Osteogenesis Imperfecta

Question 1

What is the primary goal of medical and surgical management of osteogenesis imperfecta?

Answer 1

To decrease the incident of fractures and maintain mobility

Question 2

What dental condition can occur with osteogenesis imperfecta?

Answer 2

Dentinogenesis imperfecta

Question 3

How does dentinogenesis imperfecta present?

Answer 3

Tooth enamel wears easily
Teeth are brittle and discolored

Question 4

What are additional complications associated with osteogenesis imperfecta?

Answer 4

Early hearing loss
Acute and chronic pain
Scoliosis
Respiratory problems

Question 5

What is a key nursing priority when caring for a patient with osteogenesis imperfecta?

Answer 5

Handle the patient carefully and teach the family to avoid trauma

Question 6

How should mobility be managed in children with osteogenesis imperfecta?

Answer 6

Safe mobility should be encouraged

Question 7

What supports safe mobility in osteogenesis imperfecta?

Answer 7

Use of adaptive equipment and appropriate promotion of mobility

Question 8

What therapies are used to promote independence in children with osteogenesis imperfecta?

Answer 8

Physical therapy and occupational therapy

Question 9

What do PT and OT help promote in children with osteogenesis imperfecta?

Answer 9

Find motor skills and independence and activities of living ADLs

Question 10

Why must children with osteogenesis imperfecta be handled cautiously during care?

Answer 10

To avoid trauma that could cause fractures

Question 11

What precautions should be taken when inserting IV or taking blood pressure in osteogenesis imperfecta?

Answer 11

Use caution as pressure on the arm or leg, can lead to bruising and fractures

Question 12

What is emphasized in family teaching for osteogenesis imperfecta?

Answer 12

Preventing injury, safe handling and minimizing trauma

Question 13

What is osteogenesis imperfecta (OI)?

Answer 13

A genetic bone disorder (also called brittle bone disease) characterized by low bone mass, increased bone fragility, and connective tissue problems.

Question 14

What connective tissue problem is commonly seen in osteogenesis imperfecta?

Answer 14

Joint hypermobility, which leads to joint instability.

Question 15

Why do children with osteogenesis imperfecta have frequent fractures?

Answer 15

Low bone mass, increased bone fragility, and joint instability all contribute to fracture occurrence.

Question 16

How does osteogenesis imperfecta usually occur?

Answer 16

As a result of a defect in the collagen type 1 gene.

Question 17

In children with moderate to severe osteogenesis imperfecta, what are common findings?

Answer 17

• Fractures are more likely to occur • Short stature is common

Question 18

What additional complications can occur with osteogenesis imperfecta?

Answer 18

• Hearing loss • Acute and chronic pain • Scoliosis • Respiratory problems

Question 19

What is the primary goal of medical and surgical management for osteogenesis imperfecta?

Answer 19

To decrease the incidence of fractures and maintain mobility.

Question 20

What dental condition can occur in osteogenesis imperfecta?

Answer 20

Dentinogenesis imperfecta

Question 21

How is dentinogenesis imperfecta characterized?

Answer 21

• Tooth enamel wears easily • Brittle and discolored teeth

Question 22

What hearing complication may be seen in osteogenesis imperfecta?

Answer 22

Early hearing loss

Question 23

Why is careful handling important in children with osteogenesis imperfecta?

Answer 23

To avoid trauma and prevent fractures.

Question 24

Should children with osteogenesis imperfecta be encouraged to move?

Answer 24

Yes — safe mobility should be encouraged.

Question 25

What supports safe mobility in children with osteogenesis imperfecta?

Answer 25

• Use of adaptive equipment • Appropriate promotion of mobility

Question 26

What therapies help children with osteogenesis imperfecta gain independence?

Answer 26

• Physical therapy (PT) • Occupational therapy (OT) To promote fine motor skills and independence in ADLs.

Question 27

What is a key nursing teaching point for preventing injury in children with osteogenesis imperfecta?

Answer 27

Never push or pull on an arm or leg.

Question 28

How should a baby with osteogenesis imperfecta be lifted?

Answer 28

Place one hand under the legs and buttocks and the other under the shoulders, head, and neck.

Question 29

What lifting techniques should be avoided in osteogenesis imperfecta?

Answer 29

• Lifting by the ankles • Lifting under the armpits • Bending limbs into awkward positions

Question 30

How should a suspected fracture be handled in a child with osteogenesis imperfecta?

Answer 30

Handle the limb minimally.

Question 31

Why should caution be used when inserting an IV or taking blood pressure in osteogenesis imperfecta?

Answer 31

Pressure on the arm or leg can lead to bruising and fractures.

Question 32

What is the single most important nursing priority when caring for a child with osteogenesis imperfecta?

Answer 32

Prevent injury by careful handling and trauma avoidance.