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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
Idenify the condition associated with the gross image attached.
The image shows Brown tumor The condition associated is Hyperparathyroidism
26
**What is the underlying basis of the condition described?** Blue sclerae multiple skeletal fractures hearing loss small, misshapen and blue-yellow teeth
Defective synthesis of Type I collagen | (Osteogenesis imperfecta)
27
Classic radiologic finding of Osteomalacia
pseudofractures of Milkman-Looser syndrome (radiolucent transverse defects)
28
Which bone cell type is most active in the stage of Osteitis deformans described below: initial osteolytic stage
Osteoclasts
29
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?**
FGFR3 mutation
30
Predict the following hormone levels in Rickets/Osteomalacia Serum Calcium Serum Phosphorus Serum PTH Serum ALP
31
See the attached image . What's the most likely diagnosis?
Osteogenesis imperfecta
32
How does chronic kidney disease lead to secondary hyperparathyroidism?
Decreased phosphate excretion **--\>** hyperphosphatemia **--\>** hypocalcemia **---\>**secondary hyperparathyroidism
33
What are the 3 key ways in which renal osteodystrophy occurs?
See the flow chart in the attached image
34
1. Identify the finding shown in the attached image. 2. Which condition is it associated with?
1. Loss of palisades of cartilage 2. Rickets
35
Normal sized head and vertebral column Shortened arms and legs Short stature, rhizomelic shortening of limbs, frontal bossing, midface deficiency **Mode of inheritance?**
Autosomal dominant
36
**What is the typical radiologic finding associated with the condition described below?** 2 year old child fractures anemia optic atrophy, deafness, and facial paralysis
Diffuse symmetric sclerosis (bone-in-bone)
37
Shown here is a photomicrograph of a bone biopsy stained with von Kossa stain Identify the finding shown
**Osteomalcaia** The surfaces of the bony trabeculae (black) are covered by a thicker than normal layer of osteoid (red)
38
List 1 tumor condition that may arise from Pagetic bone
osteosarcoma
39
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
Osteoblasts
40
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?**
This disorders is assoc with **impaired proliferation of cartilage at the growth plate**. Therefore, the main cell type involved is the **chondrocyte.**
41
1 consequence of accumulation of vertebral body fractures in senile osteoporosis
progressive kyphosis of the thoracic spine (Dowager's hump)
42
Best investigation to estimate bone loss in osteoporosis
Dual-energy x-ray absorptiometry
43
Why is the growth plate thickened in Rickets?
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
**Predict the following hormone levels in Paget's disease (Osteitis deformans)** Serum Calcium Serum Phosphorus Serum PTH Serum ALP
45
Why is the sclera blue in osteogenesis imperfecta?
decreased collagen content -----\>makes the sclera translucent-----\>partial visualization of the underlying choroid
46
WHO definition of Osteopenia
T-scores between −1 and −2.5
47
Identify the condition from the radiologic image attached. List 3 key findings
**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
Modality of investigation used to estimate bone mineral density
Dual-energy x-ray absorptiometry (DXA) also known as DEXA scan
49
**Predict the following hormone levels in primary hyperparathyroidism** Serum Calcium Serum Phosphorus Serum PTH Serum ALP
50
What is the technique used to measure deposition of osteoid is osteomalacia?
double tetracycline labeling
51
Why are African American women less prone to osteoporosis?
Due to higher peak bone mass
52
**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
Osteitis Deformans (The Xray shows extensive bony sclerosis in the region of the hip joint on the left)
53
Why does carbonic anhydrase II enzyme deficiency cause osteopetrosis?
interference with the process of acidification of the osteoclast resorption pit
54
**Predict the following hormone levels in Osteoporosis** Serum Calcium Serum Phosphorus Serum PTH Serum ALP
55
micromelic shortening of the limbs frontal bossing relative macrocephaly small chest cavity bell-shaped abdomen
Thanatophoric dysplasia
56
Osteitis fibrosa cystica is a combination of which 3 key events/findings in hyperparathyroidism
increased bone cell activity + peritrabecular fibrosis + cystic brown tumors

PATHPROS (39 decks)

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