13. Bond Disorders: Osteogenesis Imperfecta (OI)

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8 Terms

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Osteogenesis Imperfecta

Inherited “brittle bone disease” due to defective collagen type/ production

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Pathophysiology

Defective collagen → impaired bone matrix → weak bones prone to fracture

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Signs and symptoms

  • Multiple recurrent fractures

  • Blue sclera

  • hearing loss (ossicle involvement)

  • Dentinogenesis imperfecta (Teeth discoloration)

  • Short stature

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Risk Factors

  • Genetic (autosomal dominant)

  • Family History of OI

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Diagnostics

  1. Genetic Testing (COL1A1/ COL1A2 mutation)

  2. X-ray: Thin bones, old fractures, deformities

  3. Bone Biopsy : rare

  4. DXA Scan: Measures bone density

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Nursing Management

  • Prevent fractures (gentle handling)

  • Provide mobility support (Bracing, wheelchairs)

  • Support for hearing and dental issues

  • Educate family about safety at home/school

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Pharmacologic Treatment

  1. Bisphosphates : Increase bone density

  2. Calcium and Vitamin d: Support bone mineralization

  3. Growth Hormone

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Surgical Interventions

🔧 Intramedullary Rodding

  • Indication: Recurrent long bone fractures and limb deformities in children with moderate to severe OI

  • Purpose: Insert rods into long bones (femur, tibia) to prevent future fractures and correct deformity

  • Post-op: Early but gentle mobilization, physical therapy, monitor growth and rod integrity

🧱 Fracture Repair / Deformity Correction

  • Indication: Complex fractures or bowed limbs

  • Purpose: Align and support fragile bones using internal fixation or corrective osteotomy

  • Post-op: Extreme care during transfers, high fracture risk during rehab