Paget’s Disease Flashcards
History leading to diagnosis of Paget’s disease
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
Examination in Paget’s disease
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)
Paget’s investigations
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
Radiological findings in Paget’s Disease
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
Indications for treatment of Paget’s disease
Bone pain
Progressive deformity
Complications e.g. neural compression or high-output cardiac failure
Prelude to orthopaedic surgery
Treatment of Paget’s disease
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