Bone/Soft tissue Pathology

Question 1

scurvy results from

Answer 1

vitamin C deficiency

Question 2

impaired collagen formation leads to

Answer 2

poor vessel support, bleeding tendency
inadequate synthesis of osteoid
impaired wound healing

Question 3

measure of bone turnover (serum marker)

Answer 3

osteocalcin

Question 4

osteoprotegrin does what

Answer 4

blocks RANK

Question 5

how long does bone mineralization take

Answer 5

12-15 days

Question 6

lamellar bone

Answer 6

mature, orderly arrangement of collagen and osteoid

Question 7

woven bone

Answer 7

immature, disorderly

Question 8

cortical bone

Answer 8

on the outside

Question 9

medullary bone

Answer 9

on the inside, softer

Question 10

compact bone

Answer 10

mostly found in the cortical bone. Or flat bones

circumferential lamellae with haversian. No spaces

Question 11

cancellous (trabecular) bone

Answer 11

is in the medullary locations.
medullary bone
hematopoeisis
w/ lamellae in between (spicules)

Question 12

diseases impacting synovium

Answer 12

CVD, Lupus, RA, Lymes disease

Question 13

dysplasias

Answer 13

abnormalities of bone and cartilage organogenesis
Achondroplasia
Osteogenesis Imperfecta
Osteopetrosis

Question 14

dysostoses

Answer 14

congenital absence of bone, inappropriately fused bone, supernumerary bone

Question 15

Activating mutation of FGFR3 receptor of the growth plate:

Answer 15

inhibits cartilage proliferation and decreased endochondral ossification.
achondroplasia

Question 16

with rhizomelic shortening of the arms and legs

Answer 16

achondroplasia

Question 17

midface hypoplasia results in

Answer 17

causes dental crowding, obstructive apnea, and otitis media

Question 18

why do 3-7% of achondroplasia patients die within first year of life

Answer 18

because of brainstem compression (central apnea) or obstructive apnea

Question 19

comorbidities with achondroplasia

Answer 19

• restrictive pulmonary disease with infection and cor pulmonale
• spinal stenosis
• ear infections

Question 20

sudden death in achondroplasia caused by

Answer 20

brainstem compression

Question 21

Lack of normal maturation and columnation and the presence of a bony end-plate in the metaphysis

Answer 21

achondroplasia

Question 22

Abnormality in collagen type genes.

Answer 22

osteogenesis imperfecta

Question 23

Within all OI, the fundamental abnormality is

Answer 23

too little bone

Question 24

OI type I

Answer 24

Hearing loss (ossicles), Dental imperfections (abnormal dentin).

Question 25

type II OI

Answer 25

usually lethal in utero or within days after birth. Excessive bone fragility with multiple bone fractures while the fetus is in the uterus. Blue sclerae.

Question 26

type III OI

Answer 26

Severe clinical form in children surviving the perinatal period. Multiple fractures, progressive deformities. The sclerae are normal.

Question 27

type IV OI

Answer 27

Compatible with survival. Short stature, moderate skeletal fragility.

Question 28

which types of OI are most compatible with life

Answer 28

I, IV

Question 29

Blue sclerae, dental abnormalities

Answer 29

OI

Question 30

Brown, short teeth result from

Answer 30

failure in the formation of dentin(dentinogenesis imperfecta)

Question 31

osteoclast deficiency/abnormality resulting in

Answer 31

osteosclerosis

Question 32

There is failure to resorb calcified cartilage in the growth plate.

Answer 32

osteopetrosis

Question 33

vitamin D deficiency

Answer 33

rickets/osteomalacia

Question 34

excess of unmineralized bone matrix

Answer 34

rickets/osteomalacia

Question 35

Overgrowth of growthplate at costochondral junction

Answer 35

rickets

Question 36

Rachitic rosary due to

Answer 36

pull on costochondral cartilage –also due to the pull of the respirtaory muscles with bending of the ribs inward resulting in pidgeon breast deformity

Question 37

osteomalacia findings

Answer 37

Prominent unmineralized osteoid in bone, weak bone, vulnerable to fractures (mostly vertebrae and femoral neck).

Question 38

are there similar skeletal deformities in osteomalacia?

Answer 38

no. bone is already formed

Question 39

differentiating osteomalacia from osteopetrosis

Answer 39

osteomalacia: decreased mineralized bone, same hyper cartilage

Question 40

lucent lines (pseudo fractures)

Answer 40

osteomalacia

Question 41

When mechanical stress is placed upon bone, osteoprogenitor cells produce WNT proteins that bind to receptors on osteoblasts, increasing β-catenin. As a result, what protein is most likely to diminish osteoclast activity and increase bone formation?

Answer 41

osteoprotegerin can bind RANK ligand to reduce its binding to RANK and reduce the nuclear factor kappa B transcription factor and diminish osteoclast activity.

Question 42

cranial nerve involvement

Answer 42

osteopetrosis

Question 43

multiple osteomas associated with

Answer 43

Gardner's syndrome

Question 44

metastatic bone lesions favor | sites

Answer 44

axial skeleton, proximal long bones

Question 45

prostatic mets are (lytic or blastic)

Answer 45

blastic- denser

Question 46

thyroid mets are (lytic or blastic)

Answer 46

lytic- much looser

Question 47

gardner's syndrome findings

Answer 47

osteomas, epidermal cysts, fibromatoses and | colonic adenomatous polyposis

Question 48

sharp, nocturnal pain relieved by aspirin

Answer 48

osteoid osteoma

Question 49

duller pain not relieved by aspirin

Answer 49

osteoblastoma

Question 50

which is larger- osteoid osteoma or osteoblastoma

Answer 50

osteoblastoma > 2 cm

Question 51

enchondromatosis

Answer 51

more likely to undergo malignant transformation

Question 52

maffuccis's

Answer 52

chondromas associated with angiomas

Question 53

malignancies associated with maffucci's

Answer 53

ovary and brain malignancies

Question 54

genetic syndromes associated with soft tissue tumors

Answer 54

NF1, FAP

Question 55

HHV-8 associated with

Answer 55

Kaposi's sarcoma

Question 56

Gardner's syndrome

Answer 56

Fibromatosis of the mesentery Adenomas and Colon cancer Osteomas of bone Mutation in APC gene on chromosome 5

Question 57

polyostotic FD+ endocrinopathy (precocious puberty) and skin pigmentation

Answer 57

Albright- McCune syndrome