Bones, Joints, and Soft Tissue Tumors Flashcards
aWhat is the precursor cell of osteoblasts?
Osteoprogenitor cells
What is the precursor cell of osteoclasts?
Fusion of Preosteoclasts
(that’s how they become giant, multinucleate cells)
What is the function of osteoprotegerin?
- Blocks RANK ligand
- Can’t bind RANK receptor on preosteoclast
- Prevents bone resorption by inhibiting differentiation
NOTE:
- proteger = to protect in spanish
- Osteo- = bone
- Osteoprotegerin = bone protector
Where is woven bone normally seen?
- Fetal skeleton
- Pathologic state in adults
- healing fractures
- Infection
- Tumor
What is the major protein present in bone?
Collagen type I
Osteocalcin is produced and used as a marker of activity of which cell?
Osteoblasts
What genes control skeletal morphogenesis?
Homeobox genes
When in development does endochondral ossification begin?
8th week of gestation
How does bone enlargement occur?
Appositional growth
- deposition of new bone on a pre-existing surface
What are dysostoses? How do they occur?
- Dysosteogenesis = defective bone formation
- Localized problems in migration of mesenchymal cells and formation of condensations
What is synpolydactyly? What causes this?
- Def
- extra digit between 3rd and 4th fingers
- some syndactyly
- Cause
- mutation in homeobox gene
- defective transcription factor
Achondroplasia
- Mutation
- Symptoms
- Mutation
- in FGF receptor (constant stimulation)
- inhibits chondrocyte proliferation
- Premature deposition of horizontal struts of bone seals growth plates and inhibits growth
- Symptoms
- shortened proximal extremities
- Enlarged head
- Normal trunk length
Thanatophoric dwarfism
- Mutation
- Symptoms
- Mutation
- FGF receptor gene
- Symptoms
- Dwarfism
- Bell-shaped abdomen
- Small chest cavity
- leads to respiratory insufficiency and death
Osteogenesis Imperfecta
- Mutation
- Symptoms
- aka Brittle bone disease
- Mutation
- defects in synthesis of Type I collagen
- Symptoms (general)
- too little bone
- Osteoporosis with
- cortical thinning
- attenuation of trabeculae
What symptoms are associated with Osteogenesis imperfecta type I?
- Increased risk of fractures
- Blue sclerae
- hearing loss
- Small, mis-shapen, blue-yellow teeth
What symptoms are associated with Osteogenesis imperfecta type II?
- Multiple fractures
- Fatal in utero
What symptoms are associated with Osteogenesis imperfecta type III?
Progressive, deforming disease
- Growth retardation
- Multiple fractures
- Hearing loss
- Tooth irregularities
What symptoms are associated with Osteogenesis imperfecta type IV?
- Postnatal fractures
- Normal Sclerae
- Moderate skeletal fragility
- Short stature
Achondrogenesis II
- Mutation
- Symptoms
- Mutation
- Type II collagen
- Symptoms
- Short trunk
- Short extremities
- Enlarged cranium
- Flattened face
Schmid Metaphyseal Chondrodysplasia
- Mutation
- Symptoms
- Mutation
- type 10 collagen defect
- Symptoms
- short stature
- Bowing of lower extremities
- **Coxa Vera **(deformity of hip joint)
- Abnormal metaphysis
Patient presents with history of fractures, anemia, increased infections, and hepatosplenomegaly. Radiography shows bones with symmetric sclerosis and an erlenmeyer flask deformity. What is the disease? What is the cause of disease? What causes the symptoms?
- Disease
- Osteopetrosis
- Cause of disease
- Reduced osteoclast activity so reduced bone resorption
- Type II Carbonic anhydrase deficiency
- Failure to excrete H+ to solubilize hydroxyapatite
- Cause of symptoms
- Bones lack a medullary canal
- decreased hematopoiesis
- Extramedullary hematopoiesis
- hepatosplenomegaly
- Bones lack a medullary canal
Osteopetrosis
- Characteristics
- Symptoms
- Cause
- Characteristics
- symmetric sclerosis
- erlenmeyer flask deformity
- ends are bulbous and misshapen
- lack medullary canal
- Neural foramina compress exiting nerves
- Symptoms
- Fractures
- anemia
- increased infections
- hepatosplenomegaly
- Cause
- Reduced osteoclast activity so reduced bone resorption
- Type II Carbonic anhydrase deficiency
- Failure to excrete H+ to solubilize hydroxyapatite
Post-menopausal woman presents with height loss and history of fractures. Her bones are found to have a reduced mass and increased porosity. Radiograph shows a collapsed vertebra. What is the disease? What is the cause? What is the possible complication?
- Disease
- Post-menopausal osteoporosis
- Cause
- Estrogen deficiency increases secretion of proinflammatory cytokines
- Increased activity of osteoclasts
- Complication of fracture
- Pulmonary emboli
What is the difference in bone structure in post - menopausal and senile osteoporosis?
- Post-menopausal
- Trabeculae are thinned
- Mainly affects bone that has increased surface area
- vertebral bodies
- Senile
- Cortex is thinned
- Haversian systems are widened
What is the difference in cause of post - menopausal and senile osteoporosis?
- Post-menopausal
- Estrogen deficiency
- increased inflammatory cytokines
- (+) osteocyte activity
- Estrogen deficiency
- Senile
- decreased replication and activity of osteoblasts
- reduced physical activity
Patient presents with localized bone pain, joint pain in the knees, and history of fractures. Labs show elevated serum alkaline phosphatase and increased urine hydroxyproline. Histology shows a mosaic pattern of bone with increased bone mass. What is the disease? What is the cause?
- Disease
- Paget disease
- Cause
- high concentration of IL-6 and M-CSF
- induces osteoclast activity
- high concentration of IL-6 and M-CSF
Paget disease
- Cause
- Signs
- Symptoms
- Cause
- High concentrations of IL-6 and M-CSF
- induces osteoclast activity
- High concentrations of IL-6 and M-CSF
- Signs
- Increased bone mass
- mosaic pattern
- Elevated serum alkaline phosphatase
- Increased urine hydroxyproline
- Increased bone mass
- Symptoms
- Pain localized to affected bone
- microfractures
- compression of nerves
- Osteoarthritis in weight-bearing joints
- Pain localized to affected bone
What are the stages of Paget disease? What is the net effect?
- Osteolytic
- active bone resorption
- Osteoclastic-osteoblastic
- ends in bone formation
- Quiescent osteosclerotic phase
Net effect: increased bone mass that is disordered and unsound
What is the cause of Primary Hyperparathyroidism?
Adenoma
What is the cause of Secondary Hyperparathyroidism?
Prolonged hypocalcemia
(results in increased PTH secretion)
Osteitis Fibrosa Cystica
- Cause
- Signs
- Symptoms
- Cause
- Hyperparathyroidism
- uncontrolled bone resorption
- Signs
- Osteopenia (decreased density)
- Replacement of marrow spaces with fibrovascular tissue
- Microfractures
- Hemorrhage
- Cell influx
-
Brown tumor
- result of hemorrhage
- Symptoms
- Pain
What is the pathogenesis of Renal osteodystrophy?
- Chronic renal failure
- Phosphate retention
- Hyperphosphatemia
- Secondary hyperparathyroidism
- Phosphate retention
- Causes bone resorption
What is the difference between an closed and a compound fracture?
- Closed
- overlying tissue intact
- Compound
- break through skin
What is a comminuted fracture?
Bone is splintered
What is a displaced fracture?
Ends of bone are not aligned
What is the mechanism of fracture repair?
- Fibrin clot seals fracture site
- creates scaffolding for cells and angiogenesis
- Organization of hematoma
- Soft tissue callus or Procallus formed
- uncalcified tissue
- Chondroblasts make cartilage along fracture line
- undergoes endochondral ossification
- Bony callus formation
- Mineralization and resorption of callus sections not physically stressed
What is the most common cause of osteonecrosis?
Corticosteroid use
What is the difference between medullary and subchondral infarcts in osteonecrosis?
- Medullary
- Involve cancellous bone and marrow
- Subclinical
- Subchondral
- Wedge-shaped
- Results in chronic pain
How is osteonecrosis repaired?
Creeping substitution
- Not effective
- Results in collapse of necrotic bone
What is the most common cause of osteomyelitis?
Staph. aureus