I-thalassemia ye-Beta i- anemia ezuzwe njengefa lapho umzimba ongakwazi ukukhiqiza i-hemoglobin ngokuvamile. I-Hemoglobin A (i-hemoglobin enkulu kubantu abadala) iqukethe amaketanga amabili e-globin e-alpha kanye namaketanga amabili we-beta globin. Ku-beta thalassemia, umnto wethambo awukwazi ukukhiqiza inani elivamile lama-beta globin okwenza kancane kancane ku-hemoglobin ayikho A. Futhi, kukhona okungaphezu kwe-globin ye-alpha engabhekwa ne-beta globin ebangela ukuhlakazeka kwe-cell cell eyaziwa ngokuthi i-hemolysis.
Ubani Osesengozini?
I-thalassemia ye-Beta yisimo esizuzwe njengefa. Kudinga ukuthi bobabili abazali babe yithwala ye-disorder ebizwa nge-beta thalassemia trait noma encane. Uma bobabili abazali banomthelela we-beta thalassemia, bane-1 ku-4 amathuba okuba nengane ene-beta thalassemia isifo. Ngeshwa, ngoba isici se-beta thalassemia asiboni izimpawu, abazali bangase bangazi ukuthi le ngozi ingakafiki ngaphambi kokukhulelwa.
Yiziphi izinhlobo ze-Bhala Thalassemia?
I-thalassemia ye-Beta ingahle ibekwe ngezindlela ezimbili: ngokuguqulwa kwezakhi zofuzo ezizuzwe njengefa noma ngezidingo zokumpontshelwa igazi.
Izinguquko zomzimba
I-beta zero thalassemia : I-zero e-beta zero mutation ibonisa ukuthi ayikho i-globa ye-beta engakhiwa yilo chromosome.
I-Beta kanye ne-thalassemia : i-globeta yeBeta ikhiqizwa ngamanani aphansi. Inani le-beta globin elikhiqizwa lihlukahluka kakhulu ngokushintshwa okulutholile. Izinguquko ezithile azikhiqizi cishe i-globa ye-beta ezenza zifane nemithi efana ne-beta zero.
Izidingo zokuthambisa
I-thalassemia enkulu ye- Beta : I-thalassemia enkulu ye- Beta ichazwa isidingo sokumpontshelwa isikhathi eside, ngokuvamile ngenyanga (okuthiwa yi-hypertransfusion therapy).
I-thalassemia ye-Beta i-intermedia : Ngokujwayelekile i-anemia engalinganisa ukuthi ingadinga ukumpontshelwa ngezikhathi ezithile (ngezifo, ukukhulelwa, njll) kodwa hhayi njalo.
Isici se-thalassemia se-Beta / encane : Lokhu kubangela ukutholakala kwe-anemia emnene okuvame ukutholwa ekubaleni kwegazi okuphelele.
I-Beta Thalassemia Ithola Kanjani?
Ngokuvamile, omkhulu we-beta thalassemia ubonakala esikrinini esanda kuzalwa . Kulezi zinsana, uhlolo luzoveza kuphela i-hemoglobin F (noma i-fetal), ayikho i-hemoglobin A. Lezi zingane zibhekiswe ku-hematologist ukuze zihlolwe eduze. Ezinye ezithinteka kakhulu nge-beta thalassemia phakathi kweziguli zizobonakala ngale ndlela.
Ezinye iziguli ezithintekayo zizobonakala ngokujwayelekile kwesilinganiso segazi (CBC). I-CBC izokwethula i-anemia emnene kuya ekulinganiseni enezinqamuzana zegazi elibomvu kakhulu. Lokhu kungahle kudidaniswe nokuntuleka kwe-anemia yensimbi. Ukuxilongwa kuqinisekiswa yi-profile ye-hemoglobin (ebizwa nangokuthi i-electrophoresis). Ku-beta thalassemia intermedia kanye nokulinganisa lokhu kuhlolwa kwembula ukuphakama ku-hemoglobin A2 (ifomu lesibili le-hemoglobin yabantu abadala) futhi ngezinye izikhathi F (fetal).
Yiziphi Izindlela Zokwelashwa kwe-Beta Thalassemia?
Isici se-thalassemia se-Beta asidingi ukwelashwa. Abantu abanesici se-beta thalassemia bayoba ne-anemia engapheli yonke.
Ukuthuthwa kwegazi : Iziguli ezibhekene ne-beta thalassemia ezinkulu zidinga ukumpontshelwa impilo yonke. Ukwelashwa kwe-Transfusion kusetshenziselwa ukuvimbela ukukhiqizwa kwamaseli obomvu emangeni.
I-thalassemia ye-Beta iphazamisa iziguli ezingadinga ukwelashwa ngegazi ngesikhathi sezifo nokukhulelwa (ngesikhathi sokukhula).
I-iron chelation therapy: Iziguli ezine-beta thalassemia ezinkulu zithola insimbi engaphezu kwegazi ngokusebenzisa igazi elibomvu elisegciwane legazi. Le nsimbi engaphezulu ingabangela ukulimala kwezitho, ikakhulukazi inhliziyo. I-Iron Chelation iyasiza umzimba ekukhipheni insimbi. Lezi zinganikwa njengemithi yomlomo noma ukumnika ngaphansi kwesikhumba. Iziguli ezine-beta thalassemia intermedia zingathuthukisa ukuxhuma kwensimbi ngisho nangaphandle kokumpontshelwa igazi okwedlula ukwandiswa kwensimbi emathunjini amancane.
I-Splenectomy : I-spleen ingaba yanda kakhulu ngenxa yokudilizwa kwe-cell cell (i-hemolysis) kanye nokukhiqizwa kwe-cell cell ebomvu. Uma isidingo sokumpontshelwa igazi sanda noma ezinye izibalo zamaseli wegazi zihla ngenxa yokubamba epeni, isitha singadinga ukususwa ngokuhlinzwa.
Hydroxyurea: Hydroxyurea isetshenziselwe ukwandisa ukukhiqizwa kwe-hemoglobin fetal. Ukuphumelela kuye kwashintsha.