Alfa thalassemia ndi nthenda yotaya magazi yomwe thupi lawo silingathe kupanga hemoglobin yeniyeni. Hemoglobin A (hemoglobin yaikulu mwa anthu akuluakulu) ali ndi maunyolo awiri a alpha globin ndi mabini awiri a beta. Mu alpha thalassemia, pali kuchepa kwa alpha globes.
Mitundu ya Alpha Thalassemia ndi Yanji?
- Alfa thalassemia minima (silent carrier) amachitika pamene gulu limodzi la alpha globin limatayika.
- Alfa thalassemia wamng'ono (kapena khalidwe) amapezeka pamene magawo awiri a alpha globin akutayika. Pali mitundu iwiri. Pamene ma alfabomu awiri omwe amagonjetsedwa ali ndi chromosome yofanana 16 amatchedwa cis , koma pamene chilolezo chimodzi cha alpha globin chikusowa pa chromosome iliyonse 16 izi zimatchedwa trans .
- Matenda a Hemoglobin H (kapena alpha thalassemia intermedia) amapezeka pamene magulu atatu a alpha globin samagwira ntchito. Pankhaniyi, pali mabotolo ochuluka kwambiri. Izi zikagwirizanitsa, zimatchedwa Hemoglobin H.
- Mankhwala amtundu wa fetereza amapezeka pamene mitundu yonse ya anai ya alphabini imatayika. Zakale, izi zakhala zosagwirizana ndi moyo. Ngati chiopsezo chisanadziwike, kupatsirana kwa intrauterine (kuika magazi kwa mwana wosabadwa mkati mwa chiberekero) kukhoza kuperekedwa bwino. Ana awa amafunika mankhwala opatsirana mosalekeza atabereka ndipo kawirikawiri amawotchera mafupa.
- Hemoglobin H Constant Spring ndi mitundu yosiyanasiyana ya alpha thalassemia komwe ma alpha alphabini amagazi ali otayika kuphatikizapo alfa globin gene imasinthidwa.
Ndani Ali pangozi ya Alpha Thalassemia?
Alfa thalassemia kawirikawiri amagwirizanitsidwa ndi Asia, Africa, ndi dera la Mediterranean. Ku US, pafupifupi 30 peresenti ya African-American ali ndi alpha thalassemia minima kapena yaing'ono.
Alfa thalassemia ndi chikhalidwe chobadwira ndipo amafuna kuti makolo onsewo azitsatira.
Munthu wopanda alpha thalassemia ayenera kukhala ndi magawo anayi a alpha globin. Kuopsa kwa kukhala ndi mwana ndi matenda a alpha thalassemia kumadalira udindo wa makolo. Mtundu wa alpha thalassemia wamng'ono ndi wamba kwambiri mwa anthu a ku Africa. Zithunzizi zimakhala zofala kwambiri kwa anthu ochokera ku Asia kapena m'dera la Mediterranean.
Ngati makolo awiri ali ndi trans alpha thalassemia wamng'ono (a- / a-), ana awo onse ali ndi trans alpha thalassemia wamng'ono. Ngati kholo limodzi lili ndi alpha thalassemia wamng'ono (aa / -) ndipo kholo lina liri ndi trans alpha thalassemia wamng'ono (a- / a-), ali ndi mwayi wokhala ndi mwana mmodzi ali ndi matenda a Hemoglobin H. Mofananamo, Ngati kholo limodzi lili ndi alpha thalassemia wamng'ono (aa / -) ndipo kholo lina ali ndi thalassemia minima (aa / a-), ali ndi mwayi wokhala ndi mwana ali ndi matenda a Hemoglobin H. Mankhwala a fetus amapezeka pamene makolo onse awiri ali ndi alpha thalassemia wamng'ono.
Kodi Alpha Thalassemia Apezeka Bwanji?
Alfa thalassemia minima sachititsa ma laboratory kusintha pa CBC. Ichi ndi chifukwa chake chimatchedwa chithandizi chamtendere. Izi zimawoneka ngati mwana wabadwa ndi matenda a Hemoglobin H. Izi zingangotsimikiziridwa ndi kuyesedwa kwa majini.
Nthaŵi zina alpha thalassemia yaing'ono imadziwika pawuni yakhanda , koma osati nthawi zonse.
Mayesowa ndi othandiza kwa mabungwe a Hemoglobin Bart kapena ofulumira. Anthu ambiri okhala ndi alpha thalassemia wamng'ono sindikudziwa. Izi nthawi zambiri zimafika poyera nthawi yowerengera magazi nthawi zonse (CBC). CBC idzawonetsetsa kuchepa kwa magazi m'thupi ndi maselo ofiira ochepa kwambiri. Izi zingasokonezedwe ndi kuchepa kwa magazi m'thupi . Kawirikawiri, ngati kusowa kwa magazi m'thupi kutayika ndipo beta ya thalassemia imachotsedwa, wodwalayo ali ndi khalidwe la alpha thalassemia. Ngati ndi kotheka, izi zikhoza kutsimikiziridwa ndi kuyesedwa kwa majini.
Hemoglobini H ingadziwidwe pawonekedwe la mwana wakhanda. Ana awa amatumizidwa ku katswiri wamatenda kuti aziyang'anitsitsa mosamala.
Odwala ena amadziwika patapita nthawi m'ntchito yokhudzana ndi kuchepa kwa magazi.
Mankhwala otchedwa Hydrops fetalis sizodziwika bwino, koma zimakhala zochititsa chidwi pa neonatal ultrasound. Kutayika kwa majini anayi a alpha globin kumapezeka panthawi ya ntchito chifukwa cha hydrops.
Kodi Chipatala cha Alpha Thalassemia ndi chiyani?
Palibe mankhwala oyenera ku alpha thalassemia minima kapena aang'ono. Anthu omwe ali ndi alpha thalassemia wamng'ono amakhala ndi kuchepa kwa magazi m'thupi.
Kutsekemera: Odwala ndi Hemoglobin H amakhala ndi kuchepa kwa magazi m'thupi. Mafupa amtunduwu amafunika nthawi zina pa nthawi ya matenda ndi malungo pamene kuchuluka kwa maselo ofiira a magazi akufulumira. Kutengeka kungakhale kofunika nthawi zonse pamene munthu wamkulu. Odwala omwe ali ndi Hemoglobin H Constant Spring akhoza kukhala ndi kuchepa kwa magazi m'thupi ndipo amafunika kuikidwa magazi nthawi zonse.
Chithandizo cha Iron Chelation: Odwala omwe ali ndi matenda a Hemoglobin H akhoza kukhala ndi chitsulo chosungunula ngakhale popanda kuika magazi pokhapokha ngati kuwonjezeka kwachitsulo kumawopsa. Akhoza kuchiritsidwa ndi mankhwala omwe amatchedwa chelators kuti athandize kuchotsa chitsulo chowonjezera.