MSK Pathology Flashcards

(115 cards)

1
Q

Achondroplasia is a defect in

A

cartilage = failure of long bone growth

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2
Q

Specific achondroplasia mutation

A

Autosomal Dominant Mutation of fibroblast growth factor receptor (FGFR)–short arm of chromosome 4

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3
Q

Achondroplasia presentation

A

Short arms and legs (endochondral ossificaiton), normal torso and head (intramembranous ossification is unaffected)

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4
Q

Osteogenesis imperfecta defect

A

AD mutation resulting in defective Type I collagen synthesis

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5
Q

The classic triad of OI- type 3

A

Multiple Fractures in strange places Blue sclera Hearing loss (inner ear bones Fx)

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6
Q

Examples of type 1 collagen

A

tendons, ligaments, the endomysium of myofibrils, interstitial stroma, the organic part of the bone, the dermis, the dentin, and organ capsules.

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7
Q

Labs for Osteopetrosis

A

Pancytopenia

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8
Q

2 Neuro complications of osteopetrosis

A

Vision/hearing impariment due to compression of nerves by foramina

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9
Q

Pathogenesis of osteopetrosis. Mechanism?

A

Lack of osteoclast function

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10
Q

Rickets/Osteomalacia defect

A

Excessive unmineralized osteoid; bad quality bone

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11
Q

MCC of Rickets/OM

A

Vitamin D deficiency

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12
Q

Osteomalacia lab values (Ca, Phos, PTH, Alk Phos)

A

Low Ca and Phos (low vitD) High PTH High Alk Phos (osteoblasts are driven by PTH. They are still working fine)

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13
Q

Osteoporosis is a reduction in

A

Trabecular bone mass (endosteal and periosteal resorption)

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14
Q

3 major Risk factors for osteoporosis

A

weight bearing exercise, menopause, Age

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15
Q

Effect of estrogen on bone development

A

Estrogen inhibits RANK, Loss of estrogen after menopause = more active osteoclasts

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16
Q

2 fracture-related complications of osteoporosis

A

Vertebral compression Fx Hip Fx

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17
Q

besides vertebral fractures which vertebral complications do osteoporosis pts have

A

Kyphosis and decreased height

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18
Q

Osteoporosis labs (Ca, Phos, PTH, alk phos)

A

All normal! Normal labs in the face of bone pain distinguishes this from osteomalacia

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19
Q

Paget disease of bone underlying problem

A

Imbalance b/w osteoblasts and osteoclasts (too much), viral infection, genetics

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20
Q

paget disease progression

A

Overactive osteoclasts –> osteoclasts start burning out –> osteoblasts activate –> lay down poor structure bone

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21
Q

A 58-year-old male patient reported to the dental hospital with a complaint of difficulty in vision.

History revealed that there were swellings in the skull bones, which started 6 years back. All swellings gradually increased in size. X-Ray skull attached. Diagnosis?

A

Pagets

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22
Q

4 classic SSx of paget’s disease of bone

A

Bone Pain Hearing loss/ Vision changes Increasing hat size Coarsening of facial features

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23
Q

Characteristic lab abnormality in paget’s disease

A

Isolated elevated ALk Phos with everything else normal

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24
Q

histology of paget’s

A

mosaic pattern of lamellar bone

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25
2 major complications of paget disease
High output heart failure, Osteosarcoma
26
location of hematogenous osteomyelitis in kids (\< 2 years) vs adults
Kids = epiphysis, Adults = metaphysis
27
MCC (most common cause) of osteomyelitis overall
S. aureus
28
MCC of osteomyelitis in diabetic or IV drug user
Psudomonas
29
MCC of osteomyelitis in sickel cell
salmonella typhi
30
Xray appearance of osteomyelitis
Lytic bone lesion surrounded by sclerosis (lytic lesion = sequstrum) (sclerosis = involurcrum) The diagnosis is strongly suggested by the characteristic radiographic findings of a lytic focus of bone destruction surrounded by a zone of sclerosis.
31
Osteoma association
Gardner syndrome (FAP, fibromatosis, osteomas)
32
Osteoid osteoma is a tumor of
Osteoblasts
33
Gross appearance of osteoid osteoma
Tumor of osteoblasts surrounded by a ring of reactive bone
34
Osteoid osteoma imaging
Bony mass with a radiolucent core (osteioid from the osteoblasts)
35
Classic location of osteoid osteoma
Cortex of long bones
36
The classic presentation of osteoid osteoma
Humerus or femur pain that resolves with aspirin
37
Osteoblastoma location
Everything is the same as an ostoid osteoma, but it is located in the vertebra
38
Osteoblastoma presentation
Back pain that does not respond to aspirin (r/o osteoid osteoma)
39
diagnosis and location of origin
Osteochondroma arises from the lateral projection of the epiphyseal plate
40
Structure of osteochondroma
Bone with an overlying cartilage cap
41
Giant cell tumor location
Epiphysis of long bones. ONLY TUMOR IN THE EPIPHYSIS
42
Giant cell tumor is a proliferation of
Stromal cells
43
Xray of Giant cell tumor
"bubble" appearance of the epiphysis
44
osteosarcoma AKA
osteogenic sarcoma
45
Osteogenic sarcoma is a proliferation of
osteoblasts
46
primary osteosarcoma location
METAPHYSIS of long bones (unlike giant cell tumors)
47
3 major risk factors for sec. osteosarcoma
Paget Disease Radiation exposure LiFraumeni Sydnrome Gardner
48
diagnosis?
Xray of osteosarcoma
49
Ewing sarcoma is a proliferation of
neuroectodermal cells (poorly differentiated)
50
Ewing sarcoma location and population
Diaphysis of long bones in kids
51
Ewing sarcoma translocation
11:22
52
Xray of Ewing sarcoma
Onion skinning
53
mechanism of onion skinning
As tumor grows laterally, the periostium will continue forming new layers
54
chondrosarcoma location
pelvis or central skeleton
55
Osteoblastic bone metastasis
Prostate Cancer
56
Ewing sarcoma histology
malignant small blue cells
57
Articular cartilage is composed of
Type II collagen (hyaline)
58
The classic pattern of OA involvement
ASYMMETRIC involvement of Leg and hip joints + DIPs
59
Classic OA presentation
Morning stiffness that worsens with activity and improves with an NSAID
60
3 Xray findings of OA
Osteophytes UNILATERAL Joint space narrowing Subchondral sclerosis
61
RA HLA association
HLA DR4
62
Histology of RA
Pannus formation --\> inflammed granulation tissue
63
Pathogenesis of RA, 3 steps
autoimmune synovial Inflammation --\> pannus formation --\> bony ankylosis (cartilage and bone damage)
64
Rheumatoid factor description
IgM against IgG
65
Most specific serum marker for RA
anti CCP (cyclic citrullinated peptide)
66
A pattern of joint involvement in RA
Symmetric involvement of PIPs, Wrists, elbows and knees NO DIP!
67
3 Extraarticular manifestations of RA
Rheumatoid Nodules Amyloidosis and synovial cysts
68
Rheumatoid nodule histology
Fibrinoid necrosis surrounded by palisading histocytes
69
cell type responsible for swan neck deformity
Myofibroblasts w/in the granulation tissue of the pannus and ossification
70
Xray of RA
BILATERAL joint space narrowing, and flexion-hyperextension abnormality
71
2 complications of RA
Anemia of chronic Dz (elevated hepcidin) Secondary amyloidosis (constant inflammation)
72
Type of pain in all seronegative spondyloarthropathies
Similar to RA. Worse in morning, improves with use
73
4 subtypes of seronegative spondyloarthropathies and their pattern of joint involvement (Symmetric vs Asymmetric) HLA association :
PAIR ASAS Psoriatic (Asymmetric) Ankylosing spondylitis (Symmetric) Inflammatory Bowel Dz (Asymmetric) Reactive Arthritis (Symmetric) HLA B27
74
Psoriatic arthritis x-ray finding Other findings:
Pencil in cup deformity of DIP Nail deformities
75
Ankylosing spondylitis joints involved
Spine and bilateral sacroiliac- may lead to vertebral fusion
76
Ank Spond Extraarticular manifestation
Uveitis
77
Reactive Arthritis (Reiter) triad
Conjunctivitis Urethritis Arthritis "Cant pee, cant see, cant climb a tree"
78
CLassic reactive arthritis case
2 weeks after GI or STD, pt presents with knee or low back pain
79
Joint fluid in reactive arthritis
Negative for infection
80
3 MCC septic arthritis
Staph Strep Gonorrhea
81
Atypical Triad of gonococcal arthritis
Polyarthralgia Tenosynoviits Dermatitis (pustules)
82
Joint fluid difference in Acute Gout vs Septic arthritis
Septic = purulent w/ WBCs but no crystals Gout= WBCs + crystals
83
3 common causes of secondary gout
Leukemia/Myeloproliferative Disorders (increased cell turn over) Lesch Nyhan Renal insufficiency
84
Mechanism of Lesch Nyhan causing gout
HGPRT deficiency = no salvage pathway of purines = increased Purine metabolism into Uric Acid
85
What is the actual causative agent in Gout
Monosodium Urate Crystals
86
Major cellular mediator in gout flares
Neutrophils
87
histology/gross of tophi
Aggregates of crystals with fibrosis and giant cell reactions Essentially it is chronic granulomatous inflammation around the crystals (i.e. giant cells and fibrosis)
88
where are tophi located
soft tissues and joints
89
Gout synovial fluid analysis
Needle-shaped crystals with negative birefringence. The crystals that turn yellow are the ones that are parallel to the light The ones that are blue are perpendicular
90
Crystals in pseudogout
Calcium pyrophosphate, rhomboid-shaped, blue
91
Fibromyalgia SSx
Widespread (not just proximal) PAIN w/o weakness Sleep problems Concentration problems
92
Polymyalgia rheumatica SSx
Proximal muscle pain WITHOUT weakness
93
3 Dermatomyositis SSx
Bilateral proximal muscle WEAKNESS, Rash Gottron papules
94
Ab to differentiate dermatomyositis from SLE (both cause malar rash and can have +ANA.
anti Jo1
95
Biopsy of dermatomyositis
Perimysial inflammation "perimysium is closest to the skin, Dermatomyositis involves the skin with CD 4":
96
Cell type involved in dermatomyositis
CD4 T cells
97
Biopsy of polymyositis
Endomysial inflammation
98
Cell type in polymyositis
CD8 poly = multiple...8 is more than 4...polymyositis has CD8. dermatomyositis has CD4
99
Myasthenia gravis effects on receptor
Competitive inhibition of post synaptic Ach receptor --\> this is why high ACh can reverse sx
100
Association with MG
Thymic hyperplasia/thymoma Thymectomy will improve Non-MSK
101
Lambert Eaton defect
AutoAb to presynaptic Ca Channels, preventing Ach release
102
MCC of Lambert Eaton
Small cell lung cancer
103
How to differentiate MG from LE on PE
MG symptoms worsen with use but improve with ACHEs LE symptoms improve with use but not ACHEs
104
liposarcoma is a proliferation of which cell
Lipoblast
105
most common site for rhabdomyosarcoma
Vagina of a young girl
106
Rhabdomyosarcoma is a malignancy of
Rhabdomyoblasts
107
Rhabdomyosarcoma immunohistochemical stain
Desmin positive
108
Which bones in fibrous dysplasia
Flat bones (skull, face, ribs)
109
3 stages of osteitis deformans
Lytic - Osteoclasts go crazy Mixed - Osteoclasts start burning out, osteoblasts wake up Sclerotic - osteoblasts go crazy
110
Tumors that metastasize to bone
Breast \> Prostate \> Lung \> Thyroid \> Kidney \> Testicular
111
Tumor in the diaphysis/medulla of a small bone (metatarsal, metacarpal)
Chondroma
112
A 25-year-old man presents with lower back pain and a diastolic murmur at the right 2nd intercostal space. Diagnosis?
Ankylosing Spondyitis
113
why does alcohol precipitate gout attacks?
EtOH competes for the same excretion sites in the kidney as uric acid, leading to a build up of uric acid in the blood
114
Other name- anti-Jo 1 Ab
histidyl tRNA synthase
115
A two-month-old male infant was referred to the department of pediatric gastroenterology because of hepatosplenomegaly and thrombocytopenia. Past medical history of the patient revealed that he was hospitalized in the first day of his life in the neonatal unit because of poor feeding and onset of sepsis workup was done. X-ray shows thickened bone and decreased marrow cavities of various bones. Diagnosis?
osteopetrosis