I-Osteogenesis Imperfecta ivame ukubizwa ngokuthi yi-brittle bone disease. Ezimweni ezimbi kakhulu, ukuphuka okuningi kuvamile, kuyilapho icala elincane lihilela ukuphuka okumbalwa noma amathambo aphukile empilweni yomuntu. Abantu abanalesi simo bazalwa bengakwazi ukukhiqiza izicubu ezixhumeneyo noma ukuba nazo ngenxa yofuzo olungalungile. Ithinta ukwakheka kwe-collagen, iphrotheni yezicubu ezixhumeneyo eziqinisa amathambo. Lesi sifo sitholakala kumafomu I no-IX, mina ngimnene futhi IX ngibulala. Uphawu oluyinhloko lwe-osteogenesis imperfecta ukuphuka njalo. Ezinye izimpawu zihlanganisa ukuxega kwamalunga, amathambo akhubazekile, ukungezwa, izinkinga zokuphefumula kanye nezinkinga zamazinyo. I-Osteogenesis imperfecta ingaqinisekiswa nge-DNA noma ukuhlolwa kwe-collagen.
Akukho ukwelashwa kwalokhu ngoba kuyisimo sofuzo. Ukuzivocavoca nokuyeka ukubhema kuyizibonelo ezimbili zendlela yokuphila enempilo engasiza ekuvimbeleni ukuphuka. Izinsimbi, izidambisa-zinhlungu, nokunakekelwa okufanele kokuphuka kwamathambo konke kuyasiza. Ama-antibiotics kanye nama-antiseptics angasetshenziswa ukwelapha izifo zamathambo. Ukwenyuka kwesidingo sezindlela zokwelapha ezintsha zokwelapha i-osteogenesis imperfecta kanye nezivumelwano zamalayisense nokusebenzisana kwamasu ngabadlali ababalulekile bezimboni zokuthuthukiswa kokwelashwa kulindeleke ukuthi kuqhubekisele phambili ukukhula kwemakethe yokwelashwa kwe-osteogenesis imperfecta yomhlaba wonke esikhathini sokubikezela.
Ukwenyuka kwezimali zocwaningo kanye nentuthuko ukuthuthukisa izindlela zokwelapha ezintsha kanye nokwelapha kuyisici esibalulekile esikhuthaza ukukhula kwemakethe yokwelashwa kwe-osteogenesis imperfecta yomhlaba wonke. Ngaphezu kwalokho, izivumelwano zamalayisense nokusebenzisana kwamasu ngabadlali bemboni abaholayo ekuthuthukisweni kokwelashwa kwe-OI kungenye into eqhuba ukukhula kwemakethe. Ngaphezu kwalokho, i-osteogenesis imperfecta yisifo sofuzo, futhi umlando womndeni wabantu abane-OI ubangela ukwanda kwenani lamacala emhlabeni wonke, okulindeleke ukuthi kuqhubekisele phambili ukukhula kwemakethe.
Isibonelo, ngoMeyi 2021, i-Mereo BioPharma Group plc yamemezela idatha ekhuthazayo yezinyanga eziyisi-6 kusukela ocwaningweni lwayo lomtholampilo lweSigaba II-B ezigulini ezikhulile ezine-osteogenesis imperfecta uhlobo IV, III noma mina ngelashwe nge-setursumab (BPS-804). I-European Medicines Agency (EMA) igunyaze uhlelo lwenkampani lophenyo lwezingane lwe-BPS-804 kanye nedizayini yocwaningo lokubhalisa, futhi umuntu ozongenela umuthi uselungele Isigaba III sezingane.
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