Kazhinji Kazhinji Inobata Vana Vacheche
MuBlackfan Diamond (kana Diamond Blackfan) anemia, mutumbi wemuviri wemuviri unobereka zvishoma kana kusina masero matsvuku eropa. Blackfan Diamond anemia inokanganisa vanhu vanenge 600 kusvika ku700 munyika yose. Chikonzero chayo hachizivikanwe, kunyange zvazvo maitiro ezvakaberekwa mujeni rinonzi RPS19 pane chromosome 19 inobatanidza ne 25% yematambudziko. Munenge 10 kusvika ku20% yezviitiko, kune nhoroondo yemhuri yehutano.
Zviratidzo
Blackfan Diamond anemia iripo pakuberekwa asi zvinogona kuva zvakaoma kuziva. Muchikamu chetatu chevana vanoberekwa vane chirwere, pane zviremara zvenyama zvakadai sezvakaitwa nemaoko kana zvirema zvemoyo, asi zviratidzo zvakajeka hazvina kuonekwa. Zviratidzo zvinogonawo kusiyanisa zvikuru, kubva pane zvishoma kusvika kune zvakanyanya uye kutyisidzira upenyu.
Masero matsvuku eropa anotakura okisijeni mumuviri wose, saka mwana ane Blackfan Diamond angave ane zviratidzo zvine chokuita nekusakwana kweropa (oxygen) (anemia):
- pallor (paleness)
- kusagadzikana kwepfungwa, nekuda kwemoyo kuedza kuchengetedza okisijeni inofamba mumuviri wose
- kuneta, kusagadzikana, uye kupera .
Kuziva
Blackfan Diamond anemia inowanzoongororwa mukati memakore maviri ekutanga ehupenyu, dzimwe nguva kunyange pakuberekwa, zvichienderana nezviratidzo. Semuenzaniso, mwana angangodaro anokonzera kuti ane chirwere chepemhi kana achigara aripo uye asingakwanisi kufema apo anonwa bhodhoro kana mukoti.
Vabereki vanowanzofungidzira kuti pane "chimwe chinhu chakashata" nemwana wavo. Kuongororwa kweBlackfan Diamond anemia, kunyanya, hakugoni kuzivikanwa pakarepo, nokuti chirwere chacho hachina chakanaka uye vashoma vanachiremba vanoziva nayo.
Chiverengero chakakwana chemasero eropa (CBC) yechecheche inogona kuratidza nhamba yakadzika yemasero matsvuku eropa uyewo pasi hemoglobin.
Imwe yeropa rekuongorora ropa raizoratidza zvakanyanya adenosine deaminase basa (ADA). Mushonga wemapfupa (biopsy) unoratidza kuti mashoma masero matsvuku eropa akanga achigadzirwa.
Kurapa
Mutsara wokutanga wemishonga we Blackfan Diamond anemia ndeye kupa mwana steroid mishonga, kazhinji prednisone. Vanenge 70 muzana yevana vane Blackfan Diamond anemia vanozopindura kurapwa uku, umo mishonga inokurudzira kugadzirwa kwemamwe masero matsvuku eropa. Zvisinei, izvi zvinoreva kuti mwana anofanira kutora mishonga ye-steroid kwehupenyu hwake hwose, iyo ine zviremera zvakakomba zvakadai seshuga , glaucoma , bone kuderedza ( osteopenia ), uye kuwedzerwa kweropa . Uyewo, mishonga inogona kungomira kushanda kumunhu chero nguva.
Kana mumwe munhu asingapindi mishonga ye-steroid kana kuti ane chido chakakura kwazvo kuti aite kuti / tsvuku yake yeropa iwedzere, kurapa kunova kuwedzerwa ropa. Nguva dzose kuwedzerwa ropa kuchapa masero matsvuku eropa asiwo kunotungamirira kune iron yakawandisa mumuviri . Kazhinji, muviri unoshandisa iron iyo ichiita masero matsvuku eropa, asi sezvo munhu ane Blackfan Diamond aneemia ari kuisa masero akawanda, iron inowedzera. Munhu wacho zvino anofanira kutora mishonga iyo inotora yakawanda yimbi yakabuda mumuviri.
Chirwere chete chinowanikwa kuBlackfan Diamond anemia inonzi bone marrow transplantation , iyo inotsiva munhu asina kukwana marora marora nemafuta akanaka. Zvisinei, kuiswa kwemavara inzira inonetsa yekufamba nayo uye haiwanzoshanda nguva dzose. Inowanzochengeterwa vanhu vane steroid mishonga uye kuwedzerwa ropa hakubatsiri.
> Sources:
> Guinan, EC. "Kuongororwa uye Kutungamirirwa kweAplastic Anemia." ASH Education Book , 2011, 76-81.
> National Organisation for Rare Disorders. "Blackfan Diamond Anemia." Index yeRare Diseases, 2008.
> Diamond Blackfan Anemia Support Group. "Chii chinonzi Diamond Blackfan Anemia?" 2008.