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Types of Osteogenesis Imperfecta (Cont.)

 
Type V
Type V OI has similar symptoms to type IV, including:
 
  • A dense band seen on x-rays adjacent to the growth plate of the long bones
  • Calcification of the membrane between the radius and ulna (the bones of the forearm), leading to restriction of forearm rotation
  • Unusually large calluses, called hypertrophic calluses, at the sites of fractures or surgical procedures (a callus is an area of new bone that is laid down at the fracture site as part of the healing process)
  • White sclera
  • Normal teeth
  • Bone has a "mesh-like" appearance when viewed under the microscope.
     
Type VI
People with this type of osteogenesis imperfecta are moderately to severely affected. They have normal (white or slightly blue) sclera, and the teeth are not affected. The alkaline phosphatase (an enzyme linked to bone-forming cell activity) activity level is slightly elevated in type VI, and this can be determined by a blood test. Because the clinical features are so similar to other moderate types of osteogenesis imperfecta, a bone biopsy is the only method by which type VI can be diagnosed with certainty. The bone from patients with this form has a distinctive "fish-scale" appearance when viewed under the microscope. Eight people with this type of osteogenesis imperfecta have been identified.
 

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Written by/reviewed by: Arthur Schoenstadt, MD
Last reviewed by: Arthur Schoenstadt, MD