Biomaterial Database

[Osteogenesis imperfecta and dentinogenesis imperfecta. Apropos of 4 cases]. 1989

J C Ajacques, and P Farge, and B Kerebel

Hôpital Debrousse, Lyon.

Based on four personal cases of osteogenesis imperfecta and dentinogenesis imperfecta, the authors review the semiological elements constituting the syndrome, and assign each case to the Sillence (1979) classification. The author established his classification on clinical criteria and the genetic nature of the various forms of osteogenesis imperfecta, however, the most recent discoveries concerning collagen biochemistry confirm that the condition is without doubt more heterogenous than suggested clinically, thereby better explaining the wide range of mutations encountered.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D010013	Osteogenesis Imperfecta	COLLAGEN DISEASES characterized by brittle, osteoporotic, and easily fractured bones. It may also present with blue sclerae, loose joints, and imperfect dentin formation. Most types are autosomal dominant and are associated with mutations in COLLAGEN TYPE I.	Fragilitas Ossium,Lobstein Disease,Brittle Bone Disease,Lobstein's Disease,Osteogenesis Imperfecta Tarda,Osteogenesis Imperfecta with Blue Sclerae,Osteogenesis Imperfecta, Type 1,Osteogenesis Imperfecta, Type I,Disease, Lobstein,Disease, Lobstein's,Lobsteins Disease,Ossiums, Fragilitas,Osteogenesis Imperfecta Tardas
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003811	Dentinogenesis Imperfecta	An autosomal dominant disorder of tooth development characterized by opalescent dentin resulting in discoloration of the teeth. The dentin develops poorly with low mineral content while the pulp canal is obliterated.	Hereditary Opalescent Dentin,Capdepont Teeth,Dentinogenesis Imperfecta 1,Dentinogenesis Imperfecta without Osteogenesis Imperfecta,Dentinogenesis Imperfecta, Shields Type 2,Dentinogenesis Imperfecta, Shields Type II,Opalescent Dentin,Opalescent Teeth without Osteogenesis Imperfecta,Dentin, Opalescent,Opalescent Dentin, Hereditary,Teeth, Capdepont
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man