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83 Cards in this Set
- Front
- Back
This is a chronic disorder of the adult skeleton in which normal osseous tissue is replaced by a highly vascular fibrous osteoid.
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Pagets Disease
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What is the other name for Paget's Disease?
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Osteitis Deformans
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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 |
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Describe the osteolytic phase of Paget's Disease.
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Haphazard osteoclastic resorption of cortical and cancellous bone
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In Paget's Disease, what part of the body is most commonly affected?
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The axial skeleton (pelvis, sacrum, skull, spine). Also the femur.
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In Paget's disease, destroyed bone is replaced by what?
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fibrous tissue
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In the Active Osteolysis/Osteogenesis phase of Paget's Disease, what is bone replaced by?
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Vascular connective tissue
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What is the pathognomic indication for Paget's Disease?
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A "Tile-like or mosaic pattern" of cortical and cancellous bone.
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What disease leaves bone poorly organized, lacking structural strength (ie, soft, porous, undermineralized)?
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Paget's Disease
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What disease is associated with increased thickness of both cortical and cancellous bone (increased overall size)?
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Paget's Disease
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What disease has a phase where inactive osteoblastic/sclerosis occurs?
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Paget's Disease
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Bones are "light, soft, porous, and almost have the consistency of dry bread." What is it?
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Paget's Disease
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Bone increases in volume in this phase of Paget's Disease.
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Inactive Osteoblastic/Sclerotic phase
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A very small percentage (.9 - 2%) of Paget's Disease patients experience what type of degeneration of bone?
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Malignant degeneration
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In what areas of the body will malignant degeneration occur in Paget's Disease?
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Femur, humerus, ilia, skull, tibia
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Most patients with Paget's Disease are symptomatic. True or False?
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False. Most patients are asymptomatic
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What disease progresses slowly over years, and is often an incidental finding?
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Paget's Disease
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This disease insidiously results in pain, stiffness, fatigability, deformity, headaches, loss of auditory acuity, and increased skull size.
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Paget's Disease
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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 |
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Paget's disease affects what areas of the body?
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- Pelvis/sacrum
- femur - skull - vertebrae - tibia - clavicles - humerus |
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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 |
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Pathological fractures, pseudofractures, and spinal stenosis are associated with what disease of bone?
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Paget's Disease
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In Paget's disease, what are the other terms used to describe the pseudofractures?
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- "Loosers lines"
- "Milkman's syndrome" |
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Why do pseudofractures occur in Paget's Disease?
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Local demineralization causes Paget's Disease
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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 |
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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 |
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What pathology has a slow physiological resorption of normal bone, replaced by fibrous tissue, mixed with haphazardy arranged trabeculae.
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Fibrous Dysplasia
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What disease is known as the "great imitator of bone?"
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Fibrous Dysplasia
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In Fibrous Dysplasia, what is the average age of onset?
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8-14 years old
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What bone pathology develops lesions during skeletal growth?
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Fibrous Dysplasia
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McCune -Albright syndrome is associated with what bone disorder?
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Fibrous Dysplasia
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McCune-Albright Syndrome affects mostly what gender?
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Females
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In Fibrous Dysplasia, what is the etiology? What is happening to bone?
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There is an aberrant maturation of bone. Bone does not mature properly.
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In Fibrous Dysplasia, where are the bone defects typically found?
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In the medullary cavity.
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What is the appearance of bone in Fibrous Dysplasia?
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There is a disorganized ground-glass appearance.
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What disease has a pathological finding where the normal bone cortex erodes from within? What is that erosion called?
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Fibrous Dysplasia. Endosteal Scalloping.
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What is the characteristic pathological & radiographic finding of fibrous and cartilaginous lesions of bone?
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Endosteal scalloping
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Endosteal scalloping is found in Fibrous Dysplasia, but what other bone disease is associated with endosteal scalloping?
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Chondrosarcoma
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A thin shell of normal cortex can be found in what bone pathology?
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Fibrous Dysplasia
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The lesion expands and deforms bone, but does not break the cortex in what bone pathology?
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Fibrous Dysplasia
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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 |
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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 |
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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) |
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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" |
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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 |
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What are the other two names for Neurofibromatosis?
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1. von Recklinghausen's Disease
2. Elephant Man Disease |
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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. |
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In Neurofibromatosis, describe what a fibroma molluscum lesion looks like.
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mutiple, soft, elevated cutaneous tumors
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In Neurofibromatosis, describe what cafe au lait spots look like.
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pigmented, cutaneous lesions
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Is Neurofibromatosis a congenital disease, or is it aquired from infection by a foreign organism?
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A congenital disease
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To be diagnosed with Neurofibromatosis, what is typically the number of cafe au lait spots found on the body, and of approximately what size?
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6+ spots taht are equal to or greater than 1.5cm in diameter.
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What is the frequent and best diagnostic cutaneous feature of Neurofibromatosis?
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fibroma molluscum - soft, multiple, asymptomatic cutaneous tumors
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In a patient with Neurofibromatosis, describe the size of fibroma molluscum lesions.
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pinhead sized to greater than or equal to 5cm
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What is the most common skeletal feature found in Neurofibromatosis?
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Scoliosis (especially kyphoscoliosis of the lower thoracic spine and possible cord compression)
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A cervical kyphosis in Neurofibromatosis is uncommon...true or false?
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True. It is common
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If someone has Neurofibromatosis, there is no possibility for paraplegia...true or false?
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False. A Neurofibromatosis patient my have paraplegia.
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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?
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It is a tumor or tumors on the nerves. For example, on the optic nerve, it can cause blurred vision, scotomas, and transient blindness.
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I patient with Neurofibromatosis can also get a acoustic neuroma. What can an acoustic neuroma cause?
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deafness
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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) |
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Describe Osteopenia.
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It is a radiographic finding of decreased bone density.
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Describe Osteoporosis.
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A decrease in total bone mass greater than what is expected for a given age, race, sex.
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In osteoporosis, what is the composition of osteoid to bone mineral?
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The bone that is present is of a normal oseoid to bone mineral ratio.
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What parts of the body is osteoporosis typically found?
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In the axial skeleton: vertebrae, ribs, pelvis
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In osteoporosis, what parts of the bone are lost?
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Cortical and trabecular bone are lost. There is simply less bone overall, even though the mineral to bone ratio is normal.
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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 |
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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 |
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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?
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False
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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 |
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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 |
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What is DEXA?
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It is the acronym for Dual Energy X-ray Absorptiometry
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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?
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DEXA (Dual Energy X-ray Absorptiometry)
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What is the other name for Osteomalacia?
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Rickets
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Describe what happens to bone in osteomalacia?
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There is defective minerilation, leading to bone softening with a relative increase in osteoid.
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In osteomalacia, what is it also known as in children?
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Rickets
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What is the other name for rickets, but is used to describe the pathology in adults?
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Osteomalacia
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In a patient with osteomalatia, what is the problem that occurs in bone?
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There is a Vitamin D deficiency.
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In osteomalacia, there is a deficiency of Vitamin D. How does vitamin D contribute to bone mineralization?
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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.
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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 |
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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 |
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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 |
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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** |
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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 |
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If you had a patient with osteomalacia/rickets, what might be your primary treatment?
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Give the patient a Vitamin D plan
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