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95 Cards in this Set
- Front
- Back
- 3rd side (hint)
Deficiency in the synthesis of type-1 collagen?
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Osteogenesis Imperfecta
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Is Osteogenesis Imperfecta considered a type of osteoporosis?
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yes
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what is the basic disorder of Osteogenesis Imperfecta?
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Osteoporosis in the form of bone thinning and weakness leading to fragility and increased breakage
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What are the 4 subtypes of OI?
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OI:1 Post-natal fracture w/ Blue Sclera
OI: 2 Perinatal Lethal in Utero OI: 3 Progressive Deforming OI: 4 Post-natal fracture w/ normal sclera |
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Which of the OI subclasses is not compatible with survival?
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OI 2 : Perinatal Lethal
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Which of the 4 subtypes of OI results in short stature?
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OI 4: Postnatal Fracture w/ normal sclera
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Normal stature
Skeletal fragility Dentinogenesis Imperfecta Hearing impairment Joint laxity Blue Sclera |
OI : 1 PNF w/ blue sclera
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Growth retardation
Multiple fractures Progressive kyphoscoliosis Blue sclera @ birth --> white Hearing impairment Dentinogenesis imperfecta |
OI : 3 Progressive Deforming
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Which OI subtypes may lead to Dentinogenesis Imperfecta?
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OI: 1, 3, & 4
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What is the presentation of teeth for OI?
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opalescent teeth
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'Marble bone / Alber-Schonberg Disease'
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Osteopetrosis
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In osteopetrosis, osteoclasts decrease in number thus causing reduced bone resorption? T/F
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False:
reduced activity, but numbers stay the same or even are higher |
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reduced osteoclastic bone resorption due resulting in diffuse symmetrical skeletal sclerosis
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Osteopetrosis
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carbonic anhydrase II deficiency is a variant of what pathology?
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Osteoporosis
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What type of Osteopetrosis would exhibit:
-fractures -anemia - hydrocephaly - optic atrophy - deafness - paralysis -repeated infections |
Infantile Malignant Osteopetrosis
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What is the reason for repeated infections of patients with Osteopetrosis?
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decreased bone marrow --> (WBC production)
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_____ bone is laid down haphazardly thus decreasing durability of bone associated with ______?
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woven
Osteopetrosis |
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presents with:
- repeated fractures in an X ray - mild cranial deficits - anemia - smaller foramina |
Osteopetrosis: Autosomal Benign type
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What is a treatment for Osteopetrosis to desist increase osteoclastic activity and reverse some of the skeletal defects?
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bone marrow transplant
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X ray exhibits :
- bony enlargements - bilateral sclerosis of bone - lessening of vasculature to bony site |
Osteopetrosis
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characterized by increasesed porosity of the skeleton resulting from reduced bone mass which predisposes the bone to fracture
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Osteoporosis
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Apart from Primary Osteoporosis due to PM women, senile or idiopathic, what are the secondary categories for cause?
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1) Endocrine disorders (hypothyroidism/diabetes I, etc..)
2) Neoplasia (Multiple Myeloma) 3) Gastrointestinal ( malnutrition) 4) Rheumatologic disease drugs (DMARDS) 5) Miscellaneous (OI, anemia etc..) |
5
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What are the most common forms of Osteoporosis?
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Senile
Post-menopausal |
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what genetic factor contributes to the density of bone and is a factor in Osteoporosis cases?
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Vitamin D receptor
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What is the rate of bone loss at the point that one has met their peak/
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0.7%/ year
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What 5 factors contribute to the onset of osteoporosis?
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1) Age - related (low turnover variant)
2) Reduced Physical Activity (remod) 3) Genetic factors (Vit D receptors) 4) Calcium homeostasis (diet) 5) Hormonal influences (estrogen) |
charg
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How do estrogen levels contribute to osteoporosis?
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In PM women, an estrogen deficiency takes place that leads to the increased secretion of cytokines ( IL-1, IL-6, TNF) which stimulate osteoclastic activity recruitment and activity that exceed osteoblastic deposition
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Increased expression of _____ and _____ will lead to the increase the activity of osteoclasts
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RANK (receptor) and RANKL (ligand)
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clinical manifestations of Osteoporosis would be : (5)
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1) vertebral fractures
2) lumbar lordosis 3) kyphoscoliosis 4) pulmonary embolism 5) pneumonia |
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What may cause the clinical manifestation of pneumonia in Osteoporosis?
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fractures of:
-neck of femur -pelvis -spine |
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bisphosphonates and recombinant PTH are used for what?
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tx of Osteoporosis
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2 pathologies that result in a defect in bone matrix mineralization?
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1) Rickets
2) Osteomalacia |
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What cohort does Rickets affect?
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children
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What cohort does Osteomalacia affect?
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adults
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Children with deranged bone growth producing skeletal deformities such as:
-craniotabes -frontal bossing -square head -lumbar lordosis -bowing of legs |
Rickets
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In an adult that exhibits a matrix mineralization defect resulting in osteopenia and due bone fragility
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Osteomalacia
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What are 5 etiologies of matrix mineralization deficiency?
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1) Inadequate synthesis or dietary of Vit D
2) Decreased absorption of Vit D 3) Deranged Vit D metabolism 4) End Organ Vit D resistance 5) Phosphate depletion |
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'pigeon breast' deformity is associated with _____?
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Rickets
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inflammation of bone and its marrow
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Osteomyelitis
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In the US what 2 bacteria are common to Osteomyelitic infections?
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1) pyogenic
2) myocobacteria |
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Fever
Leukocytosis Lymphadenopathy Tissue sensitivity/swelling all lasting less than a month is the course of _______? |
Acute Osteomyelitis
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Pain
Swelling Sinus formation Purulent discharge Sequestrum formation Xray: patchy/ragged radiolucency sequestrum radiopacity |
Chronic Osteomyelitis
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What are the 3 routes of infection for Pyogenic Osteomyelitis?
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1) hematognous (most common)
2) extension from contigous site 3) direct implantation |
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What are the most common infection sites for Pyogenic Osteomyelitis?
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Long bones
Vertebral bodies |
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What pathogen is most common to Pyogenic Osteomyelitis
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S. aureus
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Who are the patients that contract the following infectious agents:
1) E. coli; Klebsiella; Pseudomonas 2) H. influenzae; B Streptococci 3) Salmonella 4) Mixed infection |
1) Urogenital Infection pts.
2) Neonatal pts. 3) Sickle cell pts. 4) Surgery pts. |
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What % of acute infections of Osteomyelitis progress to the Chronic form?
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5-25%
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1-3% of those with TB (pulmonary/ or extrapulmonary) may manifest what infection?
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Tuberculous Osteomyelitis
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Pott's disease (vertebrae destruction) causing skeletal deformities and neurological deficits is associated with _____?
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Tuberculous Osteomyelitis
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____ is characterized by:
-localized, frenzied osteoclastic activity with bone resorption followed by exuberant bone formation with a net gain of unsound bone. |
Paget's Disease (Osteitis Deformans)
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What are the 3 phases of Paget's Disease?
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1) initial phase: osteoclastic activity, hypervascularity, and bone loss
2) mixed osteoclastic / osteoblastic activity 3) osteosclerotic phase (late) |
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Basophilic, 'resting lines' characterize the bony mosaic in ______?
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Paget's Disease
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Paramyxovirus
Vitamin D dysfunction RANKL disorder -all may be culprits to what disease? |
Paget's
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Bone pain is the most common problem in what disorder?
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Paget's Disease
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-Pain (bone)
-Headache -Hearing problems -Enlargement of head (leontiasis ossea) -Bowing/chalkstick fractures of leg -Tumors |
Paget's Disease
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What % of Paget's Disease pts. may develop cancer?
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1-3%
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benign lesions of bone that usually are developmental growths composed of woven lamellar bone
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Osteoma
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What is the most common location for development of Osteomas?
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Facial bones
Skull |
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What is the cohort for Osteomas
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Middle aged (40-50 yo)
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exophytic growths that may arise within bone?
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osteomas
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Multiple Osteomas may be present in what disease, accompanied with oral problems and other malignancies?
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Gardner Syndrome
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Osteomas often undergo malignant change that may result in fast-growing manifestations. T/F
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False
Slow growing; non-malignant |
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malignant mesenchymal tumor whereby cancerous cells produce bone matrix
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Osteosarcoma
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Excluding myelomas and lymphomas, what is the most common primary malignant tumor of bone?
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Osteosarcoma
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What cohort incurs Osteosarcomas?
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<20 yo = 75%
Older are those with predispositions (Paget's disease) |
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Most osteosarcomas of the jaw arise in pts. between what age?
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20-30yo
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What gender is most affected by osteomas?
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Males
1.6:1 |
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Osteosarcomas arise in the metaphyseal region of long bones while ____% arise in the ______
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50-60%
knee |
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10-20% of pts. with Osteosarcomas already have ________
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pulmonary metastases
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The _____ yr survival rate of Osteosarcoma pts. has increased to 60-70%
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5
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What may be the tumorous factor of Osteosarcomas?
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Osteoblasts
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benign tumor like lesion that is arrested , immature developmental tissue in the medulla of bones
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Fibrous Dysplasia
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Fibrous CT from Fibrous Dysplasia pts. that is within bone medulla is replaced eventually by _______
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non-maturing bone
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What are the 3 presentations of Fibrous Dysplasia lesions?
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1) monostotic (single bone involved)
2) polystotic (multiple) 3) polystotic w/ cafe au lait skin and endocrine abnormalities |
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Accounts for 70% of Fibrous Dysplasia cases
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Monostotic form
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When in a patient's life does the asymptomatic Monostotic form of FD desist in growth?
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adolescence
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Polystotic FD w/o endocrine involvement represents ___ % of the cases and about ___% of these patients have craniofacial involvement.
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27%
50% |
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When does Polystotic FD present in a patients life?
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early age
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have propensity to develop:
-shoulder + pelvic problems -crippling -Shepard-Crook deformities -fractures |
Polystotic FD w/o endocrine dysfunction
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Polystotic FD with Skin pigmentation and Endrocrine dysfunction is also called?
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McCune Albright syndrome
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Polystotic FD pts. are often _____ in gender and present with:
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girls
-cafe au lait spots -endocrine abnormalities: sexual precocity, hyperthyroidism, pituatary adenomas, etc. |
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Monostotic FD usually presents with ____ complications while Polystotic may be associated with ______ diseases
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-minimal
-progressive |
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'ground glass' appearance in radiographs
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Fibrous Dysplasia
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benign tumors of hyaline cartilage found in hands and feet
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Chondroma
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what is the cohort for Chrondromas
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20-40 yo (young)
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Multiple chondromas (enchondromas) are seen in _________
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Ollier Disease
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Chrondromas develop from _______ often limiting growth potential
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rests of growth plates
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how might chondromas become malignant?
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enchondromas (multiple)
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chondromas are usually painful? T/F
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False
-usually not but can be at times |
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what is the tx of choice for chondromas?
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surgical excision
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malignant neoplasms populated by mesenchymal cells that produce a cartilagenous matrix
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Chondrosarcomas
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What is the cohort and incidence of Chondrosarcomas?
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Males X2
Osteosarcomas x2 in comparison Older pts. > 40 yo |
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What might be the result of an enlarging, painful mass in the shoulder, pelvis, femur or ribs and is made of cartilage?
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Chondrosarcoma
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Chondrosarcomas often metastisize thru the ______ route usually with the ____ as the most common site
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hemagenous
lungs |
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5 year survival rate of those with Chondrosarcomas in grades:
1 - 2 - 3 - |
90%
81% 43% |
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