Piaget

Question 1

defintion - is it inflammatory or metabolic?

Answer 1

DEFINITION
Paget’s disease is a metabolic disorder of bone remodeling (osteoblastic and osteoclastic activity).
Increase resorption or loss of bone results in bone deposits that are weak, enlarged, and disorganized.

Question 2

ETIOLOGY / PATHOPHYSIOLOGY - what type of disease is it? And what is the cause?

Answer 2

Metabolic disorder, cause is unknown.
A viral cause has been proposed.
40 % have a positive family history of Paget’s disease.
Paget disease is thought to be a disease of osteoclast. In PG, osteoclasts appear histologically, bizarre in appearance, multinucleated and excessive in number

Question 3

PAGET’S DISEASE: what bones are mostly affected? (Paget’s is long)

Answer 3

The bones most affected are the pelvis, long bones, spine, ribs, sternum and cranium.

Question 4

Three phases of Paget’s disease:

Answer 4

ACTIVE
MIXED
INACTIVE

Question 5

PAGET’S DISEASE: how is it diagnosed?

Answer 5

The diagnosis of PD is primarily radiologic:
Usually diagnosed incidentally on X-ray

Question 6

PAGET’S DISEASE COMPLICATIONS (think bone…)

Answer 6

The most common one is pathological fracture. It could be the first indication of the disease.
Neoplasm:
Osteosarcoma
Fibrosacroma
Osteoclastoma (giant cell tumor)

Question 7

PAGET’S DISEASE: TREATMENT - what meds?

Answer 7

No cure
Symptomatic management and correction of deformities with surgery and braces
Medication therapy
Calcitonin: Inhibits osteoclastic activity
Biphosphanates: ↓ bone resorption
Correction of deformities (braces and surgery)
Firm mattress
Corset or light brace
Good body mechanics
Assistive devices
Well balanced diet with Vit D, Ca++, and protein

Question 8

blast =

Answer 8

builders

Question 9

symptoms (Piaget waddles)

Answer 9

Symptoms include:
Pain: due to bone overgrowth, deformity and nerve compression.
C/O of fatigue and waddling gait.
HA, dementia, visual deficit, hearing loss

Question 10

are symptoms bad?

Answer 10

Depending on disease severity : from mild to intractable.

Question 11

Active phase

Answer 11

ACTIVE
A prolific increase in osteoclasts (cells that breakdown bone). Causes massive bone destruction and deformity

Question 12

mixed phase

Answer 12

MIXED
Osteoblasts (bone forming cells) form new abnormal bone.
Trabecular (spongy) bone has a mosaic pattern and is 2x’s larger than regular bone.

Question 13

inactive phase

Answer 13

INACTIVE
Osteoblastic activity exceeds osteoclastic activity

Question 14

Lab tests would include

Answer 14

↑ ALP (alkaline phosphatase) Occurs because of overactive osteoblasts ( increased bone turn over)

Question 15

what is elevated on blood tests? and what else? (Paget is uric)

Answer 15

Can cause an elevation of Calcium and uric acid due to overactive bone metabolism. (Can be misinterpreted as gout)

Question 16

lab tests - urine. (Pager tests hydro)

Answer 16

Increase in urinary hydroxyproline levels (reflects an increase in bone collagen turnover). The higher the value, the worse the Paget’s disease.