Saamai nababa, tese tinoona mwana wedu achikura, zuva nezuva. Asi, fungidzira... ko kana mwana akakura nekukurumidza kusingafungidzike, kwete nenzira yakajairika? Zvakaoma kufungidzira. Nhasi tiri kutaura nezvechirwere chisingawanzoitiki, asi chakakosha kwazvo chekuziva. Ichi chinonzi Progeria.
Chii chaizvo chinonzi Progeria?
Zvichitaurwa zviri nyore, Progeria chirwere chisingawanzoitiki chinoita kuti miviri yevana ikwegure nekukurumidza. Mwana ane chirwere ichi anotaridzika semwana ane hutano paanozvarwa. Zvisinei, mukati megore rekutanga kana maviri ehupenyu, anotanga kuratidza zviratidzo zvekukwegura uku nekukurumidza. Kukura kwavo kunoderera zvakanyanya, uye vanowana uremu zvishoma nezvishoma.
Asi chinonyanya kukosha ndechekuti, hapana kushaikwa kwenjere muvana ava. Vane hungwaru hwakanyanya. Dambudziko riri pakuchinja kwemuviri kunoitika nekuda kwekukurumidzira kwekukwegura mumuviri.
Zita rekuti "progeria" rinobva pashoko rechiGiriki rekuti "geras" rinoreva "kukwegura." Rudzi rukuru rwechirwere ichi runonzi Hutchinson-Gilford progeria syndrome (HGPS).
Iyi inyaya inosuwisa zvikuru, nekuti hapana mushonga weProgeria. Avhareji yehupenyu hwevana ava inosvika makore 14.5. Zvisinei, vamwe vana vanorarama kusvika pamakore ekutanga ekuma20. Chikonzero chikuru cherufu rwevana ava chirwere chemwoyo kana sitiroko . Chikonzero cheizvi ndechekuti atherosclerosis , chirwere chinoita kuti madziro etsinga akobvu, chinowanzoonekwa muvakuru, muvana ava vachiri vadiki.
Ndiani anowana chirwere ichi? Chinowanikwa kakawanda sei?
Progeria chirwere chemajini chinogona kukanganisa chero munhu. Asi hachisi chenhaka . Kureva kuti, hachigamuchirwi kubva kuvabereki. Chinowanzo kukonzerwa nekuchinja kwemajini kubva mukutanga kusvika pakuva nemwana. Kureva kuti, shanduko isingatarisirwi inoitika mumbeu isati yabata pamuviri.
Chirwere ichi hachiwanzoitiki zvekuti mwana mumwe chete pavana mamiriyoni mana vanozvarwa pasi rose ndiye anobatwa nechirwere ichi. Parizvino, kune vana nevechidiki vanosvika mazana mana vanorarama neProgeria pasi rose.
Ndezvipi zviratidzo zveProgeria?
Zviratidzo zveProgeria zvakafanana nezvevanhu vanokwegura, asi zvinoonekwa pazera diki kwazvo. Zviratidzo izvi zvinogona kuonekwa mumakore maviri ekutanga ehupenyu. Ngatitsanangure zviratidzo izvi.
| Rudzi rwehunhu | Zvinhu zvekuona |
|---|---|
| Zviratidzo zvinowanzoonekwa pakutanga | |
| Kunonoka kukura | Kureba kwemwana uye kuwedzera kwehuremu kwakaderera zvikuru. |
| Kukwinya kweganda | Ganda rinotsvedza uye rinoita makwinya, seremunhu mukuru. |
| Kudonha kwebvudzi | Tsiye, tsiye, uye bvudzi zvinodonha, zvichikonzera mhanza. |
| Kuomarara kwemajoini | Kuderera kwekuchinjika kwemajoini mumakumbo uye kuoma kwekufamba. |
| Kukora kweganda | Kukora uye kuoma kweganda (kufanana nescleroderma). |
| Kuderedza mafuta mumuviri | Mafuta ari pasi peganda anorasika. |
| Kuchinja kunooneka pachiso nemusoro | |
| Kuwedzera musoro | Musoro wacho unotaridzika wakakura kana tichienzanisa nechiso (macrocephaly). |
| Chiso chidiki | Chiso chacho chinoita sechidiki uye chakatetepa zvichienzaniswa nehukuru hwemusoro. |
| Chimiro chemhino | Mhino yacho yakatetepa uye yakapinza, yakaita semuromo weshiri. |
| Kuderedza shaya | Kukura kweshaya yepazasi (micrognathia) kwakaderedzwa. |
| Kunonoka kubuda kwemeno | Kunonoka kubuda kwemeno uye kuvharirana kwemazino. |
| Zviratidzo zvinoonekwa kana chirwere chichienderera mberi | |
| Hip hop | Kubuda kwehudyu. |
| Matumbu emusoro | Matumbu emusoro. |
| Arthritis | Arthritis. |
| Mafuta ari mutsinga dzeropa | Kuora kweropa. |
Chii chiri kukonzera izvi? Sei izvi zviri kuitika?
Izvi zvinokonzerwa nekuchinja kudiki kwazvo kwejini rimwe chete mumuviri wedu. Jini iri rinonzi LMNA gene . Basa rejini iri nderekugadzira puroteni inonzi lamin A, iri munucleus yesero rega rega mumuviri wedu uye inobatsira kuti irambe yakagadzikana.
Funga nezvemasero edu sezvidhinha, nucleus iri pakati pezvinhu zvinodzora mukati. Puroteni yeLamin A yakaita sefuremu yakasimba yakakomberedza nucleus iyoyo.
MuProgeria, chikanganiso chidiki mugene reLMNA chinoita kuti kopi yeprotein yelamin A isina kunaka ibudiswe panzvimbo pezvo. Izvi zvinonziProgerin . Progerin iyi isina kukwana inoita kuti nucleus yesero irasikirwe nesimba rayo uye isagadzikana. Zvishoma nezvishoma, masero aya anokuvara uye anofa nguva isati yakwana. Kana izvi zvikaitika kune sero rega rega mumuviri, muviri wese unotanga kukwegura nekukurumidza.
Ichi chirwere chinogara nhaka here?
Kwete. Kazhinji, izvi hazvigamuchirwi nhaka. Uku kuchinja kwemajini kunoitika pasina kurongeka. Uku ndiko kunonzi autosomal dominant condition. Izvi zvinoreva kuti kunyangwe kuva nekopi imwe chete yegene risina kunaka zvakakwana kukonzera chirwere ichi.
Asi, pane chimwe chinhu. Kana uine mwana ane Progeria kare, njodzi yekuti mumwe mwana awane chirwere ichi yakakwira zvishoma, iri pakati pe2% ne3%. Izvi zvinokonzerwa nechirwere chinonzi mosaicism . Izvi zvinoreva kuti mumwe wevabereki ane genetic mutation iyi mune mamwe masero emuviri wavo (semuenzaniso, mumasero anogadzira urume kana mazai), asi haaratidzi zviratidzo. Saka, kana mwana wako aine Progeria, zvakakosha kuti uongorore majini uye utaure nachiremba wako nezvazvo.
Chirwere ichi chinoonekwa sei uye chinorapwa sei?
Chiremba wako anogona kufungidzira kuti chirwere ichi chine chekuita nechitarisiko chemwana wako uye zviratidzo zvake. Kuti asimbise chirwere ichi, ropa remwana richatorwa uye kuongororwa kwemajini kuchaitwa.
Chinhu chakakosha ndechekuti hapasati pave nemushonga chaiwo weProgeria. Zvisinei, kune mishonga inogona kugadzirisa chirwere ichi uye kuvandudza hupenyu hwemwana uye hupenyu hwake.
Nzira dzekurapa
1. Mushonga: Mushonga weLonafarnib unoshandiswa pakuita izvi. Mushonga uyu, wakagadzirwa pakutanga wegomarara, wakawanikwa uchibatsira kuderedza kufambira mberi kweProgeria. Mushonga uyu wakaratidzwa kuti unowedzera avhareji yehupenyu hwevana nemakore angangoita 2.5. Izvi,
- Inowedzera kuchinjika kwetsinga dzeropa.
- Inosimbisa chimiro chemapfupa.
- Zvinobatsira kuwedzera huremu hwemuviri.
- Inovandudza kugona kunzwa.
2. Kurapa Kwemuviri uye Kurapa Kwebasa:
Kurapa muviri kunobatsira kuchengetedza kufamba kwemajoini emwana, kuenzana, uye chimiro chake. Kurapa kwemabasa kunobatsira mwana kudzidza kudya ega, kuchengetedza hutsanana hwake, uye kuita mabasa ezuva nezuva zviri nyore, akadai sekunyora.
3. Kutarisirwa kwenguva dzose kwechiremba:
Mwana ane Progeria anoda kutarisirwa nachiremba nguva dzose kuitira kuti matambudziko angangoitika aonekwe uye agadziriswe nekukurumidza.
| Chikamu Chinotarisira | Zvinhu zvekuita |
|---|---|
| Chirwere chemwoyo | Kuongororwa kweropa nguva dzose, kuongororwa kwemoyo, nezvimwewo. Kupihwa mishonga yakaita seaspirin ine mwero mudiki. |
| Sisitimu yetsinga | Kuita scan dzakadai seMRI kuti vaone kana paine chirwere chestroke. |
| Kuona maziso | Kuongororwa maziso nguva dzose nekuda kwezvirwere zvakaita sekuona kusina kunaka, maziso akaoma, nezvimwewo. Kushaikwa kwemaziso kunowedzera njodzi yekuti guruva ripinde mumaziso. |
| Kunzwa | Kurasikirwa nekunzwa kunogona kuitika uye zvinhu zvinobatsira kunzwa zvinogona kudiwa. |
| Hutano hwemazino | Matambudziko akadai sekunonoka kubuda kwemeno, kuvharika kwemazino, uye maburi anowanikwa kakawanda. Zvakakosha kushanyira chiremba wemazino nguva nenguva. |
| Kudya zvinovaka muviri | Zvakakosha kupa mwana macalorie nemvura yakakwana. Kana zvichidikanwa, chubhu yekudyisa inogona kushandiswa. |
Unotarisira sei mwana wako semubereki?
Kuziva kuti mwana wako ane Progeria chinhu chakaoma kubata nacho. Zvinogona kukuremera. Asi hausi wega parwendo urwu. Chinhu chinonyanya kukosha kugadzira mamiriro epamba akajairika uye anofadza sezvinobvira kumwana wako.
- Ipa hupenyu hwakajairika:Rega mwana wako atore chikamu muzviitiko zvese zvaanogona kuita, uye iva nechokwadi chekuti haanzwi asina kusarudzwa nevamwe vana mumhuri.
- Taura chokwadi: Taura nemwana wako nezvemamiriro ake ezvinhu nenzira yaanonzwisisa uye inoenderana nezera rake. Zvakakosha kutaura chokwadi nezvenyaya iyi nemunhu wese mumhuri. Kana zvichidikanwa, tsvaga mazano emhuri.
- Gadzirira mwana wako kuti anzwe manzwiro evamwe vanhu: Vamwe vanhu vanogona kutarisa mwana wako vachishamisika kana kutozevezera. Dzidzisa mwana wako maitiro ekugadzirisa mamiriro ezvinhu akadaro.
- Batana nechikoro: Vana vazhinji vane Progeria vanopinda chikoro. Taura nguva dzose nevatungamiriri vechikoro, vadzidzisi, uye vanamukoti kuti uve nechokwadi chekuti mwana wako akachengeteka uye anonzwa kugadzikana. Gadzira hurongwa hwezvaunofanira kuita kana paine emergency (semuenzaniso, kurwadziwa pachipfuva, kunetseka kufema) uye ramba uchizivisa chikoro.
Yeukai, kuwana rutsigiro kubva kuchikwata chevanachiremba vemwana wenyu, vanachiremba, nevamwe vabereki kuchakubatsirai zvikuru kuti rwendo urwu ruve nyore.
Mharidzo Yekuenda Nayo Kumba
- Progeria chirwere chisingawanzoitiki chemajini chinoita kuti miviri yevana ikwegure nekukurumidza.
- Izvi hazviwanzoitika nekuda kwekusagadzikana kwemajini, asi zvinokonzerwa nekuchinja kwemajini.
- Hapana dambudziko nepfungwa dzevana ava. Dambudziko riri mukukurumidzira kwekukura kwemuviri nekukwegura.
- Kunyange hazvo pasina mushonga chaiwo weizvi parizvino, mishonga, physiotherapy, uye kugara uchitariswa nachiremba zvinogona kugadzirisa zviratidzo uye kuvandudza hupenyu hwemwana uye hupenyu hwake.
- Rutsigiro rwakasimba kubva kuvabereki, mhuri, uye timu yezvekurapa rwakakosha kuti mwana ararame hupenyu hunofadza uye hwakajairika sezvinobvira.
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