Epidermolysis bullosa

Kuongorora kweEpidermolysis Bullosa

Epidermolysis bullosa iboka remamiriro ezvinhu asingawanzoitiki umo ganda rinova brittle uye nyore nyore kuputika. Misodzi, maronda, uye mapundu paganda zvinoitika kana chimwe chinhu chichikwesha kana kurova ganda. Anogona kuonekwa chero pamuviri. Muzviitiko zvakakomba, mablister anogonawo kuumba mukati memuviri, senge mumuromo, esophagus, mudumbu, matumbu, ekumusoro kufema turakiti, dundira, uye genital.

Vanhu vazhinji vane epidermolysis bullosa vanogara nhaka yakashandurwa (yakashandurwa) gene kubva kuvabereki vavo. Iyo gene mutation inoshandura maitiro anoita muviri mapuroteni anobatsira ganda kubatana uye kuramba akasimba. Kana uine epidermolysis bullosa, imwe yemapuroteni aya haina kugadzirwa zvakanaka. Matunhu eganda haawanzo kubatana, zvichiita kuti ganda ribvaruke uye blister nyore.

Chiratidzo chikuru che epidermolysis bullosa iganda risina kusimba rinotungamirira kukuputika uye kubvaruka. Zviratidzo zvechirwere zvinowanzotanga pakuzvarwa kana paucheche uye kubva pakupfava kusvika kune zvakanyanya.

Hakuchina mushonga wechirwere; zvisinei, masayendisiti anoenderera mberi achiongorora kurapa kunogona kuitika kwe epidermolysis bullosa. Chiremba wako anobata zviratidzo, izvo zvinogona kusanganisira kubvisa marwadzo, kurapa maronda anokonzerwa nemapundu nemisodzi, uye kukubatsira kurwisa chirwere.

Ndiani anowana epidermolysis bullosa?

Chero ani zvake anogona kuwana epidermolysis bullosa. Zvinoitika mumarudzi ose nemadzinza uye zvinobata varume nevakadzi zvakaenzana.

Mhando dze epidermolysis bullosa

Kune mhando ina huru dze epidermolysis bullosa. Ganda rine denga repamusoro kana rekunze rinonzi epidermis uye dermis layer iri pasi peepidermis. The basement membrane ndipo panosangana zvidimbu zveganda. Vanachiremba vanosarudza rudzi rwe epidermolysis bullosa zvichienderana nenzvimbo yekuchinja kweganda uye yakaonekwa gene mutation. Mhando dze epidermolysis bullosa dzinosanganisira:

• Epidermolysis bullosa simplex: mablisters anoitika muchikamu chezasi che epidermis.
• Borderline epidermolysis bullosa: Mabhururu anoitika pamusoro pechikamu chepasi membrane nekuda kwezvinetso zvekubatanidza pakati peepidermis uye membrane yepasi.
• Dystrophic epidermolysis bullosa: Mabhururu anoitika mudermis yepamusoro nekuda kwezvinetso zvekubatanidza pakati pe membrane yepasi uye yekumusoro dermis.
• Kindler's syndrome: mablister anoitika muzvikamu zvinoverengeka zveganda, kusanganisira yepasi membrane.

Vatsvakurudzi vakaona zvinopfuura makumi matatu subtypes echirwere, izvo zvakabatanidzwa mumhando ina huru dze epidermolysis bullosa. Nekuziva zvakawanda nezve subtypes, vanachiremba vanogona kutarisa pakurapa chirwere.  

Mhando yechishanu yechirwere, yakawana epidermolysis bullosa, chirwere chisingawanzoitiki chemuviri umo masoja ekudzivirira emuviri anorwisa rudzi rwakati rwe collagen muganda remunhu. Dzimwe nguva izvi zvinoitika nechimwe chirwere, chakadai sechirwere chinoputika. Kashoma kuti mushonga ukonzere chirwere. Kusiyana nemamwe marudzi epidermolysis bullosa, zviratidzo zvinogona kuonekwa chero zera, asi vanhu vazhinji vanoita zviratidzo mukati mezera repakati.

Epidermolysis bullosa zviratidzo

Epidermolysis bullosa zviratidzo zvinosiyana zvichienderana nerudzi rwe epidermolysis bullosa. Munhu wese ane chirwere ichi ane ganda risina kusimba rinobuda mablister uye misodzi zviri nyore. Zvimwe zviratidzo, nemhando uye subtype, zvinosanganisira zvinotevera.

• Epidermolysis Bullosa Simplex ndiyo inowanzozivikanwa yechirwere. Vanhu vane mild subtype vanoita mablister pazvanza zvemaoko avo uye pasi petsoka dzavo. Mune mamwe, akanyanya kuomarara subtypes, mablister anoonekwa muviri wese. Zvichienderana ne subtype yechirwere, zvimwe zviratidzo zvinogona kusanganisira:
  • Kukora kweganda pazvanza zvemaoko nepasi petsoka.
  • Yakakasharara, yakakora, kana kushaikwa zvinzwara kana zvigunwe.
  • Mapundu mukati memukanwa.
  • Shanduko mune pigmentation (ruvara) yeganda.
• Bullous nodular epidermolysis kazhinji inorema. Vanhu vane chimiro chakanyanya kuoma vanogona kunge vaine mablister akavhurika kumeso, torso, uye makumbo, izvo zvinogona kutapukirwa kana kukonzera kupera simba kwemvura nekuda kwekurasikirwa kwemvura. Mablister anogona zvakare kukura mumuromo, esophagus, epamusoro kufema turakiti, mudumbu, matumbu, weti system, uye nhengo dzesikarudzi. Zvimwe zviratidzo nezvinetso zvine chokuita nechirwere zvinogona kusanganisira:
  • Yakakasharara uye yakakora kana kushaikwa nzwara nezvigunwe.
  • Kuonekwa kweganda rakatetepa.
  • Matuzu emumusoro kana kurasikirwa nebvudzi ane mavanga.
  • Kushaikwa kwezvokudya zvinovaka muviri kunokonzerwa nokusadya zvakakwana macalories nemavhitamini nekuda kwekuputika kwemukanwa uye nemudumbu. 
  • Anemia
  • Kunonoka kukura kwese.
  • Kusagadzirwa zvakanaka mazino enamel.
• Bullous dystrophic epidermolysis ane zviratidzo zvishoma zvakasiyana, zvichienderana nokuti chirwere chacho chiri kutonga kana kuti chinowedzera; zvisinei, vanhu vazhinji vane recessive subtype.
  • Recessive subtype: Zviratidzo zvinotangira kunyoro kusvika kune zvakanyanya uye zvinogona kusanganisira:
    • Mablisters anowanzoonekwa panzvimbo huru dzemuviri; mune zvimwe zviitiko zvakapfava, mablisters anogona kuoneka patsoka, magokora, uye mabvi chete.
    • Kurasika kwezvipikiri kana zvipikiri zvakakasharara kana gobvu.
    • Mavanga eganda, izvo zvinogona kuita kuti ganda rive gobvu kana kutetepa.
    • Milia mapundu machena madiki paganda.
    • Itching.
    • Anemia
    • Kunonoka kukura kwese.

Zvimiro zvakakomba zve recessive subtype zvinogona kukonzera kubatanidzwa kweziso, kurasikirwa kwemazino, kuputika kwemuromo uye gastrointestinal turakiti, uye kusanganiswa kweminwe kana zvigunwe. Ngozi yekubatwa negomarara reganda yakakurawo. Gomarara iri rinowanzokura nekupararira nekukurumidza muvanhu vane epidermolysis bullosa pane vanhu vasina chirwere ichi.

• • Dominant subtype: Zviratidzo zvinogona kusanganisira:
    • Matuzu chete pamaoko, makumbo, magokora nemabvi.
    • Kushandura chimiro chezvipikiri kana kudonha kunze kwezvipikiri.
    • Milia.
    • Mapundu mukati memukanwa.

• Kindler syndrome haina subtypes, uye mablister anogona kuumba muzvikamu zvese zveganda. Mapundu acho anowanzoonekwa pamaoko nemakumbo uye, muzviitiko zvakanyanya, anopararira kune dzimwe nhengo dzemuviri, kusanganisira gurokuro uye dundira. Zvimwe zviratidzo zvinosanganisira ganda ritete, rakaunyana; scarring; milium; uye kunzwa kweganda kuchiedza chezuva.

Zvikonzero zve epidermolysis bullosa

Kuchinja (kuchinja) mumajini anogarwa nhaka kubva kuvabereki kunokonzera akawanda epidermolysis bullosa. Majini anotakura mashoko anoratidza kuti unhu hupi hunopfuudzwa kwauri kubva kuvabereki vako. Tine makopi maviri eakawanda emajini edu, imwe kubva kumubereki wega wega. Vanhu vane chirwere ichi vane jini rimwe chete kana anopfuura anotakura mirairo isiriyo yekugadzira mamwe mapuroteni muganda.

Kune marudzi maviri emhando dzenhaka:

• Inotonga, zvinoreva kuti iwe unogara nhaka imwe yakajairika kopi uye imwe kopi yejini rinokonzera epidermolysis bullosa. Iyo isina kujairika kopi yejini yakasimba kana "inotonga" iyo yakajairika kopi yejini, ichikonzera chirwere. Munhu ane shanduko yakakura ane mukana we50% (1 mu2) wekupfuudza chirwere ichi kune mumwe nemumwe wevana vake.
• Recessive, zvinoreva kuti vabereki vako havana mamiriro acho, asi vabereki vese vane geni risiri iro rinokonzera epidermolysis bullosa. Kana vabereki vese vakatakura majini akarembera, pane 25% (1 mu4) mukana wekuve nemwana ane mamiriro ekuzvitakura kwega kwega. Pane mukana we50% (2 kubva pa4) wepamuviri kuva nemwana anogara nhaka yegeni rimwechete risiri renguva dzose, richiita mutakuri. Kana mumwe mubereki ane recessive gene mutation, vana vavo vose vachatakura geni risina kujairika, asi hazvirevi hazvo kuti vane epidermolysis bullosa.

Vatsvakurudzi vanoziva kuti epidermolysis bullosa inowanikwa chirwere che autoimmune, asi havazivi kuti chii chinoita kuti muviri urwise collagen muganda remunhu. Dzimwe nguva, vanhu vane autoimmune kuzvimba bowel chirwere zvakare vanogadzira vakawana epidermolysis bullosa. Muzviitiko zvisingawanzoitiki, zvinodhaka zvinokonzera chirwere.