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107 Cards in this Set
- Front
- Back
what type of ossification is not affected in achondroplasia?
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membranous - skull, facial bones, and axial skeleton are normal
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Heberden's nodes (DIP) and Bouchard's nodes (PIP)
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osteoarthritis
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pain in weight-bearing joints after use that improves with rest
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osteoarthritis
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destruction of articular cartilage, subchondral bone formation, sclerosis, osteophytes, and eburnation are associated with what?
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osteoarthritis
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what are joint mice? what are they associated with?
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osteophytes that fracture and float into synovial fluid along with fragments of separated cartilage - seen in osteoarthritis
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pannus formation in joints, especially MCP, PIP
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rheumatoid arthritis
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morning stiffness improving with use; symmetric joint involvement, and systemic symptoms (fever, fatigue, pleuritis, pericarditis)
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rheumatoid arthritis
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Rx for severe cases of osteoporosis?
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bisphosphonates or pulsatile PTH
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type I osteoporosis?
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postmenopausal - increased bone resorption due to decreased estrogen levels
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type II osteoporosis?
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senile osteoporosis - affects men and women over 70
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what types of fractures are associated with type I osteoporosis?
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vertebral crush fractures - acute back pain, loss of height, kyphosis
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what type of fractures are associated with type II osteoporosis?
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distal radius (Colles') fractures, vertebral wedge fractures
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in this disease there is a failure of normal bone resorption that leads to thickened, dense bones
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osteopetrosis (marble bone disease)
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in this disease osteoclasts function abnormally
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osteopetrosis
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what level of alk phos is osteopetrosis associated with?
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normal
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what causes osteitis fibrosa cystica?
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hyperparathyroidism
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this disease is characterized by 'brown tumors', high serum calcium, low serum phosphorus, and high alk phos
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osteitis fibrosa cystica
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these are cystic spaces lined by osteoclasts, filled with fibrous stroma and sometimes blood
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brown tumors (seen in osteitis fibrosa cystica)
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in this disease there is abnormal bone architecture caused by an increase in both osteoblastic and osteoclastic activity
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Paget's disease (osteitis deformans)
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what level of alk phos is Paget's disease associated with?
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increased (normal serum calcium, phosphorus, and PTH)
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in this disease, bone is replaced by fibroblasts, collagen, and irregular bony trabeculae
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polyostotic fibrous dysplasia
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Albright's syndrome is a form of what?
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polyostotic fibrous dysplasia
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multiple unilateral bone lesions, unilateral pigmented skin lesions, precocious puberty
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Albright's syndrome
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pain and stiffness in shoulders and hips, often with fever, malaise and weight loss; doesn't cause muscular weakness
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polymyalgia rheumatica
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what is polymyalgia rheumatica associated with?
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temporal (giant cell) arteritis; occurs in patients over 50
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is ESR increased or decreased in polymyalgia rheumatica?
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increased
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progressive proximal muscle weakness caused by CD8+ T cell-induced injury to myofibers
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polymyositis
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muscle biopsy with evidence of inflammation is diagnostic for what?
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polymyositis
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increased CK, increased aldolase, and positive ANA, anti-Jo-1
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polymyositis/dermatomyositis
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Raynaud's, arthralgias, myalgias, fatigue, esophageal hypomotility
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mixed CT disease
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antibodies to U1RNP?
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mixed connective tissue disease
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what is the classic triad of Sjogren's syndrome?
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xerophthalmia, xerostomia, arthritis
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patients with Sjogren's syndrome are at increased risk for what?
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B cell lymphoma
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what is enlarged in Sjogren's syndrome?
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parotids
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what patients does Sjogren's primarily affect?
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females between 40-60
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autoantibodies to ribonucleoprotein antigens, SS-A (Ro), SS-B (La)
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Sjogren's
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fever, fatigue, weight loss, nonbacterial verrucous endocarditis, hilar adenopathy, and Raynaud's
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SLE
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what are the kidney findings in SLE?
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wire loop lesions in kidney with immune complex deposition (with nephrotic syndrome)
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what part of the SI does celiac sprue tend to affect?
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jejunem
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what skin finding is celiac sprue associated with?
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dermatitis herpetiformis
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what malignancy is celiac sprue most strongly affiliated with?
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T cell lymphoma
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blunting of villi and lymphocytes in the lamina propria are suggestive of what?
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celiac sprue
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what type of crystals precipitate in gout?
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monosodium urate
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what type of diuretics is gout associated with?
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thiazide
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needle shaped negatively birefringent
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gout
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with what type of crystals is pseudogout associated?
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calcium pyrophosphate
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rhomboid crystals that are weakly positively birefringent
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pseudogout
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what type of joints does pseudogout usually affect?
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large joints, especially the knee
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what is the treatment for pseudogout?
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none
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pulmonary hemorrhages leading to hemoptysis, renal lesions leading to hematuria, anemia, crescentic glomerulonephritis
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Goodpasture's syndrome
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what type of hypersensitivity reaction does Goodpasture's represent?
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type II
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what population does Goodpasture's usually affect?
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men 20-40
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what level is elevated in sarcoidosis?
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ACE
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immune-mediated, widespread noncaseating granulomas common in black females
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sarcoidosis
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what do the epithelial granulomas contain in sarcoidosis?
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microscopic Schaumann and asteriod bodies
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why is hypercalcemia associated with sarcoidosis?
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elevated conversion of vitamin D to its active form in epithelioid macrophages
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restrictive lung disease, bilateral hilar lymphadenopathy, erythema nodosum, Bell's palsy are associated with what?
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sarcoidosis
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patients with what disorder have decreased sensitivity and often anergy to skin test antigens?
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sarcoidosis
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chronic inflammatory disease of spine and sacroiliac joints -> ankylosis, uveitis, and aortic regurgitation
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ankylosing spondylitis
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this disorder is characterized by excessive fibrosis and collagen deposition throughout the body
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scleroderma (progressive systemic sclerosis)
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widespread skin involvement, rapid progression, early visceral involvement, associated with anti-Scl-70 Ab
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diffuse scleroderma
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CREST syndrome
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calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia
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idiopathic, segmental, thrombosing vasculitis of intermediate and small peripheral arteries and veins; seen in heavy smokers
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Buerger's disease
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intermittent claudication, superficial nodular phlebitis, and cold sensitivity, severe pain in affected part (may lead to gangrene) are characteristic of what?
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Buerger's disease
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what is the treatment for Buerger's disease?
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quit smoking
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who does Takayasu's arteritis primarily affect?
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Asian females under 40
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this is the most common vasculitis that affects medium and small arteries, usually branches of the carotid
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temporal/giant cell arteritis
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unilateral headache, jaw claudication, impaired vision (occlusion of ophthalmic artery, which can lead to blindness)
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temporal/giant cell arteritis
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half of patients with temporal arteritis have what?
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systemic involvement and polymyalgia rheumatica (proximal muscle pain, periarticular pain)
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what is elevated in temporal arteritis? what is the treatment?
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elevated ESR; steroids are Rx
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perforation of nasal septum, chronic sinusitis, otitis media, mastoiditis, cough, dyspnea, hemoptysis, and hematuria are associated with what disease?
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Wegener's granulomatosis
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this vasculitide is like Wegener's but lacks granulomas and is associated with P- or C-ANCA
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microscopic polyangitis
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where do giant cell bone tumors most commonly occur? in what age group?
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epiphyseal end of long bones - 20-40 years old
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this is a locally aggressive benign tumor often around the distal femur, proximal tibial region
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giant cell tumor
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what type of bone tumor has a 'soap bubble' or 'double bubble' appearance on x-ray?
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giant cell tumor
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spindle-shaped cells with multinucleated giant cells found in what type of benign bone tumor?
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giant cell tumor
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what is the most common benign bone tumor?
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osteochondroma (exostosis)
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this type of benign bone tumor is usually found in men <25 and commonly originates from long-bone metaphyses
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osteochondroma
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this is a benign cartilaginous neoplasm found in intramedullary bone, usually in the distal extremities
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enchondroma
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what is the most common malignant tumor of bone?
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osteosarcoma
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this malignant bone tumor's peak incidence is in men 10-20 years old and is commonly found in the metaphysis of long bones
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osteosarcoma
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name some predisposing factors for osteosarcoma
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Paget's disease of bone, bone infarcts, radiation, and familial retinoblastoma
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Codman's traingle or sunburst pattern on x-ray
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osteosarcoma
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this is an anaplastic small blue cell tumor of bone most common in boys <15
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Ewing's sarcoma
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what is the characteristic appearance of Ewing's sarcoma?
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onion skin
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this malignant bone tumor is aggressive with early mets but is responsive to chemo; commonly appears in diaphysis of long bones, pelvis, scapula, and ribs
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Ewing's sarcoma
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what is the chromosomal translocation associated with Ewing's sarcoma?
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11; 22
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this is a malignant cartilaginous tumor most common in men 30-60
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chondrosarcoma
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where are chondrosarcomas usually located? what can they originate from?
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pelvis, spine, scapula, humerus, tibia, or femur; may be primary or from osteochondroma (rare)
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What are the genetics of achondroplasia?
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AD inherited. defect in fibroblast growth factor receptor --> results in defect in cell signaling
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what is a prophylactic treatment for osteoporosis?
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exercise and calcium ingestion before the age of 30
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What genetic deficiency can lead to osteopetrosis?
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deficiency of carbonic anhydrase II
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Paget's disease can lead to what cancer of bone?
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osteogenic sarcoma
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a person has a new piece of bone growing on another piece of bone (in the skull). what is it called?
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osteoma - associated with Gardner's syndrome
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what is osteoid osteoma?
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interlacing trabeculae of woven bone surrounded by osteoblasts. <2 cm and found in proximal tibia and femur
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where is an osteoblastoma found?
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vertebral column
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Rheumatoid arthritis is associated with what cytochemical markers?
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anti-IgG antibody and HLA-DR4
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What is Sicca syndrome and in what disease is it found?
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dry eyes, dry mouth, nasal and vaginal dryness, chronic bronchitis, reflux esophagitis. found in Sjogren's syndrome
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conjunctivitis and anterior uveitis, urethritis, arthritis
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classic triad for Reiter's syndrome
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in what would you see a "pencil and cup" deformity on X-ray?
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Psoriatic arthritis
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in what types of patients would you see Reiter's syndrome?
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post-GI or Chlamydial infections
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If you find an anticentromere antibody, what do you have?
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CREST syndrome (scleroderma)
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What is pathogenesis of myasthenia gravis?
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autoantibodies to postsynaptic AChR. associated with thymoma. Treat with Acetocholinesterase inhibitors
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what is Lambert-Eaton syndrome?
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autoantibodies to presynaptic Ca channel results in decreased ACh release leading to proximal muscle weakness.
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with what is Lambert-Eaton syndrome associated?
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paraneoplastic diseases (small cell cancer of lung)
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how does one differentiate Lambert-Eaton syndrome from Myasthenia Gravis?
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Myasthenia has reversal of symptoms with Acetocholinesterase inhibitors, and gets worse with muscle use. Lambert Eaton syndrome improves with muscle use.
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in what disease does one see anti-histone antibodies?
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drug-induced lupus
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