chirwere chakadai yasara havaratidzi kuvazivisa. Masayendisiti pasi pose kuramba kuongorora zvinokonzera, chikuru kurapwa zviratidzo, uyewo nzira kurwisa dambudziko hachifadzi kwakadaro. Stay utano!