I-Alpha thalassemia yi- anemia ezuze ilifa apho umzimba awukwazi ukuvelisa inani eliqhelekileyo le-hemoglobin. I-Hemoglobin A (i-hemoglobin enkulu kubantu abadala) iqulethe ezimbini ezimbini ze-globin kunye neetoni ezimbini ze-globin. Kwi-alpha thalassemia, kukho inani elincinci le-alpha globe.
Ziziphi iintlobo zeAlfa Thalassemia?
- I-Alpha thalassemia minima (umthuthi wangasese) uvela xa enye i-alphabbin gene ilahleka.
- I-Alpha ye-thalassemia encinci (okanye impawu) iyenzeka xa iifayile ze-globin ze-alpha zilahlekile. Kukho iifom ezimbini. Xa iifayile ze-globin ze-alpha ezilahlekileyo zifana ne-chromosome efanayo 16 zibizwa ngokuba yi- cis , kodwa xa enye i-alpha yolbini yegazi ilahleka kwikhophi nganye ye-chromosome 16 oku kuthiwa yi- trans .
- Isifo se-Hemoglobin H (okanye i-alpha thalassemia intermedia) iyenzeka xa i-alfabhethi ze-globin zegciwane zingasebenzi. Kule meko, kukho inani eligqithisileyo le-beta globins. Xa ezi zidibene kunye, kuthiwa yiHemoglobin H.
- I-Hydrops fetalis iyenzeka xa zonke iiglob ezine zeglobin zegesi zilahlekile. Ngokomlando, oku akuhambelani nobomi. Ukuba umngcipheko uyaziwa kwangaphambili, ukumpontshelwa kwe-intrauterine (ukumpontshelwa emntwaneni xa usesiswini) kungavumela ukunikezelwa ngempumelelo. La bantwana bafuna unyango olungapheliyo emva kokuzalwa kwaye ngokuphindaphindiweyo batyelela ukutsalwa komongo.
- I-Hemoglobin H Injalo i-Spring iyinto eyahlukileyo ye-alpha thalassemia apho iifayile ze-globin ze-alpha ezilahlekileyo kunye ne-alpha alphabin gene ziguquguquka.
Ngubani Osemngciphekweni weAlfa Thalassemia?
I-alpha thalassemia idla ngokuqhagamshelana ne-Asiya, i-Afrika, kunye nommandla waseMeditera. E-US, malunga neepesenti ezingama-30 zama-Afrika-aseMelika ane-alpha thalassemia minima okanye encinci.
I-Alpha thalassemia yimeko ezuze ilifa kwaye idinga ukuba abazali bobabini babe ngabathwali.
Umntu ongenalo i-alpha thalassemia kufuneka abe nezine ze-alpha globin genes. Umngcipheko wokuba nomntwana onesifo se-alpha thalassemia uxhomekeke kwimeko yabazali. Ifom ye-alpha thalassemia encinci ixhaphake kakhulu kubantu baseAfrika. I- cis ifom yezona ziqhelekileyo kubantu abavela e-Asia okanye kwingingqi yeMeditera.
Ukuba ngaba bobabini abazali be- trans alpha thalassemia encinci (a- / a-), bonke abantwana babo baya kuba ne- trans alpha thalassemia encinci. Ukuba omnye umzali une-alpha ye-alpha thalassemia encinci (aa / -) kunye nomnye umzali une- trans alpha ye-thalassemia encinci (a- / a-), abanama-1 kwithuba eli-2 lokuba nomntwana abane-Hemoglobin H. Ngokufanayo, Ukuba umzali omnye ene-alpha thalassemia encinci (aa / -) kunye nomnye umzali unomntwana we-thalassemia (aa / a-), abanalo 1 kwithuba eline lokuba nomntwana ene-Hemoglobin H. I-Hydrops fetalis iyenzeka xa bobabini abazali be-alpha thalassemia encinci.
I-Alpha Thalassemia ichongwa njani?
I-Alpha thalassemia minima ibangela ukuba akukho tshintsho lwebhoratri kwiCBC. Yingakho kuthiwa yi-carrier carrier. Oku kudla ngokukrokrelwa emva kokuba umntwana ezalwe ne-Hemoglobin H. Oku kunokwenziwa kuphela ngovavanyo lwemizimba.
Ngamanye amaxesha i-alpha thalassemia encinci ichongiwe kwiskrini esandula kuzalwa , kodwa kungekho kuzo zonke iimeko.
Uvavanyo lukhangelekile kumaHemoglobin Bart okanye amaqhinga okukhawuleza. Abantu abaninzi abane-alpha thalassemia encinane andinayo ingcamango. Oku ngokuqhelekileyo kuza kukhanya ngexesha lexesha elipheleleyo legazi (CBC). I-CBC iya kubonakalisa i-anemia elula kunye neyomodareyitha kunye neeseli ezincinci zegazi ezibomvu. Oku kungadideka ngentswelo ye-anemia . Ngokuqhelekileyo, ukuba inkunkuma ye-anemia iyabonakala ngaphandle kwaye i-beta thalassemia isetyenziswe ngaphandle, isigulane sinomzila we-alpha thalassemia. Ukuba kuyimfuneko, oku kuya kuqinisekiswa ngovavanyo lwemizimba.
I-Hemoglobin H inokuqatshelwa kwiskrini esandula kuzalwa. Aba bantwana bathunyelwa kwi-hematologist ukuba bajongwe ngokukhawuleza.
Ezinye izigulane zibonwa kamva ebomini ngexesha lomsebenzi we-anemia.
I-Hydrops fetalis ayiyiyo i-diagnostic ethile, kodwa iindawo ezibonakalayo kwi-neonatal ultrasound. Ukulahlekelwa kwezine ze-alpha globin zegeni kufunyanwa ngexesha lokusebenza ngenxa ye-hydrops.
Ziyintoni Iingonyango ze-Alpha Thalassemia?
Akukho unyango olufunekayo kwi-alpha thalassemia minima okanye encinci. Abantu abane-alpha thalassemia encinci baya kuba ne-anemia engapheliyo.
Ukutshintshwa: Izigulane ezineHemoglobin H zihlala zine-anemia ephakathi. Iinguqulelo zifunwa ngezinye iinkhathi ngexesha lokugula kunye nomkhuhlane njengoko inani legazi elibomvu lichithwa. Ukutshintshiswa kunokudinga rhoqo rhoqo xa ukhulile. Izigulane ngeHemoglobin H Unyaka oMthombo weNkqubela unokuba ne-anemia ebalulekileyo kwaye ifuna ukumpontshelwa rhoqo ngexesha lokuphila kwabo.
I-iron chelation yonyango: Izigulane nge-Hemoglobin H isifo sinokuphuhlisa i-iron ngaphezu kokungabikho kwegazi ekumanyeni ukunyuswa kwesinyithi emathunjini amancinci. Banokunyangwa ngamachiza abizwa ngokuba yi-chelators ukukhupha umzimba we-iron eyimfuneko.