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54 Cards in this Set

  • Front
  • Back
Makes up 65% of bone?
Hydroxyapatite: 99% of bone calcium, 85% of phosphorus, 65% of sodium and magnesium
Osteoid?
Unmineralized bone
Osteoprogenitor Cells?
Differentiation?
Pleuripotent stem cells at bony surface.
Differentiate to Osteoblasts when stimulated by BMP
Osteoblast Function?
Osteoclast Function?
Synthesize, transport and arrange proteins, institute mineralization.
Bone resorption.
Activates osteoclastogenesis?
Blocked by?
RANKL binding to RANK and bloked by OPG(osteoprotegrin)
Proteins in Bone?
collagen type 1
Woven Bone?
Seen in fetal skeleton, at growth plates, pathologic in adults
Lamellar bone?
Layered, Types: circumferential, concentric, interstitial, trabecular
BMU?
Bone multicellular unit: osteoclasts and osteoblasts.
Remodel 10% of skeleton anually
Stages of bone growth?
cartilage model, endochondrial ossification, primary and secondary centers of ossification, growth plate of physis
Common congenital agenises of bone?
Phalanx, rib, clavical
Extra bones?
Ribs or digits
Fusion of two adjacent digits?
Syndactyly
Spider fingers?
Arachnodactyly
Clinical Manefistation of Achondroplasia?
Major cause of dwarfism, shortened proximal extremities, normal trunk, frontal bossing, saddle nose, no change in intelligence or reproductive status
Pathogenesis of Achondroplasia?
Abnormalities of growth plate, AD, most are new mutations, FGFR3(fibroblast growth factor) always on inhibiting cartilage proliferation
Clinial manifestations of Osteogenesis imperfecta?
Large spectrum, skeletal fragility(brittle bone disease), Blue sclera, hearing impaired, Dental abnormalities
Pathology of Osteogenesis imperfecta?
Brittle bones due to defective type 1 cartilage, systemic disorder of connective tissue disorder
Etiology of Osteogenesis imperfecta?
AD, some AR, some acquired.
Mutaion in genes for alpha1 and alpha2 chains of collagen
Treatment of Osteogenesis imperfecta?
Pain control, decrease fractures, psychiatric anlysis
Pathogenesis of Osteopetrosis?
AR and rarely AD, osteoclast dysfunction which leads to dense bones.
Pathology of Osteopetrosis?
Marble bone disease, diffuse skeletal sclerosis, boes lack medullary canal, no room for marrow.
clinical manifestation of Osteopetrosis?
Some die early, some diagnosed later in life, multiple bone fractures, anemia, blindness, deafness and CN impingement of neuronal foramina
Pathology of Osteoporosis?
Absolute reduction of bone mass, weak bones, decrease size and number of traceculae
Greatest bone loss in Osteoporosis seen in?
Vertebrae, wrists(colle's fracture), ribs and pelvis
Pathogenesis of Menopausal Osteoporosis?
decrease estrogen, decrease IL-1,6, TNF levels, Increase RANK and RANKL levels, increase osteoclast activity
Pathogenesis of aging Osteoporosis?
Decrease replicative activity, decrease osteoblast activity, decrease matrix bound growth factors, decrease physical activity
Secondary causes of Osteoporosis?
Hyperthyroid, hyperparathyroid, corticosteroids, hypogonadism, immobilization
Clinical manifestation of Osteoporosis?
Bone fractures, weight loss, shortened stature, kyphoscoliosis,
Diagnosing Osteoporosis?
X-ray:no, ca PO4 Vit D Alk Phos not diagnostic, Bone density scan(T and Z-values)
Pathology of Rickets?
Decrease mineralization, craniotabes, frontal bossing, Rachitic rosary: noby costocondrial joints, pigeon breast, bowing of legs
Pathology of Osteomalacia?
Weak and vulnerable bone, fractures and microfractures, bone pain, osteopenia
Pathogenesis of Rickets and osteomalacia?
Vit D deficiency
Pathology of Hyperparathyroidism?
unabated osteoclast activity, Dissecting osteitis(osteoclast tunnel), osteitis fibrosa cystica: peritrabecular fibrosis, cystic brown tumors
Clinical manifestation of Hyperparathyroidism?
Decrease bone mass and skeletal deformity from weight bearing stress, bone changes can regress with control of PTH
Pathogenesis of Scurvy?
Vit C deficiency
Pathology of Scurvy?
Defects in collagen synthesis, deformed bones in kids and tissue hemorrhage. Defects in Hydroxylation of procollagen
Clinical Manifestation of Scurvy?
Decreased osteoid leads to bone deformation, weakness in blood vessels leads to hemorrhage, purpura, ecchymoses, Gingina with hemorrhage, poor wound healing
Pathology of Paget's disease (osteitis deformans)
Osteolytic phase, mixed phase, osteosclerotic phase
Get poorly organized bone, highly vascularized
Clinical manifestation of Paget's disease?
middle adulthood, Bone pain, leotiasis ossea, hearing loss, hypervascularity(warm tibia): leads to high output heart failure, increased alk phos
Leontiasis ossea?
Thickening of skull and craniofacial bones in Paget's
Complications of Paget's disease?
Bone pain/ fractures
High-output cardiac failure
Hearing loss
Osteosarcoma
mosaic bone formation
Etiology of Osteonecrosis?
trauma, corticosteroids, dysbarism(air embolis), sickle cell and other myoglobinemias, gaucher
Clinical Maifestation of Osteonecrosis?
Bone or joint pain, subchondral infarcts lead to collapse of affected bone: secondary arthritis
Medullary infarcts are silent
Pathology of Osteonecrosis?
Infartion of bone, dullay oor subchondral region of epiphysis
Pott's disease?
TB in the vetebral bodies, thoracic and lumbar
Mixed lytic and blastic bone mets?
Breast, Lung, Thyroid, Kidney, Multiple myeloma
Blastic Bone Mets?
Prostate, Infamous lymphoma, Transitional cell carcinoma, Treated breast cancer, APUDoma(carcinoid)
Osteoma?
Benign, middle aged men, craniofacial bones, multiple found in Gardner's syndrome
Osteoid osteoma?
Benign, haphazardly arranged trabecullae and woven bone, cortex of femer or tibia, men<25, nocternal pain relieved by aspirin.
Osteosarcoma?
Malignant, 60% by knee, 15% by hip, shoulder, jaw, 50-60 y/opainful enlarged mass, prognosis correlates to tumor grade
EWING'S SARCOMA or PNET?
neural=PNET(Primitive neuroectodermal tumor,
No neural=Ewing's sarcoma
Pathology of EWING'S SARCOMA or PNET?
Round mesenchymal cells, found in medulary cavity of long bones, translocation in 11/22 chromasomes
Clinical manifestation of EWING'S SARCOMA or PNET?
pts<20, painful enlarging mass, aggressive, looks like lymphoma, rhabdomyosarcom
75% 5 yr survival