Ukubuyekezwa kwezinhlobo, izimpawu, ukuxilongwa nokuningi
I-Thalassemia iyisifo se-hemoglobin esibangela i-hemolytic anemia . I-Hemolysis yigama lokuchaza ukubhujiswa kwamangqamuzana egazi abomvu. Kubantu abadala, i-hemoglobin yenziwe ngamaketanga amane-amakhetho amabili e-alpha namaketanga amabili e-beta.
E-thalassemia awukwazi ukwenza amaketanga e-alpha noma i-beta ngokwanele okwenza umthambo wakho we-bone awukwazi ukwenza amangqamuzana egazi abomvu ngokufanele.
Amangqamuzana egazi abomvu nawo abhujiswa.
Ingabe Kukhona Ngaphezu Kokuthi 1 Uhlobo Lwe-Thalassemia?
Yebo, kunezinhlobo eziningi ze-thalassemia, kufaka phakathi:
- I-thalassemia ye-Beta iphakathi
- I-thalassemia enkulu ye-Beta (ukuxhomeka kwegazi)
- Isifo se-Hemoglobin H (uhlobo lwe-alpha thalassemia)
- I-Hemoglobin H-Constant Spring (uhlobo lwe-alpha thalassemia, oluvame kakhulu kune-hemoglobin H)
- I-hemoglobin I-beta thalassemia
Ziyini Izimpawu ZaseThalassemia?
Izimpawu ze-thalassemia zihlobene kakhulu ne-anemia. Ezinye izimpawu zihlobene ne-hemolysis kanye noshintsho lomnkantsha wamathambo.
- Ukukhathala noma ukukhathala
- Isikhumba esikhuphukile noma esikhuphukile
- I-yellowing yesikhumba (i-jaundice) noma amehlo (i-icterus yesikhumba) - i-bilirubin (i-pigment) ikhishwa kuma-cell obomvu uma ibhujiswa
- I-pleen ekhulisiwe (i-splenomegaly) - uma umnkantsha wezintambo ungeke ukwenze amangqamuzana egazi abomvu okwanele, isebenzisa iphleen ukwenza amanye amangqamuzana egazi abomvu
- Izinthala ze-Thalassemic - ngenxa ye-hemolysis e-thalassemia, umongo wamathambo (indawo lapho amangqamuzana egazi akhiwa khona) angena ekudleni okuningi. Lokhu kubangela ukukhulisa emathanjeni ebusweni (ikakhulukazi emathunzini nasemathambo amathambo). Le nkinga ingavinjelwa ngokuphathwa ngendlela efanele.
I-Thalassemia Ithola Kanjani?
E-United States ngokuvamile iziguli ezikhuthele kakhulu zivame ukutholakala ngehlelo lokuhlola ukuzalwa . Iziguli ezithintekile zingase zinikezwe esikhathini esizayo lapho i-anemia ibonakala ngokubala kwegazi eliphelele (i-CBC). I-Thalassemia ibangela i-anemia (i-hemoglobin ephansi) ne-microcytosis ( ivolumu elincane elincane lomzimba ).
Ukuhlolwa okuqinisekisiwe kubizwa ngokuthi i-hemoglobinopathy work-up noma i-hemoglobin electrophoresis. Lokhu kuhlolwa kubika izinhlobo ze-hemoglobin onayo. Kumuntu omdala ngaphandle kwe-thalassemia, kufanele ubone kuphela i-hemoglobin A ne-A2 (omdala). Ku-beta thalassemia intermedia futhi enkulu, une-elemount elevation in hemoglobin F (fetal), ukuphakama kwe-hemoglobin A2 enokunciphisa okukhulu kwe-hemoglobin A eyakhiwe. Isifo se-alpha thalassemia sibonakala ngokukhona kwe-hemoglobin H (inhlanganisela yezinsimbi ezine ze-beta esikhundleni se-2 alpha ne-2 beta). Uma ukuhlolwa kudideka, ukuhlolwa kofuzo kungathunyelwa ukuqinisekisa ukuxilongwa.
Ubani Ongengozini yeThalassemia?
I-Thalassemia yisimo esizuzwe njengefa. Uma bobabili abazali bephethe i- alpha thalassemia trait noma i-beta thalassemia, banalo ithuba lokuba nengane ene-thalassemia. Umuntu angazalwa ngesifo se-thalassemia noma isifo se-thalassemia-lokhu akukwazi ukushintsha. Uma unomkhuba we-thalassemia, kufanele ucabange ukuthi umlingani wakho uvivinywa ngaphambi kokuba nezingane zokuhlola ingozi yokuba nengane nge-thalassemia.
I-Thalassemia iphatheke kanjani?
Izinketho zokwelapha zisekelwe ebucayi be-anemia:
- Vala ukubuka: Uma i-anemia ilula futhi ibekezelela kahle-udokotela wakho angakubona njalo ukuze aqaphele inani lakho eliphelele legazi.
- Imithi: Imithi efana ne-hydroxyurea, eyandisa ukukhiqizwa kwe-hemoglobin yesisu, isetshenziswe ku-thalassemia ngemiphumela ehlukahlukene.
- Ukumpontshelwa igazi: Uma i-anemia yakho inzima futhi ibangela izinkinga (isb., I-splenomegaly ephawulekayo, ama-thalassemic facies), ungabekwa ohlelweni lokumpontshelwa okungapheli. Uzothola ukumpontshelwa igazi njalo ngemva kwamasonto amathathu kuya kwamane ukuze uzenzele umthambo wakho njengamaseli amasha egazi obomvu ngangokunokwenzeka.
- Ukufakelwa kwe-bone marrow (noma i-stem cell): Ukufakelwa kwezitshalo kungaba ukwelapha. Imiphumela engcono kakhulu ihambisana nabaxhasi abathandana nabo. Indodana egcwele inomunye emanzini amane okufanisa omunye umntanakho.
- I-Iron Chelation: Iziguli ezithola uketshelwa igazi okungapheli zithole insimbi eningi egazini (insimbi itholakala ngaphakathi kwamaseli obomvu egazi). Ukwengeza, abantu abane-thalassemia bathatha inani elikhulu legazi ekudleni kwabo. Lesi simo esibizwa ngokuthi i-iron overload noma i-hemochromatosis kubangela ukuba insimbi ifakwe kwezinye izicubu ezinqeni, enhliziyweni nasezinkomini, okwenza ukuba lezi zitho zilimale. Lokhu kungelashwa ngemithi ebizwa ngokuthi i-iron chelators esiza umzimba wakho ukuba ususe insimbi eyengeziwe.