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ichthyosis

Kuongorora kwe ichthyosis

Ichthyosis iboka remamiriro eganda rinoguma nekuoma, ganda rinoputika rinotaridzika, rakashata, uye rakatsvuka. Zviratidzo zvinogona kubva kune zvinyoro kusvika kune zvakanyanya. Ichthyosis inogona kukanganisa ganda chete, asi mamwe maitiro echirwere anogonawo kukanganisa nhengo dzomukati.

Vanhu vazhinji vanogara nhaka ichthyosis kubva kuvabereki vavo kuburikidza nejini rakashandurwa (rakashandurwa). Zvisinei, vamwe vanhu vanogadzira chimiro chekuwana (kwete-genetic) ichthyosis nekuda kweimwe mamiriro ehutano kana mamwe mishonga. Nepo pari zvino pasina mushonga weichthyosis, kutsvagisa kuri kuenderera mberi uye marapiro aripo kubatsira kubata zviratidzo.

Maonero evanhu vane ichthyosis anosiyana zvichienderana nerudzi rwechirwere uye kuoma kwayo. Vanhu vazhinji vane ichthyosis vanoda kurapwa kwehupenyu hwose kuti chirwere chacho chigone kugadziriswa.

Ndiani anowana ichthyosis? 

Munhu wose anogona kuwana ichthyosis. Chirwere chacho chinowanzoparadzirwa kubva kuvabereki; zvisinei, vamwe vanhu vanogona kunge vari vekutanga mumhuri kukudziridza ichthyosis nekuda kwekuchinja kwemajini matsva. Vamwe vanhu vanogadzira yakawanikwa (isina-genetic) fomu yeichthyosis, inoguma kubva kune chimwe chirwere kana zvinodhaka zvinodhaka.

Mhando dze ichthyosis

Pane zviratidzo zve20 zve ichthyosis, kusanganisira izvo zvinoitika sechikamu cheimwe syndrome kana mamiriro. Vanachiremba vanogona kuona rudzi rwe ichthyosis nekutsvaga:

  • Gene mutation.
  • Nhaka muenzaniso nekuongorora miti yemhuri.
  • Zviratidzo, kusanganisira kuoma kwavo uye nhengo dzipi dzavanobata.
  • Zera apo zviratidzo zvakatanga kuonekwa.

Mamwe marudzi echirwere anogarwa nhaka uye asiri chikamu chesyndrome anosanganisira zvinotevera:

  • Ichthyosis vulgaris ndiyo yakajairika mhando. Inowanzoenderera muchimiro chinyoro uye inozviratidza mugore rokutanga rehupenyu neganda rakaoma, rinoputika.
  • Harlequin ichthyosis inowanzoonekwa pakuzvarwa uye inokonzera gobvu, makwapa eganda kuvhara muviri wese. Ichi chimiro chechirwere chinogona kukanganisa chimiro chezviso zvechiso uye kuderedza kufamba kwemajoini.
  • Epidermolytic ichthyosis iripo kubva pakuzvarwa. Vana vakawanda vanozvarwa vaine ganda risina kusimba uye matuzu akavhara miviri yavo. Nokufamba kwenguva, mablister anonyangarika, uye peeling inooneka paganda. Inogona kunge iine mbabvu inoratidzika panzvimbo dzemuviri dzakakombama.
  • Lamellar ichthyosis iripo kubva pakuzvarwa. Mwana anozvarwa ane membrane yakaoma, inoonekera inovhara muviri wese inonzi collodion membrane. Mumavhiki mashoma, membrane inobhururuka, uye mazenji makuru akasviba eramellar anooneka pane mazhinji emuviri.
  • Congenital ichthyosiform erythroderma iripo pakuzvarwa. Vacheche zvakare vanowanzova necollodion membrane.
  • X-yakabatana ichthyosis inowanzokura muvakomana uye inotanga kutenderera mwedzi mitatu kusvika kumitanhatu yezera. Peeling inowanzoitika pamutsipa, pasi pechiso, torso, uye makumbo, uye zviratidzo zvinogona kuwedzera nekufamba kwenguva.
  • Variable erythrokeratoderma inowanzokura mwedzi yakati wandei mushure mekuzvarwa uye inofambira mberi muhuduku. Zvigamba zvakakasharara, zvakakora, kana zvitsvuku zvinogona kuoneka paganda, kazhinji kumeso, magaro, kana kumucheto. Nzvimbo dzakakanganiswa dzinogona kupararira kune ganda nekufamba kwenguva.
  • Progressive symmetric erythrokeratoderma inowanzo ratidza muhudiki ganda rakaoma, dzvuku, rine makwapa rinonyanya kumucheto, matako, kumeso, zviziso zvemakumbo, uye maoko.

Zviratidzo zve ichthyosis

Zviratidzo zveichthyosis zvinogona kubva kune zvinyoro kusvika kune zvakanyanya. Zviratidzo zvinonyanya zvinosanganisira:

  • Dry skin.
  • Itching.
  • Kutsvuka kweganda.
  • Kutsemuka kweganda.
  • Zviyero paganda chena, grey kana brown ruvara zvine zvinotevera kutaridzika:
    • Diki uye crumbly.
    • Zviyero zvakakura, dema, lamellar.
    • Zviyero zvakaoma, zvakafanana nenhumbi dzokurwa.

Zvichienderana nerudzi rweichthyosis, zvimwe zviratidzo zvinogona kusanganisira:

  • Matuzu anogona kuputika, zvichikonzera maronda.
  • Kupera kwebvudzi kana brittleness.
  • Maziso akaoma uye kunetseka kuvhara maziso. Kusagona kudikitira (dikitira) nekuti ganda rinoputika rinovhara matinji edikita.
  • Matambudziko ekunzwa.
  • Kukora kweganda pazvanza nepasi petsoka.
  • Kusimbisa ganda.
  • Kutadza kupeta mamwe majoini.
  • Vhura maronda ekukwenya ganda rinokwenya.

Chikonzero che ichthyosis

Gene mutations (shanduko) inokonzera marudzi ose enhaka yechthyosis. Mazhinji ekuchinja kwemajini akaonekwa, uye chimiro chenhaka chinoenderana nerudzi rweichthyosis. Vanhu vari kugara vachikura ganda idzva uye vachibvisa ganda rekare muhupenyu hwavo hwose. Muvanhu vane ichthyosis, majini akachinja anochinja kukura kwakajairika uye kutenderera kweganda, zvichiita kuti masero eganda aite chimwe chezvinotevera:

  • Vanokura nekukurumidza kupfuura zvavanodonha.
  • Dzinokura pamwero wakajairika asi dzinodurura zvishoma nezvishoma.
  • Vanodurura nekukurumidza kupfuura zvavanokura.

Kune marudzi akasiyana enhaka ye ichthyosis, kusanganisira:

  • Inotonga, zvinoreva kuti iwe unogara nhaka imwe yakajairwa kopi uye imwe yakashandurwa kopi yejini rinokonzera ichthyosis. 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, izvo zvinoreva kuti vabereki vako havana zviratidzo zveichthyosis, asi vabereki vose vanotakura geni rimwe chete risina kukwana, iro risina kukwana kukonzera chirwere. Kana vabereki vese vakatakura geni rimwe chete recessive, pane mukana we25% (1 mu4) panhumbu yega yega yekuva nemwana achagara nhaka ese ari maviri emajini akachinja uye kuita chirwere. Pane mukana we50% (2 kubva pa4) wepamuviri kuti ave nemwana anongogara nhaka imwe chete mutated recessive gene, zvichiita kuti ave mutakuri wechirwere chechirwere pasina zviratidzo zvinoonekwa. Kana mumwe mubereki ane recessive form of ichthyosis ine maviri mutated genes, vana vavo vose vachatakura rimwe jena risina kujairika asi kazhinji havazove nezviratidzo zvinoonekwa zveichthyosis.
  • X-yakabatana, zvinoreva kuti jini shanduko dziri paX sex chromosome. Munhu wese ane machromosome maviri epabonde: vakadzi vanowanzova maviri X chromosomes (XX) uye varume kazhinji vane imwe X chromosome uye imwe Y chromosome (XY). Amai vanogara vachipfuudza X chromosome, nepo baba vachigona kupfuudza X kana Y chromosome. Nzira yenhaka ye X-yakabatana ichthyosis inowanzorevesa; izvi zvinoreva kuti vanhurume vakatanga vaine X chromosome imwe chete vanopfuura paX chromosome yakachinja. Nekuda kwemaitiro aya, vakadzi vanowanzobatwa, uye vanowanzova neiyo yakachinja uye imwe yakajairika X chromosome.
  • Zvinongoerekana zvaitika, zvinoreva kuti gene mutation inoitika zvisina tsarukano pasina nhoroondo yemhuri yechirwere.