Junctional epidermolysis bullosa (JEB)
Junctional EB (JEB) ndi imodzi mwa mitundu inayi ikuluikulu ya epidermolysis bullosa (EB), matenda opweteka a majini omwe amachititsa kuti khungu lizing'ambika kapena kuphulika pang'ono. Mitundu inayi ikuluikulu ya EB imayamba chifukwa cha kusintha kwa majini, komwe kumabweretsa mapuloteni olakwika kapena osowa m'magawo osiyanasiyana a khungu komanso nthawi zina ziwalo zamkati.
JEB ndi mtundu wosowa kwambiri wa EB womwe umakhudza nembanemba yapansi, yomwe ndi kapangidwe kamene kamapangitsa kuti epidermis (kunja) ndi dermis zizikhala palimodzi, kutanthauza kuti khungu limasweka mosavuta kupangitsa matuza.
Pafupi ndi EB (JEB)
Munthu aliyense ali ndi makope awiri a jini iliyonse, kusintha kwa jini komwe kumayambitsa EB kungakhale mumtundu umodzi kapena onse awiri. Komabe, JEB imatengera cholowa, kutanthauza kuti majini onse awiri - imodzi kuchokera kwa kholo lililonse - imakhudzidwa.
Recessive EB nthawi zambiri imakhala yowopsa kuposa mitundu yayikulu ndipo imatha kudabwitsa kwambiri chifukwa makolo amatha kunyamula popanda kuwonetsa okha. 5% ya milandu yonse ya EB ndi JEB.
Dziwani zambiri za kusiyana pakati pa EB yolamulira ndi yobwerera m'mbuyo.
Pali mitundu iwiri ikuluikulu ya JEB yokhala ndi zizindikiro ndi zotsatira zosiyanasiyana. Matuza amawonekera pobadwa kapena atangobadwa kumene ndipo amatha kukhudza thupi lonse.
- JEB wapakatikati (yomwe poyamba inkadziwika kuti JEB Generalized Intermediate kapena Non-Herlitz JEB). Uwu ndi mtundu wocheperako kwambiri wa ziwiri zomwe matuza amatha kungokhala m'manja, m'zigongono ndi kumapazi. Alopecia (kutha tsitsi), chala cholakwika ndi zikhadabo ndi enamel ya mano osakhazikika zitha kuwonekera. A yachibadwa moyo n'zotheka ndi osiyanasiyana mankhwala zilipo kuti zithandize ndi ululu ndi kuyabwa.
- Kwambiri JEB (yomwe poyamba inkadziwika kuti JEB Generalized Severe kapena Herlitz JEB) ikhoza kukhala yoopsa kwambiri ndipo nthawi zambiri imapha munthu akamakhanda chifukwa cha zovuta zomwe zimadza chifukwa cha matuza aakulu otere thupi lonse ndi ziwalo zamkati, zomwe zimapangitsa kuti zikhale zovuta kuti makanda adye ndi kugaya chakudya.
Pakadali pano palibe mankhwala a EB, ntchito yathu ku DEBRA ikufuna kusintha izi. Komabe, pali mankhwala omwe alipo omwe amathandiza kuthana ndi ululu ndi kuyabwa . Timathandizira mapulojekiti ofufuza omwe cholinga chake ndi kupeza mankhwala ena komanso mankhwala, ndipo Gulu lathu Lothandizira Anthu a EB ladzipereka kuthandiza odwala ndi mabanja kuthana ndi mavuto omwe EB imabweretsa.
Ngati inu kapena wachibale wanu mwapezeka ndi JEB, mutha kulumikizana ndi Gulu Lathu Lothandizira Anthu Amdera kuti mupeze chithandizo china. Gulu lathu likufuna kuthandiza anthu onse a EB mosasamala kanthu za mtundu kapena kuopsa kwa matendawa, tili ndi njira zosiyanasiyana zothandizira, zamaganizo komanso zachuma.
Ngati muli ndi JEB, mutha kugwiritsa ntchito khadi lathu la 'Ndili ndi JEB'. Izi zimapangitsa kuti zikhale zosavuta kugawana ndi anthu mfundo zazikuluzikulu za mtundu wa EB wanu, monga akatswiri azachipatala ngati mukupita kukakumana.
Pali mtundu wa digito wa khadi womwe mungathe kutsitsa apa ndikusunga ngati chithunzi pafoni yanu.
Ngati mukufuna khadi yokhala ndi chikwangwani cha katundu, chonde lankhulani ndi gulu lathu la Umembala kuti mupemphe imodzi.