bone pathology Flashcards
What is ESR?
erythrocyte sedimentation rate, RBC aggegation causes increased sedimentation (falls faster in test tube)
why does elevated ESR indicate inflammation?
inflammatory products (fibrinogen) coat RBC and cause aggregation –> falls faster in test tube
increased ESR in lab could mean…
anemia infection, inflammation cancer pregnancy autoimmune(SLE)
SLE
systemic lupus erythematous autoimmune CT disease, chronic antigen / Ab complex (tyoe 3 hypersensitivity) malarbutterfly, discoid rash, oral ulcers, arthritis, raynauds , anemia, phenomenon childbearing aged women imitates many diseases! inflammation Not as damaging as RA. \+ ANA/anti-sdDNA/anti Sm
what disease/gene causes impaired cartilage proliferation in growth plate (short extremities and normal head and chest?
achondroplasia
activating mutation –> overexpression of FGFR3
what type of bone formation does achondroplasia affect?
endochondral bone formation!
intramembranous is unaffected
what disease gives you normal size head and chest but small extremities?
how are mental function, life span, and fertility
achondroplasia
osteogenesis imperfecta
congenital defect of bone formation–> weak bone
defect in collagen type 1 synthesis
features of osteogenesis imperfecta
weak bone
multiple fractures (can mimic child abuse)
blue sclera - thinning to see choroidal veins
hearing loss - middle ear factures easily
abnormal bone resorption
- -> abnormal thick heavy bone that fractures easily
- poor osteoclast [ ]
osteopetrosis
what mutation occurs in osteopetrosis?
carbonic anhydrase II mutation –>leads to loss of acidic microenvironment and bone cannot be resorbed by osteoclasts
osteopetrosis treatment?
marrow transplant
osteoclasts form from monocytes
clinical features of osteopetrosis
anemia/thrombocytopenia due to bony replacement of marrow
vision and hearing impaired - cranial nerve impingement
hydrocephalus due to narrowing of foramen magnum
renal tubular acidosis- lack of carbonic anhydrase –>decreased HCO3 resorbtion
osteoblasts –> bone?
osteoblasts form osteoid which is then mineralized w calcium and phosphate to form bone
low vit D results in low serum —- & —-?
calcium and phosphate
rickets and osteomalacia are diseases that…
defective mineralization of osteoid due to low vitamin D
vitaminD sources and activation
diet and sunlight;
activated in liver (25 hydroxylation) and proximal tubule cells of kidney (1 alpha hydoxylation)
what does ACTIVATED vit D do?
raises serum ca and phosphate by increasing reabsorption in intestine, kidney,and bone
rickets
low vit D in children leading to low bone mineralization
arises <1yo, pigeon breast deformity, frontal bossing (osteoid deposit in forehead), rachitic rosary, bowing of legs
osteomalacia
low vit D leading to low bone mineralization in adults
weak bone with increased risk of fracture
LAB: decreased serum ca and phosphate and increased PTH and alkaline phosphatase
Lab: decreased serum ca and phosphate, increased PTH and alkaline phosphatase
osteomalacia
reduction in trabecular bone mass resulting in porous bone
osteoporosis
RANKL - what does it do and what is it a sign of
increase osteoclast activity;
osteoporosis
risk of Osteoporosis
mostly senile and postmenopausal
rate based on peak bone mass (30 yo) and rate of bone loss after (normally 1% a yr)
bone loss is more rapid with lack of weight bearing exercise, poor diet, and decreased estrogen
treatment for osteoporosis
exercise, vit D, calcium, bisphosphonates
not glucocorticoids!!
what do bisphosphonates do?
induce apoptosis of osteoclasts
Lab for osteoporosis
ca, phosphotase, PTH, and allkaline phosphatase all NORMAL –> rules out osteomalacia
**measure bone density using DEXA
clinical features of osteoporosis
bone pain and fractures in weightbearing areas - vertebra, hip, distal radius
-lost of height from vertebra compression
paget disease
imbalance between osteoclast and osteoblast activity, in late adulthood
three stages - osteoclastic, mixed, and osteoblastic/sclerotic
results in thick, bone with lamellar cement lines (jig saw) that fractures easily due to rushed osteoblasts
does paget disease affect entire skeleton?
NO, just one or a few bones
-skull is common
clinical features of pagets disease?
bone pain (microfractures), increasing hat size, hearing loss (impinged cranial nerve), lion like facies,
Lab shows all normal except isolated elevated alkaline phosphotase
paget disease
treatment of pagets
calcitonin (inhibits osteoclasts)
bisphosphonates (osteoclast apoptosis)
two complications of pagets disease
osteosarcoma
AV shunts in bone –> cardiac failure
osteomyelitis
infection of marrow and bone, usually bacterial, hematogenous spread
kids>adults
pain with systemic signs of infection
lytic focus (abcess) surrounded by bone sclerosis (sequestrum surrounded by involucrum)
where does each form of osteomyelitis seed and in who?
transient bacteremia (kids) - metaphysis open wound bacteremia (adults) - epiphysis
most common cause of osteomyelitis (plus others)
**staph aureus - 90%
Ngonorrhoeae - sexually active young adults
salmonella - sickle cell
pseudomonas - diabetics or IV drug users
pasteurella - cat or dog bite
mycobacterium tuberculosis - involves vertebra