Term
| Paget's Disease is aka __ ___. |
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Definition
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Term
| The disease begins in middle age or later and usually affects the __ __ of the __ and the __. The bones __ and __, and those bearing weight become unnaturally ___. Also hat size tends to __. |
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Definition
- long bones of the leg
- skull
- enlarge and soften
- curved
- increase |
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Term
| Recent evidence suggests there are __ and __ factors that lead to Paget's Disease. |
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Definition
| - genetic and environmental |
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Term
| Paget's disease is uncommon before age __. __ to __% of Paget's disease patients have a positive family history. |
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Definition
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Term
| The second most common bone disease after osteoporosis is __ __. |
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Definition
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Term
| Paget's Disease is most common in people of __ __ descent and affects roughly 2% of the population over age of 55. |
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Definition
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Term
| Paget's in patients over the age of 85 is 5 times more common than in patients younger than 60. Incidence greatly increases with age. |
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Definition
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Term
| Paget's is more prevalent in the ___ United States than in the South. |
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Definition
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Term
| male:female ratio for Paget's is about equal, though some studies show more common in men |
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Definition
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Term
| Classical Paget's Disease is autosomal ___ . |
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Definition
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Term
| Paget's disease is a chronic progressive skeletal disorder in which there are __ __ and __ of ___. There are localized areas of excessive __ __ and ___. Pts may have only one affected bone or have multiple affected bones. New lesions __ develop in previously unaffected bone after diagnosis. |
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Definition
- increased number and size of osteoclasts
- bone resorption and formation |
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Term
| Osteoclasts in Paget's disease are numerous and too large. They are morphologically abnromal b/c they have __ __. |
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Definition
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Term
Pagetic osteoclasts are both numerous and morphologically abnormal, containing multiple nuclei.
Increased numbers of osteoblasts are recruited to the sites of resorption, causing increased bone formation. Subsequently, bone formation is also increased because of the increased numbers of morphologically normal osteoblasts.
The high population of osteoblasts rapidly deposits new bone at the affected site. The new bone is disorganized and structurally weak.
Patients may have only one affected bone (monostotic disease), or there may be pagetic lesions in multiple bones (polyostotic disease).
The disease remains localized. Patients rarely develop lesions at previously unaffected sites after the diagnosis has been made. |
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Definition
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Term
Paget's is usually ___, may be incidental finding b/c found serum ___ ___ or something on x ray. May have __, ____, ____, __ ___, __ __, and ___.
Pagetic Bone Is Weaker Than Normal Bone |
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Definition
- asymptomatic
- alkaline phosphatase
- pain, fracture, deformity, hearing loss, dental complaints, and osteoarthritis |
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Term
| More signs of Paget's Disease: |
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Definition
- Pain related to osteoarthritis in a hip or knee adjacent to a pagetic bone is a common complaint among patients with Paget’s disease.1
- Bone pain, when present, is often mild to moderate; it often persists during the night, a feature that generally differentiates it from osteoarthritis.1
- Pain in the tibia or femur may be aggravated by weight bearing.1
- Dental problems such as loosening of the teeth or swollen, infected gums can occur in patients with Paget’s disease in the skull.4 |
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Term
| Paget's disease can occur in any bone, but is most commonly in the: |
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Definition
- skull
- vertebrae
- pelvis
- femur
- tibia |
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Term
| It is important to note that alkaline phosphatase can be elevated in other disorders, notably vitamin D deficiency, osteomalacia, and gall bladder and liver disease, which must be ruled out. |
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Definition
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Term
| Radiographic findings of Paget’s disease are characteristic and rarely confused with x-ray findings suggestive of other diseases. Osteolytic lesions in long bones advance upward or downward at about 1 cm/y.1 As Paget’s disease progresses, there is cortical thickening, loss of corticomedullary distinction, and accentuated trabecular markings. The last phase of Paget’s disease is primarily sclerotic, with enlargement and thickening of long bones.3 |
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Definition
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Term
Plain radiographs of Paget’s lesions are usually confirmatory and rarely confused with x-ray findings of other conditions. Scintigraphic bone scans may be used to assess the extent of skeletal involvement.1,2
References |
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Definition
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Term
| The early stage of Paget's disease is called the ___ stage. __ __, __ __ __ lesions are typical of this stage. These lesions advance up or down the bone about __cm/year. |
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Definition
- Lytic stage
- V-shaped, blade of grass lesions
- 1 cm/year |
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Term
| With Advanced Paget's Disease you will see __ and __ lesions. __ lesions will be seen by __ and __ of the long bone affected. |
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Definition
- sclerotic and lytic lesions
- sclerotic
- enlargement and thickening |
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Term
| As Paget's disease progresses, the __ become __ and __ and the bone __ and __. |
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Definition
- cortices become trabeculated (accentuated) and thickened
- bone broadens and bows |
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Term
| CORTICAL THICKENING WITH PAGETS AND BOWING OF BONES |
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Definition
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Term
| Early lytic Paget's Disease of the skull is known as __ __. A typical lesion of this would be a __ __ border. |
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Definition
Osteoporosis circumscripta
lytic resorptive border |
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Term
| Advanced Sclerotic Paget's Disease of the Skull is known as __ __ __. |
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Definition
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Term
This radiograph shows characteristic pagetic changes, such as enlargement or expansion of bone and sclerotic changes.
Radiographs can also reveal osteoarthritis in joints adjacent to pagetic bones and identify fissure fractures, and demonstrate the degree to which lytic or sclerotic disease predominates. |
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Definition
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Term
| Paget's disease in the vertebrae can result in a __ __ appearance, where the cortex frames a lytic lesion. |
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Definition
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Term
| Physical exam of a patient with Paget's may reveal: |
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Definition
Skeletal deformity
Abnormal gait
Elevated temperature, erythema, or tenderness over affected bone
Hearing loss
Dilated scalp veins
High-output cardiac failure
Neurological findings |
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Term
| Because pagetic bone is highly vascularized, there is ___ blood flow to the entire area of involvement. Rarely, the increased demand for blood places enough stress on the heart to precipitate congestive heart failure.2 |
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Definition
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Term
| Typical changes in the skull associated with Paget’s disease include diffuse enlargement, and dilated scalp veins. Some patients may show frontal bossing or knobby deformity, or there may be enlargement of the maxilla or mandible. |
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Definition
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Term
| Lateral and/or anterior bowing also occurs in the long bones of the arm |
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Definition
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Term
| Complications of Paget's Disease: |
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Definition
Osteoarthritis adjacent to affected bones, particularly in the hips
Fracture (complete, fissure, vertebral compression)
Neurologic
Cardiac
Neoplastic (Rare) Osteosarcoma Rare finding (<1%) Giant Cell tumors (benign) |
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Term
| Neurological Disorders that may result from Paget's Disease: |
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Definition
Hearing deficit
Cranial nerve deficits
Mottled retinal degeneration;angioid streaks
Basilar impression
Hydrocephalus
Myelopathy
Radicular neuropathies
Spinal stenosis
Spinal vascular steal syndrome |
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Term
| Hearing loss associated with Paget's occurs with ___ bone involvment. There is __ __ sensory loss and ___ __ conductive loss. It is __ and __. |
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Definition
- temporal bone
- high frequency sensory loss
- low frequency conduction loss
- bilateral and progressive |
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Term
| An angioid streak is a crack in __ membrane.This occurs with Paget's disease. These are also seen with d/o called pseudoxanthoma elasticum. |
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Definition
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Term
| Cardiovascular complications associated with Paget's Disease: |
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Definition
- increased cardiac output
- congestive heart failure
- generalized atherosclerosis
- aortic valve caclifications
- endocardial calcifications |
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Term
| Cardiac output increases proportionally with degree of Paget's disease. This is due to the increased __ of the bone and overlying muscle and skin that is affected. |
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Definition
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Term
| Neoplastic complications from Paget's Disease: |
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Definition
- Sarcoma (osteosarcoma, chondrosarcoma, fibrosarcoma)
- Benign giant cell tumor |
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Term
| Paget's disease leading to osteosarcoma- death either from __ __ or __ __. |
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Definition
local extension
pulmonary metastases |
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Term
| Personal impact of paget's disease: |
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Definition
Self-report survey of 958 persons with Paget’s disease revealed:
Frequent comorbidities
Arthritis/arthrosis (64%)
Hypertension (32%)
Heart problems (28%)
Nearly half of patients (47%) reported depression
44% reported their health as “fair” or “poor”
Only 21% reported their quality of life was “very good” or “excellent” |
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Term
| Gold standard of antipagetic therapy: |
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Definition
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Term
| if you were treating a paget's disease pt what would you give them: |
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Definition
bisphosphonates
pain management: NSAIDs, COX-2 inhibitors, Opioids, analgesics |
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Term
| indications for treatment of Paget's disease: |
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Definition
Bone pain
Preparation for orthopedic surgery
Fracture of pagetic bone
Hypercalcemia and/or hypercalciuria
Neurologic deficit associated with cranial or vertebral disease
Presence of high-output congestive heart failure
Prevention of future complications |
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Term
| The use of salmon calcitonin involves continuous long-term therapy by injection. The most prominent biologic action of salmon calcitonin is a reduction of osteoclastic activity, thereby decreasing bone resorption. There is also a significant analgesic effect on bone pain. The most impressive effect is healing of osteolytic lesions. Nasal spray salmon calcitonin has not been shown to be consistently effective in treating Paget's disease |
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Definition
| should be given subcu or IM |
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Term
| contraindications to bisphosphonates: |
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Definition
Hypocalcemia
Vitamin D deficiency
Hypoparathyroidism
Severe renal insufficiency |
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Term
| adverse effects of bisphosphonates: |
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Definition
UGI intolerance, diarrhea (oral)
Acute phase reaction
Musculoskeletal/bone pain
Hypocalcemia
Renal failure reported with high dose intravenous bisphosphonates (obtain serum creatinine level before each dose of intravenous bisphosphonate)
Ocular inflammation (rare)
Osteonecrosis of jaw (ONJ) -rare |
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Term
Correct vitamin D deficiency before administering a bisphosphonate
Instruct patients to take 1500 mg of calcium and 800 units of vitamin D (preferably vitamin D3) daily during the 10 days after an infusion of zoledronic acid |
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Definition
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Term
| Bisphosphonate-associated ONJ has predominantly occurred in cancer patients receiving IV bisphosphonates4-7 |
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Definition
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Term
| Follow-Up for patients with Paget's: |
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Definition
Untreated Patients
Annual serum total or bone specific alkaline phosphatase measurement
Periodic x-rays of osteolytic lesions
Treated Patients
Serum total or bone specific alkaline phosphatase measurement every 3-6 months
Bone resorption markers are optional
Periodic x-rays of osteolytic lesions |
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