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PATHPROS > MUSCULOSKELETAL PATHOLOGY-2 > Flashcards

MUSCULOSKELETAL PATHOLOGY-2 Flashcards

Covers the Congenital and Metabolic bone disorders

1
Q

What is the most likely basis for defective osteoclastic activity in this patient?

Anemia, Optic atrophy, Facial paralysis

Xray- diffuse symmetric skeletal sclerosis

Renal tubular acidosis

Cerebral calcification

A

Carbonic anhydrase II enzyme deficiency

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2
Q

2 consequences of Scurvy in the bones and joints

A

Subperiosteal hemorrhages

Joint hematomas

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3
Q

2 key factors in the pathogenesis of senile osteoporosis

A

decreased activity of 1 alpha hydroxylase

decreased osteoblastic activity

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4
Q

1. Identify the condition described.

2 year old child

fractures

anemia

optic atrophy, deafness, and facial paralysis

diffuse symmetric skeletal sclerosis on Xray

2. What is the pathogenesis?

A
  1. Osteopetrosis
  2. impaired formation or function of osteoclasts
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5
Q

Why do patients with osteopetrosis experience optic atrophy, deafness / facial paralysis?

A

Because the neural foramina are small and compress exiting nerves

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6
Q

List the 3 sequential phases of osteitis deformans

A

(1) an initial osteolytic stage
(2) a mixed osteoclastic-osteoblastic stage, which ends with a predominance of osteoblastic activity and evolves ultimately into
(3) a final burned-out quiescent osteosclerotic stage

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7
Q

Why is there exacerbation of underlying cardiovascular disease in Osteitis Deformans?

A

Increased blood flow from Increased arteriovenous shunts may cause high-output heart failure

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8
Q

3 cardinal features of Carbonic anhydrase enzyme II deficiency

A

Osteopetrosis

Renal tubular acidosis

Cerebral calcification

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9
Q

List the 3 bone resorptive cytokines

A

IL-1, IL-6 and TNF

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10
Q

Why do patients with osteopetrosis have pancytopenia/anemia?

A

Due to due to persistence of the primary spongiosa, which fills the medullary cavity and displaces the hematopoietic marrow

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11
Q

Predict the following hormone levels in secondary hyperparathyroidism due to CKD

Serum Calcium

Serum Phosphorus

Serum PTH

Serum ALP

A
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12
Q

WHO definition of osteoporosis

A

T-score of −2.5 or lower

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13
Q

Identify the condition from the radiologic findings attached

A

Osteitis Deformans

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14
Q

Morphologic findings in osteomalacia

A

exaggeration of osteoid seams

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15
Q

3 morphologic consequences of hyperparathyroidism

A

Osteoporosis

Brown tumors

Osteitis fibrosa cystica

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16
Q

Predict the following hormone levels in hypervitaminosis D

Serum Calcium

Serum Phosphorus

Serum PTH

Serum ALP

A
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17
Q

List 5 clinical features of Osteitis deformans

A

Pain localized to the affected bone

increased hat size

hearing loss

anterior bowing of the femurs and tibiae

Chalk stick fracture

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18
Q

What is the fundamental defect in Rickets/Osteomalacia?

A

impairment of mineralization and a resultant accumulation of unmineralized matrix.

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19
Q

What is the basis of the condition described?

Child presenting with

Anemia

Optic atrophy

Facial paralysis

See attached image

A

impaired formation or function of osteoclasts

(Osteopetrosis/Marble-bone disease)

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20
Q

Identify the metabolic bone disorders from the morphologic findings shown

A

Osteoporosis

(reduction in the number and size of trabeculae)

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21
Q

How do the bone resorptive cytokines favor osteoclastic activity in post menopausal osteoporosis?

A

Expression of RANK and RANKL

Inhibition of OPG expression

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22
Q

List the 6 non modifiable risk factors for osteoporosis

A
  • Caucasian ethnicity
  • Rheumatoid arthritis
  • Advancing age
  • Postmenopausal status
  • Previous fragility fracture
  • Genetics
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23
Q

List 4 modifiable risk factors for osteoporosis

A
  • Low body weight
  • Excessive alcohol consumption
  • History of heavy/protracted glucocorticoid use
  • Current cigarette smoking
24
Q

List 2 morphologic findings of rickets

A

Deposition of osteoid matrix on inadequately mineralized cartilaginous remnants

Growth plate is conspicuously thickened, irregular and lobulated

25
Q

Idenify the condition associated with the gross image attached.

A

The image shows Brown tumor

The condition associated is Hyperparathyroidism

26
Q

What is the underlying basis of the condition described?

Blue sclerae

multiple skeletal fractures

hearing loss

small, misshapen and blue-yellow teeth

A

Defective synthesis of Type I collagen

(Osteogenesis imperfecta)

27
Q

Classic radiologic finding of Osteomalacia

A

pseudofractures of Milkman-Looser syndrome

(radiolucent transverse defects)

28
Q

Which bone cell type is most active in the stage of Osteitis deformans described below:

initial osteolytic stage

A

Osteoclasts

29
Q

Normal sized head and vertebral column

Shortened arms and legs

Short stature, rhizomelic shortening of limbs, frontal bossing, midface deficiency

What’s the etiopathogenesis of this disorder?

A

FGFR3 mutation

30
Q

Predict the following hormone levels in Rickets/Osteomalacia

Serum Calcium

Serum Phosphorus

Serum PTH

Serum ALP

A
31
Q

See the attached image .

What’s the most likely diagnosis?

A

Osteogenesis imperfecta

32
Q

How does chronic kidney disease lead to secondary hyperparathyroidism?

A

Decreased phosphate excretion –> hyperphosphatemia –> hypocalcemia —>secondary hyperparathyroidism

33
Q

What are the 3 key ways in which renal osteodystrophy occurs?

A

See the flow chart in the attached image

34
Q
  1. Identify the finding shown in the attached image.
  2. Which condition is it associated with?
A
  1. Loss of palisades of cartilage
  2. Rickets
35
Q

Normal sized head and vertebral column

Shortened arms and legs

Short stature, rhizomelic shortening of limbs, frontal bossing, midface deficiency

Mode of inheritance?

A

Autosomal dominant

36
Q

What is the typical radiologic finding associated with the condition described below?

2 year old child

fractures

anemia

optic atrophy, deafness, and facial paralysis

A

Diffuse symmetric sclerosis (bone-in-bone)

37
Q

Shown here is a photomicrograph of a bone biopsy stained with von Kossa stain

Identify the finding shown

A

Osteomalcaia

The surfaces of the bony trabeculae (black) are covered by a thicker than normal layer of osteoid (red)

38
Q

List 1 tumor condition that may arise from Pagetic bone

A

osteosarcoma

39
Q

Which bone cell type is most active in the stage of Osteitis deformans described below:

mosaic pattern (jigsaw puzzle) of lamellar bone visible due to prominent cement lines, which join haphazardly oriented units of lamellar bone

A

Osteoblasts

40
Q

Normal sized head and vertebral column

Shortened arms and legs

Short stature, rhizomelic shortening of limbs, frontal bossing, midface deficiency

What cell type is involved in the pathogenesis of this disorder?

A

This disorders is assoc with impaired proliferation of cartilage at the growth plate. Therefore, the main cell type involved is the chondrocyte.

41
Q

1 consequence of accumulation of vertebral body fractures in senile osteoporosis

A

progressive kyphosis of the thoracic spine (Dowager’s hump)

42
Q

Best investigation to estimate bone loss in osteoporosis

A

Dual-energy x-ray absorptiometry

43
Q

Why is the growth plate thickened in Rickets?

A

in children, the growth plates (physes) are open.

Osteoclastic activity functions only if the matrix is mineralized.

In rickets, the growth plate does not calcify normally–>no osteoclastic resorption—>thick growth plate

44
Q

Predict the following hormone levels in Paget’s disease (Osteitis deformans)

Serum Calcium

Serum Phosphorus

Serum PTH

Serum ALP

A
45
Q

Why is the sclera blue in osteogenesis imperfecta?

A

decreased collagen content —–>makes the sclera translucent—–>partial visualization of the underlying choroid

46
Q

WHO definition of Osteopenia

A

T-scores between −1 and −2.5

47
Q

Identify the condition from the radiologic image attached.

List 3 key findings

A

Fraying – edge of metaphysis loses its sharp border

Splaying – widening of the metaphyseal end of bone

Cupping – edge of the metaphysis changes from a flat/ convex surface to a concave surface.

Increased distance between ends of bone shafts and the epiphyseal center

48
Q

Modality of investigation used to estimate bone mineral density

A

Dual-energy x-ray absorptiometry (DXA) also known as DEXA scan

49
Q

Predict the following hormone levels in primary hyperparathyroidism

Serum Calcium

Serum Phosphorus

Serum PTH

Serum ALP

A
50
Q

What is the technique used to measure deposition of osteoid is osteomalacia?

A

double tetracycline labeling

51
Q

Why are African American women less prone to osteoporosis?

A

Due to higher peak bone mass

52
Q

What’s the diagnosis?

55 year old male

Pain in the affected bone

Hearing loss

elevated serum alkaline phosphatase levels

normal serum calcium and phosphorus

Xray findings attached

A

Osteitis Deformans

(The Xray shows extensive bony sclerosis in the region of the hip joint on the left)

53
Q

Why does carbonic anhydrase II enzyme deficiency cause osteopetrosis?

A

interference with the process of acidification of the osteoclast resorption pit

54
Q

Predict the following hormone levels in Osteoporosis

Serum Calcium

Serum Phosphorus

Serum PTH

Serum ALP

A
55
Q

micromelic shortening of the limbs

frontal bossing

relative macrocephaly

small chest cavity

bell-shaped abdomen

A

Thanatophoric dysplasia

56
Q

Osteitis fibrosa cystica is a combination of which 3 key events/findings in hyperparathyroidism

A

increased bone cell activity + peritrabecular fibrosis + cystic brown tumors

PATHPROS (36 decks)

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