Epidermolysis bullosa (EB) ndi dzina la gulu la zowawa zapakhungu zomwe zimapangitsa khungu kukhala lolimba kwambiri ndikung'ambika kapena chithuza pakukhudza pang'ono.Dzina limachokera ku 'epiderm'- khungu lakunja,'lysis' - kuwonongeka kwa maselo ndi 'bullosa'- matuza.
Pali zambiri mitundu yosiyanasiyana ya EB, zonse zomwe zimayikidwa pansi pa mitundu inayi ikuluikulu kuyambira yofatsa, yomwe manja ndi mapazi okha amakhudzidwa, mpaka zovuta kwambiri, zomwe zingakhale ndi zotsatira zowononga pa mbali iliyonse ya thupi yomwe imayambitsa kulemala ndi kupweteka kwa moyo wonse. Pazovuta kwambiri EB ikhoza kupha mwachisoni.
EB sipatsirana ndipo singapatsidwe kudzera mu kukhudzana.
Makanema athu akuwonetsa momwe moyo ungakhalire kwa anthu omwe ali ndi EB komanso amagawana mwayi womwe ulipo woti apititse patsogolo moyo wabwino kudzera mukugwiritsa ntchito mankhwala osokoneza bongo.
nkhani;
Kodi anthu amapeza bwanji EB?
EB imachokera ku jini yolakwika (gene mutation), zomwe zikutanthauza kuti khungu silingagwirizane, kotero kuvulala kulikonse kapena kukangana kungayambitse matuza opweteka, omwe amachititsa mabala otseguka ndi mabala. Zingakhudzenso zitsulo zamkati ndi ziwalo.
Munthu aliyense ali ndi makope awiri a jini iliyonse - imodzi kuchokera kwa kholo lililonse. Jini lililonse limapangidwa ndi DNA, yomwe ili ndi malangizo opangira mapuloteni ofunika kwambiri, omwe ena amamanga khungu lathu pamodzi. Kusintha kwa ma gene (ma jini olakwika) ndikusintha kosatha mumayendedwe a DNA. Mu EB, jini yolakwika imatanthawuza kuti malo okhudzidwawo alibe mapuloteni ofunikira omwe amamangiriza khungu pamodzi, zomwe zimapangitsa kuti zisawonongeke mosavuta.
EB ikhoza kukhala wolamulira kapena wopondereza, kutengera ngati jini imodzi kapena zonse ziwiri zili ndi zolakwika.
Ma jini olakwika ndi ma protein omwe akusowa amatha kuchitika m'magulu osiyanasiyana pakhungu, zomwe zimapangitsa mtundu wa EB.
Mitundu yosiyanasiyana ya EB
Pakalipano pali mitundu ingapo yamitundu yosiyanasiyana ya EB, yokhala ndi majini angapo omwe amadziwika kuti ali ndi magawo awa. Ma subtypes awa akugwera pansi pano mitundu inayi ikuluikulu ya EB kudziwika molingana ndi malo a jini yolakwika ndi mapuloteni omwe akusowa mkati mwa zigawo zosiyanasiyana za khungu.
Dinani pansipa kuti mumvetsetse zizindikiro ndi mawonekedwe amtundu uliwonse.
EB simplex (EBS)
Mtundu wofala kwambiri komanso wofatsa kwambiri wa EB pomwe puloteni yomwe ikusowa ndi kufooka kumachitika kumtunda kwa khungu - epidermis. 70% ya milandu yonse ya EB ndi EB Simplex.
Itha kukhala yofatsa kapena yolimba (yolamulira kapena yochulukirapo). Mapuloteni omwe akusowa ndi kufooka kumachitika pansi pa nembanemba yapansi mkati mwa dermis yowonekera. 25% ya milandu yonse ya EB ndi Dystrophic EB.
Junctional EB (JEB)
A osowa zolimbitsa-oopsa mawonekedwe a EB, kumene kusowa zomanga thupi ndi fragility zimachitika ndi dongosolo amasunga epidermis ndi dermis zigawo pamodzi - chapansi nembanemba. 5% ya milandu yonse ya EB ndi Junctional EB.
Kindler EB (KEB)
Amatchulidwa motero chifukwa cha jini yolakwika yomwe imayang'anira chidziwitso chofunikira kupanga mapuloteni a Kindlin1. Mtundu uwu wa EB ndi wosowa kwambiri koma fragility ikhoza kuchitika pamagulu angapo a khungu.
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Zizindikiro za EB
Zizindikiro zimatha kukhala zosiyanasiyana ndipo zimatha kukhala zofatsa mpaka zovuta kutengera mtundu wa EB. Zizindikiro zodziwika bwino ndi:
- kukhudza pang'ono kungayambitse kung'ambika kowawa ndi matuza pakhungu
- kuchiritsa kwa matuza kungayambitse kupweteka, kuyabwa kwambiri ndi mabala
- mu mitundu yocheperako ya EB, matuza amatha kuchitika makamaka m'manja ndi kumapazi kumayambitsa zovuta zakuyenda ndi zochitika zina zatsiku ndi tsiku.
- kufalikira kwa matuza pazovuta kwambiri kungapangitse khungu kukhala pachiwopsezo chotenga matenda ndipo zipsera zambiri zimatha kuchitika
- zikavuta kwambiri matuza amatha kuchitika m'thupi lonse komanso ngakhale ziwalo zamkati. Angatanthauzenso chiopsezo chachikulu chokhala ndi khansa yapakhungu
- vuto lalikulu lomwe anthu omwe ali ndi EB amakumana nawo tsiku ndi tsiku ndi kupweteka ndi kuyabwa zomwe zimachitika chifukwa cha kuchepa kwa magazi.
Kodi EB imadziwika bwanji?
Kuzindikira kwa labotale ndikofunikira kuti muzindikire ndikupeza jini yolakwika, yomwe ingatsimikizire mtundu wa EB. Kusanthula kwachitsanzo cha khungu kumatha kuchitidwa koyambirira ndipo nthawi zambiri ndi gawo loyamba la makanda obadwa kumene. Kuyezetsa mimba kumathekanso.
Magulu a Neo-natal, GPs, Dermatologists kapena EB akatswiri azachipatala azitha kulangiza njira yodziwira yomwe ili yoyenera kwambiri pazochitika zapayekha. Mutha kuwerenga zambiri pa njira zosiyanasiyana za matenda apa.
Ngati inu kapena wachibale mwapezeka ndi EB posachedwa, titha kukuthandizani kuti muthandizidwe, chonde lumikizanani ndi Gulu Lathu Lothandizira Anthu.
Dominant ndi Recessive anafotokoza
EB ikhoza kutengedwa ngati yolamulira (mtundu umodzi wokha wa jini ndi wolakwika) kapena wochulukirapo (makopi onse a jini ndi olakwika).
- In wamkulu EB, kope limodzi la jini limachokera kwa kholo limodzi, kutanthauza kuti kope lina la jini lomwelo kuchokera kwa kholo lina ndi lachibadwa. Kholo lomwe limanyamula jini nthawi zambiri limakhudzidwa ndi momwe zimakhalira ndipo pali mwayi wa 50% wopatsira ana. Mitundu yodziwika bwino ya EB nthawi zambiri imakhala yofatsa kuposa mitundu yokhazikika.
- Recessive EB ndi pamene makope awiri a jini yofanana amatengera - imodzi kuchokera kwa kholo lililonse. Mwayi wopanga recessive EB ndi 25%. Kubadwa kwa mwana wokhala ndi mawonekedwe ochulukirapo nthawi zambiri kumakhala kosayembekezereka chifukwa makolo onse amatha kunyamula jini ya EB popanda kudziwonetsa okha. Recessive EB nthawi zambiri imakhala yovuta kwambiri.
- EB imathanso kubwera kudzera mu masinthidwe osintha - palibe kholo lomwe limanyamula EB koma jini imasintha zokha mu umuna kapena dzira musanatenge mimba.
- Kawirikawiri, mtundu woopsa wa EB ukhoza "kupezedwa" chifukwa cha matenda a autoimmune, kumene thupi limapanga ma antibodies kuti awononge mapuloteni ake a minofu.
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Kodi EB imathandizidwa bwanji?
Pakali pano palibe mankhwala odziwika a EB koma pali mankhwala omwe cholinga chake ndi kuthandiza kuchepetsa zina mwazofowoketsazo. Ku DEBRA UK, ntchito yathu ikuyang'ana pa kufufuza kwa ndalama kuti tipeze mankhwala atsopano ndi machiritso, komanso kupereka chithandizo kwa anthu omwe ali ndi EB kuti apititse patsogolo moyo wawo mwa kupititsa patsogolo chithandizo chamankhwala, kupeza chidziwitso ndi chisamaliro chapadera. Dziwani zambiri za zosankha zamankhwala, momwe timathandizira gulu la EB ndi ntchito zofufuza zomwe tikupereka ndalama.
Kodi kukhala ndi EB kumakhala bwanji?
Mukakhala ndi EB, zinthu zambiri zimakhala zoletsedwa. Muyenera kuganizira chilichonse chomwe mumachita. Ana ena sayenera kuchita zimenezo.
Fazeel, wokhala ndi DEB
Kodi ndiyenera kuchita chiyani ngati ndikuganiza kuti ndili ndi EB?
Ngati mukukayikira kuti muli ndi mtundu uliwonse wa EB, mutha kukaonana ndi GP wakudera lanu, ngati akuganizanso kuti mutha kukhala ndi mawonekedwe a EB ndiye akulozerani kwa mmodzi wa
EB akatswiri malo. Gulu lachipatala ku EB center lidzazindikira momwe khungu lanu lilili ndipo adzakonza (ndi chilolezo chanu) kuti ayese chibadwa kuti atsimikizire ngati muli ndi mtundu uliwonse wa EB. Ngati EB yatsimikiziridwa, gulu lachipatala la EB lidzagwira ntchito nanu kuti mudziwe dongosolo lachipatala. Mukhozanso kupeza thandizo kuchokera ku
DEBRA EB Community Support Team.
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