Osteogenesis imperfecta/lobstein syndrome associated with dentinogenesis imperfecta.

Collagen Type I Alpha 1 Mutation Causes Osteogenesis Imperfecta from Mild to Perinatal Death in a Chinese Family

IntRoductIon Osteogenesis imperfecta (OI), also known as brittle bone disease or Lobstein syndrome, is characterized by blue or gray sclerae, variable short stature, dentinogenesis imperfecta, hearing loss, and recurrent fractures. Based on clinical, genetic, and radiological features, Sillence et al.[1] classified the OI into four subtypes including type I: Mild, common, with blue sclera; type...

Prosthetic treatment in a patient with osteogenesis imperfecta.

Osteogenesis imperfecta is a syndrome of interest to dentists because dentinogenesis imperfecta is frequently associated with not only bone disease but also tooth diseas~. Specifically, these patients usually have many dental problems such as 1) high incidence of tooth fracture, enamel chipping and cracking, 2) high caries activity, 3) inability or difficulty of endodontic treatment, 4) mastica...

Osteogenesis imperfecta with dentinogenesis imperfecta: a mistaken case of child abuse.

Dentinogenesis imperfecta associated with osteogenesis imperfecta: report of two cases.

Osteogenesis imperfecta (OI) is a heritable systemic disorder of the connective tissue. Dentinogenesis imperfecta (DI), which is sometimes an accompanying symptom of OI, belongs to a group of genetically conditioned dentin dysplasias and is characterized clinically by an opalescent amber appearance of the dentin. Although the teeth of DI cases wear more easily and excessively compared to normal...

Heterogeneity of osteogenesis imperfecta type I.

We have studied 166 patients from 71 families with Sillence type I osteogenesis imperfecta (dominant inheritance and blue sclerae). We confirm earlier findings that there are two subgroups, those with and those without dentinogenesis imperfecta; each family can be allocated to one or other group. Our confidence that the two groups represent distinct disorders is increased by finding that the pa...