Mutsva weTe-Transfusion Inotarisira Thalassemia
Thalassemia iboka rematambudziko anokonzera hemoglobin, protein, mukati memasero matsvuku eropa (RBC). Vanhu vanotora thalassemia havakwanisi kubereka hemoglobin inowanzoita kuti munhu ave nechirwere chemahyamu (pasi reRBC count) nezvimwe zvinetso.
Thalassemia inogona kuputswa muzvikamu zvitatu zvakakura:
- Tsika: Munhu anotakura geni rekuchinja asi haana chirwere. Izvi dzimwe nguva zvinonzi thalassemia maduku.
- Intermedia: Munhu ane thalassemia pane imwe nzvimbo pakati pehutano uye hukuru.
- Major: Munhu ane thalassemia anoda hupenyu hweRBC hupenyu hwose.
Thalassemia Intermedia Inowanikwa Sei?
Kunyange zvazvo, nguva huru yenguva huru thalassemia ichaonekwa pachirongwa chitsva , vanhu vane thalassemia intermedia vangave vasingazivikanwi kusvikira makore gare gare. Vanhu ava vanowanzozivikanwa pamutambo weropa rakazara (CBC). ICBC icharatidza anemia anononoka kuenzanisa nemasero matsvuku eropa. Izvi zvinogona kuvhiringidzika nekushaya simba kwesimbi.
Kuongororwa kunosimbiswa nehmoglobin profile (inonziwo electrophoresis). Mu beta thalassemia intermedia uye chiitiko ichi kuongorora kunoratidza kukwirira kwehemoglobin A2 (imwe yechipiri yevanhu vakuru hemoglobin) uye dzimwe nguva F (fetal). Alpha thalassemia intermedia inowanzonzi hemoglobin H chirwere sezvo iyi ndiyo inonzi hemoglobin inonyanya kuonekwa pane zvayo.
Ndezvipi Zvinetso zveThalassemia Intermedia?
Zvinetso zve thalassemia intermedia zvinosanganisira:
- Iron inowedzera
- Osteoporosis: Kuodza kwemafupa
- Extramedullary hematopoiesis: Izvi zvinoreva kuwedzera kwepleen, chiropa uye / kana bone marrow kuti kuwedzere kusvibiswa kwemasero eropa. Mafupa akanyanya kukanganiswa ari mudhehenya - pamusoro uye padama mapfupa.
- Kunyengedza: Kurambidzwa kwekugadzirwa kwezvipombwe. Izvi zvinogona kudzivirira kuberekwa kuti zvirege kuitika zvakasikwa.
- Gallstones:
- Ropa rinovhara
Sei Vanhu Vane Thalassemia Intermedia Kukudziridza Iron Overload?
Kune zvikonzero zviviri zvinoita kuti vanhu vane thalassemia intermedia vave nesimbi yakakura.
- Dzokorora ropa rakatsva: Kunyange zvazvo vana vane thalassemia intermedia kazhinji vasingadi kuwedzerwa ropa kwevhiki rimwe nerimwe kusvika ku4 sevana vane thalassemia makuru , vachiri kuda kuwedzerwa kuwedzerwa ropa gore negore. Kuwedzerwa kweropa rimwe nerimwe kwakagamuchira kwakafanana neyomukati (IV) muyero wesimbi. Mutumbi hauna nzira yakanaka yekubvisa iyi iron kubva mumuviri. Saka nekufamba kwemazuva aya kuwedzerwa kuwedzerwa kunogona kukonzera kukura kwesimbi kunowedzera, kunyange zvazvo kakawanda gare gare muhupenyu (vanhu vakuru) kune vanhu vane thalassemia makuru (uhu).
- Kuwedzera kunwa kwesimbi kubva pakudya: Muviri unoona kuti marora emapfupa haasi kuita basa rakanaka rinobudisa hemoglobin uye masero matsvuku eropa. Hepcidin ipuroteni inovhara kubatwa kwesimbi. Mu thalassemia, hepcidin mazinga ari pasi achibvumira kuti imwe iron iswededzwe kupfuura inodiwa. Zvinokurudzirwa kuti vanhu vane thalassemia intermedia vanotevera kudya kwakaderera kwesimbi uye kunwa tiyi pamwe chete nezvokudya setii blocks kubatwa kwesimbi.
Chii Chinorapa Zvirongwa zveThalassemia Intermedia?
- Nguva dzose kurapwa kwevanhu: Haasi munhu wese ane thalassemia intermedia achada kurapwa, asi zvakakosha kuti uenderere mberi kwehutano hwepamusoro kuti uongorore zvinetso.
- Kufuridzira: Vanhu vane thalassemia intermedia vangada kuwedzerwa, asi kazhinji havawanzoiti sevanhu vane thalassemia vakuru kusvikira vakura. Kuwedzerwa ropa kunogona kudiwa munguva dzekuwedzera kukura uye kukura (kufuma), zvirwere kunyanya avo vane fever, pamuviri, kana kugadzirira kuvhiyiwa.
- Folic acid: Vamwe vanachiremba vanogona kukurudzira folic acid zuva nezuva kutsigira RBC kukohwa.
- Splenectomy: MuThalassemia, chirwere chinogona kuwedzera (splenomegaly) mukuedza kuvandudza rBC rutsigiro. Izvi kazhinji hazvibatsiri uye zvinogona kukuvadza anemia uye / kana kuwedzerwa kuwedzerwa ropa. Mumamiriro ezvinhu aya, splenectomy inogona kufungwa.
- Hydroxyurea: Hydroxyurea imishonga yezuva nezuva inotorwa nemuromo mukuedza kuwedzera hemoglobin, zvichiita kuti kuderedza kudiwa kweRBC kuwedzerwa.
- Kudya zvakaderera kwesimbi : Nokuti vanhu vane thalassemia intermedia vari pangozi yekuwedzera simbi inowedzera kubva pakuwedzerwa kwesimbi mukati mekudya, kudyiwa kwesimbi kusinganzwi kunogona kukurudzirwa. Tea, kunyanya chai yeusiya, inoderedza kusvuta kwesimbi uye inogona kukurudzirwa nekudya.
Kudzidza kuti une thalassemia intermedia inogona kushamisa sezvo iwe usina kunge uine zviratidzo. Iva nechokwadi chekutevera nechiremba wako semarongwa kuitira kuti iwe unogona kuongororwa nekuda kwezvinetso.
> Sources:
> Benz EJ. Maitiro echiremera chethalassemia syndromes, Clinical zviratidzo uye kuongororwa kwethalassemia, uye Kurapa kwe beta thalassemia. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA.