Osteogenesis imperfecta is an autosomal dominant disorder ofthe connective tissue, which is also known as "brittle-bonedisease" because it renders those affected susceptible to fracturesafter minimal trauma. Clinically and biochemically, osteogenesisimperfecta is a generalized disorder of the connective tissue,characterized by various combinations of blue sclerae, triangularfacies, macrocephaly, hearing loss, defective dentition, barrelchest, vertebral compression and scoliosis, progressive limbdeformity and bowing, joint laxity, and varying degrees of growthretardation.
The currently accepted classification of the disease includesfour types defined according to clinical and radiographic features,with some overlap among them.1 Type I is . . . [Full Text of this Article]
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