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83 Cards in this Set

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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?
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?
- 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?
- Pelvis/sacrum
- femur
- skull
- vertebrae
- tibia
- clavicles
- humerus
What six deformities are associated with Paget's Disease?
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?
- "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?
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?
- 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?
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?
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?
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?
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?
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?
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?
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?
1. von Recklinghausen's Disease
2. Elephant Man Disease
In Neurofibromatosis, what is the physiological presentation?
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?
If you were viewing an x-ray of a patient with Neurofibromatosis, what might you see?
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?
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?
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?
If a patient with osteoporosis had fractures, where would they typically be found? Three locations.
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?
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?
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?
What is the other name for rickets, but is used to describe the pathology in adults?
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?
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?
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?
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?
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?
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