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Metabolic Bone Diseases
Osteopetrosis (Marble Bone Disease, Albers-Schonberg Disease) Paget’s Disease (Osteitis Deformans)
Definition Definition
Rare Hereditary Disease Periods of Bone Re sorption followed by Hectic Bone Formation
↓ Osteoclast Bone Resorption Later, Bone Cell Activity becomes markedly Diminished
Result in Diffuse Symmetrical Skeletal Sclerosis Result in ↑ Bone Mass
Brittle Bone, Fracture (like a piece of chalk) (but Newly Formed Bone Disordered)(Abnormal Architecture)
Types Common in Whites (England, France, Austria)
Malignant Benign Not Serious Life Threatening Disease in Absence of Malignant Transformation
Autosomal Recessive Autosomal Dominant 3 Phases
Morphology Osteosclerotic
Osteolytic Mixed Osteoclastic – Osteoblastic
Precise Nature (Remains Unknown) (Burnt Out)
Deficient Osteoclast Activity Ends with predominance of
• Bones Lack a Medullary Canal Osteoblastic Activity
• End of Long Bones – Bulbou s, Misshapen Pathogenesis
Initial Lytic Phase
1° Spongiosa Persists ↓
Fills in Medullary Cavity → No Hemopoetic Marrow Waves of Osteocla sts, Numerous Reso rptive P its
Osteoclasts Abnormal ly Large (up to 100 nucle i)
(Prevent Mature Trabeculae) ↓
Mixed Phase
Bones – Not Remodelled, Woven in Architecture ↓
Osteosclerotic P hase
Detected in Utero, Soon after Birth Detected till Adolescent, Adulthood ↓
Morphology
Paget’s Disease
Paget’s Disease
Paget’s Disease
Mosaic Pattern of Lamellar Bone
Prominent Cement Lines
Jigsaw Puzzle Like
Clinical Course
One or More Bones
Sites – Axial Skeleton, Proximal Femur (up to 80%)
Unusual Sites – Ribs, Fibula, Small Bones of Hands
Clinical Manifestations
Bone Pain
Joint Pain
Deformity
Spontaneous Fractures
Alk Phos ↑
[Ca2+] Normal Unless I mmobilised
Complications
Fractures
2° Osteoarthritis (OA)
Bowing of Femoral, Tibia Bone
Nerve Entrapment (2° to Compression Fracture of Spine)
Spinal Stenosis
Cardiac Failure (↑ Blood Flow – behaves as AV shunt)
Bone Sarcoma – Osteosarcoma, MFH, Chondrosarcoma
Hypercalcaemia
Rickets Rickets
Normal Osteomalacia