Paget’s Disease Flashcards

1
Q

History leading to diagnosis of Paget’s disease

A

Isolated elevation of ALP on routine bloods
Incidental finding on X-RAY
Bone pain
Secondary OA
Change in height or hat size
Progressive bone deformity or pathological fracture; gait abnormality due to change in length of long bone
Neurological symptoms - hearing loss, gait disturbance, headache
Symptoms of CCF
Symptoms of renal colic
Gout (secondary to increased bone turnover)
Sarcoma of the bone
Symptoms of hypercalcaemia
Pathological fractures

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2
Q

Examination in Paget’s disease

A
General inspection 
- Short stature
- Limb deformity
- Obvious osteosarcoma 
Face
- Skull diameter
- Auscultate skull (bruits)
- Fundi (angioedema streaks, optic atrophy)
- Hearing (ossicle or VIII nerve involvement)
- Other cranial nerves (foramina overgrowth or basilar invagination)
Neck
- short neck (basilar invagination)
- JVP (CCF)
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3
Q

Paget’s investigations

A

Hypercalcaemia
ALP (indicator of disease activity)
Urinary hydroxyproline level (indicator of disease activity)
Other biological markers of bone turnover e.g. Osteocalcin, urine or serum cross-links of collagen
Xray

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4
Q

Radiological findings in Paget’s Disease

A

Bones most often involved - pelvis, femur, skull, tibia
Look for bony enlargement, increased density, an irregular widened cortex & cortical infractions
Early lyric stage: flame shaped osteolytic wedge advancing along the bones
Secondary arthritic changes may occur
Bone scan is more sensitive than X-RAY in assessing extent of disease

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5
Q

Indications for treatment of Paget’s disease

A

Bone pain
Progressive deformity
Complications e.g. neural compression or high-output cardiac failure
Prelude to orthopaedic surgery

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6
Q

Treatment of Paget’s disease

A
Simple analgesics
Drugs that reduce bone absorption 
- oral bisphosphonate
- calcitonin of salmon or human origin
- mithramycin- given when rapid remission is required (e.g. spinal cord compression)
Surgery may be useful
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