Osteogenesis imperfecta

Others

• OI from the cellular and molecular perspective
• The bone in OI
  • 1. The locomotor system
  • 2. The bone under the microscope
  • 3. Bone mineral density
  • 4. Bone fragility and fractures
  • 5. Fracture management
  • Summary chapter 2
• Other orthopedic disorders caused by OI
• Oro-cranial disorders in OI
• Hearing in OI
• Vision and OI
• Other clinical features of OI
• Results of the study about osteogenesis imperfecta (Rare Commons)

Summary chapter 2

• Bones, joints, and muscles make up the locomotor system of the human body. This system allows humans to move, interact with the environment and remain still, and it holds and protects the other organs of the body.
• A bone has two types of tissue or textures: the outer part of the bone is the bone cortex, which is compact, while the trabecular bone, the inner part, has a spongy texture. Bones affected by OI have a thinner cortex and thinner and less trabeculae than healthy bones.
• Bone mineral content increases in childhood and even more so during puberty, reaching peak bone mass at 20-25 years of age, .After that age, progressive bone loss begins. Dual-energy X-ray absorptiometry (DXA) is the most widely used and standardised method to quantify bone mass.
• The resistance of a bone to suffer a fracture depends on its composition (characteristics of the collagen, degree of mineralisation,...) and the structure of the bone (size and shape of the bone, bone mass, amount of bone cortex and trabecular bone,...). In people affected by OI we will find alterations at all of these levels, which ultimately cause bone fragility.
• There are different types of fractures depending on their location in the bone, the fracture line and the degree of damage of the underlying tissues.
• Healing of fractures in patients with OI occurs as quickly as in healthy people, so the immobilization period must be the same (or shorter) in order to avoid loss of bone mass associated with lack of use of the affected limb or body part.
• Sometimes fractures may require the insertion of intramedullary rods, which provide resistance and keep the bone as straight as possible. There are different types of intramedullary rods, so we must choose the ones that are more adequate for each patient.

Last modified

20 January 2023