By Disease Name > Osteogenesis Imperfecta
Osteogenesis Imperfecta
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•
four recognized forms (I-IV) - each has an
autosomal dominant
form (types II and III also AR form)
•
most common = type I (50%)
•
basic defect = synthesis of abnormal
type I collagen
•
patients with type I and IV have normal life span
•
Pamidronate (biphosphonate)
–
a potent inhibitor of bone resorption