I-Osteogenesis Imperfecta yaziwa ngokuqhelekileyo njengesifo samathambo anqabileyo. Kwiimeko ezinzima, iifractures ezininzi ziqhelekileyo, ngelixa ityala elincinci libandakanya ukuphuka okumbalwa okanye amathambo aphukileyo ebomini bomntu. Abantu abanale meko bazalwa bengakwazi ukuvelisa izicubu ezixhumeneyo okanye babe nazo ngenxa yemfuza engalunganga. Ichaphazela ukuhlanganiswa kwe-collagen, iprotheni ye-tissue edibeneyo eqinisa amathambo. Esi sifo senzeka kwiifom I kunye ne-IX, kwaye mna ndinobulali kunye no-IX obulalayo. Uphawu oluphambili lwe-osteogenesis imperfecta kukuphuka rhoqo. Ezinye iimpawu ziquka ukuxengalala kwamalungu, amathambo aqhwalelayo, ukungeva kakuhle, iingxaki zokuphefumla kunye neengxaki zamazinyo. I-Osteogenesis imperfecta inokuqinisekiswa nge-DNA okanye uvavanyo lwe-collagen.
Alukho unyango loku kuba yimeko yemfuzo. Ukuzivocavoca kunye nokuyeka ukutshaya yimizekelo emibini yokukhetha indlela yokuphila enempilo enokunceda ukukhusela i-fractures. Iibrayisi, izithomalalisi zentlungu, kunye nokhathalelo olufanelekileyo lokuqhekeka kwamathambo zonke ziluncedo. Ii-antibiotics kunye ne-antiseptics zingasetyenziselwa ukunyanga izifo zamathambo. Ukonyuka kwemfuno yonyango lwenoveli yonyango lwe-osteogenesis imperfecta kunye nezivumelwano zelayisensi kunye nentsebenziswano yobuchule ngabadlali abaphambili boshishino kuphuhliso lonyango kulindeleke ukuba kukhuthaze ukukhula kwemarike yonyango lwe-osteogenesis imperfecta kwihlabathi ngexesha lengqikelelo.
Ukonyuka kwenkxaso-mali yophando kunye nophuhliso lokuphuhlisa unyango olutsha kunye nonyango yinto ebalulekileyo ekhuthaza ukukhula kwemarike yonyango lwe-osteogenesis imperfecta yehlabathi. Ngaphaya koko, izivumelwano zeelayisenisi kunye nentsebenziswano enobuchule ngabadlali abakhulu boshishino kuphuhliso lonyango lwe-OI yenye into eqhuba ukukhula kwemarike. Ngapha koko, i-osteogenesis imperfecta sisifo sofuzo, kwaye imbali yosapho lwabantu abane-OI ibangela ukwanda kwenani lamatyala kwihlabathi liphela, ekulindeleke ukuba iqhube ukukhula kwemarike.
Umzekelo, ngoMeyi ka-2021, i-Mereo BioPharma Group plc yabhengeza idatha ekhuthazayo yeenyanga ezi-6 kwiSigaba se-II-B solingo lwezonyango kwizigulana zabantu abadala abane-osteogenesis imperfecta uhlobo IV, III okanye ndinyangelwe nge-setursumab (BPS-804). I-Arhente yaMayeza yaseYurophu (EMA) ivume isicwangciso sophando lwabantwana senkampani ye-BPS-804 kunye noyilo lokufunda lokubhalisa, kwaye umgqatswa wechiza ngoku ulungele iSigaba sesi-III kubantu babantwana.
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