Pagets Disease Flashcards

1
Q

What is Paget’s disease?

A

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

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2
Q

What process is primarily increased in Paget’s disease?

A

Osteoclast-mediated bone resorption.

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3
Q

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

A

Increased bone formation.

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4
Q

What is the etiology of Paget’s disease?

A

The etiology is largely unknown, with hereditary factors suggested.

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5
Q

How does family history relate to Paget’s disease?

A

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

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6
Q

What genetic markers are associated with Paget’s disease?

A

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

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7
Q

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

A

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

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8
Q

At what age is Paget’s disease typically diagnosed?

A

It is typically diagnosed in mid or older age.

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9
Q

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

A

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

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10
Q

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

A

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

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11
Q

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

A

The axial skeleton

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12
Q

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

A

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

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13
Q

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

A

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

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14
Q

What percentage of Paget’s disease cases are polystotic?

A

About 80%, affecting multiple bones.

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15
Q

What characterizes the hot phase of Paget’s disease

A

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

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16
Q

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

A

Bone destruction occurs alongside new bone formation.

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17
Q

What is the cold phase of Paget’s disease?

A

It is characterized by disorganized new bone formation.

18
Q

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

A

Bowing of long bones.

19
Q

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

A

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

20
Q

How does Paget’s disease affect adjacent joints?

A

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

21
Q

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

A

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

22
Q

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

A

Head enlargement.

23
Q

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

A

Frontal bossing.

24
Q

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

A

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

25
What serious condition can arise from blood vessel compression in Paget's disease?
Stroke
26
What is vascular steal syndrome in the context of Paget's disease?
condition where external carotid blood flow to the skull occurs at the expense of blood flow to the brain.
27
What is basilar invagination in Paget's disease?
: A complication where the skull base invaginates into the upper cervical spine, potentially leading to neurological issues.
28
What neurological issues can arise from Paget's disease affecting the spine?
Compression neuropathy can lead to pain, tingling, numbness, paresis in an arm or leg, or paraplegia.
29
What spinal condition may occur due to Paget's disease?
Spinal stenosis.
30
What structural changes in the spine can occur in Paget's disease?
Vertebral compression and collapse.
31
What types of bone tumors are associated with Paget's disease?
Fibrosarcomas, benign giant cell tumors, and osteogenic sarcomas
32
What cardiac condition can result from Paget's disease due to increased bone vascularity?
High-output congestive heart failure.
33
How does Paget's disease potentially affect blood pressure?
It may lead to hypertension
34
What is a potential heart-related complication of Paget's disease?
Cardiomegaly
35
What vascular complication may occur in Paget's disease?
Hyperthermia
36
What laboratory finding is typically elevated in patients with Paget's disease?
Elevated alkaline phosphatase levels.
37
Radiology used inte diagnose
Ct/mri/ bone scan
38
Radiographic findings in the hot phase
Osteoporosis circumscripta Long bone: candle flame
39
Radiographic findings in intermediate phase in long bones
Long bones: conrtival thickening coarse trabecular pattern
40
Radiographic findings of the pelvis
Cortical thickening and sclerosis of the iliopectineal and ischiopubis line. Obliteration of the teardrop sign
41
Radiographic findings of the intermediate phase of the vertebrae
Picture fame appearance and cystic sponges
42
Radio graphic findings → cold phase
Skull→ cotton w'appearance. Spine →ivory vertebrae