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Step 1 2019 First Aid Flashcards > MuscSkCT - FA Patho p451 - 464 > Flashcards

MuscSkCT - FA Patho p451 - 464 Flashcards

1
Q

explain why estrogen is bone protective

A

estrogen blocks apoptosis in bone forming osteoblast and induces apoptosis in bone-resorbing osteoclast

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2
Q

what bone cell secretes acid and collagenases?

A

osteoclast

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3
Q

what cell is multinucleated and is derived from monocyte?

A

osteoclast

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4
Q

what bone path takes place in epiphysis?

A

giant cell tumors

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5
Q

what bone paths take place in metaphysis?

A

osteosarcoma (often around the knee), osteochondroma

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6
Q

what bone paths take place in diaphysis?

A

Ewing sarcoma, myeloma, osteoid osteoma, fibrous dysplasia

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7
Q

which bone path is associated with nighttime pain, central nidus, and pain released by aspirin?

A

osteoid osteoma

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8
Q

3 characteristics of Ewing sarcoma?

A
  1. anaplastic small blue cell malignant tumor 2. extremely aggressive with early metastases, but responsive to chemotherapy 3. “onion skin” periosteal reaction in bone
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9
Q

what population is most affected by Ewing sarcoma?

A

boys < 15 years old

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10
Q

what is the 2nd most common primary malignant tumor after multiple myeloma?

A

osteosarcoma (osteogenic sarcoma)

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11
Q

name 5 predisposing factors for osteosarcoma (osteogenic sarcoma)

A
  1. paget dz of bone 2. bone infarcts 3. radiation 4. familial retinoblastoma 5. Li Fraumeni syndrome (germline p53 mutation)
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12
Q

what is the age distribution on osteosarcoma?

A

biomodal distribution (10-20 yr old and more than 65)

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13
Q

what other malignancy has also bimodal distribution?

A

hodgkin (young adulthood and more than 55)

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14
Q

on x-ray, how will osteosarcoma look like?

A

codman triangle (from elevation of periosteum) or sunburst pattern on x-ray

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15
Q

what is the most common benign tumor?

A

osteochondroma

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16
Q

does osteochondroma transform to chondrosarcoma?

A

rarely yes

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17
Q

what is the characteristic of osteochondroma?

A

mature bone with cartilagionus (chondroid) cap

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18
Q

what bone tumor is characterized as soap bubble appearance on x-ray with multinucleated giant cells

A

giant cell tumor

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19
Q

what is the most common site of giant cell tumor?

A

epiphyseal end of long bone around knee (distal femur, prox tibia) - only 1 in this age group (20-40 yrs)

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20
Q

in paget dz of bone, hearing loss is due to

A

auditory foramen narrowing

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21
Q

name the 4 stages of paget dz of bone

A
  1. lytic (osteoclast) 2. mixed (osteoclast + osteoblast) 3. sclerotic (osteoblast) 4. quiescent (minimal osteoclast/osteoblast)
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22
Q

explain the pathophysio of the paget dz of bone

A

common, localized disorder of bone remodeling cause by inc in both osteoblastic and osteoclastic activity

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23
Q

what bone path is associated with mosaic pattern of woven and lamellar bone?

A

paget dz of bone

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24
Q

what is the most common site of osteonecrosis (avascular necrosis) and why?

A

femoral head (due to insufficiency of medial circumflex femoral artery)

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25
Q

name 7 causes for osteonecrosis

A

ASEPTIC 1. alcoholism 2. sickle cell 3. storage dz (Guacher) 4. exogenous steroid 5. Legg - Calve -Perthes 6. trauma 7. idiopathic 8. caisson (the bend)

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26
Q

what is another name for idiopathic cause of osteonecrosis?

A

Legg Calve Perthes disease

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27
Q

what is the pathophysio of achondroplasia?

A

(gain of function), constitutive activation of fibroblast growth factor receptor (FGFR3) inhibiting chondrocyte proliferation

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28
Q

mode of inheritance of achondroplasia?

A

autosomal dominant with full penetrance (homozygosity is lethal)

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29
Q

how is Laron syndrome diff from achondroplasia?

A

small genitalia, dec linear growth, everything small due to defective growth hormone receptor

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30
Q

treatment drugs for type 2 (senile) osteoporosis?

A

bisphosphonate, PTH analog (teriparatide), SERMs, calcitonin, denosumab

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31
Q

what is denosumab?

A

monoclonal antibody against RANKL

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32
Q

what bone path is associated with bone filling marrow space leading to pancytopenia and extramedullary hematopoiesis?

A

osteopetrosis (marble bone dz)

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33
Q

what is defective in osteopetrosis?

A

failure of normal bone resorption due to defective osteoclast leading to thickened, dense bones that are prone to fracture

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34
Q

what mutation is responsible for osteopetrosis?

A

mutations in carbonic anhydrase II impairing the ability of osteoclast to generate acidic environment necessary for bone resorption

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35
Q

what renal path will also have impaired carbonic anhydrase?

A

proximal (type2) urine pH

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36
Q

what is the treatment for osteopetrosis?

A

bone marrow transplant is curative as osteoclast is from monocytes

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37
Q

what path is associated with “bone-in-bone” appearance on x-ray?

A

osteopetrosis

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38
Q

what bone cell secretes acid and collagenases?

A

osteoclast

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39
Q

what cell is multinucleated and is derived from monocyte?

A

osteoclast

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40
Q

what is the diff btw osteoporosis and osteomalacia in terms of bone mineralization?

A

normal bone mineralization in osteoporosis abnormal bone minerailization in osteomalacia (rickets)

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41
Q

4 Rx causes for primary osteoporosis?

A
  1. long term steroid use 2. anticonvulsant 3. anticoagulants 4. thyroid replacement therapy
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42
Q

what symptoms/fractures can you get from osteoporosis?

A

Most commonly fractures from falls with fragile bones - hip, vertebra.

  1. vertebral compression fractures associated with acute back pain, loss of height, kyphosis 2. fractures of femoral neck 3. fractures of distal radius (Colles fractures)
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43
Q

2 bone paths with normal lab values?

A
  1. osteoporosis 2. osteopetrosis
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44
Q

what two bone paths will have the opposite lab values (Ca2+, PO43-, vs PTH/ALP?

A

osteomalacia/rickets and hypervitaminosis Osteomalacia - dec Ca2+, PO4, inc ALP,PTH Hypervitaminosis - inc Ca2+, PO4, dec ALP, PTH

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45
Q

2 causes for hypervitaminosis?

A
  1. oversupplementation 2. granulomatous dz (sarcoidosis)
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46
Q

explain how the lab values will be diff btw 1’ hyperparathyroidism vs 2’

A

1’: high PTH –> high Ca2+ –> low PO43-, high ALP 2’: high PO43- –> low Ca2+ –> high PTH, high ALP

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47
Q

In 2’ hyperparathyroidism, what causes high PO43- conc?

A

usually due to dec PO43- from ESRD

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48
Q

in 2’ hyperparathyroidism, what causes low serum Ca2+?

A

due to low activated vit D due to ESRD

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49
Q

MCP spared a. osteoarthris b. RA

A

a

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50
Q

RA which joint spared

A

DIP

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51
Q

pain improved with rest OA or RA

A

OA

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52
Q

TNF alpha inhibitors for RA List 3

A

etanercept (Enbrel), adalimumab (Humira), infliximab (Remicade), and certolizumab pegol (Cimzia)

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53
Q

what type of glucocorticoids would you give to osteoarthritis?

A

intra-articular

54
Q

list two DMARDS for RA

A

Methotrexate Sulfasalzine hydroxychloroquine, leflunomide

55
Q

most common fractures in osteoporosis type I?

A

femoral neck fracture, distal radius fracture

56
Q

marker of osteoclast activity?

A

urinary OH-proline - breakdown product of collagen

57
Q

Complications of Paget’s disease

A

inc blood flow from AV shunts –> high output heart failure and inc risk of ostogenic sarcoma

58
Q

Paget’s assoc w. with what childhood infection?

A

Paramyxo virus

59
Q

Which marker is abnormal in Paget’s

A

only an inc in ALP

60
Q

Ewing sarcoma derived from what type of cells?

A

neuroectoderm poorly differentiated cells

61
Q

RA association which HLA

A

HLA-DR4

62
Q

what type of hypersensitivity is RA

A

type III IV

63
Q

Heberden nodes in OA

A

He dips DIP

64
Q

Bouchard nodes in OA

A

PIP and CMC

65
Q

Pathology of rheumatoid nodules

A

fibrinoid necrosis with palisading histiocytes

66
Q

most Specific marker for RA

A

anti-cyclic citrullinated peptide antibody

67
Q

what is RF

A

IgM antibody against Fc portion of IgG

68
Q

antibodies of sjogren

A

SSA SSB

69
Q

what are SS-A and SS-B

A

ribonucleoproteins

70
Q

Patient with Sjogren suddenly have unilateral enlargement of parotid gland

A

B cell lymphoma (MALT lymphoma)

71
Q

Patient with acute onset monoarthritis, episodic pain

A

gout

72
Q

what crystals 1. gout 2. pseudogout

A

gout: monosodium urate pseudogout: calcium pyrophoshate (rhomboid)

73
Q

what type of joints do pseudogout affect

A

large joints, like the knee

74
Q

what type of diuretics can cause gout

A

Loops and Thiazides - hyperuricemia

75
Q

glycogen storage disease that cause gout

A

von gierke

76
Q

negative birefringent what color

A

yellow parallel (BLUE parallel in pseudo) blue perpendicular

77
Q

diseases associated with pseudogout

A
  • hemachromatosis
  • hyperparathyroid
  • joint trauma
78
Q

infectious arthritis in sexually active

A

n. gonorrheae

79
Q

Infective Arthritis in older kids and adults

A

s. aureus

80
Q

Gonococal arthrtis presentation

A

migratory arthritis with asymmetric pattern STD synovitis tenosynovitis dermatitis

81
Q

Seronegative spondyloarthropathies what HLA

A

HLA-B27

82
Q

sausage fingers pencil in a cup deformities on a Xray

A

psoriatic arthritis

83
Q

vertebral body fusion

A

ankylosing spondylitis

84
Q

see Aortic Regurg in which type of Seronegative arthritis

A

ankylosing spondylitis

85
Q

conjunctivitis, urethritis, arthritis

A

Reactive arthritis reiter

86
Q

cause of reactive arthritis

A

Post GI: shigella, salmonella, Yersinia, Campylo Chlymadia

87
Q

Circinate balanitis

A

annular dermatitis of glan penis seen in reiter

88
Q

keratoderma blennorrhagicum

A

hyperkeratotic vesicles in palms and soles reiter

89
Q

SLE die with what type of kidney disease

A

nephritic DPGN

90
Q

most sensitive SLE test

A

ANA

91
Q

most specific SLE test

A

dsDNA, anti-smith

92
Q

what is anti-Smith antibodies

A

Anti-snRNPs for splicing

93
Q

drug induced SLE what antibodies

A

anti-histone

94
Q

cause of drug-induced SLE

A

Methyldopa, Minocycline, Hydralazine, Isoniazid, Phenytoin, Sulfa drugs, Etanercept, Procainamide

95
Q

antibodies in antiphospholipid syndrome

A

lupus anticog anticardiolipin anti-B2 glycoprotein

96
Q

false positive VDRL

A

Viral infection (eg, EBV, hepatitis) Drugs (IV) Rheumatic fever Lupus and leprosy. Pregnancy

97
Q

lupus anticog produce paradoxical prolongation of PT or aPTT

A

PTT

98
Q

List the joint findings in osteoarthritis (6)

A
  1. Osteophytes (bone spurs) 2. joint space narrowing 3. subchondral sclerosis and cysts 4. eburnation (polished ivory like appearance of bone) 5. Heberden nodes 6. Bouchard nodes Synovial fluid non-inflammatory (WBC
99
Q

this marker in SLE indicate poor prognosis

A

anti-dsDNA

100
Q

MCP spared a. osteoarthris b. RA

A

osteoarthritis

101
Q

RA + pneumoconiosis

A

Caplan syndrome

102
Q

A 40 yo female comes in with a Hx of dyspnea especially after climbing stairs. HR and pulse are normal. After doing pulmonary function test, results show an elevated FEV1/FVC ratio. She also has a slight facial droop that has been worsening over the months. What pathology is associated with these symptoms, and what findings are prominent on imaging?

A

The pt has Sarcoidosis and hilar nodes are prominent on CXR known as potato sac lymph adenopathy bilaterally. The facial droop is due to Bell’s palsy.

103
Q

A physician orders a biopsy for an African descent patient with positive HX for Sarcoidosis. What will be found in the biopsy (appearance and inclusions)?

A

Epithelioid granulomas containing Asteroid and Schuamann bodies

104
Q

A physican orders lab test for a 40 yo female with interstitial fibrosis suspected of Sarcoidosis. What metabolites will be elevated and the mechanism behind the pathology

A

Hypercalcemia. Sarcoidosis causes increased activation 1 alpha hydroxylase in the kidney. This mediates Vitamin D activation and leads to increased Ca reabsorption

105
Q

A 60 yo female comes into the ER complaining of severe pain in her hips. She previously stated that she has been having intermittent pain in her temples What disease is she affected by at the moment? Which lab results will be found?

A

Polymyalgia Rheumatica –> Assoc with GCA. Lab reults will show elevated ESR, CRP.

106
Q

A 40 yo female comes to her family physician complaining of pain and stiffness in her muscles. She also feels lethargic during the day, and is having trouble sleeping at night. What Dx do the physician make and what does he prescribe?

A

Fibromyalgia TX with SNRI/TCA and anti-convulsants and regular exercise

107
Q

A patients come in a malar rash and redness on her hands. The physician also notices that the patient’s hands look roughed up as if she has been working outside with her hands. What is the Dx and TX?

A

Dermatomyositis with perimysial inflammation from CD4 Th cells. Tx with corticosteroids + MTX

108
Q

Immunopathologically how does Polymyositis differ from dermatomyositis?

A

Polymyositis- Cd8 +CTL induced endomysial inflammation Dermatomyositis - CD4+ Th cells induced permysial inflammation

109
Q

A 40 yo female comes in with a Hx of dyspnea especially after climbing stairs, and has lost 10 lbs of weight in the last 3 months. HR and pulse are normal. She also feels tired easily and feels weakness in washing dishes in the morning and prefers to do them at night time. What pathology is associated with these symptoms, and what underlying mechanism is associated with it?

A

Patient has Lambert Eaton syndrome where the Abs are destroying her presynaptic Ca Ch and decrease ACh release in the NMJ. LE syndrome is associated with Small CC of the lung which explains the dyspnea and cachexia

110
Q

How does Myasthenia Gravis differ from LE with regards to muscle movement? Why?

A

Muscle weakness worsens as the day progresses b/c Abs are against the AcH receptor. As the day ends the ACh receptors decrease which causes increased weakness later.

111
Q

A 45 yo female comes into her physician and complains of increased tightness and puffiness in her skin. She also shows that fingertips around her nails feel rougher than usual. What is the physician worried about after confirming his DX with these lab results to be positive?

A

The patient is positive for anti topoisomerase I Ab (SCl-70) as and has Diffuse systemic Scleroderma. These patients have a high mortality rate with renal and pulmonary sclerosis.

112
Q

Name the findings in Limited Scleroderma (5 S/S) and the Ab associated with the path?

A

Limited Scleroderma is associated with anti centromere AB CREST syndrome 1) Calcinosis 2) Raynaud’s phenomenon 3) Esophageal dysmotility 4) Sclerodactyly 5) Telengectasia

113
Q

Genetic issue with Ewing sarcoma?

A

t(11;12) translocation causing fusion protein EWS-FLI 1

114
Q

Rx associated with Osteoporotic fractures?

A
  • anticonvulsants that (+) p450 (phenobarbital, phenytoin, carbamazepine) - inc Vit D catabolism
  • Aromatase (-)’rs/Hydroxyprogesterone - Dec estrogen
  • GnRH (+)’r - dec testosterone/estrogens
  • PPI - dec Ca absorption
  • Glucocorticoids,
  • Unfractionated heparin,
  • Thiazolidindiones - dec bone formation
115
Q

bony mass with radiolucent core, nighttime pain, cental nidus

A

osteoid osteoma

116
Q

Causes of uric acid overproduction

A

Lesch Nyhan, PRPP excess, inc cell turnover (tumor lysis syndrome), von Gierke disease, dec G6Pase

117
Q

which gout rx works by (-) microtubule polymerization?

A

colchicine

118
Q

locations of tophus formation?

A

external ear, olecranon bursa, achilles tendon

119
Q

Rx that inc uric acid formation

A

Pyrazinamide, Thiazides, Furosemide, Niacin, Cyclosporin

120
Q

rhomboid crystals that are birefrigent?

A

calcium pyrophosphate deposition disease (pseudo gout)

121
Q

invasion of exocrine glands by lymphocytes?

A

sjogrens (why has dry eyes and mouth)

122
Q

migratory arthritis seen with infectious arthritis and what other illness?

A

Lyme disease

123
Q

Decrease of what immune components with SLE?

A

C3, C4, CH50 - due to immune complex formation

124
Q

associated with anti U1 RNP antibodies

A

mixed CT disease (speckled ANA)

125
Q

increased serum ACE levels and CD4+/CD*+ ration in bronchoalveolar lavage fluid?

A

Sarcoidosis

126
Q

why is there hypercalcemia with sarcoidosis/.

A

due to inc alpha hydroxylase mediated vitamin D (+)’n in macrophages

127
Q

why can polymyalgia rheumatica present with pain in shoulders and hips, as well as temporal pain?

A

associated with temporal arteritis (giant cell)

128
Q

markers with polymyositis/dermatomyosititis?

A

Inc CK, pos for ANA, anti-Jo1, anti-SRP, and anti-Mi2

129
Q

red, sometimes scaly, papules that erupt on any of the finger joints, made worse by sunlight exposure?

A

Gottron papules of Dermatomyositis

130
Q

What is Raynaud’s?

A

dec blood flow to the skin due to arteriolar vasospasm in response to cold or stress, often in fingers and toes

131
Q

How to treat Raynaud’s?

A

CCB

132
Q

triad of autoimmunity + noninflammatory vasculopathy + collagen deposition with fibrosis?

A

Scleroderma

Step 1 2019 First Aid Flashcards (63 decks)

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