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Osteogenesis imperfecta is an inherited disorder of connective tissue that affects the skeleton,
ligament, skin, sclerae and dentin.
It is thought to be characterized by the abnormal maturation of collagen in both mineralized and
nonmineralized tissues. The three major clinical criteria are osteoporosis with abnormal fragility of
the skeleton, blue sclerae and dentinogenesis imperfecta. The presence of two of these abnormalities
confirms the diagnosis.
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