Tsanangudzo:
Diamond Blackfan Anemia (DBA) inokonzerwa neanemia inotora nhaka iyo fufupa remafupa haigone kuita masero matsvuku eropa (anonzi pure red cell aplasia). Icho chikamu cheboka guru rematambudziko rinonzi rakagara nhaka marrow failure syndromes.
Ndezvipi zvinhu zveDBA?
Inenge vose varwere vanoonekwa mune gore rokutanga rehupenyu.
- Anemia: Inemia yeDBA yakaoma uye inowanzodikanwa kuwedzerwa kuwedzerwa ropa.
- Kukanganisa kwekuzvarwa: Kusvikira hafu yevarwere vane DBA vanewo kushaya muviri. Nzvimbo dzomuviri dzinowanzobatwa ndezvekuti:
- Musoro, mutsipa, uye chiso
- Moyo
- Thumbs
- Zviratidzo uye zvekuvhenekera
- Short stature
- Kuwedzera kwengozi yekenza: Vanhu vane DBA vanoita sevanowedzera mungozi yekenza. Idzi dzinosanganisira myeloid leukemia (AML), myelodysplastic syndromes (MDS), uye mishonga yakasimba.
Chii chinokonzera DBA?
DBA inotora nhaka marora kusakatika syndrome. Inenge hafu yezviitiko ndeyemhuri zvinoreva kuti genetic mutation yakapfuudzwa nemubereki. Mimwe mamiriro ezvinhu ari sporadic, achiitika panguva pfupi. Mune maitiro edzimhuri, kwete yese nhengo dzemhuri dzakakanganiswa zvakafanana. Mubereki angave ane kuchinja kunobatanidzwa neDBA asi haawanikwe kusvikira mushure mokunge mwana wake aonekwa.
Zvose masero mumuviri ane humwe hupenyu hunozopinda mumuviri kufa pasi pekutongwa kwakanyanya.
MuDBA chimiro chemagetsi chinokonzera masero emuviri (kuita masero matsvuku eropa uye masero matsvuku ekutanga mumaropa) kuti azviparadze nokukurumidza pane zvaanofanira kutungamirira kuderera kwekugadzirwa kwemasero matsvuku eropa.
DBA inowanikwa sei?
Nhamba yakazara yeropa (CBC) inoratidzira anemia uye inoratidza kukura kupfuura masero masero matsvuku eropa (macrocytosis).
Nenzira yakawanda iyo anemia inotarisana isina kuchinja muchena yeropa kana kuti platelet count. Reticulocyte count is low (reticulocytopenia) nokuti bone marora haikwanise kubhadhara ichi chirwere chemahyamu.
DBA inofanira kusiyaniswa kubva ku erythroblastopenia inotevera yehuduku (TEC) iyo inogona kuisa muboka rimwe chete asi rinogadzirisa zvese. Kunyanya iyo fetal hemoglobin level uye erythrocyte adenosine deaminase level inowanzokwidziridzwa muDBA asi kwete muTEC.
Kana mumwe munhu achifungidzirwa kuva neDBA, kuongorora mazita ekuberekwa kunofanira kutumirwa. Kana ruzivo rwechimiro rwuri kuonekwa, dzimwe nhengo dzemhuri dzinofanirwa kuongororwa dzakapiwa dambudziko rinowedzera rekenza.
Dzimwe nguva mapfupa emapfupa aspirate uye biopsy anoitwa kuti aongorore mamiriro ezvinhu ayo masero matsvuku eropa anoitwa. Iropa remafu rinowanzoshandiswa kunze kwekushayikwa kwemasero matsvuku eropa (masero ekutanga anowedzera kuva masero matsvuku eropa).
Zviratidzo zvekuongorora ndezvi:
- Zera risati rapera gore
- Macrocytic (yakakura kupfuura yakasarudzwa) aneemia isina kuchinja muchena masero eropa kana platelet count
- Reticulocytopenia
- Kazhinji mapfupa emarora kunze kwekushayikwa kwevatambi vatsvuku vesero
Kana murwere achifungidzirwa kuva neDBA asi asingakwanisi kugadzirisa chirongwa ichi chekuongorora, pane zviyero zvechipiri zvinogona kutsigira zvinenge zvichinyatsozivikanwa.
Ndezvipi zvirongwa zveDBA?
Kunyange zvazvo varwere vakawanda vachida kurapwa kusvikira 25% yevarwere vachava nekugadzirisa kusangana.
- Kufuridzira: Inemia yeDBA yakaoma uye inoda kuwedzerwa kuwedzerwa ropa. Iyi ndiyo inonyanya kurapwa kusvikira yakachengeteka kutanga steroid.
- Corticosteroids: Oral steroid yakafanana ne prednisone / prednisolone ndiyo musana werapi yevana vane DBA. Steroidsi inotanga uye yakurumurwa kusvika kumudziro wakaderera unoda kubvisa kudiwa kwekuwedzerwa ropa. Kana pasina mhinduro, kuwedzerwa ropa kunodzorerwa zvakare.
- Bhuna marora kuiswa : Bone marrow kupera kunogona kuva kurapa. Migumisiro yakanakisisa inobva kune vakaroorana vanobatsirana. Vana vechidiki vekuSouth Africa vanofanirwa kuedza kuongororwa kwemajini kuitira kuti ave nechokwadi chokuti haana hunyoro hweDBA.