Alpha thalassemia inotora nhaka inemia iyo muviri usingakwanisi kubereka huwandu hwakawanda hwehumoglobin. Hemoglobin A (iyo yakakura hemoglobin kuvanhu vakuru) inosanganisira alpha alpha globin maketani uye maviri beta globin maketani. Mune alpha thalassemia, pane chidimbu chakaderera che alpha alphabes.
Ndeapi Marudzi eAlfa Thalassemia?
- Alpha thalassemia minima (silent carrier) inoitika apo rimwe alpha alphabin gene rakarasika.
- Alpha thalassemia maduku (kana marati) anoitika apo maviri alpha globin mazamu evanhu akarasika. Kune miviri miviri. Apo ma alpha alpha globin mazita emararamiro akarashika ari pane imwecheteyo chromosome 16 inonzi cis , asi apo imwe alpha globin geni haikwani pane kopi imwe yekromosome 16 iyi inonzi kutengesa .
- Hemoglobin H chirwere (kana alpha thalassemia intermedia) inowanikwa apo matatu alpha globin mazamu asina kushanda. Muchiitiko ichi, kune zvakawanda zvakakwana zve beta globins. Kana izvi zvikabatana pamwe, inonzi Hemoglobin H.
- Hydrops fetalis inowanikwa apo ose mana alpha alphabin gome akarasika. Nhoroondo, izvi hazvina kufanana nehupenyu. Kana njodzi ichizivikanwa pachine nguva, intrauterine transfusions (kuwedzerwa kumucheche mushure mechiri mudumbu) kunogona kubvumira kubudirira kutumira. Idzi vana vanoda chirwere chisingaperi chine kuwedzerwa mushure mokuberekwa uye kazhinji kacho mapfupa ekutemwa.
- Hemoglobin H Inowanikwa Chinyorwa ishanduko yeAlma thalassemia apo ma alpha alpha globin mazamu akarasika pamwe nea alpha globin gene inoshandurwa.
Ndiani Ari pangozi yeAlfa Thalassemia?
Alpha thalassemia inowanzobatanidzwa neAsia, Africa, uye neMediterranean. MuUnited States, vanenge 30 muzana yeAfrica -America vane alpha thalassemia minima kana vaduku.
Alpha thalassemia inotora nhaka uye inoda vabereki vose kuti vatakure.
Munhu asina alpha thalassemia anofanira kuva neine alpha globin gome. Ngozi yekuva nemwana ane chirwere chema alpha thalassemia inobva pauri hwevabereki. Iko kufanana kweAlma thalassemia maduku kunowanzoonekwa kune vanhu veAfrica. Ikoko inowanzova yakajairika kuvanhu vanobva kuAsia kana kuMediterranean.
Kana vose vabereki vane trans alpha thalassemia vaduku (a- / a-), vose vana vavo vane vane trans alpha thalassemia vaduku. Kana mubereki mumwe ane cis alpha thalassemia maduku (aa / -) uye mumwe mubereki ane trans alpha thalassemia duku (a- / a-), vane 1 mu 2 mukana wokuva nemwana ane chirwere cheHemoglobin H. Saizvozvowo, Kana mumwe mubereki ane cis alpha thalassemia maduku (aa / -) uye mumwe mubereki ane thalassemia minima (aa / a-), vane 1 mu4 mukana wokuva nemwana ane chirwere cheHemoglobin H. Hydrops fetalis inowanikwa apo vose vabereki vane cis alpha thalassemia maduku.
Ko Alpha Inonzi Thalassemia Inowanikwa Sei?
Alpha thalassemia minima inokonzera hapana laboratori kuCBC. Ichi ndicho chikonzero chinonzi mutakuri wekunze. Izvi zvinowanzofungidzirwa mushure mokunge mwana aberekwa neHemoglobin H chirwere. Izvi zvinongogadziriswa chete nekuongorora mazita.
Dzimwe nguva alpha thalassemia maduku inoratidzwa pachirwere chitsva , asi kwete mune zvose.
Muedzo iwoyo wakanaka kune maHemoglobin Bart's kana kuti zvitsva. Vanhu vazhinji vane alpha thalassemia vaduku handina ruzivo. Izvi zvinowanzosvika pakujekesa panguva yekugara yakakwana yeropa (CBC). ICBC icharatidza anemia anononoka kuenzanisa nemasero matsvuku eropa. Izvi zvinogona kuvhiringidzika nekushaya simba kwesimbi . Nenzira yakawanda, kana kushaya simba kwesimbi kunoratidzwa kunze uye beta thalassemia mitiro inoratidzika kunze, murwere ane alpha thalassemia trait. Kana zvichidikanwa, izvi zvinogona kusimbiswa nemaitiro ekuongorora.
Hemoglobin H inogona kuonekwa pane chinyorwa chachangoberekwa zvakare. Ivana ava vanotumirwa kunonzi hematologist kuti iongororwe zvikuru.
Vamwe varwere vanozivikanwa gare gare muupenyu munguva yebasa-up anemia.
Hydrops fetalis haisi kunyatsozivikanwa, asi pane zvinonyanya kukosha pane neonatal ultrasound. Kurasikirwa kweine mana alpha globin majini kunowanikwa panguva yebasa-up nokuda kwechikonzero che hydrops.
Ndeapi Mishonga yeAlfa Thalassemia?
Hapana kurapwa kunodiwa kune alpha thalassemia minima kana kuti maduku. Vanhu vane alpha thalassemia vaduku vachararama kwenguva refu anemia.
Kufuridzira: Varwere vane Hemoglobin H vanowanzova nechirwere chinokonzera chirwere chinorambidzwa zvakanaka. Kufuridzirwa kunodiwa pane dzimwe nguva panguva dzezvirwere nefivha sezvo kuwanda kwemasero matsvuku eropa kunowedzera. Kudzingwa kunogona kudiwa nguva dzose muhukuru. Varwere vane Hemoglobin H Constant Spring vanogona kuva neanemia yakawanda uye vanoda kuwedzerwa kuwedzerwa munguva yavo yeupenyu.
Iron chelation therapy: Varwere vane chirwere cheHemoglobin H vanogona kukura simbi kuwandisa kunyange pasina kuwedzerwa kuwedzerwa ropa kunowedzera kuwedzerwa kwesimbi mumutsipa muduku. Vanogona kurapwa nemishonga inonzi chelators kubatsira kubvisa muviri wesimbi yakawanda.