Osteogenesis imperfecta (OI), izwi kandi nka indwara y'amagufwa acika intege, ni itsinda ry'indwara z'uruhererekane zituma amagufwa avunika byoroshye. Ishobora kandi gutera amaso afite ibara ry'ubururu, uburebure bugufi no kutumva neza.
Ahanini iterwa no kubura kolajeni yo mu bwoko bwa I. Nta muti uyikiza uhari, ariko ubuvuzi bufasha kwirinda no kuvura amavunika; ifata umuntu umwe ku bantu 15.000.
Ibimenyetso
Osteogenesis imperfecta (OI) ni itsinda ry'indwara ziterwa n'uturemangingo zifata cyane cyane amagufwa. Bituma amagufwa avunika byoroshye. Ubukana bwayo bushobora kuba buke cyangwa bukabije. Ibindi bimenyetso bishobora kuba birimo ibara ry'ubururu mu gice cyera cy'amaso (sclera), uburebure bugufi, ingingo zirekuye cyane, kutumva neza, ibibazo byo guhumeka n'ibibazo by'amenyo. Ingorane zishobora kuba zirimo gucikamo kw'imitsi y'amaraso yo mu ijosi no kw'umutsi munini w'amaraso (aorta).
Impamvu n'ubwoko
Ikibazo cy'ibanze akenshi ni icy'uturemangingo dufatanya umubiri (connective tissue) bitewe no kubura kolajeni yo mu bwoko bwa I. Ibi bibaho mu bantu barenga 90% bitewe n’impinduka mu miterere ya za jeni za COL1A1 cyangwa COL1A2. Ibi bibazo by’uturemangingo akenshi biragwa n’ababyeyi b’umuntu mu buryo busanzwe cyangwa bigaterwa n’impinduka nshya. Hari nibura ubwoko umunani bw'ingenzi, aho ubwoko bwa mbere ari bwo bworoheje cyane naho ubwoko bwa kabiri bukaba ari bwo bukabije cyane.
Isuzuma
Gusuzuma indwara akenshi bishingiye ku bimenyetso kandi bishobora kwemezwa hakoreshejwe isuzuma rya kolajeni cyangwa irya ADN.
Kuvura
Nta muti uhari. Kugira ubuzima bwiza, hakorwa imyitozo ngororamubiri kandi hirindwa kunywa itabi, bishobora gufasha mu kwirinda kuvunika. Ubuvuzi bushobora kuba burimo kwita ku magufwa yavunitse, imiti igabanya ububabare, ubuvuzi bw'umubiri, ibyuma bifasha amaguru (braces) cyangwa amagare y'abafite ubumuga ndetse no kubagwa. Ubwoko bw'ubuganga bushyira inkoni z'icyuma mu magufwa maremare bushobora gukorwa kugira ngo ayakomeze. Hari ibimenyetso bike bishyigikira ikoreshwa ry'imiti yo mu bwoko bwa bisphosphonate.
Ikwirakwira
OI igira ingaruka ku muntu umwe kuri 15.000. Uko indwara izagenda biterwa n'ubwoko bwayo, ariko abantu benshi bagira ubuzima bwiza.
Amateka
Iyi ndwara yasobanuwe kuva mu bihe bya kera. Ijambo "osteogenesis imperfecta" ryatangiye gukoreshwa mu 1895 kandi risobanura irema ry'amagufwa ridatunganye.
