B6-066 Pagets Disease Flashcards

1
Q

what physiologic states can be associated with ALP elevation in adults? [2]

A

pregnancy
fed state

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2
Q

first test that can be used to help differentiate the source of elevated ALP

A

ggt

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3
Q

test that is a specific marker for bone osteoblast activity

A

bone ALP

**fractionated out to be bone specific

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4
Q

causes of elevated bone ALP [5]

A

primary hyperparathyroidism- check Ca
thyrotoxicosis- check TSH
osteomalacia- check vit. D
bone malignancy
Paget’s

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5
Q

what imaging might you want to evaluated for Pagets? [2]

A

Xray
radionucleotide bone scan

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6
Q

X ray features of Pagets [2]

A

coarsened trabeculae
thickened cortical bone

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7
Q

areas most commonly affected by Pagets [3]

A

femur, knee, and pelvis

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8
Q

Paget’s is caused by overactive […]

A

osteoclasts

**leads to compensatory increase in osteoblastic formation

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9
Q

describe the structural changes in bone induced by Paget’s

A

expanded, less compact, more vascular

**more susceptible to fracture

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10
Q

focal areas of increased and disorganized bone remodeling

A

Paget’s

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11
Q

inheritance pattern of familial Pagets

A

AD w/ incomplete penetrance

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12
Q

bone pain in Paget’s is due to [2]

A

increased metabolic activity
osteoarthritis

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13
Q

indications of treatment of Paget’s [4]

A
  1. symptoms from active disease
  2. pagetic lesions involving the weight bearing skeleton
  3. metabolically active pagetic lesions as defined by bone turnover and bone scan
  4. potential consequences of bony overgrowth (hearing loss)
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14
Q

treatment for Pagets

A

bisphosphonates

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15
Q

osteoclast takes it up, rendering it inactive

A

bisphosphonates

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16
Q

most common side effects of bisphosphonates [3]

A

upper GI irritation (oral)
acute phase reaction (IV)
severe MSK pain

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17
Q

acute phase reaction that occurs with IV bisphosphonate […] with subsequent doses

A

decreases

18
Q

teeth extraction/dental abscess can be a risk factor for […] caused by bisphosphonates

A

osteonecrosis of the jaw

19
Q

if a bisphosphonate is contraindicated, what can be used as treatment

A

calcitonin take indefinitely

20
Q

in a younger patient with more limited disease, consider

A

oral bisphosphonates

21
Q

oral bisphosphonates [2]

A

risedronate
alendronate

22
Q

IV bisphosphonate

A

zoledronic acid

23
Q

abnormal “mosaic” bone archteicture

A

Paget’s

24
Q

[identify the condition based on the following lab values:

serum Ca+: high
Phos: low
ALP: high
PTH: high

A

primary hyperparathyroidism

25
Q

what imaging is used to localize the disease?

A

bone scan

26
Q

paget disease presents as […] in a majority of patients

A

incidental finding

27
Q

ALP will begin to fall within […] days of treatment

A

10

28
Q

[identify the condition based on the following lab values:

serum Ca+: normal
Phos: normal
ALP: high
PTH: normal

A

paget’s

29
Q

[identify the condition based on the following lab values:

serum Ca+: low
Phos: high
ALP: high
PTH: high

A

secondary hyperparathyroidism

30
Q

[identify the condition based on the following lab values:

serum Ca+: normal
Phos: normal
ALP: normal
PTH: normal
but decreased bone mass on DEXA

A

osteoporosis

31
Q

osteoblast precursor cells

A

mesenchymal stem cells

32
Q

ALP is higher in pregnancy due to

A

placental synthesis of ALP

**need to seek out the reference range for pregnant patients in order to adequately assess

33
Q

[…] can be used to determine if elevated ALP is due to bony or hepatobiliary origin

A

GGT

**if elevated, suggests hepatobiliary

34
Q

in Paget’s, osteoclast activity is […] and osteoblast activity is […]

A

increased
increased

**both are increased, however, osteoclast activity outweighs osteoblast activity

35
Q

Paget typically onsets after what age?

A

55

36
Q

what populations is Pagets more common in?

A

men
european descent

37
Q

first-line medication for Paget

A

bisphosphonates

38
Q

oral bisphosphonates should not be used in patients with […]

A

esophageal disorders

**use IV zolendronic acid

39
Q

second line treatment for Pagets

A

calcitonin

**if bisphosphonates are contraindicated

40
Q

bisphosphonates inhibit osteoclastic activity via attachment to […] sites on bony surfaces

A

hydroxyapatite

41
Q

when the osteoclasts resorb bone impregnanted with bisphosphonates, it impairs the activity of the […]

A

ruffled border

**cannot adhere to surface