Chirwere chechirwere chinokonzera vana
Chirwere cheBatten chirwere chisingawanzoiti uye chinouraya chinokanganisa hurongwa hwehutano. Vana vakawanda vanotanga kuratidzira zviratidzo pakati pemakore mashanu nemakore gumi, apo mwana akambova nehutano angave atanga kuratidzira zviratidzo zvekugumburwa kana nyaya dzekuona. Muzviitiko zvizhinji, zviratidzo zvepakutanga zvakanyanyisa, zvakadai sekushungurudzika, kudzidzira kuora mwoyo, uye kuipa kwekuona.
Vanhu vazhinji vane zvirwere zveBatten vanofa mumakore avo ekuyaruka kana kumashure makumi maviri.
Chirwere cheBatten ndicho chinowanzova neuronal ceroid lipofuscinoses (NCLs). Pakutanga, zvirwere zveBatten zvaifungidzirwa sevana vechidiki NCL, asi mumakore achangopfuura, vanachiremba vakashandisa chirwere cheBatten kutsanangura maitiro akawanda eCLL.
Chirwere cheBatten hachina chakanaka. Inofungidzirwa kugadzirisa 1 mumarudzi ose ekuberekwa 50,000 muUnited States. Kunyange zvazvo zviitiko zvichiitika munyika yose, zvirwere zveBatten zvinowanzoonekwa kune dzimwe nzvimbo dzekumusoro kweEurope, dzakadai seSweden kana Finland.
Chirwere cheBatten chirwere chekuzvidzivirira zvakanyanya, zvinoreva kuti zvinongowanikwa mumwana kana vabereki vose vakatakura maitiro ehutachiona . Kana mwana ane mubereki mumwe chete ane jeni, mwana uyo anotorwa seanotakura uye anogona kupfuudza jena kune mwana wake, zvichikonzera zvirwere zveBatten kana shamwari yake inotakura gemu zvakare.
Zviratidzo
Sezvo chirwere ichi chichifambira mberi, kurasikirwa kwemasimba, utachiona hwakanyanya hutano, atrophy yeuropi mashutu , kupererwa kwemaziso, uye kuora mwoyo kwekutanga.
Kuziva
Nemhaka yokuti zviratidzo zvekutanga zvechirwere cheBatten zvinowanzobatanidza maonero, zvirwere zveBatten zvinowanzotanga kufungidzirwa panguva yekuongorora kwemaziso. Zvisinei, haikwanise kuongororwa kuburikidza neziso rekuongorora chete.
Chirwere cheBatten chinowanikwa chinobva pane zviratidzo izvo mwana ari kutarisana nazvo. Vabereki kana mwana wevana vana vanogona kuona kuti mwana atanga kukura matambudziko ekuona kana kuparara .
Ongororo dzepamusoro dzemagetsi dzemeso, dzakadaro dzinoonekwa-dzakabviswa kana kuti electroretinogram (ERG), inogona kuitwa. Mukuwedzera, zvirwere zvekuongorora zvirwere zvakadai se electroencephalogram (EEG, kutsvaga kubata basa) uye magnetic resonance imaging (MRI, kutsvaga kuchinja muuropi) inogona kuitwa. Muenzaniso weganda kana mhuka (inonzi biopsy ) inogona kuongororwa pasi microscope kuti iongorore mazpofuscins.
Kurapa
Hapana mishonga chaiyo inowanikwa kurapa kana kuderedza kufambira mberi kwezvirwere zveBatten, asi kurapwa kunovapo kuti urarame zviratidzo uye kunoita kuti mwana ave akasununguka. Kushungurudzwa kunogona kudzorwa nemishonga inorwisa mishonga , uye mamwe matambudziko ezvoutano anogona kurapwa sezvinodiwa. Kurapa kwepanyama uye yebasa kunogona kubatsira murwere kubatirira kumabasa ekuita muviri kwenguva yakareba sezvinobvira pamberi pemisumbu atrophy.
Zvimwe zvidzidzo zvakaratidza dambudziko rekutanga kuti madhii eVitamin C uye E anogona kubatsira kuderedza chirwere, kunyange pasina kurapwa kwakakwanisa kuidzivisa kubva pakuuraya.
Masangano ekutsigira akadai seBatten Disease Support and Research Association anopa rubatsiro uye ruzivo pamusoro pemishonga uye tsvakurudzo. Kusangana nedzimwe mhuri dzakapinda muchimwe chinhu chimwe chete kana dziri kupfuura mumatanho akafanana zvinogona kuva rubatsiro rukuru apo uchitarisana neBatten chirwere.
Chirwere chekurapa chinoramba chichidzidza chirwere chetariro chichiziva zvirwere zvinoshanda munguva yemberi.
Sources:
"Batten Disease". National Institute of Neurological Disorders and Stroke, 2015.
"Chii chinonzi Batten Disease?" Batten Disease Research and Support Association, 2015.