Beta thalassemia inotora nhaka yeanemia iyo muviri usingakwanisi kubereka hemoglobin kazhinji. Hemoglobin A (iyo yakakura hemoglobin kuvanhu vakuru) inosanganisira alpha alpha globin maketani uye maviri beta globin maketani. Mu beta thalassemia, bone marora haikwanisi kubereka mararamiro akaenzana ebhea globin zvichiita zvishomanana pasina hemoglobin A. Uyewo, pane kuwedzera kweAlbum globin isingaiti bhaa globin inokonzera red cell breakdown inozivikanwa sehemolysis.
Ndiani Ari Pangozi?
Beta thalassemia inotorwa nhaka. Zvinoda vabereki vose kuti vatakure dambudziko rinonzi beta thalassemia trait kana vaduku. Apo vose vabereki vane beta thalassemia mitiro, vane 1 mukana wechina wekuva nemwana ane chirwere chebhea thalassemia. Zvinosuruvarisa, nokuti beta thalassemia chiito chinokonzera kusina zviratidzo, vabereki vanogona kunge vasingazivi dambudziko iri vasati vaita pamuviri.
Ndeapi Mhando dzeBeta Thalassemia?
Beta thalassemia inogona kuiswa nenzira mbiri: ne genetic mutation inopiwa nhaka kana kuwedzerwa ropa.
Genetic mutations
Beta zero thalassemia : Zero mu beta zero mutation inoratidza kuti hapana bhebhebhini inogona kubudiswa nechromosome iyoyo.
Beta pamwe thalassemia : Beta globin inobudiswa nechepfupi. Nhamba yebheta yebinibin inobudiswa inowanzosiyana zvikuru nedzimwe shanduko yakagadzirirwa. Zvimwe zviitiko zvinokonzera zvinenge zvisina beta globin zvichiita kuti zvipatara zvakafanana nebhea zero.
Kudzingiswa kunoda
Beta thalassemia guru: Beta thalassemia huru inotsanangurwa nekudiwa kwehupenyu huzhinji-kuwedzerwa, kakawanda pamwedzi (inonzi hypertransfusion therapy).
Beta thalassemia intermedia : Kazhinji kuenzanisa kunwisa ropa kunogona kudikanwa kuwedzerwa ropa (panguva dzezvirwere, kuberekwa, etc) asi kwete nguva dzose.
Beta thalassemia mitiro / maduku : Izvi zvinoguma nehupenzi hwekushaya ropa rinowanzosarudzwa purogiramu yakakwana yeropa.
Beta Thalassemia Inowanikwa Sei?
Nenzira yakawanda, beta thalassemia huru inowanikwa pachirongwa chitsva . Muzvicheche izvi, muedzo uyu unongoratidzira hemoglobin F (kana fetal), kwete hemoglobin A. Ava vana vanotumirwa kune hematologist kuti iongororwe zvikuru. Zvimwe zvakanyanya zvakakanganiswa neBeta thalassemia intermedia varwere vachaonekwawo nenzira iyi.
Vamwe varwere vanorwara zvakanyanyisa vanozoonekwa pamusana pekuverenga ropa rakazara (CBC). ICBC icharatidza anemia anononoka kuenzanisa nemasero matsvuku eropa. Izvi zvinogona kuvhiringidzika nekushaya simba kwesimbi. Kuongororwa kunosimbiswa nehmoglobin profile (inonziwo electrophoresis). Mu beta thalassemia intermedia uye chiitiko ichi kuongorora kunoratidza kukwirira kwehemoglobin A2 (imwe yechipiri yevanhu vakuru hemoglobin) uye dzimwe nguva F (fetal).
Ndeapi Mishonga yeBeta Thalassemia?
Beta thalassemia trait inoda kusarapwa. Vanhu vane beta thalassemia mitiro vachave nekurarama kwenguva refu anemia.
Kufuridzira: Varwere vane beta thalassemia zvikuru vanoda kuwedzerwa upenyu hwose. Kurapa kwechirwere kunoshandiswa kuderedza kusvibiswa kwemasero matsvuku muropa mumapfupa emapfupa.
Beta thalassemia intermedia varwere vangada kuwedzerwa kuwedzerwa ropa panguva dzezvirwere nekuyaruka (panguva yekukura).
Iron chelation therapy: Varwere vane beta thalassemia zvikuru vanowana yakawandisa simbi kuburikidza neropa rekuisira ropa. Iyi iron yakawanda inogona kukanganisa nhengo, kunyanya mwoyo. Iron chelation inobatsira muviri mukubvisa simbi. Izvi zvinogona kupiwa semushonga wemuromo kana kuvhara pasi peganda. Varwere vane beta thalassemia intermedia vanogona kuwedzera simbi pamusoro pokuti kunyange kusisina kuwedzerwa ropa kunowedzera kuwedzera kuwedzerwa kwesimbi mumutsipa muduku.
Splenectomy : The spleen inogona kuwedzera yakawanda nekuda kwekushandiswa kwema cell cell (hemolysis) pamwe chete neropa rekushanda kwemasero mupleen. Kana kuwedzerwa ropa kunoda kuwedzerwa kana imwe sero rekushaya ropa kunowira pasi nekuda kwekuvhara mupleen, chirwere chinogona kubudiswa chirwere.
Hydroxyurea: Hydroxyurea yakashandiswa kuwedzerwa fetal hemoglobin production. Kubudirira kwave kushanduka.