Harlequin ichthyosis: zviratidzo, kuongororwa uye kurapwa

Zvemukati

• Zviratidzo zveHarlequin Ichthyosis
• Maitiro ekuongororwa kunoitwa
• Harlequin Ichthyosis Kurapa
• Pane mushonga here?

Harlequin ichthyosis chirwere chisingawanzoitika uye chakakomba chemumajini chinoratidzirwa nekukora kwechikamu chekeratin chinoumba ganda remwana, zvekuti ganda rakakora uye rine tsika yekukweva nekutambanudza, zvichikonzera kuora kumeso nemuviri wese uye kuunza matambudziko yemwana, senge kunetseka kufema, kufudza uye kutora mimwe mishonga.

Kazhinji, vacheche vanozvarwa vaine harlequin ichthyosis vanofa masvondo mashoma mushure mekuzvarwa kana kupona kusvika pazera ramakore matatu zvakanyanya, nekuti nekuti ganda rine kutsemuka kwakati, basa rekudzivirira reganda rakakanganisika, riine mukana wakakura wekutangazve hutachiona.

Izvo zvinokonzeresa harlequin ichthyosis hazvisati zvanyatsonzwisiswa, asi vabereki vane simba vanowanzoita mwana seizvi. Chirwere ichi hachina mushonga, asi kune nzira dzekurapa dzinobatsira kubvisa zviratidzo uye kuwedzera tarisiro yeupenyu hwemwana.

Zviratidzo zveHarlequin Ichthyosis

Iye achangoberekwa ane harlequin ichthyosis inopa ganda rakafukidzwa neakakora kwazvo, akatsetseka uye opaque plaque iyo inogona kukanganisa mabasa akati wandei. Huru hunhu hwechirwere ichi ndeichi:

• Ganda rakaoma uye rine ganda;
• Zvakaoma mukudyisa nekufema;
• Kutsemuka uye maronda paganda, ayo anofarira kuitika kwezvirwere zvakasiyana siyana;
• Kukanganisa kwenhengo dzechiso, senge maziso, mhino, muromo uye nzeve;
• Kukanganisika kwetachiona;
• Kunyanya kupera mvura uye kukanganiswa kwe electrolyte;
• Kubvisa ganda muviri wese.

Pamusoro pezvo, mukobvu weganda unogona kuvhara nzeve, usingaoneke, pamusoro pekukanganisa minwe nezvigunwe uye piramidhi remhino. Ganda rakakora rinoitawo kuti zviomere kuti mwana afambe, achigara mune yakasununguka-kufamba.

Nekuda kwekuremara kwekudzivirira kweganda, zvinokurudzirwa kuti mwana uyu aendeswe kuNeonatal Intensive Care Unit (ICU Neo) kuti ave nechengeto rwakakosha kuitira kuti pasave nematambudziko. Nzwisisa mashandiro anoita neonatal ICU.

Maitiro ekuongororwa kunoitwa

Kuongororwa kweHarlequin ichthyosis kunogona kuitwa mukutarisirwa kwepamuviri kuburikidza nemiedzo yakadai seultrasound, iyo inogara ichiratidza muromo wakashama, kudzora kwekufema, kushanduka kwemhino, maoko anogara akagadziriswa kana kubvarurwa, kana kuburikidza nekuongororwa kweamniotic fluid kana biopsy. yeganda remucheche iro rinogona kuitwa pamasvondo makumi maviri nemaviri kana makumi maviri ekuberekwa.

Pamusoro pezvo, kurairiswa kwemajini kunogona kuitwa kuitira kuongorora mukana wekuti mwana azvarwe aine chirwere ichi kana vabereki kana hama dzikapa jeni rinokonzera chirwere ichi. Kurairidzwa nezvechibereko kwakakosha kuvabereki nemhuri kuti vanzwisise chirwere ichi pamwe nechengetedzo yavanofanira kutora.

Harlequin Ichthyosis Kurapa

Kurapa kweharlequin ichthyosis kunovavarira kudzikisa kusagadzikana kwemwana achangozvarwa, kubvisa zviratidzo, kudzivirira hutachiona uye kuwedzera hupenyu hwemwana. Iyo yekurapa inofanirwa kuitirwa muchipatara, nekuti kutsemuka uye kusvuuka kweganda kunofarira hutachiona nemabhakitiriya, izvo zvinoita kuti chirwere chiwedzere kutonyanya uye kuomarara.

Mushonga uyu unosanganisira kuyerwa kwemavhitamini A ekugadzirwa kaviri pazuva, kupa musero kuvandudzwa, zvichideredza maronda aripo paganda uye achibvumira kufamba kukuru. Tembiricha yemuviri inofanirwa kuchengetwa ichidzorwa uye ganda riiswe mvura. Kuti unyorove ganda, mvura uye glycerin kana emollients zvinoshandiswa zvega kana zvine chekuita nemafomati ane urea kana ammonia lactate, iyo inofanirwa kuiswa katatu pazuva. Hwisisa kuti marapirwo echthyosis anofanira kuitwa sei.

Pane mushonga here?

Harlequin ichthyosis haina mushonga asi mwana anogona kurapwa mushure mekuzvarwa muIonatal ICU iyo yakanangana nekuderedza kusagadzikana kwake.

Chinangwa chekurapa ndechekudzora tembiricha uye hydrate ganda. Maitiro evhitamini A ekugadzirwa anoshandiswa uye, mune dzimwe nguva, kuvhiya kweganda autograft kunogona kuitwa. Kunyangwe paine kuoma, mushure memazuva anenge gumi vamwe vacheche vakakwanisa kuyamwa, zvisinei kune vana vashoma vanosvika gore rimwe chete rehupenyu.