Paget's Disease Of The Bone Flashcards

1
Q

What is Paget’s disease?

A

It is defined as a condition in which there is increased, uncontrolled bone turnover

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2
Q

What is the pathophysiology of Paget’s disease of the bone?

A

There is increased bone formation and reabsorption due to excessive osteoclast and osteoblast activity

This excessive turnover is uncoordinated, leading to patchy areas of high density (sclerosis) and low density (lysis)

Ultimately, this results in enlarged and misshapen bones.

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3
Q

Which four bones tend to be affected by Paget’s disease of the bone?

A

Skull

Spine

Pelvis

Proximal long bones of the lower extremities

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4
Q

What four risk factors are associated with Paget’s disease of the bone?

A

Older Age

Male Gender

Family History

Northern Latitude

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5
Q

What are the three clinical features of Paget’s disease of the bone?

A

Bone Pain

Bone Deformity

Pathological Fractures

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6
Q

What are the three investigations used to diagnose Paget’s disease of the bone?

A

Blood Tests

X-Ray Scans

Isotope Bone Scans

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7
Q

What three blood test results indicate Paget’s disease of the bone?

A

Increased ALP Levels

Normal Calcium Levels

Normal Phosphate Levels

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8
Q

What are the five features of Paget’s disease of the bone on x-ray scans?

A

Bone Enlargement & Deformity

Osteoporosis Circumscripta

Skull Cotton Wool Appearance

Thickened Skull Vault

Long Bone V-Shaped Defects

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9
Q

What is osteoporosis circumscripta?

A

It is defined as well defined osteolytic lesions that appear less dense compared with normal bone

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10
Q

What are cotton wool spots?

A

They are defined as poorly defined patchy areas of sclerosis and lysis

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11
Q

What are V-shaped defects?

A

They are defined as V-shaped osteolytic bone lesions surrounded by healthy bone

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12
Q

What is the feature of Paget’s disease of the bone on isotope bone scans?

A

There is increased uptake at the site of active bone lesions – usually skull, spine, asymmetrical pelvis and proximal long bones

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13
Q

What are the four management indications of Paget’s disease of the bone?

A

Bone Pain

Skull/Long Bone Deformity

Pathological Fractures

Peri-Articular Involvement

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14
Q

What is the pharmacological management option of Paget’s disease of the bone?

A

Bisphosphonate Drugs

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15
Q

When are bisphosphonate drugs used to manage Paget’s disease of the bone?

A

They are the first line pharmacological management option

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16
Q

How are bisphosphonate drugs used to manage Paget’s disease of the bone?

A

They interfere with osteoclast activity in order to restore normal bone metabolism

17
Q

Name two bisphosphonate drugs used to manage Paget’s disease of the bone

A

Oral risedronate

IV zoledronate

18
Q

How do we montior the effectiveness of Paget’s disease of the bone management?

A

We conduct regular monitoring of serum ALP levels and review the patient’s clinical features

19
Q

What are the seven complications associated with Paget’s disease of the bone?

A

Pathological Fractures

Osteosarcoma

Spinal Stenosis

Spinal Cord Compression

Skull Thickening

Hearing Loss

High Output Cardiac Failure