Ukubukezwa kweThalassemia engekho-Transfusion
I-Thalassemia yiqembu lezinkinga ezichaphazela i-hemoglobin, iphrotheni, ngaphakathi kwamangqamuzana egazi obomvu (RBC). Abantu abadla i-thalassemia abakwazi ukukhiqiza i-hemoglobin evame ukuholela ekudleni kwegazi (isibalo esincane se-RBC) nezinye izinkinga.
I-Thalassemia ingaphulwa zibe izigaba ezintathu ezinkulu:
- Umkhuba: Umuntu uthatha isakhi sokuguquka kodwa akanalo isifo. Lokhu ngezinye izikhathi kuthiwa yi-thalassemia encane.
- I-Intermedia: Umuntu onesifo se-thalassemia phakathi kwendawo phakathi kokuziphatha nokukhulu.
- Amakhulu: Umuntu onesifo se-thalassemia esidinga ukumpontshelwa kwe-RBC impilo yonke.
I-Thalassemia Intermedia ihlolwe kanjani?
Nakuba, iningi le-thalassemia elikhulu lizobonakala esikrinini esanda kuzalwa , abantu abane-thalassemia intermedia kungenzeka bangabonakali kuze kube seminyaka. Laba bantu bavame ukubonwa ngokubala okuphelele kwegazi (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). I-alpha thalassemia intermedia ngokuvamile ibizwa ngokuthi i-hemoglobin H isifo njengoba lena yi-hemoglobin eyinhloko ebonakala kuphrofayli.
Yiziphi Izinkinga Ze-Thalassemia Intermedia?
Izinkinga ze-thalassemia intermedia zifaka:
- I-Iron overload
- Ukuphefumula kwamathambo: Ukuphuza kwamathambo
- I-hematopoiesis e-Extramedullary: Lokhu kubhekisela ekwandiseni uhlangothi lwesibindi, isibindi kanye / noma umthambo wegazi ukuze ukhiqize ukukhiqizwa kwegazi elibomvu. Amathambo amakhulu kakhulu abathintekile asemakhanda nasemahlathini.
- Ukuzikhohlisa: Ukukhiqizwa kokunciphisa kwezitho zocansi. Lokhu kungavimbela ukukhulelwa kungenzeki ngokwemvelo.
- Ama-Gallstones:
- Ama-clots egazi
Kungani Abantu Abasebenzisa I-Thalassemia Intermedia Bakhula Ukunqwabelana Kwensimbi?
Kunezizathu ezimbili zokuthi kungani abantu abane-thalassemia bethola ukungena kwensimbi.
- Phinda ukumpompela igazi elibomvu: Ngisho noma izingane ezinesifo se-thalassemia ngokuvamile zingadingi ukumpontshelwa njalo emasontweni amathathu kuya ku-4 njengabantwana abanezinkinga ezinkulu ze-thalassemia , zingadinga igazi eziningi njalo ngonyaka. Ukumpontshelwa kwegazi okubomvu okutholakele kufana ne-intravenous (IV) dose yensimbi. Umzimba awunayo indlela enhle yokususa le nsimbi emzimbeni. Ngakho ngokuhamba kwesikhathi lezi zimpompo eziphindaphindiwe zingabangela ukuthuthukiswa kwensimbi, ikakhulukazi esikhathini esizayo empilweni (abadala) kunabantu abane-thalassemia enkulu (ebuntwaneni).
- Ukwandiswa kwe-iron ekudleni: Umzimba uyaqaphela ukuthi umthambo wethambo awukwenzi umsebenzi omuhle okhiqiza i-hemoglobin namaseli abomvu egazi. I-Hepcidin iyi-protein evimbela ukumunca kwensimbi. E-thalassemia, amazinga we-hepcidin aphansi avumela ukuba insimbi eningi ingenele kunokuba iyadingeka. Kunconywa ukuthi abantu abane-thalassemia bahambisane nokudla okuncibilika kwe-iron futhi baphuze itiye ngokudla njengama-tea blocks ukunwabelana kwensimbi.
Yiziphi Izinketho Zokwelapha Thalassemia Intermedia?
- Ukunakekelwa kwezokwelapha njalo: Akuwona wonke umuntu one-thalassemia intermedia ozodinga ukwelashwa, kodwa kubalulekile ukuqhubeka nokunakekelwa kwezempilo okuseduze ukuqapha izinkinga.
- Ukuthungathwa: Abantu abane-thalassemia intermedia bangadinga ukumpontshelwa igazi, kepha ngokuvamile abavami njalo njengabantu abane-thalassemia enkulu kuze kube abantu abadala. Ukuguqulwa kungadingeka ngezikhathi zokukhula nokuthuthuka okukhulayo (ukukhulelwa), izifo ikakhulukazi ezinomkhuhlane, ukukhulelwa, noma ukulungiselela ukuhlinzwa.
- I-Folic acid: Abanye odokotela bangase batuse folic acid nsuku zonke ukusekela ukukhiqizwa kwe-RBC.
- I-splenectomy: E-thalassemia, i-spleen ingahle ikhulise (i-splenomegaly) ngomzamo wokuthuthukisa ukukhiqizwa kwe-RBC. Lokhu kuvame ukungasebenzi futhi kungadala ukwanda kwe-anemia kanye / noma ukwandise izidingo zokumpontshelwa igazi. Kulezi zimo, i-splenectomy ingacatshangwa.
- I-Hydroxyurea: I- Hydroxyurea imithi yansuku zonke ethathwe umlomo ngomzamo wokwandisa i-hemoglobin, ngaleyo ndlela ukunciphisa isidingo sokumpontshelwa kwe-RBC.
- Ukudla kwe-iron okuphansi: Ngenxa yokuthi abantu abane-thalassemia intermedia basengozini yokuthuthukisa insimbi engaphezu kokungenwa kwensimbi ekudleni, kungase kuphakanyiswe ukudla okuncane kwe-iron. Ihlobo, ikakhulukazi itiye elimnyama, linciphisa ukumunca kwensimbi futhi kunganconywa ngokudla.
Ukufunda ukuthi une-thalassemia intermedia ingasabeka ngoba kungenzeka ukuthi awunayo izimpawu. Qinisekisa ukulandelana nodokotela wakho njengohlelo ukuze ukwazi ukuqapha izinkinga ezingase zibe khona.
> Imithombo:
> Benz EJ. I-molecular pathology ye-thalassemia syndromes, ukubonakaliswa kwemitholampilo nokuxilongwa kwe-thalassemia, kanye nokwelashwa kwe-beta thalassemia. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA.