Osteogenesis Imperfecta (OI), inowanzonzi brittle bone chirwere, chirwere chinokonzera kukanganisa mukugadzirwa kwe collagen protein. Collagen ipuroteni inokosha inobatsira kutsigira muviri; funga nezvazvo sechinhu chinogadziriswa nemuviri. Kune mhando dzakasiyana dzegorogen, iyo yakawanda kwazvo iyo inonzi I collagen.
Tora I collagen inowanikwa mune bone, the sclera yeziso, ligaments, uye meno.
Osteogenesis imperfecta inowanikwa kana pane chiremera chekugadzirwa kwegorgen I.
Unowana Sei Osteogenesis Imperfecta?
Osteogenesis imperfecta inokonzerwa nehutano hwemagetsi mujeni rinotungamirira muviri kubudisa collagen. Osteogenesis imperfecta inogona kupfuudzwa kubva kumubereki ane chirwere ichi, kana kuti inogona kuitika sekuchinja kwemazvirokwazvo mumwana. Nyaya dzakawanda dze osteogenesis imperfecta dzinotapudzwa kubva kuvabereki dzinokonzerwa nekuchinja kukuru , kunobva kune mumwe mubereki ane osteogenesis imperfecta, kunyange zvazvo nhamba shomanana yezviitiko inokonzerwa nekuchinja kwezvinhu zvakanyanya uye inouya kubva kune geni kubva kuvabereki vose, uye isina chirwere.
Zviratidzo nezviratidzo
Matambudziko anowanzoonekwa nevarwere vane osteogenesis imperfecta vanosanganisira bone fragility, nguva shoma, scoliosis, mazino mazino, kupererwa kwekunzwa, bluish sclera, uye kusununguka kwemitsipa . Osteogenesis imperfecta inowana zita rayo rakawanda, brittle bone chirwere nokuti vana ava vanowanzoongororwa mushure mekusimbisa mafupa akawanda akaputsika .
Zviratidzo zve osteogenesis imperfecta zvakasiyana zvikuru pakati pevanhu. Vamwe vana vanoongororwa kare kare muupenyu, asi vamwe vanogona kuva zvakaoma kuti vaone mamiriro acho ezvinhu. Kuwanda kwezviputi zvinokonzerwa nekukuvara kwepasi-simba kunofanira kusimudzira kusava nechokwadi kwemamiriro ezvinhu akadai sa osteogenesis imperfecta.
Mutsika, osteogenesis imperfecta yakarongerwa kuva chimwe chezvikamu zvina zvikuru:
- Tanga I: mildest form; inoonekwa muvana vane makore ekuchikoro
- Rudzi rwepiri: rinouraya pakuberekwa
- Rudzi rwechitatu: kuonekwa pakuberekwa; zvizhinji zvakaputsika; kazhinji haakwanisi kufamba
- Rudzi rweV IV: kushungurudzika pakati peiyo I uye firipi III
Kwemakore gumi adarika, mamwe maitiro e osteogenesis akave akarondedzerwa. Parizvino, pane 8 sub-types of osteogenesis imperfecta.
Kurapa
Parizvino, hapana chirwere chakazivikanwa che osteogenesis imperfecta. Chinonyanya kukoshesa kurapwa ndechekudzivirira kukuvara nekuchengetedza mapfupa ane utano. Kuvimbisa kuti vana vane osteogenesis imperfecta vanodya kudya kunovaka muviri, vanogara vachirovedza muviri, uye kuchengetedza hutano hwakanaka hunokosha zvikuru. Kakawanda vana vane osteogenesis imperfecta vachatsvaka rubatsiro rwekuvharorera, mabhesi, kana maguruvhiri.
Kuvhipwa kweasteogenesis imperfecta kunoenderana nekuoma kwemamiriro acho uye nguva yemurwere. Muvana vaduku zvikuru, kuputsika kwakawanda kunorapwa zvakafanana, sokunge kuti mwana akanga asina mamiriro ezvinhu. Zvisinei, kuparadzanisa kugadzikana kwezvipuka kunowanzova kwakajairika, kunyange pazera remakore.
Kuvhuniwa mapfupa kuvana vane osteogenesis imperfecta vanowanzorapwa nekuvhiyiwa kuitira kuti vagadzikane mapfupa uye kudzivirira kuora mwoyo.
Intramedullary rodding , apo tsvimbo yesimbi inogadzirirwa pasi chivako chepfupa, inowanzoshandiswa kubatsira kutsigira tete rakaoma uye kudzivirira kuora kwefupa. Scoliosis inowanzobatwa nechisimba sezvo dambudziko rinowedzera kufambira mberi kune vana vane osteogenesis imperfecta.
Basa remishonga mukurapa kwe osteogenesis imperfecta riri kubuda, asi zvidzidzo zvenguva pfupi zvinoratidza kubatsirwa kwerapisphonates uye calcium supplements . Chikonzero ndechekuti idzi kurapwa dzichasimbisa pfupa uye kuderedza kuwanda kwezviputsi. Kutsvakurudza kuri kuitwawo mukushandiswa kwekukura kwehomoni uye maitiro ekurapa.
Sources:
Burnei G, et al. "Osteogenesis Imperfecta: Kuziva uye Kurapa" J. Am. Acad. Ortho. Surgery., June 2008; 16: 356 - 366.