msk

Question 1

achondroplasia mutation

Answer 1

AD activation of FGFR3

Question 2

why are sclera blue in OI

Answer 2

exposure choroidal veins

Question 3

abnormally thick bone no resportption, easy to fracture, pancytopenia and extramedullary hematopoeiss

Answer 3

osteoetrosis
too much osteoblast
not enough osteoclast

Question 4

osteopetrosis mutation

Answer 4

carbonic anyhdrase II (bone needs acid in order to undergo resorption) so no resorption

Question 5

rx osteopetrosis

Answer 5

bone marrow transplant (gives bone ability to make normal osteoclasts (monocytes)

osteoclasts dervie from monocytes

Question 6

pigeon breast deformity, frontal bossing, rachitic rosary leg bowing

Answer 6

rickets (children)

Question 7

osteoid depostions abnormal

Answer 7

rickets

Question 8

low Ca, low phos, increased PTH, incersaed alk phos

Answer 8

osteomalacia (vit D def)

Question 9

increasd alk phos =

Answer 9

increasd osteoBLAST activity (creates alkaline environment in order to minerLize bone)

Question 10

pathway smooth muscle CONTRACTION

Answer 10

Ca enters Ltype voltage gated Ca channels increases intracellular Ca , increases Ca calmodulin compelx, increases MYOSIN LIGHT CHIAN KINASE = contraction

Question 11

pathwya smooth muscle relaxation

Answer 11

No increases cGMP (from gtp), incresas myosin light chain PHOSPHATASE

light chain kinase -= kontraction
light chain phosphatase = relaxation

Question 12

ossification defective in achondro

Answer 12

endochondral ossification (axial skeleton, appendicular skeleton, base of skull

Question 13

which bones undergo membranous ossificaiton

Answer 13

bones of calvarium , facila, bones clavicle

Question 14

abnormal labs in osteoporosis

Answer 14

NONE

ALL ARE NORMAL

Question 15

MOA bisphos

Answer 15

inhibit osteoclasts

bind hydroxapatitie

Question 16

MOA paget disesae

Answer 16

imablane between osteolast and osteoblast

increased osteoclast and then mixed osteoblast and clast then later increased ostoblast (osteoclast gets burns out)

Question 17

increasing hat size, lion liek faces, hearing loss

Answer 17

paget

Question 18

isoalted alk phos

Answer 18

paget (normal Ca PTH

Question 19

cardiac complciation paget

Answer 19

high output cariac failure (av shunts) (weird bone = increased av shunts)

Question 20

paget increases risk of…

Answer 20

osteogenic sarcoma

Question 21

MCC site aseptic necrosis

Answer 21

femoral head due to insufficeincy of medial circumfelx femoral artery

Question 22

increase risk of what in dermatomyositis

Answer 22

OCCULT MALIGNANCY (usually stomach)

Question 23

can’t comb hair, can’t climb stairs…rash (malar) or upper eyelids, red papules on elbows

Answer 23

dermatomyositis

Question 24

ANA+ +antijo1 increasd CK

Answer 24

dermatomyositis

Question 25

perimysial inflmmation adn atrophy with CD4 t cells

Answer 25

dermatomysitis

Question 26

T cells involved in dermatomyositis

Answer 26

CD4

Question 27

proximal muscle weakness without rash ENDOmysial inflammation (CD*)

Answer 27

polymositis

CD8 inflammation

Question 28

replaced skeletal muscle by adipose tissue

Answer 28

x-linked muscular dystrophy

Question 29

largest gene human genome

Answer 29

dystorphin DMD links cytoskeletin to transmembrane with a and b dysroglyacan (ECM)

Question 30

calf psudohypertrophy

Answer 30

duchenne

Question 31

MCC death duchenne

Answer 31

dilated cardiomyopathy or respiratory failure

Question 32

how will ACHE affect mg VS LAMERT EATON

Answer 32

ache will reverse myastehnia

will not reverse lambert eaten

Question 33

thymic hyperplasia or thymoma

Answer 33

myasthenia gravis

Question 34

cardiac rhabdomyoma

Answer 34

tuberous sclerosis

Question 35

MCC soft tissue tumor children

Answer 35

rhabdomyosarcoma

Question 36

desmin psoitive

Answer 36

rhabdomyoblast

Question 37

characteristic of RA

Answer 37

inflmamatioon induces formaiton of PANNUS (prliferative granulation tissue) which erodes articular artilage and bone

Question 38

OA vs RA presentation

Answer 38

oa - pain worsens with use,asymmetric, no systemic systmpsoms
ra - pain IMPROVES with use, morning stiffness, systmeic symptoms, symmetrical

Question 39

OA vs RA - dsitrubution

Answer 39

OA - DIP and PIP

RA - DIP spared

Question 40

OA vs RA - findings in joint

Answer 40

OA - osteophytes (bone spurs),

RA - erosions, osteopenia, soft tissue swelling

Question 41

rhematoid factor antibody structure

Answer 41

IgM antibody against Fc portion of IgG (rheumatoid factor) marker of disease

Question 42

RA synovial fluid

Answer 42

neutrophils and high protein

Question 43

bamboo spine

Answer 43

ankylosing spondylitis

Question 44

cardiac complication of ankylosing spondylitsi

Answer 44

aortititis (aortic regurg)

Question 45

orgaisms that can cause reactive arthritis

Answer 45

ShY ChiCS
shigella
yersenia
chlamydia
campylobacter
salmonella

Question 46

skin psorisais and nail lesions…sausage finger toes

Answer 46

psoriatic arthritis

Question 47

MCC infectious arthritis

Answer 47

gonarrhea

s aureus

Question 48

monosodium urate crystals in joints

Answer 48

gout

Question 49

needle shaped crystals negative bifringence underpolarized light

Answer 49

gout (crystals law LOW YELLOW) paraLLel YeLLow

Question 50

rhomboid crystals postiive bifrignetn

Answer 50

pseudogout (calcium pyrophosphate stones)

Question 51

MOA allopurinol

Answer 51

competitive inhibits xanthine oxidase (decreasd PRODUCTION)

useful in PREVENTING gout not acute

Question 52

MOA febuxostat

Answer 52

inhibits xanthin oxidase

Question 53

MOA probenecid

Answer 53

inhibits REABSORPTION of uric acid(pee it out…can precpitate uric acid calculi

Question 54

acute got drugs

Answer 54

NSAIDs glucocorticoids, colciine

Question 55

MOA colchicine

Answer 55

inhibits microtubule polymerazition