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100 Cards in this Set
- Front
- Back
What bone cells originate from hematopoeitic progenitor cells?
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osteoclasts
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The histological unit of bone is called what?
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osteon
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What mineralizing protein is a specific serum marker for osteoblast activity?
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osteocalcin
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Unmineralized bone matrix is called what?, What type of collagen predominates in this tissue?
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osteoid tissue, type 1 collagen
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Bone with less organized structure and an absense of haversian systems is called what?
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woven bone
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When is woven bone seen?
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Children, Pathology (post-fracture callus, infections, bone forming tumors)
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Where does intramembranous ossification occur?
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skull and parts of clavicle
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Defects in nuclear proteins and transcription factors cause what type of bone pathologies?
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dysostoses
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Defects in nhormones and signal transduction mechanisms cause what type of bone pathologies?
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dysplasias
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Defects in extracellular structural matrix proteins cause what type of bone pathologies?
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dysplasias
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Failure of the spinal column closure is called what?
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craniorachischisis
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Synpolydactyly is associated with what gene mutation and what phenotype?
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HOXD-13, Extra digit with fusion
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Waardenburg syndrome is associated with what gene mutation and what phenotype?
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PAX-3 , Craniofacial abnormalities associated with hearing loss and abnormal pigmentation
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What are the complications associated with nail-patella syndrome?
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Hypoplastic nails, Hypo/Aplastic patellae, Dislocated radial head, Progressive nephropathy
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What are the complications associated with Ulnar-Mammary syndrome?
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Hypo/Aplastic Ulna, 3rd and 5th digits, breast, and teeth, Delayed puberty
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What genetic defect is characterized by PTHrp receptor mutations and involves short, bowed limbs, clinodactyly, facial abnormalities, hypercalcemia, hypophosphatemia
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Jansen Metaphyseal Chondroplasia
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What syndrome is associated with craniosynostosis and FGFR2 gene mutation?
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Crouzon syndrome
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What mutation causes achondroplasia?
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FGFR3 on chromosome 4, (arg for gly 375)*
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What is change in gene expression is involved in achondroplasia?
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constant activation of FGFR3 which inhibits cartilage proliferation
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Rhizomelia, normal trunk, and macrocephaly with a depressed root of the nose is associated with what genetic abnormality?
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achondroplasia
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A missense point mutation in FGFR3 with micromelia and respiratory insufficiency is associated with what genetic abnormality?
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thanatophoric dwarfism
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Hypertrophy of the growth plate with clusters of large chondrocytes is associated with achondroplasia or thanatophoric dwarfism?
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achondroplasia
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Diminished chondrocyte proliferation with normal chondrocyte size is associated with achondroplasia or thanatophoric dwarfism?
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thanatophoric dwarfism
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Defect in COL2A1 results in what defect?, What is the phenotoype?
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achondrogenesis, Short trunk, Severely shortened extremities, Relatively enlarged cranium, Flattened face
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Defect in COL1A1 results in what defect?, What is the phenotype?
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Osteogenesis Imperfecta, Bone fragility, Hearing loss, Blue Sclera
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Mutations of the genes coding for alpha1 and alpha2 chains of collagen molecules cuasuing decreased quantity but normal quality of collagen is associated with what condition?
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osteogenesis imperfecta
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How is osteogenesis imperfecta inherited?
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3 of the four types are autosomal dominant, Type II is autosomal recessive
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What type of osteognesis imperfecta is incompatible with life?
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Type II
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Brittle bones and laxity and subluxation of other tissues are associated with what condition?
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osteogenesis imperfecta
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Metaphyseal corner fractures are characteristic of what condition?
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child abuse
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Muchopolysacharoidoses is due to what enzyme deficiency?, What are the clinical manifestations?
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lysosomal acid hydrolases, Short stature and skeletal deformities, Course facial features, corneal clouding, mental retardation, organ involvement
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What is the most definitive test in differentiating between child abuse and osteogenesis imperfecta?
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Gel electrophoresis for analysis of collagen production
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Morquio syndrome is an example of what kind of disease?
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Muchopolysacharoidosis, Lysosomal storage disease
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Deficiency of carbonic anhydrase II or mutation in the CIC-7 chloride channel gene results in what bone pathology?
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osteopetrosis
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What types of bone cells are dysfunctional in osteopetrosis?
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osteoclasts
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What are the major effects of obliteration of the medullary cavities associated with osteopetrosis?
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repeated infections, hepatosplenomegaly
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Bulbous long bones (Erlenmeyer flask deformity), repeated infections, hepatosplenomegaly, nerve compression and frequent fracture may lead to what diagnosis?
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osteopetrosis
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What are the 3 types of osteopetrosis?
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Infantile malignant osteopetrosis (autosomal recessive), Carbonic anhydrase, Autosomal dominant type I and II (detected at puberty)
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How is osteopetrosis treated?
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bone marrow transplants (sometimes effective)
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What are the primary causes of osteoporosis?
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postmenopause, aging
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What are the important secondary causes of osteoporosis?
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endocrine disorders, Neoplasia, Gastrointestinal disorders, Rheumatologic disease, Drugs, Immobilization
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What categories of drugs contribute to osteoporosis?
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Anticoagulants, Chemotherapy, Corticosteroids, Anticonvulsants, Alcohol
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What is the major difference in the mechanism of osteoporosis associated with aging opposed to Menopausal osteoporosis?
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senile-low bone turn over (decreased osteoblasts), postmenopausal-high bone turn over (increased osteoclasts)
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Why is early diagnosis of osteoporosis difficult?
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Often assymptomatic, X-ray unreliable until 30-40% bone loss
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If osteoporosis is suspected with normal x-rays, what tests can be done?
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DEXA scan, Quantitative CT scan
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How is osteoporosis best prevented?
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Exercise, Estrogen replacement, Bisphosphonates, Recombinanant parathyroid hormone
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What are the possible serious complications of osteoporosis?
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Femoral neck fracture, pulmonary embolism, fracture
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What disease is associated with a mosaic pattern of lamellar bone or woven bone?
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Paget's disease
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What virus may be linked to the etiology of Paget's disease?
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paramyxovirus
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What are the 3 stages of pathogenesis of Paget's disease?
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Initial osteolytic stage, Mixed osteoclastic-osteoblastic stage, Osteosclerotic stage
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Paget's disease is often associated with increased levels of what enzyme?
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alkaline phosphatase
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How will x-rays of Paget's disease differ from osteopetrosis?
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Both hypertrophic and hypotrophic bone will be seen
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What are some of the major complications associated with Paget's disease?
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Pain from bone fragility and nerve compression, Secondary osteoarthritis , Tumor transformation (benign: giant cell tumor, malignant osteosarcoma), High output heart failure
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Mild symptoms of Paget's disease are usually suppressed by what treatment?
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Calcitonin & bisphosphonates
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What are the two main roles of Vitamin D?
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enahance absorbtion of Ca and increase mineralization
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Deficiency in vitamin D will lead to an increase or decrease in osteoid tissue?
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Increase
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Decreased calcium in the serum will have what effect on the parathyroid gland?
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Increased PTH secretion
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What effect does parathyroid hormone have on calcium and phosphorous?
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Increased calcium resorbtion from bone, Increased excretion of phosphorous
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What are the clinical manifestations of rickets?
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Craniotabes, Delayed fonatelle closure, Rachitic rosary, Harrison's groove , Pigeon chest , Bowing of legs
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Vitamin D deficiency in adults leads to what disease?
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Osteomalacia
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Persistent failure of bone mineralization in adults leading to loss of skeletal mass is called what?
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osteopenia
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Primary hyperparathyroidism results form what?
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Autonomous hyperplasia, Tumor
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Osteomalacia due to chronic renal failure is called what?
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renal osteodystrophy
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The severe form of hyperparathyroidism affecting the bones is called what?
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osteitis fibrosa cystica
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The increased osteoclast activity in hyperparathyroidism affects what kind of bone most?
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cortical bone (more than cancelous bone)
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An X-ray showing radiolucency along the radial aspect of the index and middle fingers is most commonly associated with what disease?
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osteitis fibrosa cystica
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What are the major skeletal changes that occur due to chronic renal failure?
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Increased osteoclastic bone resorption , Osteomalacia, Osteoporosis, Growth retardation
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What are the 3 major elements of the pathogenesis of renal osteodystrophy?
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Decreased Renal alpha hydroxylase leads to hypocalcemia and bone resorption, Hyperphosphataemia leading to secondary hyperparathyroidism, Metabolic acidosis leading to bone resorption
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How does dialysis of renal failure patients contribute to osteomalacia?
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iron and aluminum deposit in the bone matrix
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What are the histopathological hallmarks of osteitis fibrosa cystica?
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Dissecting osteitis (Railroad track appearance), Brown tumor
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What are the three phases of bone healing?
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Inflammatory phase, Reparative phase, Remodeling phase
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The procallus and bony callus formation of bone healing happens in what phase of bone healing?
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Reparative phase
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What kind of bone is present at the site of a fracture at the start of the remodeling phase of bone healing?
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Woven bone (formed at the end of the reparative phase)
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What are some of the common causes of osteonecrosis?
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Idiopathic, Congenital (Gaucher Disease), Traumatic, Corticosteroid therapy, Infections, Sickle cell and other anemias, Thrombosis/Embolism (dysbarism, pregnancy, pancreatitis), Connective tissue disorders, Increased intraosseus pressure
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Osteonecrosis of the head of the second metatarsal is called what?
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Frieberg-Kohler
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Fibrous tissue and reactive bone deposited surounding a sequestrated piece of bone is called what?
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involucrum
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What is the most common route of infection in Pott's disease?
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hematogenous from lungs
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Massive destruction involving the vertebra and vertebral disc is most likely infection or tumor?
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infection
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A subacute osteomyelitis, which may persist for years before converting to a frank osteomyelitis is called what?
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Brodie's abscess
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A cold fluctuant psoas abscess may be due to what disease?
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Tuberculosis
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What causes syphilis?
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Treponema Pallidum, Treponema Pertenue
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Tibias affected by skeletal syphylis are called what?
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saber shin
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Spirochetes stain with what kind of stain?
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silver stain
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What is the major benign tumor of bone of unknown origin?
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Giant cell
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What are the most frequently occuring primary malignant tumors?
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osteosarcoma
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What common metastatic tumor is primarily osteoblastic?
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prostate
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What common metastatic tumors are primarily osteolytic?
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kidney, liver, thyroid gland
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Are breast tumors primarily osteoblastic or osteolytic?
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equal
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What is the most common benign tumor (hamartoma) of the bone?
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osteochondroma
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Osteochondroma arises most often at what sites?
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metaphysis of long bones particularly lower end of femur & upper end of tibia
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Multiple osteochondroma (exostosis) may be an indication of what condition?
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familial osteochondromatosis
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A 20-40 year old woman with swelling close to the knee joint with a bubble soap appearance on x-ray probably has what condition?
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Giant Cell Tumor (Osteoclastoma)
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A 10-25 year old male with nocturnal pain sensitive to aspirin with a growth on the femur or tibia most likely has what type of neoplasm?
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osteoid osteoma
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A benign tumor larger than 2 cm with a central nidus that usually affects irregular bone like the lumbar spine is probably what?
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Osteoblastoma
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Intramedullary, multilobular, lytic lesion s with flexes of calcification in the hands and feat are most likely what type of growth?
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Encondroma
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A 10-20 year old male with swelling around the knee joint with lifted periostium and Codman's triangle and a sunburst appearance on x-ray most likely has what neoplasm?
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osteosarcoma
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What diseases may lead to osteosarcoma?
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Paget's disease, Familial Retinoblastoma
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A 30-60 year old man with swelling around a central skeletal bone with x-rays showing periostial erosion but no sunburst pattern on x-ray is most likely what malignant neoplasm?
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chondrosarcoma
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A clear cell variant of chondrosarcoma affects what parts of what bones?
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Epiphysis of long bones
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A boy under 15 years old with a tumor of a long bone or flat bone of the pelvis with an onion skin appearance on x-ray and small blue cells on histological preparation most likely has what condition?
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Ewings Sarcoma
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