Dzimwe nguva unonzwa here kuti mwana wako ari kusaririra zvishoma mukukura? Kana kuti chimiro chake chechiso nemuviri wake zvinoita sezvakasiyana zvishoma nevamwe vana vezera rake? Dzimwe nguva, kuseri kwezvinhu izvi, panogona kunge paine chirwere chisingawanzoitika chatisati tambonzwa nezvacho. Nhasi, tiri kutaura nezvechirwere chisingazivikanwe nevanhu vazhinji, asi zvakakosha kuti isu sevabereki tizive nezvacho. Ndiyo Hunter Syndrome.
Zvichitaurwa zviri nyore, chii chinonzi Hunter Syndrome?
Hunter syndrome chirwere chisingawanzoitiki, chinoitika mumajini. Apa ndipo apo muviri wemwana wako usingakwanise kupwanya zvakanaka uye kugaya mamwe mamorekuru akaomarara eshuga. Funga nezvazvo semahorse madiki ari mukati memiviri yedu, atinoti maenzymes. Basa rawo nderekupwanya nekuchenesa zvinhu zvinopinda mumiviri yedu, zvinhu zvatisingade.
Mwana ane chirwere cheHunter syndrome anozvarwa aine enzyme shoma inodiwa kuti aparadze mhando yemorekuru yeshuga. Saka chii chinoitika ipapo? Mamorekuru eshuga asingagone kupatsanurwa anotanga kuungana munhengo dzemuviri nematishu emwana. Kungofanana nemarara asingabviswi, anoungana. Nekufamba kwenguva, kuunganidzwa uku kunogona kukanganisa kukura kwemwana mumuviri nepfungwa.
Vanachiremba vanopatsanura chirwere ichi muzvikamu zviviri zvikuru:
1. Rudzi rwakakomba rwezviratidzo: Urwu ndirwo rudzi rwakajairika (runenge 60%). Zviratidzo zvevana ava zvinokura nekukurumidza, uye hunyanzvi hwavo hwepfungwa hunokanganiswawo. Kazhinji, pazera remakore 6-8, mwana anotanga kuva nematambudziko nemabasa ekutanga.
2. Rudzi rusina kunyanya kuoma: Zviratidzo zvinoonekwa zvishoma nezvishoma. Njere dzemwana hadziwanzo kanganiswa zvakanyanya.
Chirwere ichi chiri muboka rezvirwere zvinonzi mucopolysaccharidoses. Ndosaka chirwere cheHunter chichinziwo mucopolysaccharidosis type II (MPS II) .
Chirwere ichi chakajairika zvakadii? Ndiani anonyanya kuchibata?
Ichi chirwere chisingawanzoitiki . Zvakare, chinonyanya kubata vakomana . Zvichienderana nehuwandu, munhu mumwe chete pavakomana 100,000 kusvika 170,000 vanoberekwa anowanikwa aine chirwere ichi.
Zvisinei, vasikana vanogona kutakura majini asina kunaka anokonzera chirwere ichi. Muchidimbu, musikana ane machromosome maviri eX, nepo mukomana aine rimwe chete. Saka kunyangwe musikana akagara nhaka chromosome yeX isina kunaka, imwe chromosome yake yeX ine hutano inogona kugadzira enzyme yaanoda. Asi kana mukomana akagara nhaka chromosome yeX isina kunaka, haana imwe sarudzo uye anova nezviratidzo.
Ndezvipi zviratidzo zvechirwere ichi?
Zviratidzo zvinowanzotanga kuonekwa mumwana ari pakati pemakore maviri nemana. Zviratidzo izvi zvinogona kusiyana kubva pamwana kuenda kumwana. Vamwe vana vane zviratidzo zvishoma, nepo vamwe vaine zvakawanda.
| Chiratidzo | Tsananguro |
|---|---|
| Chitarisiko chemuviri | Chiso chakakora (mhino dzakakora, miromo, nerurimi), musoro wakakura kupfuura avhareji, chipfuva chakafara, uye mutsipa mupfupi. |
| Mapfupa nemapfupa | Kuomarara kwemajoini emakumbo, kuoma kwekukotama. |
| Kukura | Kukura kunonoka. Kukura kwehurefu kunomira kana kuitika zvishoma nezvishoma, kunyanya mushure memakore mashanu. |
| Kunzwa | Kunzwa kunopera simba zvishoma nezvishoma. |
| Nhengo dzemukati | Kukura kwechiropa nespleen (kubuda kwedumbu). |
| Ganda nemazino | Kuonekwa kwemapundu machena paganda. Kunonoka kubuda kwemeno kana mipata mikuru pakati pemazino. |
Sei chirwere ichi chichiitika chaizvo?
Izvi zvinokonzerwa nekuchinja kwejini reIDS . Jini reIDS ndiro rinoita basa rekudzora kugadzirwa kweenzyme inonzi iduronate 2-sulfatase (I2S), iyo muviri wedu unoda.
Iyi enzyme yeI2S inopwanya mamorekuru akaomarara eshuga anonzi glycosaminoglycans (GAGs). Vana vane Hunter syndrome (MPS II) havagadziri iyi enzyme yeI2S zvachose, kana kuigadzira muhuwandu hushoma.
Izvi zvinoita kuti mamorekuru eshuga anonzi maGAG aunganire mu lysosomes, ayo ari nzvimbo dzekudzokorodza masero. Lysosomes dzakafanana nenzvimbo dzekudzokorodza masero. Zvirwere zvinoitika nekuda kwekuungana kwezvinhu mukati me lysosomes zvinonziwo lysosomal storage disorders . Nekufamba kwenguva, kuunganidzwa uku kunokuvadza nhengo dzemuviri.
Ndezvipi zvimwe zvinetso zvinogona kuitika nekuda kwechirwere ichi?
Zvichienderana nekuoma kwechirwere, mwana anogona kusangana nematambudziko akasiyana-siyana. Vanachiremba vanoshandisa mishonga uye dzimwe nguva vanotovhiya kuti vagadzirise matambudziko aya.
Chinhu chakakosha ndechekuti havasi vana vese vanozosangana nematambudziko aya. Saka musazvidya mwoyo. Zvakakosha kugara muchitaurirana nachiremba uye kugara muchitarisa mwana wenyu.
| Dambudziko | Tsananguro |
|---|---|
| Kuoma kufema | Kukora kwenyama yekufema kunogona kuvhara nzira dzekufema. |
| Chirwere chemwoyo | Mavharuvhu emwoyo anogona kukuvara. |
| Matambudziko emapfupa nemajoini | Kukanganisika kwemapfupa nemapfundo kunogona kuitika. |
| Basa rehuropi | Muzviitiko zvakakomba zvechirwere, mashandiro europi anogona kukanganisika. |
| Mamwe matambudziko | Carpal tunnel syndrome, hernias, pfari, uye matambudziko ehunhu zvinogona kuitika. |
Nzira yekuziva sei chirwere ichi?
Chiremba wemwana wenyu achaita bvunzo dzakawanda kuti aone kana chirwere ichi chiripo.
- Kuongororwa kweweti: Izvi zvinoongorora huwandu hweshuga hwakanyanya (GAGs) hwatakambotaura nezvahwo muweti.
- Kuongororwa kweropa: Izvi zvinogona kuona kana basa re enzyme inodiwa muropa riri shoma kana kuti risiripo.
- Kuongororwa kwemajini: Kuongororwa uku kunoitwa kuti pave nechokwadi chekuti kushanduka kwemajini kunokonzera chirwere ichi kuripo here.
Inorapwa sei?
Hapana mushonga weHunter syndrome parizvino . Zvisinei, kune nzira dzekurapa dzinodzora mafambiro echirwere, kuona matambudziko nekukurumidza sezvinobvira, uye kuvandudza hupenyu hwemwana.
Mushonga wakanakisa weizvi iEnzyme Replacement Therapy (ERT) . Izvi zvinosanganisira kugadzira enzyme inoshaikwa mumuviri woipa mwana mushonga uyu. Mushonga uyu unonzi idursulfase (Elaprase®) . Mushonga uyu unowanzo pihwa nemutsinga kamwe chete pasvondo.
Pamusoro pezvo, tsvakiridzo pamusoro pekurapa majini iri kuitikawo pasi rose, uye pane tariro yekuti zvichatungamira mukurapa kuri nani mune ramangwana.
Ungati chii nezveramangwana remwana?
Ndinoziva kuti mubvunzo uyu wakaoma zvikuru kubvunza. Muzviitiko zvakakomba zvechirwere, hupenyu hwemwana hunogona kuva hupfupi. Kazhinji pakati pemakore gumi nemakumi maviri. Zvisinei, vana vane zviratidzo zvishoma vanogona kurarama kusvika vakura.
Chinonyanya kukosha ndechekuti kurapwa kunogona kubatsira mwana wako kugadzirisa matambudziko aanosangana nawo uye kuvandudza hupenyu hwake. Saka usambofa wakarasa tariro.
Mibvunzo yekubvunza chiremba wako
Kana mwana wako akaonekwa aine chirwere ichi, zvakajairika kuva nemibvunzo yakawanda mupfungwa dzako. Bvunza chiremba wako nezvezvinhu izvi zvakajeka.
- Urwu rudzi rwechirwere rwakanyanya here kana kuti rusina kunyanya kuoma?
- Chii chichaitika kumwana wangu kwenguva pfupi uye kwenguva refu?
- Chirwere ichi chichakanganisa sei hupenyu hwemwana wangu?
- Ndezvipi zvingasarudzwa pakurapa?
Zvakajairika kuti mhuri ishamiswe uye ishungurudzike kana ikanzwa nezvechirwere chakadai. Yeuka kuti hausi wega panguva ino. Taura nachiremba wako, mhuri, neshamwari dzepedyo nezveizvi. Tose tinofanira kushanda pamwe chete kuti tipe mwana wako rubatsiro rwakanakisisa.
Mharidzo Yekuenda Nayo Kumba
- Hunter syndrome chirwere chisingawanzoitiki, chine majini akawanda, chinonyanya kubata vakomana.
- Chirwere ichi chinokonzerwa nekushaikwa kwe enzyme yakakosha mumuviri, zvichiita kuti mamwe mamorekuru eshuga aungane mumuviri uye akuvadze nhengo dzemuviri.
- Zviratidzo zvinowanzotanga kuonekwa pakati pemakore maviri kusvika mana. Zviratidzo zvikuru kunonoka kukura, kushanduka kwechiso, uye kuomarara kwemajoini.
- Kunyange hazvo pasina mushonga wakakwana weizvi, mishonga yakaita se enzyme replacement therapy (ERT) inogona kudzora zviratidzo uye kuvandudza hupenyu hwemwana.
- Kana mukaona chimwe chinhu chisina kunaka mukukura kwemwana wenyu, kurumidzai kuonana nachiremba. Kuongororwa kwechirwere ichi nekukurumidza kwakakosha zvikuru pakurapwa.
💬 Comments (0)
Hapana zvakataurwa parizvino. Isa mhinduro yako pano kekutanga.
Wedzera zvaunofunga