Uri kuona mavara asinganzwisisike paganda rako here? Zvingave zvichikonzerwa ne (Incontinentia Pigmenti) here?

Wakambonzwa nezvechirwere ichi chinonzi `(Incontinentia Pigmenti)` kana `(IP)` here? Pamwe paunonzwa zita iri, rinogona kunzwika serakaoma uye risinganzwisisike. Asi iyi inyaya inokosha yatinofanira kutaura nezvayo, nekuti chirwere chemajini chisingawanzoitika chinogona kukanganisa vamwe vana, kunyanya vasikana. Saka nhasi tichataura nezvezvinhu zvakawanda zviri nyore, zvakaita sekuti `(Incontinentia Pigmenti)` chii, zviratidzo zvacho ndezvipi, uye kuti chinorapwa sei.

Chii chinonzi `(Incontinentia Pigmenti)`? Ngatizvinzwisise zviri nyore!

Zvichitaurwa zviri nyore, Incontinentia Pigmenti chirwere chegene chinokonzerwa neX-linked dominant. Ungave uchifunga kuti, "Ah, chiremba, handizvinzwisisi izvi." Zvakanaka, ngatizvirerutsei zvakanyanya. Idambudziko rinopfuudzwa kuburikidza nemajini, zvichireva kuti rinopfuudzwa kubva kumubereki kuenda kumwana. Iri muboka rezvirwere zvinonzi neurocutaneous disorders. Izvi zvinoreva kuti mhando idzi dzezvirwere dzinogona kukanganisa sisitimu yedu yetsinga (ndiko kuti, uropi nemusana), ganda, maziso, mazino, uye skeletal system .

Kana paine `(IP)`, shanduko dzakati wandei (kuchinja) mumajini edu dzinokonzera kuoneka zvishoma nezvishoma kwemavanga nemavanga paganda. Ichi ndicho chiratidzo chikuru uye chinowanzoonekwa chechirwere ichi. Mavanga aya paganda anogona kuonekwa pakuzvarwa, uye dzimwe nguva anogona kuonekwa kunyange mavhiki mashoma mushure mekuzvarwa.

Kune marudzi mana emavanga paganda!

Mavara aya eganda anogona kuoneka rimwe nerimwe, muzvikamu zvina. Dzimwe nguva zvikamu izvi zvinogona kuuya zvakarongwa, kana kuti chimwe chikamu chinogona kuoneka mushure mechimwe. Funga nezvazvo semwaka unochinja, asi dzimwe nguva mwaka mumwe unopera usati watanga mumwe.

1. Danho reVesicular: Apa ndipo panoonekwa maronda madiki matsvuku, azere nemvura paganda. Akafanana nemaronda anoonekwa panguva yehuku. Anogona kuonekwa semitsetse mumuviri wese, kunyanya pamakumbo nemaoko.

2. Danho reVerrucal: Nekufamba kwenguva , mavanga akaita semapundu, akasimudzwa, uye akakwidzwa anotanga kuoneka paiva nemapundu. Dzimwe nguva aya anogona kugara kwemwedzi yakati wandei.

3. Danho reHyperpigmentation: Tevere, mavara egrey, ebhuruu-brown anoonekwa paganda. Aya anoonekwa zvisina kurongeka pamuviri nepamakumbo, akafanana nemapatani ane machena kana mitsetse ine mafungu . Mavara aya ndechimwe chezvikonzero nei zita rekuti `(Incontinentia Pigmenti)` rakagadzirwa. "Pigmenti" zvinoreva pigment. Danho iri rinogona kugara kwemakore.

4. Danho rekuchinja ruvara rweganda: Pakupedzisira, mavara matema anotsiviwa nemavanga asina kucheneruka, dzimwe nguva akafanana nemavanga . Aya anogona kugara kweupenyu hwese.

Chinhu chakakosha ndechekuti havasi vese vanozowana nguva dzese ina idzi dzakatevedzana seizvi. Vamwe vanhu vanogona kuona nguva imwe chete pachena, vamwe vachiona dzakawanda panguva imwe chete.

Kazhinji, IP inokonzerwa nekuchinja kwemajini kunonzi NEMO (NF-kappaB essential modulator). Iri jini reNEMO ijini rinokosha rinobatsira kudzora mashandiro emasero mumuviri wedu uye rinobatsirawo mukufa kwemasero kwakarongwa. Zvinosuwisa kuti hapana mushonga chaiwo weIP parizvino.

Ndezvipi zvinetso zvinogona kuitika nekuda kwe `(Incontinentia Pigmenti)`?

IP haisi chirwere cheganda chete. Dzimwe nguva inogona kukonzerawo mamwe matambudziko ehutano. Zvakakosha kuti tizive izvozvo zvakare.

Matambudziko etsinga

• Kupera simba kweuropi.
• Maburi madiki muchinhu chichena chiri pakati peuropi.
• Kuparadzwa kwemasero etsinga mu cerebellar cortex.

Nekuda kwezvikonzero izvi, vanenge 20% yevana vane `(IP)` vanogona kuva nezvirwere zvinotevera:

• Kunonoka kwehunyanzvi hwekufambisa makumbo (semuenzaniso, kunonoka kukambaira, kunonoka kufamba).
• Kuremara kwepfungwa (kwakadai sekuremara pakudzidza).
• Kushaya simba kwetsandanyama.
• Kuva nepfungwa dzakapfava (pfari).

Matambudziko ekuona

Vamwe vana vane `(IP)` vanogonawo kuva nematambudziko ekuona. Semuenzaniso:

• Maziso akapatsanurwa (strabismus).
• Matumbu emusoro.
• Mune zvimwe zviitiko zvakakomba, kurasikirwa zvachose kwekuona kunogona kuitika .

Matambudziko emazino

Matambudziko emazino anogonawo kuonekwa ne `(IP)`.

• Mamwe mazino haasipo.
• Mazino acho akaita se "peg" (kana kuti "peg").
• Kunonoka kubuda kwemeno.

Ndosaka zvichikosha zvikuru kubvunza chiremba wemazino.

Chii chinonzi `(Incontinentia Pigmenti Achromians)`?

Chimwe chirwere chakafanana neichi ndechekuti `(incontinentia pigmenti achromians)`. Muizvi, ganda rinoita makwinya akachenuruka, akachenuruka (hypopigmented) uye mitsetse . Kusiyana nemavanga matema ari mu`(IP)`, chirwere ichi chine mavara akachenuruka chete. Zvisinei, se`(IP)`, chirwere ichi chinogonawo kusanganiswa nematambudziko mutsinga dzemuviri.

`(Incontinentia Pigmenti)` inorapwa sei?

Sezvatakambotaura, hapana mushonga chaiwo we `(IP)` parizvino. Zvisinei, kune nzira dzakasiyana-siyana dzekurapa dziripo kudzora zviratidzo nematambudziko anokonzerwa nechirwere ichi. Kurapa kunoenderana nezviratidzo zvemunhu mumwe nemumwe.

• Mavara eganda: Kazhinji, mavara aya eganda, kunyanya maronda nemavanga akakombama anoonekwa pakutanga, anonyangarika zvishoma nezvishoma pasina kurapwa, kungave panguva yekuyaruka kana yekukura.Mavara iwayo matema (hyperpigmentation) uye mavara machena (hypopigmentation) anogona kugara kwenguva yakareba, dzimwe nguva kweupenyu hwese. Asi kazhinji haakonzeri marwadzo.
• Zviratidzo zvetsinga: Kana uine pfari, kusasimba kwetsandanyama, kana kuputika kwetsandanyama kusingawanzoitiki, chiremba wetsinga anogona kunyora mishonga nezvimwe zvishandiso zvekurapa .
• Matambudziko ekuona: Kana usina kuona zvakanaka, chiremba wako anogona kukukurudzira magirazi, mishonga, kana, kana zvakanyanya, kuvhiyiwa .
• Matambudziko emazino: Nyanzvi dzehutano hwemazino dzinogona kurapa matambudziko emazino. Dzinogona kuita zvinhu zvakaita sekuzadza mazino nemazino ekugadzira mazino.

Hupenyu huchava hwakaita sei kana paine chirwere chinonzi `(Incontinentia Pigmenti)`? (Kufungidzira)

Iri idambudziko revanhu vazhinji. Havasi vese vane IP vanowana matambudziko akakomba. Vanhu vazhinji, kunyanya avo vasingawani matambudziko akakomba vachiri vacheche kana kuti vari vadiki, vanogona kurarama hupenyu hwakanaka uye hwakanaka. Mavara eruvara anoonekwa paganda anowanzopera nekufamba kwenguva, uye dzimwe nguva anonyangarika zvachose.

Zvisinei, vamwe vanhu vanogona kuramba vaine matambudziko etsinga nemuviri. Saka, kana iwe kana mwana wako aine IP, zvakanaka kutaura nachiremba wako kana chipangamazano wezvemajini kuti vasarudze hurongwa hwehutano hwakanakira iwe.

Vatsvagurudzi vari kuita zvidzidzo zvekubatanidza majini kuti vaone kuti majini ane chekuita nezvirwere zvetsinga dzakadai seIP ari kupi. Vari kudzidzawo kuti kuchinja kwemajini uku kunokanganisa sei kukura nekushanda kweuropi nehurongwa hwetsinga. Tsvagiridzo yakaita seiyi ichatibatsira kunzwisisa zvakawanda nezvezvirwere zvemajini uye inogona kugadzira nzira yekurapwa mune ramangwana.

Zvinhu zvakakosha zvekurangarira (Mharidzo Yekuenda Kumba)

• Incontinentia Pigmenti chirwere chisingawanzoitiki, chine majini chinonyanya kukanganisa ganda, asi chinogonawo kukanganisa tsinga, maziso, nemazino.
• Mavara eganda anogona kuoneka muzvikamu zvina. Anowanzo nyangarika nekufamba kwenguva.
• Havasi vese vanowana matambudziko akakomba. Vanhu vazhinji vanogona kurarama hupenyu hwakajairika.
• Kurapwa kunoenderana nezviratidzo. Unogona kutsvaga rubatsiro kubva kune nyanzvi dzezveuropi, dzemaziso, uye dzemazino.
• Kana iwe kana mwana wako muine zvinokunetsa nezvechirwere ichi, tsvagai mazano echiremba nekukasika. Chinhu chakakosha ndechekusatya, kuwana ruzivo rwakakodzera, uye kutsvaga kurapwa kwakakodzera.

Ndinovimba ruzivo urwu ruchakubatsira. Kana uine mibvunzo, usanyara kubvunza chiremba. Tese tiripo kuti tikubatsire.

Incontinentia Pigmenti, IP, zvirwere zveganda, zvirwere zvemajini, jini reNEMO, mavara eganda, zvirwere zvetsinga, zvirwere zvevana