osteogenesis imperfecta type 3

Rare Disease

MONDO:0009804

Also Known As

OI type 3OI3osteogenesis imperfecta type 3osteogenesis imperfecta type IIIprogressive deforming osteogenesis imperfectaprogressively deforming osteogenesis imperfecta with normal sclerasevere osteogenesis imperfectaOI type IIIOI, type 3Oi3osteogenesis imperfecta, progressively deforming with normal scleraeosteogenesis imperfecta, progressively deforming, with normal scleraeosteogenesis imperfecta, type 3osteogenesis imperfecta, type IIIprogressively deforming OI

Definition

Osteogenesis imperfecta type III is a severe type of osteogenesis imperfecta (OI), a genetic disorder characterized by increased bone fragility, low bone mass and susceptibility to bone fractures. The main signs of type III include very short stature, a triangular face, severe scoliosis, grayish sclera, and dentinogenesis imperfecta (DI).