Bone & Joint Final
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- This is a chronic disorder of the adult skeleton in which normal osseous tissue is replaced by a highly vascular fibrous osteoid.
- Pagets Disease
- What is the other name for Paget's Disease?
- Osteitis Deformans
- What are the four phases of Paget's Disease?
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1. Osteolytic Phase
2. Active Osteolysis/Osteogenesis Phase
3. Inactive Osteoblastic/Sclerotic Phase
4. Malignant Degeneration Phase - Describe the osteolytic phase of Paget's Disease.
- Haphazard osteoclastic resorption of cortical and cancellous bone
- In Paget's Disease, what part of the body is most commonly affected?
- The axial skeleton (pelvis, sacrum, skull, spine). Also the femur.
- In Paget's disease, destroyed bone is replaced by what?
- fibrous tissue
- In the Active Osteolysis/Osteogenesis phase of Paget's Disease, what is bone replaced by?
- Vascular connective tissue
- What is the pathognomic indication for Paget's Disease?
- A "Tile-like or mosaic pattern" of cortical and cancellous bone.
- What disease leaves bone poorly organized, lacking structural strength (ie, soft, porous, undermineralized)?
- Paget's Disease
- What disease is associated with increased thickness of both cortical and cancellous bone (increased overall size)?
- Paget's Disease
- What disease has a phase where inactive osteoblastic/sclerosis occurs?
- Paget's Disease
- Bones are "light, soft, porous, and almost have the consistency of dry bread." What is it?
- Paget's Disease
- Bone increases in volume in this phase of Paget's Disease.
- Inactive Osteoblastic/Sclerotic phase
- A very small percentage (.9 - 2%) of Paget's Disease patients experience what type of degeneration of bone?
- Malignant degeneration
- In what areas of the body will malignant degeneration occur in Paget's Disease?
- Femur, humerus, ilia, skull, tibia
- Most patients with Paget's Disease are symptomatic. True or False?
- False. Most patients are asymptomatic
- What disease progresses slowly over years, and is often an incidental finding?
- Paget's Disease
- This disease insidiously results in pain, stiffness, fatigability, deformity, headaches, loss of auditory acuity, and increased skull size.
- Paget's Disease
- What are the clinical signs of a patients with Paget's Disease?
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- Most asymptomatic
- Progresses slowly over years
- Bone pain is deep and aching
- Because of increased blood flow, surrounding skin is warm - Paget's disease affects what areas of the body?
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- Pelvis/sacrum
- femur
- skull
- vertebrae
- tibia
- clavicles
- humerus - What six deformities are associated with Paget's Disease?
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1. Bowing deformities of long bones
2. Enlarged calvarium (skull cap)
3. Basilar invagination with brain stem compression
4. Flattened vertebral bodies
5. Kyphosis, sloliosis
6. Hobbling gait - Pathological fractures, pseudofractures, and spinal stenosis are associated with what disease of bone?
- Paget's Disease
- In Paget's disease, what are the other terms used to describe the pseudofractures?
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- "Loosers lines"
- "Milkman's syndrome" - Why do pseudofractures occur in Paget's Disease?
- Local demineralization causes Paget's Disease
- If you were to take an X-ray on someone with Paget's Disease, what six things might you see?
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1. Changes in density
2. Cortical thickening
3. Bone expansion
4. Pathological fractures
5. Bowing deformities
6. Pseudofractures - If you did a Lab on someone with Paget's Disease, what might you find?
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- Increased alkaline phosphatase activity (indicative of osteoblastic activity)
- Urinary excretion of hydroxyproline when collagen is destroyed
- Normal serum calcium - What pathology has a slow physiological resorption of normal bone, replaced by fibrous tissue, mixed with haphazardy arranged trabeculae.
- Fibrous Dysplasia
- What disease is known as the "great imitator of bone?"
- Fibrous Dysplasia
- In Fibrous Dysplasia, what is the average age of onset?
- 8-14 years old
- What bone pathology develops lesions during skeletal growth?
- Fibrous Dysplasia
- McCune -Albright syndrome is associated with what bone disorder?
- Fibrous Dysplasia
- McCune-Albright Syndrome affects mostly what gender?
- Females
- In Fibrous Dysplasia, what is the etiology? What is happening to bone?
- There is an aberrant maturation of bone. Bone does not mature properly.
- In Fibrous Dysplasia, where are the bone defects typically found?
- In the medullary cavity.
- What is the appearance of bone in Fibrous Dysplasia?
- There is a disorganized ground-glass appearance.
- What disease has a pathological finding where the normal bone cortex erodes from within? What is that erosion called?
- Fibrous Dysplasia. Endosteal Scalloping.
- What is the characteristic pathological & radiographic finding of fibrous and cartilaginous lesions of bone?
- Endosteal scalloping
- Endosteal scalloping is found in Fibrous Dysplasia, but what other bone disease is associated with endosteal scalloping?
- Chondrosarcoma
- A thin shell of normal cortex can be found in what bone pathology?
- Fibrous Dysplasia
- The lesion expands and deforms bone, but does not break the cortex in what bone pathology?
- Fibrous Dysplasia
- What are 5 pathological findings in Fibrous Dysplasia?
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1. Edosteal Scalloping
2. Spiculated bone
3. Thin shell of normal cortex
4. Disorganized ground-glass appearance
5. Expands and deforms bone, but does not break the cortex - What are the three clinical patterns for Fibrous Dysplasia?
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1. Monostotic fibrous dysplasia
2. Polyostotic fibrous dysplasia
3. Polyostotic fibrous dysplasia with endocrine abnormality - In Fibrous Dysplasia, McCune-Albright syndrom is presents with what signs?
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1. Skin pigmentation (cafe au lait spots)
2. Early onset of sexual development (irregular menses, secondary sexual characteristics at 5-6 YOA) - If you were in clinic doing a patient history, what might you see on the body or in x-rays in a person with the polyostotic form of Fibrous Dysplasia?
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1. bowing deformities which can cause pain and walking problems
2. pathological fractures
3. cranial enlargement
4. cafe au lait spots (with irregular margins: "coast of Maine" - If you were to look at an x-ray of a person with the polyostotic form of fibrous dysplasia, what are six things you might see?
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1. Radiolucent, loculated, irregular trabeculated lesions
2. ground-glass appearance of bone.
3. well demarcated "ring" of sclerosis
4. widened medullary cavity
5. bone expansion
6. pathological fractures - What are the other two names for Neurofibromatosis?
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1. von Recklinghausen's Disease
2. Elephant Man Disease - In Neurofibromatosis, what is the physiological presentation?
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1. Cafe au lait spots
2. Fibroma Molluscum
3. Osseous deformities of axial & appendicular skeleton
4. tumors of peripheral, optic, and acoustic nerves. - In Neurofibromatosis, describe what a fibroma molluscum lesion looks like.
- mutiple, soft, elevated cutaneous tumors
- In Neurofibromatosis, describe what cafe au lait spots look like.
- pigmented, cutaneous lesions
- Is Neurofibromatosis a congenital disease, or is it aquired from infection by a foreign organism?
- A congenital disease
- To be diagnosed with Neurofibromatosis, what is typically the number of cafe au lait spots found on the body, and of approximately what size?
- 6+ spots taht are equal to or greater than 1.5cm in diameter.
- What is the frequent and best diagnostic cutaneous feature of Neurofibromatosis?
- fibroma molluscum - soft, multiple, asymptomatic cutaneous tumors
- In a patient with Neurofibromatosis, describe the size of fibroma molluscum lesions.
- pinhead sized to greater than or equal to 5cm
- What is the most common skeletal feature found in Neurofibromatosis?
- Scoliosis (especially kyphoscoliosis of the lower thoracic spine and possible cord compression)
- A cervical kyphosis in Neurofibromatosis is uncommon...true or false?
- True. It is common
- If someone has Neurofibromatosis, there is no possibility for paraplegia...true or false?
- False. A Neurofibromatosis patient my have paraplegia.
- If a patient has Neurofibromatosis, what is a neurofibroma? What if there is a neurofibroma on the optic nerve? What might happen to the patient?
- It is a tumor or tumors on the nerves. For example, on the optic nerve, it can cause blurred vision, scotomas, and transient blindness.
- I patient with Neurofibromatosis can also get a acoustic neuroma. What can an acoustic neuroma cause?
- deafness
- If you were viewing an x-ray of a patient with Neurofibromatosis, what might you see?
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1. "dumb-bell" neurofibroma of spinal nerves
2. Enlarged IVF's (dumb-bell shaped)
3. scoliosis
4. kyphosis (in thoracic or cervical) - Describe Osteopenia.
- It is a radiographic finding of decreased bone density.
- Describe Osteoporosis.
- A decrease in total bone mass greater than what is expected for a given age, race, sex.
- In osteoporosis, what is the composition of osteoid to bone mineral?
- The bone that is present is of a normal oseoid to bone mineral ratio.
- What parts of the body is osteoporosis typically found?
- In the axial skeleton: vertebrae, ribs, pelvis
- In osteoporosis, what parts of the bone are lost?
- Cortical and trabecular bone are lost. There is simply less bone overall, even though the mineral to bone ratio is normal.
- If your patient had osteoporosis, what might be your clinical findings upon examination?
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1. Acute onset of mid-back pain
2. Decreased ROM, esp. in flexion
3. Decreased height of patient
4. Increased thoracic kyphosis and decreased lumbar lordosis
5. Palpable vertebral muscle spasms - What are the two main clinical indicators of osteoporosis?
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1. Decreased height of the patient
2. Increase thoracic kyphosis, and decreased lumbar lordosis - If your patient has osteoporosis, although there is a loss of mass of bone, the remaining bone is normal, therefore there is no chance for fractures...true or false?
- False
- If a patient with osteoporosis had fractures, where would they typically be found? Three locations.
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1. In the vertebrae (vertebral compression fractures)
2. Hip fractures
3. Distal radius fractures - If you were looking at your patient's x-ray, and he/she had osteoporosis, what would you likely see?
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1. Decreased bone density
2. Pencil thin cortex
3. Apparent increase in weight-bearing trabeculae
4. Multiple compression fractures - What is DEXA?
- It is the acronym for Dual Energy X-ray Absorptiometry
- What would be the best x-ray type to use if you wanted to take an x-ray of the bone density of a patient with osteoporosis?
- DEXA (Dual Energy X-ray Absorptiometry)
- What is the other name for Osteomalacia?
- Rickets
- Describe what happens to bone in osteomalacia?
- There is defective minerilation, leading to bone softening with a relative increase in osteoid.
- In osteomalacia, what is it also known as in children?
- Rickets
- What is the other name for rickets, but is used to describe the pathology in adults?
- Osteomalacia
- In a patient with osteomalatia, what is the problem that occurs in bone?
- There is a Vitamin D deficiency.
- In osteomalacia, there is a deficiency of Vitamin D. How does vitamin D contribute to bone mineralization?
- Vitamin D stimulates proteins to transport calcium from the lumen of the intenstine into the blood. It also maintains the balance of calcium and phosphorus in bone.
- If a patient came into the clinic with Rickets, what would be the presentation?
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1. Muscle tetany and weakness
2. Listlessness and irritability
3. Delayed skeletal development
4. Bone deformity - If a patient came into the clinic with Osteomalacia, what would be the presentation?
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1. Diffuse bone pain, tenderness to palpation
2. Muscle weakness
3. Skeletal deformities - If you took x-rays on a patient with rickets, what might you see?
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1. Diffuse osteopenia
2. Coarsened trabeculae
3. Cortical thinning
4. Bowing deformities
5. **Wide epiphyseal plate
6. **"Frayed" metaphysis - If you took x-rays on a patient with osteomalacia, what might you see?
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1. Diffuse osteopenia
2. Coarsened trabeculae
3. Cortical thinning
4. Bowing deformities
5. **Pseudofractures** - If you did a Lab test on a patient with osteomalacia/rickets, what might you find?
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1. Decreased serum phosphorus
2. Decreased serum calcium
3. Increased alkaline phosphatase - If you had a patient with osteomalacia/rickets, what might be your primary treatment?
- Give the patient a Vitamin D plan