MSK Path High Yield Flashcards

1
Q

“failure of longitudinal bone growth (endochondrial ossification) leading to short limbs”

A

Achondroplasia

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2
Q

What is the mutation in achondroplasia?

A

a constitutive activation of fibroblast growth that inhibits chondrocyte proliferation

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3
Q

“trabecular (spongy) bone loses mass and interconnections despote normal bone mineralization”

A

Primary osteoporosis

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4
Q

Which 4 drugs classes cause osteoporosis?

A

1- antconvulsants
2- steroids
3- anticoagulants
4- Thyroid replacement therapy

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5
Q

Which type of osteoporosis is associated with increased osteoclast activity?

A

Type I post menopausal

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6
Q

Which type of osteoporosis is associated with decreased osteoblast activity?

A

Type II (senile)

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7
Q

“failure of normal bone resportion due to defective osteoclasts–> thickened dense bone”

A

osteopetrosis

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8
Q

What are associated findings with osteopetrosis?

A

Visual and

Pancytopenia and estramedullary hematopoiesis

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9
Q

What is the mutation in osteopetrosis?

A

Carbonic anhydrase II

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10
Q

“defective bone mineralization/ calficification of osteoid–> soft bones that bow out”

A

Osteomalacia/ rickets

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11
Q

What is the PTH level in osteomalacia/ rickets? The ALP level?

A

Increased!!!

ALP is increased because of the osteoblasts

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12
Q

“increase in both osteoblastic and osteoclastic activity leading to a mosaic bone pattern of woven and lamellar bone”

A

Paget disease of bone

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13
Q

What are the 4 stages of paget disease?

A

1- Lytic (osteoclasts)
2- Mixed (both)
3- Sclerotc (osteoblasts)
4- Quiescent (minimal activity)

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14
Q

What cancer is there an increased risk of with pagent disease of bone?

A

osteogenic sarcoma

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15
Q

What is a common cause of death in paget disease?

A

High Output cardiac failure

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16
Q

What is the most common site of osteonecrosis? What is the causative artery?

A

Femoral head

Medial circumflex femoral artery

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17
Q

What is Leff- Calve Perthes disease?

A

Idiopathic osteonecrosis

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18
Q

What are the abnormal lab values in osteoporosis?

A

None

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19
Q

What is the abnormal lab value in paget disease?

A

Increased ALP

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20
Q

What are the abnormal lab values in osteomalacia?

A

Decreased Ca
Decreased PO4
Increased ALP
Increased PTH

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21
Q

What are the abnormal lab values in Primary hyperparathyroidism?

A

Increased Ca
Decreased PO4
Increased PTH
Increased ALP

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22
Q

What are the abnormal lab values in secondary hyperparathyroidism?

A

Decreased Ca
Increased PO4
Increased ALP
Increased PTH

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23
Q

“brown tumor due to fibrous replacement of bone and subperiosteal thinning”

A

Osteitis fibrosa cystica

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24
Q

What are the 3 actions of vit D?

A

Intestine: Increased absortion of Ca and PO4

Kidney: increased reab of Ca and PO4

Bone: increased reab of calcium and phosphate

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25
Location of osteoma?
facial bones
26
What is condition is osteoma associated with?
Gardner syndrome
27
"benign tumor of osteoblasts surrounded by a rim of reactive bone"
Osteoid osteoma
28
Bone tumor associated with nighttime pain that improves with aspirin
Osteoid osteoma
29
Where are osteoid osteomas typically located?
Diaphysis
30
Where is osteoblastoma usually located?
Vertebrae
31
What is the major difference between osteoblastoma and osteoid osteoma?
Pain does not resolve with aspirin in osteoblastoma
32
Where are osteochondromas usually found?
metaphysis (lateral projection)
33
"Tumor of bone with overlying cartilage cap"
Osteochondroma
34
"malignant proliferation of osteoblasts"
Osteosarcoma
35
Where are Osteosarcomas usually located?
Metaphysis
36
What is the X ray appearance of osteosarcoma on Xray?
Codman triange or sunburst pattern
37
"bone tumor composed of multinucleated giant cells and stromal cells"
giant cell tumor
38
Where are giant cell tumors usually located?
Epiphysis of long bones
39
What is the appearance of giant cell tumors on xray?
soap bubble appearance
40
"malignant proliferation of poorly differentiated cells derived from neuroectoderm"
Ewing sarcoma
41
Where are ewing sarcomas usually located?
diaphysis
42
Joint pain that improved with use?
Rheumatoid arthritis
43
Joint pain that worsens with use?
Osteoarthrtis
44
What type of hypersensitivity is RA?
Type III and Type IV
45
Which joints are spared in osteoarthritis?
MCP
46
What joints are spared in RA?
DIP
47
"inflammatory granulation tissue formation in joints seen in RA"
Pannus
48
What type of necrosis do rheumatoid nodules exhibit?
Fibrinoid necrosis
49
What HLA type is associated with RA?
HLA DR4
50
What are the 2 autoantibodies associated withRA?
Anti- IgG antibody Anti- cyclic citrullinated peptide antibody
51
"reactive bony formation seen in osteoarthritis"
Osteophyte
52
"reactive bony formation seen in osteoarthritis"
Osteophyte
53
Autoantibodies seen in sjogrens?
Anti SS-A and SS-B (ANA antibodies)
54
What are 2 possible complications of Sjogrens?
Dental carries MALT (parotid enlargement)
55
Which genetic conditions cause an over production of uric acid?
Lesch- Nyhan syndrome von Gierke disease PRPP excess
56
What color are urate crystals under parallel light? Perpendicular light?
Parallel--> yellow Perpendicular--> blue
57
"white chalky aggregates of uric acid crystals with fibrosis and giant cells seen in gout"
tophus
58
:"pain and effusion in a joint caused by deposition of calcium pyrophosphate crystals within the joint space"
Pseudogout
59
Shape of pseudogout crystals?
rhomboid crystals
60
What are 3 possible causes of pseudogout?
1- hemochromatosis 2- hyperparathyroidism 3- osteoarthitis
61
What color are pseudogout crystals in parallel light?
Blue
62
Most common cause of infectious arthritis in older children and adults?
S. aureus
63
What is the most common cause of infectious arthritis in young adults?
Neisseria gonorrhea Migratory infections arthritis
64
What HLA type are Psoriatic arthritis, ankylosing spondyliti, IBD and Reactive arthritis associated wth?
HLA- B27
65
"chronic inflammatory disease of spine and sacroiliac joints"
Ankylosing spondylitis
66
What are 2 common findings of ankylosing spondylitis?
bamboo spine (vertebral fusion) aortic regurgitation
67
"conjunctivitis + urethritis + arthritis"
Reactive arthritis
68
What are 4 organisms associated with reactive arthritis?
Shigella Salmonella Yersinia Campylobacter Chlamydia
69
What 2 renal presentations are seen in lupus?
Membranous glomerulonephritis---> nephrotic DPGN--> Nephritic
70
What autoantibody is MOST SPECIFIC for lupus?
anti- dsDNA/ anti- smith
71
What are anti-smith antibodies against?
snRNPs
72
What is antihistone antibodies associated with?
drug induced lupus
73
What is needed for a diagnosis of antiphospholipid syndrome?
Hx of thrombosis Lupus anticoagulant/ anticoardiolipin/ ant beta2 glycoprotein sntibodies
74
Anticardiolipin antibodies can cause a false positive in what test?
VDLR
75
:immune mediated, widespread noncaseating granulomas with elevated ACE levels"
Sarcoidosis
76
What is the ratio of CD4/ CD8 in sarcoidosis?
Increased!
77
Is sarcoidosis a restrictive or obstructive lung disease/
restrictive
78
What causes hypercalcemia in sarcoidosis?
Increased 1alpha hydroxylase mediated vit D activation in macrophagges
79
"pain andstiffness in soulders and hips with fever, malaise and weightloss"
Polymyalgia rheumatica
80
What disease is polymyalgia rheumatica associated with?
temporal (giant cell) arteritis
81
"chronic widespread musculoskeletal pain associated with stiffness, paresthesias, poor sleep and fatigue"
fibromyalgia
82
What are the antibodies associated with polymyosiitis and dermatomyositis?
ANti-Jo-1 Anti SRP Anti Mi-2
83
"progressive PROXIMAL muscle weakness, characterized by endomysial inflammation with CD8+ cells"
Polymyositis
84
"progressive PROXIMAL muscle weakness with associated malar rash, grotton papules on elbows and knees, "shawl and face" rash"
Dermatomyositis
85
What is the cell type associated with polymyositis?
CD8+
86
What cel type is associated with dermatomyositis?
CD4+
87
Autoantibodies to presynaptic Ca channel leading to decreaed ACh release?
Lambert- Easton Syndrome
88
What condition is myasthenia gravis associated with?
Thymoma
89
"metaplasia of skeletal muscle into bone following muscular trauma"
Myositis ossificans
90
"autoimmunity, noninflammatory vasculopathy, collagen deposition with fibrosis"
Scleroderma Tightening of the skin without wrinkles
91
What autoantibody is associated with diffuse scleroderma?
anti- Scl-70 (anti- DNA topoisomerase I antibody)
92
What is seen in limited scleroderma?
CREST ``` Calcinosis Raynaud phenomenon Esophageal dysmotility Sclerodactyly Telandiectasia ```