Pagets Disease

Question 1

What is Paget’s disease?

Answer 1

A disorder of bone remodeling characterized by increased osteoclast-mediated bone resorption followed by increased bone formation.

Question 2

What process is primarily increased in Paget’s disease?

Answer 2

Osteoclast-mediated bone resorption.

Question 3

What follows the increased bone resorption in Paget’s disease?

Answer 3

Increased bone formation.

Question 4

What is the etiology of Paget’s disease?

Answer 4

The etiology is largely unknown, with hereditary factors suggested.

Question 5

How does family history relate to Paget’s disease?

Answer 5

There is a higher occurrence in patients’ relatives than in controls, with 15-30% of patients having a positive family history.

Question 6

What genetic markers are associated with Paget’s disease?

Answer 6

HLA DQw 1 and DR antigens, as well as the sequestrosome 1 gene on chromosome 5.

Question 7

What potential trigger is associated with the onset of Paget’s disease in genetically predisposed individuals?

Answer 7

Viral infection may be necessary to trigger the disease in those who have inherited the associated gene.

Question 8

At what age is Paget’s disease typically diagnosed?

Answer 8

It is typically diagnosed in mid or older age.

Question 9

What is the male-to-female ratio of Paget’s disease in the US?

Answer 9

The ratio is approximately 3:2, with men being more affected than women.

Question 10

How does the prevalence of Paget’s disease vary by age?

Answer 10

The disease is more common in mid to older age groups.

Question 11

What part of the skeleton is more commonly affected by Paget’s disease?

Answer 11

The axial skeleton

Question 12

Which specific bones are commonly affected by Paget’s disease?

Answer 12

The skull, spine, pelvis, and proximal long bones like the femur.

Question 13

What percentage of Paget’s disease cases are monostotic, and which bones are typically involved?

Answer 13

Approximately 20% are monostotic, commonly affecting the tibia and iliac bones.

Question 14

What percentage of Paget’s disease cases are polystotic?

Answer 14

About 80%, affecting multiple bones.

Question 15

What characterizes the hot phase of Paget’s disease

Answer 15

An initial phase of increased osteoclastic activity affecting both the cortex and cancellous bone.

Question 16

What happens during the intermediate or mixed phase of Paget’s disease?

Answer 16

Bone destruction occurs alongside new bone formation.

Question 17

What is the cold phase of Paget’s disease?

Answer 17

It is characterized by disorganized new bone formation.

Question 18

What deformity is commonly seen in long bones affected by Paget’s disease?

Answer 18

Bowing of long bones.

Question 19

What percentage of Paget’s disease patients experience fractures, and which bones are commonly affected?

Answer 19

Approximately 7%, commonly in the femur, tibia, humerus, and forearm.

Question 20

How does Paget’s disease affect adjacent joints?

Answer 20

can lead to osteoarthritis (OA) of nearby joints due to stress from enlarged bones.

Question 21

What is acetabular protrusion in the context of Paget’s disease?

Answer 21

A complication where the acetabulum (hip socket) protrudes due to bone changes, potentially affecting hip function.

Question 22

What is a common result of Paget’s disease in the skull?

Answer 22

Head enlargement.

Question 23

What deformity is associated with Paget’s disease of the skull?

Answer 23

Frontal bossing.

Question 24

What percentage of patients may experience deafness due to Paget’s disease?

Answer 24

Approximately 13%, along with headaches and dizziness from auditory nerve entrapment.

Question 25

What serious condition can arise from blood vessel compression in Paget’s disease?

Answer 25

Stroke

Question 26

What is vascular steal syndrome in the context of Paget’s disease?

Answer 26

condition where external carotid blood flow to the skull occurs at the expense of blood flow to the brain.

Question 27

What is basilar invagination in Paget’s disease?

Answer 27

: A complication where the skull base invaginates into the upper cervical spine, potentially leading to neurological issues.

Question 28

What neurological issues can arise from Paget’s disease affecting the spine?

Answer 28

Compression neuropathy can lead to pain, tingling, numbness, paresis in an arm or leg, or paraplegia.

Question 29

What spinal condition may occur due to Paget’s disease?

Answer 29

Spinal stenosis.

Question 30

What structural changes in the spine can occur in Paget’s disease?

Answer 30

Vertebral compression and collapse.

Question 31

What types of bone tumors are associated with Paget’s disease?

Answer 31

Fibrosarcomas, benign giant cell tumors, and osteogenic sarcomas

Question 32

What cardiac condition can result from Paget’s disease due to increased bone vascularity?

Answer 32

High-output congestive heart failure.

Question 33

How does Paget’s disease potentially affect blood pressure?

Answer 33

It may lead to hypertension

Question 34

What is a potential heart-related complication of Paget’s disease?

Answer 34

Cardiomegaly

Question 35

What vascular complication may occur in Paget’s disease?

Answer 35

Hyperthermia

Question 36

What laboratory finding is typically elevated in patients with Paget’s disease?

Answer 36

Elevated alkaline phosphatase levels.

Question 37

Radiology used inte diagnose

Answer 37

Ct/mri/ bone scan

Question 38

Radiographic findings in the hot phase

Answer 38

Osteoporosis circumscripta
Long bone: candle flame

Question 39

Radiographic findings in intermediate phase in long bones

Answer 39

Long bones: conrtival thickening coarse trabecular pattern

Question 40

Radiographic findings of the pelvis

Answer 40

Cortical thickening and sclerosis of the iliopectineal and ischiopubis line. Obliteration of the teardrop sign

Question 41

Radiographic findings of the intermediate phase of the vertebrae

Answer 41

Picture fame appearance and cystic sponges

Question 42

Radio graphic findings → cold phase

Answer 42

Skull→ cotton w’appearance. Spine →ivory vertebrae