Kuongorora mhando, zviratidzo, kuongororwa & nezvimwe
Thalassemia chirwere che hemoglobin chinokonzera hemolytic anemia . Hemolysis ishoko rekutsanangura kuparadzwa kwemasero matsvuku eropa. Muvanhu vakuru, hemoglobin yakaitwa nemaketani mana-maviri maaketani emaketani nemaketani maviri ezvita.
Mu thalassemia haugone kuita maketani eAlfa kana beta mumutokwana wakakwana kuita kuti mafuta ako emarara asakwanisa kuita masero matsvuku eropa zvakanaka.
Masero matsvuku eropa anoparadzwa zvakare.
Pane Pane Zvakawanda Kupfuura 1 Rudzi rweThalassemia?
Hungu, kune marudzi akawanda e thalassemia, kusanganisira:
- Beta thalassemia intermedia
- Beta thalassemia guru (kuwedzerwa ropa)
- Hemoglobin H chirwere (chimiro che alpha thalassemia)
- Hemoglobin H-Constant Spring (imwe yeropa thalassemia, kazhinji yakaoma kupfuura hemoglobin H)
- Hemoglobin E-beta thalassemia
Ndezvipi Zviratidzo zveThalassemia?
Zviratidzo zve thalassemia zvinowanzobatana neanemia. Zvimwe zviratidzo zvakabatana ne hemolysis uye marrow bone inoshanduka.
- Kuneta kana kuneta
- Pallor kana ganda ganda
- Kupenya kweganda (jaundice) kana maziso (scleral icterus) - bilirubin (pigment) inosunungurwa kubva mumasero matsvuku eropa kana yaparadzwa
- Yakapararira spleen (splenomegaly) - apo mafuta emapfupa haagoni kuita masero matsvuku eropa, anoshandisa chirwere ichi kuita mamwe masero matsvuku eropa
- Thalassemic facies - nekuda kwe hemolysis mu thalassemia, marora (nzvimbo umo masero eropa anoumbwa) anowedzera kupinda. Izvi zvinokonzera kukura mumapfupa echiso (kunyanya pamberi nematama mapfupa). Dambudziko iri rinogona kudzivirirwa nekurapa kwakakodzera.
Thalassemia Inowanikwa Sei?
MuUnited States kazhinji vanonyanya kuvhiringidzwa nevarwere vanowanzoongororwa nehurongwa hwekuzvarwa patsva . Vanosangana nevarwere vanokanganisa vanogona kuendeswa kune imwe nguva yechirwere apo chirwere chekushaya ropa chinowanikwa pahuwandu hwekuverenga ropa (CBC). Thalassemia inokonzera anemia (low hemoglobin) uye microcytosis (low mean corpuscular volume ).
Kuedzwa kwakasimba kunonzi hemoglobinopathy work-up kana hemoglobin electrophoresis. Iyi kuedza inorondedzera mhando dze hemoglobin yaunayo. Mune munhu mukuru asina thalassemia, unofanira kungoona hemoglobin A uye A2 (munhu mukuru). Mu beta thalassemia intermedia uye chikuru, iwe une kukwirira kwepamusoro mu hemoglobin F (fetal), kukwirira kwe hemoglobin A2 ine kunyanya kuderedza muwandu weHemoglobin A yakagadzirwa. Chirwere cheAfa thalassemia chinoratidzwa nekuvapo kwe hemoglobin H (kusanganiswa kwe 4 beta maketani pane 2 alpha uye 2 beta). Kana kuedzwa kuchivhiringidza, kuongorora mazita evanhu kunogona kutumirwa kusimbisa kuongororwa.
Ndiani Ari panjodzi yeThalassemia?
Thalassemia inowanikwa nhaka. Kana vose vabereki vane alpha thalassemia trait kana kuti beta thalassemia trait, vane rimwe mukana mana yekuva nemwana ane thalassemia chirwere. Munhu anozvarwa ane thalassemia mitiro kana chirwere chethalassemia-izvi hazvigoni kuchinja. Kana iwe une thalassemia mamiriro, iwe unofunga kufunga kuti wawakaroorana naye akaedzwa vasati vava nevana kuti vaongorore njodzi yekuwana mwana ane thalassemia.
Thalassemia Inotarirwa Sei?
Mishonga yekurapa inobva pakusimba kweanemia:
- Kunyatsocherechedza: Kana chirwere chemahyamu chiine nyoro-yepamusoro uye chakanyatsorambidzwa-chiremba wako angakuona nguva dzose kuti uongorore ropa rako rakazara.
- Mishonga: Mishonga yakafanana ne hydroxyurea, inowedzera kubereka kwako kwe fetal hemoglobin, yakashandiswa mu thalassemia nemigumisiro yakasiyana.
- Kuwedzerwa ropa: Kana kushaya ropa kwako kwakanyanya uye kuri kukonzera matambudziko (semuenzaniso, inokosha splenomegaly, thalassemic facies), unogona kuiswa purogiramu isina kuwedzerwa ropa. Iwe uchagamuchira kuwedzerwa ropa kwevhiki nhatu kusvika kuchina, uchiedza kuita kuti mafuta ako emapfupa aite se mashoma masero matsvuku eropa sezvinobvira.
- Iropa marora (kana stem cell) kuiswa: Kuiswa kwepakati kunogona kuva kurapa. Zvakanakisisa zvinowanikwa nevanonyatsokodzera vakaroorana vanopa. Mhuri yakakwana ine rimwe mukana mana yekufananidza imwe hama.
- Iron chelation: Varwere vanowana kuwedzerwa ropa kusingaperi vanowana iron yakawandisa kubva muropa (iron inowanikwa mukati memasero matsvuku eropa). Uyezve, vanhu vane thalassemia vanowana ropa rakawandisa kubva mukudya kwavo. Izvi zvinonzi iron inowedzeredza kana hemochromatosis inokonzera simbi inofanira kuiswa mune dzimwe tishu muropa, mwoyo, uye pancreas, zvichikonzera kukuvara kune nhengo idzi. Izvi zvinogona kurapwa nemishonga inonzi iron chelators iyo inobatsira muviri wako kuti ubvise dare rakawanda.