Lecture 9- Musculoskeletal pathology 2

Question 1

…. is progressive replacement of normal bone by fibrous CT and abnormal bone trabeculae. typically seen in adolescents and young adults

Answer 1

fibrous dysplasia

Question 2

is fibrous dysplasia usually monostotic or polyostotic ?

Answer 2

monostotic (70%)

Question 3

what are the 2 types of polyostotic fibrous dysplasias ?

Answer 3

Jaffe-Lichtenstein syndrome (cafe-au-lait)

McCune-Albright syndrome (cafe-au-lait + endocrine disorders)

Question 4

tx of fibrous dysplasia

Answer 4

observation (many will stabilize at puberty)

anti-resorptive therapy

surgical reduction (usually delayed until after skeletal maturity)

Question 5

…. is a bone and osteoid producing malignancy. outside of lymphoproliferative diseases, this is the most common primary malignant tumor of bone

Answer 5

osteosarcoma

Question 6

usual age of affected patient of osteosarcoma?

Answer 6

less than 20 (mean age of 18)

Question 7

what is the most frequent clinical presentation of osteosarcoma

Answer 7

painful enlarging mass

Question 8

most frequent site of osteosarcoma

Answer 8

knee (60%)

jaws (8%)

Question 9

the radiographic appearance of osteosarcoma is a large, destructive, mixed radiolucent-radiopaque lesion that may present with a … appearance.

Answer 9

“sunburst”

Question 10

… is a characteristic feature of osteosarcoma andis an acute angle formed btn neoplastic bone and the cortex

Answer 10

codmans triangle

Question 11

tx of osteosarcoma

Answer 11

surgery and chemo

5 yr survival 60-70%

Question 12

…. aka degenerative joint disease, most common joint disorder, occurs in people over 50

Answer 12

osteoarthritis

Question 13

tx of osteoarthritis

Answer 13

NSAIDS

intrajoint hyaluronic acid; cortisone shots

physical therapy; braces

joint replacement

Question 14

… is disorder caused by tissue accumulation of excessive amounts of uric acid

Answer 14

gout

Question 15

tx of gout

Answer 15

diet modification (beer)

NSAIDS, corticosteroids, colchicine, allopurinol

Question 16

…. is an X-linked recessive disorder caused by the absence of a structural protein termed dystrophin

Answer 16

duchenne muscular dystrophy

Question 17

… is an autoimmune disease which results in destruction of acetylcholine receptors in the NMJ and is manifested by weakness and prominent fatiguability of voluntary muscles

Answer 17

myasthenia gravis

Question 18

tx for myasthenia gravis

Answer 18

cholinesterase inhibitors (improves transmission)

immunosuppression

thymectomy may induce remission

spontaneous remission in some cases

Question 19

prognosis for myasthenia gravis

Answer 19

good, respiratory compromise was a past major cause of death; currently 5 year survival is 95%