Ch. 28: Osteogenesis Imperfecta (OI)

Question 1

Heterogenous, autosomal dominant disorder of the bones resulting in fractures and deformity

Answer 1

Osteogenesis imperfecta

Question 2

What are the classic manifestations?

Answer 2

Multiple bone fractures
Blue sclera
Early hearing loss

Question 3

What are the more severe manifestations of OI?

Answer 3

Bowed legs and arms
Kyphosis
Scoliosis

Question 4

What treatment is used to increase bone density?

Answer 4

Med of Pamidronate–Administer IV

Question 5

What are the adverse efffects we need to monitor for when giving the Pamidronate for OI?

Answer 5

Hypokalemia
Hypomagnesemia 
Hypocalcemia 
Hypophosphatemia
General malaise

Question 6

What kind of exercises should we teach the family and client about?

Answer 6

Low impact exercises

Question 7

What are surgical interventions (for severe cases)?

Answer 7

Correct bone deformities, placement of rods

Question 8

What are some complications of OI?

Answer 8

• Disuse osteoporosis (limit time in casts; encourage activity)
• Hearing loss
• Permanent deformities