Kawirikawiri Amakhudza Ana
Mu Blackfan Diamond (kapena Diamond Blackfan) kuchepa kwa magazi, thupi la fupa limapanga maselo ofiira aang'ono kapena opanda magazi. Mankhwala a Blackfan Diamond amakhudza anthu pafupifupi 600 mpaka 700 padziko lonse lapansi. Chifukwa chake sichikudziwika, ngakhale kuti cholakwika cha majeremusi mu jini yotchedwa RPS19 pa chromosome 19 ikugwirizana ndi pafupifupi 25% mwa milandu. Pa 10% mpaka 20 peresenti ya milandu, pali mbiri ya banja ya matendawa.
Zizindikiro
Mankhwala a Blackfan Diamond alipo pomwe anabadwa koma akhoza kukhala ovuta kuzindikira. Pafupifupi theka la ana obadwa ndi matendawa, pali zofooka zakuthupi monga zopweteka manja kapena zofooka za mtima, koma zizindikiro zomveka sizidziwika. Zizindikiro zingakhalenso zosiyana kwambiri, kuyambira zofatsa mpaka zoopsa komanso zowopsya.
Maselo ofiira amanyamula mpweya m'thupi, kotero mwana yemwe ali ndi Blackfan Diamond akhoza kukhala ndi zizindikiro zokhudzana ndi magazi osaperewera.
- zovuta (kupweteka)
- kupsa mtima kwapadera, chifukwa cha mtima kuyesera kuti mpweya uziyenda mthupi lonse
- kutopa, kukwiya, ndi kutaya .
Kudziwa
Blackfan Diamond magazi amapezeka kawirikawiri m'zaka ziwiri zoyambirira za moyo, nthawi zina ngakhale atabadwa, pogwiritsa ntchito zizindikiro. Mwachitsanzo, mwana angaganize kuti ali ndi vuto la kuperewera kwa magazi ngati nthawi zonse amakhala wotumbululuka ndipo amatha kupuma pang'ono akamamwa botolo kapena namwino.
Nthawi zambiri makolo amakayikira kuti pali "cholakwika" ndi mwana wawo. Kuzindikira kwa Blackfan Diamond magazi, makamaka, sikungadziwike pomwepo, chifukwa matendawa ndi osowa kwambiri ndipo madokotala ochepa amadziwa bwino.
Kuwerengera kwathunthu kwa maselo a magazi (CBC) kwa mwanayo kungasonyeze chiwerengero chochepa kwambiri cha maselo ofiira a magazi komanso hemoglobin yapamtunda.
Mayeso ena a magazi angasonyeze ntchito yotchuka ya adenosine deaminase (ADA). Chitsanzo cha mkaka wa fupa (biopsy) chikanawonetsa kuti maselo ofiira atsopano a magazi apangidwa.
Chithandizo
Mzere woyamba wa chithandizo cha magazi a Blackfan Diamond ndi kupereka mankhwala a mwana steroid, kawirikawiri prednisone. Pafupifupi 70 peresenti ya ana omwe ali ndi magazi a Blackfan Diamond adzalandira mankhwalawa, omwe mankhwalawa amachititsa kupanga maselo ofiira ambiri. Komabe, izi zikutanthauza kuti mwanayo adzalandira mankhwala a steroid kwa moyo wake wonse, omwe ali ndi zotsatira zoyipa monga shuga , glaucoma , fupa lofooka ( osteopenia ), ndi kuthamanga kwa magazi . Komanso mankhwalawa amatha kugwira ntchito kwa munthu nthawi iliyonse.
Ngati wina sakuyankha mankhwala a steroid kapena amafunikira mlingo waukulu kuti magazi ake asapitirire, mankhwalawo amayamba kuikidwa magazi. Kuikidwa magazi nthaŵi zonse kumapereka maselo ofiira a magazi komanso kumabweretsa chitsulo chochuluka m'thupi . Kawirikawiri, thupi limagwiritsa ntchito chitsulo popanga maselo ofiira atsopano, koma popeza munthu yemwe ali ndi magazi a Blackfan Diamond sakupanga maselo ambiri, chitsulo chimamangirira. Munthuyo amafunika kumwa mankhwala omwe amachotsa zitsulo zochulukira kunja kwa thupi.
Chithandizo chokha chopezeka ku Blackfan Diamond ndi kuchepa kwa mafupa a mafupa , komwe kumalowetsa mafupa osakwanira. Komabe, kuziika ndizovuta kuti zichitike ndipo sizigwira ntchito nthawi zonse. Kaŵirikaŵiri zimasungidwa kwa anthu omwe mankhwala ochizira steroid ndi kuikidwa magazi samathandiza.
> Zotsatira:
> Guinan, EC. "Kusanthula ndi Kulamulira kwa Mapuloteni a Plastiki." ASH Education Book , 2011, 76-81.
> Bungwe la National for Disturbances Rare. "Blackfan Diamond Anemia." Mndandanda wa Zachilombo Zambiri, 2008.
> UK Diamond Blackfan Anemia Support Group. "Kodi Diamond Blackfan Anemia ndi chiyani?" 2008.