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27 Cards in this Set

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Osteoporosis: Menopause & aging effects
osteogenesis imperfecta
brittle bone dz
*abnormal dev of type 1 collagen
*multiple bone fractures, blue sclear, hearing loss, dentinogenesis imperfecta, scoliosis, short stature

*subtypes:
OI type 1: compatible w/ life
OI type 2: perinatal lethal
OI type 3: progressive & deforming but compatible w/ survival
*OI type 4: compatible w/ survival
Osteopetrosis
marble bone dz & albers schonberg dz
*rare bone disorder w/ reduced bone resorption & diffuse symmetric skeletal sclerosis due to impaire formation/fxn oclasts.

*stonelike/brittle bones
*mutation interfere w/ acidification of oclast resorption pit needed to dissolve Ca hydroxyapatite in matrix
*No medullary canal on bones. Bulbous ends of long bones (erlenmeyer flask deformity). small neural foramina--> compressed nerves

*primary spongiosa fills medullary cavity interfereing w/ hematopoietic bone marrow & prevent formation of mature traveculae. deposited bone not remodeld & woven into archeture. These lead to brittle bones
*dep on subtype: oclasts inc dec or normal
Infantile Malignant osteopetrosis
*fracture anemia, hydrocephaly--> postpartum mortality
*if child survives: Cranial nerve defects, inadequate bone marrow--> severe infx. Extramedullary hematopoiesis --> hepatosplenomegaly.

*milder forms may not be detected until adolescence or young adulthood.
Osteoporosis
* Dec in bone mass. Structural change--> bone fragility.
*loss of horizontal trabeculae & thick vertical trabeculae

*GENETICS: RANKL, OPG, RANK--> regulate oclasts. MHC locus & estrogen receptor
*AGE: oblasts in elderly= dec prolif & biosynth
*Dec weight bearing exercise & adolescents w/ dec Ca+ diet
*HORMONE: decade after menopause: estrogen dec--> +CK--> inc RANKL & dec OPG--> inc oclasts--> yearly inc lost bone mass
Osteomalacia
* Rickets
*children w/ open epiphyseal plates--> shape deformity on xray
*accum unmineralized bone by defective mineralization.
* dec Vit D--> Dec serum Ca/P
Paget's :)
* >55yo, England/Australia/ N. Europe
*affect L-S spine, pelvis, femur, skull
*Cause: uncertain, genetic & env connection

*15% have family hx.
*mut in SQSTM1 gene (inc + by RANK--> inc oclast & inc susceptibility to dz
*may dev secondary osteosarcomas/ fibrosarcomas

*osteolytic phase: marrow replaced by CT w/ oclasts
*mixed phase: bone resorption & bone formation
*osteosclerotic phase: irreg bone deposition--> mosaic pattern

*usu asymp. Serum alkaline Phosphatase inc.
hypervascular bone lesions: warm skin, inc CO. enlarged head: headache, visual disturb, deaf. Transverse fractures of long bones
Osteomyelitis
*Bacterial: STAPH AUREUS. e. coli, klebsiella or proteus.
*salmonella: sickle cell pt
* h influenza: newborns
*pseudomonas: IVDA
*TB: pott's dz
* hematogenous spread. direct extension from joint/tissue. traumatic implantation from surgery/trauma

*Acute: Nphil inflam, necrosis, sub periosteal abscesses, disrupt blood supply, spread to joint capsule
*Chronic: sequela of Acute infx.
Sequestrum = residual necrotic bone
Involucrum= rim of reactive bone
Brodie's Abscess: abscess surrounded by sclerotic bone
*bacteria may be present in sequestered area
Osteomas
* 40-50y, M>F
*exophytic lesion of dense mature bone on flat bonse of skull/face. may protrude into sinuses
*assoc w/ Gardner's Syndrome.
*cosmetic problems. not malignant
Osteoid Osteoma
* M>F, 10-30yo
*femur, tibia in metaphysis
*painful, relieved by aspirin, central radiolucent nidus.
*high levels of prostaglandins
Osteoblastoma
*sim to osteoid osteoma
*large central nidus
*spine, large bones of legs.
* PAINLESS
*may be diff to distinguish from osteosarcoma
osteosarcoma
*most common primary bone malignancy (except hematopoietic lesions)
*M: F = 3: 2, usu 10-25y w/ 2nd peak >40y
*metaphysis, lower femur, upper tibia
*malignant cells form osteoid. Extend from marrow to cortex to soft tissie to epiphysis to joint
*Codman's triangle: elevation of periosteum.
*may have satellite nodules

*destroy preexisting bone/grow around it.
*met via blood to Lung or other bones. Regional LN not involved

*predispositions: Paget's Dz (>50yo), Radiation: 10yr post, Chemo: children w/ retinoblastoma. Trauma does not cause but brings attention to it.
Osteochondroma
* exostoses
*10yo, M>F
*metaphysis, lower femur, upper tibia, humerus.
*grow in opposite direction of joint. cap of cartilage w/ bone underneath.

*asymp. spont regress. Rare malig transform if single. If multiple = Garder's syndrome & inc malig.
Chondroma
*common benign lesions
* Xray = popcorn like densities
*small bones: hands, feet, phalanges
*usu not painful.
*lobules of hyaline cartilage, may have calcification

*syndromes w/ mult chondromas= inc risk malig
1. Ollier's dz: U/L chondromas & ovarian tumors
2. Maffucci's syndrome: chondromas & soft tissue hemangiomas
chondroblastoma
*10-20yr, M>F
*painful
*Epiphyseal: distal femur, proximal humerus. Very cellular. Rarely aggressive
*immature cartilage=chicken wire pattern
chondromyxoid fibroma
* young adults, long bones, small bones of feet
* xray: well defined lytic lesion
*solid yellowish to tan. reselmble low grade chondrosarcoma
*recur if not completely removed
Chondrosarcoma
*30-60yo = pelvis, ribs, shoulder
*In children = extremities
*xray: osteolytic lesions w/ calcification
*2+ nuclei per cell. permeate Bone marrow

*must correlate w/ xray. better prognosis than osteosarcoma. Recurrence may occur up to 20y later. Met to lung, usu not LN via blood
Giant cell tumor
* F> M, 20-30y, Asian>western
*most at metaphyseal-epiphyseal jxn. Ends of bones: lower femur, upper tibia, lower radius.
*xray = lytic w/o sclerosis
*solid, tan --> brown w/ hemorrhage

*giant cells in spindled stromal cells.
*all potentially malignant. Met to lung. Radiation tx may induce malig.
Ewings Sarcoma
* 5-20yo
*long bones, pelvis, ribs, vertebrae
*common bone malig in children
*t (11:22) translocation
*arise in medullary cavity. involve entire bone.

*xray: onion skin layering of new bone
*Gross= white, fleshy
*micro: sheets of small uniform cells separated by strands of fibrous stroma. "small blue cell tumor
*met to lungs, other bones, cns, LN
chordoma
*M>F, 40-50 yo
* malignant, destroys bone
* Gross: gelatinous, soft, hemorrhage
*micro: cells grow in cords & lobules separated by mucoid matrix
*physaliferous cells: large tumor cells w/ bubbly cytoplasm

*met late to skin, bone.
*vertebral bodes, discs, sacrum. sphenooccipital area in kids
fibrous dysplasia
*uncommon benign tumor like lesion of bone
1. monostotic: most common, teens, ribs, tibia, femur.
2. polystotic: craniofacial area, pelvis, femur.
3. polystotic w/ endocrine abnorm: least common
4. McCune-Albright syndrome: UL bone lesions, cafe au lait spots, precocious puberty

*lesions circ &radiolucent. Prolif of fibroblasts & collegen w/ islands of woven bone. may fracture. asymp
Adamantinoma
*tibia femur ulna fibula
* in jaw = ameloblastoma
*poorly defined lytic lesion. sclerosis outlining lucent areas. spindle cells surround basaloid cells.
*low grade malig: local recurrence, rare LN met
Plasma cell myeloma
most common primary tumor of bone skull spine & ribs
Malignant lymphoma
patchy cortical & medullary involvement

most = large cell type
hemangioma
common
skull, vertebrae, jaw
Metasteses
*most common of all malig tumors of bone
*mostly from breast, lung, prostate,
*70% in axial skeleton, 30% in extremities.
*usu osteolytic
*osteoblastic: prostate, bone
*painful
osteoarthritis
*enlargement &disorganization of chondrocytes
*splitting of articular surface, erosion of articular cartilage, eburnation of underlying bone,
*osteophytes: bony excrescences on bone margins

*clinic: hips knees lumbar cervical vert. prox/dist interphalangeal joints of fingers. first carpometacarpal joints, first tarsometatarsal joints. most <50yo = asymp. deep aching pain